Blindness, tetraspasticity, and other signs of irreversible brain damage in hereditary angioedema

Hereditary angioedema (HAE) caused by a genetic C1 esterase inhibitor (C1-INH) deficiency (HAE-C1-INH) is clinically characterized by recurrent skin swellings, abdominal pain attacks, and, more rarely but potentially life-threatening, upper airway obstruction (supraglottic edema, laryngeal attacks). Cerebral symptoms are rare in HAE-C1-INH.1,2 HAE-C1-INH attacks, including laryngeal attacks, usually occur unpredictably. They may reach their maximal intensity usually 1 to 12 hours after onset, reflecting some variability in the development time.

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