Tolosa–Hunt syndrome

Tolosa-hunt ophtalmoplegia

Tolosa–Hunt syndrome (THS) is a rare neurological disorder characterized by severe headache and ophthalmoplegia (paralysis or weakness of the eye muscles). The syndrome is named after Eduardo Tolosa and William Hunt, who first described the condition in 1954 and 1961, respectively.

Presentation

Patients with Tolosa–Hunt syndrome typically present with a sudden onset of severe, unilateral periorbital pain that may be accompanied by cranial nerve palsies. The pain is often described as sharp or stabbing and can be debilitating. The affected cranial nerves usually include the oculomotor nerve (CN III), trochlear nerve (CN IV), and/or the abducens nerve (CN VI), leading to symptoms such as ptosis, diplopia, and restricted eye movements.

Pathophysiology

The exact cause of Tolosa–Hunt syndrome is not well understood, but it is believed to involve an inflammatory process in the cavernous sinus or the superior orbital fissure. This inflammation can lead to compression and damage of the cranial nerves passing through these structures.

Diagnosis

The diagnosis of Tolosa–Hunt syndrome is primarily clinical, supported by imaging studies such as magnetic resonance imaging (MRI) or computed tomography (CT) scans. These imaging modalities may show signs of inflammation in the cavernous sinus or superior orbital fissure. Lumbar puncture and blood tests are often performed to rule out other causes of similar symptoms, such as infection, neoplasm, or autoimmune disease.

Treatment

The mainstay of treatment for Tolosa–Hunt syndrome is high-dose corticosteroids, which can lead to rapid improvement in symptoms. The typical course of treatment involves an initial high dose followed by a gradual tapering over several weeks. In some cases, other immunosuppressive agents may be used if corticosteroids are not effective or if the patient has contraindications to their use.

Prognosis

The prognosis for patients with Tolosa–Hunt syndrome is generally good, with most patients experiencing significant relief of symptoms with appropriate treatment. However, recurrences can occur, and some patients may have residual deficits.

Related Pages

• Cranial nerve
• Ophthalmoplegia
• Cavernous sinus
• Corticosteroids
• Magnetic resonance imaging
• Computed tomography