Documenta Ophthalmologica 44,1 : 167-172, 1977 THE TOLOSA-HUNT SYNDROME J.T.W. VAN DALEN & G.M. BLEEKER

(Amsterdam) Keywords: Cavernous sinus, Oculomotor palsy, Orbital Phlebography, Tolosa-Hunt syndrome ABSTRACT The signs and symptoms of the Tolosa-Hunt syndrome are described and the results of orbital phlebography discussed. Emphasis is placed on the importance of systemic administration of corticosteroids, both as a diagnostic test and as a therapeutic measure. A case history is presented. The Tolosa-Hunt syndrome was first described in 1954 by Tolosa of Barcelona. His patient was a 49-year-old man, who had suffered for approximately three years from recurrent, self-limiting left-sided retro-orbital pain. The pain recurred once more after an interval of several months and became associated with ptosis and subsequently with total ophthalmoplegia. Two days after an exploratory craniotomy the patient died and the report of the autopsy (the only available autopsy) stated (literally quoted): 'The intracavernous portion of the left carotid artery was wrapped in granulomatous tissue which, however, did not completely obstruct the lumen of the cavernous sinus. No endo-arterial or meso-arterial lesions were found.' In 1961 Hunt reported 6 similar cases and reviewed Tolosa's slides. He concluded that the case was not primarily an arteritis but an inflammatory process limited to the cavernous sinus. Lakke (1962) showed that the inflammatory process may extend into the superior orbital fissure. In 1966 the term 'Tolosa-Hunt syndrome' was applied to this entity by Lawton-Smith. He stressed the importance of corticosteroid administration as a diagnostic test because of the dramatic therapeutic response to such medication. The main signs of the syndrome are: recurrent unilateral orbital pain; diplopia due to neurologic involvement, including the III rd, IV th and Vlth cranial nerves; - diminished visual acuity, - abnormal orbital phlebography: obstruction of the superior ophthalmic -

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vein in its third part and non-opacification of the cavernous sinus on the affected side. In general, the conditions which may give rise to this kind of orbital phlebogram are: 1) space-occupying lesions in the orbital apex or in the middle cranial fossa; 2) carotid-cavernous sinus fistula; 3) thrombosis or an inflammatorY process in the cavernous sinus. It is apparent that orbital phlebography does not give conclusive evidence of the Tolosa-Hunt syndrome. The syndrome, however, is a distinct entity defined by its clinical manifestations and the dramatic response to systemic steroids. The disease typically runs a recurrent and remitting course. Exacerbations last for days or weeks and spontaneous remissions may be maintained for months or years. The pain usually precedes the ophthalmoplegia and is most commonly retro-orbital, but may extend further. The ophthalmoplegia may be manifested by a III rd, IV th or VI th nerve palsy. Dysfunction of the ophthalmic division of the V ttl cranial nerve is not uncommon and involvement of the optic nerve is approximately 50%. Since the signs and symptoms of the syndrome are mainly ophthalmological, it is particularly important that they should be recognized. As an example we present the case-historY of a 25-year-old woman. After a febrile period in December 1975 our patient complained of slight exophthalmos and constant retro-orbital pain in the right eye. In January 1976 a VI th nerve palsy appeared and in March 1976 palsies of the III rd and IV th cranial nerves developed. There was a decrease in visual acuity to 1/60 and slight exophthalmos, while the visual fields showed several defects. Neurological examination revealed no further abnormality. The blood-pressure was 160/100 and the ESR was 40 mm/hr. All these signs and symptoms may be attributed to a lesion in either: the orbital apex, or the superior orbital fissure, or - the middle cranial fossa. F o r this reason an EMI-scan and carotid angiography were performed. Neither of these showed any gross pathology, while ultrasonography failed to provide further information. Orbital phlebography showed.Ahe above mentioned typical r6ntgenological appearance: obstruction of the fight superior ophthalmic vein in its third part and non-opaciflcation of the cavernous sinus on the right side (Fig. 1). The diagnosis: syndrome of Tosola-Hunt, was made. The patient was given -

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Fig. 1. Orbital phlebography shows obstruction of the ophthalmic vein ip. its third part (R-side).

Fig. 2. Visual fields, April 21, 1976. 169

Fig. 3. Visual fields, April 27, 1976.

Fig. 4. Visual fields, May 5, 1976. 170

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corticosteroids intramuscularly. This led to a dramatic response; the pain subsided a few hours after the treatment was started. The visual acuity improved from 1/60 on April 20 to 6/4 on April 29. The visual fields and the ocular motility returned to almost normal in approximately 14 days (Figs. 2, 3, 4 and 5).

REFERENCES Tolosa, E. Periarteritic lesions of the catorid siphon with the clinical features Of a catotid infraclinoidal aneurysm. Neurol. Neurosurg. Psychiat. 17:300-302 (1954). Hunt, W.E., J.N. Meagher, H.E. LeFever & W. Zeman. Painful ophthalmoplegia. Neurology 11:56-62 (1961). Lakke, J.P.W.F. Superior Orbital Fissure Syndrome. Arch. Neurol. 7:289-300 (1962). (1962).

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Smith, J. Lawton & D.S.R. Taxdal. Painful Ophthalmoplegia, The Tolosa-Hunt Syndrome.Amer. J. Ophthal. 61:1466-1472 (1966). Authors' address: Eye Department Ophthalmological Clinic of the University of Amsterdam Wilhelmina Gasthuis 104, le Helmersstraat Amsterdam The Netherlands

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The Tolosa-Hunt syndrome.

Documenta Ophthalmologica 44,1 : 167-172, 1977 THE TOLOSA-HUNT SYNDROME J.T.W. VAN DALEN & G.M. BLEEKER (Amsterdam) Keywords: Cavernous sinus, Oculom...
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