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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., (Copyright 2003 S. Karger AG, Basel)

Nine individuals with Parkinson's disease (PD) and nine normal control subjects, matched for sex and age, were compared on a standardized assessment protocol for dysarthria. Simple measures of hand and gait motility were also obtained. A kinematic analysis of vertical jaw movements during speech was performed by using an optoelectronic technique. The PD subjects were examined during both induced off L-dopa and on L-dopa states, and their performance was compared between the two conditions. Significant group differences were found for all kinematic measures during a syllable repetition task, as well as for the total dysarthria test scores and certain individual test items. Implications and clinical usefulness of the kinematic approach to the study of oral motor functions are discussed.

The phonation and articulation disorders in a group of aphasics with aphonia/dysarthrophonia could be considerably reduced by the use of medication. The group consisted of 10 apoplectic patients whose resulting aphasia could not be classified because of the vocal impairment. Extrapyramidal motion disorders were proved by laryngoscopy and stroboscopy. A definite improvement of phonation and articulation was observed after L-dopa medication.