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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 10X Case 10 Denouement
Not shown: Only few beta-amyloid plaques are present in the neocortex. Tau IHC reveals few neurofibrillary tangles or diffusely tau-immunoreactive neurons and numerous tau-immunoreactive tufted astrocytes both in the cortex and white matter. A few coiled bodies, are seen largely in the white matter. No neuritic plaques are identified. No pTDP-43-immunoreactive neuronal cytoplasmic inclusions (NCIs) are identified. Several alpha-synuclein-positive Lewy bodies and neurites are detected in the locus ceruleus and tegmentum. Frequent alpha-synuclein immunoreactive Lewy bodies and Lewy neurites are present in the dorsal nucleus of the vagus and lateral reticular nucleus. Neuro Final Diagnosis: Progressive supranuclear palsy; Diffuse Lewy body disease (see comment); Alzheimer disease neuropathologic change (Low; A2, B1, C0; see comment); Aging-related tau astrogliopathy (ARTAG) in the occipital lobe, amygdala and basal ganglia; Small vessel disease ---- Neuro Diagnosis Comment: There is modest evidence of Alzheimer disease. Sparse beta-amyloid plaques and few neurofibrillary tangles, but no neuritic plaques are present. This very modest AD pathology is insufficient to meet the neuropathologic criteria for AD according to Khachaturian, CERAD, and NIA-Reagan Institute criteria. In the more recent NIA-AA criteria, this case has AD neuropathological change A2, B1, C0 ---- This case displays severe alpha-synucleinopathy in the form of Lewy bodies and Lewy neurites, largely in the brainstem, with more modest involvement of limbic and neocortical regions. The distribution of Lewy bodies is consistent with a 'neocortical stage' according to the McKeith criteria. In the Braak et al staging of Parkinson disease, this case represents stage 5/6. ---- In conclusion, the cognitive and motor deficits experienced during life in this patient may be explained largely by two diseases: PSP and diffuse Lewy body disease with a modest contribution from ADNC.
