Diagnostic criteria
- Unilateral orbital or periorbital headache fulfilling criterion C
- Both of the following:
- granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit, demonstrated by MRI or biopsy
- paresis of one or more of the ipsilateral IIIrd, IVth and/or VIth cranial nerves
- Evidence of causation demonstrated by both of the following:
- headache is ipsilateral to the granulomatous inflammation
- headache has preceded paresis of the IIIrd, IVth and/or VIth nerves by ≤2 weeks, or developed with it
- Not better accounted for by another ICHD-3 diagnosis.
Numbers
- Any age from the 1st to the 8th decades of life
- No sex predilection.
- Either side may be affected, with case reports of bilateral simultaneous involvement.
Clinical features
- Painful ophthalmoplegia.
- Pain
- Defining symptom.
- Lasts an average of 8 weeks if untreated.
- Ocular motor cranial nerve palsies may coincide with the onset of pain or follow it within a period of up to 2 weeks.
- It is usually described as “intense”, “severe”, “boring”, “lancinating”, or “stabbing”. It is periorbital in location, frequently extending into the retro-orbital, frontal, and temporal regions.
- CN3-6 may be involved, in various combinations.
- Pupillary reactions
- most of the time is normal,
- If abnormal can be either
- parasympathetic (oculomotor nerve) dysfunction OR
- sympathetic (third order neuron Horner's syndrome) dysfunction
- If CN2 affected then orbital apex may be involved
- Optic disc may be normal, swollen, or pale in appearance, and visual decline may be minimal or lead to blindness.
- Loss of acuity is variable
- Infrequently, other cranial nerves not located within the cavernous sinus/superior orbital fissure may be affected. These include the maxillary and mandibular branch of the trigeminal nerve, and the facial nerve.
- There are no reports of systemic or other neurological involvement in Tolosa-Hunt syndrome, with the exception of nausea and vomiting. These symptoms are probably due to the intense periorbital pain; they promptly resolve with cessation of pain.
Natural history
- unpredictable course.
- Acute at onset, the patient's symptoms typically last from days to weeks. B
- Recurrences are common, taking place in about a half of reported patients, usually at an interval of months or years from the initial attack.
- These recurrences may be ipsilateral, contralateral, or rarely, bilateral.
- A self limited illness, it does cause considerable morbidity. Infrequently, residual cranial nerve palsies persist. With the institution of corticosteroid therapy, the natural course is altered.
Pathology
- An idiopathic inflammatory process.
- Granulomatous inflammation, with epithelioid cells and occasional giant cells.
- Necrosis may also be seen.
- Not associated with infectious organism
- of the cavernous sinus and orbital apex, with thickening of the sinus dura mater.
Mechanism
- Unknown cause
- No information is available as to what triggers the inflammatory process
- Inflammation of the
- Superior orbital fissure
- Cavernous sinus.
Imaging
- MRI findings (may show evidence of inflammatory changes in the anterior cavernous sinus, superior orbital fissure and orbital apex):
- T1: Iso to hypo intense.
- T2: Hyperintense (hypointense if fibrous tissue substitution).
- Postcontrast T1: Asymmetrical enhancement.
- Narrowing of the cavernous ICA may be present.
- Specific case findings (as depicted in the images):
- Postcontrast axial T1 + 6 (A) and coronal T1 (B) T2 (A) show an asymmetrical enhancing enlargement of the left cavernous sinus (red circle).
- There is reduced T2 signal due to fibrous tissue proliferation (C, red arrow).
- The left ICA was occluded, which is noted by the flow void loss in the coronal sequences (B, C, yellow arrows).
- Confirmed occlusion on 3D TOF-MRA (D).
Treatment
- Steroids
- Although there is no conclusive evidence to show that steroids lessen the degree or duration of the ophthalmoplegia, there is dramatic reduction of pain, often within 24 hours.
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