|Year : 1994 | Volume
| Issue : 4 | Page : 207-209
Computerised tomography in Tolosa-Hunt syndrome
Lekha Pandit, Suryanarayana Rao
Department of Neurology, Kasturba Hospital, Manipal, India
Department of Neurology, Kasturba Hospital, Manipal 576 119
Source of Support: None, Conflict of Interest: None
Twelve patients who satisfied the clinical criteria of Tolosa-Hunt syndrome (THS), underwent axial computerised tomography (CT) scan of the head. Two patients had abnormalities detected in the cavernous sinus on the affected side which supported the clinical diagnosis. This coupled with steroid responsiveness and long-term asymptomatic follow-up firmly established the diagnosis of THS. We discuss the computerised tomographic findings seen in THS and the relevance of using accurate imaging techniques as the first line of investigation in these cases
Keywords: Tolosa-Hunt syndrome - Painful ophthalmoplegia - Computerised tomography
|How to cite this article:|
Pandit L, Rao S. Computerised tomography in Tolosa-Hunt syndrome. Indian J Ophthalmol 1994;42:207-9
Tolosa-Hunt syndrome (THS) consists of painful ophthalmoplegia caused by steroid responsive, nonspecific inflammation in the region of the cavernous sinus. Tolosa  in 1954 first described the angiographic and postmortem findings in a patient who presented with severe retro-orbital pain followed by paralysis of ipsilateral third, fourth, sixth cranial nerves and diminished corneal reflex. Hunt  in 1961 described an additional 6 cases and laid down the clinical criteria for diagnosis of THS. They are: (1) steady gnawing retro-orbital pain, (2) defects in cranial nerves third, fourth, sixth or first branch of fifth cranial nerve with less common involvement of optic nerve or sympathetic fibres around the cavernous carotid artery, (3) symptoms lasting days to weeks, (4) occasional spontaneous remission, (5) recurrent attacks, and (6) prompt response to steroid therapy.
Prior to the advent of computerised tomography (CT) scan, orbital venography and angiography were used for imaging of the middle fossa in such patients which yielded inconsistent and nonspecific findings. The cavernous sinus by virtue of its location is not easily accessible to biopsy. Thus, the diagnosis of THS was one of exclusion till high resolution CT scans and MRI came into the picture. CT scan was initially thought to be insensitive to THS findings. However, modern high resolution CT has been able to demonstrate soft tissue abnormalities in the cavernous sinus and since 1978 sporadic cases of positive CT studies have been reported .,
| Materials and methods|| |
In five years, between 1989 and 1993, 12 patients presented with THS. All patients underwent plain and contrast-enhanced axial CT scan of the head (Toshiba TCT 80). With the exception of 2 patients described below, all patients had normal CT scans. Response to steroid therapy accompanied by long-term followup confirmed the diagnosis of THS in these patients.
A 29-year-old male presented with acute onset of left frontal and retrobulbar pain of 3 weeks' duration accompanied by nausea. He had photophobia and excessive tearing from the affected eye. Ten days after onset of pain he developed progressive ptosis of the left eye accompanied by diplopia in the left upward gaze. He also had numbness of the left forehead. He had no preceding history of migraine, diabetes, weight loss, or fever. On examination, relevant neurological findings included normal visual acuity, visual fields, and colour vision. Fundus was normal. He had complete ptosis of the left eye with total ophthalmoplegia. There was diminished sensation in the distribution of the ophthalmic division of the fifth nerve. He was afebrile, not anaemic, and had no lymphadenopathy or organomegaly.
Investigations were as follows - Hb 13.8 gm %; PCV 41.5; total count: 9,600/cu mm; differential count: N56%, L40%, E4%; ESR 5 mm/hr; and normal peripheral smear. Blood VDRL and TPHA were negative. ELISA for detection of HIV infection was negative. LE cell, ANF, and Rh factor were negative in serum. Fasting blood sugar was 73 mg% and postprandial blood sugar was 101 mg%. Renal and liver functions were normal. Plain CT scan of the head was normal. Contrast scan showed an irregular enhancing lesion in the anterior part of the left cavernous sinus [Figure - 1]. X-ray chest was normal. Mantoux test was negative. CSF analysis showed 2cells/cu mm, protein 73 mg%, sugar 71 mgs (corresponding blood sugar was 102 mgms%). CSF smear and culture were negative for pyogenic, fungal and AFB cultures. India ink staining for Cryptococcus was negative.
The patient was started on prednisolone at dose of 1 mg/kg body weight. Within 24 hours headache had diminished significantly. At the end of 1 week, ptosis had recovered completely and left pupil became smaller in size and reactive to light. At the end of 3 weeks, he had only mild restriction of vertical eye movements on the affected side. Steroids were tapered and stopped over the next 3 weeks. He was totally asymptomatic at the end of 2 months on steroid therapy and remained so at follow-up, 6 months later.
A 20-year-old male presented with recent onset of right frontal and periorbital "boring" pain of 25 days' duration accompanied by nausea and photophobia. Ten days after onset of headache he developed progressive ptosis and diplopia in the right gaze. On examination, he had complete ptosis of the right eye with total external ophthalmoplegia. Other cranial nerves including vision were normal. General and other systemic examinations were normal. Investigations revealed - Hb 12.6 gm%; total count: 6,920/cu mm, differential count - N54%, L42%, E3%, M1%; ESR 50 mm/hr; and normal peripheral smear. Blood urea was 36 mg%, creatinine 0.8 mg%, fasting blood sugar 96 mg% and postprandial blood sugar 115 mgms%. Blood VDRL, TPHA, and ELISA for HIV infection were negative.
Collagen vascular workup was negative. Mantoux test was negative. CT scan of the head, following contrast administration showed an enlarged and densely enhancing right cavernous sinus [Figure - 2]. Plain X-ray of the chest was normal. CSF analysis showed: 2 cells/cu mm, sugar 69 mgms% (corresponding blood sugar was 105 mgms%), protein 89 mg%, smear and cultures for fungus, AFB and pyogenic organisms were negative.
He was started on steroids 1 mg/Kg body weight. He improved rapidly in neurological deficits and was totally asymptomatic 2 weeks following treatment. Steroids were tapered off within the next 10 days. He remained well at last follow-up, 1 year later.
| Discussion|| |
Tolosa-Hunt syndrome has been reported sparsely in the western literature. , However, there are many reports from Asia .,
The diagnostic criteria for THS has its limitations since painful ophthalmoplegia may be encountered in a host of other conditions like intracavernous carotid aneurysm, parasellar neoplasms, infections, polyarteritis nodosa, and sarcoidosis. Also, steroid responsiveness is not without its pitfalls. Other lesions like giant cell tumours, lymphoma, chordoma, aneurysms, and nasopharyngeal carcinoma show steroid responsiveness. Earlier, orbital venography was the popular choice of investigation. It revealed variable and inconsistent findings which included occlusion of the superior ophthalmic vein, collateral venous flow through small venous channels or collateral veins and poor opacification of the ipsilateral cavernous sinus.
CT scan of the head was infrequently performed and was often reported to be normal in cases of THS. In recent times, it has been found that satisfactory imaging of the cavernous sinus by CT scan requires scrupulous attention to technique and standard axial scan of the head may be insufficient in detecting abnormalities. Narrow columnated contrastenhanced CT with reformatted images in the axial and sagittal planes has become a superior method of visualising the cavernous sinus. If tomographic studies of 5 mm are inadequate, overlapping 5 mm sections or non-overlapping 1.5 mm sections should be done.
Visualisation of the cavernous sinus requires large dose of contrast. Combining a rapid intravenous bolus followed by a continuous drip of contrast is best suited for this purpose. Using this technique it is possible- to diagnose and more importantly exclude other conditions that simulate THS.
Kwan et al have reported positive findings in all 5 patients in their series presenting with THS using these refined imaging techniques. Two of these patients had initial scans reported as normal when done elsewhere using standard methods. Repeat CT showed resolution in all these cases. Spector et al11 have clearly demonstrated the fallacy of using nonsophisticated CT imaging methods.
Various CT findings have been reported in THS. These findings include enlargement of the affected cavernous sinus, enhancing lesions in the cavernous sinus, orbital apex and pre-pontine cisterns, effacement of the Meckels cave and increased soft tissue in the orbital apex .,
Computerised tomographic findings in THS are not specific. However, it easily excludes a host of other differential diagnosis. Also, it avoids the necessity of invasive and less reliable investigations such as orbital venography and angiography. Though THS is considered a benign disorder, delay in diagnosis and initiation of steroids can lead to permanent neurologic sequelae including optic atrophy. Thus, it is imperative that the primary modality of investigation should be a high resolution scanning of the orbit and cavernous sinus region in all patients presenting with THS. CT is also invaluable in monitoring response to treatment and it has often been stressed to repeat the CT scan after completion of steroid therapy ,,
Two of the 12 patients in our series showed abnormalities on low resolution CT of the head. We believe that the yield would certainly have been higher if all the patients had been scanned paying careful attention to technique.
The diagnostic criteria for THS prior to the advent of CT scan requires revision. Besides the clinical criteria, it should include CT/MRI findings of THS and reversibility of abnormalities on posttreatment scans. In conclusion, we believe that THS by definition is an ambiguous entity not encompassed by clear boundaries or strict criteria. It is essentially a diagnosis of exclusion and computerised tomography helps to do so.
| References|| |
Tolosa E. Periarteric lesions of the cavernous syphon with clinical features of carotid infra clinoid aneurysms. J Neurosurg Psychiatry 17:300-302, 1954.
Hunt WE, Meaghes JN, Lefever HE, et al. Painful ophthalmoplegia: Its relation to indolent inflammation of the cavernous sinus. Neurology 11:56-62, 1961.
Neigel JM, Rootman J, Robinson RG, et al. The TolosaHunt Syndrome: Computed tomographic changes and reversal after steroid therapy. Can J Ophthalmol 21:287290, 1986.
Kwan ESK, Wolpert SM, Hedges TR. III, et al. TolosaHunt Syndrome revisited: Not necessarily a diagnosis of exclusion. AJR 150:413-418, 1988.
Juncos JL, Beal MF. Idiopathic cranial polyneuropathy - a fifteen year experience. Brain 110:197-211, 1987.
Thomas JE, Yoss RE. The parasellar syndrome: Problems in determining aetiology. Mayo Clin Proc. 45:617 623, 1978.
Mathew NT, Chandy J. Painful Ophthalmoplegia. J Neurol Sci 11:243-256, 1970.
Krishna AG, Mekhri MB. Painful Ophthalmoplegia: A clinical analysis of 180 cases. Neurol India 21:518-520, 1973.
Kline LB, Chandra-Sekhar B. Pitfalls in computed tomographic evaluation of the cavernous sinus. Surv Ophthalmol 29:293-296, 1985.
Kline LB, Acker JD, Post MJD, et al. The cavernous sinus: a computed tomographic study. 89:374-385, 1982.
Spector RH, Fiandaca MS. The sinister Tolosa-Hunt syndrome. 36:198-203, 1986.
Thomas DJB, Charlesworth MC, Afshar F, et al. Computerised axial tomography and magnetic resonance scanning in the Tolosa-Hunt syndrome. Br J Ophthalmol 72:299-302, 1988.
[Figure - 1], [Figure - 2]