Protein Information

ID 289
Name episodic ataxia type 2
Synonyms APCA; BI; Brain calcium channel 1; Brain calcium channel I; CACH 4; CACH4; CACN 3; CACN3…

Compound Information

ID 332
Name 4-aminopyridine
CAS 4-pyridinamine

Reference

PubMed Abstract RScore(About this table)
15136697 Strupp M, Kalla R, Dichgans M, Freilinger T, Glasauer S, Brandt T: Treatment of episodic ataxia type 2 with the potassium channel blocker 4-aminopyridine. Neurology. 2004 May 11;62(9):1623-5.
Patients with episodic ataxia type 2 (EA2) can often be successfully treated with acetazolamide. The authors report three patients with EA2 (two with proven mutations in the CACNA1A gene) whose attacks were prevented with the potassium channel blocker 4-aminopyridine (4-AP; 5 mg tid). Attacks recurred after treatment was stopped; subsequent treatment alleviated the symptoms (mean follow-up time 6 months). These effects might be due to an improvement of the impaired functioning of Purkinje cells.
88(1,1,2,3)