title: Presenile dementia with progressive supranuclear palsy tangles and Pick bodies: an unusual degenerative disorder involving the cerebral cortex, cerebral nuclei, and brain stem nuclei.
abstract: Degeneration of heterogeneous systems in the central nervous system, with widespread distribution of argyrophilic neuronal fibrillary inclusions, was found in a patient with presenile dementia. Atrophy was circumscribed in the frontal and temporal lobes. Neuronal loss was severe in the basal ganglia, subthalamic nucleus, and substantia nigra. Immunocytochemical study using anti-phosphorylated tau and anti-ubiquitin antibodies in conjunction with ultrastructural observations revealed two types of inclusions: neurofibrillary tangles (NFTs) of progressive supranuclear palsy (PSP) in the Edinger-Westphal nucleus, locus coeruleus, cerebellar dentate nucleus, inferior olivary nucleus, and posterior horn of the spinal cord; and Pick bodies (PBs) in the atrophied cerebral cortex and red nucleus. PSP-type NFTs and PBs have been demonstrated in a single case for the first time. Despite their pathognomonic significance in certain disorders, we suggest that these inclusions may reflect a form of cytoskeletal disorganization, which is not entirely restricted to a single disease entity.
authors.affiliation: Division of Psychiatry, National Center Hospital for Mental, Nervous, and Muscular Disorders, Tokyo, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Arima
authors.forename: S
authors.initials: S
authors.lastname: Murayama
authors.forename: S
authors.initials: S
authors.lastname: Oyanagi
authors.forename: T
authors.initials: T
authors.lastname: Akashi
authors.forename: T
authors.initials: T
authors.lastname: Inose
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology; D001933:Brain Stem / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D003704:Dementia / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008854:Microscopy, Electron; D016874:Neurofibrillary Tangles / Q000473:pathology*; D009929:Organ Size; D013194:Staining and Labeling; D013494:Supranuclear Palsy, Progressive / Q000473:pathology*