Intended for healthcare professionals

Rapid response to:

Clinical Review

Cluster headache

BMJ 2012; 344 doi: (Published 11 April 2012) Cite this as: BMJ 2012;344:e2407

Rapid Response:

Re: Cluster headache

Dear Editor,

We read with interest the recent article by Dr. Nesbitt and Prof. Goadsby on cluster headaches, however as Ophthalmologists, we would highlight a number of treatable ophthalmic conditions missing from the listed differential diagnoses.

Several ocular conditions can mimic the presentation of cluster headache, and present with recurrent pain, lacrimation, conjunctival injection, periorbital oedema, ptosis and/or pupillary abnormalities. The disorders that best match these features are scleritis, uveitis, orbital inflammatory disease (including dacryoadenitis and sino-orbital disease), intermittent angle closure glaucoma and blepharochalasis.

Scleritis presents with a severe pain, which can radiate to other parts of the head and often wakes the person at night. Recurrent episodes are common and in its more severe forms, early recognition and aggressive management of the underlying condition will not only reduce ocular damage but may also save the patient’s life.[1] Uveitis can also present in a similar manner.

Patients with orbital inflammatory disease usually present with acutely painful periorbital swelling, but may also describe subactute (over weeks) or more insidious symptoms over a period of months.[2] Orbital imaging is helpful in the diagnosis, and CT will often demonstrate extraocular muscle thickening, with occasional involvement of the sclera, orbital fat or lacrimal gland.[3] CT would also be a helpful adjunct in excluding sinus or sino-orbital disease as a cause of cluster headache-type symptoms, especially when nasal congestion or rhinorrhoea are present. Dacryoadenitis can present in a similar fashion, although the pain, redness and swelling are typically located over the outer third of the upper eyelid, and lacrimation is more commonly associated. There may be a palpable tender mass in this region (the enlarged lacrimal gland).

A severely painful eye with circumcorneal injection and pupillary abnormalities might also be caused by intermittent angle closure glaucoma. Vision is often reduced when this occurs with a third of patients also describing halos around bright lights.[4]

Finally, blepharochalasis is an intermittent swelling of (usually) the upper eyelid, due to recurrent inflammation. The average attack lasts up to 48 hours, although bouts are more commonly painless.[5]

In summary, we would recommend that an ophthalmological assessment be considered before reaching the diagnosis of cluster headache. History and appearance alone (such as the gentleman in Figure 1) may be insufficient without specialist ophthalmic input.


1. Pavesio CE, Meier FM. Systemic disorders associated with episcleritis and scleritis. Current Opinion in Ophthalmology, 2001;12:471-8.
2. Ahn Yuen SJ, Rubin PAD. Idiopathic Orbital Inflammation: Distribution, Clinical Features, and Treatment Outcome. Arch Ophthalmol. 2003;121:491-499.
3. Voyatzis G, Chandrasekharan L, Francis I, Malhotra R. The Importance of Clinicians Reviewing CT Scans in Suspected Lacrimal Gland Disease Causing Eyelid Swelling, Even if Radiologists Previously Interpreted them as Normal. Open Ophthalmol J. 2009;3:26–28.
4. Shindler KS, Sankar PS, Volpe NJ, Piltz-Seymour JR. Intermittent headaches as the presenting sign of subacute angle-closure glaucoma. Neurology, 2005;65:757-8.
5. Koursh DM, Modjtahedi SP, Selva D, Leibovitch I. The blepharochalasis syndrome. Survey of Ophthalmology, 2009;54:235-44.

Competing interests: No competing interests

25 April 2012
Andre S Litwin
Raman Malhotra
Queen Victoria Hospital NHS Trust
East Grinstead