Tremor in X-linked recessive spinal and bulbar muscular atrophy (Kennedy's disease)
Clinics
;
66(6): 955-957, 2011. tab
Article
in English
| LILACS
| ID: lil-594361
ABSTRACT
OBJECTIVE:
To study tremor in patients with X-linked recessive spinobulbar muscular atrophy or Kennedy's disease.METHODS:
Ten patients (from 7 families) with a genetic diagnosis of Kennedy's disease were screened for the presence of tremor using a standardized clinical protocol and followed up at a neurology outpatient clinic. All index patients were genotyped and showed an expanded allele in the androgen receptor gene.RESULTS:
Mean patient age was 37.6 years and mean number of CAG repeats 47 (44-53). Tremor was present in 8 (80 percent) patients and was predominantly postural hand tremor. Alcohol responsiveness was detected in 7 (88 percent) patients with tremor, who all responded well to treatment with a β-blocker (propranolol).CONCLUSION:
Tremor is a common feature in patients with Kennedy's disease and has characteristics similar to those of essential tremor.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Tremor
/
Bulbo-Spinal Atrophy, X-Linked
Type of study:
Practice guideline
Limits:
Adult
/
Humans
/
Male
Language:
English
Journal:
Clinics
Journal subject:
Medicine
Year:
2011
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Genetika Laboratory/BR
/
University of Paraná/BR
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