Biochemical and Neurophysiological Investigations in Two Forms of Segawa's Disease

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Abstract
In two 14-year-old children with the typical clinical picture of Segawa's syndrome the metabolism of L-DOPA was examined and compared to an age matched control. The very different responses to DOPA- and benser-acid-medication underline the hypothesis, that Segawa's disease may result from at least two different pathological conditions. Since this disease mimics hereditary degenerative nervous tissue disorders, evoked potentials of both patients are demonstrated, showing that those pathways of the CNS, who can be examined by these methods, are unaffected. Bei zwei 14 Jahre alten Kindern mit der typischen Symptomatik des Segawa-Syndroms wurde der DOPA- Stoffwechsel untersucht. Die sehr unterschiedlichen Spiegelverläufe von L-DOPA und seinem Metaboliten 3-o-methyl- DOPA nach DOPA- und Benserazid-Gabe bestätigen die Hypothese, daß es mindestens zwei unterschiedliche Ursachen für das Segawa-Syndrom gibt. Normale elektrodiagnostische Befunde helfen, dieses Krankheitsbild von anderen degenerativen Nervensystemerkrankungen zu unterscheiden.

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