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Year : 2015  |  Volume : 8  |  Issue : 6  |  Page : 810-812  

A rare cause of symptomatic cluster headache

Department of Neurology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India

Date of Web Publication19-Nov-2015

Correspondence Address:
Venkatesan Prasanna Eswaradass
3, Vijayarahavachari Road, Gandhi Road, Salem - 636 007, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-2870.169943

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Cluster headache (CH) is characterized by recurrent attacks of short-lasting excruciating pain accompanied by signs of autonomic dysfunction. Although neuroimaging results are usually normal in primary headaches, rarely structural lesions may be associated with CH like presentation. Most symptomatic CH is due to pituitary tumors and less commonly due to arteriovenous malformations (AVM) and aneurysms. Here we describe a case of 44-year-old male who presented to us with new onset episodic CH with headache features typical. Initially, headache responded to treatment, but later the headache became continuous, severe in intensity and refractory to treatment. Since magnetic resonance imaging was normal, he was subjected to digital substraction angiography (DSA) to rule out aneurysm or AVM. DSA revealed indirect carotid-cavernous fistula (CCF). Once the fistula was obliterated with onyx embolization, headache subsided completely. MRI brain is often routinely performed in CH to rule out secondary causes. When MRI brain is normal, detailed vascular imaging with DSA must be performed in patients with CH with especially in those with atypical features. Like continuous pain refractory to treatment, sudden increase in severity in the first episode, minimal or absent autonomic features and abnormal physical findings like persistent ptosis in the headache-free period. Although many cases of symptomatic CH have been reported in literature we report the first case of CCF presenting as CH. Early identification is essential as it is completely curable with endovascular treatment.

Keywords: Cluster headache, indirect carotid-cavernous fistula, trigeminal autonomic cephalgia

How to cite this article:
Eswaradass VP, Gnanashanmugham G, Pranesh MB, Parimalam N. A rare cause of symptomatic cluster headache. Med J DY Patil Univ 2015;8:810-2

How to cite this URL:
Eswaradass VP, Gnanashanmugham G, Pranesh MB, Parimalam N. A rare cause of symptomatic cluster headache. Med J DY Patil Univ [serial online] 2015 [cited 2023 Dec 5];8:810-2. Available from:

  Introduction Top

Cluster headache (CH) is a primary headache disease like migraine. In primary headaches, pain is due to spontaneous activation of nociceptive pathways. CH is characterized by recurrent attacks of short-lasting excruciating pain accompanied by signs of autonomic dysfunction as described in the second version of the International Classification Headaches second edition (ICHD-II) criteria. [1] An underlying cause has sometimes been found in a patient with typical CH features which include pituitary tumors, meningiomas, metastases, orbital infection, orbital tumors, arteriovenous malformations (AVM) and aneurysms. Here we describe a rare, treatable case of symptomatic CH due to indirect carotid-cavernous fistula (CCF) which completely improved following onyx embolization.

  Case Report Top

A 44-year-old male a known hypertensive on treatment came with episodic headache of 1-month duration. Pain was exclusively localized to the right periorbital region never on the left side. Pain was severe, pulsatile about 2-3 episodes/day. With each episode, pain peaked in 15 min, lasted with maximum severity for 2 h and then gradually abated. It woke him up from sleep few times. Pain was associated with narrowing of right palpebral fissure during peak of a headache with mild lacrimation and few episodes of vomiting but not associated with redness, nasal congestion, photophobia, phonophobia, tinnitus or periorbital swelling. During the episode, patient was restless and agitated.

On examination, he was distressed, afebrile and had right partial sided ptosis. Visual acuity, field of vision, color vision and fundi were normal. Pupils were 3 mm symmetrical and reacted to light. Extraocular movements were full. Other central nervous system examination was normal. His routine blood investigations including erythrocyte sedimentation rate and computed tomography brain with contrast were normal. Patient was clinically diagnosed as CH - first episode. Initially, there was some relief with analgesics and 100% oxygen but later headache became continuous, persistent and refractory to treatment.

Due to persistent headache, we did a magnetic resonance imaging (MRI) with magnetic resonance angiogram to rule out secondary cause. As it was normal, we decided to do digital substraction angiography (DSA) to rule out aneurysm or AVM. DSA revealed an indirect (type III) right CCF supplied by meningeal branch of right internal maxillary artery ([Figure 1]). Fistula was treated by endovascular embolization by with Onyx ([Figure 2]). Patient's headache relieved immediately and completely after the procedure and ptosis recovered in the next day. Patient's headache and ptosis completely subsided following procedure, and he is on regular follow-up since then.
Figure 1: (a) Venous phase showing dilated superior ophthalmic vein. (b) Digital substraction angiography right common carotid artery (CCA) injecion showing fistula

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Figure 2: (a) Onyx cast in fi stula. (b) Postembolization right common carotid artery (CCA) injection — showing no fi lling of fi stula

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  Discussion Top

Trigeminal autonomic cephalalgias (TACs) are primary headache syndromes characterized by severe short-lasting headaches accompanied by paroxysmal facial autonomic symptoms. [2] The group includes CH, paroxysmal hemicranias, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing. By far, CH is the most frequent syndrome and is characterized by attacks of intense periorbital pain accompanied by ipsilateral autonomic symptoms. [3] These attacks may come clustered in periods of several weeks or months, alternating with attack-free periods of months to years (episodic CH) or without such attack-free periods (chronic CH). The headache is accompanied by one ipsilateral autonomic symptom among the following: conjunctival injection and lacrimation, nasal congestion, forehead sweating, miosis and ptosis. In the absence of autonomic signs, CH can be diagnosed if a sense of restlessness is present during the attacks. The pathophysiologic mechanism of TACs is largely unknown; hypothalamic and trigeminovascular mechanisms have been implicated. [4]

Although neuroimaging results are usually normal in TACs as they are primary headaches, rarely it may be associated with structural lesions. Favier et al. analyzed 31 previously published cases of clinical TACs and TAC like syndromes who were found to have structural lesions on neuro imaging. [5] Abnormal attack duration, absence of autonomic symptoms, continuous headache, sudden change in headache or abnormal physical examination would give a clue to underlying structural abnormalities. In addition, onset of first episode at fifth decade would suggest a secondary TAC. Of 31 cases 11 patients had a pituitary tumor, 4 had AVM, 4 had aneurysm, and 10 had SOL like the meningioma, pilocytic astrocytoma, aspergilloma, mucocele, nasoparngeal carcinoma and so forth. There was 1 case of subclavian steal syndrome and another metallic foreign body in the maxillary sinus. [5]

In our case, patient initially presented with classical features of CH fulfilling the ICHD-II criteria. However since the onset of first episode of cluster was in the fourth decade and patient later developed continuous pain refractory to standard treatment, we suspected secondary cause. When MRI was normal, we went ahead with DSA to rule out vascular malformations. Clinically, we did not suspect CCF as there was no exophthalmos, chemosis or pulsatile tinnitus. Kuromoto et al. reported one case of cavernous sinus (CS)-dural arteriovenous fistula (dAVF) with CH-like ophthalmalgia without chemosis and exophthalmos that was successfully treated by transvenous embolization. [6] CCF generally causes periorbital aching with ocular symptoms due to high venous pressure in the CS, while TAC like pain is caused by arterial dilatation-stimulating trigeminal nerves around the vessels. [7] Although there are many causes for symptomatic CH the most common is pituitary tumor and indirect CCF is a very rare cause. To the best of our knowledge, this is the first case of CCF presenting as only CH without ophthalmoplegia.

A CCF is an abnormal communication between the CS and the carotid arterial system. CCFs can be classified by etiology (traumatic vs. spontaneous), velocity of blood flow (high vs. low flow), and anatomy (direct vs. dural). [8] Some fistulas Direct CCFs are characterized by a direct connection between the cavernous segment of the internal carotid artery (ICA) and the CS. These fistulas usually are of the high flow type. Most and often caused by a single, traumatic tear in the arterial wall, they are called a direct CCF. [9] The indirect or dural fistula may be due to congenital AVM that develop spontaneously, often in the setting of atherosclerosis, systemic hypertension or connective tissue disease. Dural CCF consists of a communication between the CS and one or more meningeal branches of the ICA, external carotid artery or both. [8] These fistulas usually have low rates of arterial blood flow with spontaneous regression. Otherwise, conservative treatment by means of carotid or the jugular compression is recommended initially but in cases with rapidly deteriorating ocular symptoms urgent interventional therapy is necessary.

  Conclusion Top

Cluster headache is predominantly primary headaches but rarely symptomatic CH can occur. Most symptomatic CH is due to pituitary tumors and less commonly due to AVM and aneurysms. Though MRI imaging is often performed in TACs to rule out vascular anomalies, it can be easily missed in routine MR angiography. Though MR imaging is often performed in TACs to rule out secondary causes, vascular anomalies like dural AV fistulas and aneurysms can be easily missed in routine MR angiography. When potentially treatable vascular anomalies are missed patient may go undiagnosed, and disabling hemicranial pain may persist without responding to conventional therapies for CH. Hence, we suggest when MRI is normal; it is mandatory to perform detailed vascular imaging with DSA for all patients with TACs with atypical features.

  References Top

Headache Classification Subcommittee of the International Headache Society. The International classification of headache disorders: 2 nd edition. Cephalalgia 2004;24 Suppl 1:9-160.  Back to cited text no. 1
Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain 1997;120( Pt 1):193-209.  Back to cited text no. 2
Matharu MS, Goadsby PJ. Trigeminal autonomic cephalgias. J Neurol Neurosurg Psychiatry 2002;72 Suppl II:ii19-26.  Back to cited text no. 3
Goadsby PJ. Pathophysiology of cluster headache: A trigeminal autonomic cephalgia. Lancet Neurol 2002;1:251-7.  Back to cited text no. 4
Favier I, van Vliet JA, Roon KI, Witteveen RJ, Verschuuren JJ, Ferrari MD, et al. Trigeminal autonomic cephalgias due to structural lesions: A review of 31 cases. Arch Neurol 2007;64:25-31.  Back to cited text no. 5
Kuramoto Y, Adachi H, Sakai N, Ueno Y, Sakai C, Imamura H, et al. Cluster-like headache cause by cavernous sinus dural arteriovenous fistula, cured by transvenous embolization: A case report. No Shinkei Geka 2013;41:493-8.  Back to cited text no. 6
Yamada SM, Masahira N, Shimizu K. A migraine-like headache induced by carotid-cavernous fistula. Headache 2007;47:289-93.  Back to cited text no. 7
Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg 1985;62:248-56.  Back to cited text no. 8
Kwan E, Hieshima GB, Higashida RT, Halbach VV, Wolpert SM. Interventional neuroradiology in neuro-ophthalmology. J Clin Neuroophthalmol 1989;9:83-97.  Back to cited text no. 9


  [Figure 1], [Figure 2]

This article has been cited by
1 Secondary (Symptomatic) trigeminal autonomic cephalalgia
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[Pubmed] | [DOI]


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