MovDisordV3, PascalFrancis, Corpus, bibRecord, 001189

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

Identifieur interne : 001189 ( PascalFrancis/Corpus ); précédent : 001188; suivant : 001190

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

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

Source :

Movement disorders [ 0885-3185 ] ; 2008.

RBID : Pascal:08-0396197

Descripteurs français

Pascal (Inist)
- Pathologie du système nerveux, Antagoniste canal potassium, Ataxie cérébelleuse, Etude cas.

English descriptors

KwdEn :
- Case study, Cerebellar ataxia, Nervous system diseases, Potassium channel antagonist.

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.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

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C01	`01`		`ENG`	@0 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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Format Inist (serveur)

NO :	PASCAL 08-0396197 INIST
ET :	Potassium Channel Blocker 4-Aminopyridine is Effective in Interictal Cerebellar Symptoms in Episodic Ataxia Type 2 : A Video Case Report
AU :	LÖHLE (Matthias); SCHREMPF (Wiebke); WOLZ (Martin); REICHMANN (Heinz); STORCH (Alexander)
AF :	Department of Neurology, Technical University of Dresden/Dresden/Allemagne (1 aut., 2 aut., 3 aut., 4 aut., 5 aut.)
DT :	Publication en série; Courte communication, note brève; Niveau analytique
SO :	Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2008; Vol. 23; No. 9; Pp. 1314-1316; Bibl. 7 ref.
LA :	Anglais
EA :	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.
CC :	002B17; 002B17E
FD :	Pathologie du système nerveux; Antagoniste canal potassium; Ataxie cérébelleuse; Etude cas
ED :	Nervous system diseases; Potassium channel antagonist; Cerebellar ataxia; Case study
SD :	Sistema nervioso patología; Antagonista canal potasio; Ataxia cerebelosa; Estudio caso
LO :	INIST-20953.354000196264160220
ID :	08-0396197

Links to Exploration step

Pascal:08-0396197

Le document en format XML

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<front><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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<ET>Potassium Channel Blocker 4-Aminopyridine is Effective in Interictal Cerebellar Symptoms in Episodic Ataxia Type 2 : A Video Case Report</ET>
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<AF>Department of Neurology, Technical University of Dresden/Dresden/Allemagne (1 aut., 2 aut., 3 aut., 4 aut., 5 aut.)</AF>
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	Movement Disorders (revue)
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Movement Disorders (revue)

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

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

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