Inflammatory glial activation in the brain of a patient with hereditary sensory neuropathy type 1 with deafness and dementia.
The brain of a patient with hereditary sensory neuropathy type 1 (HSN-1) associated with sensorineural deafness and early-onset dementia was neuropathologically investigated. Widespread neuronal degeneration in cerebral neocortex, hippocampus and basal ganglia was revealed, accounting for the clinical features. Loss of neurons with ballooning of residual neurons was remarkable in the hippocampus and frontal, parietal, and occipital lobes. Neuronal degeneration in these regions was accompanied by axonal dystrophy and glial reactions such as microgliosis and astrocytosis, however, only glial responses were prominent in the basal ganglia, brain-stem and cerebellum with mild neuronal loss. These results indicate that the widespread neuronal degeneration may be accelerated by inflammatory processes including glial activation in the brain of a patient with HSN-1 associated with deafness and dementia.
Hojo
Kaori
K
Department of Environmental Health and Safety, Division of Psychiatry and Neurology, Faculty of Medical Sciences, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-Ku, 650-0017, Japan.
Kawamata
Toshio
T
Tanaka
Chikako
C
Maeda
Kiyoshi
K
D003638:Deafness / Q000150:complications*; D003704:Dementia / Q000150:complications*; D006681:HLA-D Antigens / Q000145:classification / Q000378:metabolism; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000150:complications / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D007249:Inflammation / Q000209:etiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009457:Neuroglia / Q000378:metabolism / Q000473:pathology*; D009474:Neurons / Q000473:pathology; D013194:Staining and Labeling / Q000379:methods
D006681:HLA-D Antigens