Movement Disorders (revue)

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Potassium Channel Blocker 4-Aminopyridine is Effective in Interictal Cerebellar Symptoms in Episodic Ataxia Type 2 : A Video Case Report

Identifieur interne : 003740 ( Main/Merge ); précédent : 003739; suivant : 003741

Potassium Channel Blocker 4-Aminopyridine is Effective in Interictal Cerebellar Symptoms in Episodic Ataxia Type 2 : A Video Case Report

Auteurs : Matthias Löhle [Allemagne] ; Wiebke Schrempf [Allemagne] ; Martin Wolz [Allemagne] ; Heinz Reichmann [Allemagne] ; Alexander Storch [Allemagne]

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RBID : Pascal:08-0396197

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English descriptors

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.

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Le document en format XML

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<div type="abstract" xml:lang="en">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.</div>
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