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Orbital Myositis Posing as Cluster Headache
Michael S. Lee, MD;
Simmons Lessell, MD
Arch Neurol. 2002;59:635-636.
ABSTRACT
Objective To describe the case of a patient with recurrent orbital myositis who
was thought to have cluster headaches for 6 years.
Design and Setting Case report in an outpatient neuro-ophthalmology clinic.
Patient A 24-year-old man developed unilateral supraorbital pain, lacrimation,
conjunctival hyperemia, nasal congestion, proptosis, and painful eye movements.
The pain intensity varied over the course of each day and disappeared after
1 month. He had multiple attacks responsive to prednisone that were separated
by months over the ensuing 6 years. Neuroimaging revealed an enlarged extraocular
muscle.
Conclusions Overlap in symptoms between recurrent orbital myositis and cluster headache
delayed the diagnosis in this patient. Orbital myositis should be considered
in patients with atypical cluster headache characterized by proptosis, painful
eye movements, and pain that does not completely resolve after 3 hours.
INTRODUCTION
THE INTERNATIONAL Headache Society has established criteria for the
diagnosis of cluster headache (Figure 1).1 It predominantly affects men between 20 and 40 years
of age and centers in and around 1 eye. Episodes begin acutely, last 1 to
2 hours, and occur several times a day. They occur daily for an average of
2 weeks to 3 months (International Headache Society criteria are 1 week to
1 year). Associated findings include ipsilateral conjunctival hyperemia, lacrimation,
ptosis, miosis, nasal stuffiness, and facial sweating.2
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Figure 1. International Headache Society
criteria for cluster headaches.1
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Recurrences almost always occur on the same side, but 10% to 15% may
alternate. Remissions should last at least 2 weeks, but they often will last
months to years. About 10% of patients suffer chronic cluster headaches, with
"clusters" occurring for more than 1 year without remission or with remissions
lasting less than 2 weeks.1
Orbital myositis is a subgroup of idiopathic orbital inflammatory syndrome.
It is the second most common cause of extraocular muscle enlargement, after
thyroid-associated ophthalmopathy. Women are affected twice as often as men,
and the mean age of onset is approximately 30 years. Mombaerts and Koornneef3 followed up 16 patients with myositis for a mean of
9.7 years and found 56% with recurrence. Clinical findings include painful
eye movements, lid edema, conjunctival hyperemia and chemosis, poor extraocular
motility, and proptosis.
We present the case of a patient with recurrent orbital myositis, which
was mistakenly diagnosed and treated as cluster headache for 6 years.
REPORT OF A CASE
In 1994, a 24-year-old man developed paroxysmal headache in the right
supraorbital region associated with swelling of his right eyelid and eye.
The headache was severe, pounding, and not preceded by an aura. The patient
also noted blurring of the vision in his right eye and painful eye movements.
The headache fluctuated in intensity over the course of the day and lasted
approximately one month. Recurrences were separated by 2 months and precipitated
by alcohol use. He denied having sinus disease. Apart from 2 uncles with migraine,
his family history was noncontributory.
Results of ophthalmologic evaluation for proptosis between episodes
were reportedly normal. A computed tomographic scan of the brain and orbits
during an attack demonstrated enlargement of the right medial rectus muscle.
Medical history was unremarkable. Treatment with over-the-counter nonsteroidal
anti-inflammatory medicine and acetaminophen/butalbital/caffeine gave only
minor relief.
In 1996, neurologic consultation revealed tenderness to palpation of
the right eye with fullness of the right upper lid, conjunctival hyperemia,
lacrimation, and nasal stuffiness. The results of a pupil examination were
normal, and there was no optic nerve edema. The neurologic examination was
otherwise unremarkable. Episodic cluster headache was diagnosed, and the patient
was treated with prednisone. The results of thyroid function tests and the
erythrocyte sedimentation rate were normal.
The headaches recurred in the spring and fall of each year for the next
5 years, each time on the right side with a good response to prednisone. In
August 2000, the patient developed left retro-orbital headache with blurred
vision and lacrimation of the left eye associated with proptosis. This recurred
on the left side 4 times in 6 months with increasing severity. Examination
revealed mild proptosis and ptosis of the left eye with conjunctival hyperemia
and lacrimation. The rest of the examination findings remained unchanged.
The patient was treated with verapamil hydrochloride to prevent attacks, and
acute episodes responded well to prednisone. Magnetic resonance imaging and
angiography of the brain with contrast revealed an enlarged left superior
rectus muscle (Figure 2). The results
of repeated thyroid function tests were normal.
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Figure 2. Postgadolinium coronal magnetic
resonance imaging demonstrating a thickened and enhancing left superior rectus
muscle (arrow) when compared with the right.
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COMMENT
Cluster headache, a disorder of unknown cause and pathogenesis, can
be mimicked by other disorders. Instances in which vertebral artery aneurysm,4 cavernous sinus pseudoaneurysm,5
and arteriovenous malformations6 were confused
with cluster headache have been reported. It is easy to see why, as in our
case, inflammatory orbital pseudotumor could be confused with cluster headache.
The patient was a young man with recurrent, unilateral, high-intensity pain
accompanied by eyelid edema, lacrimation, ocular hyperemia, and nasal stuffiness
sometimes precipitated by the ingestion of alcohol. As in some cases of cluster
headache, the disorder was responsive to corticosteroids.7
The correct diagnosis is established when imaging of the orbit demonstrates
enlargement of an extraocular muscle. It is important that orbital pseudotumor
be diagnosed expeditiously because prompt treatment with oral corticosteroids
is apt to provide quick relief and prevent reduced ocular motility.7 Furthermore, an inflamed orbit may be a manifestation
of orbital cellulitis or a systemic disorder such as Wegener granulomatosis.8 In light of this, it behooves neurologists to consider
obtaining either computed tomographic scans or magnetic resonance images of
the orbits in cases of cluster headache in which eyelid edema, ptosis, and
conjunctival hyperemia are prominent features or in which the duration of
pain is prolonged, as with our patient.
AUTHOR INFORMATION
Accepted for publication October 19, 2001.
Author contributions: Study concept and design (Drs Lee and Lessell); acquisition of data (Drs Lee and Lessell); analysis and interpretation of data (Drs Lee and Lessell); drafting of the manuscript (Dr Lee); critical revision of the manuscript for important intellectual
content (Dr Lessell); administrative, technical,
and material support (Dr Lee); study supervision (Dr Lessell).
This study was supported by a grant from the Heed Foundation, Cleveland,
Ohio (Dr Lee).
Corresponding author: Michael S. Lee, MD, Massachusetts Eye and Ear
Infirmary, Department of Neuro-ophthalmology, 243 Charles St, Boston, MA 02114
(e-mail: michael_lee{at}meei.harvard.edu).
From the Department of Neuro-ophthalmology, Massachusetts Eye and Ear
Infirmary, Harvard University Medical School, Boston.
REFERENCES
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1. Solomon S. Diagnosis of primary headache disorders: validity of the International
Headache Society criteria in clinical practice. Neurol Clin. 1997;15:15-26.
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3. Mombaerts I, Koornneef L. Current status in the treatment of orbital myositis. Ophthalmology. 1997;104:402-408.
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a case report. Headache. 1982;22:184-185.
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7. Mannor GE, Rose GE, Mosely IF, Wright JE. Outcome of orbital myositis: clinical features associated with recurrence. Ophthalmology. 1997;104:409-414.
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8. Shovlin JP. Orbital infections and inflammations. Curr Opin Ophthalmol. 1998;9:41-48.
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