In:
Epileptic Disorders, Wiley, Vol. 23, No. 4 ( 2021-08), p. 579-589
Abstract:
Objective . To unveil current medical and psychosocial conditions of patients with West syndrome in Japan. Methods . A cross‐sectional analysis was performed in patients with West syndrome registered in the Rare Epilepsy Syndrome Registry (RES‐R) of Japan. Furthermore, new‐onset patients registered in the RES‐R were observed prospectively and their outcomes after one and two years of follow‐up were compared with data at onset. Results . For the cross‐sectional study, 303 patients with West syndrome were included. Seizures (such as spasms, tonic seizures and focal seizures) occurred daily in 69.3% of the patients at registration. Seizure frequency of less than one per year was observed in cases of unknown etiology (22.6%), genetic etiology (23.8%) and malformation of cortical development (MCD; 19.1%). Neurological findings were absent in 37.0%, but a high rate of abnormality was seen in patients with Aicardi syndrome, hypoxic‐ischemic encephalopathy (HIE), genetic etiology and MCD other than focal cortical dysplasia, accompanied by a 〉 50% rate of bedridden patients. Abnormal EEG was found in 96.7%, and CT/MRI was abnormal in 62.7%. Treatments included antiepileptic drug therapy (94.3%), hormonal therapy (72.6%), diet therapy (8.3%) and surgery (15.8%). Intellectual/developmental delay was present in 88.4%, and was more severe in patients with Aicardi syndrome, genetic etiology and HIE. Autism spectrum disorder was found in 13.5%. For the longitudinal study, 27 new‐onset West syndrome patients were included. The follow‐up study revealed improved seizure status after two years in 66.7%, but worsened developmental status in 55.6%, with overall improvement in 51.9%. Significance . The study reveals the challenging neurological, physical and developmental aspects, as well as intractable seizures, in patients with West syndrome. More than a half of the children showed developmental delay after onset, even though seizures were reduced during the course of the disease.
Type of Medium:
Online Resource
ISSN:
1294-9361
,
1950-6945
DOI:
10.1684/epd.2021.1301
Language:
English
Publisher:
Wiley
Publication Date:
2021
detail.hit.zdb_id:
2118181-0
