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A Chinese patient with epilepsy and WWOX compound heterozygous mutations.
He, Jing; Zhou, Wenjing; Shi, Jie; Zhang, Bingqing; Wang, Haixiang.
Affiliation
  • He J; Department of Epilepsy Center, Tsinghua University Yuquan Hospital, Shijingshan District, Beijing, China.
  • Zhou W; Department of Epilepsy Center, Tsinghua University Yuquan Hospital, Shijingshan District, Beijing, China.
  • Shi J; Department of Epilepsy Center, Tsinghua University Yuquan Hospital, Shijingshan District, Beijing, China.
  • Zhang B; Department of Epilepsy Center, Tsinghua University Yuquan Hospital, Shijingshan District, Beijing, China.
  • Wang H; Department of Epilepsy Center, Tsinghua University Yuquan Hospital, Shijingshan District, Beijing, China.
Epileptic Disord ; 22(1): 120-124, 2020 Feb 01.
Article in En | MEDLINE | ID: mdl-32051108
Early infantile epileptic encephalopathy type 28 is a refractory epilepsy with early onset, poor prognosis, and hereditary causes. WW domain-containing oxidoreductase (WWOX) gene mutation can result in epileptic encephalopathy, but the mechanism remains unclear. We present the case of a patient with epilepsy and WWOX compound heterozygous mutations. The seizures manifested as tonic-clonic, convulsive and were refractory to drugs. Magnetic resonance imaging showed a widened subarachnoid space and thin corpus callosum. The patient died from asphyxia at the age of one year and 23 days. Peripheral blood was taken from the patient and his parents, and whole-exome sequencing was investigated to determine possible gene mutation. Two compound heterozygous mutations were identified: c.172+1G>C (with no amino acid change) and c.984C>G (amino acid change: p.Tyr328Ter). The pathophysiology of epileptic encephalopathy related to the WWOX gene remains to be determined, and further studies are required to elucidate possible mechanisms.
Subject(s)
Key words

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Tumor Suppressor Proteins / Epileptic Syndromes / WW Domain-Containing Oxidoreductase Type of study: Prognostic_studies Limits: Humans / Infant / Male Country/Region as subject: Asia Language: En Journal: Epileptic Disord Journal subject: CEREBRO / NEUROLOGIA Year: 2020 Document type: Article Affiliation country: China Country of publication: United States

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Tumor Suppressor Proteins / Epileptic Syndromes / WW Domain-Containing Oxidoreductase Type of study: Prognostic_studies Limits: Humans / Infant / Male Country/Region as subject: Asia Language: En Journal: Epileptic Disord Journal subject: CEREBRO / NEUROLOGIA Year: 2020 Document type: Article Affiliation country: China Country of publication: United States