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Fluctuation of Computed Tomographic Findings in White Matter in Alexander's Disease

Department of Pediatrics Yamagata University School of Medicine, Department of Pediatrics Yamagata Prefectural Nihonkai Hospital

Mitsuhiro Kato, MD, PhD

Department of Pediatrics Yamagata University School of Medicine

Tomomi Honma, MD

Department of Pediatrics Yamagata University School of Medicine, Department of Pediatrics Yamagata Prefectural Nihonkai Hospital

Shinsuke Ohtaki, MD, PhD

Department of Pediatrics Yamagata Prefectural Nihonkai Hospital Sakata, Japan

Yukio Sawaishi, MD, PhD

Department of Pediatrics Akita University School of Medicine Akita, Japan

Kiyoshi Hayasaka, MD, PhD

Department of Pediatrics Yamagata University School of Medicine Yamagata, Japan

A Japanese boy developed febrile seizures and gait disturbance at 2 years of age and dysarthria a year later. He had generalized tonic-clonic seizures once or twice a year from the age of 4 years. Brain computed tomography (CT) showed symmetric low-density areas in the white matter of the frontal lobes. However, abnormal CT findings fluctuated occasionally, with no apparent change in clinical manifestations. Clinical evaluation at 9 years of age revealed hyperreflexia, psychomotor retardation, megalencephaly, and slurred nasal speech. Magnetic resonance imaging showed white matter abnormalities, predominantly in the frontal lobes. He was a heterozygote of the Arg239Cys mutation of the glial fibrillary acidic protein gene and was diagnosed with Alexander's disease. Fluctuation of CT findings in white matter may reflect blood-brain barrier dysfunction in Alexander's disease. (J Child Neurol 2002;17: 227-230).

Journal of Child Neurology, Vol. 17, No. 3, 227-230 (2002)
DOI: 10.1177/088307380201700316


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