Abstract
We present a Norwegian family with familial hemiplegic migraine (FHM) with possibly four affected in three generations. The family had a point mutation in the ATP1A2 gene that caused a change of the amino acid valine to methionine (V628 M). The symptoms were pure FHM with intra- and interindividual variability, and epilepsy is not part of the clinical picture. Attacks could be provoked by physical activity. The proband had prolonged attacks of FHM, and was hospitalized due to such an attack provoked by a minor head trauma. The initial management was conservative, but due to persistence of the hemiplegia on day 9, a continuous nimodipine infusion was initiated in order to prevent cerebrovascular vasospasm. However, the nimodipine infusion worsened the patient's symptoms and possibly provoked a generalized tonic-clonic seizure due to vasodilatation and reduced cerebral blood flow. The MRI showed cortical edema and the SPECT showed reduced perfusion on the contralateral side of the hemiplegia. We conclude that nimodipine is contraindicated in the management of prolonged FHM attacks, and recommend conservative management and supplement of sufficient intravenous fluid in nauseated patients in order to avoid hypovolemia.