Defining neuralgiform headache with ipsilateral autonomic symptoms: case report in a headache center

Patients who fulfill the diagnostic criteria for primary headaches of the IHS Classification of Headache Disorders (2nd edn [1]) often consult an internal medicine specialist [2]. Headache is a common complaint in the general population. The 1-year prevalence of tension-type headache in men is 30–60% and in women 35–85% while the corresponding prevalence for migraine approximates 5% in men and 15% in women [3–5]. In addition to migraine and tension-type headache the IHS classification comprises a group called Trigeminal Autonomic Cephalalgias (TACs) including cluster headache, paroxysmal hemicrania and Short lasting Unilateral Neuralgiform headache with Conjunctival injection and Tearing (SUNCT). Clinically, they share halfsided head pain with prominent autonomic features. Particularly, SUNCT is very rare [6], sometimes can coexist with trigeminal neuralgia [7] and generally the differentiation between these two syndromes, which require specific treatment, is difficult. Diagnostic criteria for SUNCT are: (a) at least 20 attacks fulfilling criteria b–d, (b) attacks of unilateral orbital, supraorbital or temporal stabbing or pulsating pain lasting 5–240 s, (c) pain is accompanied by ipsilateral conjunctival injection and lacrimation, (d) Attacks occur with a frequency from 3 to 200 per day, (e) not attributed to another disorder. We present the case of a 65 year-old male admitted to our Headache Centre with a 8-year history of headache phases characterized by severe strictly left-side pain involving the eye, and nose with some extension to upper jaw. The patient’s past medical history indicated only arterial hypertension that had commenced ten years prior that was well controlled with drug therapy (association of delapril 30 mg and indapamide 2.5 mg/day). There was no family history of migraine or other headache syndromes. When he was 59 years old, the first period of headache attacks appeared. The pain was excruciating with a stabbing quality but not throbbing, and was associated with ipsilateral prominent conjunctival injection, tearing and nasal obstruction The attacks lasted from 1 to 10 s with absence of pain between attacks. The frequency of the crisis was 20–100 a day, and some attacks were triggered by speaking, swallowing, chewing. The pain remitted for variable periods. At the time of our observation, general and neurological examinations were normal. Routine blood tests and urinalysis were unremarkable. This included thyroid stimulating hormone, free thyroxine, adrenocorticotrophic hormone, testosterone, prolactin at 0 and 30 min, growth hormone (because of some case reports where SUNCT was associated with pituitary microadenomas [8–10]). MRI of brain revealed bilaterally multiple, ischemic areas in the white matter, no alterations of pituitary gland and the MRI of the cerebral district showed any alterations. The patient experienced four active periods of headaches between 59 and 62 years of age, which lasted from 10 to 15 days with the same symptoms. Before coming under our care, in his long lasting evolution the patient had been treated with different symptomatic medications e.g. ketorolac sublingual 30 mg, and tramadole 15 mg three times a day and prophylactic drugs without significant improvement. Carbamazepine had little effect and was abandoned because of side effects (particularly constipation); oral pregabalin (75 mg two times a day) and gabapentin (400 mg two times a day), not given in F. De Cesaris (&) M. Fanciullacci U. Pietrini B. Anselmi E. Del Bene Headache Center, Department of Internal Medicine, University of Florence, Villa Monna Tessa, Viale Pieraccini, 18, 50139 Florence, Italy e-mail: f.decesaris@dmi.unifi.it