Brain abnormalities after vigabatrin for infantile spasms

2021 ◽  
Vol 1883 (1) ◽  
pp. 7-7
2021 ◽  
Author(s):  
Tomer Stern ◽  
Liora Kornreich ◽  
Hadassa Goldberg

Abstract Background We aimed to find the clinical significance of brain abnormalities on magnetic resonance imaging (MRI) in epilepsy and the lateralization of these findings with electroencephalogram (EEG). Methods We retrospectively analyzed the results of all EEGs and brain MRIs of 600 consecutive epilepsy patients from 1998 to 2020. Results Data were available for 563 cases (267 females). Ninety percent of the patients were 18 years old or younger. A total of 345 patients (61.3%) had focal epilepsy, 180 (32%), generalized, and 38 (6.7%), inconclusive. In 187 (33.2%), the first MRI was abnormal and in 81 (out of 108 repeated MRI), the second was pathological. The most frequent brain abnormalities were cortical dysplasia in 41 (18.1%), other structural abnormalities in 25 (11%), various phacomatoses in 23 (10.1%), and mesial temporal sclerosis in 17 (7.5%). Among 226 patients with abnormal MRI, 171 (75.6%) had focal epilepsy when compared with 36 (15.9%) with generalized epilepsy (p <0.001). In 121 patients (53.5%), the result of the abnormal MRI contributed significantly to the understanding of the epilepsy etiology. The side of abnormality was lateralized to the EEG focus in 120 cases (53%); in 10/15 cases with infantile spasms (66%), MRI was significantly abnormal. In 33, in whom the first MRI was normal, a second MRI revealed a significant abnormality. Conclusion Brain MRI is an important tool in epilepsy diagnosis, mainly in focal seizures and infantile spasms. A repeat MRI is mandatory in intractable focal cases to improve the yield of this test.


Epilepsia ◽  
2017 ◽  
Vol 58 (4) ◽  
pp. 674-682 ◽  
Author(s):  
Shaun A. Hussain ◽  
Jackie Tsao ◽  
Menglu Li ◽  
Madeline D. Schwarz ◽  
Raymond Zhou ◽  
...  

Author(s):  
Divya Nagabushana ◽  
Aparajita Chatterjee ◽  
Raghavendra Kenchaiah ◽  
Ajay Asranna ◽  
Gautham Arunachal ◽  
...  

Abstract Introduction IQSEC2-related encephalopathy is an X-linked childhood neurodevelopmental disorder with intellectual disability, epilepsy, and autism. This disorder is caused by a mutation in the IQSEC2 gene, the product of which plays an important role in the development of the central nervous system. Case Report We describe the symptomatology, clinical course, and management of a 17-month-old male child with a novel IQSEC2 mutation. He presented with an atypical Rett syndrome phenotype with developmental delay, autistic features, midline stereotypies, microcephaly, hypotonia and epilepsy with multiple seizure types including late-onset infantile spasms. Spasms were followed by worsening of behavior and cognition, and regression of acquired milestones. Treatment with steroids led to control of spasms and improved attention, behavior and recovery of lost motor milestone. In the past 10 months following steroid therapy, child lags in development, remains autistic with no further seizure recurrence. Conclusion IQSEC2-related encephalopathy may present with atypical Rett phenotype and childhood spasms. In resource-limited settings, steroids may be considered for spasm remission in IQSEC2-related epileptic encephalopathy.


2006 ◽  
Vol 37 (S 1) ◽  
Author(s):  
KL Lin ◽  
HS Wang
Keyword(s):  
Low Dose ◽  

2006 ◽  
Vol 37 (S 1) ◽  
Author(s):  
F Buruiana ◽  
D Plesca ◽  
R Teleanu ◽  
AM Davitoiu ◽  
M Moiceanu ◽  
...  
Keyword(s):  

2006 ◽  
Vol 37 (S 1) ◽  
Author(s):  
K Darke ◽  
S Edwards ◽  
E Hancock ◽  
A Johnson ◽  
C Kennedy ◽  
...  

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