Infections & Myelopathy

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INFECTIONS & MYELOPATHY

Abscess
AIDS
Central European encephalitis
CSF eosinophilia
HIV
HTLV I & II
Parasites
Poliomyelitis: Acute
Spirochete
Tuberculosis
Varicella zoster
West Nile
Whipple's

Neuromuscular Disorders

Tabes dorsalis

From Bramwell: Atlas of Clinical Medicine

Polio: Arm atrophy

Polio

Human susceptibility: 2° to Polio virus receptor
Events after viral exposure
- Asymptomatic: 90% to 95%
- Minor illness: 1 to 5 days after exposure
  - Fever
  - Malaise, Myalgia
  - Sore throat, GI upset
- Major illness: 4 to 10 days
  - Aseptic meningitis
  - Fever
  - Pain: Headache, stiff neck, back pain
- Paralytic disease: 50% of those with major illness
  - After 2 to 5 days of illness
  - Fasciculations: Localized
  - Pain: Intense myalgia, hyperesthesia
  - Weakness
    - Onset: Fulminent, Acute, or Subacute
    - Focal or Asymmetric
    - Legs > Arms > Bulbar (20%)
  - Acute dysautonomia
    - Blood pressure instability
    - Cardiac arrhythmia
    - GI: Atony, Constipation
    - Urinary retention
    - Sweating: Increased or Decreased
  - Differential diagnosis
    
    From Bramwell: Diseases of the Spinal Cord
    
    Polio: Small ventral horn
    - Viral: Anterior horn cell disease
      - Enterovirus 71
      - Coxsackie A & B
      - Echoviruses
      - West Nile
    - Immune: Acute neuropathies
    - Porphyria
    - Toxins
    - Myelitis
  - Lab
    - CSF pleocytosis: Acute - Polys; > 72 hours - lymphocytes
    - Serology: Serum 4-fold rise; CSF IgM poliovirus specific
    - Culture: Stool positive in 90% by 20 days
    - Electrodiagnostic: Selective loss of motor axons @ 7 to 10 days
Also see
- Post-polio syndrome
- External links: Polio virus image; PBS

Other Viruses

Varicella zoster

Transverse myelitis

Acquired Immunodeficiency Syndrome (AIDS)

R. Schmidt

HIV myelopathy

Cytomegalovirus
- Lumbosacral polyradiculopathy
HIV: Chronic myelopathy
- Frequency: 5% to 20% of late stage AIDS
- Onset: Late stages of AIDS
- Clinical
  - Weakness: Legs; Symmetric or asymmetic
  - Pyramidal signs: Leg spasticity
  - Urinary frequency/incontinence: May be presenting feature
  - Sensory loss: Mild
  - Associated CNS features: Dementia
  - Course: Progressive
- Pathology
  - Vacuolar
  - Prominent posterior column involvement

Human T-cell lymphotrophic virus, Type I (HTLV I)

Epidemiology
- Common in West Indies (Caribbean basin), Japan, Centrla Africa, Iranian Jews
- US risk groups
  - Intravenous drug abusers
  - Immigrants from high-risk areas
  - Often associated with coinfection with HTLV-II or HIV
  - Endemic in certain parts of the southeastern US (Native Amerindian population)
- Myelopathy in 1% of patients with infection
- Most infected patients are asymptomatic
- Transmission
  - Vertical transmission: Mother to child
    - Through breast-feeding: Especially past 6th month
    - Related to: High maternal provirus levels & High HTLV-I antibody titer
  - Sexual
    - More common male to female
    - Inefficient
  - Parenteral: Via lymphocytes
    - Blood
    - Organ transplantation
Clinical
- Myelopathy: Progressive spastic paraparesis
  - Nosology
    - Tropical spastic paraparesis
    - HTLV-1-associated myelopathy (HAM)
  - Onset
    - Age: 3rd to 5th decade
    - 20 to 30 years after initial infection
    - Abnormal gait
    - Slow development: Usual pattern; Over years
    - Rapid development: Reported after blood transfusion or organ transplantation
  - Clinical
    - Motor: Leg weakness
    - Pyramidal signs
      - Arms & Legs
      - Hyperreflexia
      - Spasticity
    - Symmetric
    - Autonomic: Urinary & fecal incontinence
    - Sensory: Minor changes
    - Other occasional neural features
      - Optic atrophy
      - Sensorineural hearing loss
      - Cerebellar symptoms
      - Cognitive deficits
  - Pathology
    - Chronic inflammation: Middle to lower thoracic cord
    - Spinal cord atrophy: Involvement of pyramidal tracts & posterior columns
    - Meningeal thickening
- Motor disorder: "ALS-like syndrome"; Often atypical features
  - Epidemiology: Described in few patients
  - Clinical: Variable syndromes
    - Pyramidal features
    - Lower motor neuron
      - Weakness: Often proximal
      - Muscle wasting
      - Fasciculations
    - Urinary incontinence
  - Pathology: Anterior gray horn of spinal cord; Ventral spinal nerve roots
  - EMG: Denervation, acute & chronic, in lower thoracic paraspinal muscles
- Polyneuropathy³
  - Frequency
    - Clinical: 1% to 6% of infected patients; ? More with associated myelopathy
    - Electrophysiological findings: Up to 40% to 50%
  - Clinical
    - Sensory loss: Distal legs
    - Tendon reflexes: Less at ankles than at knees
    - May occur in absence of myelopathy
    - Treatment: Reports of improvement when inflammation found on biopsy
  - NCV: Axon loss
    - Normal velocities
    - Mildly reduced SNAP amplitudes in legs
  - Pathology
    - Reduced density of large and small myelinated axons
    - Regenerating clusters
    - Inflammation: Perivascular; CD4 & CD8 cells
  - CSF: High protein; Pleocytosis
- Myopathy
  - Inclusion body myopathy
    - Weakness: Proximal
    - Course: Slowly progressive
    - Serum CK: Mildly elevated
    - Muscle biopsy: Myopathy; Vacuoles; Endomysial inflammation
  - Inflammatory myopathy
    - Variable association with HTLV-1 infection
    - Occcasional patients: Asymmetric proximal weakness
    - Serum CK: May be high
    - Treatment: Reports of improvement with corticosteroids
    - HAM/TSP patients: Inflammation in muscle may be found
- Other syndromes with HTLV-1 infection
  - Sjögren's syndrome
  - T-cell leukemia: Adult
  - Uveitis
  - Arthropathy & synovitis
  - Pulmonary abnormalities
  - Thyroiditis
Laboratory
- CSF: Mild increase in protein & cells; High IgG
- MRI
  - Spinal cord: Increased T2 signal; Cord atrophy
  - Subcortical: Leukoencephalopathy
- False positive: Lyme; Syphilis; Antiphospholipid antibody
Possible treatments
- Methylprednisolone, IV
- Interferon-alpha¹

Human T-cell lymphotrophic virus, Type II²

African-American, often Indian descent
Onset: 34 to 70 years
Clinical
- Most patients asymptomatic
- Spastic paraparesis or quadriparesis
- Spinal sensory loss
- Progressive
  - Non-ambulatory in < 5 years
  - Faster than HTLV I
CSF: ± high protein, ± oligoclonal bands

Spirochetes

Syphilis
- Meningovascular
- Arachnoiditis
- Tabes dorsalis
Lyme
- Spastic paraparasis
- Bladder dysfunction

Tuberculosis

Arachnoiditis & meningitis
- Often progresses to complete block
- Very high CSF protein
Tuberculoma: Paraspinal or Vertebral; Rarely intramedullary

Parasitic infections

Types
- Schistosomiasis (Trematode): Granulomatous intrathecal mass lesions
- Echinococcus granulosis (Cestode): Hydatid cysts
- Taenia solium: Leptomeningeal cysts
- Paragonimus westermani (Trematode): With pulmonary & cerebral disease
Laboratory: Eosinophilic cells in CSF

Abcesses

Locations
- Extradural
- Intramedullary (rare)
Associated with bacteremia

Whipple's disease

Organism: Tropheryma whippelii
Clinical
- Epidemiology
  - Middle age
  - Males > Females
  - More common in farmers or with expoure to dirt
  - Noth America & Europe
- Cervical myelopathy: Spastic quadriparesis
- Other signs of CNS Whipple's: May be absent
  - CNS
    - Dementia
    - Myoclonus
    - Ataxia
  - Eye: Supranuclear ophthalmoplegia; Visual loss; Nystagmus; Ptosis
  - Polyneuropathy: Rare
  - Oculofacial skeletal myorhythmia
  - GI signs: Diarrhea; Abdominal pain
  - Systemic: Arthralgias; Fever; Weight loss
- Progression: Months
Lab
- CSF: Often normal
- MRI: High signal in CNS, Spinal cord (cervical)& Medulla
- Diagnosis
  - PCR of small intestine cells
  - PAS positive GI mucosal cells
External link: Ophthalmic Whipple's

CSF eosinophilia

Helminthic infections
- Nematode: Rarely cause 1^o spinal disease
  - Angiostrongyliasis
  - Gnathostomiasis
  - Strongyloidiasis
  - Toxocariasis
  - Trichinosis
- Cestodes
  - Cysticercosis
  - Echinococcosis
- Trematodes
  - Paragonimiasis
  - Schistosomiasis
Lupus erythematosis
Sarcoidosis
Drug or contrast agent reactions
Hodgkin's lymphoma
Idiopathic hypereosinophilic syndrome

Also see Infections & Neuromuscular Disease
Return to Myelopathies
Return to Neuromuscular Home Page

1. Neurology 1996;46:1016-1021
2. Ann Neurol 1996;40:714-723
3. J Neurological Sci 2003;206:17–21, Semin Neurol 2005;25:315-327

4/4/2009