Acute encephalopathy in familial hemiplegic migraine with ATP1A2 mutation

Merwick, Aine
Fernandez, Desiree
McNamara, Brian
Harrington, Hugh

¹Neurology Department, National Hospital for Neurology and Neurosurgery, London, UK
²Nelson Hospital, Nelson-Marlborough DHB, Nelson, New Zealand
³Department of Neurology, Wellington Regional Hospital, Capital and Coast District Health Board, Newtown, Wellington, New Zealand
⁴Neurophysiology Department, Cork, University Hospital, Cork, Ireland
⁵Neurology Department, Mercy University Hospital, Cork, Ireland

BMJ Case Reports 2013, 2013. | DOI: 10.1136/bcr-2013-009750

Familial hemiplegic migraine is a rare subtype of migraine with aura which includes motor weakness. A 32-year-old woman with known familial hemiplegic migraine (point mutation in Exon 22 of the ATP1A2 gene) presented with an acute confusional state, after an initially typical migraine. On examination, she had fever (38°C), agitated, with a right hemiparesis and dysphasia. Electroencephalography showed slowing of α rhythm and continuous rhythmical δ activity in the left hemisphere. She recovered 48 h after the onset of encephalopathic episode. Electroencephalography after recovery showed resolution of the abnormal slowing of the α waveforms.