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Episodic ataxia and channelopathies.
- Source :
-
Brain & development [Brain Dev] 1998 Jan; Vol. 20 (1), pp. 9-13. - Publication Year :
- 1998
-
Abstract
- Clinical details are given of different types of episodic ataxia: type 1, with myokymia, and attacks which usually last a few minutes, and may occur several times a day, and treatment with acetazolamide can reduce the number of attacks; type 2, with interictal nystagmus, and attacks which last for several hours to a day or more, and treatment with acetazolamide is very effective; paroxysmal choreoathetosis with episodic ataxia, with attacks lasting for about 20 min and occurring at varying intervals; and familial hemiplegic migraine, with transient hemiplegia presenting during the aura of a migraine headache, the symptoms improving on treatment with acetazolamide. Their inheritance is of dominant type; and the gene for type 1 is mapped to chromosome 12p near to a cluster of potassium channel genes, and that for type 2 and for familial hemiplegic migraine to chromosome 19p near to calcium channel genes. The differential diagnosis from other conditions with a periodic symptomatology is discussed, especially from a number of metabolic disorders. Treatment is effective for many of these, and the treatment of the episodic ataxias with acetazolamide can sometimes have a dramatic effect. The possible role of the channelopathies in the causation of some periodic neurological disorders is considered; with the expectation that further research will improve the identification of specific diseases, and lead to more effective treatment.
- Subjects :
- Acetazolamide therapeutic use
Ataxia complications
Athetosis complications
Athetosis genetics
Cerebellar Ataxia genetics
Cerebellar Ataxia physiopathology
Chorea complications
Chorea genetics
Hemiplegia drug therapy
Hemiplegia genetics
Humans
Migraine Disorders drug therapy
Migraine Disorders genetics
Time Factors
Ataxia drug therapy
Ataxia physiopathology
Ion Channels genetics
Nervous System Diseases genetics
Subjects
Details
- Language :
- English
- ISSN :
- 0387-7604
- Volume :
- 20
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Brain & development
- Publication Type :
- Academic Journal
- Accession number :
- 9533553
- Full Text :
- https://doi.org/10.1016/s0387-7604(97)00086-7