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A 71 y/o woman with downward gaze palsy, dementia, and recurrent falls

Kam Newman, Hesam Tabrizi, Parham Naghedechi, Kia Javaheri, Saeed Kahkeshani
San Jacinto Methodist Hospital, Baytown, TX

Learning Objectives Case History Images Discussion


1. Understanding the differences between The patient is a 71 year old Caucasian female Patients with PSP have an akinetic rigid Parkinson-
progressive supranuclear paly (PSP) and with past history of walking problem and like syndrome, rest tremor is uncommon. The
parkinson disease (PD). dementia who was admitted hospital for altered symptoms consist of bradykinasia, decreased fine
mental status. Her problems started with motor skills, and hypophonia. Gait difficulties
2- Recognizing the movement disorder recurrent falls and the ability to walk gradually progress faster than in PD. Unlike typical PD, falls
patterns in PSP. declined to the point that she stopped walking begin within the first year. Presentation can range
completely about 5 to 6 month ago and she had to from unexpected falls, to visual (blurry or double
use wheel chair. She has been declining in her vision), cognitive (apathy, dementia), or
Oculocephalic maneuver demonstrates intact Parkinsonian symptoms with little or no tremor.
mental function since a couple of years ago, and
reflex downgaze in a patient unable to look down Compared with PD, PSP patients have more axial
was treating for Alzheimer disease for about a
Summary year. According to her family, she has also been
voluntarily. than appendicular rigidity, more dystonia and a
using diapers for several months. Also they have more symmetric exam. Weak voice with
noticed that during the past several month, her psudobulbar features can develop early;
Olszewski, Steele, and Richardson reviewed swallowing problem develop later. Downgaze is
arms became stiff and fingers flexed so that she
autopsied of patients who had a syndrome of affected before upgaze, whereas lateral movements
could not open her fist.
pseudobulbar palsy, supranuclear ocular of eye are usually preserved. Motor examination
On physical exam, patient stares at the examiner
palsy, extrapyramidal rigidity, gait ataxia, reveals axial hypertonia that is out of proportion to
all the time and is not communicative. She
and dementia.They found a consistent pattern limb tone, which may be normal in contrast to PD
recognizes some family members around her, but
of neuronal degeneration and neurofiblliray Patients. Eyelid opening and closing aparaxias are
completely disoriented to the place and time, and
tangles, chiefly affecting the pons and far more common in PSP than in any other
follows just some simple commands like
midbrain. It is clear that PSP continues to be extrapyramidal disorder. “Applause sign” helps to
squeezing fingers. Her speech is slow and New radiologic sign for the diagnosis of PSP.
an under-recognized disorder with multilevel discriminate PSP from FTD and PD. Pseudobulbar
somewhat slurred. She has a very poor eye Midsagittal MR image of a patient with PD (A) does
involvement of the neuraxis that helps palsy is an important symptom of PSP associateed
movement in all directions. In motor exam, she is not show any apparent abnormality, while that’s of a
differentiate it from other akinetic-rigid with high morbidity and decreased survival.
rigid in a generalized fashion, and her rigidity patient with PSP (B) shows marked atrophy of
syndromes such as PD. After PD, PSP is the The pathology of PSP is characterized by
was seen in all extremities and trunk. She holds midbrain tegmentum, and a patient with multiple-
most common form of Parkinsonism with a widespread neurodegeneration associated with tau
her right hand in a completely flexed position. system atrophy of the Parkinson type (MSA-P) shows
prevalence of 10% of that of PD. It is an protein deposition in subcortical regions that
Deep tendon reflexes are exaggerated in all four marked atrophy of pons (C). The midbrain to pons
increasingly recognized akinetic-rigid include the SN, globus pallidus, subthalamic
extremities. CBC, CMP, cardiac profile, LFT, ratio is always small in the patients with PSP. In pts
syndrome with features that may initially nucleus, midbrain, pontine reticular formation,
SPEP, RPR, CEA, and spinal tap all were with PSP, the shapes of midbrain tegmentum (bird’s
mimic those of idiopathic PD. The clinical dentate nucleus of the cerebellum and the superior
unremarkable. MRI of the brain revealed head) and pons (bird’s body) on midsagittal MR
hallmarks of PSP are vertical gaze palsy, cerebellar peduncle.
atrophy of the midbrain. images look like a lateral view of a standing penguin
psudobulbar palsy, axial rigidity, and Compared with PD, PSP pts drive a limited benefit
cognitive impairment. (especially the king penguin) with a small head and
Reference: big body. from dopaminomimetics; nonethless in individual
2. Clinical aspects of progressive supranuclear Recognition of this penguin silhouette sign should patients this benefit can be clinically important.
Acknowledgements
palsy. Handb of Clin Neurol. 2008; 89:461-73. strongly raise suspicion for the diagnosis of PSP. Beyond dopamine, GABA receptors seem to be
The authors wish to thank Dr. Hamid Iranmanesh, and Dr. Kaveh
Bahmanpour, Department of Family Medicine for their support. decreased in the frontal cortex of PSP patients.