Tremor in X-linked recessive spinal and bulbar muscular atrophy (Kennedy's disease)
| Autor: | Francisco A. Dias, Renato P. Munhoz, Salmo Raskin, Lineu César Werneck, Hélio A. G. Teive |
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| Jazyk: | angličtina |
| Rok vydání: | 2011 |
| Předmět: | |
| Zdroj: | Clinics, Vol 66, Iss 6, Pp 955-957 (2011) |
| Druh dokumentu: | article |
| ISSN: | 1807-5932 1980-5322 |
| DOI: | 10.1590/S1807-59322011000600006 |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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