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  • Print publication year: 2011
  • Online publication date: May 2011

Case 38 - Young man with slow cognitive decline


This chapter speaks about a 75-year-old woman admitted with a 4-year history of progressive social withdrawal, decreased fluency, and difficulty handling complex tasks. Magnetic Resonance Imaging (MRI) of the brain showed prominent mesencephalic atrophy, dilation of the third ventricle, and mild cerebral atrophy. Based upon her history and examination, the patient met National Institute of Neurological Disorders and Stroke - Society for Progressive Supranuclear Palsy (NINDS-SPSP) diagnostic criteria for probable Progressive Supranuclear Palsy (PSP). Available data showed that the diagnosis of PSP was pathologically confirmed in essentially 100% of patients who meet these criteria. The patient's motor function progressively worsened, and she died from respiratory complications, approximately 6 years following symptom onset. Progressive supranuclear palsy is a sporadic neurodegenerative disease, which is defined clinicopathologically by the constellation of atypical parkinsonism, supranuclear vertical gaze palsy, and a characteristic pattern of tau accumulation within the brainstem and basal ganglia.

Further reading

ArataJ, TakashimaH, HiranoR, et al. (2006). Early clinical signs and imaging findings in Gerstmann–Sträussler–Scheinker syndrome (Pro102Leu). Neurology, 66, 1672–8.
HsiaoK, BakerHF, CrowTJ, et al. (1989). Linkage of a prion protein missense variant to Gerstmann–Sträussler syndrome. Nature 338, 342–5.
IronsideJW, HeadMW (2004). Neuropathology and molecular biology of variant Creutzfeldt–Jakob disease. Curr Top Microbiol Immunol, 284, 133–59.
KovacsGG, PuopoloM, LadoganaA, et al. (2005). Genetic prion disease: the EUROCJD experience. Hum Genet, 118, 166–74.
RossorMN, FoxNC, MummeryCJ, SchottJM, WarrenJD (2010). The diagnosis of young-onset dementia. Lancet Neurol, 9(8), 793–806.
WadsworthJD, CollingeJ (2010). Molecular pathology of human prion disease. Acta Neuropathol, in press.