Potassium channel blocker 4-aminopyridine is effective in interictal cerebellar symptoms in episodic ataxia type 2 — A video case report
✍ Scribed by Matthias Löhle; Wiebke Schrempf; Martin Wolz; Heinz Reichmann; Alexander Storch
- Book ID
- 102945212
- Publisher
- John Wiley and Sons
- Year
- 2008
- Tongue
- English
- Weight
- 749 KB
- Volume
- 23
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
Abstract
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