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Tolosa-Hunt syndrome (THS)

Tolosa-Hunt Syndrome (THS) is a rare disorder characterized by painful ophthalmoplegia, which is the combination of severe eye pain and weakness or paralysis of the muscles that control eye movement. It is caused by inflammation in the cavernous sinus or superior orbital fissure, areas located near the base of the skull. The syndrome is typically idiopathic, meaning its exact cause is unknown, although it is believed to be related to a nonspecific inflammatory process

Tolosa-Hunt Syndrome (THS) is a rare disorder characterized by painful ophthalmoplegia, which is the combination of severe eye pain and weakness or paralysis of the muscles that control eye movement. It is caused by inflammation in the cavernous sinus or superior orbital fissure, areas located near the base of the skull. The syndrome is typically idiopathic, meaning its exact cause is unknown, although it is believed to be related to a nonspecific inflammatory process.

Key Features

  1. Eye Pain: Severe, deep, and sharp orbital or periorbital pain, often unilateral.
  2. Ophthalmoplegia: Weakness or paralysis of one or more cranial nerves that control eye movements, including the third (oculomotor), fourth (trochlear), and sixth (abducens) cranial nerves.
  3. Visual Symptoms: Diplopia (double vision) is common due to misalignment of the eyes caused by cranial nerve palsy.
  4. Other Neurological Deficits: May include ptosis (drooping eyelid) or sensory loss in the distribution of the ophthalmic branch of the trigeminal nerve.

Diagnostic Criteria

The International Headache Society outlines the following criteria:

  • Unilateral orbital pain persisting for weeks if untreated.
  • Paired with paresis of cranial nerves responsible for eye movements (III, IV, VI), with or without involvement of other cranial nerves.
  • Symptoms improve with corticosteroid treatment.
  • Exclusion of other causes via imaging (e.g., MRI, CT scans).

Causes

The exact cause of THS is unknown. It is considered idiopathic but is linked to granulomatous inflammation in the affected areas (cavernous sinus, superior orbital fissure, or orbital apex).

Diagnosis

  • Imaging: MRI with gadolinium contrast is the preferred imaging modality, often showing inflammatory thickening in the cavernous sinus or orbital apex.
  • CSF Analysis: Lumbar puncture may be done to rule out other conditions like infections or malignancy.
  • Exclusion of Other Causes: Differential diagnosis includes tumors, infections (e.g., fungal or bacterial), aneurysms, and vasculitis.

Treatment

  • Corticosteroids: High-dose corticosteroids are the primary treatment and usually result in a rapid improvement of symptoms.
  • Immunosuppressive Therapy: In cases where corticosteroids are insufficient or for long-term management, immunosuppressive agents may be used.
  • Pain Management: Analgesics for symptomatic relief of pain.

Prognosis

With appropriate treatment, the prognosis is generally good, and symptoms often resolve. However, recurrences are possible, and long-term follow-up may be required.

Complications

  • Persistent cranial nerve deficits in some cases.
  • Risk of recurrence.
  • Side effects related to long-term corticosteroid use.

If you suspect THS or experience its symptoms, seeking prompt evaluation by a neurologist or ophthalmologist is crucial.