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Cluster Headache with Short-Lived Visual Symptoms

A 72-year-old retired woman was evaluated for recurrent bouts of severe left temporal-orbital headaches that began about 32 years earlier. She described the headaches as throbbing with an occasional stabbing quality. Her headaches reached their maximum intensity within a few minutes. Each attack lasted from 30 to 45 min, then subsided, only to be followed by another attack within 30–60 min, totalling up to eight attacks per day. The bouts of headache persisted for several weeks only to remit for several months. Each of the headaches was accompanied by lacrimation and injection in the left eye, rhinorrhoea in the left nostril, osmophobia and occasional photophobia. She denied nausea, vomiting, sensory or motor symptoms. She denied any personal or family history of headaches suggestive of migraine. However, she reported a first cousin with cluster headache (CH). For over 30 years, approximately 50% of the headache cycles were preceded by a brief visual disturbance consisting of ‘flashing lights’ or ‘zigzagging lines’ that occurred 3–7 days before the onset of the first headache in the bout. These visual symptoms lasted from 30 s to about 3 min and never occurred without a subsequent headache cycle. Furthermore, her visual symptoms never occurred after the headache cycle had started or within an hour prior to an individual headache. In addition to the visual symptoms, she described a feeling of scalp tenderness on the left side of her head that occurred either with or soon after the visual symptoms and preceded the headache cycle’s onset by several days. Initially, headache bouts were sporadic and occurred unpredictably at any time of the year, but during the 5 years prior to presentation, her headache bouts became seasonal (autumn and spring only) and became more excruciating. She could recall neither any triggering factors nor any relationship between the headaches and her menstrual cycle prior to menopause. The patient had tried a number of treatments including O2, indomethacin and lowdose verapamil without success. She obtained some relief from her headaches by applying ice pack to the painful side of her head. Magnetic resonance imaging (MRI) of the head with and without contrast showed small vessel white matter changes in the frontal subcortical white matter. No abnormalities were present in the occipital cortices. There was a right frontal arachnoid cyst and an ovoid 1–2-cm area of bright T1 signal in the anterior parasagittal frontal region that may represent calcified meningioma. Magnetic resonance angiography (MRA) showed no significant carotid or vertebrobasilar stenosis or arteriovenous malformation (AVM). EEG was performed and was normal and without epileptiform discharge or focal dysrhythmia. A transoesophageal echocardiogram showed no patent foramen ovale. The patient was treated with verapamil 80 mg three times a day because the initial trial of verapamil was inadequate in dose and duration. The patient was interviewed over the phone after 3 months and seen in follow-up 9 months after her initial visit. She denied having headaches or visual symptoms since her initial visit even at the expected times, during the autumn or in the spring.