Print version ISSN 1807-5932
DIAS, Francisco A. et al. Tremor in X-linked recessive spinal and bulbar muscular atrophy (Kennedy's disease). Clinics [online]. 2011, vol.66, n.6, pp. 955-957. ISSN 1807-5932. http://dx.doi.org/10.1590/S1807-59322011000600006.
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.
Keywords : Kennedy's disease; X-linked recessive bulbospinal neuronopathy; Spinal and bulbar muscular atrophy; Motor neuron disease; Tremor.