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Qu es la Parlisis Supranuclear Progresiva?

La Parlisis Supranuclear Progresiva (PSP) es un raro desorden del cerebro que ocasiona graves y
permanentes problemas con el control de la forma de caminar y el equilibrio. El signo ms evidente
de la enfermedad es la incapacidad de orientar los ojos de forma adecuada, que ocurre debido a
las lesiones en el rea del cerebro que coordina los movimientos de los ojos. Algunos pacientes
describen este efecto como visin borrosa. Los pacientes de PSP presentan a menudo
alteraciones del humor y el comportamiento, entre ellas la depresin y la apata, as como
demencia leve progresiva.
El nombre largo del desorden indica que la enfermedad comienza lentamente y contina
empeorando (progresiva), ocasiona dao en el cerebro por encima de ciertas estructuras del
tamao de un guisante llamadas ncleos (supranuclear) y produce parlisis. Sin embargo, los
pacientes de PSP no experimentan una verdadera parlisis.
La Parlisis Supranuclear Progresiva (PSP) fue descrita inicialmente como desorden distintivo en
1964, cuando tres cientficos publicaron una ponencia que distingua la condicin de la enfermedad
de Parkinson. A veces se aludi a esta condicin como Sndrome de Steele-Richardson-Olszewski,
reflejando los nombres combinados de los cientficos que definieron el desorden. An cuando la
Parlisis Supranuclear Progresiva (PSP) empeora progresivamente, nadie muere de ella.
Quin adquiere la Parlisis Supranuclear Progresiva (PSP)?
Aproximadamente 20,000 norteamericanos tienen PSP, lo que la hace mucho menos comn que la
enfermedad de Parkinson que afecta a ms de 500,000 norteamericanos. Los pacientes tienen
edad mediana o son ancianos y los hombres se ven ms frecuentemente afectados que las
mujeres. La PSP es a menudo difcil de diagnosticar debido a que sus sntomas se asemejan
mucho a los de otros desrdenes del movimiento ms comunes y debido a que algunos de los
sntomas ms caractersticos pueden desarrollarse posteriormente en la vida o no desarrollarse en
absoluto.
Cules son los sntomas?
El primer sntoma ms frecuente de la PSP es la prdida de equilibrio al andar. Los pacientes
tienen cadas inexplicadas o rigidez y dificultad en el caminar. A veces las personas que
experimentan las cadas las describen como ataques de mareos. Esto lleva a menudo a sospechar
un problema del odo interior.
Otros sntomas iniciales comunes son cambios de personalidad tales como prdida de inters en
actividades ordinariamente agradables o una mayor irritabilidad, malhumor y olvido. Los pacientes
pueden rer o llorar repentinamente sin razn aparente, pueden ser apticos o pueden tener
explosiones de coraje ocasionalmente, tambin sin razn aparente. Hay que subrayar que el patrn
de signos y sntomas puede ser bastante diferente de una persona a otra.
A medida que progresa la enfermedad, la mayora de los pacientes comienzan a desarrollar una
visin borrosa y a tener problemas en controlar el movimiento de los ojos. En realidad, los
problemas visuales ofrecen generalmente la primera indicacin definitiva de que la PSP es el
diagnstico apropiado. Los pacientes de PSP tienen problemas en cambiar voluntariamente la
mirada hacia abajo y tambin pueden tener problemas en controlar los prpados. Esto puede
conducir al cierre involuntario de los ojos, a parpadeos prolongados o involuntarios o a una
dificultad en abrir los ojos.

Otro problema visual comn es la incapacidad de mantener contacto visual durante una
conversacin. Esto puede dar la impresin errnea de que el paciente es hostil o no est
interesado.
La persona usualmente muestra dificultades al hablar y se le hace difcil tragar alimentos slidos o
lquidos. En casos raros, algunos pacientes advertirn temblores en las manos.
Qu ocasiona la Parlisis Supranuclear Progresiva (PSP)?
Sabemos que los sntomas de la PSP son ocasionados por el deterioro paulatino de las clulas
cerebrales en unos cuantos lugares diminutos pero importantes de la base del cerebro, en la regin
llamada tallo enceflico. Una de estas reas, la sustancia nigra, tambin es afectada en la
enfermedad de Parkinson y el dao a esta regin del cerebro explica los sntomas motores que
tienen en comn la PSP y la enfermedad de Parkinson.
Los cientficos no saben qu ocasiona la degeneracin de estas clulas cerebrales. No existen
pruebas de que la PSP sea contagiosa y los factores genticos no se han visto implicados. Ningn
grupo tnico o racial ha sido afectado ms frecuentemente que otro y la PSP no tiene mayores
probabilidades de ocurrir en algunas zonas geogrficas que en otras.
Hay, sin embargo, varias teoras acerca de la causa de la PSP. Una posibilidad es la de que un
agente no convencional similar a un virus infecta al cuerpo y lleva aos o dcadas para comenzar a
producir efectos visibles. La enfermedad de Creutzfeldt-Jakob es una enfermedad que se sabe es
ocasionada por dicho agente. Otra posibilidad es la de que las mutaciones genticas fortuitas, del
tipo que ocurren en todos nosotros en todo momento, ocurren accidentalmente en determinadas
clulas o en ciertos genes, en la combinacin adecuada para lesionar estas clulas. Una tercera
posibilidad es la de que hay exposicin a algn producto qumico desconocido en los alimentos, la
atmsfera o el agua que lentamente daa ciertas zonas vulnerables del cerebro. Esta teora emana
de una pista hallada en la Isla de Guam en el Pacfico donde una enfermedad neurolgica comn
que ocurre slo all y en unas cuantas islas vecinas comparte algunas de las caractersticas de la
PSP, la enfermedad de Alzheimer, la enfermedad de Parkinson y la esclerosis lateral amiotrfica
(enfermedad de Lou Gehrig). Se considera que su causa es un factor diettico o una sustancia
txica hallada slo en dicha zona.
Otra causa probable de la PSP es el dao celular ocasionado por radicales libres, molculas
reactivas producidas continuamente por todas las clulas durante el metabolismo normal. Aun
cuando el cuerpo tiene mecanismos integrales para eliminar los radicales libres del sistema, los
cientficos sospechan que, en ciertas circunstancias, los radicales libres pueden reaccionar y daar
a otras molculas. Una gran cantidad de investigacin est dirigida a comprender el papel del dao
por los radicales libres en las enfermedades humanas.
Cmo se diagnostica la Parlisis Supranuclear Progresiva (PSP)?
Las quejas iniciales en la PSP son tpicamente vagas y siempre se le hace difcil al mdico hacer
un diagnstico temprano. Las quejas principales caen dentro de estas categoras: 1) sntomas de
desequilibrio, tales como caminar inseguro o cadas abruptas o inexplicadas sin prdida de
conciencia; 2) quejas visuales, entre ellas visin enturbiada, dificultades en mirar hacia arriba o
hacia abajo, visin doble, sensibilidad a la luz, quemazn en los ojos y otros problemas visuales; 3)
dificultad al hablar; y 4) distintas quejas mentales tales como lentitud del pensamiento, falta de
memoria, cambios de personalidad y cambios de humor.
La Parlisis Supranuclear Progresiva (PSP) es diagnosticada errneamente a menudo debido a
que algunos de sus sntomas son muy similares a los de la enfermedad de Parkinson, la
enfermedad del Alzheimer y desrdenes neurodegenerativos ms raros tales como la enfermedad

de Creutzfeldt-Jakob. En realidad, la PSP es ms frecuentemente diagnosticada errneamente


como enfermedad de Parkinson al principio de la enfermedad. Los problemas de memoria y los
cambios de personalidad tambin pueden llevar a un mdico a confundir la PSP con depresin o
incluso atribuir los sntomas a alguna forma de demencia. La clave para establecer el diagnstico
de la PSP es la identificacin de inestabilidad temprana al caminar y la dificultad en mover los ojos,
distintivo de la enfermedad, as como descartar otros desrdenes similares, algunos de los cuales
son tratables.
Cmo difiere la PSP de la enfermedad de Parkinson?
Tanto la PSP como la enfermedad de Parkinson ocasionan falta de flexibilidad, dificultades del
movimiento y torpeza. Sin embargo, los pacientes con PSP tienen comnmente una postura
erguida y ocasionalmente incluso inclinan la cabeza hacia atrs (y tienden a caer de espaldas),
mientras que los que sufren la enfermedad de Parkinson se inclinan generalmente hacia adelante.
Los problemas relacionados al habla y con tragar son mucho ms comunes y agudos en la PSP
que en la enfermedad de Parkinson y tienden a presentarse antes en el curso de la enfermedad.
Ambas enfermedades tienen en comn otras caractersticas: comienzan a fines de la edad
mediana, bradicinesia (lentitud de movimiento) y rigidez muscular. El temblor, casi universal en los
pacientes de Parkinson, es raro en la PSP. Aunque los pacientes de Parkinson se benefician
marcadamente del medicamento levodopa, los pacientes de PSP responden deficientemente y slo
de forma transitoria a este medicamento.
Cul es el pronstico de la Parlisis Supranuclear Progresiva?
La PSP empeora progresivamente pero no pone de por s en peligro la vida. S predispone, no
obstante, a los pacientes a graves complicaciones tales como a la pulmona secundaria y a la
dificultad de tragar (disfagia). Las complicaciones ms comunes son el atragantamiento y la
pulmona, la lesin en la cabeza y fracturas ocasionadas por las cadas. La causa ms comn de la
muerte es la pulmona. Con buena atencin a las necesidades mdicas y nutricionales, es posible,
que la mayora de los pacientes de PSP vivan ms de una dcada despus de haber sentido los
primeros sntomas.
Hay algn tratamiento?
Actualmente no hay tratamiento eficaz para la PSP, aunque los cientficos estn investigando
formas mejores de controlar la enfermedad. En algunos pacientes, la lentitud, la falta de flexibilidad
y los problemas de equilibrio de la PSP pueden responder a agentes contra el Parkinson tales
como la levodopa, pero el efecto es generalmente temporero. Las dificultades al hablar, tragar y de
la visin no responden comnmente a ningn tratamiento a base de medicamentos.
Otro grupo de medicamentos que ha tenido algn xito modesto en la PSP son los medicamentos
antidepresores. Los ms comnmente utilizados son la fluoxetina (Prozac), la amitriptilina (Elavil) y
la imipramina (Tofranil). El beneficio contra la PSP de estos medicamentos parece no estar
relacionado con su capacidad de aliviar la depresin.
El tratamiento no medicamentoso para la PSP puede adoptar muchas formas. Algunos pacientes
utilizan pesas para ayudarles a caminar debido a su tendencia a caer hacia atrs. A veces se
prescriben para los pacientes de PSP lentes bifocales o especiales llamados prismas para
remediar la dificultad de mirar hacia abajo. La terapia fsica formal no proporciona beneficio
demostrado en la PSP, pero pueden efectuarse ciertos ejercicios para mantener las articulaciones
flexibles.
Un procedimiento quirrgico que puede ser necesario cuando se producen perturbaciones de la
accin de tragar es la gastrostoma. Una gastrostoma (o una jejunostoma) es un procedimiento

mnimamente invasivo que se realiza cuando el paciente tiene dificultad en tragar o cuando el
atragantamiento agudo es un claro riesgo. Esta ciruga entraa la colocacin de un tubo a travs
de la piel del abdomen hasta el estmago (intestino) para alimentar al paciente. Palidotoma y otros
procedimientos quirrgicos sometidos a prueba en los tratamientos contra la enfermedad en
pacientes con Parkinson's han probado no ser eficaces en la PSP.
Qu investigacin se est realizando?
En fecha reciente se han realizado estudios para mejorar el diagnstico de la PSP en el Instituto
Nacional de Trastornos Neurolgicos y Accidentes Cerebrovasculares (NINDS- National Institute of
Neurological Disorders and Stroke). En la actualidad se estn llevando a cabo experimentos para
identificar la causa o causas de la PSP. Se tiene proyectado realizar en el futuro ensayos
teraputicos con agentes limpiadores de radicales libres (agentes que pueden liberar al cuerpo de
radicales libres potencialmente nocivos).
Adems, hay una gran cantidad de investigacin en curso sobre las enfermedades de Parkinson y
Alzheimer en los Institutos Nacionales de Salud (NIH) y en los centros mdicos universitarios de
todo el pas. Una mejor comprensin de esos desrdenes, los cuales tienen caractersticas
comunes, contribuir grandemente a resolver el problema de la PSP, al igual que el estudio de la
PSP puede ayudar a esclarecer las enfermedades de Parkinson y Alzheimer.

Parlisis supranuclear
progresiva
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INTRODUCCIN
La parlisis supranuclear progresiva (PSP) es un trastorno neurodegenerativo que se caracteriza
clnicamente por un cuadro progresivo de parkinsonismo con alteraciones de la mirada de origen
supranuclear, deterioro cognitivo y una marcada inestabilidad postural con cadas.
La primera descripcin clnico-patolgica detallada fue realizada por Steele, Richardson y el
patlogo Olszewski (Steele et al 1964), por lo que inicialmente esta enfermedad fue conocida como
sndrome de Steele-Richardson-Olszewski, aunque existen otros casos descritos clnicamente con
anterioridad.

EPIDEMIOLOGA
La PSP es el parkinsonismo atpico ms frecuente. Su prevalencia ajustada por edad se estima
que se encuentra entre 5-6 casos/100000 habitantes, lo que supone aproximadamente un 5-10%
de la prevalencia de la enfermedad de Parkinson idioptica (Schrag et al 1999; Nath et al 2001;
Litvan I 2003). Sin embargo se piensa que estas cifras son inferiores a las reales por las
dificultades diagnsticas que presenta esta enfermedad.

No existen aparentes diferencias de gnero y la mayora de casos son espordicos aunque existen
descritos alguna minora de pacientes con presentacin familiar.
La mayora presentan los sntomas iniciales de la enfermedad en la sptima dcada de la vida
(media de presentacin 63 aos) y no han sido descritos casos en menores de 40 aos, siendo un
criterio de exclusin presentaciones por debajo de esa edad segn los criterios diagnsticos
actuales (Ver Criterios diagnsticos para PSP.jpg).

HALLAZGOS PATOLGICOS
La PSP se clasifica anatomo-patolgicamente como una taupata. El principal hallazgo histolgico
es la presencia de acmulos de protena tau (fundamentalmente isoformas de 4R) formando ovillos
neurofibrilares, junto con prdida neuronal y gliosis en regiones de los ganglios basales y el
mesencfalo. Estos hallazgos son ms intensos y sistemticos en la sustancia negra, el ncleo
subtalmico, el globo plido, el rea pretectal y los colculos superiores.
La presencia de cuerpos de Lewy, inclusiones argirfilas, cuerpos de Pick, alteraciones
espongiformes o placas de amiloide positivas para protena prinica son criterios histolgicos de
exclusin para el diagnstico de PSP (Hauw et al 1994).
Estos hallazgos histolgicos no son patognomnicos y alteraciones similares se pueden encontrar
en otras patologas (principalmente otros parkinsonismos atpicos muy infrecuentes como el
postencefaltico y los parkinsonismos atpicos de Guam y Guadalupe) aunque combinados con la
informacin clnica tienen una gran fiabilidad y se consideran actualmente el gold standard
diagnstico (Litvan et al 1996a).

ETIOPATOGENIA
La etiopatogenia de la PSP permanece desconocida y aunque se han realizado avances en los
ltimos aos acerca de posibles mecanismos fisiopatolgicos el papel de muchos de ellos est an
por aclarar.
- factores ambientales: el consumo de ciertas frutas tropicales y ts se ha relacionado con el
desarrollo de cuadros de parkinsonismo atpico similares a la PSP en la isla de Guadalupe
(Caparros-Lefebvre et al 1999). Parece que la sustancia responsable sera la annonacina, una
molcula que producira alteraciones a nivel del complejo I de la cadena respiratoria (Lannuzel et al
2003).
- alteraciones mitocondriales: existen diferentes evidencias de que alteraciones en el
funcionamiento del complejo I mitocondrial podran tener un papel en la patogenia de la PSP
(Albers et al 2002). Esto llevara a un proceso de estrs oxidativo que provocara la acumulacin de
la protena tau y en ltima instancia la muerte celular.
- factores genticos: dadas las caractersticas histopatolgicas de la PSP los esfuerzos genticos
se han orientado en el estudio del gen de la protena tau. Existen 6 isoformas diferentes de la

protena que se clasifican en dos grupos en funcin de si el dominio de unin a los microtbulos
presenta tres repeticiones (isoforma 3R) o cuatro (isoforma 4R). En los individuos normales existe
una relacin similar de ambas isoformas, sin embargo en individuos con PSP y tambin en la
degeneracin corticobasal, la proporcin de la isoforma 4R es muy superior.
Mutaciones en el gen de la protena tau se han relacionado con otras taupatas (fundamentalmente
el cuadro de demencia fronto temporal-parkinsonismo ligado al cromosoma 17) y se han descrito
una minora de casos familiares de cuadros de parkinsonismo atpico similares a la PSP que se
deben a mutaciones en este gen. Sin embargo, la verdadera influencia gentica de la PSP est
an por aclarar, y en los casos espordicos no parece ser ms que un leve factor predisponente
(Ludolph et al 2009).

MANIFESTACIONES CLNICAS
La PSP produce clnicamente varios sntomas y signos, algunos de ellos muy caractersticos, y que
presentes conjuntamente hacen relativamente fcil el diagnostico. Sin embargo al inicio muchos de
ellos pueden no estar presentes o tener la enfermedad presentaciones mas atpicas, por lo que en
ocasiones el diagnostico precoz es muy difcil y solo se consigue varios aos despus del inicio de
los sntomas. Los principales signos y sntomas son:

- inestabilidad postural y cadas: es el sntoma de presentacin ms frecuente en la mayora de los


pacientes (Nath et al 2003) y se debe a una combinacin de bradicinesia y rigidez, alteracin de los
reflejos posturales y alteraciones en la motilidad ocular. Es tambin uno de los sntomas ms
incapacitantes y produce de forma precoz cadas (segn los criterios diagnsticos en el primer ao
de la enfermedad, aunque algunos individuos lo hacen ms tarde) que suelen ser hacia atrs. A
ello contribuye el hecho de que los pacientes no tienen conciencia de su falta de equilibrio y se
mueven sin precaucin pese a las mltiples cadas. Esto en ocasiones se hace evidente a la
exploracin mediante el signo del cohete (al pedir al paciente que se levante de la silla, ste se
pone bruscamente en pie para volver a caer inmediatamente sentado). Con el sujeto en pie y el
examinador a su espalda, mediante un empujn brusco desde los hombros hacia atrs (estando
previamente el paciente avisado de la maniobra) se puede explorar la alteracin de los reflejos
posturales. Los individuos con PSP necesitarn mltiples pasos hacia atrs para corregir la
posicin o incluso caern directamente hacia atrs como un rbol. Esta inestabilidad postural se
puede observar en pacientes con enfermedad de Parkinson pero siempre en estadios tardos de la
enfermedad.

- alteraciones oculares: no suelen ser frecuentes los sntomas oculares aunque durante el curso de
la enfermedad pueden aparecer quejas de diplopia, visin borrosa o fotofobia debidos a varios
factores (reduccin del parpadeo, dificultad para enfocar, alteraciones oculomotoras,). Sin
embargo, uno de los hallazgos exploratorios ms conocidos de la PSP es la alteracin de los
movimientos sacdicos oculares en el plano vertical aunque al comienzo puede ser sutiles y pasar
desapercibidos, e incluso en algunos pacientes puede no aparecer a lo largo del curso de la
enfermedad. En las fases iniciales, a la exploracin se puede observar un enlentecimiento en las

sacadas verticales (no en los movimientos de seguimiento) que se explora pidiendo al paciente que
mire hacia arriba y hacia abajo. Se considera que las sacadas oculares estn enlentecidas cuando
el explorador es capaz de ver el desplazamiento de los ojos del paciente en el plano vertical y no
simplemente que los ojos pasan de una posicin a otra. Este enlentecimiento es progresivo hasta
producir una limitacin en la mirada vertical, mientras que los movimientos en el plano horizontal
suelen estar respetados. Una limitacin en la supraversin de la mirada se puede encontrar en
sujetos sanos de cierta edad pero la limitacin en la infraversin siempre va a ser un hallazgo
patolgico. Estas alteraciones tienen un origen supranuclear, lo que quiere decir que se deben a
una alteracin de las estructuras superiores que controlan los movimientos oculares y no a
alteraciones de los nervios o los ncleos que se encargan de la motilidad ocular. Esto se
demuestra en la exploracin comprobando que la limitacin en la mirada vertical se corrige con los
movimientos culo-ceflicos (se pide al paciente que mantenga la mirada al frente en un punto fijo
y realizamos movimientos en flexin y extensin de la cabeza del paciente para ver que no existe
limitacin de la mirada vertical)

- parkinsonismo: los pacientes con PSP desarrollan un cuadro de bradicinesia y rigidez cuyas
principales caractersticas son el predominio axial (en el tronco y en el cuello), su distribucin
simtrica y la presencia muy infrecuentemente de temblor de reposo. Esto va a permitir diferenciar
el cuadro de la enfermedad de Parkinson, donde el temblor de reposo es un hallazgo frecuente y la
rigidez suele predominar en los miembros sobre el tronco y con una distribucin asimtrica.

- alteraciones cognitivas y del comportamiento: suelen estar presentes desde las fases iniciales de
la enfermedad aunque al principio pueden ser muy sutiles. (LINK DEMENCIAS)
Un signo relativamente especifico presente en estos pacientes a la exploracin es el signo del
aplauso (Dubois et al 2005) en el que el medico realiza tres aplausos y pide al paciente que haga
exactamente lo mismo. Estos pacientes, debido a la falta de planificacin y la desinhibicin motora,
continan aplaudiendo ms de tres veces.

- sintomas bulbares: alteraciones del habla en forma de disfona (con una voz similar a un gruido)
y disartria, junto con el desarrollo posterior de disfagia son frecuentes en el curso de la
enfermedad.

- expresin facial y aspecto general: los pacientes con PSP tienen una expresin facial
caracterstica que les da una apariencia de asombrados o asustados por la combinacin de
hipomimia facial, con disminucin del parpadeo, y retraccin palpebral con hiperactividad del
msculo frontal. Otras alteraciones que pueden presentar son blefaroespasmo y apraxia de la
apertura palpebral que pueden producir una gran limitacin visual. Adems la rigidez axial que
presentan estos pacientes puede estar combinada con distona cervical en forma de retrocollis lo
que les confiere una postura peculiar.

OTRAS FORMAS CLNICAS


La forma clnica arriba detallada, inicialmente descrita por Steele, es la forma de presentacin ms
frecuente, representando en torno al 50% de los casos (Williams et al 2005), y se la conoce como
forma clsica o sndrome de Richardson. Sin embargo, recientemente se han descrito otros
sndromes clnicos muy diferentes respecto a la forma clsica y que presentan cambios
histolgicos diagnsticos de PSP. Estas variantes clnicas en cierta medida se podran explicar por
la variacin en la distribucin y la severidad de los hallazgos patolgicos en las diferentes reas del
sistema nervioso central (Williams et al 2009).
La variante ms conocida y ms frecuente tras la forma clsica, con aproximadamente un 30% de
los casos, es la forma PSP-parkinsonismo (PSP-P). En ella el parkinsonismo domina el cuadro,
presentando los pacientes bradicinesia y rigidez tanto axial como de los miembros con una buena
respuesta a levodopa, aunque sta no suele ser intensa ni prolongada (Williams et al 2005). Los
pacientes pueden presentar temblor pero suele ser postural e irregular. Por todas estas
caractersticas, estos pacientes pueden ser inicialmente diagnosticados de forma errnea de
enfermedad de Parkinson. Los hallazgos que los diferencian incluyen la rpida progresin, la
importante afectacin axial y la incompleta respuesta a levodopa. Inicialmente estos pacientes no
presentan alteraciones oculares, cadas, alteraciones de la estabilidad postural o alteraciones
cognitivas, lo que les diferencia de la forma clsica de PSP. Sin embargo con el paso de los aos,
ambos cuadros clnicos se van solapando y su distincin puede ser ms difcil. La PSP-P tiene un
curso ms benigno que la forma clsica con una progresin ms lenta y una esperanza de vida
mayor.
Otras variantes clnico-patolgicas minoritarias descritas (Williams et al 2009) presentan
alteraciones de la marcha con congelacin (freezing), micrografia e hipotona (PSP con akinesia y
freezing de la marcha), distona, apraxia y alteraciones sensitivas corticales de distribucin
asimtrica similares a la degeneracin crtico-basal (sndrome PSP-corticobasal) o apraxia del
lenguaje (PSP con afasia progresiva no fluente).

DIAGNSTICO
El diagnstico de la PSP contina siendo clnico, permaneciendo como gold standard el examen
anatomopatolgico. Existen unos criterios clnicos universalmente aceptados (Litvan et al 1996b)
basados en casos confirmados mediante autopsia que clasifican el grado de certeza diagnstica en
definitivo (confirmacin histopatolgica de un caso clasificado clnicamente como posible o
probable), probable o posible [Criterios diagnsticos para PSP.jpg]. As mismo, existen unos
criterios de exclusin y otros criterios que apoyan el diagnstico de PSP si estn presentes. Sin
embargo estos criterios fueron creados como una herramienta uniforme para la seleccin de
pacientes en ensayos clnicos debido a su gran especificidad, por lo que su utilidad en la prctica
clnica es escasa.
Lamentablemente no hay ningn test diagnstico para la PSP aunque existen distintas pruebas
complementarias que pueden mostrar datos que apoyen el diagnstico o, sobre todo, que
descarten otras causas secundarias. Los resultados de los anlisis rutinarios en sangre, orina y
lquido cefalorraqudeo son normales.

- Resonancia magntica (RM): se debe realizar ante todo paciente con sospecha de PSP, ya que
va a permitir descartar otras causas secundarias (tumores, hidrocefalia, enfermedad vascular,) y
en algunas ocasiones, encontrar otros hallazgos que apoyen el diagnstico. Se puede objetivar una
marcada atrofia del mesencfalo y los pednculos cerebrales respecto a la protuberancia que
algunos autores han propuesto como marcadores radiolgicos de PSP (signo del colibr o del
pingino en cortes sagitales y signo de Mickey Mouse en cortes axiales), junto con aumento de
seal en el mesencfalo, globo plido y ncleo rojo. Sin embargo estos hallazgos no han sido
evaluados en las fases precoces de la enfermedad por lo que su valor esta an por determinar
(Seppi et al 2010).

- PET y SPECT: los estudios moleculares de imagen mediante PET y SPECT suelen mostrar una
reduccin del sistema dopaminrgico tanto a nivel presinptico (transportador de dopamina-DAT)
como a nivel postsinptico (receptor de dopamina D2). Estos estudios son tiles para diferenciar la
PSP de la enfermedad de Parkinson, donde slo existe un dficit presinptico, pero sin embargo no
permite diferenciarla de otros parkinsonismos degenerativos (atrofia multisistema, degeneracin
corticobasal) donde tambin existe un dficit postsinptico aadido (Kagi et al 2010, Tatsch 2010).

- Ecografa transcraneal: en los ltimos aos se est generalizando el uso de la ecografa


transcraneal para la visualizacin del parnquima cerebral por su rapidez, disponibilidad y escaso
coste. La presencia de hiperecogenicidad en el ncleo lenticular y normalidad de la sustancia negra
es un patrn sugerente de PSP o la variante parkinsoniana de la atrofia multisistema, y permite
diferenciarlas de la enfermedad de Parkinson, donde existe una hiperecogenicidad de la sustancia
negra sin alteraciones de seal en los ganglios basales (Berg et al 2008). Sin embargo la tcnica
tiene varios inconvenientes, como son la ausencia de ventana sea en un 10% de sujetos y la gran
dependencia de los resultados en el explorador.

DIAGNSTICO DIFERENCIAL
Al inicio de la enfermedad, cuando todo el cuadro clnico de la PSP todava no esta desarrollado, y
especialmente en las formas PSP-P, el diagnostico puede ser complicado con otras entidades
[Diagnostico diferencial de parkinsonismo.jpeg] (Edwards et al 2008). Las otras entidades ms
frecuentemente relacionadas con estos errores diagnsticos son:
- enfermedad de Parkinson: es la enfermedad que ms frecuentemente se diagnostica de forma
errnea, sobre todo con las formas PSP-P. La marcada afectacin axial, la rpida progresin de la
enfermedad y la incompleta respuesta a levodopa junto con la demostracin mediante pruebas de
imagen molecular de un defecto dopaminrgico postsinptico ayudan a su diferenciacin.
- atrofia multisistema: en ambas enfermedades existen formas clnicas en las que predomina el
parkinsonismo, por lo que pueden ser confundidas inicialmente. La PSP no presenta disautonoma
y las alteraciones oculomotoras suelen ser ms marcadas.

- degeneracin corticobasal: presenta una marcada asimetra de los sntomas y alteraciones


aprxicas y sensitivas corticales.
- enfermedad por cuerpos de Lewy: suele presentar hallazgos claros de deterioro cognitivo con
marcadas alucinaciones no relacionadas con medicacin.
- enfermedad cerebrovascular: inicio y deterioro de la enfermedad bruscos, presencia de infartos
en RM que afectan al tronco del encfalo o los ganglios basales, presencia de factores de riesgo
vascular, hallazgos de focalidad neurolgica a la exploracin.
Otras entidades pueden compartir algunas de las caractersticas clnicas de la PSP por lo que en
ocasiones pueden simular este cuadro y deben ser tenidas en cuenta ante presentaciones
incompletas o atpicas: enfermedad de Wilson, enfermedad de Whipple, hidrocefalia a presin
normal, enfermedad de Creutzfeldt-Jakob, degeneracin hepatocerebral adquirida,... (Golbe
2007a).

EVOLUCION Y PRONSTICO
El pronstico de los pacientes con PSP es malo, presentando una supervivencia media desde el
inicio de los sntomas de unos 6 aos, siendo la neumona aspirativa la causa ms frecuente de
muerte. La presencia de sntomas bulbares se ha relacionado con un peor pronstico (Nath et al
2003) mientras que las formas PSP-P tienen una evolucin ms lenta con una supervivencia en
torno a tres aos mayor (Williams et al 2005).
El pronstico de los pacientes con PSP puede ser estimado mediante una escala (PSP rating
scale) que evala su situacin clnica (Golbe 2007b).

MANEJO TERAPETICO
Dado el limitado conocimiento de la fisiopatologa de la PSP, el nico tratamiento del que se
dispone actualmente es un manejo sintomtico de la enfermedad. Pese a sus limitaciones, ste
puede ofrecer un gran aumento en la calidad de vida de los pacientes y prolongar su supervivencia.
El manejo de estos pacientes es complejo, por lo que lo recomendable es que se lleve a cabo en
centros especializados y a cargo de un equipo multidisciplinar incluyendo medidas farmacolgicas
y no farmacolgicas (Warren et al 2007, Golbe 2007a).
- medidas no farmacolgicas:
- logopedas para el manejo de la disfagia y la disartria mediante tcnicas deglutorias y de
comunicacin.
- fisioterapeutas y terapeutas ocupacionales: para mejorar la deambulacin y adaptar el entorno
para evitar cadas
- gastrostoma percutnea: debe plantearse para el manejo de la disfagia severa cuando existe
prdida de peso o se ha producido el primer episodio de aspiracin o neumona.

- los sntomas oculares pueden mejorar mediante la aplicacin de prismas para suplir los dficits en
la motilidad ocular y la administracin de lubricantes oculares para evitar el ojo seco.
- informacin y apoyo adicional para el paciente y los familiares pueden obtenerse a travs de las
asociaciones de pacientes.
- medidas farmacolgicas:
- levodopa: se debe realizar una prueba con levodopa en todo paciente con PSP ya que existe una
proporcin importante de pacientes que van a mejorar su rigidez y bradicinesia, aunque esta
respuesta suele ser incompleta y poco duradera. Algunos de estos pacientes necesitan dosis ms
altas que los pacientes con enfermedad de Parkinson, por lo que se deben alcanzar dosis de hasta
1g/dia de levodopa para descartar que exista una respuesta. El resto de frmacos dopaminrgicos
no han demostrado mayor beneficio que la levodopa y son mucho peor tolerados, por lo que no
suelen emplearse (Lubarsky et al 2008).
- amantadina: puede mejorar los sntomas motores en dosis de 100mg/12h en algunos pacientes.
- amitriptilina: puede mejorar el estado de nimo as como los sntomas motores. Otros
antidepresivos (ISRS) no han mostrado beneficios en los sntomas motores pero si son tiles en el
manejo de los trastornos del nimo relacionados.
- toxina botulinica: puede ser til en el manejo de la apraxia de la apertura palpebral, el
blefaroespasmo y el retrocollis, aunque se debe aplicar con precaucin y hay que vigilar que no se
produzca como efecto secundario un empeoramiento de la disfagia que provoque una aspiracin.

Progressive Supranuclear Palsy


Posted on: January 30, 2006
View Comments (2)Print Article
Vol. 16 Issue 5 Page 6
Progressive Supranuclear Palsy
Safe swallow and aspiration are key concerns
By Alyssa Banotai
Progressive supranuclear palsy (PSP) is a neurodegenerative syndrome and an atypical Parkinsonian
disorder. Most patients present with swallowing, feeding and communication difficulties and require the direct
supervision and treatment of a speech-language pathologist.
PSP, also known as Steele-Richardson-Olszewski syndrome and nuchal dystonia dementia syndrome, may be
confused with or misdiagnosed as Parkinson's disease. "Progressive supranuclear palsy is often referred to as
a Parkinson's plus syndrome with multi-system atrophy in the brainstem, basal ganglia, cerebellar nuclei, midbrain and pontine tegmentum," said Barbara Sonies, PhD, CCC-SLP, BRS-S, a scientist emeritus and former
chief of the Speech-Language Pathology Section at the National Institutes of Health (NIH). "It is initially
confused with Parkinson's disease."

"There are reports of one to six individuals per 100,000 in the literature, comprising about 5 to 6 percent of the
Parkinsonian world," said Beth Solomon, MS, CCC-SLP, chief of the Speech-Language Pathology Section,
Rehabilitation Medicine Department, at NIH.
Symptoms of PSP can appear as early as age 40, but onset typically occurs in the late 50s or 60s. Men usually
are more affected by the disorder than women.
"The presentation of PSP is usually an unsteady gait with a tendency to fall backwards," she told ADVANCE.
"The gait is characterized as staggering or reeling due to stiffness of the neck or legs."
Most patients also present with visual disturbances, such as a downward gazing disturbance, though visual
acuity generally remains intact. Feeding and swallowing problems can arise simply from a patient's inability to
gaze downward in order to see food on the plate.
People with PSP may lose control of their swallowing mechanism, and behavior changes can result in
unintentional rapid drinking and food stuffing. Due to body stiffness and tremors, these patients are poor selffeeders and require mealtime monitoring.
"The biggest concern for speech-language pathologists is the ability to swallow safely and the concern for
aspiration," Solomon explained. "Issues with swallowing, such as aspiration or other medical sequelae
associated with lack of movement, usually are the cause of death."
Differences in the swallowing difficulties experienced by patients with PSP and those with Parkinson's have
been the focus of research by Dr. Sonies, who currently is a research professor in the Hearing and Speech
Sciences Department at the University of Maryland, in College Park, and an adjunct professor in the
Department of Speech and Hearing Sciences at George Washington University in Washington, DC.
She collaborated with Irene Litvan, MD, on oral-motor function research. Dr. Litvan is director of the Movement
Disorder Program, Department of Neurology, at the University of Louisville, in Louisville, KY, and formerly was
a neurologist at NIH.
They interviewed 22 patients with PSP using a swallowing questionnaire developed by Dr. Sonies; conducted
an oral examination to determine swallowing ability for liquids, solids and pastes; and examined wet and dry
swallows and tongue movements using ultrasound due to its noninvasive nature.
The researchers also conducted a standard modified barium swallow study (MBSS) to look at the dysphagia
components of PSP and compare them to those of patients with Parkinson's who have dysphagia.
"We found that there were differences between these populations," Dr. Sonies stated. "The swallowing
questionnaire seemed to differentiate the two just on the basis of observation of swallowing difficulty between
Parkinson's disease and PSP because they are frequently mixed up."
The research indicated that patients with PSP are more aware of their feeding and swallowing difficulties than
people with Parkinson's.
"They reported on their swallowing questionnaire that they were aware that they took longer to prepare and
initiate a swallow, that they were slow eaters, and that they had a lot of coughing and choking," she said.

"Parkinson's disease patients weren't having the awareness that they were having as much trouble."
Patients with PSP were more aware that food fell out of their mouth during meals, Dr. Sonies said. "This was
very interesting. They would try to prepare something, and it would spread all over their mouth and then fall out
before they actually swallowed. This was not characteristic of the Parkinson's patients."
Patients with PSP also complained about chewing problems, which they tried to eliminate by avoiding hard,
crunchy foods. The most common complaints were frequent coughing or choking following a swallow and an
excess of saliva or mucus.
"They were all highly aware of having these difficulties," Dr. Sonies noted.
Approximately 85 percent of the patients with PSP had difficulty with the oral phase of swallowing, according to
the MBSS results.
"They also had pharyngeal swallowing problems at the same 85 percent level because oral phase problems
are related and often the cause of pharyngeal phase problems," she explained. "In the pharyngeal phase they
had pooling in the vallecula space and the pyriform sinuses. In addition, they had trouble just getting the
swallow going."
Citing the results of Dr. Sonies' research, Solomon emphasized the importance of early involvement for
patients with PSP by a speech-language pathologist. If the disease is even suspected, physicians, speechlanguage pathologists or other professionals should request a full swallowing evaluation.
"In addition to serving as a baseline, the evaluation would serve as a measurement of success and treatment
outcomes as well as progression of the disease," she said.
The evaluation should consist of an intensive medical history, eating and swallowing history, comprehensive
oral-sensorimotor assessment, respiratory system observation and clinical feeding assessment. An
instrumental assessment also may be needed.
Clinicians first should determine whether the patient is a safe eater and what food textures are most
appropriate, Solomon recommended. "Chewing and eating solid foods can be laborious and slow, so we try to
have patients avoid foods like red meats, raw vegetables and hard breads. Coughing while eating and drinking
during and following the meal is often problematic. We also recommend avoiding mixed texture foods such as
cereals, with little bits in floating milk."
Though every case is treated individually, most patients require a diet of pureed or smoother textured foods,
she noted.
Other general feeding strategies can be used with the PSP population:
ensure patients are sitting up straight in their chair during a meal;
eliminate distractions such as television and conversations;
maintain a steady, slow feeding rate; and

keep the plate in the patient's line of vision; if a patient has difficulty with downward gaze, put the plate on a
phone book or replace the patient's reading glasses with bifocals.
Thin liquids present a particular challenge to patients with PSP, Solomon cautioned. "They move the fastest
through the mouth, and there is a slight delay in the pharyngeal onset and the closure of the airway. Many
patients with PSP have a lot of recurrent chest infections in conjunction with the coughing and drinking issues."
Using a mirror will show patients how to control problems such as food falling from the mouth, adequate
chewing of the bolus, and overstuffing while eating, Dr. Sonies said.
Speech-language pathologists should demonstrate airway protection strategies to patients, she suggested.
"Help them use the cough that they have to clear their throat to make sure that they have a throat-clearing
maneuver. Show them how to position the bolus in their mouth and make sure that they swallow effortfully.
Make them aware of the oral phase. Make them aware of closing their mouth and manipulating the bolus, not
letting it drip outprimarily by closing their lips and keeping their head in the proper position."
Clinicians also can write down feeding and swallowing problems they observe during mealtime or note the
patient's complaints and use the notations as a teaching tool.
"With some of my patients with PSP, I had to write down very simply, 'This is what you do to swallow: step 1,
step 2, step 3,'" she said.
Speech-language pathologists should use the awareness patients have of their oral-sensory problems to their
advantage during therapy, Dr. Sonies stated. "Use the better or more intact oral sensation that patients with
PSP have in the treatment you provide them."
Family members and caregivers should have an informed, active role in feeding, Solomon said. "Once the
swallowing problem has been identified and therapeutic interventions have been instituted, the caregiver
needs to know the warning signs."
Warning signs include excessive drooling, food collection in the mouth that previously was not present,
increased wetness in the voice following meals, increased mealtime duration, difficulty swallowing medication,
low-grade fevers of unknown origin, chest congestion or coughing during meals, and weight loss.
"These signs should ignite caregivers to seek further assistance from either their speech- language pathologist
or their physician with a referral back to the speech-language pathologist," she said.
The family member or caregiver should be present for at least part of the therapy session, Dr. Sonies
suggested. "If the family member cannot be there, we have wonderful instant e-mails and faxes, but it is
important that there is communication."
In addition to feeding and swallowing difficulties, patients with PSP can present with speech and language
disturbances. Perceptual speech disturbances include hypernasality, hoarseness, and slow rate of speech.
"In terms of language function, one of the cardinal features is a reduction in spontaneous speech, a lack of
initiation in speech production, limitations in the amount and range of narrative expression, and loss of verbal
fluency," stated Solomon.

Certain characteristics of PSP should guide decisions about communication aids and equipment, reported
Roberta Kelley, MS, CCC-SLP, COM, on staff at the Virginia Mason Medical Center in Seattle, WA. These
characteristics include postural difficulties, visual disturbances and possible cognitive decline.
"By the time high-tech augmentative and alternative communication [AAC] devices or speech-generating
devices [SGDs] are needed, the cognitive decline often precludes complex equipment, and it's time to try
simpler SGDs," she said. "In terms of treatment considerations with the PSP population, one needs to be very
proactive and plan ahead for a communication crisis or an increase in the severity of dysarthria because of the
nature of this progressive, degenerative neurological disease."
The individual needs of a patient, as well as the willingness to use an alternate means of communication, will
determine AAC use, Kelley stressed.
She recalled a patient with PSP who spent the week at an assisted living facility while his wife worked and
went home on weekends. When Kelley recommended an AAC device, the patient's wife expressed doubt that
her husband would use it and eventually decided against purchasing the equipment.
As a result, "we're looking at something less complicated that will allow him to get information out to the staff at
the assisted living facility as well as to his wife," she said. "I follow patients and try to be proactive in
recommending AAC equipment sooner rather than later."
Speech-language pathologists and caregivers should make every effort to encourage communication in
patients with PSP, Solomon advised.
Although a "blank expression" due to rigidity of the facial musculature is more typical of Parkinson's disease,
patients with PSP may present with it as well. Solomon stressed that the expression has no bearing on the
patient's ability to comprehend conversation.
"It doesn't mean the patient doesn't understand what the communicator is saying, and that's one of the keys in
educating the communicator," she said. "Just because they have that blank look on their face doesn't mean
they don't understand."
Question-and-answer dialogues about television shows and newspaper articles of interest to the patient can
provide opportunities for communication interchanges in the home. Shorter sentences and questions that
present limited choices often may stimulate short sentence production from the patient.
"These patients are slow in their timing in terms of getting the speech out, so allowing time for them to
communicate is very important," Solomon said.

Question
Recently a patient was admitted to our rehabilitation department with a diagnosis of
corticobasal degeneration. She presents with dysarthria, resembling that of ataxic
dysarthria and mild apraxia (oral greater than verbal). Although her speech sounds abe

Answer
Corticobasal degeneration (also known as corticobasal ganglionic degeneration) is one of the
parkinsonism-plus syndromes (PPS) which also include progressive supranuclear palsy (PSP),
Shy-Drager syndrome, multiple system atrophy (MSA) and olivopontocerebellar degeneration.
These diseases affect multiple neural systems and produce varying symptoms from patient to
patient. All affect the basal ganglia to some extent, producing some symptoms similar to
idiopathic Parkinson's disease (PD). In fact, patients may initially be diagnosed with PD with a
diagnosis of PPS coming later as the disease progresses. Corticobasal degeneration is one of
the rarer of the parkinsonism-plus syndromes and typically the progression of symptoms is
more rapid than those seen in PD. Unlike PD, patients with corticobasal degeneration typically
do not respond to antiparkinsonism drugs. Dementia is also seen in the later stages of the
syndrome. The dysarthria associated with corticobasal degeneration is of the mixed variety and
varies from patient to patient.
With regard to treatment, there is very little research investigating the effectiveness of
behavioral therapy with patients with this specific diagnosis. Because the components of the
mixed dysarthria may vary, a clinician could identify the primary dysarthria and use treatments
that have been designed for that type of dysarthria. For example, speaking rate control
techniques or other prosodic therapies for patients with predominately ataxic symptoms.
Countryman, et al (1994) did provide some evidence that Lee Silverman Voice Therapy LSVT
is effective in improving voice and intelligibility in patients with parkinsonism-plus syndromes,
but caution that it should only be used in patients who have voice symptoms typical of PD.
However, as this disease often shows rapid progression, it is likely that speech will also show
rapid deterioration. With this in mind, a speech therapy program should be simple and target
functional communication as quickly and efficiently as possible. It may be more beneficial to
spend therapy time teaching the patient ways to compensate for speech deficits, as well as
repair strategies to use when intelligibility breaks down, as well as working with family members
to assure maximum communicative effectiveness. An excellent resource is Management of
Speech and Swallowing in Degenerative Disorders by Yorkston, Miller and Strand. All patients
with dysarthria, regardless of etiology, can benefit from these communication-centered

approaches to therapy to improve their quality of life. Patients who receive speech therapy in
early stages of the disease progression may be able to continue to communicate verbally for a
longer period of time. Augmentative communication will likely be necessary in later stages.

Managing Symptoms
Behavioural Changes
PSP can lead to changes in behaviour or personality. For some people, these changes may be
very subtle, whilst others may notice obvious changes. This can be distressing for the person
living with PSP, their carers, friends and families.
This information aims to inform you about the types of changes in behaviour that may occur
and offers practical advice to enable you to develop your own coping strategies:

Cognition and Mood

Speech and Communication


Bowel and Bladder
People will be increasingly immobile as PSP progresses and as a result, constipation, lack of
bowel control and urinary problems may develop.
The following information offers practical advice to help manage some of these symptoms:

bladder and Bowel


PEG Feeding
In PSP, the muscles involved in chewing and swallowing can become slow, weak and/or
uncoordinated.
As a result it can take longer to eat a meal. You may also experience episodes of coughing
whilst eating or drinking.

If swallowing becomes difficult you may eat and drink less than you need which results in
weight loss, thus making you weaker and more vulnerable to infections.
To ease these problems alternative feeding methods may be suggested to you by your health
care team this may include PEG feeding.
A PEG (percutaneous endoscopic gastrostomy) is a feeding tube which passes through the
abdominal wall into the stomach. This means nutrition can be provided without swallowing. In
some cases a PEG can be used to supplement ordinary food.
For further information on PEG feeding please telephone our Helpline on 0300 0110 122 or
email helpline@pspassociation.org.uk

Swallowing, eating and drinking


Saliva
Saliva helps break down food and protects the mouth from drying out and cracking.
On average we produce around a litre and a half of saliva each day. People living with PSP
have a reduced swallow reflex, resulting in a build up of saliva inside the mouth. Sometimes
saliva can also be thick and difficult to swallow and some medication can cause saliva to dry
up, resulting in a dry mouth.
Oral hygiene is essential as it will help minimise any excess bacteria from the mouth entering
the lungs.
The following information offers practical ideas on managing saliva problems. If you continue to
experience problems with saliva, then do contact your GP who may be able to provide
medication to alleviate some of the symptoms:

Saliva, drymouth and oral hygiene


Sight
People living with PSP may experience visual problems which cant be corrected by glasses.
The main symptoms include:

Double vision

Tunnel vision where the field of vision is reduced

Blurred or misty vision

Slow jerky eye movement

Difficulty looking down


This information aims to offer practical advice on coping with these symptoms:

Vision
Speech
In PSP, areas of the brain controlling movement of the tongue, lips and throat can impact on
speech making it slurred and difficult to understand.
There are three main types of speech problems:

Voice sounding strained or harsh and speech is slow and slurred

Speech becoming quiet, to the point of being difficult to hear and very fast. The voice
may also sound croaky or breathy

Speech sounds slurred and people can often struggle with longer words
A speech and language therapist (SLT) can show you how to modify your speech and provide
advice and equipment for alternative ways of communicating.
The following aims to inform and offer you practical help and advice on speech and
communication:

Alternative Ways of Communicating

Swallowing Difficulties
Eating, drinking and swallowing difficulties are common in PSP and can occur fairly soon after
diagnosis.

Coughing when eating or drinking is an indication that food or drink has gone down the wrong
way into the airway. If this occurs regularly, it can lead to chest infections or pneumonia.
A speech and language therapist (SLT) can assess swallowing and suggest strategies for
coping.
The following aims to inform you in more detail about swallowing difficulties. It also introduces
some simple changes to make eating and drinking less troublesome:

Swallowing, eating and drinking

Scale content
The PSPRS comprises 28 items in six areas. The available total score ranges from 0 (normal)
to 100. Six items are rated on a 3-point scale (02) and 22 are rated on a 5-point scale (04).
The History/Daily Activities area includes seven items with a total maximum of 24 points, the
mentation area four items with 16 points, the bulbar area two items with 8 points, the ocular
motor area four items with 16 points, the limb motor area six items with 16 points and the gait
area five items with 20 points.
Table 2 shows the items and the definitions of each score level along with details regarding
administration of the test.

Enlarge table

Table 2
The progressive supranuclear palsy rating scale with comments and instructions
Item name and score definitions

Comments, instructions

I. History
1. Withdrawal
0 None

Relative to baseline personality

1 Follows conversation in a group, may


respond spontaneously, but rarely if ever initiates

Consider lack of conversation due to dementia or

exchanges

bradyphrenia as withdrawal

Item name and score definitions

Comments, instructions

2 Rarely or never follows conversation in


a group
2. Irritability
0 No increase in irritability

Relative to baseline personality

1 Increased, but not interfering with


family interactions

Ask if patient shouts or loses temper easily

2 Interfering with family interactions


3. Dysphagia for solids
0 Normal; no difficulty with full range of
food textures

Ignore difficulty related to overloading mouth

1 Tough foods must be cut up into small

If certain foods like bread crusts or leafy vegetables

pieces

must be avoided, but meats OK, score 2

2 Requires soft solid diet


3 Requires pureed or liquid diet
4 Tube feeding required for some or all
feeding
4. Using knife and fork, buttoning clothes,
washing hands and face
0 Normal

Rate the worst of the 3


If difficulty is related to downgaze, score as if it were

1 Somewhat slow but no help required


2 Extremely slow; or occasional help
needed
3 Considerable help needed but can do

purely motor

Item name and score definitions

Comments, instructions

some things alone


4 Requires total assistance
5. Falls
Average frequency if patient attempted to walk
0 None in the past year

unaided

1 <1 per month; gait may otherwise be


normal

Assume no access to walking aids

2 14 per month

Ignore near-falls

3 530 per month


4 >30 per month (or chairbound)
6. Urinary incontinence
If daytime pad used as precaution but no recent
0 None or a few drops less than daily

wetting, score 3

1 A few drops staining clothes daily


2 Large amounts, but only when asleep;
no pad required during day
3 Occasional large amounts in daytime;
pad required
4 Consistent, requiring diaper or
catheter awake and asleep
7. Sleep difficulty
0 Neither 1 nor 2 insomnia

1 insomnia is difficulty falling asleep

1 Either 1 or 2 insomnia; averages 5

2 is difficulty remaining asleep

Item name and score definitions

Comments, instructions

h sleep nightly
2 Both 1 and 2 insomnia; averages 5

Ignore trips to bathroom after which pt. returns to

h sleep nightly

sleep easily

3 Either 1 or 2 insomnia; averages <5


h sleep nightly
4 Both 1 and 2 insomnia; averages <5
h sleep nightly
II. Mentation
Items 811 use this scale:
Estimate the degree to which each deficit would
0 Clearly absent

interfere with performance of daily cognitive tasks

1 Equivocal or minimal
2 Clearly present, but does not interfere
with activities of daily living (ADL)
3 Interferes mildly with ADL
4 Interferes markedly with ADL
Use MMSE items 110 or history to estimate
8. Disorientation

interference in ADLs
If delayed responses prompt the caregiver to answer
for the patient or limit your ability to interview patient,

9. Bradyphrenia

rate at least a 3
If there is a history of inappropriate laughing or crying
but none at the time of the examination, rate a 1 or 2,

10. Emotional incontinence

depending on its frequency

11. Grasping/imitatative/utilizing behaviour

If none is displayed spontaneously (e.g. grabbing

Item name and score definitions

Comments, instructions
your coat or arm, or the wheelchair arm), ask patient to
rest hands on thighs, palms up. Hold your hands 510
cm above his and say nothing.
If he grabs them, rate a 3
If he only imitates your actions during the exam, rate
a2

III. Bulbar
12. Dysarthria
0 None

Ignore palilalia and dysphonia

1 Minimal; all or nearly all words easily


comprehensible

Comprehensible means to examiner, not caregiver


If generally silent but can be coaxed to speak a few

2 Definite, moderate; most words

words, rate a 4 no matter how clear those words may

comprehensible

be

3 Severe; may be fuent but most words


incomprehensible
4 Mute; or a few poorly comprehensible
words
13. Dysphagia
0 None

Give 3050cc of water in a cup, if safe


Do not give water if secretions are audible with

1 Single sips, or fuid pools in mouth or

breathing, if there is a history of frequent aspiration or if

pharynx, but no choking/coughing

caregiver is apprehensive

2 Occasionally coughs to clear fuid; no


frank aspiration

1 cough rates 2, multiple coughs 3

Item name and score definitions

Comments, instructions

3 Frequently coughs to clear fuid; may


aspirate slightly; may expectorate frequently
rather than swallow secretions
4 Requires artificial measures (oral
suctioning, tracheostomy or feeding gastrostomy)
to avoid aspiration
IV. Ocular motor
Items 1416 use this scale:
0 Saccades not slow or hypometric; 86
100% of normal excursion

Use a stationary target and a verbal command

1 Saccades slow or hypometric; 86

If improves with repetition, use the initial (i.e. worst)

100% of normal excursion

result

2 5185% of normal excursion

May hold lids to observe downward saccades

3 1650% of normal excursion

Normal range of gaze is 50 in each direction

4 15% of normal excursion or worse

Ignore square-wave jerks

14. Voluntary upward command movement


15. Voluntary downward command movement
16. Voluntary left and right command movement
17. Eyelid dysfunction
0 None

Recruitment of frontalis muscle rates at least 2.

1 Blink rate decreased (<15/min) but no

Isolated difficulty closing lids on command rates at

other abnormality

least 2

2 Mild inhibition of opening or closing or


mild blepharospasm; no visual disability

Item name and score definitions

Comments, instructions

3 Moderate lid-opening inhibition or


blepharospasm causing partial visual disability
4 Functional blindness or near-blindness
because of involuntary eyelid closure
V. Limb motor
18. Limb rigidity
0 Absent

Rate the worst of the four limbs


Count fexion contracture in advanced pts as

1 Slight or detectable only on activation

dystonia, not rigidity

2 Definitely abnormal, but full range of


motion possible
3 Only partial range of motion possible
4 Little or no passive motion possible
19. Limb dystonia
0 Absent

Rate the worst of the four limbs

1 Subtle or present only when activated

When subtle, may be evident only with activating

by other movement

tasks such as sustention task or tapping by other limbs

2 Obvious but not continuous


3 Continuous but not disabling
4 Continuous and disabling
20. Finger tapping
0 Normal (>14 taps/5s with maximal
amplitude)

If asymmetric, rate worse side

Item name and score definitions

Comments, instructions

1 Impaired (614 taps/5s or moderate


loss of amplitude
2 Barely able to perform (05 taps/5s or
severe loss of amplitude)
21. Toe tapping
0 Normal (>14 taps/5s with maximal
amplitude)

If asymmetric, rate worse side

1 Impaired (614 taps/5s or moderate


loss of amplitude
2 Barely able to perform (05 taps/5s or
severe loss of amplitude)
22. Apraxia of hand movement
0 Absent

Test for ideomotor apraxia


Two tasks with each hand (e.g. salute, throw ball,

1 Present, not impairing most functions

hitchhike, V-for-victory)

2 Impairing most functions


23. Tremor in any part
0 Absent

Upper extremities extended

1 Present, not impairing most functions

Finger-to-nose with each hand

2 Impairing most functions


VI. Gait and midline
24. Neck rigidity or dystonia
0 Absent

Rate the resistance to passive antero-posterior

Item name and score definitions

Comments, instructions
rotation

1 Slight or detectable only when

Ignore spontaneous posture (kyphosis, dystonic

activated by other movement

rotation, retrocollis)

2 Definitely abnormal, but full range of


motion possible
3 Only partial range of motion possible
4 Little or no passive motion possible
25. Arising from chair
If patient must use hands, do not allow hands to
0 Normal

contact arms of the chair

1 Slow but arises on first attempt

If cane needed, to arise, rate 4

2 Requires more than one attempt, but

If patient can arise unassisted but falls forward

arises without using hands

(rocket sign), rate 4

3 Requires use of hands


4 Unable to arise without assistance
26. Gait
If patient staggers across room, using wall or
0 Normal
1 Slightly wide-based or irregular or
slight pulsion on turns
2 Must walk slowly or occasionally use
walls or helper to avoid falling, especially on turns
3 Must use assistance all or almost all
the time

furniture when possible, rate 3

Item name and score definitions

Comments, instructions

4 Unable to walk, even with walker; may


be able to transfer
27. Postural stability
If pt. can remain standing unassisted, pull backward
0 Normal (shifts neither foot or one foot)

by shoulders and be ready to catch him/her

1 Must shift each foot at least once but

Pull should be hard enough to make normal adult

recovers unaided

take one step back to retain balance

2 Shifts feet and must be caught by


examiner
3 Unable to shift feet; must be caught,
but does not require assistance to stand still
4 Tends to fall without a pull; requires
assistance to stand still
28. Sitting down
May use hands to touch seat of chair, but not arms or
0 Normal
1 Slightly stiff or awkward
2 Easily positions self before chair, but
descent into chair is uncontrolled
3 Has difficulty finding chair behind
him/her and descent is uncontrolled
4 Unable to test because of severe
postural instability

back of chair

THE ROLE OF THE SLP IN EVALUATING AND TREATING PD


There are many professionals that care for the person with PD. Speechlanguage
pathologists (SLP) are healthcare professionals trained to evaluate and
treat
individuals with speech, voice, language, and swallowing problems. A
SLP has a

graduate degree and is certified by the American Speech-Language and


Hearing
Association (ASHA).
Developing and improving effective communication skills and swallowing
function
are the primary roles of a SLP when treating an individual with PD. Many
SLPs have specialized training, specifically in treatment of the PD
population.
Where Can I Find an SLP?
Local hospitals and rehabilitation centers employ SLPs to provide both
inpatient
and outpatient services. In addition, many SLPs have private practices,
and many
university clinics specialize in treatment of PD.
Evaluating Speech and Voice
Healthy speech, voice, and swallowing production require that the brain
and
muscles that work the structures of the head, neck and pulmonary
system are all
functional. If a referral to a SLP is made, it is most likely that he/she will
be
working closely with an otolaryngologist (a physician who diagnoses
disorders of the ear, nose and throat) or a neurologist (a physician who
diagnoses
and treats disorders of the nervous system). The SLPs job is to
determine if there
are problems with the way the structures in the oral cavity (tongue, lips,
jaw etc)
3
are working to produce speech, voice and swallow. Questions about
medical history
will be asked and specific questions about the changes in speech, voice,
or
swallow following the onset of PD will need to be
documented. Particular emphasis will be on how speech and voice
quality has
changed and what circumstances or situations make it get better or
worse.
Questions about changes to eating and nutrition will also be asked.
A SLP may perform an oral peripheral examination, to look at the
structure and
function of the lips, tongue, jaw and other parts within the mouth.
Following the
oral peripheral examination, the speech pathologist may perform an
examination

of the vocal cords. This is done in the physicians office and allows the
medical
team to determine if the vocal cords are moving normally.
The SLP is the person who will provide detailed information about the
changes
that will occur in speech, voice and swallowing as the disease progresses
and will
be able to offer solutions and treatment for improving speech, voice and
swallowing if problems are being experienced.
4
WHAT IS NORMAL?
Normal Breathing
In order to breathe without effort the lungs, muscles of the ribcage,
diaphragm and
abdominal muscle all need to be healthy and function normally. Each of
these parts
makes up a big portion of the respiratory system. When air is taken into
the lungs
it is called primary inspiration or inhalation. To breathe-in, the lungs have
to
expand in volume. Movement of the diaphragm achieves this. When air
is moved
out of the lungs it is called expiration or exhalation. To breathe-out the
lungs have
to decrease in volume. Movement of the abdomen and the ribcage
achieves this.
When all parts of the respiratory system are functioning normally, the
effort to
breathe is minimal and automatic. When problems arise, the effort of
breathing
increases and this is when a physician should be consulted immediately.
Normal Voice Production
Respiration (breath support) is often referred to as the power-source
for voice
production. When speaking, air must be brought into the lungs
(inhalation).
During exhalation, the air rushes through the vocal cords. The vocal
cords sit right
at the top of the windpipe, therefore, when air rushes out of the lungs it
moves
through the vocal cords. When speaking, the vocal cords move close
together. The
air rushing through the vocal cords causes the vocal cords to vibrate
generating
sounds. Depending on age and sex, the vocal cords will vibrate from 100
to 250

times per second. Articulators (tongue, lips, teeth) move in specific


patterns to
shape the sound generated from the vocal cords into speech sounds.
The brain is
responsible for sending a message by way of a nerve to the vocal cords
to be in the
appropriate position to vibrate when the air rushes though them during
exhalation.
If there is damage to the brain, or changes in the way it functions then
this
message may not be sent, resulting in a change in vocal quality. The
nerve that
supplies the information to the vocal cords regarding their position may
be
damaged as well.
Voice Quality
As a result of PD voice quality may sound hoarse, breathy or low in
loudness. This
is caused by the vocal cords in the larynx/throat not firmly meeting in a
regular
rhythm or closing pattern when the voice is produced. Below are two
checklists
(Hogikyan & Sethuraman, 1999; Hogikyan & Rosen, 2002, Jacobsen,
Johnson,
Grywalski, Silbergleit, Jacobsen & Benninger, 1997) that can be used to
determine
if changes in the voice are presenting a handicapping condition.
5
6
Voice Handicap Index: This index was developed for patients that
experience
difficulty with their voice. Sometimes the problems can become severe
enough
that it imposes a handicapping condition. The Voice Handicap Index can
be used
as a way to see if voice problems associated with PD are adversely
affecting daily
activities.
Instructions: These are statements that many people have used to
describe their
voices and the effects of their voices on their lives. Circle the response
that
indicates how frequently you have the same experience.
0 = Never 1 = Almost Never 2 = Sometimes 3 = Almost Always 4 =
Always
Part I-Functional

1. My voice makes it difficult for people to hear me.


01234
2. People have difficulty understanding me in a noisy room.
01234
3. My family has difficulty hearing me when I call them throughout the
house.
01234
4. I use the phone less often than I would like to.
01234
5. I tend to avoid groups of people because of my voice.
01234
6. I speak with friends, neighbors, or relatives less often because of my
voice.
01234
7. People ask me to repeat myself when speaking face-to-face.
01234
8. My voice difficulties restrict personal and social life.
01234
9. I feel left out of conversations because of my voice.
01234
10. My voice problem causes me to lose income.
01234
Part II-Physical
1. I run out of air when I talk.
01234
2. The sound of my voice varies throughout the day.
01234
3. People ask, What is wrong with your voice?
01234
4. My voice sounds creaky and dry.
01234
5. I feel as though I have to strain to produce voice.
01234
6. The clarity of my voice is unpredictable.
01234
7. I try to change my voice to sound different.
01234
8. I use a great deal of effort to speak.
01234
9. My voice sounds worse in the evening.
01234
10. My voice gives out on me in the middle of speaking.
01234
Part III-Emotional
1. I am tense when talking to others because of my voice.
01234

2. People seem irritated with my voice


01234
3. I find that other people dont understand my voice problem.
01234
4. My voice problem upsets me.
01234
5. I am less outgoing because of my voice problem.
01234
6. My voice makes me feel handicapped.
01234
7
7. I feel annoyed when people ask me to repeat.
01234
8. I feel embarrassed when people ask me to repeat
01234
9. My voice makes me feel incompetent.
01234
10. I am ashamed of my voice problem.
01234
Often it is the caregiver who realizes the change in communication
before the
person with PD. A care partner, family member or friend who has regular
contact
can help complete the form below. The respondent should carefully read
each
statement, thoughtfully consider if it applies, and make a check mark if
it does:
Caregiver Questionnaire
1. I have difficulty hearing my Parkinson partner when he/she speaks.
2. I have difficulty understanding his/her speech.
3. My Parkinson partner does not talk as much as in the past.
4. My Parkinson partner does not attend social functions as frequently as
in the past.
5. He/she often asks me to make phone calls or order from a menu for
him/her.
6. My Parkinson partner clears his/her throat often.
7. My Parkinson partner often sounds as if he/she is running out of
breath
when speaking.
8. My Parkinson partner suspects that I need a hearing aid.
9. My Parkinson partner thinks I ignore what he/she has to say.
Multiple yes answers, or a yes answer to even one issue could
interfere with
daily communication. This may be sufficient to request referral for a
complete

speech and voice evaluation because the difficulties that are being
experienced
may have an underlying medical cause.
Try these suggestions to improve voice quality:
Try to produce a staccato or sharp sound while producing voice.
Keep the loudness level of the voice up by pushing air from the
abdomen.
Practice speech drills and lip and tongue strengthening exercises (see
Appendices)
Protect the vocal cords by avoiding excessive coughing, throat clearing
or yelling.
Protect the vocal cords by keeping the home air moist. Use a
humidifier, if necessary
8
VOCAL LOUDNESS
There are many aspects of speech or voice that can be affected because
of PD.
Low vocal loudness level means that the vocal output is softer than
normal. Often
time persons with PD indicate that they cannot be heard or that they
have a hard
time speaking over crowd noise like that, which occurs at a social
gathering. Also,
family members or a spouse may complain that the person with PD is
not speaking
up loud enough and it can lead to frustration during communication
interaction.
The difficulties that persons with PD have with vocal loudness have been
well studied. Some believe that it results because of the changes that
occur to the
muscles that help the voice get loud, like the muscles of the vocal cords
or the
muscles that control breathing. Others believe that the person with PD
needs to be
trained how to speak louder and that with adequate training using a
louder voice
can be achieved. The training programs that are used to help persons
with PD will
be discussed later in this section.
There are some strategies that can be used to increase the
loudness level of
the voice such as:
Take a big breath before beginning to speak. This helps give enough
air
to speak on and will actually make the voice come out louder. Start by
trying to hear the voice as soon as the breath is allowed to exhale. Begin

by breathing in and then slowly exhaling. Try to control the air that is
released when you exhale (slow and controlled). Next, say the vowels
ah or ee with the lips slightly parted on a steady flow of exhaled air.
Try to hold the sound for at least 15-20 seconds or make that an
eventual
goal as the practice continues. This can be done three times in a row
with a 1-2 minute rest after each of the three sound productions. This
should be done 3-4 times a day to help gain control of the breathing and
develop an awareness of how speech is produced.
Make sure that the sentences/phrases that are spoken are not too
long.
The longer the phrase the more air that is used. If the amount of air runs
out then it will require more physical effort to keep the voice loud. A
phrase like: The other day I went to the store would be a phrase of
adequate length. After finishing the phrase, another breath needs to be
taken, if not it will be very difficult to make the voice stay loud.
It can help if muscles in the abdomen are used to help force air out the
lungs. This is especially true when the voice is used to yell or talk very
loud over crowd noise. Try speaking aloud a short staccato counting
phrase hut, 2,3,4. Keep a hand on the abdomen and feel the in- and
out- movement of the abdominal wall.
Maintain a good posture. It is most efficient if the body and head are
facing straight. If the head is tilted to one side or the posture is slouched
it does not allow the muscles that are working together to produce
speech
to be in an optimal position.
Open the mouth when speaking. If the mouth (jaw and lips) are not
moving very much during speaking it actually makes the sound come
out
9
less loud. Try at first to over exaggerate lip and mouth movements when
saying vowels such as ah or the words found in Appendix C. Hear and
feel the difference when the mouth is open wide compared to trying to
speak through nearly closed lips.
If it is still difficult to make the voice loud after trying some or all of
these
exercises, then there could be some other reasons that are preventing
progress.
First, discuss these difficulties that are being experienced with a
neurologist
and/or speech pathologist. They may make a referral to other specialists
to
determine if:
The vocal cords are weak and unable to close properly. A consult with an
Ear, Nose and Throat doctor (otolaryngologist) may be made to examine
the function of the vocal cords.

The lungs are not functioning normally. A consult with a pulmonologist


may be made to test the function of the lungs.
See Appendix B for some functional phrases and sentences to use at
home to
practice projecting vocal loudness.
Voice Amplifiers
There are some circumstances where the voice needs additional help in
being
made loud. These circumstances include work environments that are
noisy and
require the voice to be loud for long periods of time. With PD the voice
will tire
over time and even the strategies listed above may not be enough to
keep the voice
loud. In these situations augmentative devices can be used to give the
voice that
extra power it needs. These devices are called amplification units or
voice amplifiers.
The amplifiers can be personal amplifiers worn on the body or placed in
a
room, such as classroom or conference room. Sometimes the
microphone is worn
on the lapel of a shirt or is part of a headset system. Remember the
amplifiers will
not improve the quality of the voice, it will simply make it louder. Most of
the
devices on the market can at least double the loudness level of the
voice. A speech
pathologist and family members can help decide which type is the best
device for
the situation. Additionally, the amplifier should be comfortable to the
person with
PD. The following list is a sample of companies that market voice
amplification
systems, which might be useful in providing compact personal
amplification
systems for people with low-volume speech.
Insurance coverage for amplifiers: MEDICARE does not usually cover
amplifiers,
but they are considered on an individual basis and some people have
been
reimbursed, so it may be worthwhile to submit a claim.
Here are some other suggestions that can be used to enhance
communication.
1. Choose the best spot to communicate. Areas with appropriate lighting
and very little noise are the best;

2. Look for visual cues to determine what the communication partner is


trying to get across;
3. Ask for written words if needed to get on the same topic;
4. Provide feedback to the communication partner that the message has
been understood or that a part of the message has not been understood;
5. Do not pretend that you understand;
6. Try not to interrupt the person who is talking with you;
7. Avoid putting objects in front of the face while speaking;
8. Avoid having objects in the mouth (i.e. gum, cigarettes, and food)
while
speaking;
9. Use gestures;
10. Provide clues to the communication partner when a subject changes;
11. Rephrase what is being said if the message is not being understood.
11
There are other types of devices that are used when a persons ability to
communicate
becomes very limited. These are also called augmentative or assistive
devices. The devices can be used in addition to continued efforts at
speech
improvement or by themselves. Some systems are quite simple and
inexpensive,
while others are complex and more expensive.
Writing - This is most common way to communicate without speech.
Keep writing materials accessible at all times. These could include paper
and pencil, clipboard, small chalkboard with chalk and eraser or a magic
slate (found in toy departments). When writing or printing, concentrate
on
keeping the letters large and well spaced.
Pointing Systems - This includes such items as a large board,
notebook,
binder or photo album with pictures of family, friends and commonly
used
items. Alphabet boards or notebooks with words for frequently needed
items organized into categories are other helpful pointing systems.
Electronic/Computer Systems - These are the most sophisticated
systems
and may consist of a keyboard, display screen and printer. Many are
portable. Size of keyboard and display/printer varies
Before purchasing a device, consult with a speech-language pathologist.
The
following companies are some of those marketing communication
devices.
Programs To Help Improve Voice Loudness
One of the most widely used programs that has been tested and shown
to have

positive outcomes for persons with PD is called the The Lee Silverman
Voice
Treatment (LSVT) program. Lorraine O. Ramig, Ph.D., CCC-SLP, and
Carolyn
Mead, M.A., CCC-SLP developed this program, in 1987. Both are speech
pathologists. It was named after one of the first patients to receive this
treatment.
It is an intensive behavioral treatment program that requires a person
with PD to
engage in 16 therapy sessions in one month. The major goal of the
technique is to
help improve vocal loudness, but as a result of the therapy,
improvements in
speech clarity and breathing often result. While the number of sessions
to be
completed in one month is very intensive the outcomes of the program
is long lasting
(Ramig, Sapir, Countryman, Pawlas, Obrien, Hoehn, Thompson, 2001).
More
information on the program can be found at www.lsvtglobal.com
The LSVT program consists of five essential concepts:
Concept 1: focuses on the VOICE. This concept works on helping the
vocal cords
close better, reinforces the idea of THINKING LOUD/THINKING SHOUT
and
attempts to train the speech to be as clear as possible.
Concept 2: focuses on HIGH EFFORT with emphasis on vocal and
physical
effort. This focus on effort is thought to override the muscles lack of
movement
and
slowness of movement to new effort levels particularly as the disease
progresses.
The clinician helps the person with PD scale the effort needed to perform
the tasks
during therapy.
Concept 3: sixteen sessions are done on an individual basis for a period
of one
month. This provides daily opportunity to practice and over the 16
sessions,
allows the degree of vocal effort to be increased. It helps maintain a
certain level
of motivation and accountability. It also provides adequate time for the
skill(s)
to be learned and transferred to other environments such as home or
work.

Finally it allows the SLP to document any fluctuations in the patients


perform13
ance.
Concept 4: focuses on defining what the patient knows and accepts
about the
amount of effort needed to consistently increase vocal loudness to a
level that is
within normal limits. The relationship between increased vocal effort and
vocal
output is then established. Those with PD often have problems knowing
how loud
their voice is.
Concept 5: focuses on quantification. The key is to motivate the patient
and
provide feedback about the improvement or the need for more
improvement.
During this phase all five concepts are integrated and all focus on high
effort voice
used daily.
Some persons with PD indicate that LSVT has been an excellent program
for
improving voice. There is ome research as well to suggest that LSVT may
improve
swalling function, however, the most direct outcome with LSVT are
imrpovements
in speech and voice.
14
VOCAL PITCH
Pitch Variation
The speech of people with PD often has very little melody or pitch
variation. This
is called monotone speech. It can be hard to listen to because it is the
inflection of
speech that keeps listeners involved in a conversation. Speech, which is
monotone,
or produced almost constantly on the same note, lacks liveliness. It can
be boring
to listen to and sometimes it is misunderstood. The pitch of the voice is
what controls
the variation of the highs and lows of the voice. Pitch is controlled by
how
fast the vocal cords vibrate, so it is understandable that someone with
PD that has
muscle impairment may have difficulty varying the pitch of the voice.

When practicing the words, phrases and sentences found in the


Appendices try to
put pitch changes into the speech. Think about the melody of the speech
while
talking. Remember these principles for voice inflection:
When making a statement, start the sentence at a slightly higher pitch
so that the pitch can be brought down at the end. For example:
Its time to go home.
I am happy to meet you.
The dog had seven puppies.
When asking a question that could be answered yes or no, do the
opposite. Start lower and raise the pitch at the end. For example:
Are you ready to go?
Should we go to the movie today?
Would you like more coffee?
When asking a question that needs more than a yes or no answer,
lower the pitch at the end of the question. For example:
Should we have soup or sandwich?
What would you like for breakfast?
Do they live in Minneapolis or New York?
Emphasize important words by bringing the pitch level up when they
are said.
When reading the speech practice material, draw arrows to aid in
indicating
when to change the pitch. Draw to bring the pitch up; draw
to bring the pitch down.
Practice pitch changes when singing. Try to exaggerate the pitch range
when singing.
Biofeedback for Improving Voice Production
Biofeedback is a technique that gives the person with PD information
about how
speech sounds or how it is being produced to make it sound the way it
does.
Biofeedback can also be a therapy tool drawing on relaxation techniques
or other
techniques to help an individual gain voluntary control so that a desired
response
can be achieved. An example would be trying to achieve a certain vocal
loudness
15
or vocal pitch. Biofeedback techniques will help the individual target the
loudness
level that is appropriate for the speaking task. Visual and auditory
biofeedback is
a major component of patient education, self-monitoring skills and
documentation

of treatment effectiveness. Technology is rapidly developing and there


are many
adept devices at surprisingly low cost. Software for biofeedback of
speech production
is available at:
www.kayelemetrics.com
1-800-289-5297
16
ENHANCING SPEECH
Slow and Even Speech Rate
Sometimes people with PD experience rushes of speech very rapid
speech
with an uneven tempo. It is very difficult to keep precise speech sound
production
when the speech is moving very fast. Speech becomes difficult to
understand.
Precise and Clear Speech Sounds
All talkers differ in the way they speak. Some people talk fast, some
people use
more gestures with their hands to get their point across, while some use
pauses to
make a point. The speech of people who have PD is often difficult to
understand
because the individual speech sounds are not made clearly or precisely.
The result
is speech that sounds, slurred. Any of the following reasons can
contribute to
this difficulty. Recall that the disease affects the movements of the oral
structures
that produce speech and some of the symptoms that may make it
difficult to speak
clear are:
Slow and imprecise lip movements.
Slow and imprecise tongue movements.
Poor ability to move the tongue and lips together in a way that is
coordinated and rapid enough to be able to produce all of the speech
sounds clearly.
There is a technique called Clear Speech (Picheny, Durlach & Braida,
1985;
Schum, 1996) that is used by persons who talk to those who are hearingimpaired,
and it can be used by anyone wishing to better their pronunciation so
that their listeners
may understand them. The program focuses on key skills needed to
improve

the clarity of speech and also helps build confidence in the speaker. Not
being able
to be understood is frustrating for the speaker and can, at times, make
the speaker
withdrawal from communication interactions because of the difficulty of
being
understood. The following exercises can be tried to improve the clarity of
speech.
Clear Speech Exercises
Practice lip strength and flexibility exercises (see Appendix C).
Practice tongue strength and flexibility exercises (see Appendix D).
Before starting to speak, swallow all excess saliva in the mouth.
Say all sounds clearly and firmly exaggerate the sounds and do not
leave any sounds of any words out.
Start by practicing single words, then two and three word phrases,
short
sentences and paragraphs. (see Appendix B).
Make sure the lips meet firmly for b, p, and m sounds.
Try to explode the sounds t, d, k, and g.
Pause between words and remember to keep the vocal loudness up
until
the end of the sentence.
17
When talking, remember to use shorter sentences.
Simplify the message for the listener.
Speak at a slightly slower than normal rate, so the words can be said
better.
Do not strain to say every word perfectly.
Clear Speech is naturally louder, lively and full of distinct and meaningful
pauses.
It has been found that simply training with these exercises for 5 -10
minutes a day
has a positive carryover to conversation activities and can last up to a
few months
with some re-training needed if the person starts to lose the Clear
Speech
strategies once learned.
Other strategies that have been offered to help produce clear speech is
to practice
the following:
Say short sentences while tapping out an even rhythm with a finger on
the table, chair or leg.
Say one syllable, or one word part, for each tap.
Say short sentences in time with a slowly ticking metronome. Say one
word or syllable per beat.
Put a row of colored dots on a piece of paper. Say one word or syllable

as you touch each dot in a slow and even rhythm.


There is a system called the Facilitator which provides Delayed Auditory
Feedback that can also be used to help slow down speaking rate. For the
individual
whose speech improves with delayed auditory feedback (DAF), the DAF
on
the Facilitator makes the instrument a powerful assistive speaking
device. Details
on a DAF system can be found on www.kayelemetrics.com.
18
SWALLOWING FUNCTION
The term that is used for swallowing dysfunction is dysphagia. In order
to determine
if a swallowing dysfunction is occurring there are certain symptoms to
pay
attention to. It is not uncommon for people with PD to report difficulty
with
chewing and swallowing. That is because the physical changes in the
muscles used
for speech also can affect the ability to chew and swallow safely. The
primary
problems reported are:
Slow rate of eating.
Fatigue during eating.
Food sticking in the throat.
Coughing or choking on food or liquid.
Difficulty in swallowing pills.
Other swallowing symptoms that can be encountered include:
Difficulty initiating swallowing.
Unexplained weight loss.
Change in dietary habits.
Recurrent pneumonia.
Change in voice or speech.
Nasal regurgitation.
Sensation of food sticking in the chest.
Oral or pharyngeal regurgitation.
When someone is having difficulty with swallowing you may notice
symptoms
when they are drinking and eating. There are some very common
questions that
can either be asked or answered to determine if the patient with PD is
having
these difficulties. Below are some questions that can be used to obtain
an initial
impression of weather swallowing difficulty exists.

If you answer true to one or more of these then consult with a primary
care
physician/neurologist about these problems immediately. A swallowing
evaluation
may also be recommended.
1. I feel weak and tired often.
2. Most days, I dont care if I eat or not.
3. I have recently experienced an unintentional loss of weight.
4. It takes me longer to eat than other people.
5. It takes me forever to eat a meal.
6. I still have food remaining in my mouth after several attempts to
swallow.
7. I pocket food on either side of my mouth.
8. I dont enjoy eating anymore.
9. I cough before, during, and after I swallow.
10. I choke often when I eat food.
19
11. I choke or gag when I drink liquids.
12. I have thick or excess saliva or phlegm.
13. I drool sometimes.
14. I have problems chewing.
15. I have to clear my throat often.
16. Sometimes I have problems breathing.
17. I get pneumonia more than once a year.
18. Food sticks in my throat.
19. Food sticks in my mouth.
20. Food or liquid dribbles out of my mouth.
21. Food or liquid comes out of my nose.
22. I cough food or liquid out of my mouth when it gets stuck.
23. Figuring out what I can or cannot eat is a problem for me.
24. Its difficult to find food that I both like and can eat.
25. People have a hard time understanding me.
26. My voice sounds gurgly.
27. Its been difficult for me to speak clearly.
28. I fear I may start choking when I eat food.
29. I am afraid of choking when I drink liquids.
30. I never know when Im going to choke.
The following is a checklist for the caregiver to help identify if there are
issues
with swallowing in the person with PD.
1. My partner with PD seems uninterested in food.
2. He/she coughs during meals.
3. He/she takes longer to eat a meal than previously.
What is a swallowing evaluation?
A swallowing evaluation includes a radiologist and SLPs examination.
This exam

could include an x-ray in motion, or videofluoroscopy, to detect the


specific
location of problems in the swallowing mechanism. Fiberoptic endoscopic
evaluation of swallowing (FEES) is another type of test that could be
performed
during a swallowing exam. It is a procedure that allows for the direct
viewing of
swallowing function when regular food materials are eaten. The FEES is a
separate
procedure, but may be used in conjunction with videofluoroscopy.
During the videofluoroscopy, the patient will be asked to eat and drink
various
consistencies (pudding, water, milk, cookies, etc.). The x-rays will be
taken during
the chewing and swallowing process.
For the FEES examination, a very thin flexible fiberoptic tube will be
passed
through the nasal passage that is hooked up to a camera and light
source. The tube
does not go down the throat, but provides a bright light so the
swallowing can be
20
observed. This procedure is painless and well tolerated by most
individuals.
Following a complete swallowing evaluation, an intervention program is
established for the patient and family support members.
Recommendations may
include:
Best feeding techniques.
Exercises to strengthen oral and throat muscles.
Compensatory techniques to assist in safe swallowing.
Oral and mouth care techniques.
Recommendations for modification of food texture.
Safe positioning strategies.
Patient/family education.
Assessment of the adequacy of swallowing.
Swallowing Food and Liquids
Below are techniques to make eating easier and safer:
Sit upright during all eating and drinking, even when taking pills.
Tilt the head slightly forward, not backward, as you swallow.
Take small bites of food, chew thoroughly, and do not add any more
food
until everything from the first bite has been swallowed.
Take small sips of liquid. Hold the liquid in the mouth for a short time
to
prepare for the swallow.

Concentrate on moving the food backward in the mouth with the


tongue.
Double swallow (swallow a second time) if the food did not go down
completely with the first swallow. Sometimes taking a sip of liquid
between bites of food can help to wash the food down.
If eating is very tiring, try several smaller meals spaced out during the
day instead of three large meals. Nutritional supplements periodically
during the day may be helpful in keeping calorie count high enough for
good energy level.
Consider selecting the following types of foods to help ease the
swallowing
process. Choosing the right kinds of foods will also help in making eating
easier,
safer and more pleasurable. Choose foods that dont require vigorous
chewing.
Texture should be slippery rather than dry and crumbly.
Moderate textured wheat breads instead of very coarse, nutty breads
or
very soft, white breads.
Oatmeal, cream of wheat or moistened dry cereals instead of very
coarse,
dry cereals.
Well-cooked, tender chicken/turkey, well-cooked fish without bones,
chopped and ground meats, instead of stringy, tough meats that require
a
lot of chewing.
21
Soft casseroles and poached or scrambled eggs instead of fried eggs.
Mashed potatoes or rice, moistened with gravy or margarine, instead
of
wild rice or French fried potatoes.
Soft, cooked pasta elbows, instead of long, dry spaghetti.
Soft, well cooked vegetables, cut up or creamed, instead of raw
vegetables
or those with a hard texture.
Pureed or mashed fruits, fruit juices and fruit sauces, instead of fruits
with seeds or hard outer skins.
Custard, yogurt, ice cream or other soft desserts, without pineapple,
nuts,
seeds or coconut.
Nutritional Risk Factors
When a person is sick or suffering from a debilitating disease process it
changes
his/her ability to accomplish tasks, which appear easy to a healthy
person. Persons

with PD are often sedentary, their activity levels drop and the need and
energy for
eating can diminish. The drop in energy can lead to a lack of desire to
prepare
foods, thus reducing the amount of food eaten and the nutritional
consistency.
This, along with difficulty chewing foods and swallowing them, can lead
to
substantial reductions in weight. Coupled possibly with dietary restriction
due to
medication use and/or other drug side effects such as nausea, vomiting
and
constipation can place the person with PD at high-risk for malnutrition.
Nutritional Suggestions
(http://www.wemove.org/par_nphm.html)
Eat a balanced diet, including all food groups
Consume sufficient calories to maintain weight
Consume adequate fiber and fluids to avoid constipation
Take vitamin D and calcium to prevent osteoporosis
Reduce protein to minimum daily allowance Take it with
the evening meal
Drooling
Poor control of saliva is known as sialorrhea. The reason that persons
with PD
have problems controlling saliva is because, in some cases, the muscles
of the oral
cavity, face, and neck have less control than normal. Sometimes there is
excess
saliva in the mouth because swallowing is less frequent. Problems
encountered
with drooling may be due to the fact that swallowing is less frequent or
there is a
delay in the persons ability to trigger the swallowing process. There are
certain
drugs that can be prescribed to help reduce the amount of saliva
produced, but one
should not take a drug that will result in completely drying the mouth
(Dworkin &
Nadal 1991). Close communication with a primary care physician or
neurologist
is recommended in order to best treat this condition.
The following techniques may help control the saliva that is not being
controlled
adequately:
Keep the chin up and the lips closed when not speaking or eating.
Do lip & tongue exercises (see Appendix C & D) to strengthen the lip

muscles.
22
Swallow often.
Always swallow before starting to speak and as often as needed while
speaking.
Avoid sugary foods because they cause more saliva to develop.
Chew food well.
Ask a doctor about medications that may help the problem.
Have family members learn the Heimlich maneuver.
23
HEARING
Hearing problems are not directly caused by PD but certainly are
associated with
aging. Hearing loss can be experienced by the person with PD or affect
the spouse
or caregiver of the person with PD. Approximately one in every ten
people in this
country has a hearing loss. Projections indicate that this number and
percentage
will grow as baby boomers age and increasing noise pollution continues
to take its
toll. Hearing loss affects everyone that needs to interact with the
hearing impaired
individual. Poor hearing seriously interferes with communications and
when
compounded with the difficulties that persons with PD already have with
their
speaking it can significantly diminish the communication interaction. The
most
common type of hearing loss that occurs with age is called a
sensorineural hearing
loss. A sensorineural hearing loss is caused by damage to the inner ear
and/or the
auditory nerve. Noise, certain medications, as well as age can contribute
to
this permanent hearing loss. Most sensorineural hearing losses can be
treated
effectively with hearing aids. If you suspect a hearing loss or are the
spouse or
caregiver of a person with PD, seek a professional consultation with an
audiologist.
An audiologist is a professional who specializes in hearing evaluation and
treatment.
The techniques below will be helpful when communicating with someone
who has
difficulty hearing:

Always let the listener see the face of the speaker. A distance of three
to
six feet between speaker and listener is best.
Do not speak to a person who has difficulty hearing from another
room.
Get his or her attention before you start to speak.
Dont try to communicate in a noisy environment. Turn off the radio or
the TV or go to a quiet place for conversation.
Dont shout. Raise the volume a little and speak clearly.
Never talk directly into a hearing impaired persons ear.
Keep the rate of speech slow.
Repeat the message, if needed. If the person with hearing impairmen
does not appear to understand what is being said, rephrase the
statement
rather than simply repeating the misunderstood words.
Above all, treat the person with hearing impairment as an adult, and
particularly those that are elderly, with respect.
24
Types of Hearing Aids
There are four basic styles of hearing aids.
Behind-The-Ear (BTE) hearing aids are extremely flexible and work for all
types
of hearing loss. The hearing device is housed within a curved shell that
sits behind
each ear and delivers sound through a clear tube. The clear tube fits into
a mold
that has been customized to comfortably fit inside each ear.
In-The-Ear (ITE) hearing aids are very easy to operate, even if the user
has poor
control of the hands. These are the most widely recommended hearing
aid style.
The hearing device is housed within a custom-made shell that fits
comfortably
inside each ear and delivers sound directly to the ear.
In-The-Canal (ITC) hearing instruments can barely be seen and are very
easy to
operate, even if the user has poor dexterity. The hearing device is
housed within a
custom-made shell that fits comfortably inside each ear canal and
delivers sound
directly to the ear.
Completely-In-The-Canal (CIC) hearing aids are the newest and smallest
type,
virtually invisible. The hearing device is housed in a tiny shell that fits
comfortably

and completely into each ear canal. The device is removed from the ear
canal
by pulling a tiny cord.
Open-Fit Hearing Devices are new technology hearing aids that are
similar to
BTE, however, they are used with patients with close to normal low
frequencies
and a sloping high frequency hearing loss. The device has a wire that
travels down
into the ear canal making it less noticeable and leaving the natural
sound to be
routed through the ear canal.
25
BODY LANGUAGE
Not all communication takes place with speech and words. People also
communicate
with each other by their body language and gestures. Facial masking, or
lack
of facial expression that is common in those with PD, is a result of
rigidity and
reduced range of movement in the muscles of the face. Some friends
and family
members report that the listener who displays no facial expression does
not seem
interested in their conversation. Facial expressions, as well as other
nonverbal
gestures, contribute to and enhance the communication process. We can
use facial
expressions, head movements, hand and arm gestures, and body
posture and body
position to clarify or add to what we are saying.
Using body language in communication can be a special challenge to
people who
have PD because of facial masking, slowness of body movements and
rigidity of
muscles. Practice body language and gestures and then make a point of
using them
when you are talking.
Practice and remember the following:
Eye contact is probably the most important type of non-spoken
communication.
Always make eye contact when you are talking. Making eye
contact shows that you are interested in communicating and in the
listener.
Practice exaggerating facial expressions. What does the face feel and

look like when expressing emotions such as happiness, sadness, worry


or
concern? Practice these facial expressions: raise the eyebrows, wrinkle
the forehead, open the eyes widely, squint the eyes, smile broadly,
purse
the lips. Use these movements of facial muscles when talking.
Keep the lips closed when listening. Having lips closed indicates
concentration
and attention.
Use body posture to show feelings when talking. Leaning slightly
forward in the chair shows interest; leaning back in the chair may show
that a state of relaxation and comfort. Leaning back and breaking eye
contact can show that a lack of interest in communicating or that the
communication should stop.
Use gestures to add to the spoken message. Shrug the shoulders,
clasp the
hands, turn the head, raise the arms.
Simply pointing to what is being talked about can be effective ways to
add to the spoken message.
26
MEDICATION EFFECTS ON SPEECH AND VOICE
While medications are the primary tool for management of PD, the
effects of those
pharmacological agents on speech, voice and swallowing deficits have
been
difficult to measure. Additionally, there are some side effects of PD
medications
that may negatively impact voice, speech and swallowing functions. It is
of
extreme importance that persons with PD realize the cycle of their
medication in
terms of peak and off peak effects that accompany the cycles of
medicine regimen.
Helpful Strategies:
Be aware of the on cycle of PD medications as the voice, speech and
swallowing will be at its peak performance.
Avoid too much vocal communication when tired or fatigued, or during
the off cycle of the PD medications.
Schedule important phone calls and other conversational interaction
during the on-cycle of the medication regimen.
Take time to rest between multiple phone calls and important
communication
interactions.
27
APPENDICES
A. Voice Strategies: A Home Practice Routine

Integrating family members or friends is an important part of the


therapy process.
Having this type of support makes it easier to carryover the therapeutic
strategies
from a treatment session to daily activities. Also, family and friends can
lend a
hand by reminding you when the voice is not loud enough or clear
enough.
Sometimes a simple hand gesture can be a reminder to increase the
loudness.
These helpful reminders can help motivate a change in communication
effectiveness.
Below are some homework routines that can be completed. Its helpful
to
be consistent, therefore try to choose a time of day to practice these
exercises for
10 minutes without interruptions. You may wish to practice once in the
morning
and once in the afternoon. If you are unable to practice with a partner
who can
provide feedback then try to practice with a recording device so that you
can play
back and listen to the voice and speech production.
1. Take a big deep breath and say the vowel ah in a loud voice. Try to
hold
the vowel as long as possible;
2. Repeat #1, except this time glide from the lowest possible tone (pitch)
to
the highest tone (pitch). Keep the voice loud and steady;
3. Practice reading short phrases, sentences and reading paragraphs in a
loud, high energy voice.
4. Try talking in conversation in a high-energy voice;
5. Try making phone calls to friends or family members and be sure to
use
a high energy voice, be aware of the number of times repetition is
requested;
6. While driving or riding in a car, practice saying aloud the street signs
or
places passed
7. Read short books or newspaper articles out loud. Read books to
children
or grandchildren in a loud voice.
28
B. Exercises for speech practice
Single words
Phrases

Sentences
Paragraphs
Start with the shortest speech material first, and then move to the
longer and more
complicated exercises. Work independently or with someone else, if
possible.
Read the words, phrases or sentences aloud or repeat them after
someone else. It
doesnt help if the material is read silently.
Use a tape recorder so progress can be listened to.
Try to use all of the principles from this booklet when practicing.
Make up independent speech practice material for more variety.
Rememberthe best practice is using speech!
Words and Functional Phrases. *When reading aloud, hold paper
upright
in order to project the voice.
Hello Good-bye Good morning
Goodnight How are you? See ya later
Who is this? This is ___________ Whats up?
Help me! Thank you No thank you
Thats okay Very well, thanks Youre welcome
Sure I hope so No
Take care! Im ready Not today
Can you get that Youre kidding? Never mind
Why do you ask? Do you see it? Im not sure
Could you get that? Do you know him/her Happy Birthday
Not too good Its over there Wheres the paper
Please go to the store Better than before Its time to go
Whos on the phone I have to go to the bathroom I am not feeling well
29
Functional Sentences
Lets go out tonight.
How was your day?
Lets have chicken for dinner tonight.
It was nice to meet you.
Could you help me with this?
Did you get that?
I dont need any help.
Where are you going?
Who was that?
Have you met ___________?
Have a good day!
What time is it?
Please pass the __________.
Would you do that for me?
Did ________ call today?

This is my friend, __________.


Its so good to see you!
Did you lock the door?
That was so nice of you!
Could you turn off the T.V.?
Are all of the windows closed?
Is the air conditioning on?
What do you have to drink?
Id like to order a pizza.
What time will you be home?
Whats the weather like outside?
Im hungry. How about you?
Turn off the lights when you come up.
I cant find my glasses. Have you seen them?
I was wondering what time you would be home.
Could you direct me to the restrooms?
Would you like a cup of coffee? I could go for one.
30
Paragraphs
1. Bird watching can be fun and a rewarding hobby. All that is needed to
get
started is some type of bird feeder, some birdseed, a window to watch
from and
patience. Once the birds discover your bird feeder, they pass the word
along to
other birds. Before long, your patio or deck can become a regular stop
for the
neighborhood birds. Look for chickadees, nuthatches, blue jays,
sparrows, and
wrens in the summer. More unusual birds like goldfinches and bluebirds
may also
appear. In the winter enjoy the bright red of the cardinal or the black and
white
juncos.
2. Your library card can be your ticket to entertainment, current events
and new
ideas. Almost every city has a public library and there is no charge for a
library
card. Libraries have books about many subjects, but there are also other
things at
the library. These include books on cassette tapes or CDs, videotapes,
large print
books, music CDs or tapes and magazines. Many have free programs in
the afternoon
or evening about travel, hobbies or other topics of interest. Some cities
also

have a bookmobile service, which brings the library right into your
neighborhood.
3. Any magazine or newspaper can be used to find additional and
interesting
reading material
31
C. Lip Movements
These exercises will help increase the flexibility and strength of the lips.
This will
help both speech and eating. Practice in front of a mirror. Always keep
the lips
closed when you are not eating or speaking. This will help maintain lip
strength.
Rub the lips together firmly.
Smack the lips. Try to make a loud smacking sound.
Pucker the lips-as for a kiss-then relax.
Alternate smiling and lip puckering. Saying the sounds eee-ooo may
make this easier to do.
Practice whistling.
When with family and friends, SMILE.
D. Tongue Movements
The tongue is a muscle. It needs exercise to improve strength and
flexibility. This
will help when speaking and eating. Use a mirror during practice so to
see and feel
what is being done. Repeat each exercise 5 to 10 times or as often as
possible
Stick the tongue out and pull it back in;
Stick the tongue straight out and push it against resistance, such as
the
back of a spoon;
Stick the tongue straight out and move it slowly from corner to corner
of
the mouth;
Do the same movement inside the mouth, alternating pushing against
each cheek;
Open the mouth and raise the tongue tip up to the ridge behind the
front
teeth. Bring the tongue down to rest;
Raise the tongue tip up to the roof of the mouth and pull it back along
the
roof;
Bring the tongue down to rest;
Lick all of the way around the lips, making a smooth, even circle with
the
tongue.

32