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.

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 …

https://jnnp.bmj.com/content/jnnp/71/5/577.full.pdf

The Tolosa-Hunt syndrome.

The Tolosa-Hunt syndrome

✓ The author reports 12 cases of Tolosa-Hunt syndrome, a benign steroid-resistant cryptogenic granuloma that presents as painful ophthalmoplegia. This syndrome is differentiated from other causes of painful ophthalmoplegia including tumors, aneurysms, collagen disease, specific infections, mucoceles, and benign granulomas of unknown etiology. These other conditions should be excluded by appropriate tests. Surgical exploration is not necessary if there is a prompt remission on steroid therapy.

Tolosa-Hunt syndrome: one cause of painful ophthalmoplegia

A patient is described with features of a superior orbital fissure (Tolosa Hunt) syndrome and a coexistent intraorbital lesion. There was radiological evidence both of narrowing of the carotid artery and of an intraorbital obstruction of venous return from the orbit. The diagnostic value of orbital venography and carotid angiography in the investigation of granulomata in the region of the orbit is stressed. The condition described here is responsive to corticosteroids and it is also inferred that there may be a clinicopathological spectrum encompassing both the Tolosa Hunt syndrome and pseudotumour of the orbit.

https://jnnp.bmj.com/content/jnnp/36/3/486.full.pdf

Superior orbital fissure syndrome: Some clinical and radiological observations

The Tolosa-Hunt syndrome (THS), a steroid-responsive painful ophthalmoplegia secondary to idiopathic granulomatous inflammation, historically has been categorized as a diagnosis of exclusion because of its nonspecific radiologic presentation. Five patients who satisfied the anatomic and clinical criteria of this syndrome underwent high-resolution CT of the orbital apex/cavernous sinus region. Two patients were diagnosed as having orbital apex pseudotumor, two as having cavernous sinus inflammation, and one as having a cavernous sinus epidermoid by the characteristic CT and clinical findings. Follow-up studies while the patients were asymptomatic demonstrated complete resolution of the CT abnormalities in four patients and clinical improvement in all five patients. Our data suggest that orbital apex pseudotumor and granulomatous inflammation of the cavernous sinus have similar clinical features and should be considered as part of the spectrum of THS. With the advent of high-resolution CT, THS may now be a diagnosis of inclusion. Symptomatic improvement after steroid therapy is an essential but not absolute proof of the syndrome, since lesions such as lymphomas may also respond to steroids. Resolution of the soft-tissue inflammation on CT is an additional criterion for diagnosis.

http://www.ajnr.org/content/ajnr/8/6/1067.full.pdf

Luis B. Morretin

Papers

Report of a case of sphenoid fissure syndrome studied by orbital venography.