Review of Tolosa-Hunt Syndrome, Recent Updates.

PURPOSE OF REVIEW: We aim to present diagnostic considerations and management insights on Tolosa-Hunt syndrome. We highlight recent controversies in this field and emerging literature. RECENT FINDINGS: The diagnostic categorization of Tolosa-Hunt syndrome remains controversial, with imaging negative cases described in the literature and an increasing literature of secondary causes falsely diagnosed as Tolosa-Hunt syndrome. Response to steroids can fulfill diagnostic criteria, but newer management strategies are available in treatment-resistant patients, such as steroid-sparing agents or radiotherapy. Tolosa-Hunt syndrome has become controversial; the entity of granulomatous inflammation of the cavernous sinus and possible extension into orbital fissure and posterior orbit of late has been re-evaluated. Recent case series and reviews have outlined multiple false positive (neoplasm or infection) and negative (no imaging findings) diagnoses. In the future, when assessing whether a patient has this entity, we caution readers to closely follow patients for secondary causes and consider this entity in differential even if MRI is non-revealing. Additionally, biopsy should be regarded as a gold standard for diagnosis and utilized, especially in uncertain cases. The diagnostic categorization of Tolosa-Hunt syndrome may need reconsideration in future versions of the ICHD.

Orbital inflammation following COVID-19 vaccination: A case series and literature review.

PURPOSE: The purpose of the study was to report three cases of orbital inflammation following administration of the COVID-19 vaccination, manifesting as Tolosa-Hunt syndrome (THS) and orbital myositis. METHOD: A retrospective case series and literature review of patients who developed orbital inflammation following a COVID-19 vaccination. RESULTS: One patient presented with Tolosa-Hunt syndrome (THS) 14 days following her third (booster) COVID-19 vaccination, one patient developed orbital myositis 10 days following his first COVID-19 vaccination and one patient developed recurrent orbital myositis 1 and 7 days following her second and fourth COVID-19 vaccination. All patients received the Comirnaty vaccine (Pfizer-BioNTech). A thorough systemic autoimmune disease workup in both patients was unremarkable. Two patients had a prior history of orbital inflammation, with previous involvement of other different orbital structures. Characteristic MRI features for each pathology were observed, supporting the clinical presentation of THS and orbital myositis. There was complete resolution of THS following corticosteroids, with no recurrence at 2 months. Meanwhile, one case of orbital myositis self-resolved at 2 months without use of systemic corticosteroids, while the other patient with orbital myositis required treatment with intra-orbital steroid injections and oral corticosteroids. CONCLUSION: Orbital inflammation has been recognised as a rare adverse effect following COVID-19 vaccination. We present a case series of THS and orbital myositis as varied presentations of this entity.

A 57-year-old man with painful ophthalmoplegia and cavernous sinus involvement: Why this is not Tolosa-Hunt syndrome.

Tolosa-Hunt syndrome (THS) is an idiopathic condition included in the differential diagnosis of painful ophthalmoplegia. Although this was once a common diagnosis, the increasing availability of tests reveals an alternative etiology in many cases. Exclusion of treatable disorders is important, because the prognosis may otherwise be poor. We here describe a patient who presented with painful ophthalmoplegia with an infiltrating lesion in the cavernous sinus. Initially suspected of THS, he had a fatal evolution, and postmortem evaluation revealed cervicocephalic actinomycosis. Actinomycosis diagnosis is often missed, and still represents a challenge to the clinician. We highlight pearls and pitfalls to establish a proper diagnosis to avoid missing a treatable condition in patients with suspected THS.

Hypopituitarism and cranial nerve involvement mimicking Tolosa-Hunt syndrome as the initially presenting feature of diffuse large B-cell lymphoma: a case report.

BACKGROUND: Early diagnosis of lymphoma involving the central nervous system is sometimes difficult but emergent to avoid the delay of therapeutic initiation. Pituitary insufficiencies are usually associated with lymphoma in the pituitary gland. There have been no cases of lymphoma originating from extra pituitary gland with hypopituitarism that simultaneously presenting unilateral upper cranial nerve palsies and ophthalmalgia. These symptoms are mostly caused by neoplastic involvement of the skull base or benign diseases such as Tolosa-Hunt syndrome (THS). We report a case of lymphoma with unique clinical courses initially presenting hypopituitarism and symptoms mimicking THS with a mass in sphenoidal and cavernous sinuses accompanying sphenoidal bone erosion. CASE PRESENTATION: A 71-year-old woman visited our hospital with left ophthalmalgia, ptosis, and diplopia. Neurological findings revealed left oculomotor, trochlear and abducent nerve palsies. Endocrine tests indicated partial hypopituitarism. Initial CT and MRI revealed that a mass in sphenoidal and cavernous sinuses had invaded the sella with osteolysis of the sphenoid bone. At around four weeks, almost all the symptoms of cranial nerve palsies were relieved. Seven weeks later, she had a high fever and cervical lymph node (CLN) swellings. CLN biopsy revealed CD20-positive B-cells. She was diagnosed with diffuse large B-cell lymphoma (DLBCL). 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) revealed elevated uptake at the erosion lesion of the sphenoidal bone, but not the pituitary gland. After chemotherapy, all the symptoms related to systemic lymphoma were relieved, but partial hypopituitarism remained. The mass in sphenoidal and cavernous sinuses and elevated uptake by PET/CT were dissolved. CONCLUSION: This case of DLBCL had a unique clinical course; initial presentation of hypopituitarism and symptoms mimicking THS. There was also rare demonstration of mass lesions related to DLBCL in the sphenoidal and cavernous sinuses compressing the pituitary gland through an eroded area of the sphenoidal bone. It should be clinically cautioned that DLBCL can be associated with erosion of the sphenoidal bone and cause both hypopituitarism and THS-mimicking symptoms.

Clinical and Cerebrospinal Fluid Characteristics in 55 Cases of Tolosa-Hunt Syndrome: A Retrospective Analytical Study.

BACKGROUND: Several case series of patients with Tolosa-Hunt syndrome have been described in the literature; however, few studies have focused on the cerebrospinal fluid (CSF) characteristics. This study aimed to analyse the CSF characteristics of patients with Tolosa-Hunt syndrome. METHODS: Fifty-five patients who fulfilled the 3rd Edition of the International Classification of Headache Disorders diagnostic criteria for Tolosa-Hunt syndrome were included in this study. We retrospectively analysed data on CSF parameters, imaging findings, and clinical characteristics of these patients. RESULTS: Oligoclonal bands (OBs) were detected in the CSF of 13 (13/44, 29.5%) patients. The sex ratio was balanced. The mean age at onset of Tolosa-Hunt syndrome was 46.9 ± 10.23 (range 22-72) years. Eight (8/13, 61.5%) patients had multiple cranial nerve palsies. Lesions limited to the cavernous sinus were found on magnetic resonance imaging in 7 (7/13, 53.8%) patients. OBs were significantly detected more frequently in patients whose samples were evaluated less than 30 days after the onset of this diseases (p = 0.026); however, there were no significant differences in the protein level (p = 0.360) and IgG synthesis rate (p = 0.614). CONCLUSIONS: The detection of OBs in the CSF of patients with Tolosa-Hunt syndrome was not rare. It would be interesting to follow-up patients with OBs to determine whether they eventually developed an otherwise more specific inflammatory diagnosis.

Steroid responsive cavernous sinus syndrome due to Rosai-Dorfman disease: beyond Tolosa-Hunt syndrome - a case report.

BACKGROUND: The term "Tolosa-Hunt syndrome" (THS) has been used to refer to painful ophthalmoplegia associated with nonspecific inflammation of the cavernous sinus and many processes can result in a similar clinical picture, including infectious, inflammatory and neoplastic diseases. Rosai-Dorfman disease (RDD) is a lymphoproliferative disorder that rarely affects the central nervous system. We report a case of isolated CNS Rosai-Dorfman disease involving the cavernous sinus and presenting as "Tolosa-Hunt syndrome". CASE PRESENTATION: Our patient presented with horizontal diplopia due to impairment of cranial nerves III, IV and VI and a stabbing/throbbing headache predominantly in the left temporal and periorbitary regions. There was a nonspecific enlargement of the left cavernous sinus on MRI and the patient had a dramatic response to steroids. Biopsy of a frontal meningeal lesion was compatible with RDD. CONCLUSIONS: We highlight the importance of including Rosai-Dorfman disease as a differential diagnosis in cavernous sinus syndrome and demonstrate a satisfactory long-term response to steroid treatment in this disease.

Tolosa-Hunt Syndrome in Childhood and Adolescence: A Literature Review in the Last 10 Years.

OBJECTIVES: The aim of the study is to review the articles published in the last 10 years on Tolosa-Hunt syndrome (THS) in childhood and adolescence to learn about its clinical and epidemiological characteristics and its therapeutic management. METHODS: Based on the literature search in the major medical databases and using the descriptors "Tolosa-Hunt syndrome and children" and "Tolosa-Hunt syndrome and adolescent" we included articles published between 2009 and 2019. We found 51 articles, but only 11 met the inclusion criteria. RESULTS: There were eight women and four men with THS, whose average age was 12.0 ± 4.3 years, ranging from 4 to 17 years. Magnetic resonance imaging showed inflammation in the cavernous sinus (100%), orbit (25%), and superior orbital fissure (8.3%), unilateral in all patients. The internal carotid artery (58.3%), third (66.7%), sixth (33.3%), and fourth (25%) cranial nerves were injured. Headache was located in the orbital region, ipsilaterally to nerve involvement and had mild to severe intensity. Corticosteroid was the treatment used in 91.7% of the patients. They were followed for an average of 13.4 ± 12.4 months. Only two patients had recurrence of the disease. CONCLUSION: THS is infrequent in childhood and adolescence and knowledge of its clinical and epidemiological characteristics and its therapeutic management is necessary for pediatricians.

Multiple Spontaneous Visceral Arterial Dissections in a Patient With Tolosa-Hunt Syndrome on Corticosteroid Therapy.

Multiple spontaneous visceral arterial dissections are an infrequent occurrence. The etiology, risk factors and natural history of these dissections have not been elucidated, and the optimal therapeutic strategy has not been established. We report a rare case of multiple spontaneous visceral arterial dissections involving the celiac artery, splenic artery, superior mesenteric artery, and right renal artery in a patient with Tolosa-Hunt syndrome on short-term corticosteroid therapy. The patient was subjected to conservative treatment and endovascular repair, achieving good clinical and radiological outcomes during the long-term follow-up period.

The clinical features and outcomes of Tolosa-Hunt syndrome.

BACKGROUND: The objective of this study was to investigate the clinical features and outcomes of Tolosa-Hunt syndrome (THS). METHODS: A retrospective review of the medical records was performed on patients with THS between March 2016 and January 2020. A total of eleven patients fulfilling the International Classification of Headache Disorders (ICHD-3 beta) diagnostic criteria for THS were included in this study. RESULTS: The average age of the patients with THS was 57.18 ± 15.32 years and the mean duration of recovery was 26.91 ± 24.35 days. All eleven patients had orbital or periorbital pain as the first symptom followed by diplopia. Ptosis was found in five patients (45.45 %) in the involved eye. Sixth cranial nerve (CN) palsy was most common (eight cases, 72.73 %), followed by third and fourth CN palsy (five cases, 45.45 %, respectively), optic neuritis (two cases, 18.18 %), and trigeminal nerve and facial nerve palsy (one case, 9.09 %, respectively). One patient with optic neuritis failed to recover visual acuity and the other ten patients completely recovered their ocular motor limitation. All patients were initially treated with steroids. One patient relapsed after five weeks and one patient had a history of THS five years earlier. CONCLUSIONS: THS responded well to steroid treatment, but if it was accompanied by optic neuritis as optic nerve involvement, we suggest follow-up with high dose steroid treatment especially was important.

Multiple Cranial Neuropathies and Pachymeningitis in a Patient With a Pathogenic Nucleotide-Binding Oligomerization Domain 2 Polymorphism.

ABSTRACT: An 11-year-old boy presented with 2 weeks of intermittent headache, right orbital pain, and constant diplopia. Brain MRI showed dural thickening and enhancement of the right lateral cavernous sinus, right orbital apex, and tentorium. Initial cerebral spinal fluid analysis showed only mild pleocytosis, and serum diagnostics were unrevealing. The working diagnosis was Tolosa-Hunt syndrome. His pain and sixth nerve palsy resolved with corticosteroids. Five months after initial presentation, he developed new numbness of the right cheek, complete right ophthalmoplegia, and weakness and numbness of his right hand and leg, all of which were responsive to steroids. Fifteen months later, he returned to the emergency department with 2 weeks of left-sided headaches and acute diplopia. On examination, he had a left cranial nerve 6 palsy. Dural biopsy showed diffuse mononuclear inflammatory cell reaction consisting mostly of lymphocytes with no signs of granuloma formation, nor any epithelioid or giant cells. His clinical course was consistent with an autoinflammatory condition of unknown etiology. Genetic testing with an immunodeficiency panel showed a risk allele in NOD2 (nucleotide-binding oligomerization domain 2) c.3019dup (p.Leu1007Prof*2) that is associated with an increased risk for Crohn disease. His clinical condition had similarities to central nervous system sarcoidosis. Because of the similarities between our patient's clinical, imaging, and genetic findings and neurosarcoidosis, he was switched to a more targeted therapy-infliximab. His condition has since been stable for nearly 2 years. In conclusion, genetic testing should be considered in patients with suspected occult autoimmunity.

Tolosa-Hunt Syndrome Misdiagnosed as Surgical Complication After Frontal Balloon Sinuplasty.

ABSTRACT: Tolosa-Hunt syndrome (THS) is a rare benign disease caused by granulomatous inflammation in the craniofacial region. It is mostly idiopathic and generally presents with painful ophthalmoplegia, ipsilateral oculomotor paresis, and steroid responsiveness. There are few reports of THS after sinus surgery. Here, we present a case of THS in an adult immunocompetent patient with severe ophthalmic pain and diplopia after frontal balloon sinuplasty. The patient was initially misdiagnosed as having a surgical complication. The patient was treated with massive corticosteroid pulse therapy, and the symptoms resolved dramatically. There were no complications or recurrence in the 7-month follow-up period.

Tolosa-Hunt syndrome as initial presentation of Systemic Lupus Erythematosus.

PURPOSE: Case presentation of newly diagnosed systemic lupus erythematosus (SLE) presenting initially as Tolosa-Hunt syndrome (THS). STUDY DESIGN: Retrospective clinical case. METHOD: Case report. RESULTS: A healthy young man developed acute binocular diplopia within 2 days without other neurological deficits. Bilateral 6th cranial nerve palsy was observed with general reduction in the visual field test. Emergent brain magnetic resonance image (MRI) was performed, which revealed severe inflammation in the cavernous sinus, superior orbital fissure, and apex of the orbit. No cavernous thrombosis or intracranial lesion was shown in the MRI. THS was diagnosed and the patient's CN 6 palsy recovered quickly after corticosteroid treatment. However, severe anaemia was discovered during admission (Hb=6.0), so the patient was evaluated by profound laboratory tests, which revealed SLE. CONCLUSION: With painful ophthalmoplegia, cavernous sinus syndrome is highly suspected. THS is one of the differential diagnoses for cavernous sinus syndrome. THS is a rare disease, recognized by the National Organization for Rare Disorders, and characterized by inflammatory changes in the cavernous sinus, superior orbital fissure and/or orbital apex under image study. The inflammatory changes are mostly idiopathic, but secondary causes such as sarcoidosis or other autoimmune diseases need to be ruled out. Physicians should be aware of possible underlying conditions, such as immunosuppressed status as in SLE, as the true cause of THS.

Proposed modified diagnostic criteria for recurrent painful ophthalmoplegic neuropathy: Five case reports and literature review.

BACKGROUND: Recurrent painful ophthalmoplegic neuropathy (RPON) is an uncommon disorder characterized by recurrent unilateral headache attacks associated with ipsilateral ophthalmoplegia. We intend to study the clinical picture in our case series along with the published literature to discuss the pathogenesis and propose modified diagnostic criteria for recurrent painful ophthalmoplegic neuropathy. METHODS: We reported five cases diagnosed as ophthalmoplegic migraine/RPON in our medical centers and reviewed the published literature related to RPON from the Pubmed database between 2000 and 2020. In one of these cases, a multiplanar reformation was performed to look at the aberrant cranial nerve. RESULTS: The mean onset age for RPON was 22.1 years, and the oculomotor nerve was the most commonly involved cranial nerve (53.9%) in 165 reviewed patients. In most patients, ophthalmoplegia started within 1 week of the headache attack (95.7%, 67/70). Additionally, 27.6% (40/145) of patients presented enhancement of the involved nerve(s) from MRI tests. Finally, 78 patients received corticosteroids, out of which 96.2% benefited from them. CONCLUSION: This is the first time multiplanar reformation has been performed to reveal the distortion of the oculomotor nerve. Modified diagnostic criteria are proposed. We hope to expand the current knowledge and increase the detection of recurrent painful ophthalmoplegic neuropathy in the future.

Reappraising the Tolosa-Hunt Syndrome Diagnostic Criteria: A Case Series.

INTRODUCTION: Tolosa-Hunt syndrome (THS), a condition characterized by painful ophthalmoplegia and accompanied by cranial nerve palsies, remains as a diagnosis of exclusion. Nevertheless, the 3rd Edition of the International Classification of Headache Disorders (ICHD) has refined its diagnostic criteria to require the demonstration of granulomatous inflammation on magnetic resonance imaging or biopsy. We sought to assess the effectiveness of the new criteria in arriving at accurate diagnoses. METHODS: We extracted all patient charts from our institution's electronic medical record associated with ICD-9 code 378.55 (external ophthalmoplegia). We then completed the retrospective diagnostic workups to determine if subjects met ICHD-3 criteria for THS and compared our findings with their final diagnoses. RESULTS: Of 62 patients associated with ICD-9 code 378.55, 10 (16%) was identified to have presenting symptoms concerning THS. The average age at the first onset of THS-like symptoms was 58 years. Five of the 10 (50%) met ICHD-3 criteria for THS. Two of the 5 (40%) meeting ICHD-3 criteria for THS were discovered to have other diagnoses. Two of the 5 (40%) patients not meeting ICHD-3 criteria for THS nevertheless received a final diagnosis of THS. DISCUSSION: Our false-negative rate of 40% is consistent with previous case series found in the literature. Our false-positive rate of 40% is, to our knowledge, a new contribution to the literature as no other case series includes more than a single false-positive. The false-positive rate is most concerning, as a preliminary misdiagnosis of THS can delay treatment tailored to the true etiology. Furthermore, infectious etiologies can be exacerbated with steroid treatment. CONCLUSION: Our case series suggests that ICHD-3 criteria are suboptimal for the accurate diagnosis of THS. We recommend a close follow-up for all patients with symptoms concerning THS until a definitive responsible etiology is discovered and we encourage further studies assessing ICHD-3 guidelines to optimize their sensitivity and specificity in the diagnosis of THS.

Tolosa-Hunt syndrome with general myasthenia gravis involvement.

Tolosa-Hunt syndrome is an uncommon disease that exhibits unilateral periorbital pain or headache, accompanied by cranial nerve palsies. Myasthenia gravis is an acquired immune system disease involving the neuromuscular junction. One rare case of Tolosa-Hunt syndrome combined with ocular myasthenia gravis had been reported in the literature, but not general myasthenia gravis. We present a patient with a probable coincidence of Tolosa-Hunt syndrome and general myasthenia gravis. A 63-year-old male exhibited episodes of unilateral headache with double vision, bilateral ptosis, vision decrease in the left eye and left facial hypoesthesia, muscle weakness in limbs and neck. The muscle weakness was fluctuating and could be relieved by rest. Blood analysis, cranial magnetic resonance imaging, magnetic resonance angiography/venogram) and orbit/mediastinum computed tomography demonstrated no abnormalities. Serum myasthenia gravis related antibodies detection showed positive titin- antibodies and ryanodine receptor antibodies. Corticosteroid and pyridostigmine bromide treatments were effective. Each of the patient's symptoms had almost disappeared at the third-month follow-up. We speculate on the etiology of Tolosa-Hunt syndrome with general myasthenia.

[Orbital myositis, a cause of pediatric painful ophthalmoplegia]. / Miositis orbitaria, una causa de oftalmoplejia dolorosa en pediatría.

INTRODUCTION: Orbital myositis (OM) is a serious inflammation of extraocular muscles with unknown etiology. Pe diatric presentation is rare and often affects more than one individual in a family, suggesting a genetic predisposition. OBJECTIVE: To describe a pediatric case of orbital myositis, its clinical characteristics, and the usefulness of MRI for confirming the diagnosis. CLINICAL CASE: A 13-year-old female patient presenting with acute headache, right periorbital pain, exacerbated by eye movements, and blurred vision. We ruled out thyrotoxic myopathy, infectious diseases, autoimmunity, and malignancy. An MRI showed right medial rectus muscle myositis and no evidence of optic neuritis. She was treated with intravenous systemic glucocorticoids followed by oral steroids with complete clinical resolution. CONCLUSIONS: OM has unknown etiology and can present a malignant course. Due to its unspecific clinical presentation, a comprehensive differential diagnosis should be made and it should consider performing MRI. Early treatment avoids permanent damage of extraocular muscles.

The aetiologies of unilateral oculomotor nerve palsy: a clinical analysis on 121 patients.

Background and purpose: To strengthen the understanding, increase the early diagnostic rate, and improve the outcome of unilateral oculomotor nerve palsy through the analysis of the 121 patients suffering from this disease in our hospital. Methods: A retrospective study was performed on the 121 patients with unilateral oculomotor nerve palsy diagnosed at the Affiliated Hospital of Xuzhou Medical University from October 2014 to October 2015. The clinical data, such as gender, age, aetiology, clinical features, laboratory tests, and six months follow up reports were analyzed. Results: The main causes identified in the 121 patients with unilateral oculomotor nerve palsy were intracranial aneurysm (29.8%), diabetic peripheral neuropathy (26.5%), painful ophthalmoplegia (9.9%), and other causes (33.9%). The results from the six month follow up showed that in all the patients, 53.7% were fully recovered, 38.0% improved, and 8.3% had no significant change in symptoms. The results also indicated that the patients with diabetic peripheral neuropathy had the best outcome with 71.9% full recovery rate, which was significantly higher than that in the patients with intracranial aneurysm (36.1%, p < .05), and idiopathic causes (44.5%, p < .05). Conclusions: Our data indicates that intracranial aneurysm is the leading cause of unilateral oculomotor nerve palsy, and that diabetic peripheral neuropathy has better outcome. Understanding the common causes and clinical features of unilateral oculomotor nerve paralysis is helpful for its early diagnosis and treatment.

Carotid-cavernous fistula(CCF) presenting as paroxysmal painful ophthalmoplegia.

BACKGROUND: Painful ophthalmoplegia can be caused by various etiologies, and broad differential diagnosis is needed. Carotid-cavernous fistula (CCF) is a rare cause of painful ophthalmoplegia, and early diagnosis is quite difficult. CASE PRESENTATION: Here, we present a case of paroxysmal painful ophthalmoplegia caused by CCF. The episodic symptoms were nonstereotypical and lasted minutes to hours. Magnetic resonance imaging (MRI) and computed tomography angiography (CTA) results were normal, which confounded efforts to determine a diagnosis. Subsequently, digital subtraction angiography (DSA) revealed a posterior-draining CCF. The CCF was treated at an early stage without residual symptoms. CONCLUSIONS: We propose that symptoms could be relapsing or remitting during an early stage of CCF and that posterior-draining CCF is prone to misdiagnosis due to atypical manifestations. Normal CTA results cannot exclude carotid-cavernous fistula, and DSA should be performed once CCF is suspected.

Tolosa-Hunt Syndrome: Appraising the ICHD-3 beta diagnostic criteria.

Introduction The term Tolosa-Hunt Syndrome was first used more than half a century ago to describe painful ophthalmoplegia accompanied by cranial nerve palsies. In the decades since, its diagnostic criteria have evolved considerably. The beta version of the 3rd Edition of the International Classification of Headache Disorders narrows these criteria to require the demonstration of granulomatous inflammation on MRI or biopsy. We believe this may introduce challenges to accurate diagnosis. Discussion Requiring the demonstration of granulomatous inflammation for a diagnosis of Tolosa-Hunt Syndrome may introduce the potential for false negative and false positive diagnoses. Although the disorder presents secondary to granulomatous inflammation, MRI technology may not be able to identify it reliably, and biopsy is not always indicated for its symptomatology. Additionally, several cases have been reported of Tolosa-Hunt Syndrome diagnosed with MRI-confirmed granulomatous inflammation that later prove to be attributable to other pathologies. The emphasis on neuroimaging may therefore exclude some true Tolosa-Hunt Syndrome cases and include others resulting from other latent pathologies that are not visible on MRI. Conclusion We wish to offer several potential modifications to the International Classification of Headache Disorders guidelines for Tolosa-Hunt Syndrome, including making the demonstration of granulomatous inflammation on MRI or biopsy non-mandatory and lengthening patient follow-up to two years for cases in which MRI is unrevealing.