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Differential Diagnosis of Nervous System Disorders

= progressive fatal disease characterized by destruction / alteration of gray and white matter

Etiology: genetic; viral infection; nutritional disorders (eg, anorexia nervosa, Cushing syndrome); immune system disorders (eg, AIDS); exposure to toxins (eg, CO); exposure to drugs (eg, alcohol, methotrexate + radiation)

Myelinoclastic / Demyelinating Disease!!navigator!!

= disease that destroys normally formed myelin

◊Usually affects older children / adults

  1. infectious
    1. Progressive multifocal leukoencephalopathy
    2. Subacute sclerosing panencephalitis (SSPE)
    3. Acute disseminated encephalomyelitis (ADEM)
  2. noninfectious
    1. Radiation
    2. Anoxia
    3. Hypertensive encephalopathy
    4. Disseminated necrotizing leukoencephalopathy (from methotrexate therapy)
  3. others
    1. Multiple sclerosis (most frequent of primary demyelinating disease)
    2. Alzheimer disease (most common of diffuse gray matter degenerative diseases)
    3. Parkinson disease (most common of subcortical degenerative disease)
    4. Creutzfeldt-Jakob disease
    5. Menkes disease (sex-linked recessive disorder of copper metabolism)
    6. Globoid cell leukodystrophy
    7. Spongiform degeneration
    8. Cockayne syndrome
    9. Spongiform leukoencephalopathy
    10. Myelinoclastic diffuse sclerosis (= Schilder disease)

Dysmyelinating Disease!!navigator!!

= metabolic disorder (= enzyme deficiency) resulting in deficient / absent myelin sheaths

  • Usually presents in first 2 years / 1st decade of life!
  • Associated with white matter atrophy
  1. macrencephalic:
    1. Alexander disease (frontal areas affected first)
    2. Canavan disease (white matter diffusely affected)
  2. hyperdense thalami, caudate nuclei, corona radiata
    1. Krabbe disease
  3. family history (X-linked recessive)
    1. X-linked adrenoleukodystrophy
    2. Pelizaeus-Merzbacher disease
  4. others
    1. Metachromatic leukodystrophy (most common hereditary leukodystrophy)
    2. Binswanger disease (SAE)
    3. Multi-infarct dementia (MID)
    4. Pick disease
    5. Huntington disease
    6. Wilson disease
    7. Reye syndrome
    8. Mineralizing microangiopathy
    9. Diffuse sclerosis

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