A Chinese patient with epilepsy and WWOX compound heterozygous mutations.
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.
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