Nelson Textbook of Pediatrics 22nd Edition | Prion diseases are fatal neurodegenerative disorders caused by misfolded prion protein (PrPSc). Human forms: sporadic CJD (most common), variant CJD (vCJD, related to BSE/mad cow disease), iatrogenic CJD, familial CJD, Gerstmann-Sträussler-Scheinker (GSS), fatal familial insomnia (FFI), and kuru. Diagnosis: clinical features, MRI (cortical ribboning, pulvinar sign), CSF 14-3-3, RT-QuIC. No treatment. Prevention: avoid contaminated neural tissues, blood donor deferral for vCJD risk.
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