title: Autopsy case of hereditary spastic paraplegia with thin corpus callosum showing severe gliosis in the cerebral white matter.
abstract: We report an autopsy case of a 51-year-old man clinically diagnosed with a complicated type of hereditary spastic paraplegia. His sister showed similar manifestations. Gait disturbance was manifested at 14 years of age. Subsequently, slowly progressive spastic tetraplegia developed with mental deterioration, neuropathy and amyotrophy. Marked cerebral atrophy with thin corpus callosum was shown by cranial MRI. Autopsy revealed a severely atrophic brain with extreme thinning of the whole corpus callosum. Microscopically, neurodegeneration was found in the corticospinal tract, thalamus, cerebral white matter and substantia nigra, as well as in the anterior horn and posterior column of the spinal cord. The remaining neurons contained large amounts of lipofuscin and eosinophilic granules. Unique to this patient was the severe gliosis in the cerebral white matter and substantia nigra, suggesting that sufficient development had been established when the degenerative process occurred. The predominant feature of the present case is the neurodegeneration process rather than hypoplasia.
authors.affiliation: Department of Neurology, Suzuka National Hospital, Kasado, Suzuka-shi, Mie, Japan. kuru@suzuka.hosp.go.jp
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kuru
authors.forename: Motoko
authors.initials: M
authors.lastname: Sakai
authors.forename: Masaaki
authors.initials: M
authors.lastname: Konagaya
authors.forename: Mari
authors.initials: M
authors.lastname: Yoshida
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Hashizume
mesh_terms: D001344:Autopsy; D002540:Cerebral Cortex / Q000473:pathology*; D003337:Corpus Callosum / Q000473:pathology*; D005911:Gliosis / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009410:Nerve Degeneration / Q000473:pathology; D009474:Neurons / Q000473:pathology; D015419:Spastic Paraplegia, Hereditary / Q000473:pathology* / Q000503:physiopathology; D013116:Spinal Cord / Q000473:pathology