Tolosa.html


    
        
            
                
                    

                

            

            
                Tolosa-Hunt syndrome

                Ben Barnard, MB ChB
            

            Delme Hurter, MB ChB, MMed (D Rad)

            Department of Radiology, Kimberley Hospital Complex, Kimberley

            Francois Roux, MB ChB, DA (SA)
        

        Tygerberg Academic Hospital, University of Stellenbosch, Tygerberg

        Shaheer Aboobaker, MB ChB, Dip Opth (SA)
    

    Department of Ophthalmology, Kimberley Hospital Complex, Kimberley

    
        Corresponding author: B Barnard (benwbarnard@gmail.com)

        Introduction

        Tolosa-Hunt syndrome (THS) is a rare disorder
        indicated by recurrent painful ophthalmoplegia caused by non-specific
        inflammation of the cavernous sinus or superior orbital fissure (SOF).
        The disease shares histopathological features with idiopathic orbital
        pseudotumour; however, owing to its anatomical location, it produces
        characteristic clinical manifestations.1 
        Recurrent retro-orbital pain, with palsies of the third, fourth or
        sixth cranial nerves as well as the first and second divisions of the
        trigeminal nerve, are typical. Clinically, immediate response to
        steroid therapy is a hallmark of the condition.

        The clinical presentation of THS has a wide
        differential diagnosis, and timely and appropriate imaging – as
        an adjunct to pertinent laboratory investigations – can greatly
        assist clinicians with early accurate diagnosis and management.

        Case report

        Our patient was a 17-year-old girl who presented with a 2-week history of
        a sharp peri-ocular headache and drooping of the left eyelid. She also
        complained of a decrease in eye movement as well as visual acuity. She
        indicated that she had had similar episodes of headache preceded by
        blurring of vision in the same eye for more than a year. She was
        previously healthy.

        On clinical examination, her vitals were
        normal. She had a left-sided ptosis and impairment of adduction and
        elevation of the left eye, consistent with an oculomotor (IIIrd) nerve
        palsy. The left pupil was mildly dilated but responsive to light. No
        facial sensory or motor loss in the distribution of the trigeminal
        (Vth) nerve was detected. Fundoscopy was normal.

        Bloodwork and lumbar puncture (LP) were
        non-specific. Of note, the white cell count (WCC) was mildly elevated
        and the erythrocyte sedimentation rate (ESR) significantly increased.
        She tested retroviral negative.

        The patient was referred for an urgent brain
        CT; this showed a diffusely thickened left optic nerve with associated
        dilatation of the left superior ophthalmic vein (SOV). In addition, a
        soft-tissue mass was noted in the SOF, extending into the left
        cavernous sinus. An MRI of the brain and orbits was then performed on a
        1.5T Toshiba Vantage machine. Whole-brain FLAIR and T2W imaging was
        done, followed by thin-slice (2.5 mm slice thickness) coronal, axial
        and sagittal CE (Magnevist) FS T1W sequences through the cavernous
        sinus and orbits. This demonstrated an avidly enhancing mass lesion in
        the orbital apex extending into the left SOF, causing compression on
        the neurovascular elements. The lesion extended into the cavernous
        sinus. The diffusely thickened optic nerve was secondary to oedema but
        was not infiltrated. There was no luminal narrowing of the
        intracavernous segment of the internal carotid artery (ICA), and no
        evidence of cavernous sinus or SOV thrombosis. The rest of the study
        was non-contributory.

        The patient was treated with oral steroids
        and showed significant relief of symptoms over the following 48 hours.
        She had complete resolution of the ptosis as well as the oculomotor
        nerve palsy within one month. Further follow-up showed no recurrence of
        symptoms.

        Discussion

        Tolosa first described the condition in 1954, in a
        patient with unilateral recurrent painful ophthalmoplegia involving
        cranial nerves III, IV, VI and V1. The patient was imaged using carotid angiography, and segmental narrowing of the carotid siphon was seen.1
    

    Hunt et al.
    described 6 patients with similar clinical findings in 1961, and
    proposed a low-grade non-specific inflammation of the cavernous sinus
    and its walls as the cause of the syndrome. Pathologically,
    infiltration of lymphocytes and plasma cells as well as thickening of
    the dura mater was seen.1  The condition was termed Tolosa-Hunt syndrome by Smith and Taxdal in 1966.2  The latter authors stressed the importance of the dramatic rapid response to steroid therapy.

    In 1988, THS criteria were provided by the International Headache Society (IHS), and further revised in 2004 (Table I).3
    ,
    4





Neuro-imaging – in particular MRI
– is an essential part of the workup of any patient presenting
with features of THS, as these features are non-specific and have a
wide differential diagnosis, including meningioma, sarcoidosis,
pituitary tumours, tuberculous meningitis (TBM) and lymphoma.2


MRI findings classically demonstrate a
soft-tissue mass lesion involving the SOF or cavernous sinus. Signal
characteristics are typically hypointense to fat and isointense to
muscle on short TR/TE sequences and isointense to fat on long TR/TE
sequences.5 
Significant enhancement of the mass lesion is demonstrated on CE
sequences. Of particular value is the post-contrast fat-saturated
thin-slice coronal images through the orbital apex and cavernous sinus.

THS essentially remains a diagnosis of
exclusion. The role of the radiologist is to exclude other conditions
causing similar clinical features. Distinctive MRI findings and rapid
resolution of clinical symptoms with steroid therapy are characteristic.




1.	Som PD, Curtin HD. Head and Neck Imaging. 4th ed. St Louis: Mosby, 2003:587-591.

1.	Som PD, Curtin HD. Head and Neck Imaging. 4th ed. St Louis: Mosby, 2003:587-591.

2. Sathyanathan BP, Rajasundaram R, Sankaravadivelu ST, Nadhamuni K. A
case of Tolosa-Hunt Syndrome – MR imaging appearance. Ind J
Radiol Imag 2006;16(1):97-98.

2. Sathyanathan BP, Rajasundaram R, Sankaravadivelu ST, Nadhamuni K. A
case of Tolosa-Hunt Syndrome – MR imaging appearance. Ind J
Radiol Imag 2006;16(1):97-98.

3. Mendez JA, Arias CR, Sanchez D, et al. Painful ophthalomoplegia of
the left eye in a 19-year-old female, with an emphasis on Tolosa-Hunt
syndrome: a case report. Cases Journal 2009;2:8271.

3. Mendez JA, Arias CR, Sanchez D, et al. Painful ophthalomoplegia of
the left eye in a 19-year-old female, with an emphasis on Tolosa-Hunt
syndrome: a case report. Cases Journal 2009;2:8271.

4. Mora-de-Onate J, Pascual-Perez-Alfaro R, Izquierdo-Vazquez C,
Gonzalez-Ruiz M, Aguirrebena-Olmos A, Diez-Villalba R. Painful
ophthalmoplegia (pseudotumour of the orbit and Tolosa-Hunt Syndrome).
Arch Soc Esp Oftalmol 2007;82:509-512.

4. Mora-de-Onate J, Pascual-Perez-Alfaro R, Izquierdo-Vazquez C,
Gonzalez-Ruiz M, Aguirrebena-Olmos A, Diez-Villalba R. Painful
ophthalmoplegia (pseudotumour of the orbit and Tolosa-Hunt Syndrome).
Arch Soc Esp Oftalmol 2007;82:509-512.

5. Yousem DM, Atlas SW, Grossman RI, Sergott RC, Savino PJ, Bosley TM.
MR imaging of Tolosa-Hunt Syndrome. AJNR 1990;154:167-170.

5. Yousem DM, Atlas SW, Grossman RI, Sergott RC, Savino PJ, Bosley TM.
MR imaging of Tolosa-Hunt Syndrome. AJNR 1990;154:167-170.

6. Dornam TL, Espir MLE, Gale EAM, Tattersall RB, Worthington BS.
Remittent painful ophthalmoplegia: the Tolosa-Hunt syndrome? J Neurol
Neurosurg Psychiatry 1979;42:270-275.

6. Dornam TL, Espir MLE, Gale EAM, Tattersall RB, Worthington BS.
Remittent painful ophthalmoplegia: the Tolosa-Hunt syndrome? J Neurol
Neurosurg Psychiatry 1979;42:270-275.




    
        
    

    
        Fig. 1. Axial CT brain demonstrates a soft-tissue
        mass in the left superior orbital fissure. Note also the thickened
        optic nerve.

    




    
        
    

    
        Fig. 2. Axial CE FS T1W image reveals intense
        enhancement of the soft-tissue mass within the left SOF. The lesion
        extends into the cavernous sinus.

    




    
        

    




    



    Fig. 3. Coronal CE FS T1W image again shows the intensely enhancing soft-tissue mass.




    




    



    Fig. 4. Axial CE FS T1W follow-up image 4 months later demonstrates complete resolution of the enhancing soft-tissue mass.




    



    Fig. 5. Coronal CE FS T1W follow-up image again shows the resolution of the mass.

    
    

    	
                Table I. THS diagnostic criteria

            
	
                A

            	
                One or more episodes of unilateral orbital pain persisting for weeks if untreated

            
	
                B

            	
                Paresis of one or more of the third, fourth and/or sixth cranial nerves and/or demonstration of granulomas by MRI or biopsy

            
	
                C

            	
                Paresis coincides with the onset of pain or follows it within 2 weeks

            
	
                D

            	
                Pain and paresis resolve within 72 hours when treated adequately with corticosteroids

            
	
                E

            	
                Other causes have been excluded by appropriate investigations