Tolosa-Hunt syndrome—a rare cause of headache and ophthalmoplegia

A 54-year-old African American woman came to our hospital with a 1-week history of severe right-sided retroorbital pain and blurry vision. 2 days after onset of unilateral throbbing eye pain she developed intermittent blurry vision, which was worse with rightward gaze. She had no facial droop or language impairment, and no previous episodes of headache or visual changes. She had no history of orbital trauma. Her only previous medical diagnoses were schizophrenia and depression. She was taking aripiprazole and escitalopram daily. On admission she had normal vital signs. Neurological examination showed a right-sided abduction defi cit that was consistent with sixth-nerve palsy (fi gure) and impaired facial sensation on the right in the distribution of trigeminal nerves V1 and V2. Visual acuity was 20/50 in both eyes, which was unchanged from baseline, with no papilloedema seen. Strength, sensation, and cerebellar function were intact. Complete blood count, basic metabolic panel, and coagulation studies were normal. CT of the head showed no evidence of infarction, haemorrhage, or mass. Lumbar puncture was done with normal opening pressure and no white blood cells. Cerebrospinal fl uid analysis showed no oligo clonal bands. Angiotensin con verting enzyme inhibitor and tests for venereal disease were negative. Autoimmune workup showed antinuclear antibody titre of 1 in 64, negative double stranded DNA, and negative anti-Smith antibodies. Antinuclear cyto plasmic antigen was negative. Chest radiography was normal. Plain MRI and MRI angiography showed normal optic nerves, but revealed asymmetric dural enhancement along the lateral aspect of the right cavernous sinus wall, which was refl ective of an underlying infl am matory process (appendix). The patient was started on a trial of prednisone 80 mg orally daily on day 3 of hospital isation and showed good improvement in symptoms within 48 h. Her cranial nerve defi cits were believed to be secondary to infl ammation of the cavernous sinus aff ecting the nerves along the lateral cavernous sinus wall (appen dix), with a presumed diag nosis of Tolosa-Hunt syndrome. International Headache Classifi cation defi nes TolosaHunt syndrome as episodic orbital pain associated with paralysis of one or more of the third, fourth, and sixth cranial nerves, which usually resolves spontaneously but tends to relapse and remit. Unilateral retro-orbital pain and ophthalmoplegia are secondary to granulomatous (and occasionally non-granulomatous) material in the caver nous sinus, supraorbital fi ssures, or orbit in some biopsied cases. Tolosa-Hunt syndrome is extremely rare, and may present at any age with estimated incidence of one per million. Diagnostic criteria as determined by the International Headache Society in 2004 are: one or more episodes of unilateral orbital pain persisting for weeks if untreated; paresis of one or more of the third, fourth, and sixth cranial nerves, with or without demon stration of granuloma by MRI or biopsy; paresis that coincides with the onset of pain or follows it within 2 weeks; pain and paresis resolve within 72 h when treated with corticosteroids; and other causes excluded by appropriate investigations. The diff erential diagnosis for painful opthal moplegia is broad and includes orbital infl ammatory syndromes, neoplasms, vascular anomalies, and autoimmune disease. Defi nitive diagnosis via biopsy is reserved for severe cases because of the high risks associated with surgery. Treatment consists of high dose oral prednisone tapered over 4 weeks. Noticeable improvement is often evident within the fi rst 24 h of treatment. Failure to respond to steroids suggests an alternative diagnosis. Repeat imaging should be done every 1–2 months until there is resolution of the imaging abnormalities. Patients should be told that relapses can occur and long term steroid treatment may be required. 1 month after discharge our patient had a follow-up MRI, which showed resolution of the previous cavernous sinus enhancement (appendix). She reported no further headaches and had normal extraocular movements. Although Tolosa-Hunt syndrome is a rare diagnosis, unilateral pain and cranial nerve fi ndings without an alternative explanation should prompt consideration of the syndrome.