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Journal of Child Neurology
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Progressive Leukoencephalopathy Associated With Aluminum Deposits in Myelin Sheath

Masayuki Itoh, MD, PhD

National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan, itoh{at}ncnp.go.jp

Yoshiko Suzuki, MD, PhD

Department of Pediatrics, Aichi Aoitori Medical and Welfare Center, Nagoya, Japan

Kenji Sugai, MD, PhD

National Hospital for Mental, Nervous and Muscular Disorders, Tokyo, Japan

Naofumi Ozuka, PhD

National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan

Maki Ohsawa, MD

National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan

Taisuke Otsuki, MD, PhD

National Hospital for Mental, Nervous and Muscular Disorders, Tokyo, Japan

Yu-ichi Goto, MD, PhD

National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan

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.

Key Words: leukoencephalopathy • aluminum • electron spectroscopic imaging

Journal of Child Neurology, Vol. 23, No. 8, 938-943 (2008)
DOI: 10.1177/0883073808315413
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