Exceptional stroke-like episodes in a patient with type I autosomal angioedema

We report on a man with type I hereditary angioedema (HAE) who presented with repeated transient neurologic deficits, totally regressive after a specific C1-inhibitor (C1 INH) injection. Hereditary angioedema, first described in 1888,1 is an autosomal dominant disease, affecting up to 1/50,000 persons, defined in type I as a C1 INH deficiency.2 It is clinically characterized by recurrent edematous crisis at various body sites: mainly relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstructions. Neurologic manifestations are exceptionally reported in this affection.3 ### Case report. A 61-year-old man was admitted for the sudden onset of a right sensory-motor deficit. Since childhood, he had had serpiginous erythema and recurrent abdominal pain attacks. The diagnosis of HAE type I was established when he was 14 years old. The disease was still active in 2008 (2 abdominal or respiratory attacks during 3 or 4 days/month), despite a high dose of prophylactic treatment with danazol (600 mg/day). He was an active smoker (40 packs/year) and had a dyslipidemia probably due to danazol. He had no arterial hypertension. At admission, the deficit was …