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Carotid artery dissection is a frequent non-atherosclerotic cause of stroke in young adults. In up to 20% of cases it is bilateral or associated with vertebral artery dissection.1 Common clinical features include unilateral pain on the side of the dissection, signs of cerebral ischaemia, ear bruits, and Horner's syndrome,2 which is usually incomplete and sometimes transient.3 Horner's syndrome is thought to result from a lesion of the periarterial sympathetic plexus caused by the dissection.2 Rhinorrhoea and nasal congestion have not yet been reported as symptoms of spontaneous carotid artery dissection but have been seen in patients undergoing cranial base surgery.4 We here describe a patient with spontaneous bilateral carotid artery dissection in whom rhinorrhoea, nasal congestion, and headache were the first symptoms, which preceded the development of incomplete Horner's syndrome for several weeks.
A previously healthy 33 year old man was referred for evaluation of headache. He admitted to having had rhinorrhoea and nasal stuffiness for 7 weeks. Treatment with antibiotics and corticosteroids before admission had been unsuccessful. An infectious or allergic cause could not be determined. Four weeks later he developed severe periodical retro-orbital headache lasting for hours, and pulsatile tinnitus on the right side. Two weeks before admission he noted severe stabbing retro-orbital pain on the left side irradiating to the neck. This did not respond to aspirin. Pain attacks and vomiting occurred regularly during the afternoon, progressing during the night without fluctuations. Intake of alcohol and nicotine triggered the attacks. He had no history of cluster headache or migraine.
On examination he showed rhinorrhoea, increased lacrimation of the left eye without redness, left eyelid swelling, incomplete Horner's syndrome on the left side, and ear bruits on the right side that were not detected by auscultation of the skull. There were no other focal neurological deficits, in particular no anhydrosis. Routine laboratory blood tests, screening for cardiovascular diseases, and Doppler sonography were all normal. Cranial magnetic resonance angiography and cerebral digital subtraction angiography showed bilateral dissection of the internal carotid artery with dissecting aneurysms on both sides (figure A-D).
Anticoagulation with heparin and then dicumarole was initiated and the patient's symptoms gradually improved. On follow up examination 6 months and 14 months later, he showed marginal ptosis and a slightly smaller pupil (2 mm) in the dark on the left side, but no headaches, rhinorrhoea, or tinnitus. Cerebral magnetic resonance angiography of the left internal carotid artery was now normal but the dissecting aneurysm of the right internal carotid artery was unchanged. Therefore anticoagulation was continued.
We suggest that in our patient bilateral carotid artery dissection led to a lesion and dysfunction of the sympathetic pericarotid plexus leading to a parasympathomimetic state with nasal hypersecretion and congestion of nasal vessels. This condition was reversible after recanalisation of the left internal carotid artery. Nasal stuffiness or rhinorrhea may be rarely reported by patients because this phenomenon may be associated only with sympathetic pericarotid lesions after infrequent bilateral internal carotid artery dissections whereas unilateral dissections may cause only a mild and clinically often inapparent irritation.1
Cusimano and Sekhar described a syndrome they termed “pseudocerebrospinal fluid rhinorrhoea” with ipsilateral nasal hypersecretion and nasal stuffiness after surgery of the cranial base.4 In these patients, the pericarotid sympathetic plexus, the petrous or cavernous carotid artery parts, and the greater petrosal nerve had been removed or dissected. Experimental selective parasympathetic nerve activation in the nasal mucosa of the cat leads to an increase in nasal secretion and vascular congestion.5 Lung found nasal congestion to be related to a withdrawal of sympathetic discharge rather than to an overactivity of the parasympathetic nerves.6 Thus the nasal hypersecretion and stuffiness in our patient is in agreement with the assumption of a lesion of pericarotid sympathetic nerve fibres after carotid artery dissection.
In the differential diagnosis, cluster headache and paroxysmal hemicrania have to be considered. Our patient's symptoms differed from typical cluster headache and paroxysmal hemicrania in that headache followed the beginning of autonomous symptoms after several days and pain was progressing over hours during bouts without fluctuations.7
We conclude that structural lesions of sympathetic nerve fibres should be considered when (1) the headache profile is not typical for cluster headache and paroxysmal hemicrania, and (2) autonomous symptoms precede and outlast headache.
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