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Table of Contents    
Year : 2014  |  Volume : 62  |  Issue : 6  |  Page : 681-683

Cluster-like headache as presenting feature of Vogt-Koyanagi-Harada disease

1 Department of Neuro Ophthalmology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Submission16-Oct-2014
Date of Decision20-Oct-2014
Date of Acceptance24-Oct-2014
Date of Web Publication16-Jan-2015

Correspondence Address:
Vimal K Paliwal
Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.149407

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How to cite this article:
Kanaujia V, Paliwal VK, Aneez A, Agarwal R. Cluster-like headache as presenting feature of Vogt-Koyanagi-Harada disease. Neurol India 2014;62:681-3

How to cite this URL:
Kanaujia V, Paliwal VK, Aneez A, Agarwal R. Cluster-like headache as presenting feature of Vogt-Koyanagi-Harada disease. Neurol India [serial online] 2014 [cited 2021 Mar 9];62:681-3. Available from:


A 26-year-old lady presented with acute, severe, right periorbital/frontal headache for 1 week. She had three to four headache episodes/day, each lasted an hour and usually clustered around night, sometimes waking her from sleep. Headache was piercing-type associated with intense lacrimation/redness of right eye. Three days later, she complained of transient blurring of vision from right eye which improved after subsidence of headache. There was no double vision, painful eye movements, or past/family history of migraine. General/neurological examination was normal. Fundus examination revealed normal optic disc. A trial of oxygen inhalation (8l/min) promptly relieved her headache. Indomethacin failed to reduce headache frequency. During the hospital course, she developed mild continuous headache overlying cluster-like attacks and blurred vision both eyes. Ophthalmic evaluation revealed vision 6/18 both eyes and multiple areas of exudative retinal detachments that were confirmed on fluoresce in angiography (FA) and optical coherence tomography (OCT) [Figure 1]. Cerebrospinal fluid (CSF) examination, cranial magnetic resonance imaging (MRI) and immunological work-up were normal. With diagnosis of Vogt-Koyanagi-Harada disease (VKH), she was prescribed oral prednisolone (1 mg/kg). Within 1 week of initiation of steroids, her headache disappeared. At 1 month, her vision improved to 6/6 andretinal exudative detachments showed near-complete recovery.
Figure 1: (a) Extensive exudative detachments around disc (arrows) with adjoining serous detachments (arrowheads) of neurosensory retina, erythematous optic disc, and chorioretinal folds (b) Flourescein angiography shows pinpoint hyperfluorescent areas (arrowheads) filling the retinal detachments (arrows) with dye (c) Detachments confirmed on OCT. OCT = Optical coherence tomography

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VKH disease is a multisystem disease of presumed autoimmune etiology that commonly involves eye in the form of anterior uveitis, vitritis, optic nerve edema/hyperemia, and subretinal exudates with multiple retinal detachments. VKH also produce recurrent aseptic meningitis, encephalitis, deafness, blindness, and cutaneous manifestations. VKH evolves in phases starting from prodromal phase, uveitis, convalescent, and recurrent phase. Prodromal phase is characterized by headache, fever, and other nonspecific systemic features and may include meningeal/cranial neuropathy signs. Three to five days later, bilateral uveitis occurs with characteristic findings as seen in our patient. Convalescent phase is characterized by disappearance of subretinal exudates, retinal detachments, but depigmentation of choroid/retinal epithelium ensues. Most ocular/neurological complications occur during recurrent phase. A revised diagnostic criterion for VKH was proposed by international committee on nomenclature that classifies VKH into complete, incomplete, and probable VKH [Table 1]. [1] Owing to characteristic clinical picture and exclusion of other causes like sympathetic ophthalmia, sarcoidosis, Lyme disease, tuberculosis, and lymphoma and due to absence of neurological/cutaneous manifestation (more commonly during recurrent phase); our patient was classified into probable VKH.
Table 1: Diagnostic criteria for Vogt-Koyanagi-Harada disease and cluster headache

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VKH may present with headache before the appearance of ophthalmic complaints, but presentation with cluster-like headache is not known. [2] Cluster headache is severe/very severe unilateral supraorbital frontal headaches with characteristic ipsilateral autonomic features (lacrimation, conjunctival injection, ptosis, etc.). Our patient fulfills the international classification of headache disorder (3 rd edition) criteria for diagnosis of cluster headache. [3] The term 'cluster' refers to a specific feature, whereby in majority with episodic primary cluster headache, attacks occur during specific months every year (circannual rhythm). Cluster headache is a primary headache; however, many secondary causes like pituitary tumors, other intracranial space-occupying lesions, infections, arterial/venous infarcts, etc., are known to produce cluster headache. Many ophthalmic conditions like Tolosa-Hunt syndrome, orbital exenteration, and orbital myositis can produce cluster-like headache. [4],[5],[6] Pathogenesis of cluster headache in orbital conditions is not known. Pain-sensitive structures in orbit are supplied by ophthalmic branch of trigeminal nerve which when stimulated by appropriate triggers, produces pain and autonomic features via trigeminal-autonomic reflex (through connections of trigeminal nucleus to superior salivatory nucleus). These secondary cluster headaches are usually isolated bouts as they lack circannual rhythmicity of pain attacks which is thought to be due activation of hypothalamus. Most secondary cluster headaches have certain atypical features like blurring of vision, background mild continuous headache that may favor the need to search for secondary causes of cluster headache.

  References Top

Read RW, Holland GN, Rao NA, Tabbara KF, Ohno S, Arellanes-Garcia L, et al. Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: Report of an international committee on nomenclature. Am J Ophthalmol 2001;131:647-52.  Back to cited text no. 1
Tavsanli M, Uluduz D, Saip S, Kendiroglu G. Vogt-Koyanagi-Harada disease: Headache as an initial manifestation. J Headache Pain 2008;9:255-6.  Back to cited text no. 2
Headache classification committee of the International Headache Society (IHS). The international classification of headache disorders, 3 rd edition (beta version). Cephalalgia 2013;33:629-808.  Back to cited text no. 3
Lee MS, Lesell S. Orbital myositis posing as cluster headache. Arch Neurol 2002;59:635-6.  Back to cited text no. 4
Harley JS, Ahmad F. Cluster-like headache heralding inflammatory orbital pseudotumour. Cephalalgia 2008;28:401-2.  Back to cited text no. 5
Evers S, Sörös P, Brilla R, Gerding H, Husstedt IW. Cluster headache after orbital exenteration. Cephalalgia 1997;17:680-2.  Back to cited text no. 6


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