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Clinics ; 66(6): 955-957, 2011. tab
Artículo en Inglés | LILACS | ID: lil-594361

RESUMEN

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


Asunto(s)
Adulto , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Atrofia Bulboespinal Ligada al X/fisiopatología , Temblor/fisiopatología , Edad de Inicio , Antagonistas Adrenérgicos beta/administración & dosificación , Debilidad Muscular/fisiopatología , Propranolol/administración & dosificación , Temblor/tratamiento farmacológico
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