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
| Autor: | Daichi Sone, Tomoko Maekawa, Miho Murata, Atsuhiko Sugiyama, Mikako Enokizono, Hiroshi Matsuda, Noritaka Wakasugi, Hidehiro Mizusawa, Noriko Sato, Yukio Kimura, Yuji Takahashi |
|---|---|
| Rok vydání: | 2017 |
| Předmět: |
Pathology
medicine.medical_specialty Mutation Thalamus Disease computer.software_genre medicine.disease medicine.disease_cause Gerstmann–Sträussler–Scheinker syndrome 030218 nuclear medicine & medical imaging 03 medical and health sciences 0302 clinical medicine Atrophy Neurology Voxel 99mtc ecd medicine Dementia Neurology (clinical) Psychology computer 030217 neurology & neurosurgery |
| Zdroj: | Journal of the Neurological Sciences. 373:27-30 |
| ISSN: | 0022-510X |
| DOI: | 10.1016/j.jns.2016.12.021 |
| Popis: | Gerstmann-Straussler-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 two cases of genetically proven GSS102L, both of which present with atrophy and decreased blood flow of the thalamus as determined by voxel-based specific regional analysis system for Alzheimer's disease (VSRAD) advance software and easy Z-score analysis for 99mTc-ethyl cysteinate dimer-SPECT, respectively. These thalamic abnormalities have not been fully evaluated to date, and detecting them might be useful for differentiating GSS102 from other neurodegenerative disorders. |
| Databáze: | OpenAIRE |
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