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13. CRANIAL NEURALGIAS AND CENTRAL CAUSES OF FACIAL PAIN 13.1. Trigeminal neuralgia 13.2. Glossopharyngeal neuralgia 13.3. Nervus intermedius neuralgia [G51.80] 13.4. Superior laryngeal neuralgia [G52.20] 13.5. Nasociliary neuralgia [G52.80] 13.6. Supraorbital neuralgia [G52.80] 13.7. Other terminal branch neuralgias [G52.80] 13.8. Occipital neuralgia [G52.80] 13.9. Neck-tongue syndrome 13.10. External compression headache 13.11. Cold-stimulus headache 13.12. Constant pain caused by compression, irritation or distortion of cranial nerves or upper cervical roots by structural lesions [G53.8] + [code to specify aetiology] 13.13. Optic neuritis [H46]  13.14. Ocular diabetic neuropathy [E10-E14]  13.15. Head or facial pain attributed to herpes zoster 13.16. Tolosa-Hunt syndrome 13.17. Ophthalmoplegic “migraine” 13.18. Central causes of facial pain 13.19. Other cranial neuralgia or other centrally-mediated facial pain [code to specify aetiology] Bibliography and reference14. OTHER HEADACHE, CRANIAL NEURALGIA, CENTRAL OR PRIMARY FACIAL PAIN 

IHS	Diagnosis	ICD-10
13.16	Tolosa-Hunt syndrome	G44.850

Description:

Episodic orbital pain associated with paralysis of one or more of the third, fourth and/or sixth cranial nerves which usually resolves spontaneously but tends to relapse and remit.

Diagnostic criteria:

One or more episodes of unilateral orbital pain persisting for weeks if untreated
Paresis of one or more of the third, fourth and/or sixth cranial nerves and/or demonstration of granuloma by MRI or biopsy
Paresis coincides with the onset of pain or follows it within 2 weeks
Pain and paresis resolve within 72 hours when treated adequately with corticosteroids
Other causes have been excluded by appropriate investigations¹

Note:

Other causes of painful ophthalmoplegia include tumours, vasculitis, basal meningitis, sarcoid, diabetes mellitus and ophthalmoplegic "migraine".

Comments:

Some reported cases of Tolosa-Hunt syndrome had additional involvement of the trigeminal nerve (commonly the first division) or optic, facial or acoustic nerves. Sympathetic innervation of the pupil is occasionally affected.

The syndrome has been caused by granulomatous material in the cavernous sinus, superior orbital fissure or orbit in some biopsied cases.

Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia.

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