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Malformations of cortical development and epilepsy: A cohort of 150 patients in western China
Wenyu Liu1, Dongmei An1, Jiahe Xiao2
1Departments of Neurology, West China Hospital, Sichuan University, No. 37 Guo Xue Alley, Chengdu 610041, China.
Insights
Malformations of cortical development (MCDs) cause refractory epilepsy, especially in resource-limited areas. Multiple MCDs present with more severe symptoms and poorer outcomes than simple MCDs.
Area of Science:
- Neuroscience
- Developmental Neuroscience
- Epileptology
Background:
- Malformations of cortical development (MCDs) are a significant cause of refractory epilepsy.
- Understanding the spectrum, distribution, and clinical features of MCDs is crucial, particularly in resource-limited settings.
- Existing knowledge on MCDs in diverse populations and their long-term prognosis remains limited.
Purpose of the Study:
- To investigate the distribution and classification of MCDs in a Chinese epilepsy cohort.
- To compare the clinical features and long-term prognosis of Simple versus Multiple MCD forms.
- To identify specific MCD subtypes and their associated clinical characteristics.
Main Methods:
- Retrospective analysis of 150 epilepsy patients with radiologically diagnosed MCDs at a tertiary center in western China.
- Classification of MCDs based on the Barkovich classification system.
- Categorization into Simple (single MCD type) and Multiple (co-existing abnormalities) forms for comparative analysis.
Main Results:
- Focal cortical dysplasia was the most common MCD type; perinatal insults were noted in Group III.
- Multiple MCD (36/150 patients) was linked to delayed milestones, cognitive impairment, and neurological deficits compared to Simple MCD.
- Multiple MCD patients exhibited earlier seizure onset, younger assessment age, lower seizure-free rates, and worse overall outcomes, with extra-temporal epilepsy being more prevalent.
Conclusions:
- MCDs represent a critical and challenging cause of epilepsy, especially in resource-limited countries.
- Advanced imaging is vital for diagnosing and classifying cortical malformations.
- The presence of multiple malformations significantly worsens clinical features and long-term prognosis, necessitating individualized treatment strategies.
Purpose:
Malformations of cortical development (MCDs) are abnormalities of the cerebral cortex that arise from abnormal formation of the cortical plate, and have become increasingly identified as an important etiology for refractory epilepsy. Little is known about the spectrum, distribution and clinical features of MCDs, especially in resource-limited regions. This study investigates the distribution of MCDs and compares the clinical features and long-term prognosis between the two forms of MCDs: Simple and Multiple.
Method:
One hundred and fifty epilepsy patients (138 adults, 12 pediatric patients) with radiologically diagnosed MCDs were identified at a tertiary epilepsy center in western China. Patients were divided into three subtypes according to the Barkovich classification. They were further divided into either Simple or Multiple MCD forms based on whether they had a single type of MCDs or other co-existing developmental brain abnormalities.
Results:
The most common type of MCD is focal cortical dysplasia. We found perinatal insults more common in group III patients. Multiple MCD was identified in 36 of 150 patients, and was associated with higher rates of delayed milestones (p=0.005), cognitive impairment (p=0.023) and neurological deficits (p=0.002) compared to Simple MCD. Extra-temporal epilepsy was more commonly seen among individuals with Multiple MCD (p=0.017). Participants with Multiple MCD were younger at time of seizure onset (p=0.003) and at assessment (p=0.002), had a lower seizure-free rate (p=0.033) and had worse outcomes overall. Patients with heterotopias were more commonly associated with other abnormalities.
Conclusion:
MCDs are a critical cause of epilepsy and pose a big challenge for resource-limited countries. Imaging techniques are crucial in diagnosing and classifying cortical deformities. Multiple malformations lead to more severe clinical features and worse prognosis. Identifying and classifying MCDs can help physicians to better estimate patient prognosis and seek the best, individualized therapeutic options.
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