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Brain atrophy following hemiplegic migraine attacks
Nadine Pelzer1, Evelien S Hoogeveen2, Michel D Ferrari1
11 Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands.
Severe hemiplegic migraine (HM) attacks can cause brain atrophy. Early prophylactic treatment may prevent permanent neurological damage in patients with CACNA1A mutations.
Area of Science:
- Neurology
- Neurogenetics
Background:
- Hemiplegic migraine (HM) is a rare subtype of migraine with aura.
- Pathophysiology of progressive neurological deterioration in HM remains unclear.
Observation:
- A 16-year follow-up of a patient with a de novo p.Ser218Leu CACNA1A mutation and severe HM attacks.
- Patient experienced nine severe HM attacks with seizures and consciousness decline between ages 3-12.
Findings:
- Neuroimaging revealed cytotoxic edema during HM attacks, leading to hemisphere atrophy.
- Progressive cerebellar atrophy was observed; brain atrophy halted after attack cessation.
- Prophylactic treatment with flunarizine and sodium valproate may have halted atrophy progression.
Implications:
- Severe HM attacks can cause irreversible brain damage, including atrophy.
- Early prophylactic treatment is crucial to prevent permanent neurological deficits in HM patients.
- This case highlights the importance of early intervention in managing severe HM and preventing long-term consequences.
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