1. Dopa-responsive dystonia with depigmentation of the substantia nigra and formation of Lewy bodies.
- Author
-
Olsson JE, Brunk U, Lindvall B, and Eeg-Olofsson O
- Subjects
- Adult, Bromocriptine therapeutic use, Cerebrospinal Fluid Proteins metabolism, Dopamine cerebrospinal fluid, Dopamine metabolism, Drug Therapy, Combination, Dystonia cerebrospinal fluid, Dystonia pathology, Female, Humans, Neuropeptides cerebrospinal fluid, Dihydroxyphenylalanine therapeutic use, Dystonia drug therapy, Lewy Bodies pathology, Pigmentation, Substantia Nigra pathology
- Abstract
The patient, who died at 23 years of age, was first diagnosed when she was 12 year old as having a dopa-responsive dystonia with decreased concentrations of monoamine metabolites in the cerebrospinal fluid. Also the concentrations of other neurotransmitters including somatostatin, substance P and metenkefalin were lowered indicating a more widespread damage of the cerebral neurotransmitter systems. At autopsy the brain was essentially intact except for pronounced gliosis and extreme loss of melanotic nerve cells in the substantia nigra. Several remaining nerve cells showed the presence of Lewy bodies.
- Published
- 1992
- Full Text
- View/download PDF