Trigeminal autonomic cephalgia with periorbital ecchymosis, ocular hemorrhage, hypertension and behavioral alterations

We describe a 38-year-old male in whom severe unilateral headache was associated with marked palpebral edema, periorbital ecchymosis, lacrimation, conjunctival injection, nasal congestion and rhinorrhea. A second, less severe headache form developed subsequently. The patient often presented severe labile hypertension and behavioral disturbances during the crises, and there was an episode of intra-ocular hemorrhage. General, neurological and ophthalmological examinations revealed nothing remarkable. We discuss possible pathogenetic mechanisms and the nosology of this case within the trigeminal autonomic cephalgias.