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Article

Thalamic involvement determined using VSRAD advance on MRI and easy Z-score analysis of 99mTc-ECD-SPECT in Gerstmann-Sträussler-Scheinker syndrome with P102L mutation.

Journal of the neurological sciences - 15 Feb 2017

Sugiyama Atsuhiko, Sato Noriko, Kimura Yukio, Maekawa Tomoko, Wakasugi Noritaka, Sone Daichi, Enokizono Mikako, Takahashi Yuji, Murata Miho, Mizusawa Hidehiro, Matsuda Hiroshi

Abstract excerpt

Gerstmann-Sträussler-Scheinker syndrome caused by the P102L mutation in the prion protein gene (GSS102) is usually characterized by the onset of slowly progressive cerebellar ataxia, with dementia occurring much later. Because of the relatively long disease course and the prominence of progressive cerebellar ataxia in the early stage, GSS102 is often misdiagnosed as other neurodegenerative disorders. We present...

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Identifiers

PMID: 28131204
DOI: 10.1016/j.jns.2016.12.021

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ECD

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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