The Hidden Trap: Tolosa–Hunt Syndrome Masking Entrapped Great Occipital Nerve—A Case of Headache Surgery

Summary:

Headache syndromes may coexist with peripheral nerve entrapment, which can obscure the true pain generator and delay effective treatment. Tolosa–Hunt syndrome is a rare, painful ophthalmoplegia syndrome characterized by unilateral orbital pain and cranial nerve palsy, often showing a marked response to corticosteroids. We report the case of a 56-year-old woman with a long history of migraine who developed recurrent right-sided orbital pain, maxillary pain, and ophthalmoplegia. She was diagnosed with Tolosa–Hunt syndrome by an experienced neurologist after skull magnetic resonance imaging and clinical evaluation. Because steroids and anti-inflammatory treatment produced only partial relief, and because she also had occipital pain with a positive Tinel sign over the greater occipital nerve, occipital neuralgia was suspected. A diagnostic block of the right greater occipital nerve provided partial improvement, after which right-sided surgical decompression of the nerve was performed. At 1-year follow-up, the patient had no residual surgical sensory deficit, no recurrent severe orbital or maxillary pain, and a marked reduction in attack frequency and intensity. Her Migraine Disability Assessment score improved from 18 to 4, and medication use decreased to sporadic use of simple analgesics and triptans. In selected patients, rare headache syndromes may coexist with occult peripheral nerve entrapment. Persistent symptoms despite appropriate medical treatment should prompt reconsideration of the diagnosis and targeted evaluation for occipital neuralgia.

Surgical decompression of entrapped peripheral cranial nerves has become an established option for carefully selected patients with refractory headache disorders.¹ The finest outcomes depend on accurate localization of the trigger site and complete decompression of the affected nerve, most commonly in the frontal, temporal, or occipital regions.^2,3

Occipital neuralgia can overlap clinically with migraine, tension-type headache, and cervicogenic headache, which makes diagnosis difficult and may delay treatment.^4,5 Positive Tinel testing over the occipital nerve and a response to a diagnostic nerve block are useful bedside clues supporting this diagnosis.⁶

Tolosa–Hunt syndrome is a rare diagnosis of exclusion characterized by unilateral orbital or periorbital pain associated with painful ophthalmoplegia due to inflammatory involvement of the cavernous sinus or superior orbital fissure.^7,8 It is classically steroid-responsive, but recurrence may occur, and steroid response alone is not sufficient to exclude other causes of pain.⁸

We present a rare case in which Tolosa–Hunt syndrome masked concomitant greater occipital nerve entrapment and describe the diagnostic reasoning that led to surgical decompression after incomplete medical response. The aim of this report is to highlight the importance of reevaluating persistent or atypical headache symptoms for coexisting peripheral nerve entrapment, even when a rare central diagnosis has already been established.

CASE DESCRIPTION

A 56-year-old woman with no significant systemic disease presented with long-standing recurrent headache. Her family history was notable for migraine in her father and a paternal cousin. Headache onset occurred at 10 years of age, with increasing frequency and severity over time. Based on laterality, duration, and recurrence, she was diagnosed with migraine and treated with paracetamol and nonsteroidal anti-inflammatory drugs. Due to increasing disability, candesartan, an angiotensin II receptor blocker used off-label for migraine prophylaxis, was initiated but discontinued due to fatigue and nausea. Tricyclic antidepressants were also poorly tolerated. She increasingly relied on triptans, serotonin receptor agonists used as abortive migraine therapy.

In addition to migraine-like headaches, she developed recurrent right-sided severe orbital pain with painful ophthalmoplegia and deep maxillary pain lasting several days, partially responsive to nonsteroidal anti-inflammatory drugs and corticosteroids. After clinical evaluation and skull magnetic resonance imaging, an experienced neurologist diagnosed Tolosa–Hunt syndrome, a rare idiopathic granulomatous inflammation of the cavernous sinus or superior orbital fissure typically responsive to corticosteroids but prone to recurrence.

Despite medical therapy, disabling pain persisted. She also reported occipital radiation of pain, and examination revealed a positive Tinel sign over the right greater occipital nerve. A diagnostic block with 1.5 mL of lidocaine resulted in partial symptom relief, supporting a peripheral neuralgic component. Based on these findings, right-sided decompression of the greater occipital nerve was performed. Surgery involved neurolysis and release of compressive soft tissues; no abnormal tissue was identified, and no specimen was obtained for histopathological analysis (Fig. 1).

Fig. 1.

Intraoperative view of the right greater occipital nerve and adjacent artery at the decompression site. The figure illustrates the anatomical structures released during the surgery.

The postoperative course was uneventful. At 1-year follow-up, there was no sensory deficit and no recurrence of orbital or maxillary pain. Migraine-like headaches persisted but were less frequent and less severe. Her Migraine Disability Assessment (MIDAS) score improved from 18 to 4, and she required only occasional symptomatic medication.

DISCUSSION

This case highlights the challenge of recognizing more than 1 headache disorder in the same patient. Tolosa–Hunt syndrome can dominate clinical attention due to its association with orbital pain and ophthalmoplegia.⁹ However, when symptoms are only partially explained, clinicians should remain alert for additional pain generators such as occipital neuralgia.

Steroid therapy is the first-line treatment for Tolosa–Hunt syndrome.^7,8 In this patient, only partial improvement weakened the assumption that Tolosa–Hunt syndrome alone accounted for all symptoms and supported reevaluation of the diagnosis. Current literature also emphasizes that steroid responsiveness does not prove the diagnosis by itself, because recurrence may occur and the underlying cause may remain unresolved.^7,8

The suspicion of occipital nerve entrapment in this patient was based on the combination of occipital pain, a positive Tinel sign, and partial response to a local anesthetic block. This approach is consistent with clinical descriptions of occipital neuralgia, in which percussion over the occipital nerve can reproduce radiating pain and a nerve block can help confirm the diagnosis.¹⁰

Additionally, surgical decompression was performed at the symptomatic site with release of the greater occipital nerve from the surrounding compressive tissues, which is intended to address the mechanical component of nerve irritation. Nevertheless, as with all decompression procedures, long-term recurrence cannot be completely excluded, and this should be acknowledged as a limitation of a single case report.

We did not obtain tissue for histopathologic analysis. This is a limitation, but it is not unusual in nerve decompression surgery, where the goal is functional release rather than excision of a lesion. The absence of a specimen prevents microscopic confirmation of the entrapment tissue, yet the clinical findings and postoperative improvement supported the diagnosis.

The generalizability of this report is limited because it describes only 1 patient. Even so, the case is instructive because it demonstrates how a rare syndrome such as Tolosa–Hunt may coexist with a separate peripheral neuralgia and how a partial steroid response should prompt reconsideration of the pain source. Future studies with larger patient series are needed to clarify how often occult nerve entrapment coexists with refractory headache syndromes.

CLINICAL IMPLICATIONS

This case supports structured reassessment of persistent headache symptoms. A prior diagnosis should not prevent reevaluation when additional pain topographies or focal findings emerge. Surgery may improve 1 component of a complex headache disorder even when another phenotype persists, which is important for patient counseling.

CONCLUSIONS

Tolosa–Hunt syndrome may coexist with greater occipital nerve entrapment and obscure a surgically treatable pain source. In patients with a partial steroid response and persistent occipital pain, targeted examination for occipital neuralgia should be considered.

DISCLOSURE

The authors have no financial interest to declare in relation to the content of this article.

PATIENT CONSENT

The patient provided written informed consent for publication of this case report.