Nervenarzt

[Acute intermittent porphyria with transient paresis and contracture. Evidence for initial myopathic dysfunction?]

Year 1998
Poersch M.
Dr. von Ehrenwall'sche Klinik, Fachkrankenhaus fur Psychiatrie, Psychotherapie und Neurologie, Ahrweiler.
A 70-year old man with acute intermittent porphyria had acute transient bibrachial paresis with moderate contracture of the involved muscles, which showed electrical silence in a conventional electromyographic investigation with surface electrodes placed over the contracted muscle belly (filter: 20 Hz-2 Hz, amplifier 50 microV). Slow finger movements were still possible and showed typical muscle-action potentials. This electroclinical correlation points to myopathic localized dysfunction, perhaps similar to rare case reports with patients having metabolic myopathy of McArdle's type.

[Schizophrenia-like symptoms in the Westphal-Strumpell variation of Wilson disease]

Year 1998
Muller J. Landgraf F. Trabert W.
Universitatsnervenklinik Psychiatrie-Psychotherapie, Homburg/Saar.
Wilson's disease is a rare, autosomal recessive disorder of copper metabolism due to low serum ceruloplasm, resulting in increased copper deposition, especially in the liver and basal ganglia in the brain. The pseudosclerotic type of Wilson's disease, also known as the Westphal-Strumpell form, is distinguished by positional tremor, ataxia and dysarthria as the main symptoms. We use the example of a 23-year-old patient whose neurological symptoms were preceded by a long history of a schizophrenic-like disorder. Clinical symptoms are presented. MRI, SPECT and PET images are illustrated. Therapy and outcome are discussed.