Transverse Myelitis

Emily O. Jenkins MD, PGY3

AM Report
12.18.09
Transverse Myelitis (TM)
Immune-mediated
process results in neural Multi-
injury to the spinal cord focal CNS
Varying degrees of disease
(eg. MS)
weakness, sensory Idiopathic
alterations and autonomic Entity
Systemic
dysfunction disease
Up to half of idiopathic (eg. SLE)
cases will have a
preceding respiratory or
gastrointestinal illness
Spectrum of Neuroimmunologic
Disorders
MUSCLE SPINAL CORD PERIPHERAL BRAIN
NERVE
Polymyositis Transverse AIDP 1 MS
myelitis
Dermatomyositis Tropical spastic CIDP 2 Paraneoplastic
paraparesis encephalomyelitis
Myasthenia gravis Stiff person Hashimotos
syndrome encephalomyelitis
Neuromyelitis Rasmussens
optica encephalomyelitis
ADEM 3
PANDAS 4
1. Acute inflammatory demyelinating polyneuropathy 2. Chronic inflammatory demyelinating polyneuropathy
3. Acute disseminated encephalomyelitis 4. pediatric autoimmune neuropsychiatric disorders associated with
streptococcal infections
TM: Incidence
Rare: Estimated between 1 and 8 cases per
million people per year
1400 new cases reported in US each year
Affects individuals of all ages with a bimodal
peak between ages 10-19 and 30-39
Presentation
50% will lose all movement in legs
Nearly all have some degree of bladder dysfunction
80-94% have numbness, paresthesias, or band-like
dysethesias
Autonomic symptoms may include: urgency,
incontinence, difficulty or inability to void,
incomplete evacuation of bowel and/or bladder,
sexual dysfunction
80% of patients reach clinical nadir within 10 days
of symptom onset
Thoracic spinal cord most typically involved in
adults, cervical spinal cord in children
TM Diagnostic Criteria
Alternative diagnostic considerations
B12 deficiency: slowly progressive
weakness, sensory ataxia, paresthesias
Radiation myelopathy
Hepatic myelopathy: rare neurologic
complication of chronic liver disease with
portal hypertension
Decompression sickness: complication of
deep sea diving
Neurolathyrism: prolonged consumption
of grass or chickling pea; slowly
developing paraparesis with paresthesias;
no treatment
Konzo: acute spastic paraparesis from
high exposure to cyanogenic compounds
in diets containing insufficiently
processed bitter cassava
Etiology
Acquired alteration in the innate or acquired immune system
Cellular injury and dysfunction
Infectious trigger: infectious agent triggers breakdown of
immune tolerance for self-antigens
TM and ADEM: Superantigen-mediated activation of T
lymphocytes
Suspected that multiple immune system components
contribute to observed dysfunction including T and B
lymphocytes, macrophages, and NK cells
Mechanism of injury also probably involves multiple
pathways including T lymphocyte killing of neural cells,
cytokine injury, activation of toxic microglial pathways,
immune-complex deposition, and apoptosis
 Diseases associated with TM
Disease Examples
Bacterial Infections Mycoplasma pneumoniae, Lyme borreliosis,
syphilis (tabes dorsalis), tuberculosis
Viral Infections herpes simplex, herpes zoster, cytomegalovirus,
Epstein-Barr virus, enteroviruses (poliomyelitis,
Coxsackie virus, echovirus), human T-cell,
leukemia virus, human immunodeficiency virus,
influenza, rabies
Post-Vaccination Rabies, cowpox
Autoimmune diseases SLE, Sjogrens syndrome, sarcoidosis
Multiple Sclerosis
Paraneoplastic syndromes
Vascular Thrombosis of spinal arteries, vasculitis secondary
to heroin abuse, spinal AVM
Distinguishing TM and GBS
TM and MS
TM can be the presenting feature of MS
Patients ultimately diagnosed with MS are more
likely to have:
asymmetric clinical findings
predominant sensory symptoms with relative
motor sparing
MRI findings extending over fewer than two
spinal segments
abnormal brain MRI
oligoclonal bands
Pathology
Treatment
No consensus guidelines
Mainstays include:
corticosteroids: no randomized trials
plasmapheresis: moderate to severe cases, or those
who do not respond to steroids after 3-5 days
Pulse dose IV cyclophosphamide
CSF filtration therapy: spinal fluid is filtered for
inflammatory factors (not available in US)
For severe, refractory cases: 2 year course of
azothioprine, methotrexate, mycophenolate, or oral
cyclophosphamide
Prognosis
Most will have
monophasic disease Severe
Full
Up to 20% will have recovery permanent
disability
recurrent inflammatory
episodes within the
spinal cord
Moderate
Significant recovery is permanent
unlikely if no disability
improvement by 3
months
Recurrence
Predictors of recurrence:
Multifocal lesions within the spinal cord
Demyelinating brain lesions
CSF oligoclonal bands
Mixed connective tissue disorder
SS-A antibodies
Persistently high IL-6 levels in CSF: thought to lead to high NO
production and subsequent neural injury
Predictors of poor outcome:
Initial complaint of back pain
Rapid progression to maximal symptoms within hours of onset
Spinal shock
14-3-3 protein, a marker of neuronal injury, in CSF during acute
phase