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Abstract
Cluster headache is a rare very severe disorder that is clinically well characterized
with a relatively poorly understood pathophysiology. In this study patients with episodic
cluster headache fulfilling the criteria of the International Headache Society were
examined during an acute spontaneous attack of headache to determine the local cranial
release of neuropeptides. Blood was sampled from the external jugular vein ipsilateral
to the pain before and after treatment of the attack. Samples were assayed for calcitonin
gene-related peptide (CGRP), vasoactive intestinal polypeptide (VIP), substance P
and neuropeptide Y. Attacks were treated with either oxygen inhalation, sumatriptan
or an opiate. Thirteen patients were studied of whom 10 were male and three female.
All had well-established typical attacks of cluster headache when blood was sampled.
During the attacks external jugular vein blood levels of CGRP and VIP were raised
while there was no change in neuropeptide Y or substance P. Calcitonin gene-related
peptide levels rose to 110 +/- 7 pmol/l (normal: < 40) while VIP levels rose to 20
+/- 3 pmol/l (normal: < 7). Treatment with both oxygen and subcutaneous sumatriptan
reduced the CGRP level to normal, while opiate administration did not alter the peptide
levels. These data demonstrate for the first time in vivo human evidence for activation
of the trigeminovascular system and the cranial parasympathetic nervous system in
an acute attack of cluster headache. Furthermore, it is shown that both oxygen and
sumatriptan abort the attacks and terminate activity in the trigeminovascular system.