Abstract
A case of striatonigral degeneration with transient response to levodopa is described. Although the response might appear unexpected in view of the widespread degeneration of putaminal neurones, the neuronal population in the caudate nucleus was only moderately reduced; the striatal neurones were presumed to be the receptor cells for the actions of dopamine formed from levodopa. Differentiation of this case from idiopathic paralysis agitans is not readily made on clinical grounds, but atypical features may suggest the possibility of this diagnosis. The results of chemical analyses (dopamine, homovanillic acid, serotonin, 5-hydroxyindoleacetic acid, l-dopa decarboxylase, l-glutamic acid decarboxylase) disclosed similarities but also important differences between the present case and cases with Parkinson's disease. The neurochemical findings in the striatum and other brain areas, and degenerative changes in the brain stem tegmentum are reported for the first time, and their significance is discussed.
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