title: Pathological and biochemical studies on a case of Pick disease with severe white matter atrophy.
abstract: We report on a male patient with Pick disease who had shown severe white matter atrophy and dilatation of the lateral ventricle in the frontal lobe from an early stage. Upon admission to our hospital 2 years after disease onset, the patient showed apathy, and MRI revealed severe atrophy of the cortex and white matter of the frontal lobe. He died at age 74, 11 years after disease onset. Autopsy revealed severe atrophy of the frontal and temporal lobes, severe loss of white matter in the frontal lobe, dilatation of the lateral ventricles, and cortical thinning. Histopathological examination showed severe loss of myelinated fibers in the frontal white matter and severe neuronal loss with gliosis in the frontal and temporal cortices. Many Pick bodies were seen. Our patient had a rare case of Pick disease predominantly affecting the frontal lobe with severe involvement of the white matter from an early stage. This case suggests that myelinated fibers in the white matter as well as cerebral neurons are primarily affected in Pick disease.
authors.affiliation: Department of Neurology, Juntendo Urayasu Hospital, Chiba, Japan.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Yamakawa
authors.forename: Masashi
authors.initials: M
authors.lastname: Takanashi
authors.forename: Masao
authors.initials: M
authors.lastname: Watanabe
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Nakamura
authors.forename: Tomonori
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Masato
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Yoshikuni
authors.initials: Y
authors.lastname: Mizuno
authors.forename: Shigeki
authors.initials: S
authors.lastname: Tanaka
authors.forename: Hideo
authors.initials: H
authors.lastname: Mori
mesh_terms: D000368:Aged; D001284:Atrophy; D017809:Fatal Outcome; D005625:Frontal Lobe / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology; D020547:Lateral Ventricles / Q000378:metabolism / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009413:Nerve Fibers, Myelinated / Q000378:metabolism / Q000473:pathology; D020774:Pick Disease of the Brain / Q000378:metabolism* / Q000473:pathology*; D016622:Silver Staining; D016875:tau Proteins / Q000378:metabolism
chemical_list: D016875:tau Proteins