Headache and/or pain in the face and/or neck caused by dissection of a cervical carotid or vertebral artery. The pain is usually ipsilateral to the dissected vessel and generally has a sudden (even thunderclap) onset. It can remain isolated or be a warning symptom preceding ischaemic stroke.
A. Any new headache and/or facial or neck pain fulfilling criterion C
B. Cervical carotid or vertebral dissection has been diagnosed
C. Evidence of causation demonstrated by at least two of the following:
1. pain has developed in close temporal relation to other local signs of cervical artery dissection, or has led to the diagnosis of cervical artery dissection
2. either or both of the following:
a) pain has significantly worsened in parallel with other signs of the cervical artery lesion
b) pain has significantly improved or resolved within 1 month of its onset
3. either or both of the following:
a) pain is severe and continuous for days or longer
b) pain precedes signs of acute retinal and/or cerebral ischaemia
4. pain is unilateral and ipsilateral to the affected cervical artery
D. Not better accounted for by another ICHD-3 diagnosis.
Headache with or without neck pain can be the only manifestation of cervical artery dissection. It is by far the most frequent symptom (55-100% of cases), and the most frequent inaugural symptom (33-86% of cases), of this disorder.
6.5.1 Headache or facial or neck pain attributed to cervical arterial dissection is usually unilateral (ipsilateral to the dissected artery), severe and persistent (for a mean of 4 days). However, it has no constant specific pattern and it can sometimes be very misleading, mimicking other headaches such as 1. Migraine, 3.1 Cluster headache or 4.4 Primary thunderclap headache. Associated signs (of cerebral or retinal ischaemia and local signs) are common: a painful Horner’s syndrome, painful tinnitus of sudden onset or painful XIIth nerve palsy are highly suggestive of carotid artery dissection.
Cervical artery dissection may be associated with intracranial artery dissection, which is a potential cause of subarachnoid haemorrhage. 6.7.4 Headache attributed to intracranial arterial dissection may be present in addition to 6.5.1 Headache or facial or neck pain attributed to cervical arterial dissection.
6.5.1 Headache or facial or neck pain attributed to cervical arterial dissection usually precedes the onset of ischaemic signs, and therefore requires early diagnosis and treatment. Diagnosis is based on cervical MRI with fat suppression, Duplex scanning, MRA and/or CTA and, in doubtful cases, conventional angiography. Several of these investigations are commonly needed since any of them can be normal. There have been no randomized trials of treatment, but there is a consensus in favour of heparin followed by warfarin for 3-6 months according to the quality of the arterial recovery.