A 66-year-old man presents with rapidly progressive dementia, myoclonic jerks, and gait ataxia over 4 months. Vital signs: BP 138/82, HR 88, RR 16, Temp 37.2°C, SpO2 98%. Neurologic exam shows cogwheel rigidity and hyperreflexia; no focal weakness. EEG demonstrates periodic sharp-wave complexes. MRI reveals cortical ribboning and basal ganglia atrophy. CSF 14-3-3 protein is elevated. Which of the following best explains the underlying pathophysiology of this patient's condition?
- A)Intracellular accumulation of alpha synuclein in cortical neurons
- B)Loss of cholinergic neurons in the basal forebrain
- C)Expansion of a CAG trinucleotide repeat on chromosome 4
- D)Autoimmune antibodies to postsynaptic NMDA receptors
- E)Accumulation of a misfolded protein that converts normal proteins into beta pleated sheetsGABARITO
Explicação
Accumulation of a misfolded protein that converts normal proteins into beta pleated sheets is correct. Creutzfeldt Jakob disease is a prion disease characterized by rapidly progressive dementia, myoclonus, ataxia, and periodic sharp waves on EEG. Pathology sho... Ver explicação completa e trilha adaptativa →