Tolosa-Hunt syndrome

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Tolosa-Hunt syndrome
Classification and external resources
ICD-10 [1]44.8
DiseasesDB 31164
MeSH D020333


Summary

Tolosa-Hunt syndrome describes episodic orbital pain associated with paralysis of one or more of the third, fourth and/or sixth cranial nerves which usually resolves spontaneously but can relapse and remit.

History

It was first described in 1954 by Tolosa. In 1961, Hunt reported 6 cases of painful ophthalmoplegia that rapidly resolved with steroids.

Disease Entity

Tolosa-Hunt syndrome was first classified by International Headache Society in 2004 and now is a part of Classification ICHD – II. ICD-10 for Tolosa Hunt Syndrome is G44.850

Disease

The diagnostic criteria for Tolosa-Hunt syndrome is as follows:

  • One or more episodes of unilateral orbital pain persisting for weeks if untreated.
  • Paresis of one or more of the third, fourth and/or sixth cranial nerves and/or demonstration of cavernous sinus granuloma by MRI or biopsy.
  • Paresis coincides with the onset of pain or follows it within 2 weeks.
  • Pain and paresis resolve within 72 hours when treated adequately with corticosteroids.
  • Other causes have been excluded by appropriate investigations (please see below for [[Differential Diagnosis|differential diagnosis).


Of note:

Some reported cases of Tolosa-Hunt syndrome had additional involvement of the trigeminal nerve (commonly the first division) or optic, facial or acoustic nerves. Sympathetic innervation of the pupil is occasionally affected.

The syndrome has been caused by granulomatous material in the cavernous sinus, superior orbital fissure or orbit in some biopsied cases.

Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia.

Etiology

Tolosa-Hunt syndrome is idiopathic.

Risk Factors

A possible risk factor for Tolosa-Hunt syndrome is a recent viral infection.

Pathophysiology

Tolosa-Hunt syndrome is idiopathic, sterile inflammation of the cavernous sinus. Its pathology is described as fibroblastic, lymphocytic, and plasmocytic infiltration of the cavernous sinus. Granulocytic and giant-cell infiltrations have also been described. Pathology may extend to involve the superior orbital fissure (sphenocavernous or parasellar syndrome) or orbital apex and affect the optic nerve.

Diagnosis

Symptoms

The patient may complain of double vision worse at distance, headaches, dizziness, nausea, neck stiffness, photophobia, blurred vision, and a “boring” pain may be associated with the headache.

Physical Exam

In addition to the standard ophthalmic examination of the patient including vision, IOP, pupil check for APD and nystagmus, slit-lamp and dilated fundus exam, a complete sensorimotor exam should be done. This includes oculomotor exam (to check for esotropia, exotropia, hypertropia or hypotropia), ductions, vergence, saccades, pursuit, and head tilt/turn. A common finding is abduction deficit associated with esodeviation that increases with gaze to the affected side. Lids should be checked for ptosis or lid retraction or any change in lid aperture during eye movements (to check for aberrant regeneration). Lid strength, fatigue or variability should be noted. Facial sensation should be checked. Stereopsis and color plates should also be evaluated.

Clinical diagnosis

Involvement of multiple contiguous cranial nerves strongly suggest a lesion in the cavernous sinus or subarachnoid space. Only one nerve may be involved, most likely the sixth cranial nerve, which is the only one not protected within the dural wall of the cavernous sinus.

In addition to the complete ophthalmic exam as described, the physician must closely look for Horner syndrome, facial hypoesthesia or engorgement of ocular surface vessels, orbital venous congestion, increased IOP or pulse pressure.

All positive findings should be noted and the differential diagnoses listed below should be considered.

Diagnostic Procedures

The most appropriate imaging includes MRI /MRA (DWI series) which provides information about the cavernous sinus and orbital apex in greater detail than a CT.

A CTA w/ and w/o contrast can also be obtained if an MRI/MRA is not available. A lumbar puncture is recommended to check for opening pressure and CSF should be evaluated for infection/ oligoclonal bands.

Laboratory Tests

Work up should include tests that can rule out the various diseases listed above given the history and context of the patient. This can include CBC w diff, RPR, FTA-ABS, ACE, ANA, p-ANCA, c-ANCA, Anti dsDNA, RF, myasthenia antibodies (binding/blocking/modulating antibodies and anti-MUSK antibodies), TFTs, and a fasting glucose.

Differential Diagnosis

Tolosa-Hunt syndrome is considered a diagnosis of exclusion. Thus, the following entities must be considered and ruled out before a diagnosis of Tolosa-Hunt syndrome is made:

  • Ischemic disease: Hemorrhage, ischemic mononeuropathy
  • Infectious process: post viral syndrome, chronic inflammation of petrous bone (recurrent ear infections), syphilis, basal meningitis
  • Anatomical malformation: aneurysm, AVM, carotid-cavernous fistula, cavernous sinus thrombosis, pseudotumor cerebrii, Duane syndrome/Moebius syndrome, Chiari malformation
  • Inflammatory disease: Sarcoidosis, granulomatosis with polyangiitis (formerly Wegener's), Behcet’s disease
  • Autoimmune condition: myasthenia gravis, thyroid disease, lupus
  • Neoplastic disease: meningioma, neurogenic tumor, hemangioma, lymphoma/leukemia, schwannoma, pituitary adenoma, metastasis, CPA lesion, nasopharyngeal carcinoma, chordoma, chondrosarcoma, brain stem glioma in children
  • Demyelinating disease: MS
  • Others: Diabetes mellitus, Head trauma, BBPV, Meniere’s, ophthalmoplegic migraine

Management

Oral steroids are the mainstay of treatment. Both symptoms and physical exam findings (headache, ptosis, ophthalmoplegia, etc) can be expected to resolve rapidly with an oral steroid taper regimen over 3-4 months. The patient can be co-managed with the Neurology service to rule out other entities listed in the differential diagnosis of Tolosa-Hunt syndrome.

Complications

A rare complication is aberrant regeneration of cranial nerves.

Prognosis

Prognosis of Tolosa-Hunt syndrome is excellent. Full recovery is expected with steroid treatment. The disease may have a relapsing-remitting course.

References

  1. Albert et al. Principles and Practice of Ophthalmology. Third Edition. © 2008.
  2. Aligluo Z et al. Tolosa-Hunt syndrome: A case report. Journal of Neuroradiology. 1999. 26 (1): 68.
  3. BCSC series. Neuro-Ophthalmology. Section 5. American Academy of Ophthalmology.
  4. Dach F et al. Tolosa-Hunt syndrome: critical literature review based on IHS 2004 criteria. Cephalalgia an international journal of headache. 2007. 27 (8): 960-961.
  5. Duane’s Ophthalmology. Lipincott Williams & Wilkins. CD Rom 2006 Edition. www.oculist.net
  6. http://ihs-classification.org/de/
  7. Mendez et al. Painful ophthalmoplegia of the left eye in a 19-year-old female, with an emphasis in Tolosa-Hunt syndrome: a case report. Cases J. 2009; 2: 8271.
  8. Takahashi Y et al. Tolosa-Hunt Syndrome with Atypical Intrasellar and Juxtasellar Lesions:Two Case Reports. The Kurume Medical Journal. 1996. 43: 165-174.