Thalamic degeneration with negative prion protein immunostaining.
A 34-year-old woman presented with an insidious 5-year history of cognitive decline and apathy, associated with hypersomnia, ataxia, and dysarthria. Magnetic resonance imaging of the brain showed cortical and subcortical atrophy. At autopsy we found abnormalities in the subcortical grey matter and brainstem, with a relatively preserved cerebral cortex. The thalami showed symmetrical neuronal loss and astrocytosis, particularly severe in the dorsal medial nucleus, followed by the lateral nuclei group. Prion protein immunostaining was negative, and there was no spongiform change. No mutations were detected in the prion protein gene.
Janssen
J C
JC
Department of Clinical Neurology, Institute of Neurology, University of London, Imperial College School of Medicine, Queen Square, UK.
Lantos
P L
PL
Al-Sarraj
S
S
Rossor
M N
MN
D000328:Adult; D001921:Brain / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D011328:Prions / Q000032:analysis*; D013786:Thalamic Diseases / Q000473:pathology*
D011328:Prions