Craniocervical junction (CCJ) malformations are common pathologies in Northeast of Brazil, predominating Basilar Invagination (BI) and Chiari Malformation (CM), sometimes associated to syringomielia (SM).

Analyse the headache pattern in cases with CCJ malformation operated.

Retrospective study of 65 cases with CCJ malformation, operated between 1994 and 2015, with analysis of headache pattern.

Of 65 cases operated, 29 patients (44.6%) had BI and CM, 26 (40%) had BI, CM and SM, 2, only CM and 1, CM and SM. Of all patients, 39 (49.2%) presented headache and 43 (66.1%), nuchal pain. Among those who presented BI and CM, 12 (41.4%) presented headache and nuchal pain association. Of 26 with BI, CM and SM associated, 11 (42.3%) presented headache and nuchal pain association. We notice yet the presence of brachycephaly in 44 cases (67,7%) and brevis collis in 42 (64.6%). Headache, nuchal pain, brachycephaly and brevis collis were all present in 9 patients (31%) of BI and MC group, and in 8 (30.8%) of IB, CM and SM group.

Several denominations referred to psychogenic headache: muscle contraction headache, stress headache and, finally, tension headache, well defined in the classification of International Headache Society (IHS) with clear diagnosis criteria. Bilateral location, predominantly occipital, is an important differential diagnosis with holocranial headache or occipital secondary to craniocervical malformations, and when associated to brachycephaly and brevis colis, should be carefully investigated. Diagnostic failure in symptomatic or neurological deficit’ patients can sequelae due to chronic compression of structures adjacent to CCJ.

The authors have not supplied their declaration of competing interest.