1. Henrik Schytz Staff specialist, MD, PhD, DMSc Danish Headache Center, Department of Neurology Rigshospitalet Glostrup Danish Headache Center

2. Case 20-year old man want to be treated for migraine by his G.P. Mother and sister have severe migraine. Patient has had severe left-sided headache localized to temporal region and eye for one year. Additional history? Attacks last typically 45 minutes and occurs most often during night. From 1-5 attacks per day. May occur periodically. Pain is relieved by physical activity. Nose is running, eye gets red. Diagnosis and treatment? Cluster headache. At next cluster period oxygen 10-12 litres per minute for 15 minutes. Sumatriptan s.c. Prophylactic treatment with verapamil

3. Cluster headache, what is it? Affects 0.1-0.3 % Age at onset: 2.decade (range 3-67 years) M/F ratio: 3/1 Underdiagnosed and undertreated 9 years diagnostic delay with a wide range in DK: 2 months-62 years (Jensen et al 2006)

4. Neuronal activation during cluster attacks May et al., Nat Med 1999

5. DANISH HEADACHE CENTER Cortex Caudal trig. Nc. Thalamus Trigeminal ganglion Brainstem Dura mater Sphenopalatine ganglion Sup sal. Nc. Hypothalamus Cavernous sinus ICA n. interm. lat. Sup Cerv. Ganglion Eye Pathogenesis of CH -The Trigeminal Autonomic Reflex Martins, Gouveia and Parreira 2005

6. Severe unilateral, orbital, supraorbital and/or temporal pain lasting 15- 180 minutes when untreated, occurring 0.5-8 times a day Either or both of the following: 1. At least one of the following ipsilateral to the headache a) conjunctival injection and/or lacrimation b) nasal congestion and/or rhinorrhoea c) eyelid oedema d) forehead and facial sweating e) forehead and facial flushing f) sensation of fullness in the ear g) miosis and/or ptosis 2. A sense of restlessness or agitation Cluster headache (Horton’s headache)

7. TEMPORAL PATTERNS OF HEADACHE Migraine Chronic Tension- Type Headache Cluster Headache

8. Treatment of cluster headache Acute treatment –Inhalation of 100% oxygen 10-12 l/min for 15-20 min –Sumatriptan injection Transitional treatment (days-weeks) –Steroids (GON blockade or tablets) Prophylactic treatment –Verapamil R 200 mg x 2 (240-960 mg) NB EKG –Lithium

9. Sphenopalatine ganglion stimulation

10. Pain relief and freedom following initiation of full, sub-perception, and sham SPG stimulation (F, SP, and S, respectively). Schoenen J et al. Cephalalgia 2013 Sumatriptan injection 74% Sumatriptan næsespray 46% Oxygen 78%

11. Picturing the torment of cluster headache Slattery & Schott, Neurology 2015