Tremor in X-linked recessive spinal and bulbar muscular atrophy (Kennedy's disease)

AUTOR(ES)

Dias, Francisco A., Munhoz, Renato P., Raskin, Salmo, Werneck, Lineu César, Teive, Hélio A. G.

FONTE

Clinics

DATA DE PUBLICAÇÃO

2011

RESUMO

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%) patients and was predominantly postural hand tremor. Alcohol responsiveness was detected in 7 (88%) 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.

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