|Year : 1977 | Volume
| Issue : 2 | Page : 22-26
Tolosa Hunt syndrome
KM Zafrulla, SP Dhir, IS Jain
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
K M Zafrulla
Department of Ophthalmology, Postgraduate Institute of Medical Education & Research, Chandigarh-160011
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Zafrulla K M, Dhir S P, Jain I S. Tolosa Hunt syndrome. Indian J Ophthalmol 1977;25:22-6
Painful acute ophthalmoplegia with or without involvement of the optic nerve and ophthalmic division of trigeminal nerve and responding promptly to steroid treatment has come to be regarded as a distinct clinical entity, often called Tolosa Hunt Syndrome.
Tolosa in his case demonstrated on autopsy non specific inflammation of the anterior cavernous sinus around the internal carotid artery. However, the lesions of the superior orbital fissure, orbital apex and anterior cavernous sinus are indistinguishable clinically and can give rise to more or less similar presentation. It is important to recognise that many other pathologies in the same region may give rise to many features of Tolosa Hunt Syndrome where steroids are not necessarily the proper treatment.
We report six cases of painful ophthalmoplegia with good response to steroids. Two of these on investigation had different pathologies than Tolosa Hunt Syndrome.
| Methods and Materials|| |
Six cases of painful ophthalmoplegia admitted in the Eye Department of Nehru Hospital, Postgraduate Institute of Medical Education & Research, Chandigarh, satisfying the following criteria of diagnosis of Tolosa Hunt Syndrome during the last three years, are reviewed.
(a) Gnawing pain over the distribution of ophthalmic division of trigeminal nerve.
(b) Neurological involvement of II, III, IV, V, VI, nerve and oculo-sympathetic in any combination.
(c) Symptoms lasting from days to weeks.
(d) Spontaneous remission with or without residual damage.
(e) May recur
(f) All investigations to localise lesions are negative.
Clinical presentation, investigations and progress of these patients are summarised in [Table - 1].
All patients were below 45 years with equal distribution among males and females. 4 had left and 2 right sided involvement.
In all patients pain preceeded the ophthalmoplegia and proptosis. There was no diplopia in case 2, 4, 6 due to poor visual acuity in one eye.
Extraocular muscles were involved in various combinations but superior oblique was spared in all cases except case 5 where all movements were restricted. In case 2 the only movement present was intortion. Pupil was reacting well in all cases except case No. 2. In case 4 no mention of pupil was made as she had aphakia with complete iridectomy and incarceration of pillars. All cases had pain along the distribution of ophthalmic division of trigeminal nerve. Case 3 had dull corneal sensatlon. Functional damage to optic nerve was present in case 2 and 6 and cannot be ruled out in case 4 which had unilateral degenerative myopia. In case I and 3 disc oedema was present.
In case 1,2 and 6 proptosis was mild. In case 4 there was moderate proptosis but probably the high amount of anisometropia also contribute to the appearance. In all cases including case 3 where there was no proptosis, increase in retro ocular resistance with tenderness was a constant feature. In case 1 during recurrence there was no proptosis but retro ocular resistance and tenderness was present.
| Investigations|| |
Haemogram, urine, stool, X-rays of skull including superior orbital fissure, X-ray chest and Mantoux Test were done in all cases. Case 2 was a diabetic. Cases 3, 4, 5 showed raised ESR. In case No. 1 orbito venography did not reveal any abnormality.
In case 6 carotid angiography revealed a mass lying in relation to lesser wing of sphenoid. Craniotomy revealed a fungal (Aspergillous) granuloma.
| Response to treatment and recurrence|| |
All patients had systemic steroids with antibiotics. All patients responded well with disappearance of pain and restoration of movement to considerable extent. Proptosis also disappeared except in case 5 where there was mild proptosis and chemosis. Cases 2 and 6 had no perception of light initially. Disc oedema in case 1 and 3 disappeared.
| Discussion|| |
Hunt described in 1961 six cases of painful ophthalmoplegia and attributed it to indolent inflammation of anterior cavernous sinus on basis of the nerves involved and on autopsy report of a similar case by Tolosa. He ruled out an orbital lesion by the absence of significant proptosis, absence of palpable mass and absence of retroocular resistance. He did not explain the cause of occasional involvement of second nerve including primary optic atrophy by an inflammation in cavernous sinus. Lakke described a case similar to Tolosa's but on exploration there was pachymeningtitis of superior orbital fissure. In a subsequent paper Hunt mentioned that minor variation in site like anterior cavernous sinus, superior orbital fissure and orbital apex are unimportant.
In our 6 cases the first 4 are cases of classical Tolosa Hunt Syndrome. In these cases retroocular resistance was a constant feature, 11 nerve was involved in 3 cases and cannot be ruled out in 4th case. 1V nerve was invariably spared. So, it can be inferred that the lesion was localised in the orbital apex inside the muscle cone. We did not include it under pseudo tumour because the proptosis was never marked inspite of long standing history in cases 3 and 4 and there was no conjunctival congestion or chemosis. But the distinction may at best be arbitrary.
Smith and Taxdal reported cases of Tolosa Hunt Syndrome and advocated steroids as a therapeutic test. From South India Mathew and Chandy reported 22 cases and confirmed the value of steroids in 13 cases.
We have followed the advice of Smith and Taxdal and put all our cases on sterodis and the response in these 4 cases were satisfactory and are doing well on follow up.
Case 2 inspite of being a diabetic cannot be put under diabetic ophthalmoplegia because such an onset with second nerve involvement is unknown. The proptosis and retro ocular resistance cannot be explained if it is pure diabetic ophthalmoplegia. This patient also responded very well to steroids which were rapidly discontinued.
Case 5 and 6 call for caution in the diagnosis. Fowler et al reported cases simulating Tolosa Hunt Syndrome with good response to steroids but later proved to be lymphoma in one case and aneurysm in another. But it was mentioned that the response to steroid was not rapid. Case 5 does not flit into classical Tolosa Hunt because of
(a) Proptosis was marked
(b) Chemosis of conjunctiva
(c) Even though it responded to combined steroid and antibiotic therapy some amount of proptosis and chemosis persisted. On exploration pus was found.
Case 6 had in addition to ophthalmic findings history of behavioural abnormalities and one convulsion. So a carotid angiography was done which revealed mass in relation to lesser wing to sphenoid. After angiography the patient was put on steroids and surprisingly had very prompt relief of pain and ocular movements improved. On craniotomy a fungal granuloma was found. These last two cases warn us to be cautious in the diagnosis of Tolosa Hunt Syndrome.
| Summary|| |
Six cases of acute painful ophthalmoplegias fitting the clinical description of Tolosa Hunt Syndrome are presented. An excellent response to steroids was obtained. However, two cases which also fitted the clinical discription of Tolosa Hunt Syndrome and responded to steroids were found to have pathology of different nature. One was due to a suppurative lesion at the apex of the orbit and the other case was due to an intracranial mass. Thus the diagnosis of Tolosa Hunt Syndrome should be made with great caution on clinical grounds alone.
| References|| |
Fowler, T.J., Earl C.J., Mc Allister, V.L., Mc Donald, W.I. 1975 Brit. Ophthal, 59,149.
Hunt, W.E., Meagher, J.N. ; Lefever, H.E. and Zeman, W., 1961, Neurology, 11, 56.
Hunt, W. E. 1976, Jour. Neuro. Surg., 44, 544.
Lakke, J.P.W.F., 1962, Arch Neurology, 7: 289.
Mathew, N.T. and Chandy, J., 1970.
Jr. Neurol Sciena 11, 243.
Smith, J.L. and Taxdal, O.S.R. 1966, Amer. Jour. Ophthal., 61, P966
Tolosa, E., 1954, Jour. Neurol. Neuro. Surg.,
Psychiat, 17, 300.
[Figure - 1], [Figure - 2], [Figure - 3]
[Table - 1]