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Journal of the Korean Child Neurology Society 2002;10(2):338-343.
Published online November 30, 2002.
A Case of Childhood Ataxia with Diffuse Central Nervous System Hypomyelination(CACH) Syndrome.
Kyu Tae Kim, Hyung Suck Kim, Sang Ook Nam, Hak Jin Kim
1Department of Pediatrics, Pusan National University, College of Medicine, Pusan, Korea. pedkkt@yahoo.co.kr
2Department of Radiology, Pusan National University, College of Medicine, Pusan, Korea.
Abstract
Childhood ataxia with diffuse central nervous system hypomyelination(CACH) syndrome is a recently described leukodystrophy of unknown etiology. The patients show normal development until the age from 1.5 to 5 years, and sudden deterioration of all motor abilities with irritability is presented after a viral infection or minor head trauma. Brain magnetic resonance imaging(MRI) shows generalized hypointensity of the white matter in T1-weighted image, which turns hyperintense in T2-weighted image, and Proton Magnetic Resonance Spectroscopic Imaging(1H-MRSI) shows reduced signal of N- acetylaspartate, choline, and creatine only in white matter. Dementia is not present and peripheral nerves are normal. We report a case of CACH syndrome who was born with no perinatal problem, and showed normal development until the age of 16 months. She suddenly lost all motor abilities after exanthem subitum who recovered fully over two months. At the age of 18 months she experienced similar attack after chicken pox, and developed seizures at age of 18 months.
Key Words: CACH syndrome, Childhood Ataxia with Diffuse Central Nervous System Hypomyelination syndrome


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