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

Authors

  • Francisco A. Dias University of Paraná; Hospital de Clínicas; Internal Medicine Department; Neurology Service
  • Renato P. Munhoz University of Paraná; Hospital de Clínicas; Internal Medicine Department; Neurology Service
  • Salmo Raskin Genetika Laboratory
  • Lineu César Werneck University of Paraná; Hospital de Clínicas; Internal Medicine Department; Neurology Service
  • Hélio A. G. Teive University of Paraná; Hospital de Clínicas; Internal Medicine Department; Neurology Service

DOI:

https://doi.org/10.1590/S1807-59322011000600006

Keywords:

Kennedy's disease, X-linked recessive bulbospinal neuronopathy, Spinal and bulbar muscular atrophy, Motor neuron disease, Tremor

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%) 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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Published

2011-01-01

Issue

Vol. 66 No. 6 (2011)

Section

Clinical Sciences

How to Cite

Tremor in X-linked recessive spinal and bulbar muscular atrophy (Kennedy’s disease) . (2011). Clinics, 66(6), 955-957. https://doi.org/10.1590/S1807-59322011000600006