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A Longitudinal Exploration of CACNA1A -related Hemiplegic Migraine in Children
Insights
The course of CACNA1A-related hemiplegic migraine (HM) is unpredictable in children, with no clear patterns in timing or severity. Close monitoring is crucial, even after symptom-free periods, as events can change drastically.
Area of Science:
- Neurology
- Genetics
- Pediatric Neurology
Background:
- Hemiplegic migraine (HM) associated with CACNA1A gene variants has a broad clinical spectrum.
- The longitudinal progression of HM throughout childhood remains poorly understood.
Purpose of the Study:
- To analyze the longitudinal course, frequency, and severity of HM in individuals with CACNA1A-related disorders.
- To assess the impact of epilepsy on HM and evaluate medication efficacy for HM management.
Main Methods:
- Retrospective analysis of HM and seizure history in 15 individuals (3-29 years) with CACNA1A variants.
- Standardized monthly assessment of HM events and medication data.
- Statistical analysis to determine correlations and treatment responses.
Main Results:
- HM onset ranged from 14 months to 13 years; 25% of events were severe (>3 days).
- HM timing and severity showed significant variability between events; epilepsy (in 53%) did not correlate with HM patterns.
- Levetiracetam and acetazolamide were ineffective; verapamil showed efficacy in preventing HM episodes.
Conclusions:
- CACNA1A-related HM exhibits unpredictable longitudinal patterns in timing and severity.
- Epilepsy does not influence HM course, emphasizing the need for continuous surveillance.
- Verapamil may be a potential treatment option for preventing HM episodes in this cohort.
Introduction:
Since the initial description of CACNA1A- related hemiplegic migraine (HM), the phenotypic spectrum has expanded from mild episodes in neurotypical individuals to potentially life-threatening events frequently seen in individuals with developmental and epileptic encephalopathies. However, the overall longitudinal course throughout childhood remains unknown.
Methods:
We analyzed HM and seizure history in individuals with CACNA1A -related HM, delineating frequency and severity of events in monthly increments through a standardized approach. Combining these data with medication prescription information, we assessed the response of HM to different agents.
Results:
Our cohort involved 15 individuals between 3 and 29 years (163 patient years) and included 11 unique and two recurrent variants (p.R1349Q and p.V1393M; both n= 2). The age of first confirmed HM ranged from 14 months to 13 years (average 3 years). 25% of all HM events were severe (lasting >3 days) and 73% of individuals had at least 1 severe occurrence. Spacing of HM events ranged from 1 month to 14 years and changes in HM severity over time of showed increases or decreases of >2 severity levels in 12/122 events. Eight individuals had epilepsy, but severity of epilepsy did not correlate with frequency and severity of HM events. While levetiracetam ( n= 6) and acetazolamide ( n= 5) were the most frequently used medications, they did not show efficacy in HM prevention or HM severity reduction. However, verapamil ( n= 3) showed efficacy in preventing HM episodes (OR 2.68, CI 1.39-5.67).
Significance:
The longitudinal course of CACNA1A -related HM lacks recognizable patterns for timing and severity of HM events or correlation with seizure patterns. Our data underscores the unpredictability of CACNA1A -related HM, highlighting the need for close surveillance for reoccurring HM events even in individuals with symptom-free periods.
Key Points:
24% of hemiplegic migraines (HM) in CACNA1A- related disorders are severe, involving cerebral edema and greater than 4 days to recover Timing and severity of HM are unpredictable, with large changes in severity between events, and age of onset ranging from 1-13 yearsEpilepsy occurred in 53% of individuals, with neither the timing nor severity of seizures correlated with HM.
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