Paroxysmal neuralgic upper cervical pain attacks: the lower syndrome of cluster headache

We present a group of seven patients with a lower syndrome (LS) of cluster headache (CH). Seventy-three newly diagnosed patients with CH were subjected to a 9 years follow-up study; 66 patients were classified as upper syndrome (US) and only seven patients (9.5%) as LS. We focus on the characteristics of this group of seven patients with LS and compare them with existing literature. The seven cases with LS illustrate the wider spectrum of clinical manifestations that can occur in CH, namely infraortibal symptoms or these outside the territory of the trigeminal branches such as the upper cervical region or the craniocervical margin. The duration of the attacks can last more than 180 min. The attacks show a circadian/circannual regularity and a stereotypic pattern of symptoms in most of the patients. The severity and duration of the attacks may increase over the years. The neck pain can overshadow all the other symptoms of CH. Because of the regularity of the attacks at particular times of day or night in some patients, even abortive therapy can be used as prevention when taken some hours before the suspected attack. We hypothesize that some patients with LS may represent an anatomical-functional variant of a primary chronic neurovascular pain disorder originating from the central nervous system, with possible involvement of the hypothalamus and the trigeminovascular (TV) system, with inputs from the cervical roots C(0)-C(2). Clinicians should consider CH when pain attacks are located outside the orbitotemporal regions, but fulfil the other diagnostic criteria for CH and should try ergotamine, oxygen, sumatriptan, verapamil, steroids or even combinations in these patients.