British Journal of Ophthalmology, 1988, 72, 299-302

Computerised axial tomography and magneticresonance scanning in the Tolosa-Hunt syndromeD J B THOMAS, M C CHARLESWORTH, F AFSHAR, AND D J GALTON

From the Department of Medicine, Radiology, and Neurosurgery, St Bartholomew's Hospital, London EC]

SUMMARY A 50-year-old Asian male presented with a left sixth nerve palsy, left temporal pain,and rapidly deteriorating visual acuity in the left eye. A high resolution CT scan and magneticresonance scan showed a left retro-orbital enhancing lesion extending from the lateral margin ofthe cavernous sinus on to the greater wing of the sphenoid and into the left orbit. Arteriographywas normal. On high dose steroid therapy there was total resolution of the lesion. The value ofimaging techniques in this condition is discussed.

The Tolosa-Hunt syndrome is a rare but wellrecognised cause of painful ophthalmoplegia'characterized by a granulomatous deposit in theregion of the anterior cavernous sinus2 which isusually steroid responsive. The position of this lesionmakes histological confirmation difficult anddangerous, and as the differential diagnosis includesinflammatory vascular and neoplastic lesions3accurate imaging techniques are invaluable. CTscanning is of value in the diagnosis of this condition,but the appearances are not specific. This paperincludes magnetic resonance scans, which have notpreviously been reported in this condition.

Case report

An Asian male aged 50 presented with a four-monthhistory of diplopia and decreasing visual acuity whichwas preceded by three years of left-sided headaches.He dated the diplopia from an accident at work whensome machine oil was splashed in his eye. The onlypast history was of a head injury three years prior topresentation. Physical examination revealed a left-sided sixth cranial nerve lesion with some proptosison that side. Visual acuity was 6/6-9, and Goldmannperimetry was normal. There was reduced sensationto pinprick over the first branch of the trigeminal onthe left.

Shortly after admission there was a rapiddeterioration in the visual acuity of the left eye (from6/6-9 to 6/36). He was immediately started on high-Correspondence to Dr D J B Thomas, Mount Vernon Hospital,Northwood, Middlesex.

dose steroids (16 mg dexamethasone/day), whichproduced a dramatic improvement in visual acuity.An initial high resolution IGE 9800 CT scan

showed an enhancing lesion at the lateral marginsof the cavernous sinus, the greater wing of thesphenoid, and extended into the orbit (Figs. 1A, 1B).The magnetic resonance (MR) scan showed slightproptosis of the left eye with a pseudotumour in theposterior part of the left orbit (Fig. 1C). Arterio-

rig. IA Axial C I scan showing enhancing lesion extendinginto the left orbit.

299

on May 31, 2020 by guest. P

rotected by copyright.http://bjo.bm

j.com/

Br J O

phthalmol: first published as 10.1136/bjo.72.4.299 on 1 A

pril 1988. Dow

nloaded from

D J B Thomas, M C Charlesworth, FAfshar, and DJ Galton

rig. ZA Repeatlsteroid treatment.

Fig. 18B Axial (Ciscan showing marked distortion ofcavernous sinus.

graphy was normal. High resolution CT and MRscans repeated six months after initiation of steroidtreatment were normal (2A, 2B, 2C). Visual evokedresponses (VER) from the left eye were compatiblewith a compressive lesion before steroids werestarted. There was normal latency, depressed ampli-tude, and distorted diphasic waveform. After sixmonths on reducing steroid dosage the VER hasreturned to normal. High-dose steroid therapy wasmaintained for the whole of the four-month period.An early attempt to reduce steroids precipitated a

Fig. IC Ti modeMR scan showing lesion extending intoleft orbit.

Scan ofFig. JA cut six months after

marked deterioration in visual acuity. However,prolonged high-dose steroids resulted in a cushingoidappearance and marked proximal myopathy, andprobably contributed to another admission tohospital, when the patient presented with an acutefebrile illness, dyspnoea, and radiological evidenceof diffuse bilateral pneumonia. The pneumoniaresponded to intravenous ampicillin and erythro-mycin, and the radiological appearance returned tonormal. The only blood titres to change during thisperiod were cytomegalovirus (from 1/64 to 1/1024).The patient is at present well and on a small dose ofsteroids.

Other investigations. Erythrocyte sedimentationrate, 10 mm in the first hour. Mantoux test -ve.

1-ig. LB Repeat C I scan of rig. ]B cutsix months ajiersteroid treatment.

300

on May 31, 2020 by guest. P

rotected by copyright.http://bjo.bm

j.com/

Br J O

phthalmol: first published as 10.1136/bjo.72.4.299 on 1 A

pril 1988. Dow

nloaded from

Computerised axial tomography and magnetic resonance scanning in the Tolosa-Huntsyndrome

4

Fig. 2C RepeatMR scan six months aftersteroid treatment.

Kveim test -ve. Cerebrospinal fluid: protein 0-40 g/l,cells-1 mononuclear cell, cerebrospinal fluidglucose 3-5 mmol/l. VDRL test -ve. Bronchialbiopsy normal.

METHODSHigh-resolution CT scanning was carried out with theIGE 9800 using 2 mm cuts. Scans were taken in theaxial plane and reconstructed in the coronal andsagittal planes. 150 ml of iohexol (Omnipaque) wasused for contrast. Magnetic resonance scanning wascarried out on the MD800 (0-80 Tesla resistivemagnet scanner),in the axial plane on Ti mode (TR1000 ms, T1+200 ms). The visual evoked responsewas performed with a standard checkerboard on fullfields.

Discussion

In 1961 Hunt et al.' collected six cases with thefollowing features. Gnawing retro-orbital pain whichmight precede the ophthalmoplegia and neurologicalinvolvement of any combinations of nerves passingthrough the cavernous sinus. The symptoms mightlast for days or weeks, and although spontaneousremission did occur there was sometimes residualdeficit and attacks might recur subsequently. EarlierTolosa2 had reported on the histology from a patientwith similar symptoms who had died. Histologyshowed a non-specific granulomatous lesioncharacterised by proliferative fibroblasts and byinfiltration of the septa on the walls of the sinus withlymphocytes and plasma cells. Since then there havebeen sporadic reports of non-specific histology.Although the Tolosa-Hunt syndrome is a well recog-nised but rare cause of painful ophthalmoplegia, it

remains a diagnosis of exclusion,4 with many otherlesions such as aneurysms, tumour and even diabeticmicroangiopathy being reported as mimicking it.'When this syndrome was first recognised it was not

possible to obtain direct views of the anteriorcavernous sinus, and indirect procedures such asorbital venography and angiography were necessary.CT scanning has now made it possible to visualise thislesion accurately but the appearances are non-specific. However, they are invaluable in monitoringthe responses to treatment. Magnetic resonancetomography is a non-invasive method for soft tissueimaging, and, as the images are not degraded bybone, it provides an excellent technique for visualis-ing bony structures such as the orbit. The mainfactors influencing soft tissue contrast in magneticresonance are proton density (PD), proton spinlattice relaxation time (Ti), and spin-spin relaxationtime (T2). The last two constants describe theexponential return to equilibrium of proton magnet-isation after excitation. The structures of the normalorbit are easily recognised because of the contrastprovided by orbital fat. This has a high protoncontent and short relaxation time, which makes itappear black .on T modes. The lens also appearsblack, and the coats of the eye are contrasted againstthe vitreous, which is white because of its slowrelaxation time. Orbital bone is not seen on T images.Intraorbital tumours such as metastatic carcinomaor lymphoma appear grey on the T image, and,although the appearance for carcinoma is the sameon the PD image, that for lymphoma may appearwhite. Smith et al.' have reported two orbital pseudo-tumours due to presumed inflammatory changewhich also appear grey on the T image, and this issimilar to the magnetic resonance wan appearance inour patient. Pseudotumours appear less well deline-ated than real tumours, and there is no bone erosion.The virtual disappearance of the lesion after steroidtreatment makes it likely to be a steroid responsiveinflammatory disease.Although histological examination is desirable, the

inaccessibility of the lesion makes the proceduredifficult and hazardous. In the past attempts at biopsyhave been associated with high morbidity andmortality.7 Recently a new technique has been des-cribed, in which a fine needle is directed under CTguidance.4 This appears to have been most successfulin the user's hands diagnosing unsuspected asper-gillosis, but has not been used widely outside thatunit.There are several interesting points in the patient's

response to treatment and the clinical course of thedisease. The subject was a middle-aged Asian whopresented with a sixth nerve lesion, headache, andrapidly deteriorating visual acuity. It has been sug-

301

on May 31, 2020 by guest. P

rotected by copyright.http://bjo.bm

j.com/

Br J O

phthalmol: first published as 10.1136/bjo.72.4.299 on 1 A

pril 1988. Dow

nloaded from

D J B Thomas, M C Charlesworth, FAfshar, and D J Galton

gested that this condition is common in patients ofAsian descent, but this is disputed.' There was adramatic response to high-dose steroid therapy, withrapid clearing of pain and improvement of vision.The diplopia has been slow to improve and still hasnot entirely disappeared. It is interesting that thepatient continually mentions strange colour percep-tion and abnormalities of vision, which have per-sisted in spite of normal visual evoked responses,visual acuities, and Goldmann perimetry. Somepatients with the Tolosa-Hunt syndrome have abnor-malities of contrast sensitivity which are not identi-fied by conventional visual function tests. The precisemechanism for this remains obscure, but it has beensuggested the lesion is due to damage to large fast-conducting fibres which project from the retinalganglion to magnocellular layers of the lateralgeniculate ganglion.9

Although cases of Tolosa-Hunt syndrome mayremit spontaneously, in general they require treat-ment with steroids and are very steroid sensitive.Pain is the most responsive symptom and usuallydisappears within 48 hours of high-dose steroidtreatment. Some authors have suggested this couldbe used as a therapeutic trial because results are soconsistent."' In our patient high levels of dexametha-sone were required (16 mg per day), and this resultedin profound proximal myopathy with a cushingoidfacies. However, there was no worsening of thepatient's mild diabetes or hypertension. Finally the

presumed cytomegalovirus pneumonia was probablyrelated to steroid induced immunosuppression,though it has been reported in healthy persons.''References

1 Hunt WE, Meagher JN, Lefever HE, Zerman Z. Painfulophthalmoplegia: its relation to indolent inflammation of thecavernous sinus. Neurology 1961; 11: 56-62.

2 Tolosa E. Periarteriotic lesions of the carotid siphon with clinicalfeatures of a carotid infra-clinoidal aneurysm. J NeurolNeurosurg PsYchiatrY 1954; 19: 30)-2.

3 Lahke J. Superior orbital fissure syndrome. Arch Neurol 1962; 7:289-300.

4 Rowed DW, Kassel EE, Lewis AJ. Transorbital intracavernousneedle biopsy in painful ophthalmoplegia. J Neurosurg 1985; 62:776-80.

5 Jabs DA, Miller NR, Green WR. Ischaemic optic neuropathywith painful ophthalmoplegia in diabetes meilitus. Br JOphthallmol 1981; 65: 673-8.

6 Smith FW, Cherryman GR, Singh AK, Forrester JV. Nuclearresponse tomography of the orbit at 3-4 MHz. BrJ Radiol 1985;58: 947-57.

7 Dornan TL, Espir LE, Gale EAM, Tattersall RS. Remittentpainful ophthalmioplegia: and Tolosa-Hunt syndrome. J NeurolNeuro.surg Psychiatry 1979; 42: 270-5.

8 Spillane JD. The geography of neurology. Br Med J 1972; ii:506- 12.

9 Fallowfield LJ. Rees JE. Psychophysical assessment of a patientwith Tolosa-Hunt syndrome. J Neurol Neurosurg P.sychiatry1983; 46: 576-8.

1t) Mathew NT, Chandy J. Painful ophthalmoplegia. J Neurol Sci1970;11:243-56.

11 Idell S. Johson M, Beauregard L, Leamur N. Pneumoniaassociated with rising cytomegalovirus antibody titres. Thorax1983;38:957-8.

Accepted for publication 10 February 1987.

302

on May 31, 2020 by guest. P

rotected by copyright.http://bjo.bm

j.com/

Br J O

phthalmol: first published as 10.1136/bjo.72.4.299 on 1 A

pril 1988. Dow

nloaded from