Three children with prolonged hemiplegia following severe unilateral headache and having mutations in ATP1A2 are reported from UCLA School of Medicine, Los Angeles, CA; University Children’s Hospital, Zurich, Switzerland; and Wake Forest University School of Medicine, Winston-Salem, NC. Patient 1, a 9-year-old girl developed left-sided numbness and paralysis after 2 days severe right-sided headache. The hemiparesis lasted 5 days and then gradually resolved. Brain MRI and angiography and lumbar puncture were normal. EEG showed slowing in right hemisphere. No previous attacks were reported in the child, but her mother had a history of episodic hemiparesis lasting 30 min to an hour, and accompanied by headache and vomiting, since age 32 years. Patient 2, a 7-year-old boy had headache, somnolence, confusion and left hemiplegia, associated with viral gastroenteritis. Lumbar puncture and initial MRI were normal. Repeat MRI 2 days later showed increased signal intensity on diffusion weighted imaging in right parietal region. Hemiplegia persisted 7 days and gradually resolved over weeks. A generalized tonic-clonic seizure occurred 6 days after onset of hemiplegia. The child had a prior history of typical hemiplegic migraine, lasting 30 min and followed by unilateral headache, often induced by minor head trauma. Family history was negative. Patient 3, a 10-year-old girl developed a headache a few hours after striking her head in a fall while skating. She did not lose consciousness. Two-3 hours later, she developed right-sided numbness, hemiplegia and global aphasia. Symptoms persisted 10 days, the weakness resolved in 3 weeks, and language returned to normal after 7 weeks. Brain MRI on days 2 and 10 were normal. EEG showed slowing on the left that persisted several weeks. Her father had a history of typical hemiplegic migraine beginning at age 7 years; also, episodic visual aura without headache, and a single seizure.

Three distinct ATP1A2 mutations were identified in the FHM2 gene, but none in the FHMl gene of the patients. Affected parents of patients 1 and 3 also had the mutation. In Patient 2, neither parent had the mutation, indicating that the mutation in the child arose de novo. [1]

COMMENT. Episodes of hemiplegic migraine usually last from 30 to 60 min, but patients with more prolonged hemiparesis are reported. FHMl is typically associated with cerebellar findings, and FHM2 with seizures. More than 20 mutations in ATP1A2 have been found to cause FHM2. The MRI is generally normal, but the EEG may show slowing over the affected hemisphere.

Altered arterial function in migraine of recent onset was demonstrated using ultrasound and applanation tonometry in 50 patients and 50 controls, at Ghent University, Belgium. Brachial artery diameter was decreased, while carotid arterial wall properties were unchanged during a headache-free interval. Flow-mediated vasodilation of the brachial artery was decreased in patients with migraine for >1 yr and <6 yrs duration, indicative of endothelial vasomotor dysfunction. [2, 3]