Internal carotid artery dissection presenting as cluster headache

Dissecção da artéria carótida interna apresentando-se como cefaléia em salvas

CLINICAL/SCIENTIFIC NOTE

Internal carotid artery dissection presenting as cluster headache

Dissecção da artéria carótida interna apresentando-se como cefaléia em salvas

Clecio Godeiro-Junior^{I, II}; Gustavo W. Kuster^{I, II}; Andre C. Felício^I; Pedro P. Porto Jr^II; Alexandre Pieri^{I, II}; Fernando M.S. Coelho^{I, II}

^IDepartment of Neurology and Neurosurgery, Federal University of São Paulo, São Paulo SP, Brazil

^IICritical Care Unit, Hospital Israelita Albert Einstein, São Paulo SP, Brazil

Cluster headache (CH) is a clinical entity characterized by strictly unilateral head pain attacks accompanied by ipsilateral autonomic phenomena. The attacks are severe, short-lasting (15–180 minutes), and may occur several times a day¹. Symptomatic CH cases have been described in association with different kind of lesions located in the middle fossa, near the sellar or parasellar structures although lesions of the internal carotid artery (ICA) were also described: aneurysma of ICA and internal carotid artery dissection (ICAD)².

We report a patient with symptomatic CH secondary to ICAD, and complete remission of his symptoms with usual treatment for CH. We obtained informed constent from the patient for publication.

CASE

A 53-year-old white man presented to the emergency room of Hospital Israelita Albert Einstein with 10-day history of recurrent right peri-orbital headache. Headaches were sudden, severe, pulsating, of thirty minutes duration and occurred once or twice a day. Headache was accompanied by ipsilateral ptosis, conjunctival injection, lacrimation and nasal stuffiness. He was on treatment for mild hypertension with hidroclorotiazide 25 mg once a day. Examination during headache revealed right Horner's syndrome with partial ptosis and miosis, accompanied by ipsilateral lacrimation, eyelid edema and conjunctival injection. After this episode, he remained with mild right ptosis. The remaining cranial nerves and neurological examination were normal.

The above features satisfied criteria "A to D" for CH of the International Classification of Headache Disorders (number, site and duration, accompanying symptoms, frequency of headaches)¹. Criterion E stipulates that other causes for the headache must be ruled out. We therefore performed further investigations. Computed tomographic imaging of the brain was normal. Fat supression T1-weighted magnetic resonance imaging of the carotid canal level displayed high signal compatible with blood products within the wall of the right internal carotid what was sugestive of arterial dissection (Figure).

He was admitted in our critical care unit and complementary investigation was performed. Blood cell count, erythrocyte sedimentation rate, coagulogram, protein C and protein S, lipidogram were normal. Results from a heart survey, including Holter's monitor and echocardiography, were normal.

The patient was anti-coagulated. Verapamil and prednisone were initiated as prophylactic therapy for cluster headache in our patient. There was complete resolution of headaches in one week and the patient was entirely asymptomatic at follow-up six months later.

DISCUSSION

Clinical signs of ICAD are often dissociated with initial pain and local signs followed by retinal or cerebral ischaemic events³. Headache is the most common symptom preceding stroke in patients with ICAD⁴. Frontal and parietal localizations were significantly associated with internal carotid artery dissection⁵. Pain in ICAD is postulated to be caused by stimulation of trigemino vascular system and it can mimic different primary headaches, including CH⁶. In a series of 65 ICAD patients, pain was the presenting symptom in 58.5%, typically unilateral and localized, a painful Horner's syndrome was the initial sign in 29%, and in two cases, pain mimicked CH⁶. Similar cases have been reported: in all Horner's syndrome persisted in pain free intervals, autonomic parasympathetic signs variably included lacrimation, conjunctival injection and nasal congestion, duration and character of pain was less stereotyped than in CH².

Examination during both primary cluster headache and ICAD may demonstrate a Horner's syndrome. Persistent ptosis and miosis between headaches are widely accepted as features of primary cluster headache but data regarding carotid vasculature in such circumstances is lacking⁷. In routine practice, individuals with cluster headache have diagnosis made upon clinical grounds with structural neuroimaging requested in a substantial proportion. Few patients have carotid imaging. Identification of ICAD is important as it has substantial risk of stroke (55%) with alternate management and prognosis³. It is postulated that certain features may help to distinguish ICAD from cluster headache. Headache duration more than 3 hours, lack of diurnal periodicity and absence of precipitation by alcohol intake should raise suspicion of ICAD. Atypical pain, localisation of pain over face or eye, and accompanying neck pain suggest ICAD rather than cluster headache. Lack of anhidrosis accompanying Horner's syndrome during a headache has been postulated to suggest ICAD (as fibres pass along the external carotid plexus)⁸.

In conclusion, individuals presenting with new onset of a cluster headache disorder may have an underlying internal carotid artery dissection. Once clinical features may not be helpful in distinguishing ICAD from primary cluster headache, and further investigation must be performed, ideally an carotid angiogram. We also belive that in patients with a previous history of CH, but with atypical symptoms in the presenting attack, complementary investigation must also be performed.

Received 14 May 2008, received in final form 25 July 2008. Accepted 5 August 2008.

Dr. Clecio Godeiro-Junior – Rua Dr. Diogo de Faria 650 / 33 - 04037-002 São Paulo SP - Brasil. E-mail: [e-mail]

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Publication Dates

• Publication in this collection
  15 Oct 2008
• Date of issue
  2008