Painful ophthalmoplegia …

Ravindra Kumar Garg DM FRCP

Neuro-Ophthalmology & Neuro-Otology

Updated 06.28.2024
Released 05.08.1995
Expires For CME 06.28.2027

Painful ophthalmoplegia

Author: Ravindra Kumar Garg DM FRCP

See Contributor Disclosures

Editor: Jonathan D Trobe MD

Painful ophthalmoplegia

In This Article

Quick Link

Introduction

Overview

Painful ophthalmoplegia is the term applied to unilateral periocular or cranial pain together with ipsilateral single or multiple ocular motor palsies. Also present sometimes are hypesthesia in the ophthalmic and sometimes maxillary divisions of the trigeminal nerve, Horner syndrome, and optic neuropathy. The offending lesion is usually in the cavernous sinus or superior orbital fissure. There are many causes, including idiopathic pachymeningitis (formerly known as Tolosa-Hunt syndrome), intracavernous carotid aneurysm, arteriovenous fistula, malignancy, vasculitis, and bacterial or fungal infection. An important distinction must be drawn between these entities and the self-limited ischemic cranial mononeuropathy in adults with diabetes or essential hypertension, in which case imaging will not reveal any pertinent abnormalities. In children and younger adults, recurrent single unilateral ocular motor neuropathy with periocular pain has been labeled “recurrent painful ophthalmoplegic neuropathy,” an idiopathic condition in which brain imaging may show extra-axial inflammation of the affected ocular motor nerve. Other mimickers are inflammations and tumors of the orbit, which should be evident on imaging. Imaging is critical to localize the process, but an exact diagnosis often depends on biopsy or discovery of other relevant factors. Idiopathic pachymeningitis generally improves on treatment with corticosteroids, but so do the many other causes of this clinical syndrome.

Key points

	• Painful ophthalmoplegia is characterized by unilateral periocular or cranial pain; ipsilateral single or multiple ocular motor palsies; and sometimes first or second trigeminal hypesthesia, Horner syndrome, and optic neuropathy.
	• The process affects the cavernous sinus, superior orbital fissure, and/or orbit.
	• A common cause is idiopathic pachymeningitis (formerly known as Tolosa-Hunt syndrome), but other causes include cavernous aneurysms, arteriovenous fistulas, malignancies, vasculitis, infections, and ischemia, as well as orbital inflammation and tumors.
	• Localization and pathologic diagnosis depend on imaging, biopsy, and other contributory factors.
	• When only a single ocular motor palsy is present in older adults with diabetes or hypertension, an important consideration is a self-limited ischemic process affecting the extra-axial nerve; brain imaging typically shows no pertinent abnormalities.
	• When only a single ocular motor palsy occurs repeatedly without contributory factors, a diagnosis of “recurrent painful ophthalmoplegic neuropathy” has been assigned; imaging may show enhancement of the affected cranial nerve.
	• Idiopathic pachymeningitis often improves clinically and radiologically with corticosteroid treatment.

Historical note and terminology

In 1858, Hirschfeld first described the “syndrome of the superior orbital fissure” in a patient with a head injury (13). At autopsy, a hematoma was demonstrated near that structure. In 1955, Jackson described four cases of ocular motor palsy associated with severe headache in patients with diabetes (17). In 1890, the term “ophthalmoplegic migraine” was first used by Charcot to describe cases of headache with ocular motor nerve palsy (06).

In 1954, Tolosa described a 47-year-old man with recurrent periocular pain and dysfunction of the third, fourth, fifth, and sixth cranial nerves (32). There had been a 3-year spontaneous remission between symptomatic episodes. The patient died a few days after an intracranial exploratory operation that had yielded no definite pathology. The autopsy, however, later showed inflammation in the intracavernous portion of the internal carotid artery and adjacent cranial nerves.

In 1961, Hunt and colleagues reported six patients with periocular and brow pain along with various combinations of third, fourth, and sixth cranial nerve dysfunction on the same side (15). They reviewed Tolosa's cases and judged that their patients had the same condition based on rapid and dramatic relief of pain and ophthalmoplegia produced by systemic corticosteroid administration.

Lakke described clinically similar cases and presented pathologic material from a 47-year-old male patient with severe right periocular pain followed by progressive ipsilateral ophthalmoplegia and Horner syndrome (22). In 1966, Smith and Taxdal used the term “Tolosa-Hunt syndrome” for the first time and described an excellent response to corticosteroids (29). However, many other cavernous sinus conditions, including aneurysms, arteriovenous fistulas, malignancies, and fungal infections, have a similar clinical presentation and may improve with corticosteroid treatment. Imaging often cannot easily distinguish among them. Consequently, many observers have urged that the eponym Tolosa-Hunt be discarded. In fact, the terms “idiopathic pachymeningitis,” “idiopathic hypertrophic pachymeningitis,” or “idiopathic granulomatous pachymeningitis” are now preferred.

The idea that orbital inflammation could also cause periocular pain and ipsilaterally diminished eye movements was long ago recognized by Birch-Hirschfeld, who coined the term "pseudotumor" to distinguish orbital inflammation from orbital tumor (05). In the early 1940s, a distinction was drawn between inflammation primarily involving the extraocular muscles and inflammation primarily involving orbital fat or other tissues (08). The term "orbital myositis" was coined to describe cases with primarily extraocular muscle involvement. The term “idiopathic orbital inflammation” has been applied when no underlying cause is identified.

Clinical manifestations

Presentation and course

Painful ophthalmoplegia is a clinical syndrome that includes ipsilateral single or multiple ocular motor nerve palsies involving the third, fourth, or sixth cranial nerves, in addition to unilateral periocular pain. Patients may also have Horner syndrome and reduced sensation in the first or second divisions of the trigeminal nerve. If there is optic nerve involvement as well, the condition is considered to extend to the orbital apex. The causes include a variety of vascular, neoplastic, infectious, ischemic, and autoimmune inflammatory disorders (11).

Idiopathic pachymeningitis (formerly Tolosa-Hunt syndrome). This a sometimes granulomatous inflammation in and around the cavernous sinus or superior orbital fissure with MRI evidence of thickening and enhancement in those regions. Imaging typically cannot rule out specific inflammatory or neoplastic causes. Because obtaining tissue from this region is dangerous, examiners must look for evidence of a similar process in an accessible region (such as a paranasal sinus) or settle for empiric anti-inflammatory treatment. Sarcoidosis, IGG4 disease, and primary vasculitides should be identified as they may have specific treatments (25). Otherwise, the diagnosis remains idiopathic pachymeningitis (idiopathic hypertrophic meningitis).

Acute painful ischemic ocular motor mononeuropathy. A painful, single third, fourth, or sixth cranial nerve palsy may occur in patients with diabetes or systemic hypertension. Attributed to ischemia of the extra-axial portion of the nerve, the process is self-limiting, with spontaneous recovery within 12 weeks. The onset may be somewhat more acute than in idiopathic pachymeningitis, but otherwise the distinction depends on imaging, which will be negative in this condition. The reversibility of this deficit is attributed to a minimal degree of ischemia compatible with axon regeneration.

Recurrent painful ophthalmoplegic neuropathy (ophthalmoplegic migraine). When there are recurrent bouts of a single ocular motor palsy with ipsilateral periocular pain in a patient without vasculopathic risk factors, the label “recurrent painful ophthalmoplegic neuropathy” has been assigned (14; 01). The pain may precede the palsy by as much as 2 weeks (07). Neuroimaging sometimes demonstrates inflammatory changes in the extra-axial portion of the affected nerve (30). Formerly known as “ophthalmoplegic migraine,” this condition has been associated with elevated serum calcitonin gene-related peptide (CGRP) levels in the periods between headache attacks (31), but the imaging abnormality favors an inflammatory disorder.

Idiopathic orbital inflammation. Idiopathic orbital inflammation (also known as “orbital pseudotumor”) is believed to represent an autoimmune process. It is characterized by thickening and enhancement within the orbital intraconal fat, extraocular muscles, lacrimal gland, or sclera. Congestive orbital features are often present, including proptosis, ocular adnexal tissue swelling, conjunctival hyperemia, and ptosis (16). Imaging cannot easily differentiate this process from Graves disease or from malignancy, especially lymphoma.

Orbital cellulitis. Orbital cellulitis is an infection of orbital tissues that frequently affects children but may also occur in adults. In children, the infection usually arises in infected paranasal sinuses (mostly the ethmoid sinus). In adults, a common source is the facial skin. The presumptive pathogens are common skin and mucosal organisms. Antibiotic treatment is empirical, as smears and cultures are not typically drawn. Full resolution is usual. However, in immunocompromised individuals and in infections arising from unconventional sources, such as the mouth, orbital cellulitis may lead to vision loss, cavernous sinus thrombosis, meningitis, cerebral venous thrombosis, subdural empyema, and brain abscess. Mucormycosis and aspergillosis are important infectious causes in patients who have uncontrolled diabetes or who are immunocompromised (12; 02). Imaging typically shows sino-orbital abnormalities, but differential diagnosis can be challenging.

Carotid-cavernous fistula. Carotid-cavernous arteriovenous fistula is an anomalous communication between the intracavernous carotid artery or cavernous dural arterioles and the cavernous sinus. High-flow fistulas, which emanate from a hole in the intracavernous carotid artery, arise in head trauma, atherosclerotic aneurysms, or elastotic degenerations, especially Ehlers-Danlos type IV. Apart from periocular pain and ophthalmoplegia, manifestations include some combination of an audible bruit, pulsatile exophthalmos, high intraocular pressure, optic neuropathy, dilated retinal veins, and hyperemic (“arterialized”) conjunctival vessels. Low-flow fistulas, which emanate from newly opened channels between the dural arterioles and the cavernous sinus, generally have similar but less florid clinical features. Attributed to a change in hormonal status, low-flow fistulas most often arise spontaneously in women in the postpartum or post-menopausal period (33). Cross-sectional imaging frequently shows a dilated superior ophthalmic vein, but definitive diagnosis depends on dynamic vascular imaging that reveals the arteriovenous communications, with early filling of the venous channels in and around the cavernous sinus, including the superior ophthalmic vein. The clinical manifestations are attributed to an altered arteriovenous gradient that leads to arterial ischemia (“steal”) and venous hypertension.

Intracavernous aneurysms. Intracavernous aneurysms represent atherosclerotic fusiform dilation of the internal carotid artery. Sudden expansion of the vessel wall typically compresses the trigeminal nerve, causing the periocular pain. Compression of the ocular motor nerves, most commonly the sixth nerve, produces ductional deficits and diplopia. Vascular imaging is distinctive.

Fungal infection. Mucormycosis and aspergillosis are sino-orbital fungal infections that arise in patients with poorly controlled diabetes and other immunocompromised states. The infection begins in the paranasal sinuses, spreading into the orbit and often the cranial base, including the cavernous sinus. Rapidly progressive vascular occlusion with necrosis manifests as infarction of orbital and adjacent intracranial tissues. Careful examination of nasal and sinus tissues may reveal the characteristic necrotic ulcers. Rapid institution of anti-fungal treatment is essential to prevent death. Orbital exenteration was formerly the favored treatment for disease limited to the orbit, but aggressive intravenous and direct intraorbital infusion may avert that disfiguring intervention. A single case of cavernous sinus actinomycosis has been reported (26). Mycobacterial infection is a rare cause that must be considered in endemic areas (27).

Vasculitis. ANCA-positive and ANCA-negative primary and secondary vasculitides may selectively invade the cavernous sinus to cause painful ophthalmoplegia. Imaging may show only subtle enhancement, sometimes with involvement of a paranasal sinus, allowing biopsy confirmation. The necrotizing vasculitides pose a special threat and must be promptly diagnosed. Serologic testing is a valuable lead, and systemic clinical features are crucial in diagnosis (25).

Malignancy. Primary and metastatic cancer often involves the cavernous sinus. The neoplasms may extend from the pituitary fossa, sphenoid sinus, or orbit. Lymphoma and histiocytosis are commonly implicated. Imaging is rarely diagnostic. Evaluation heavily depends on finding tumors in sites accessible to biopsy. Cerebrospinal fluid may be positive for malignancy.

Prognosis and complications

The prognosis of painful ophthalmoplegia depends on the cause. In idiopathic pachymeningitis, recovery is generally good, but not uniformly so. Moreover, some patients require prolonged corticosteroid or other immunosuppressive treatment because they have a relapsing-remitting course. A few patients do not recover at all. In one study, approximately 50% of patients with “Tolosa-Hunt syndrome” reported recurrence (03). Steroid-sparing immunosuppressive treatment is required to prevent recurrence in such cases. Corticosteroids are effective in relieving facial pain within 24 to 72 hours following initiation of treatment.

Orbital myositis typically recovers more completely than idiopathic pachymeningitis, but in many cases, there is recurrence when treatment is discontinued. The patients who do not respond may need immunosuppressive drugs or even radiotherapy (23). In 209 cases of idiopathic orbital inflammation, male gender and degree of proptosis predicted a higher relapse rate (34).

Necrotizing vasculitis and mucormycosis are most threatening because they advance by invading arteries and infarcting tissue. Early treatment is essential.

With sudden expansion, intracavernous aneurysms may cause florid clinical manifestations and severe trigeminal pain, but these manifestations often subside spontaneously. Although frequently treated with endovascular stents, the indications for intervention are debated. High-flow fistulas are usually successfully treated with coils introduced into the cavernous sinus transvenously. Low-flow fistulas may spontaneously resolve over many months but can also be successfully treated with transvenous coil embolization if diplopia, periocular pain, very high intraocular pressure, disfiguring congestion, or optic neuropathy are intractable.

Biological basis

Painful ophthalmoplegia predominantly represents damage to the ocular motor nerves and the first division of the trigeminal nerve, which lie in the outer dural wall of the cavernous sinus and enter the superior orbital fissure. Lesions in that vicinity do their damage by inflammation, ischemia, or compression. In the active phase of inflammation, appropriately dosed corticosteroid therapy is usually effective; in the chronic (cicatricial) phase of inflammation, deficits are less reversible with treatment. Rapid expansion of tumors, aneurysm, or cysts may cause acute deficits, but more often such lesions produce slowly progressive worsening. High-flow fistulas, which often follow head trauma, typically produce acute deficits, probably by creating ischemia via arterial steal; low-flow fistulas are more likely to produce chronic evolving deficits by slowly mounting orbital venous pressure. Aspergillus infections and vasculitides generally cause slowly progressive deficits by inflammation, whereas mucormycosis causes rapidly evolving deficits by vascular invasion and infarction. Malignancies generally manifest a slow tempo of progression.

Etiology and pathogenesis

In a series of 151 patients with a cavernous sinus syndrome, the most frequent cause was malignancy (30%) (18). Head trauma was next most common (24%), followed by idiopathic inflammation (23%). The remaining 12% of patients were diagnosed with carotid aneurysms and carotid-cavernous fistulas, infection, and other uncommon causes, such as pituitary apoplexy. In another series of 126 patients, malignancies were again the most frequent cause, followed by aneurysms, carotid-cavernous fistulas, and idiopathic inflammation (called Tolosa-Hunt syndrome) (09). In a retrospective analysis of 77 patients, the most common diagnosis was idiopathic inflammation, followed by aneurysm and “diabetic ophthalmoplegia” (35). Middle cranial fossa tumors and one fungal infection accounted for the remaining cases. Of the 46 patients with idiopathic cavernous sinus inflammation (Tolosa-Hunt syndrome), only 52% had MRI abnormalities in the appropriate region. In a review of 73 patients, 86% had a definitive cause, most commonly malignancies, fungal infections, and idiopathic inflammation (Tolosa-Hunt syndrome) (04).

Epidemiology

Keane analyzed causes of cavernous sinus syndrome in 151 patients (18). He noted that classic causes, such as aneurysm, meningioma, and bacterial infection, were uncommon. In this series, the most frequent cause of cavernous sinus syndrome was neoplastic (30%; 45/151). Traumatic disorders (24%; 36/151) were the next most common cause. Idiopathic inflammatory diseases were diagnosed in 34 (23%) patients. The remaining 12% of patients were diagnosed with carotid aneurysms and carotid-cavernous fistulas, infection, and other uncommon causes, like pituitary apoplexy. Fernandez and colleagues evaluated 126 patients and noted that neoplastic disorders were the most frequent cause of cavernous sinus syndrome (09). The next most common causes were vascular diseases (aneurysms and carotid-cavernous fistula) and Tolosa-Hunt syndrome. Zhang and colleagues undertook a retrospective analysis of 77 patients with painful ophthalmoplegia (35). The most common final diagnosis was idiopathic cavernous sinus inflammation in 46 (Tolosa-Hunt syndrome; mean age 44.4 years), followed by intracranial aneurysm in 13 (posterior communicating artery in 10, internal carotid artery in three; mean age 58.8 years) and diabetic ophthalmoplegia in nine (mean age 63.2 years) as well as an assortment of middle cranial fossa tumors and one fungal infection. Of the 46 patients with idiopathic cavernous sinus inflammation (Tolosa-Hunt syndrome), 24 had abnormal MRIs, of which 19 involved the cavernous sinus and parasellar region, nine the orbital apex and superior orbital fissure, and eight the orbital apex and cavernous sinus. Bhatkar and colleagues evaluated 73 patients with cavernous sinus syndrome and were able to establish a definitive cause in 86% of patients (04). In this series, neoplastic diseases, fungal infections, and Tolosa-Hunt syndrome were the most frequent to affect the cavernous sinus.

Diagnostic workup

Painful ophthalmoplegia is not a disease but a clinical syndrome. There are many causes. The diagnostic challenge is that the lesion is in an area that cannot be accessed for biopsy without serious hazard unless it has a component in the adjacent paranasal sinuses or an identifiable remote source. Orbital extension, if discrete and forward enough, might permit biopsy, but the procedure may cause permanent ophthalmoplegia, ptosis, and optic neuropathy. Therefore, evaluation depends on finding contributory abnormalities on imaging, serology, and spinal fluid examination (28).

Tolosa Hunt syndrome (MRI)

Clinical and radiological images from a patient presenting with cavernous sinus syndrome, later diagnosed as Tolosa Hunt syndrome. (A-D) Showing ptosis and ophthalmoplegia involving the left eye. (E, F) Post-contrast T1 images sho...
Fungal infiltration of cavernous sinus presenting as painful ophthalmoplegia

(A, B) Post-contrast T1- and T2-weighted images showing signal alteration and contrast enhancement of left orbital apex and ethmoid sinus (white arrow). (C) Hematoxylin and eosin staining showing fungal hyphae (black arrow). (Cont...
Pituitary apoplexy

MRI without contrast in a patient presenting with pituitary apoplexy. There is a fluid level within the tumor that indicates hemorrhage in a pituitary tumor. (Contributed by Dr. Ravindra Kumar Garg.)
Optochiasmatic tuberculous meningitis

Contrast-enhanced MRI brain showing tuberculous basal exudates in the perichiasmal region. Patient presented with headache, vision loss, and ophthalmoplegia. (Contributed by Dr. Ravindra Kumar Garg.)
Nasopharyngeal carcinoma with skull base erosion

Nasopharyngeal carcinoma with skull base erosion, infiltrating the meninges and presenting with multiple cranial nerve palsies. (Contributed by Dr. Ravindra Kumar Garg.)

In addition to neuroimaging, diagnostic evaluation in a case of painful ophthalmoplegia requires testing for a battery of blood and cerebrospinal fluid parameters (Table 1).

Table 1. Suggested Workup for a Patient with Painful Ophthalmoplegia

Blood parameters
	Complete blood count Erythrocyte sedimentation rate Blood glucose Electrolytes Liver function tests Kidney function tests C-reactive protein Antinuclear antibody assay Antineutrophil cytoplasmic antibodies assay Serum protein electrophoresis Angiotensin-converting enzyme
Cerebrospinal fluid parameters
	Protein, glucose, and cells Gene-Xpert test for tuberculosis Fungal culture Malignant cells
(10)

Media

References

01: Aleksic DZ, Drakulic SM, Ljubisavljevic S. Recurrent painful ophthalmoplegic neuropathy: migraine, neuralgia, or something else? J Oral Facial Pain Headache 2020;34(4):374-8. PMID 33290443
02: Aloua R, Kerdoud O, Slimani F. Cavernous sinus thrombosis related to orbital cellulitis serious complication to prevent: a case report and literature review. Ann Med Surg (Lond) 2021;62:179-81. PMID 33532066
03: Arthur A, Sivadasan A, Mannam P, et al. Tolosa-Hunt syndrome: long-term outcome and role of steroid-sparing agents. Ann Indian Acad Neurol 2020;23(2):201-5. PMID 32189862
04: Bhatkar S, Goyal MK, Takkar A, et al. Cavernous sinus syndrome: a prospective study of 73 cases at a tertiary care centre in Northern India. Clin Neurol Neurosurg 2017;155:63-9. PMID 28260625
05: Birch-Hirschfeld A. Zur diagnostic and pathologic der orbital tumoren. Ber Dtsch Opthalmol Ges 1905;32:127–35.
06: Charcot JM. Sur un cas de migraine ophthalmoplegique (paralysie oculo-motrice periodique). Progr Med (Paris) 1890;31:83-6;32:99-102.
07: Dhoot R, Sengupta S. Recurrent painful ophthalmoplegic neuropathy with headache and oculomotor palsy 18 years apart. Headache 2022;62(10):1434-6. PMID 36373830
08: Dunnington J, Berke R. Exophthalmos due to chronic orbital myositis. Arch Ophthalmol 1943;30:446-65.
09: Fernandez S, Godino O, Martínez-Yélamos S, et al. Cavernous sinus syndrome: a series of 126 patients. Medicine (Baltimore) 2007;86(5):278-81. PMID 17873757
10: Gladstone JP. An approach to the patient with painful ophthalmoplegia, with a focus on Tolosa-Hunt syndrome. Curr Pain Headache Rep 2007;11(4):317-25.** PMID 17686398
11: Gladstone JP, Dodick DW. Painful ophthalmoplegia: overview with a focus on Tolosa-Hunt syndrome. Curr Pain Headache Rep 2004;8(4):321-9.** PMID 15228894
12: Hauser A, Fogarasi S. Periorbital and orbital cellulitis. Pediatr Rev 2010;31(6):242-9. PMID 20516236
13: Hirschfeld L. Epanchement de sang dans le sinus caverneux du cote gauche diagnostique pendant la vie. Compt Rend Soc Biol 1858;5:138.
14: Huang C, Amasanti M, Lovell B, Young T. Recurrent painful ophthalmoplegic neuropathy. Pract Neurol 2017;17(4):318-20. PMID 28473604
15: Hunt WE, Meagher JN, LeFever HE, Zeman W. Painful ophthalmoplegia: its relation to indolent inflammation of the cavernous sinus. Neurology 1961;11:56-62. PMID 13716871
16: Infante R, Donadio V, Nucera B, et al. Clinical reasoning: young woman with orbital pain and diplopia. Neurology 2020;94(7):e752-7. PMID 32015178
17: Jackson WP. Ocular nerve palsy with severe headache in diabetics. Br Med J 1955;2(4936):408-9. PMID 13240125
18: Keane JR. Cavernous sinus syndrome. Analysis of 151 cases. Arch Neurol 1996;53(10):967-71. PMID 8859057
19: Kemeny AA. Tolosa-Hunt syndrome: another legitimate target for radiosurgery? Acta Neurochir (Wien) 2016;158(1):141. PMID 26611688
20: Kim HJ, Lee S, Koo YJ, et al. Tacrolimus for treating orbital and cranial form of idiopathic inflammatory pseudotumors. J Clin Neurol 2020;16(4):674-80. PMID 33029975
21: Kwon HS, Kim TY, Kim JH, et al. Use of methotrexate in the management of recurrent Tolosa-Hunt syndrome: two case reports. Medicine (Baltimore) 2020;99(17):e19882. PMID 32332660
22: Lakke JP. Superior orbital fissure syndrome: report of a case caused by local pachymeningitis. Arch Neurol 1962;7:289-300. PMID 13928165
23: Montagnese F, Wenninger S, Schoser B. “Orbital around” the orbital myositis: clinical features, differential diagnosis and therapy. J Neurol 2016;263(4):631-40. PMID 26477021
24: Piamonte BLC, Ong KL, Cenina AR. Tolosa-Hunt syndrome relapse during pregnancy following chronic intake of combined oral contraceptives. BMJ Case Rep 2021;14(1):e238944. PMID 33462037
25: Prete B, Sowka J. Painful ophthalmoplegia as an initial presentation of sarcoidosis. Clin Exp Optom 2017;100(3):291-3. PMID 27628944
26: Rodriguez SS, Gómez-Muga JJ, Onandi RR, Diez Gallarreta Z, Garcia-Monco JC. A 57-year-old man with painful ophthalmoplegia and cavernous sinus involvement: why this is not Tolosa-Hunt syndrome. Eur J Neurol 2022;29(10):3127-9. PMID 36073199
27: Sasi S, Nair AP, Kolleri J, Alzibdeh A, Maslamani MSRA. Disseminated tuberculosis masquerading as Tolosa-Hunt syndrome in initial presentation: a case report with literature review. Radiol Case Rep 2023;18(3):1353-7. PMID 36712191
28: Sharifi A, Kayfan S, Clarke RL, Mehta A, Pfeifer CM. Recurrent painful ophthalmoplegic neuropathy: MRI findings in 2 patients. Radiol Case Rep 2019;14(8):1039-42. PMID 31236187
29: Smith JL, Taxdal DS. Painful ophthalmoplegia. The Tolosa-Hunt syndrome. Am J Ophthalmol 1966;61(6):1466-72. PMID 5938314
30: Smith SV, Schuster NM. Relapsing painful ophthalmoplegic neuropathy: no longer a "migraine," but still a headache. Curr Pain Headache Rep 2018;22(7):50. PMID 29904898
31: Takkar A, Kumar KVA, Katoch D, et al. Serum calcitonin gene-related peptide is elevated in patients with migraine and ophthalmoplegia. J Neuroophthalmol 2023;43(3):399-405. PMID 36255114
32: Tolosa E. Periarteritic lesions of carotid siphon with clinical features of a carotid infraclinoidal aneurysm. J Neurol Neurosurg Psychiatry 1954;17:300-2. PMID 13212421
33: Williams ZR. Carotid-cavernous fistulae: a review of clinical presentation, therapeutic options, and visual prognosis. Int Ophthalmol Clin 2018;58(2):271-94. PMID 29517654
34: Yan J, Lu Z, Wu Z, et al. Features associated with recurrence of idiopathic orbital inflammatory pseudotumor. Yan Ke Xue Bao 2007b;23(1):58-64. PMID 17444043
35: Zhang X, Zhou Z, Steiner TJ, et al. Validation of ICHD-3 beta diagnostic criteria for 13.7 Tolosa-Hunt syndrome: analysis of 77 cases of painful ophthalmoplegia. Cephalalgia 2014;34(8):624-32. PMID 24477599

Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Author

Ravindra Kumar Garg DM FRCP

Dr. Garg of King George's Medical University in Lucknow, India, has no relevant financial relationships to disclose.
See Profile

Editor

Jonathan D Trobe MD

Dr. Trobe of the University of Michigan has no relevant financial relationships to disclose.
See Profile

Former Authors

Heather E Moss MD PhD

Patient Profile

Age range of presentation: 6 to 65+ years
Sex preponderance: female>male, >1:1
Heredity: heredity may be a factor
Population groups selectively affected: none selectively affected
Occupation groups selectively affected: none selectively affected

ICD & OMIM codes

ICD-9: Orbital pseudotumor: 376.11
ICD-10: Granuloma of orbit: H05.1

Other paralytic strabismus: H49.8

Tolosa-Hunt syndrome: H49.40
ICD-11: Painful cranial neuropathies or other facial pains: 8A85

Granulomatous orbital inflammation: 9A22.2

Other specified non paralytic strabismus: 9C80.Y

This is an article preview.
Start a Free Account
to access the full version.

Nearly 3,000 illustrations, including video clips of neurologic disorders.
Every article is reviewed by our esteemed Editorial Board for accuracy and currency.
Full spectrum of neurology in 1,200 comprehensive articles.
Listen to MedLink on the go with Audio versions of each article.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com

ISSN: 2831-9125

Painful ophthalmoplegia

Also Relevant

Combined third, fourth, and sixth nerve palsies
Eye-related headache
Recurrent painful ophthalmoplegic neuropathy

Painful ophthalmoplegia …

Painful ophthalmoplegia

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Biological basis

Etiology and pathogenesis

Epidemiology

Diagnostic workup

Table 1. Suggested Workup for a Patient with Painful Ophthalmoplegia

Management

Outcomes

Special considerations

Pregnancy

Media

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

This is an article preview.
Start a Free Account
to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

Common maculopathies

Primary headache associated with sexual activity

Toxic and nutritional deficiency optic neuropathies

Third nerve palsy

Nystagmus

Primary cough headache

Medications and substances causing headache

Painful ophthalmoplegia

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Biological basis

Etiology and pathogenesis

Epidemiology

Diagnostic workup

Table 1. Suggested Workup for a Patient with Painful Ophthalmoplegia

Management

Outcomes

Special considerations

Pregnancy

Media

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

This is an article preview. Start a Free Account to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

Common maculopathies

Primary headache associated with sexual activity

Toxic and nutritional deficiency optic neuropathies

Third nerve palsy

Nystagmus

Primary cough headache

Medications and substances causing headache

This is an article preview.
Start a Free Account
to access the full version.