광범위한 대뇌병터를 보인 대뇌형 부신척수신경병증 1예

Abstract

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.