Tolosa-Hunt syndrome: an arcane pathology of cavernous venous sinus

Abstract

Tolosa-Hunt syndrome, an idiopathic granulomatous inflammation of the cavernous sinus, is primarily a diagnosis of exclusion. The majority of patients present with unilateral orbital pain and features suggestive of paralysis of one or more of the cranial nerves passing through the cavernous sinus and/or superior orbital fissure. MRI of the head may show unilateral enhancement of the cavernous sinus and orbital apex. Treatment is with high-dose intravenous steroids followed by tapering oral steroids. Rapid amelioration of pain within 24–48 h supports this rare diagnosis. Resolution of neuropathies may take longer. We describe a case of a young man who presented with left periorbital pain, complete ophthalmoplaegia and ptosis of the left eye. MRI showed enhancement of the left cavernous sinus and orbital apex. High dose steroids led to complete resolution of pain, while ptosis and ophthalmoplaegia improved gradually.

Tolosa Hunt syndrome followed by facial nerve palsy

“…Rapid resolution of the pain within two to three days from starting the steroid therapy is a characteristic feature that can help to confirm the diagnosis 3,6 although other causes can respond to steroid initially, The improvement of cranial nerve palsy and radiological changes can take a longer time. 1,3 Few case reports described the rare association of extracavernous cranial nerve involvement in THS suggesting a more diffuse systemic inflammation. [7][8][9][10][11] A case series of 14 cases of THS mentioned the involvement of facial nerve in four cases.…”

“…2 This inflammation causes painful cranial nerve palsy involving all or some of the following nerves: the third, fourth or sixth cranial nerves, V1 and/or V2 divisions of the fifth cranial nerve. 3 Here we describe a case of THS that has atypical clinical presentation, in the form of extracavernous cranial nerve involvement.…”

Case Report: Tolosa-Hunt Syndrome Associated with a Chest Mass in a Pediatric Patient

“…The pathology report identified the presence of fibrosis and non-specific inflammation, but did not show any signs of malignancy and was unable to give a clear diagnosis of the mass ( Figure 3A and Figure 3B). A lymphoma profile of the sample was also negative for MUM1, BCL2 and 6, CD3, 4,5,8,15,19,20,30,43,45, and 79a, denying the presence of abnormal T and B lymphocytes.…”

“…Evidence of causation demonstrated by a headache also must have preceded paresis of the third, fourth, and/or sixth nerves by less than 2 weeks, or developed with it, along with the headache localizing around the ipsilateral brow and eye [1,2]. Since a headache and paresis can be indicative of other, more common diseases, it is especially critical to rule out all other possible diagnoses that may present with similar symptoms which include, but are not limited to anisocoria, diabetic neuropathy, Lyme disease, meningioma, venous thrombosis, skull tumors, Systemic Lupus Erythematosus, and Lymphoma [3,4].…”

Tolosa-Hunt Syndrome, the Hunt for One in a Million

“…We present a case of Tolosa-Hunt syndrome in an 18year-old woman. The syndrome is characterized by a severe unilateral periorbital headache, pain with eye movement, double vision and sensory loss in the V1 branch of the trigeminal nerve which traverses the cavernous sinus where there is granulomatous inflammation [2]. While the syndrome is usually a temporally limited condition, relapses may occur requiring extended periods of immunosuppressive therapy.…”