Our results confirm the long-term response to onabotA in three-quarters of CM patients. After one year, lack of response occurs in about one out of 10 patients and injections can be delayed, but not stopped, to four months in around 40% of patients. Except for local muscle atrophy in two cases treated more than five years, adverse events are comparable to those already described in short-term clinical trials.

Increased interictal VIP level measured in peripheral blood could be a biomarker helping in CM diagnosis, though it does not clearly differentiate between EM and CM.

Objectives: To analyze the trajectory to diagnosis and information provided in a series of cluster headache (CH) patients from five headache clinics. Methods: CH patients were asked to fill in an ad hoc questionnaire. Results: Seventy-five patients (mean age 41.5 years, 67 males) completed the questionnaire. Patients had visited during an average of 4.9 years a mean of 4.6 physicians who had obtained 2.5 neuroimaging procedures per patient before getting a diagnosis of CH. Sixty-three (84%) had received no diagnosis (21 cases; 28%), while 43 (57%) had been given an average of 2.1 alternative diagnoses. Migraine, trigeminal neuralgia and sinusitis were the most frequent mistakes. After diagnosis, 55% had subjectively received poor/very poor information on CH. Ninety-five percent had poor or incorrect information about the nature of the disease, or acute (70%) and preventive (61%) treatments. Etiology (90%), management options (36%) and potential adverse events of medications (29%) were their main information demands. Conclusions: Although CH is an invalidating and clinically clear-cut disorder suffered by around 1/1,000 people, it is still frequently unrecognized and/or mistaken for other disorders, which calls for a better knowledge and education in the diagnosis of the main primary headaches.

Activity-related headaches can be provoked by Valsalva maneuvers ("cough headache"), prolonged exercise ("exertional headache") and sexual excitation ("sexual headache"). These entities are a challenging diagnostic problem as can be primary or secondary and the etiologies for secondary cases differ depending on the headache type. In this paper we review the clinical clues which help us in the differential diagnosis of patients consulting due to activity-related headaches. Cough headache is the most common in terms of consultation. Primary cough headache should be suspected in patients older than 50 years, if pain does not predominate in the occipital area, if pain lasts seconds, when there are no other symptoms/signs and if indomethacin relieves the headache attacks. Almost half of cough headaches are secondary, usually to a Chiari type I malformation. Secondary cough headache should be suspected in young people, when pain is occipital and lasts longer than one minute, and especially if there are other symptoms/signs and if there is no response to indomethacin. Every patient with cough headache needs cranio-cervical MRI. Primary exercise/sexual headaches are more common than secondary, which should be suspected in women especially with one episode, when there are other symptoms/signs, in people older than 40 and if the headache lasts longer than 24 hours. These patients must have quickly a CT and then brain MRI with MRA or an angioCT to exclude space-occupying lesions or subarachnoid hemorrhage.

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