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Acute Myelopathies

Darrell Laudate
12/4/09 AM Report
Overview of Acute Myelopathy

Spinal cord dysfunction or myelopathy, can occur


due to a lesion arising within the spinal cord or due to
compression of the spinal cord originating outside of
it

Frequently devastating, often producing quadriplegia,


paraplegia, and sensory deficits

Many spinal cord diseases are reversible if


recognized and treated at an early stage
Transverse Myelopathy vs. Myelitis

Transverse Myelopathy refers to clinical presentation


of severe motor, sensory, and autonomic dysfunction
(bowel, bladder, and sexual abnormalities ) below a
spinal cord lesion due to any acute/ subacute process
affecting the spinal cord, compressive or not
Often associated with back pain

Transverse Myelitis refers to an inflammatory process


of the grey and white matter of the spinal cord
Transverse Myelopathies (cont.)
Localization of the lesion depends upon the level of the spinal cord involved and
the extent of the involvement of the various long tracts. In some cases, there is
almost total paralysis and sensory loss below the level of the lesion, others only
partial loss

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

Imaging is indicated in all patient with suspicion for myelopathy


MRI is generally the most appropriate study as it images the spine,
paraspinal region, and spinal cord; also may reveal evidence of intrinsic
lesions
most patients with suspected cord compression should have total spinal
cord imaging
sole imaging of the lumber spine is often ordered with cord compression
suspicion, but recall the spinal cord ends at L1, thus visualization of the
spinal cord is not possible with a lumbar MRI alone
lumbar MRI is useful however to exclude cauda equina
compression (lower extremity weakness and sensory and
bladder disturbances without upper motor neuron signs)
Exceptions may include pts with upper and lower extremity symptoms as
cervical localization is likely -> Cervical MRI
Other Studies

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

Clinical criteria alone cannot distinguish spinal cord


compression and intrinsic cord lesions
Acute Viral Myelitis
Two Forms
Enteroviruses (poliovirus, coxsackie virus, and enterovirus 71), Flaviviruses
(West Nile virus and Japanese encephalitis virus) have been known to target
the gray matter (Anterior horn cells) of the spinal cord, producing acute lower
motor neuron disease.1
usually accompanied with fever, headache, and meningismus
produces asymmetrical flaccid weakness with reduced or absent reflexes
and few sensory symptoms or signs
MRI often shows hyperintensities in the anterior horns of the spinal cord
on T2-weighted imaging Cerebrospinal fluid (CSF) analysis
demonstrates a moderate pleocytosis

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

Treatment - Penicillin G 3 to 4 million units IV every four hours or 24 million


units continuous IV infusion for 10 to 14 days
Connective tissue Associated
Myelopathies
SLE
May be the initial feature but onset is usually present with other active lupus signs.
thought to be due to an arteritis, with resultant ischemic necrosis of the spinal cord
ANA, ds-DNA, anti-Sm, Anti-neuronal (may correlate with active CNS lupus)
Has been associated with antiphospholipid antibodies in some studies but not all. 5
Treatment: Prednisone (1.5 mg/kg per day), plasmapheresis, and cyclophosphamide. 6
Antiphospholipid antibodies a may also benefit with warfarin as well as steroids and immunosuppressive
treatment.7
Mixed connective tissue disease
Sjogren's syndrome (antibodies to the Ro/SSA or La/SSB)
Scleroderma (ANA, anti-Scl-70, anti-centromere (ACA), anti-RNA polymerase III, and anti-beta2-glycoprotein I
antibodies)
Ankylosing spondylitis
Acute myelopathy will typically occur in the setting of fracture of ankylosed spine or atlantoaxial-axial subluxation
cauda equina sydrome rare but associated with long standing disease
Rheumatoid arthritis
atlantoaxial subluxation, atlantoaxial impaction, and/or subaxial subluxation
Rarely associated with CNS vasculitis and more rarely with myelopathy from vasculitis
Sarcoid
Neurosarcoidosis
Typically occur perivascularly, but they can be extramedullary or
intramedullary, and can involve the cauda equina.
Occurs 5% of Sarcoid patients

MRI signal abnormalities are not specific


neurosarcoid lesions can appear similar to transverse myelitis or
can resemble a tumor

CSF profile consists of variable lymphocytic pleocytosis; oligoclonal


bands are present in one-third of case

Generally treated with corticosteroids and other immunomodulatory


agents and can improve
B12 Deficiency (subacute combined
degeneration of spinal cord)

Damage to peripheral nerves caused by demyelination and irreversible


nerve cell death.

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.)

Usually established by the presence of decreased Vit B12


level
in the cases of low-normal B12, the presence of elevated MMA
and homocysteine levels may be useful

Treatment: 1mg Cbl IM once daily for 1 week, followed by


1 mg IM every week for four weeks
if the underlying disorder persists, 1 mg every month
for the remainder of the patient's life.
Hypocuric Myelopathy (Copper
Deficiency)

Very similar to subacute combined degeneration


Progressive spasticity, severe gait abnormalities including ataxia,
and a neuropathy.
Also associated with anemia and neutropenia in certain patients

More common after gastric bypass, also with zinc supplementation

Diagnosis usually confirmed with low levels of serum copper are found
and often there is also a low level of serum ceruloplasmin

Symptoms are potentially reversible with copper supplementation and


reversal of underlying cause
Multiple Sclerosis

Most common autoimmune inflammatory demyelinating disease


of the CNS

Women of Northern European descent who are of child-bearing


age.

Histological examination of active plaques reveals perivascular


infiltration of lymphocytes (predominantly T cells) and
macrophages with occasional plasma cells. Perivascular and
interstitial edema may be prominent.
Multiple Sclerosis (cont.)
Multiple sclerosis (MS) is a clinical diagnosis. There are no clinical findings
that are unique to this disorder, but some are highly characteristic
Older criteria considered clinical characteristics and a number of
laboratory studies
these findings were then used to place patients in categories ranging
from clinically definite to laboratory supported definite to clinically
probable to laboratory supported probable MS
McDonald criteria focus on a demonstration with clinical, laboratory and
radiologic data of the dissemination of MS lesions in time and space, also
incorporated specific MRI findings into the diagnostic scheme

A diagnosis cannot be made until other possible conditions have been


ruled out and there is evidence of demyelinating events separated
anatomically and in time
Multiple Sclerosis (cont.)
CSF
oligoclonal bands using isoelectric focusing is the most important diagnostic CSF study
when determining a diagnosis of MS
2/3 will have normal leukocyte count
>50cells/microliter occurs only rarely and should raise suspicion of alternative etiology
Will also see elevation of the CSF immunoglobulin level relative to other protein
components, suggesting intrathecal synthesis, generally IgG
MRI
Cerebral or spinal plaques that are ovoid and hyperintense on proton density and T2-weighted
studies, and they are hypointense (if visible at all) on T1-weighted images.
Conventional T2-weighted MRI techniques may underestimate MS plaque size and
burden, Diffusion tensor imaging and MR spectroscopy may correct this
Evoked potentials (EPs): CNS electrical events generated by peripheral stimulation of a
sensory organ)
Can detect abnormal CNS function that may be clinically undetectable or help define the
anatomical site of the lesion in tracts not easily visualized by imaging (e.g., optic nerves,
dorsal columns).
Neuromyelitis optica (Devics Disease)

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

Criteria: Optic neuritis, Myelitis, and one of the following:


MRI evidence of a contiguous spinal cord lesion three or more segments in length, or
Seropositivity for NMO-IgG

Treatment: Steroids, Cytoxan, PLEX, +/- Rituximab


Cervical Spondylosis
Chronic degenerative and hypertraphic changes that involve intervertebral disks,
vertebral bodies, facet joints, and ligaments
if severe, can result in narrowing of cervical spinal canal and cause spinal
cord compression
In many case series, cervical spondylotic myelopathy is the most common
cause of myelopathy, particularly in older adults

Cervical Spondylotic Myelopathy


clinical syndrome associated with spondylosis but with spinal cord
dysfunction
commonly in pts > 55y, perhaps most common form of myelopathy
insidious onset of numbness, parathesias in upper extremities, spastic or
stiff-legged gait that is often not associated with pain
surgical decompression is generally used to treat if symptomatic, although
there is no evidence from RCT proving the efficacy of this therapy 7
Metastatic Disease to Spinal Cord
Oncologic emergency

usually requring treatment with corticosteroids (usually


dexamethasone), and emergent radiation therapy or surgery

indications for decompressive surgery for metastatic epidural spinal


cord compression includes: when tissue diagnosis is needed,
presence of spinal instability, or tumor is known to be radioresistant

One study showed patients who underwent anterior


decompressive surgical resection of metastatic epidural spinal
cord compression due to cancer had better ambulatory
outcomes than with radiation therapy alone.8
Epidural Abscess
Rare, occurring in only 1 patient per 10,000 admitted to the hospital.9
Most common pathogen is Staphylococcus aureus, which accounts for
about two-thirds of cases 9
Typically originate via contiguous spread from infections of skin and
soft tissues or as a complication of spinal surgery and other invasive
procedures, including indwelling epidural catheters.
Expected back and/or radicular pain usually but not always
accompanied systemic signs of infection
MRI preferred test
Requires emergent surgical decompression and antibiotic therapy are
indicated to treat epidural abscess
Spinal Cord Infarction
Rare compared with CVA
Most frequently caused by surgical procedures and pathologies
affecting the aorta
May also occur in the setting of vascular risk factors or aortic
disease
Presents with sudden spinal cord dysfunction that typically
corresponds to the territory of the anterior spinal artery
Weakness and pinprick loss below the level of the infarction but
sparing vibration and position sense

No treatment available and prognosis is variable and dependent upon


severity of presenting deficit
Dural AVM of Spinal Cord

Rare cause of ischemic spinal cord dysfunction


Obstructs venous outflow of the spinal cord
May progress over months to years

Surgical obliteration of the fistula can potentially


reverse this condition
References
1) Solomon et al. Infectious causes of acute flaccid paralysis. Curr Opin Infect Dis. 2003 Oct;16(5):375-81

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.