Diagnostic and Therapeutic Challenges in a Child with Suspected Hemiplegic Migraine

Background/Purpose: An 11-year-old girl presented with headache, vomiting, and somnolence after a minor head trauma. Neuroimaging revealed left hemispheric cortical edema, EEG showed a severe slowing of the left hemisphere, and CSF analysis was unremarkable. Six years earlier, the patient had suffered focal status epilepticus with Todd's paresis after a minor head trauma, with right hemispheric cortical swelling, and complete recovery. With suspected hemiplegic migraine, therapy with methylprednisolone 100 mg/d for 5 days and levetiracetam was started, and genetic testing initiated. Because of persistent somnolence after 4 days, follow-up imaging was performed, which showed new high-grade stenosis of the left distal internal carotid artery, middle cerebral artery, and anterior cerebral artery with wall enhancement DD vasculitis, with perfusion delay, and diffusion restriction in the left media stream area. Hours later, the patient manifested aphasia, hemiplegia of the left arm, and focal seizures.

Methods: Literature search.

Results: Two publications (1 case report, 1 case series including 8 patients) on vasospasm in children with familial hemiplegic migraine were found. Treatment was started to cover both the neuroradiologically suspected vasculitis with consecutive stroke as well as vasospasm. High-dose corticosteroids, acetylsalicylic acid, and IV verapamil were initiated. The vascular stenosis resolved within days. Over the course of months, the girl showed complete normalization of clinical, neuroimaging, and electroencephalographic findings. A mutation in the ATP1A2 gene was detected (FHM2), confirming the suspected diagnosis.

Conclusion: In familial hemiplegic migraine, severe vasospasms can occur and may lead to dramatic neuroimaging findings, leading to confusion in diagnosis. The ideal therapy is not known. High-dose corticosteroids, verapamil IV (especially in CACNA1A mutation), or ketamine intranasally is discussed. Prophylactic treatment between attacks can be useful.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
13 November 2023