Episodic ataxia type 4 (EA4)

Evidence-based neurology checklist on episodic ataxia type 4 (ea4): Genetics Genetic mutation is unknown It causes late onset vertigo and ataxia: the onset age is 30-60 years Clinical features Treatment Synonyms

Genetics

  • Genetic mutation is unknown
  • It causes late onset vertigo and ataxia: the onset age is 30-60 years

Clinical features

Treatment

Synonyms

References

  1. Jen JC, Graves TD, Hess EJ, et al. Primary episodic ataxias: diagnosis, pathogenesis and treatment. Brain 2007; 130:2484-2493.
  2. Merrill MJ, Nai D, Ghosh P, Edwards NA, Hallett M, Ray-Chaudhury A. Neuropathology in a case of episodic ataxia type 4. Neuropathol Appl Neurobiol 2016; 42:296-300. 
  3. Damji KF, Allingham RR, Pollock SC, et al. Periodic vestibulocerebellar ataxia, an autosomal dominant ataxia with defective smooth pursuit, is genetically distinct from other autosomal dominant ataxias. Arch Neurol 1996; 53:338-344. 
  4. Farmer TW, Mustian VM. Vestibulocerebellar ataxia. A newly defined hereditary syndrome with periodic manifestations. Arch Neurol 1963; 8:471-480.
  5. Coin JT, Vance JM. Gabapentin relieves vertigo of periodic vestibulocerebellar ataxia: 3 cases and possible mechanism. Mov Disord 2021 (Online ahead of print).

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