Restless legs syndrome in spinocerebellar ataxia types 1, 2, and 3.
To identify the prevalence and determinants of restless legs syndrome (RLS) in spinocerebellar ataxia (SCA) we studied 58 patients with a molecular diagnosis of SCA1, SCA2 and SCA3. Data on the symptoms of RLS were collected by a standardized questionnaire, and RLS was diagnosed when patients met the four minimal criteria of the syndrome as recently defined by an international study group. In addition, we studied the relationship between RLS and age, age at ataxia onset, CAG repeat length, and nerve conduction and evoked potentials data. RLS was significantly more frequent in SCA patients than in controls (28% vs. 10%). Age at RLS onset in SCA was 49.0 +/- 10.9 years. There were no significant differences in nerve conduction or evoked potentials between RLS and non-RLS SCA patients. The probability of developing RLS increased with age but not with CAG repeat length or higher age of ataxia onset. The data provide evidence that patients with SCA1, SCA2 and SCA3 are per se more susceptible to RLS than non-affected individuals. The probability of developing RLS is related principally to the period over which the CAG repeat mutation exerts its effect and not to CAG repeat length or age of ataxia onset.
Abele
M
M
Department of Neurology, University of Tübingen, Hoppe-Seyler-Strasse 3, 72076 Tübingen, Germany. michael.abele@uni-bonn.de
Bürk
K
K
Laccone
F
F
Dichgans
J
J
Klockgether
T
T
D000328:Adult; D017668:Age of Onset; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D017827:Machado-Joseph Disease / Q000150:complications* / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012148:Restless Legs Syndrome / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D020754:Spinocerebellar Ataxias / Q000150:complications* / Q000235:genetics / Q000473:pathology; D018911:Trinucleotide Repeats / Q000235:genetics*