Cervical artery dissection accounts for up to one fifth of ischemic strokes occurring before the age of 45 years.1 Although head and neck trauma and primary diseases of the arterial wall are predisposing factors, cervical artery dissection can occur spontaneously. Moreover, 28% to 66% of patients present with multiple dissections.2-4 The prognosis of cervical artery dissection is mainly determined by the occurrence and severity of ischemic stroke following the dissection. We describe a patient with an unusual presentation of multiple spontaneous cervical artery dissections.
We describe a patient with atypical headache as the only presenting symptom of spontaneous triple cervical artery dissection. As the patient suffered from arterial hypertension, a causative relation between headache and arterial hypertension was initially taken into consideration. However, four-vessel arteriography disclosed a dissection of both internal carotid arteries and the right vertebral artery.
This unique case highlights the value of conventional arteriography for diagnosing cervical artery dissection. Since multiple cervical artery dissections are not rare, all cervical arteries should be examined by means of conventional arteriography when a dissection is suspected.
A 53-year-old man was admitted to a general hospital. Three weeks before admission, the patient awoke with severe, bifrontally localized headache accompanied by phonophobia. Since onset, headaches were steady and not influenced by analgesics. The current symptomatology was not preceded by head or neck trauma. Medical history included diabetes mellitus type 2 and a transient ischemic attack 25 years ago. Except for arterial hypertension (170/100 mm Hg), the general physical examination was normal, including palpation of the superficial temporal arteries. Percussion of the sinuses was painless. Clinical neurological examination only revealed minimal anisocoria without ptosis (relative miosis of the right pupil). The anisocoria was considered to be physiological as, according to the patient, the pupil asymmetry was a premorbid manifestation.
As a causative relation between headaches and arterial hypertension was taken into consideration, treatment with antihypertensive drugs was started. Despite significant reduction of blood pressure values to normal, headaches persisted. Given the nonspecific clinical symptomatology, the differential diagnosis included sinusitis, subarachnoid hemorrhage, cervical artery dissection, temporal arteritis, and tension headache.
Laboratory studies demonstrated increased serum glucose and hemoglobin A1c values and mildly elevated liver enzyme values. The sedimentation rate was normal. A standard electroencephalogram was normal, and computed tomography of the brain showed an old lacunar infarction in the left thalamus. Magnetic resonance imaging of the brain did not reveal additional lesions. Four-vessel arteriography disclosed dissection of both internal carotid arteries and the right vertebral artery (Figure 1). All dissections were localized extracranially. No secondary embolisms were seen. A skin biopsy did not reveal any abnormalities suggestive of a vasculitis or a connective tissue disease. Therapy with acenocoumarol was started, and relative bed rest was prescribed. After 2 weeks, the patient was asymptomatic and was discharged. Six months later, four-vessel arteriography was repeated. As it showed aneurysmal dilatation of the extracranial segment of the left internal carotid artery, therapy with acenocoumarol was continued.
Most cases with cervical artery dissection present with unilateral headaches or neck pain followed by focal cerebral ischemic symptoms, retinal ischemia, or oculosympathetic palsy.5-7 However, pain as the only symptom of cervical artery dissection is rare and only sporadically reported.8-11 In a series of 44 patients with cervical artery dissection, only 2 presented with pain alone.12 Headache or neck pain typically is ipsilateral to the side of dissection. In patients with an internal carotid artery dissection, pain is limited to the anterior head in 60%, whereas headaches are distributed posteriorly in 83% of patients with a vertebral artery dissection. Headaches are mostly described as steady and have a mean duration of 3 days.13 Thus, the distribution and duration of headaches in our patient were exceptional.
Multiple cervical artery dissections have been described in 28% to 66% of reported cases.2-4 Multiple-vessel involvement can be, in part, asymptomatic at the time of diagnosis.4 However, a case with spontaneous triple cervical artery dissections with headache as the only presenting symptom has never been described.
In conclusion, atypical headaches can be the only presenting symptom of multiple, spontaneous cervical artery dissection. This case report also highlights the value of conventional arteriography for diagnosing cervical artery dissection. Since multiple dissections are not rare, all cervical arteries should be examined by means of conventional arteriography when cervical artery dissection is suspected.
Acknowledgments: Appreciation is expressed to Mrs. I. Bats (Born-Bunge Foundation). Dr. Engelborghs is a Research Assistant of the Fund for Scientific Research–Flanders (F.W.O.–Vlaanderen).