AOCCN2017

講演情報

Poster Presentation

[P1-142~216] Poster Presentation 1

2017年5月11日(木) 09:30 〜 16:00 Poster Room B (1F Argos F)

[P1-185] Extensive White Matter Lesions in Wilson’s Disease

Wen-Yu LU (Department of Pediatrics, Min-Sheng General Hospital, Taoyuan, Taiwan)

Wilson’s disease (WD) is an autosomal recessive neurodegenerative disease secondary to abnormal copper metabolism with variable phenotypic expression. It is caused by mutations in ATB7B. The MRI changes in WD predominantly affect the subcortical structures and basal ganglia. Neurological forms with extensive cortical and subcortical white matter involvement represent a rare subgroup. According to previous reports, only approximately10% of WD have destruction predominantly over the cerebral white matter and cerebral cortex. The white matter lesions were present with a predilection to the frontal lobes. Besides, increased seizure occurrence and poor outcome were seen in these patients. We present a rare case with WD, who had extensive cortical and subcortical lesions accompanied with unusual seizures as epilepsia partialis continua. We subsequently review the literature concerning extensive white matter lesions and their presentations in WD.