title: Progressive cerebral atrophy in neuromyelitis optica.
abstract: We report two cases of neuromyelitis optica patients with progressive cerebral atrophy. The patients exhibited characteristic clinical features, including elderly onset, secondary progressive tetraparesis and cognitive impairment, abnormally elevated CSF protein and myelin basic protein levels, and extremely highly elevated serum anti-AQP-4 antibody titer. Because neuromyelitis optica pathology cannot switch from an inflammatory phase to the degenerative phase until the terminal phase, neuromyelitis optica rarely appears as a secondary progressive clinical course caused by axonal degeneration. However, severe intrathecal inflammation and massive destruction of neuroglia could cause a secondary progressive clinical course associated with cerebral atrophy in neuromyelitis optica patients.
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan youko_warabi@tmhp.jp.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Warabi
authors.affiliation: Department of Neurology, Tohoku University Graduate School of Medicine, Japan Department of Neurology, National Yonezawa Hospital, Japan.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Takahashi
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.
authors.forename: Eiji
authors.initials: E
authors.lastname: Isozaki
mesh_terms: D000368:Aged; D001284:Atrophy / Q000473:pathology; D054022:Cerebrum / Q000473:pathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008875:Middle Aged; D009471:Neuromyelitis Optica / Q000097:blood / Q000134:cerebrospinal fluid / Q000473:pathology* / Q000503:physiopathology; D066127:White Matter / Q000473:pathology*
keywords: Multiple sclerosis; anti-aquaporin-4 antibody; astrocyte; axonal degeneration; inflammation; neuromyelitis optica; progressive cerebral atrophy; secondary progressive