Establishing diagnostic criteria for Perry syndrome.

Objective: To establish international diagnostic criteria for Perry syndrome, a disorder characterised by clinical signs of parkinsonism, depression/apathy, weight loss, respiratory symptoms, mutations in the gene and TAR DNA-binding protein 43 (TDP-43) pathology.

Methods: Data from the published literature and newly identified patients were gathered and analysed during and after the International Symposium on Perry syndrome in Tokyo to identify diagnostic criteria for Perry syndrome.

Results: Eighty-seven patients with Perry syndrome carrying mutations from 20 families were included in this study, and common signs of the disorder were identified, including parkinsonism (95.

Communication aid device utilizing event-related potentials for patients with severe motor impairment.

The purpose of the present study was to develop a communication aid device utilizing event-related potentials (ERPs) for patients with severe motor disabilities. Preliminary experiments were performed on eight healthy volunteers to determine the optimal methods of presentation of visual stimuli and an off-line analysis of electroencephalography signals. Three clinical experiments were performed online on 17 healthy volunteers, 5 patients with amyotrophic lateral sclerosis, and 1 patient with olivopontocerebellar atrophy.

[Optimum dosage of pergolide in patients with Parkinson's disease].

For the purpose of determining the optimal dose, pergolide was administered to patients with Parkinson's disease using a dose-escalation protocol up to the maximum dosage permissible in Japan. The study was completed in 16 subjects with Parkinson's disease. Prior to the start of the dose-escalation study, the daily dose of pergolide was 500 microg in 6 patients and 750 microg in 10 patients.

[Communication device utilizing event-related potentials].

We had developed a communication system using a theory of P300 through a computer. 17 healthy subjects participated in all 3 experiments, while 4 ALS and 1 OPCA patients took part in the first 2 experiments only. In the 1st experiment, 4 short sentences of 'yes very much', 'yes' 'no' and 'can't answer' were shown on the display.