A 36-year-old man was referred to the Danish Headache Center (DHC), Department of Neurology, Glostrup Hospital, University of Copenhagen, by his general practitioner. He had previously been seen by a practicing neurologist, but the treatment response obtained was not satisfactory and he asked for referral to a higher level of the health care system in Denmark. DHC is a national center of excellence that receives patients from all over Denmark and from other countries who have been difficult to treat or have a rare headache disorder or in whom there is doubt regarding the diagnosis.

The patient’s mother and one of her sisters, and possibly also the grandfather (who was deceased), suffered from migraine of uncertain type,.

No previous hospitalizations were reported by the patient, who had recurrent rhinitis diagnosed as pollen allergy and treated with nasal spray. He had no arterial hypertension, asthma, diabetes, or any other chronic diseases.

The patient distinguished between three different types of headache. Some headaches were mild, bilateral, pressing, and induced by stress, and responded to plain analgesics. He experienced such headaches approximately four times a month but they were not bothersome. His main problem was attacks of neurological symptoms followed by severe headaches, occurring about twice a month. These attacks had already begun in childhood, at which time they were rare. About 5 years previously the attack frequency began to slowly increase to the level of the current frequency, which had been present for approximately 2 years. Recently the attacks had become so severe that he was losing 2 days of work every month. These attacks were similar in nature from time to time, varying mainly in severity, with some occurring after vigorous exercise or after exposure to bright light, but the majority of attacks had no obvious cause. The patient described blurring of vision lateral to the point of fixation, which then expanded slowly and developed a serrated edge. As it moved from the central field of vision toward the periphery it enlarged further, and he would notice additionally a blind spot where he was unable to see anything. For example, when he looked at faces, half of the face would sometimes not be visible. Usually this was on the right side but sometimes would also be on the left. The edge was yellow-white and flickering, with no other colors. It took approximately 20 min until all the visual disturbances had disappeared. In some of the attacks he described sensory symptoms usually starting toward the end of the visual disturbance or slightly earlier, which usually began in the fingers of the right hand and moved slowly up the arm to the elbow. He then experienced a tingling sensation in the right side of the chin spreading slowly across the face and into the right half of the tongue. On very rare occasions, and usually when the sensory disturbances affected his tongue, he would also have problems with speech which became slurred, and he would find it difficult to produce the correct words and sentences. The sensory symptoms lasted approximately half an hour. In most of these episodes a severe headache would follow the neurological disturbances, but in some others the headache was only modest, and in a few he would have visual disturbances without any headache. The headache was invariably half-sided but the patient was uncertain as to whether it was on the right or left. He tended to think that it was on the same side (right) as the neurological disturbances. The headache began approximately 20 min after the visual disturbances had disappeared, at a time when he would still experience the sensory and/or speech problems. The pain was severe, throbbing, and aggravated by physical activity. He also experienced nausea and, sometimes, vomiting, and was hypersensitive to light and sound. He was unable to function during attacks, and had to retire to bed in a dark and cool room. The duration of pain was 12–24 h. As treatment he had taken paracetamol and aspirin, without effect. The neurologist had prescribed sumatriptan tablets 50 mg, which he took as soon as the neurological disturbances began, but they had only a minor effect. The neurologist had also prescribed prophylactic treatment with propranolol, which in fact did reduce the attack frequency somewhat, but the patient gained weight and his libido decreased. He had therefore given up to taking sumatriptan.