Journal ofNeurology, Neurosurgery, and Psychiatry 1989;52:1290-1293

Short report

Clinicopathological correlation in a case of painfulophthalmoplegia: Tolosa-Hunt syndromeP J GOADSBY, J W LANCE

From the Department ofNeurology, The Prince Henry Hospital and School ofMedicine, University ofNewSouth Wales, Sydney, Australia

SUMMARY A case of painful ophthalmoplegia due to idiopathic granulomatous involvement of thesuperior orbital fissure (Tolosa-Hunt syndrome) is described. The clinical features of recurrent pain,ocular motor nerve palsies and proptosis correlated well with the eventual demonstration of anenhancing mass in the region of the cavernous sinus. Removal of the lesion led to a resolution of theclinical picture and demonstration ofa non-caseating granuloma with no other detectable cause. Theoriginal observation of Tolosa was thus re-affirmed.

Recurrent painful ophthalmoplegia is a rare conditionfirst described by Tolosa' in a male patient who haddied soon after an operation to explore the sella turcicafor left retro-orbital pain and ophthalmoplegia. Atnecropsy there was granulomatous tissue surroundingthe left intracavernous carotid. Hunt2 further charac-terised the syndrome by noting its responsiveness tosteroid therapy. Although there have been over twohundred cases3 reported since, pathological descrip-tions have been limited to Tolosa's and to two furtherconfirmatory descriptions of granulation tissue liningthe superior orbital fissure.45 Another example isdescribed in which the clinical and CT findingscorrelate closely and pathological examination con-firmed the presence of a granuloma.

Case report

The patient, a 56 year old retired soldier, presented initially in1982 with a 10 week history of left-sided throbbing headache,retro-orbital pain, nausea and diplopia. A left sixth nervepalsy was the only finding on physical examination. Bloodcount, erythrocyte sedimentation rate (ESR), serum elec-

Correspondence to: Dr P J Goadsby, Department of Neurology,Clinical Sciences Building, The Prince Henry Hospital, Little BayNSW 2036 Australia.

Received 10 January 1989 and in revised form 29 March 1989.Accepted 6 April1989

trolytes, fasting blood glucose level, liver enzymes, glucosetolerance test, coagulation profile and electrocardiographwere all within normal limits. At lumbar puncture the CSFwas clear and colourless with no cells and a protein content of0-55 g/l. An endrophonium (Tensilon) test was negative andradiography of the paranasal sinuses unremarkable. Acerebral CT scan and bilateral carotid angiogram demon-strated no abnormalities. The provisional diagnosis of tem-poral arteritis was made and treatment with prednisolonewas started. The pain resolved over the ensuing two weeksand the diplopia over the next eight months. Twelve monthslater, steroid therapy was discontinued but the headachesrecurred six months later, on this occasion without anyocular signs. Steroid therapy was recommenced, again with agood response and he experienced few headaches over thefollowing years until 1985. In October, 1985, he presented forreview of polydipsia and polyuria. Diabetes mellitus wasdiagnosed and the steroid therapy was withdrawn. Hisheadache soon became much worse while his blood sugarlevel returned to normal. In January, 1986, he was referred tothe Neurology Clinic for review with a daily throbbingheadache and a left proptosis. A cerebral CT scan was againnormal. He was started on pizotofen (15 mg nocte) withamelioration of his headaches for three months after whichthey recurred. At review in May, 1986, proptosis hadresolved and therapy with methysergide was initiated with sixmonths of relief. By early 1987 the headaches returned and atrial of naproxen proved unsuccessful. By mid-1987 theheadaches were a major problem; a left proptosis and leftsixth nerve lesion had recurred with the addition of a partialleft third nerve palsy. Cerebral CT and left carotid angiogramwere normal. The third nerve and retro-orbital space wereexplored and some thickening of the third nerve was noted,although no mass lesion was seen. Post-operatively both hiseye signs and headache resolved.

1290

Protected by copyright.

on July 12, 2020 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.52.11.1290 on 1 N

ovember 1989. D

ownloaded from

Clinicopathological correlation in a case ofpainful ophthalmoplegia: Tolosa-Hunt syndrome

4:A

AiFig 1 A cerebral CTscan with intravenous contrast showing an enlarged view of the orbit. A contrast-enhancing lesion isdemonstrated against the medial wall ofthe left cavernous sinus.

His headaches returned in late 1987 and were refractory toall medical therapy. The pain had changed somewhat andbecome constant with sharp jabs in the distribution ofophthalmic division ofthe trigeminal nerve. A posterior fossaexploration (Jannetta procedure) provided no relief andsection ofthe ophthalmic division ofthe trigeminal nerve wasthen undertaken with abolition of the headache. In Septem-ber, 1988, he returned with a two week history of diplopia.On examination there was left proptosis and left third nerveand sixth nerve palsies. CT demonstrated an enhancing lesionalong the left cavernous sinus (figs 1 and 2). A left carotidangiogram was normal. At craniotomy a 5 mm diametergranuloma was removed (fig 3). At follow up two monthslater he was pain-free and his ocular signs had resolvedsignificantly with some slight weakness remaining in the leftlateral rectus.

Discussion

A case of painful ophthalmoplegia due to non-specificgranuloma of the retro-orbital region, the Tolosa-Hunt syndrome, is described. The clinicopathologicalcorrelation in this case is precise in that the lesionsuspected clinically was eventually demonstrated onCT and then excised, leading not only to resolution ofsigns but also to a clear pathological diagnosis, a rarityin the case-reports of this disorder.

Recently the aetiology has been called into questionby the suggestion that the syndrome may be caused byvasculitis6 and orbital venography had been used in thediagnosis.' This case, however, certainly re-affirms the

1291

Protected by copyright.

on July 12, 2020 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.52.11.1290 on 1 N

ovember 1989. D

ownloaded from

Goadsby, Lance

Fig2 Acerebra CT scan with intravenous contrast showing a corona s through the anterior cranialfossa. contrast-enhancing lesion is again demonstrated near the medial wall of the cavernous sinus.

original findings reported by Tolosa.' The case wassomewhat atypical in that the patient's retro-orbitalpain was often throbbing whereas the pain in thiscondition has usually been described as boring orstabbing.8 The patient's pain did assume a stabbingquality for a short period before operation.The main differential diagnoses in this setting are:

diabetic ophthalmoplegia (excluded by the normalfasting blood sugar levels and normal glucosetolerance test); carotid aneurysm (excluded by therepeatedly normal angiograms) and tumours of thenasopharynx and orbit (not demonstrated by repeatedCT examinations). In this instance after repeated

examinations, CT revealed the diagnosis. Similar CTfindings have been reported by others,9"' but withoutthe benefit of pathological correlation.

In summary, a case of Tolosa-Hunt syndrome withpainful ophthalmoplegia caused by idiopathic retro-orbital granuloma, is presented. The case history isalmost identical to that described by Tolosa andprovides a rare affirmation of the pathology of thisunusual condition.

The authors thank the Department of Medical Illus-tration, University ofNew South Wales, for photogra-phing the figures.

1292

Protected by copyright.

on July 12, 2020 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.52.11.1290 on 1 N

ovember 1989. D

ownloaded from

Clinicopathological correlation in a case ofpainful ophthalmoplegia: Tolosa-Hunt syndrome

~~~~~~~~~4.~~ ~ ~ ~ ~ ~ ~ ~~~~~4o

#l~~ ~ ~ ~ ~ ~ M"

iv.i

Fig 3 A photomicrograph of the mass removed at operation.

A granulomatous lesion with some multinucleated giant cells

is demonstrated (A: x 78; B: x 120).

References

1 Tolosa E. Periarteritic lesions of the carotid siphon withthe clinical features of a carotid infraclinoidalaneurysm. J Neurol Neurosurg Psychiatry 1954;17:300-2.

2 Hunt WE, Meagher JN, LeFever HE, Zeman W. Painfulophthalmoplegia. Its relation to indolent inflammationof the cavernous sinus. Neurology 1961;11:56-62.

3 Hoes MJAJM, Bruyn GW, Vielvoye GJ. The Tolosa-Hunt syndrome- literature review: seven new cases anda hypothesis. Cephalalgia 198 l;1: 181-94.

4 Lakke JP. Superior orbital fissure syndrome: report of acase caused by local pachymeningitis. Arch Neurol1962;7:289-300.

5 Schatz NJ, Farmer P. Tolosa-Hunt syndrome: the patho-logy of painful ophthalmoplegia. In: Smith JL ed.Neuro-ophthalmology Vol. 6. St Louis: Mosby Co.,1972:102-12.

6 Hannerz J. Pathoanatomic studies in a case of Tolosa-Hunt syndrome. Cephalalgia 1988;8:25-30.

7 Sondheimer FK, Knapp J. Angiographic findings in theTolosa-Hunt syndrome: painful ophthalmoplegia.Radiology 1973;106:105-12.

8 Hannerz J. Pain characteristics of painful ophthalmo-plegia (the Tolosa-Hunt syndrome). Cephalalgia 1985;5:103-6.

9 Thomas DJB, Charlesworth MC, Afshar F, Galton DJ.Computerised axial tomography and magnetic reson-ance scanning in the Tolosa-Hunt syndrome. Br JOphthalmol 1988;72:299-302.

10 Neigel JM, Rootman J, Robinson RG, Durity FA,Nugent RA. The Tolosa-Hunt syndrome: computedtomographic changes and reversal after steroidtherapy. Can J Ophihalmol 1986;21:287-90.

1293

Protected by copyright.

on July 12, 2020 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.52.11.1290 on 1 N

ovember 1989. D

ownloaded from