transverse myelitis emily o. jenkins md, pgy3 am report 12.18.09
Post on 19-Dec-2015
218 views
TRANSCRIPT
Transverse MyelitisEmily O. Jenkins MD, PGY3AM Report12.18.09
Transverse Myelitis (TM)
• Immune-mediated process results in neural injury to the spinal cord
• Varying degrees of weakness, sensory alterations and autonomic dysfunction
• Up to half of idiopathic cases will have a preceding respiratory or gastrointestinal illness
Multi-focal CNS
disease (eg. MS)
Systemic disease
(eg. SLE)
Idiopathic Entity
Spectrum of Neuroimmunologic Disorders
MUSCLE SPINAL CORD PERIPHERAL NERVE
BRAIN
Polymyositis Transverse myelitis
AIDP 1 MS
Dermatomyositis
Tropical spastic paraparesis
CIDP 2 Paraneoplastic encephalomyeli
tis
Myasthenia gravis
Stiff person syndrome
Hashimoto’s encephalomyeli
tis
Neuromyelitis optica
Rasmussen’s encephalomyeli
tis
ADEM 3
PANDAS 41. 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 TMDisease 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, Sjogren’s 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•Up to 20% will have
recurrent inflammatory episodes within the spinal cord
•Significant recovery is unlikely if no improvement by 3 months
Full recov-
ery
Moderate per-manent disabil-
ity
Severe permanent disability
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