Familial Hemiplegic Migraine

抄録

A 13 year old boy with hemiplegic migraine is reported. Three other family members, a grandfather, an aunt and an uncle are similarly affected.
The patient had an attack of severe nausea and vomiting at age 4. He had episodes of nausea, vomiting and headaches every 3-4 months since the age of 5. At 11 years of age he started to have transient hemiparesis and hypesthesia associated with his attacks. The attack would usualy be initiated by blurred vision in the right half of the visual field of each eye. This was followed by numbness around the mouth with difficulty in speech. Numbness and weakness ascended to the right leg to involve the right arm. Then severe throbbing headache developed in about 30-45 minutes. At the height of headache he would vomit repeatedly. During headache the patient felt drowsy and muddled. As these symptoms faded away, visual disturbance and then numbness and weakness gradually decreased. The condition cleared completely in 24-48 hours.
Phsical examination revealed convergence insufficiency of the left eye, horizontal nystagmus on lateral gaze and vertical nystagmus on upward gaze. Otherwise neurological examination was unremarkable. EEG's recorded at age 13 and 14 were normal while the waking record performed at age 16 revealed a few 3.5-4.5c/s slow waves in both occipital and post-temporal areas. Carotid angiogram was normal. Vertebral angiography reveald insufficient filling of vertebro-basilar system.