Increase of substance P and met-enkephalin in a severely atrophied striatum without clinical expression of chorea.
In a severely demented patient, with a family history of dementia, a severe striatal atrophy was observed. Neither this patient, nor his relatives, had choreoathetosis but he displayed rigidity and bradykinesia. A selective increase of the number of detectable striatal substance P and met-enkephalin neurones was found. These neurones also exhibited a striking increase of the intensity of these peptides' immunoreactivities. Simultaneously, the dense networks of substance P and met-enkephalin nerve fibres were well preserved in the globus pallidus and the substantia nigra. The absence of choreoathetosis, despite severe striatal atrophy, is described in several basal ganglia diseases. Our results are in contrast with the well established reductions of substance P and enkephalins in the atrophied striatum as well as in the globus pallidus and the substantia nigra reported in classical cases of Huntington's disease expressing choreoathetosis.
Schiffmann
S N
SN
Laboratories of Neuropathology and Neuropeptide Research and Pathology and Electron Microscopy, Faculty of Medicine, Erasme and Brugmann Hospitals, Université Libre de Bruxelles, Brussels, Belgium.
Vanderhaeghen
J J
JJ