Face / Head / Neck

Tolosa-Hunt Syndrome

Definition

Tolosa-Hunt syndrome is defined by a peri-orbital or unilateral headache combined with ipsilateral oculomotor nerve deficit, oculo-sympathetic paralysis, and loss of sensibility in the area of the first and second branch of the trigeminal nerve.

Depending on the involvement of a process near the orbital fissure and/or cavernous sinus, other cranial nerves, such as the optic nerve, may also be affected.

Aetiology

The cause is frequently is located in the cavernous sinus or in the orbital fissure and may be traumatic, vascular (aneurysm), inflammatory or neoplastic in origin.

Signs and symptoms

Patients usually complain of a piercing pain around the eye, which may later be followed by ophthalmoplegia. This pain may spread to the frontal and temporal regions. As well as loss of the third cranial nerve, the fourth, sixth, and first division of the trigeminal nerve may also be part of the clinical neurological picture.

Symptoms may last from days to weeks, with the occurrence of spontaneous remission. A permanent neurological deficit is usually left behind. Attacks may recur at intervals ranging from months to years.

Diagnostics

Physical Examination

Extensive general physical and neurological examination, with emphasis on the cranial nerves, is always indicated.

Additional Somatic Diagnostics

Additional examinations should include blood tests, CSF examination, neuroradiological (CT and MRI scans, cerebral angiography) and, when indicated, biopsy of the nasopharynx and cavernous sinus.

Somatic Treatment

Treatment depends on the primary aetiology of Tolosa-Hunt syndrome. Long-term corticosteroid therapy is usually indicated.

Pharmacological treatment:

  • Tricyclic antidepressants
  • Anticonvulsives: carbamazepine, oxcarbazepine, gabapentine en pregabaline.