Brain, Vol 120, Issue 1 193-209, Copyright © 1997 by Oxford University Press
PJ Goadsby and RB Lipton
The short-lasting primary headache syndromes may be conveniently divided
into those exhibiting marked autonomic activation and those without
autonomic activation. The former group comprise chronic and episodic
paroxysmal hemicrania, short-lasting unilateral neuralgiform headache with
conjunctival injection and tearing (SUNCT syndrome) and cluster headache.
These headache syndromes are compared with other short-lasting headache
disorders, such as hypnic headache, and persistent headache with milder
autonomic features such as hemicrania continua. Cluster headache is
included with the shorter-lasting headaches to attempt a nosological
analysis of these syndromes. The paroxysmal hemicranias are characterized
by frequent short-lasting attacks of unilateral pain usually in the
orbital, supraorbital or temporal region that typically last minutes. The
attack frequency usually ranges from 5 to 40 attacks per day. The pain is
severe and associated with autonomic symptoms such as conjunctival
injection, lacrimation, nasal congestion, rhinorrhoea, ptosis or eyelid
oedema. Almost all reported cases respond to treatment with indomethacin,
but respond poorly to other treatments including other nonsteroidal anti-
inflammatory drugs. A recent case study demonstrated the release of both
trigeminal and parasympathetic neuropeptides during a bout of pain in the
same pattern previously described in cluster headache. The SUNCT syndrome
is a distinctive rare condition characterized by less severe pain but
marked autonomic activation during attacks. Consistent with previous
reports, the present case of SUNCT syndrome was intractable to therapy. The
similarites of these syndromes suggests a considerable shared
pathophysiology. It is suggested that the syndromes are sufficiently well
established for inclusion in the International Headache Society
Classification system and that trigeminal-autonomic cephalalgias should be
classified as a group together. A proposed re- classification is presented.
ARTICLES
A review of paroxysmal hemicranias, SUNCT syndrome and other short- lasting headaches with autonomic feature, including new cases
Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, UK.
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