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A. Definition and Presentationnavigator

  1. Dysfunction of spinal cord at specific level(s), often due to inflammatory process
  2. Typically affects thoracic or lumbar levels, less commonly cervical
  3. Usually begins with paresthesias ascending from feet, leg weakness
  4. May include bladder and bowel disturbance
  5. Most patients are 20-40 years old
  6. Prognosis poorer with severe pain or rapid progression

B. Etiologynavigator

  1. Multiple Sclerosis
  2. Inflammatory Diseases
    1. Anti-Phospholipid Antibody Syndrome (APLS)
    2. Systemic Lupus Erythematosus (with APLS) [2]
    3. Neurosarcoidosis
  3. Infectious
    1. Lyme Disease of the CNS - Borellia burgdorferi
    2. Mycoplasma pneumoniae
    3. Atypical mycobacteria [5]
    4. Viral: EBV, CMV, rubella, hepatitis, measles, HIV, HTLV-1, VZV, poliovirus
    5. Spinal syphilis is very rare now
  4. Neoplasms
    1. Intramedullary - such as ependymoma
    2. Extramedullary - such as lymphoma
    3. Paraneoplastic Syndromes
  5. Drug Reaction - penicillin (rare)
  6. Anterior Spinal Artery Injury or Occlusion
    1. Frequently seen with occlusion of Artery of Adamkowitch
    2. Spares posterior columns
  7. Direct Compression
    1. Disc Disease
    2. Spondylosis
    3. Often associated with (congenital) spinal stenosis
  8. Differential [3]
    1. Syringomyelia - hereditary (usually) constriction of central canal
    2. ALS (Lou Gherig's Disease)
    3. Tangier Disease
    4. Inflammatory Polyneuropathies

C. Laboratory Evaluationnavigator

  1. Complete blood count, PT/PTT, ESR, ANA, and Anti-Cardiolipin Antibodies
  2. Specific Serologies: Mycoplasma and Lyme Titers, RPR (Syphilis Testing)
  3. Spinal Fluid: protein level often elevated, may show mononuclear cells (PMNs uncommon)
  4. Specific detection of infectious agent DNA by polymerase chain reaction (PCR)

D. Radiographic Studiesnavigator

  1. MRI is best study
  2. T2 (water bright) images show high signal along spinal cord (central inflammation)
  3. Typically does not enhance with Gadolinium except for very recent lesions
  4. Spinal angiogram can be performed to rule out tumor or spinal artery injury

E. Treatmentnavigator

  1. Mainly depends on cause
  2. Autoimmune
    1. Multiple sclerosis or vasculitis suspected
    2. 500-1000mg IV Methylprednisolone given daily x 3 days
  3. For CNS Lyme Disease, 1-2gm iv ceftriaxone qd usually given

F. HTLV-1 Associated Myelopathy [4] navigator

  1. Previously called Tropical Spastic Paraparesis
  2. Chronic progressive myelopathy and atrophy of spinal cord
  3. Subcortical white matter lesions and cerebrospinal fluid changes
  4. Immune mediated mechanisms likely play a role in myelin destruction
  5. Test patients with idiopathic progressive myelopathy for serum/CSF HTLV-1 Abs
  6. Major competing diagnosis is multiple sclerosis
  7. No current therapy available

References navigator

  1. Logigian EL and Murray MB. 1994. NEJM. 331(21):1437
  2. Lavalle C, et al. 1990. J Rheumatol. 17:34 abstract
  3. Siao PTC, Cros DP, Lees RS. 1996. NEJM. 334(21):1389 (Case Report)
  4. Levin MC, Lehky TJ, Flerlage AN, et al. 1997. NEJM. 336(12):839 abstract
  5. Von Reyn CF and Mark EJ. 2001. 345(17):1263 (Case Record)