alpha-synuclein and Parkinson-Disease--Postencephalitic

Topics: alpha-Synuclein; Creatine; Deep Brain Stimulation; Disease Progression; Dopamine; Fetal Tissue Transplantation; History, 19th Century; History, 20th Century; History, 21st Century; Humans; Levodopa; Lewy Bodies; Neostriatum; Neurons; Parkinson Disease; Parkinson Disease, Postencephalitic; Prion Diseases; Substantia Nigra; Thalamus

Postencephalitic parkinsonism (PEP), a chronic complication of encephalitis lethargica, is a tauopathy characterized by multisystem neuronal loss and gliosis with widespread neurofibrillary lesions composed of both 3- and 4-repeat (3R and 4R) tau isoforms. Previous immunohistochemical studies in a small number of PEP cases demonstrated absence of Lewy bodies as well as the lack of other alpha-synuclein pathology, classifying PEP as a "pure" tauopathy. Neuropathologic examination of 10 brains with clinico-pathologically verified PEP confirmed widespread neurodegeneration in subcortical and brainstem areas associated with multifocal neurofibrillary pathology comprising both 3R and 4R tau. Very rare beta-amyloid deposits were observed in two elderly patients, while Lewy bodies and neurites or any other alpha-synuclein deposits were completely absent. The causes and molecular background of total absence of alpha-synuclein pathology in PEP, in contrast to most other tauopathies, remain as unknown as the pathogenesis of PEP.

Topics: Adult; alpha-Synuclein; Amyloid beta-Peptides; Brain; Brain Stem; Female; Humans; Immunohistochemistry; Male; Middle Aged; Nerve Degeneration; Neurons; Parkinson Disease, Postencephalitic; Tauopathies

Topics: Aged; Aged, 80 and over; alpha-Synuclein; Basal Ganglia; Cerebral Cortex; Diagnosis, Differential; Humans; Male; Mesencephalon; Middle Aged; Nerve Tissue Proteins; Parkinson Disease, Postencephalitic; Supranuclear Palsy, Progressive; Synucleins