Treatment of non-fluctuating progressive dystonia: a neuropharmacological approach

HVA and 5-HIAA in CSF were determined in five patients with non-fluctuating progressive dystonia before and after treatment with L-Dopa. The response to L-Dopa was unequivocally favourable in one patient. The HVA-level in her CSF was significantly reduced before treatment, whereas in the other four non-responding patients the pre-treatment HVA was normal. It is postulated, that the biochemical delineation of clinical responsiveness to a neurotransmitter may provide useful clues towards the classification of extrapyramidal disease in childhood.