A 60-year-old man presents with progressive autonomic dysfunction (orthostatic hypotension, urinary retention), parkinsonism, and cerebellar ataxia. He dies 7 years later. Autopsy reveals alpha-synuclein-positive glial cytoplasmic inclusions (GCIs) in oligodendrocytes. The diagnosis is:
- A Parkinson disease
- B Multiple system atrophy ✓
- C Dementia with Lewy bodies
- D Progressive supranuclear palsy
Correct answer: B. Multiple system atrophy
Explanation
Multiple system atrophy (MSA) is an alpha-synucleinopathy characterized by glial cytoplasmic inclusions (GCIs, Papp-Lantos bodies) in oligodendrocytes, distinct from the neuronal Lewy bodies of Parkinson disease and DLB. MSA presents with autonomic failure plus parkinsonism (MSA-P) or cerebellar ataxia (MSA-B). PSP is a tauopathy. The presence of GCIs in oligodendrocytes is pathognomonic for MSA.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.