Potassium channel blocker 4-aminopyridine is effective in interictal cerebellar symptoms in episodic ataxia type 2--a video case report.
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.
Löhle
Matthias
M
Department of Neurology, Technical University of Dresden, Dresden, Germany. matthias.loehle@neuro.med.tu-dresden.de
Schrempf
Wiebke
W
Wolz
Martin
M
Reichmann
Heinz
H
Storch
Alexander
A
D015761:4-Aminopyridine / Q000627:therapeutic use*; D005260:Female; D005684:Gait / Q000187:drug effects; D020234:Gait Ataxia / Q000188:drug therapy*; D006801:Humans; D008875:Middle Aged; D026902:Potassium Channel Blockers / Q000627:therapeutic use*; D016896:Treatment Outcome
D026902:Potassium Channel Blockers; D015761:4-Aminopyridine