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Darrell Laudate
12/4/09 AM Report
Overview of Acute Myelopathy
If cervical area is involved, all four limbs may be involved and there is risk of
respiratory paralysis (segments C3,4,5 to diaphragm)
Lhermitte's sign: an electric shock-like sensation down the neck, back,
or extremities that occurs with bending of the neck
Lesions of the lower cervical (C2-T1) region will cause a combination of
upper and lower motor neuron signs in the upper limbs, and exclusively
upper motor neuron signs in the lower limbs.
A lesion of the thoracic spinal cord (T1-12) will produce a spastic paraplegia.
A lesion of the lower part of the spinal cord (L1-S5) often produces a
combination of upper and lower motor neuron signs in the lower limbs
Upper vs Lower Motor Neuron Lesions
Lower motor neuron lesion is a lesion which affects nerve fibers traveling from the anterior horn of
the spinal cord to the relevant muscle(s)
Associated with areflexia
leads to flaccid paralysis (paralysis accompanied by muscle loss)
Upper motor neuron lesion is a lesion of the neural pathway above the anterior horn cell or motor
nuclei of the cranial nerves and are marked by:
Spasticity, increase in tone in the extensor muscles (lower limbs) or flexor muscles (upper limbs)
Clasp-knife response where initial resistance to movement is followed by relaxation
Weakness in the flexors (lower limbs) or extensors (upper limbs), but no muscle wasting
Brisk tendon jerk reflexes
Babinski or Hoffman sign is present
increase deep tendon reflex
Pronator drift
(Spinal Shock - loss of sensation accompanied by motor paralysis with initial loss but gradual recovery
of reflexes
Occurs following a spinal cord injury, thus what may have looked like a lower motor neuron lesion
can later reveal itself to be an upper motor lesion)
Important Dermatomal Landmarks
C2 - posterior half of the skull cap
C3 - area correlating to a high turtle neck shirt
C4 - area correlating to a low-collar shirt
C6 - (radial nerve) 1st digit (thumb)
C7 - (median nerve) 2nd and 3rd digit
C8 - (ulnar nerve) 4th and 5th digit, also the funny bone
T4 - nipples.
T5 - Inframammary fold.
T6/T7 - xiphoid process.
T10 - umbilicus (important for early appendicitis pain)
T12 - pubic bone area.
L1 - inguinal ligament
L4 - includes the knee caps
Evaluation of suspected myelopathy
Lumbar Puncture
Serologic Studies as clinically indicated
CT myelogram may be beneficial in
patients with suspected inflammatory
or demyelinative lesions of the spinal
cord
Noncompressive Myelopathies
Vascular
Arteriovenous malformation
Antiphospholipid syndrome and other hypercoagulable states
Inflammatory
Multiple sclerosis
Neuromyelitis optica (Devics Disease)
Transverse myelitis (idiopathic)
Sarcoidosis
Vasculitis
Infectious/Postinfectious
Viral: VZV, HSV-1 & -2, CMV, HIV, HTLV-I, enteroviruses, flaivaviruses
Bacterial and mycobacterial: Borrelia, Listeria, syphilis, Mycoplasma pneumoniae
Parasitic: schistosomiasis, toxoplasmosis
Metabolic
Vitamin B12 deficiency (subacute combined degeneration)
Copper deficiency
Compressive Myelopathies
Cervical spondylosis
Epidural, intradural, or intramedullary neoplasm
Epidural abscess
Epidural hemorrhage/hematoma
Herniated disc
Posttraumatic compression by fractured or displaced vertebra or
hemorrhage
These features help to distinguish this form of viral myelitis from Guillain-
Barr syndrome, which usually produces symmetric deficits, with no MRI
abnormalities, and is associated with elevated CSF protein levels without
pleocytosis.
Acute Viral Myelitis
CMV, VZV, HSV I &II, HCV, and EBV are associated with a second
form of viral myelitis has clinical and diagnostic test features that
are similar to transverse myelitis
Association between the myelitis and the virus is not always
clear, some may represent post-infectious transverse myelitis,
others, a positive polymerase chain reaction (PCR) test in the
CSF suggests that the myelitis is directly related to the viral
infection
Treated with Herpes zoster, HSV, and EBV myelitis are treated
with intravenous acyclovir (10 mg/kg q8h) or oral valacyclovir (2
gm tid) for 1014 days; CMV with ganciclovir (5 mg/kg IV bid)
plus foscarnet (60 mg/kg IV tid), or cidofovir (5 mg/kg per week
for 2 weeks).2
Other Infectious Myelopathies
HIV
More of a chronic myelopathy, Often found mostly in late stages of AIDS and associated with AIDS
related dementia in half
slowly progressive spastic paraparesis is accompanied by loss of vibration and position sense and
urinary frequency, urgency, and incontinence
CSF may show nonspecific protein elevation
ART may reverse the symptoms3
Bacterial
Mycoplasma (acute and post infectious), Listeria monocytogenes
TB
via secondary cord compression from verterbral osteomyelitis, aka Potts disease
Also via compressive tuberculomas
Lyme disease
Cases have been described in which clinical and MRI features resembling acute transverse
myelitis have been attributed to Lyme disease. 4
CSF in these cases typically demonstrates a lymphocytic pleocytosis and elevated protein
Schistosomiasis (in endemic areas)
Tabes Dorsalis (Locomotor Ataxia)
Form of tertiary neurosyphilis in which the nerves of the dorsal (or posterior)
columns degenerate
Loss of sense of position (proprioception), vibration, and discriminative touch
Latency period of 3-20 years
Cardinal signs of tabes are loss of reflexes in the legs; impaired position and
vibratory sense; Romberg's sign
also:
bilateral Argyll Robertson pupils
fleeting and repetitive lancinating pains, primarily in the legs
Paresthesias/ formincation
visceral crisis (Bladder disturbances, and acute abdominal pain with vomiting
personality changes, dementia, deafness, visual
skeletal musculature is hypotonic due to destruction of the sensory limb of the
spindle reflex
Ataxia of the legs and (tabetic) gait due to loss of position sense occurs in half of
patients
Tabes dorsalis (cont.)
VDRL & RPR (nontreponemal tests) may be nonreactive in late neurosyphilis
If suspcion suspicion for neurosyphilis, serum FTA-ABS or TPPA (treponemal
tests) are preferred
CSF may be completely normal in tabes dorsalis, or may show mild
lymphocytic pleocytosis with 10 to 50 cells/microL and protein concentrations
of 45 to 75 mg/dL.
Syphilitic meningovascular myelitis can represent an earlier form of syphilis
infection
focal inflammation of the meninges can secondarily affect the adjacent
anterior spinal artery thus result in a CVA or spinal cord infarction
Symptoms include
paresthesias in the hands and feet
loss of vibration and position sensation
progressive spastic and ataxic weakness
Loss of reflexes due to an associated peripheral neuropathy in a patient
who also has Babinski signs, is an important diagnostic clue
Optic atrophy and irritability or other mental changes may be prominent
in advanced cases
This myelopathy tends to be diffuse rather than focal; signs are generally
symmetric and reflect predominant involvement of the posterior and lateral
tracts, including Romberg's sign
B12 deficiency (cont.)
Diagnosis usually confirmed with low levels of serum copper are found
and often there is also a low level of serum ceruloplasmin
Autoimmune, inflammatory disorder in which the optic nerves and spinal cord are targeted but may
also affect the brain,
Resembles multiple sclerosis (MS) as it has varying degrees of weakness or paralysis in the legs or
arms, loss of sensation (including blindness), and/or bladder and bowel dysfunction.
Lesions are different from those observed in MS in that the attacks appear to be not mediated by
the immune system's T cells but rather by antibodies called NMO-IgG that target aquaporin 4 of
astrocytes.6
Acts as a channel for the transport of water across the cell membrane
Found in the processes of the astrocytes that surround the blood-brain barrier,
This blood-brain barrier is weakened in Devic's disease, but it is unclear how NMO-IgG
immune response leads to demyelination
2) Hauser et al. "Chapter 372. Diseases of the Spinal Cord" (Chapter): Harrison's Principles of Internal Medicine, 17e:
http://www.accessmedicine.com/content.aspx?aID=2904373.
3) Meurs et al. Acute transverse myelitis as a main manifestation of early stage II neuroborreliosis in two patients. Eur Neurol 2004;
52:186.
4) Staudinger et al. Remission of HIV myelopathy after highly active antiretroviral therapy. Neurology 2000; 54:267.
5) Lennon et al. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J. Exp. Med. 2005. 202 (4): 473
7.
6) Transverse myelitis as the first manifestation of systemic lupus erythematosus or lupus-like disease: good functional outcome and
relevance of antiphospholipid antibodies. AU D'Cruz DP; Mellor-Pita S; Joven B; Sanna G; Allanson J; Taylor J; Khamashta MA;
Hughes GR SO J Rheumatol 2004 Feb;31(2):280-5.
7) Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature. AU Kovacs B; Lafferty
TL; Brent LH; DeHoratius RJ SO Ann Rheum Dis 2000 Feb;59(2):120-4.
8) Acute transverse myelopathy in systemic lupus erythematosus: clinical presentation, treatment, and outcome. AU Mok CC; Lau CS;
Chan EY; Wong RW SO J Rheumatol 1998 Mar;25(3):467-73
9) Porter et al. Endocrine and reproductive manifestations of sarcoidosis. QJM 2003; 96:553.
10) McCormick et al. Cervical Spndylotic myelopathy: make the difficult diagnosis, then refer to surgery. Cleve Clin J Med. 2003;70:899-
904
11) Patchell et al. Direct decompressive surgical resection in the treatment of spinal cord compression caused by metastatic cancer: a
randomised trial. Lancet. 2005;366:643.
12) Darouiche, RO. Spinal epidural abscess. N Engl J Med 2006; 355:2012.