North Carolina Neurological Foundation (NCNF)

Major Neurocognitive Disorder Due To Prion Disease

Major neurocognitive disorder due to prion disease is a rapidly progressive neurodegenerative disorder caused by misfolded prion proteins leading to neuronal loss and spongiform changes. Etiology includes sporadic, inherited, or acquired prion infections. Clinical features include rapidly progressive dementia, ataxia, myoclonus, and neuropsychiatric disturbances. Diagnosis relies on clinical evaluation, EEG, MRI, cerebrospinal fluid biomarkers, and exclusion of other dementias. Management is supportive and palliative, as there is currently no disease-modifying therapy, with prognosis typically poor.