Classification of cerebellar disorders

Classification of cerebellar disorders

How can cerebellar disorders be classified?

  • Congenital/developmental (Joubert syndrome, Dandy–Walker syndrome, Chiari malformations 

Chiari Malformations

TypeCharacteristics
IExtension of tonsils into foramen magnum without brain stem involvement
May be asymptomatic
IIExtension of tonsils and brain stem into foramen magnum, vermis may be partially complete or absent
Myelomeningocele is present
IIICerebellum and brain stem herniate through foramen magnum and into spinal canal
Can have occipital encephalocele with severe neurologic deficits
IVIncomplete or underdeveloped cerebellum
  • Acquired:
    • 1. Vascular (infarction [thrombotic > embolic] or ischemia; hemorrhage [from hypertension, vascular malformation, or tumor]; vertebral dissection; basilar migraine; vascular malformation; systemic vasculitides)
    • 2. Neoplasms (see later section)
    • 3. Infections (acute cerebellar ataxia of childhood [possibly viral]; tuberculosis or tuberculoma; cysticercosis; bacterial infection/abscess [extension of mastoid infection]; chronic panencephalitis of congenital rubella; viral encephalitis [involving cerebellum or brainstem]; Whipple’s disease)
    • 4. Inflammatory/autoimmune (multiple sclerosis, acute postinfectious cerebellitis, acute disseminated encephalomyelitis, Miller–Fisher variant of acute inflammatory polyneuropathy, anti-GAD-associated cerebellar ataxia, celiac and gluten ataxia)
    • 5. Paraneoplastic (paraneoplastic cerebellar degeneration [see later section]; opsoclonus–myoclonus [secondary to neuroblastoma])
    • 6. Metabolic (hypothyroidism, hyperthermia, hypoxia, deficiencies of thiamine [in those with alcoholic abuse], niacin [pellagra], vitamin E, essential amino acids, zinc)
    • 7. Drugs/toxins (antiepileptic drugs [phenytoin, carbamazepine, barbiturates]; chemotherapeutic agents [5-fluorouracil, cytosine arabinoside]; heavy metals [thallium, lead, organic mercury])
    • 8. Trauma (postconcussion syndrome, intracranial hematoma, brain contusion)
  • • Degenerative (multiple systems atrophy [cerebellar type], Creutzfeld–Jakob disease)
  • • Inherited/genetic (autosomal dominant, autosomal recessive, episodic mutations)
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