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Review series
NOSOLOGICAL ENTITIES?
The Tolosa-Hunt syndrome
  1. L B Klinea,
  2. W F Hoytb
  1. aDepartment of Ophthalmology, University of Alabama School of Medicine, 601 700 South 18th Street, Birmingham, Alabama, USA, bNeuro-ophthalmology Unit, University of California School of Medicine, San Francisco, California, USA
  1. Dr L Klinelkline{at}uabmc.edu

https://doi.org/10.1136/jnnp.71.5.577

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    Defining the syndrome

    The syndrome of painful ophthalmoplegia consists of periorbital or hemicranial pain, combined with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the trigeminal nerve. Various combinations of these cranial nerve palsies may occur, localising the pathological process to the region of the cavernous sinus/superior orbital fissure.

    The constellation of findings described may be due to four major causes: trauma, neoplasm, aneurysm, and inflammation. Comprehensive patient evaluation is essential in establishing the correct diagnosis.

    Within the last diagnostic category there is a specific subset of patients who develop painful ophthalmoplegia due to a non-specific inflammatory process in the region of the cavernous sinus/superior orbital fissure. Infrequently, they experience involvement of additional cranial nerves ipsilateral to the ophthalmoplegia, including the optic nerve, mandibular branch of trigeminal nerve, and facial nerve. Having a relapsing and remitting course, they respond promptly to systemic corticosteroid therapy. The diagnostic eponym Tolosa-Hunt syndrome has been applied to these patients,1 and it is this entity which forms the basis of this review.

    Historical review

    Almost 50 years ago Tolosa2 reported a patient with left orbital pain, ipsilateral progressive visual loss, total left ophthalmoplegia, and reduced sensation over the first division of the trigeminal nerve. Cerebral angiography disclosed narrowing of the intracavernous segment of the left internal carotid artery. Surgical exploration of the left parasellar region was unremarkable, but the patient died 3 days later. At postmortem, granulomatous inflammation of the affected carotid artery and cavernous sinus was found.

    Seven years later, Hunt et al 3defined a clinical entity “of somewhat obscure aetiology” on the basis of six patients. In one patient surgical exploration of the parasellar region showed all structures to be “intact and healthy”. The authors thought that this …

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