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Potassium channel blocker 4‐aminopyridine is effective in interictal cerebellar symptoms in episodic ataxia type 2 — A video case report
Author(s) -
Löhle Matthias,
Schrempf Wiebke,
Wolz Martin,
Reichmann Heinz,
Storch Alexander
Publication year - 2008
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.22071
Subject(s) - ataxia , vertigo , gait ataxia , cerebellar ataxia , cerebellum , medicine , intention tremor , 4 aminopyridine , psychology , pediatrics , psychiatry , surgery , potassium channel
Episodic ataxia type 2 (EA2) is an autosomal‐dominant hereditary disorder clinically characterized by recurrent attacks of vertigo, imbalance and ataxia. Studies have shown that 4‐aminopyridine (4‐AP) is capable to prevent these attacks. However, there are no reports whether 4‐AP is able to attenuate interictal cerebellar ataxia. Using the scale for assessment and rating of ataxia (SARA), we examined the efficacy of 4‐AP on interictal ataxia in a 63‐year‐old female patient who suffered from EA2 since the age of 57. EA2 was diagnosed based on clinical criteria and not genetically proven. When treatment with 4‐AP was paused the patient was suffering from marked gait and stance ataxia. After re‐initiation of treatment with 5 mg 4‐AP t.i.d., there was pronounced improvement in gait and stance ataxia. Within 24 hours SARA score lowered from 8.5 to 4.5 points. We conclude that 4‐AP may be beneficial for interictal cerebellar ataxia in late onset EA2. © 2008 Movement Disorder Society