This article by Japanese researchers describes a 78-year-old woman with what looks like early PSP. Since the pathological changes were in the substantia nigra and the subthalamic nucleus in this woman, the authors suggest that this is where PSP starts in everyone. The SN plays a major role in movement. The SN is also affected in Parkinson’s Disease. Both the SN and the STN are parts of the basal ganglia.
Neuropathology. 2010 Jun 21. [Epub ahead of print]
Early-stage progressive supranuclear palsy with degenerative lesions confined to the subthalamic nucleus and substantia nigra.
Sakai K, Yamada M.
Department of Neurology, Noto General Hospital, Nanao, Japan.
We describe a 78-year-old Japanese woman with early-stage progressive supranuclear palsy (PSP). She had a 3-week history of postural instability and gait disturbance. On examination, upper vertical gaze palsy, akinesia, hyperreflexia with pathological reflexes, hesitation, and postural instability were observed. Rigidity and resting tremors were not apparent. Brain MRI revealed atrophy of the frontotemporal lobes and dilatation of the third ventricle. A month later, she died of cerebral infarction. The total duration of her clinical course was approximately 2 months. The brain weighed 1180 g after fixation. Macroscopically, mild atrophy of the frontal lobes and mild depigmentation of the substantia nigra were observed. The conspicuous findings included degeneration confined to the subthalamic nucleus and substantia nigra and widespread but infrequent tau-positive neurofibrillary tangles/pretangles and glial fibrillary tangles (tuft-shaped astrocytes, coiled bodies and argyrophilic threads) in the brain. It has been reported that the most affected areas in PSP are the globus pallidus, subthalamic nucleus and substantia nigra. We suggest that degeneration in PSP would start with involvement of the substantia nigra and subthalamic nucleus.
PubMed ID#: 20573028