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KMID : 1022820120030010026
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Journal of Multiple Sclerosis and Neuroimmunology
2012 Volume.3 No. 1 p.26 ~ p.29
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Adult-Onset Alexander Disease Mimicking Neuromyelitis Optica
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Jung Hyun-Jung
Nam Tai-Seung
Lee Seung-Han
Kim Myeong-Kyu
Cho Ki-Hyun
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Abstract
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Alexander disease (AxD) has been grouped as the leukodystrophy that affects the white matter of central nervous system, and is caused by the mutation of glial fibrillary acidic protein gene. Adult-onset AxD manifests pseudobulbar signs, palatal myoclonus, spastic paralysis, or ataxia by the involvement of medulla or spinal cord. Adult-onset AxD can be initially misdiagnosed as multiple sclerosis or tumor of brainstem and spinal cord. We report a case of genetically-proven adult-onset AxD initially mimicking neuromyelitis optica.
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KEYWORD
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Alexander disease, Glial fibrillary acidic protein, Neuromyelitis optica
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