RESUMO
Background and aims We examined the neurological manifestations, treatment and outcomes of a subset of 25 patients within the largest ever outbreak of wound botulism in Europe. Methods and results All 25 cases were intravenous drug users. The most common presenting symptom was dysarthria in 19/25 (76%), followed by dysphagia in 12/25 (48%), blurred vision in 10/25 (40%) and double vision in 8/25 (32%). Microbiological analysis confirmed the diagnosis in nine cases (36%). Duration of admission positively correlated with time to antitoxin, time to wound debridement and female sex. Conclusion As the outbreak continued, hospital stays shortened, reflecting growing awareness of the outbreak and quicker treatment initiation.
Assuntos
Botulismo/microbiologia , Transtornos de Deglutição/microbiologia , Surtos de Doenças/estatística & dados numéricos , Disartria/microbiologia , Saúde Pública , Transtornos da Visão/microbiologia , Infecção dos Ferimentos/microbiologia , Adulto , Botulismo/mortalidade , Botulismo/fisiopatologia , Desbridamento , Transtornos de Deglutição/mortalidade , Disartria/mortalidade , Feminino , Dependência de Heroína , Humanos , Masculino , Escócia/epidemiologia , Resultado do Tratamento , Transtornos da Visão/mortalidade , Infecção dos Ferimentos/mortalidadeRESUMO
Thirty patients with Wilson's disease (WD) were observed at a movement disorder clinic between 1970 and 2000. Disease onset was at the mean age (SD) of 14.5 (+/-5.9) years. Presentation with hepatic disease occurred in 12 of 30 patients and with neurologic disease in 15. Three patients were asymptomatic at the time of diagnosis. The mean (SD) delay to diagnosis was 5.9 (+/-5.7) years. Five patients diagnosed in an advanced stage of disease died before initiating treatment. Eighteen patients were followed and treated with D-penicillamine alone or in combination with zinc sulphate. Treatment improved most of neurological symptoms. Dystonic postures, behavioural disturbances and dysarthria were the most resistant neurological signs. 'Pseudo-sclerotic' neurologic involvement predicted a good outcome, whereas hepatic onset and 'classic' neurologic involvement were associated with a poorer prognosis. Two of the 18 treated patients died of hepatic failure due to voluntary discontinuation of therapy. Both D-penicillamine and zinc sulphate were well tolerated. No teratogenic effect of D-penicillamine was observed throughout 5 pregnancies. Our results suggest that D-penicillamine or a combination of D-penicillamine and zinc sulphate is a safe and effective long-term treatment in patients with WD.
