Annals of Clinical Neurophysiology
- 제9권2호
- /
- Pages.97-101
- /
- 2007
- /
- 2508-691X(pISSN)
- /
- 2508-6960(eISSN)
광범위한 대뇌병터를 보인 대뇌형 부신척수신경병증 1예
A Case of Cerebral Adrenomyeloneuropathy with Extensive Cerebral Lesions
- Kim, Hyun-Jung (Department of Neurology, Seoul National University College of Medicine) ;
- Min, Ju-Hong (Department of Neurology, Chung Ang University Medical Center) ;
- Lee, Kwang-Woo (Department of Neurology, Seoul National University College of Medicine)
- 발행 : 2007.12.30
초록
We report a 31-year-old man with cerebral adrenomyeloneuronopathy variant, who presented as progressive gait disturbance. He had spastic paraparesis, hyperreflexia without Babinski's sign and sensory symptom. No adrenal insufficiency was noted. Brain MRI showed extensive high signal intensities in bilateral temporal lobes and posterior periventricular white matter in T2 weighed imaging without cerebrospinal fluid abnormality. His nerve conduction study showed sensorimotor demyelinating polyneuropathy and the level of saturated very-long-chain fatty acids was high in his plasma, although neuropsychological test was normal.