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J Neurol Sci. 2010 May 15;292(1-2):81-4. doi: 10.1016/j.jns.2010.01.021. Epub 2010 Feb 23.

The effect of 3,4-diaminopyridine on the patients with hereditary pure cerebellar ataxia.

Author information

1
Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan. ttsunemi@ucsd.edu

Abstract

BACKGROUND:

Downbeat nystagmus (DBN) is often seen in patients with pure cerebellar type of spinocerebellar ataxia (SCA) like spinocerebellar ataxia type 6 (SCA6). DBN frequently presents with other cerebellar symptoms such as postural imbalance or ataxia. A potassium channel blocker 3,4-diaminopyridine (3,4-DAP) has been reported to reduce DBN by increasing the excitability of Purkinje cells.

OBJECTIVE:

The objective of this study is to determine whether 3,4-DAP has a beneficial effect on DBN along with postural imbalance and ataxic symptoms in 10 patients with SCA6 and five patients with chromosome 16q22.1-linked autosomal dominant cerebellar ataxia (16q-ADCA).

RESULTS:

The patients took 20mg of 3,4-DAP twice a day for a week. DBN was observed in seven patients with SCA6 and two with 16q-ADCA. Although 3,4-DAP significantly reduced DBN (P<0.05), other ataxic symptoms did not improved. However, 3,4-DAP showed benefit in two patients with oscillopsia.

CONCLUSION:

3,4-DAP may be effective on DBN and oscillopsia, although it was not proved to be effective on other symptoms of ataxia in SCA patients.

PMID:
20181362
DOI:
10.1016/j.jns.2010.01.021
[Indexed for MEDLINE]

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