Pick's disease with Pick bodies combined with progressive supranuclear palsy without tuft-shaped astrocytes: a clinical, neuroradiologic and pathological study of an autopsied case.

摘要: We report clinical, neuroradiologic features, and neuropathologic findings of a 76-year-old man with coexistent Pick’s disease progressive supranuclear palsy. The patient presented loss recent memory, abnormal behavior change in personality at the age 60. symptoms were progressive. Three years later, repetitive or compulsive became prominent. About 9 years after onset, he had difficulty moving bed-ridden because fracture his left leg. His condition gradually deteriorated developed mutism vegetative. died from pneumonia 16 years onset symptoms. Serial MRI scans showed cortex atrophy, especially bilateral frontal temporal lobes. Macroscopic inspection severe atrophy whole brain, including cerebrum, brainstem cerebellum. Microscopic observations extensive superficial spongiosis neuronal gliosis second third cortical layers frontal, parietal cortex. There Pick cells argyrophilic bodies, which tau- ubiquitin-positive neurons II–III above-mentioned Numerous bodies observed hippocampus, dentate fascia. In addition, moderate to was found lot Gallyas/tau-positive globus neurofibrillary tangles caudate nucleus, pallidus, thalamus, substantia nigra, locus coeruleus nucleus. thorned-astrocytes coiled but no-tuft shaped astrocytes noted basal ganglion, cerebellar white matter. conclusion, these histopathological features compatible classical coexistence palsy without tuft-shaped astrocytes.