Progressive leukoencephalopathy associated with aluminum deposits in myelin sheath.
A 20-year-old woman with progressive leukoencephalopathy developed mental and motor disabilities and fell into a coma after suffering head trauma and febrile episodes from infancy. Brain imaging showed massive abnormal signals in the white matter. The electron spectroscopic imaging of biopsied brain tissue confirmed the electron-dense deposits to be associated with aluminum accumulation in the myelin sheath. Her brain pathology, which showed ferritin- and naphtochrome green-positive deposits, supported the imaging analysis. The clinicopathological features indicate a new form of progressive leukoencephalopathy.
Itoh
Masayuki
M
National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan. itoh@ncnp.go.jp
Suzuki
Yoshiko
Y
Sugai
Kenji
K
Kozuka
Naofumi
N
Ozuka
Naofumi
N
Ohsawa
Maki
M
Otsuki
Taisuke
T
Goto
Yu-Ichi
Y
D000328:Adult; D000535:Aluminum / Q000032:analysis* / Q000633:toxicity*; D001927:Brain Diseases / Q000139:chemically induced* / Q000473:pathology*; D003711:Demyelinating Diseases / Q000139:chemically induced* / Q000473:pathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008854:Microscopy, Electron; D046032:Microscopy, Energy-Filtering Transmission Electron; D009186:Myelin Sheath / Q000187:drug effects / Q000473:pathology*; D019636:Neurodegenerative Diseases / Q000139:chemically induced* / Q000473:pathology*; D009460:Neurologic Examination; D013702:Temporal Lobe / Q000187:drug effects / Q000473:pathology; D014057:Tomography, X-Ray Computed
D000535:Aluminum