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• W2893875453 abstract "Abstract We report clinico‐pathological features of a 65‐year‐old woman and a 56‐year‐old man with a 5‐year clinical history who had clinical and neuropathological characteristics of upper and lower motor neuron disease consistent with amyotrophic lateral sclerosis, and a frontotemporal atrophy pattern in case 2 without TDP ‐43 pathology. Instead, spongiform change and pathological prion protein deposits were observed in several brain regions. No prion protein gene mutations were found. Western blot analysis showed a five‐band profile compatible with variably protease‐sensitive prionopathy. We conclude that this disease can display prolonged disease duration and clinico‐pathological features within the ALS / FTLD spectrum." @default.
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• W2893875453 date "2018-09-21" @default.
• W2893875453 modified "2023-10-14" @default.
• W2893875453 title "Variably protease-sensitive prionopathy presenting within ALS/FTD spectrum" @default.
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• W2893875453 doi "https://doi.org/10.1002/acn3.632" @default.
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