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

• Autor(a) principal: Dias, Francisco A.
• Data de Publicação: 2011
• Outros Autores: Munhoz, Renato P., Raskin, Salmo, Werneck, Lineu César, Teive, Hélio A. G.
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Clinics
• Texto Completo: https://www.revistas.usp.br/clinics/article/view/19510
• topic: Kennedy's disease; X-linked recessive bulbospinal neuronopathy; Spinal and bulbar muscular atrophy; Motor neuron disease; Tremor

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.