Tolosa-Hunt Syndrome as the Initial Presentation of Systemic Lupus Erythematosus.

Tolosa-Hunt syndrome (THS), also known as painful ophthalmoplegia, recurrent ophthalmoplegia, or ophthalmoplegia syndrome, is described as severe and unilateral peri-orbital headaches associated with painful and restricted eye movements. THS is an uncommon disorder due to granulomatous inflammation of the cavernous sinus. Although THS is primarily idiopathic, it has rarely been reported in association with systemic lupus erythematosus (SLE). This case report describes a unique case of THS presenting as the initial manifestation of SLE, a multi-system autoimmune disease. We present a detailed case report of a 54-year-old female patient who presented with THS with the classical symptoms of THS including unilateral headache, double vision, and orbital pain. A cranial nerve examination revealed right oculomotor nerve palsy with the inability to adduct, raise, or depress her right eye. A detailed clinical examination revealed alopecia areata and erythematous macular lesions on her right earlobe. Laboratory investigations were unremarkable except for an increased erythrocyte sedimentation rate (ESR). Diagnostic investigations, including MRI and serological tests, were conducted to explore the underlying causes and systemic involvement. The patient's MRI showed characteristic findings consistent with THS, while serological tests revealed positive antinuclear antibodies, anti-ds-DNA antibodies, and anti-Smith antibodies and low complement levels leading to a concurrent diagnosis of SLE. There were no other systemic manifestations of lupus at the time of presentation.  Treatment with high-dose corticosteroids led to rapid improvement in ocular symptoms and headaches. Maintenance immunosuppressive therapy was initiated for the management of SLE. The patient had no relapses on follow-up. This case report underscores THS as a potential initial manifestation of SLE. It highlights the need for comprehensive diagnostic evaluation in patients presenting with atypical cranial neuropathy to consider systemic autoimmune disorders like SLE. Early diagnosis and management are crucial for improving outcomes in such intertwined pathologies. This case emphasizes the need for clinicians to be aware of the possibility of THS as the initial manifestation of SLE. This extended abstract provides a comprehensive overview of the article, laying out the significance of the case in broadening the clinical understanding of the overlap between localized inflammatory syndromes and systemic autoimmune conditions like SLE.

Recurrent Headache With Diplopia: A Common Presentation of an Uncommon Entity.

Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare headache syndrome, the diagnosis of which can be daunting to those who are not familiar with it. It presents characteristically with recurrent ocular motor weakness and ipsilateral head pain without an underlying etiology and often has unique imaging findings. Even after the successful diagnosis of this entity, there are no published management guidelines. Here, we present the case of a 31-year-old man whom we diagnosed with RPON following two episodes of unilateral headache with ophthalmoplegia over a three-month period and treated successfully with high-dose steroids on both occasions. We highlight the lack of prior migraine history and seeming antecedent viral infection as potential supporting evidence that this condition has a unique pathophysiology different from migraine. We also highlight his dramatic and reproducible response to steroids as additional evidence that steroids are good acute treatment options for this condition. Finally, as our patient lacked the expected cranial nerve imaging abnormalities on head MRI, we suggest that cranial nerve thickening and/or enhancement on MR imaging is not a sine qua non for this diagnosis, contrary to the opinion of some experts.

Recurrent Tolosa-Hunt syndrome: a case report.

Introduction and importance: Tolosa-Hunt syndrome is a rare condition with unknown aetiology that manifests clinically as unilateral orbital pain and ophthalmoplegia. It is a diagnosis of exclusion that resolves spontaneously but can recur and respond dramatically to systemic steroids. Case presentation: The authors herein report a case of a 38-year-old male who presented with horizontal diplopia, limited outward movement of the right eye, and blurry vision for two days which was managed with oral Prednisolone. The patient visited 3 months later with progressive ptosis and vertical diplopia with periorbital pain over the right eye. It was eventually diagnosed via magnetic imaging resonance studies and successfully treated for Tolosa-Hunt syndrome with IV methylprednisolone followed by oral prednisolone. Clinical discussion: Hence, the typical clinical presentation of the case with significant response to steroids, exclusion of other conditions from investigation and imaging, and subsequent recurrence of similar symptoms were crucial for making the diagnosis of Tolosa-Hunt syndrome. Conclusion: Tolosa-Hunt syndrome is a syndrome of painful ophthalmoplegia which responds well to steroid therapy but has a tendency to recur. Hence, patients must be adequately informed about the reoccurrence and kept under follow-up.

A Case of Painful Diplopia after COVID-19 Vaccination: Could It Be Tolosa-Hunt Syndrome?

Herein, we report a rare case of Tolosa-Hunt syndrome (THS) following coronavirus disease 2019 (COVID-19) vaccine administration. A 64-year-old patient presented with recurrent horizontal diplopia and ipsilateral orbital pain, 2 weeks after being administered the COVID-19 vaccination. A diagnosis of THS was based on the relevant criteria after ruling out the differential diagnoses. The clinical presentation improved with corticosteroid administration. THS must be recognized as a complication of COVID-19 vaccination. This association can be explained by an autoimmune response.

A case report on recurrent alternating Tolosa-Hunt syndrome due to bacterial sphenoid sinusitis: rediscussing the diagnostic terminology and classification.

BACKGROUND: Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia caused by idiopathic granulomatous inflammation involving the cavernous sinus region. Patients respond well to steroid therapy. THS is included in the differential diagnosis of cavernous sinus syndrome, so it is important to fully exclude other lesions in this area before treatment, otherwise steroid treatment may lead to fatal outcomes. Here we describe a patient who initially presented with symptoms that simulated THS symptoms and developed recurrent alternating painful ophthalmoplegia during follow-up, and the patient was finally diagnosed with cavernous sinusitis caused by bacterial sphenoid sinusitis. CASE PRESENTATION: A 34-year-old woman presented with left painful ophthalmoplegia. Magnetic resonance imaging (MRI) revealed abnormal signals in the left cavernous sinus area, and these abnormal signals were suspected to be THS. After steroid treatment, the patient obtained pain relief and had complete recovery of her ophthalmoplegia. However, right painful ophthalmoplegia appeared during the follow-up period. MRI showed obvious inflammatory signals in the right cavernous sinus and right sphenoid sinus. Then nasal sinus puncture and aspiration culture were performed, and the results showed a coagulase-negative staphylococcus infection. After antibiotic treatment with vancomycin, the painful ophthalmoplegia completely resolved, and the neurological examination and MRI returned to normal. CONCLUSION: Some other causes of painful ophthalmoplegia also fulfill the diagnostic criteria for THS in the International Classification of Headache Disorders third edition (ICHD-3) and respond well to steroid therapy. Early diagnosis of THS may be harmful to patients, and clinicians should exercise great caution when dealing with similar cases without a biopsy. Using "cavernous sinus syndrome" instead of "Tolosa-Hunt syndrome" as a diagnostic category may provide a better clinical thinking for etiological diagnosis.

Single internal carotid cavernous sinus aneurysm presented as bilateral painful ophthalmoplegia: a case report.

BACKGROUND: Intracranial aneurysms are the most common vascular cause of painful ophthalmoplegia. Symptoms include retro-orbital pain, diplopia, ophthalmoplegia, trigeminal neuropathy, or a combination of these. Most single aneurysms cause ipsilateral, painful ophthalmoplegia. Here, we report the first, to our knowledge, case of bilateral painful ophthalmoplegia possibly caused by an aneurysm of the cavernous segment of the left internal carotid artery. CASE PRESENTATION: A 62-year-old male patient presented with headache and bilateral ptosis. Laboratory tests revealed hypopituitary function. Computerized tomography angiography showed a large aneurysm in the cavernous sinus segment of the left internal carotid artery. Aneurysm embolization was performed in the Nerve Interventional Department. Four months after surgery, the patient's symptoms returned to normal. CONCLUSIONS: This case suggests that patients with bilateral painful ophthalmoplegia should be screened for aneurysms using computed tomography angiography or magnetic resonance angiography immediately.

Invasive sphenoid sinus aspergillosis with normal findings on initial diagnostic tests that mimics Tolosa-Hunt syndrome-a diagnostic dilemma: a case report.

Invasive sphenoid sinus aspergillosis can mimic Tolosa-Hunt syndrome (THS), leading to frequent misdiagnoses and potentially fatal consequences. We report a case of invasive sphenoid sinus aspergillosis initially misdiagnosed as THS. A 79-year-old man presented with right periorbital pain, ophthalmoplegia, and loss of vision. Initial evaluations including magnetic resonance imaging (MRI), were normal. He was first diagnosed with THS based on clinical features. The disease progressed despite high-dose intravenous steroid treatment, and an enhancing mass-like lesion was found in the right orbital apex, cavernous sinus, and sphenoid sinus on follow-up MRI. Aspergillosis was eventually confirmed by sphenoid sinus biopsy. The patient developed cerebral infarction and finally died despite being treated with amphotericin B. Given that invasive sphenoid sinus aspergillosis may initially resemble THS, high suspicion and rapid histological examination are important for diagnosis.

Síndrome de oftalmoplejía dolorosa, más allá del síndrome de Tolosa Hunt. Una serie de casos / Painful ophthalmoplegia syndrome, beyond Tolosa-Hunt syndrome. A case series

El síndrome de oftalmoplejía dolorosa se caracteriza por la presencia de dolor periorbital o pericraneal, que se acompaña de parálisis de los nervios oculomotores. El diagnóstico diferencial es amplio y requiere un estudio riguroso. Se describen 3casos clínicos de pacientes con síndrome de oftalmoplejía dolorosa en los que se requirieron múltiples estudios de extensión para obtener un diagnóstico etiológico. El síndrome de oftalmoplejía dolorosa es un trastorno complejo que tiene múltiples causas, entre las que se incluyen infecciones, procesos inflamatorios y tumorales. Debe considerarse la biopsia en aquellos pacientes en los que los estudios no invasivos no son conclusivos (AU)

Painful ophthalmoplejía syndrome is characterised by the presence of peri-orbital or peri-cranial pain that is accompanied by paralysis of the oculomotor nerves. The differential diagnosis is broad, and requires a rigorous study. Three clinical cases of patients with painful ophthalmoplejía syndrome are described, in which multiple extension studies were required to obtain an aetiological diagnosis. Painful ophthalmoplejía syndrome is a complex disorder that has multiple causes, including infections, inflammatory, and tumour processes. Biopsy should be considered in those cases in which non-invasive studies are inconclusive (AU)

Painful ophthalmoplegia syndrome, beyond Tolosa-Hunt syndrome. A case series.

Painful ophthalmoplegia syndrome is characterised by the presence of peri-orbital or peri-cranial pain that is accompanied by paralysis of the oculomotor nerves. The differential diagnosis is broad, and requires a rigorous study. Three clinical cases of patients with painful ophthalmoplegia syndrome are described, in which multiple extension studies were required to obtain an aetiological diagnosis. Painful ophthalmoplegia syndrome is a complex disorder that has multiple causes, including infections, inflammatory, and tumour processes. Biopsy should be considered in those cases in which non-invasive studies are inconclusive.

Painful ophthalmoplegia in a patient with a history of marginal zone lymphoma.

A 73-year-old man with a history of marginal zone lymphoma was admitted to the emergency room for diplopia and ipsilateral headache. The Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) demonstrated intense and symmetrical hypermetabolism of the cavernous sinuses, and hypermetabolic lesions diffusely in the lymph nodes and bones. The diagnosis of high-grade relapse of lymphomatous disease was made. In this context, the homogenous and symmetric lesion of the cavernous sinuses, without any other encephalic or meningeal lesions, raised the hypothesis of a paraneoplastic origin. A plausible paraneoplastic link between the neuro-ophthalmological lesion and the malignant disorder is IgG4-related disease, a condition that may be associated with lymphoma. As in our case, this diagnosis is often presumptive because histopathological confirmation is difficult to obtain.

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.

Recurrent Tolosa-Hunt syndrome.

The Tolosa-Hunt syndrome (THS) is caused by granulomatous inflammation in the cavernous sinus, superior orbital fissure, or orbit characterized by painful ophthalmoplegia. Here, we report a case of recurrent THS in a 48-year-old female, who initially showed a poor response to low-dose steroids. Each episode involved different cranial nerves and painful ophthalmoplegia. Neuroimaging showed enhancement in the right cavernous sinus. The patient was treated with glucocorticoid pulse therapy and azathioprine. THS was previously considered to be responsive to glucocorticoids. This report demonstrates a case of THS with poor response to steroids and unique presentation of frequent recurrence with different cranial nerve involvement.

Severe Bilateral Photophobia and Unilateral Abducens Nerve Palsy: An Unusual Presentation of Herpes Zoster Ophthalmicus.

Herpes zoster ophthalmicus (HZO) is an uncommon neurocutaneous condition resulting from reactivation of the varicella zoster virus in the ophthalmic division of the trigeminal nerve. Typical presentation of HZO includes a characteristic painful vesicular dermatomal rash. However, the appearance of isolated neurologic complications in the absence of ocular findings has not been previously emphasized. We observed a 47-year-old female patient with established HZO who presented with 1 week of worsening bilateral photophobia and double vision following completion of antiviral treatment. Her motility examination revealed near-complete abduction deficit of her left eye with no other signs of neurologic deficit. Slit lamp biomicroscopy, magnetic resonance imaging, and all laboratory tests were negative. After 2 tapering cycles of oral corticosteroid treatment, her photophobia resolved, and ophthalmoplegia significantly improved. The failure of antiviral therapy in preventing our patient's neurologic sequelae highlights the importance of concurrent steroid therapy in suspected HZO patients. Furthermore, the resolution of symptoms following administration of systemic glucocorticoids supports consideration of HZO complications as immune-mediated. Finally, the unusual presentation of bilateral photophobia in the absence of ocular inflammation warrants further investigation into the pathogenesis of HZO.

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.

Tolosa-Hunt Syndrome: A Non-Classical Presentation of a Rare Cause of Unilateral Headache and Painful Ophthalmoplegia.

Tolosa-Hunt syndrome is a rare condition involving the orbital and retro-orbital space. The typical symptoms are orbital pain, swelling, headache, palsies of the cranial nerves, and sensory loss in the distribution of the trigeminal nerve. Tolosa-Hunt syndrome relapses and remits with episodes separated by months to years. It is a diagnosis of exclusion after ruling out other causes of painful ophthalmoplegia and is treated with high-dose steroids. We present a case of a 43-year-old woman with a history of recurrent headaches and vision disturbances for the past 4 years presenting with worsening left-eye pain that radiated to the back of her head, swelling around the left eye, double vision, and a unilateral left-sided headache. Visual acuity was decreased in the left eye compared to the right and the patient reported left-eye pain when at extreme medial and lateral gaze. Erythrocyte sedimentation rate (ESR) was found to be elevated with all other laboratory testing within normal limits. Computed tomography (CT) scan of the orbits was unremarkable. Ophthalmology excluded other differential diagnoses and she was treated with intravenous methylprednisolone with significant improvement of symptoms. The triad of one or more episodes of unilateral orbital pain, paresis of one or more of the cranial nerves, and granulomas by magnetic resonance imaging (MRI) or biopsy is 95-100% sensitive at diagnosing the syndrome; however, our patient did not present with the "classic" triad. Initial treatment of Tolosa-Hunt is with high-dose steroids which as in our case lead to rapid and significant improvement of symptoms.

Tolosa-Hunt Syndrome: Long-Term Outcome and Role of Steroid-Sparing Agents.

BACKGROUND: Tolosa-Hunt Syndrome (THS) is one of the causes of cavernous sinus syndrome causing painful ophthalmoplegia. Literature on long-term outcome of this rare condition is scarce. AIMS AND OBJECTIVES: The aim is to study the recurrence and role of steroid-sparing agents in THS. METHODOLOGY: All cases of THS treated at a tertiary-level teaching hospital during a 10-year period were studied. Clinical and radiological profile, response to treatment and recurrences were noted. RESULTS: A total of 44 cases were studied. The mean age was 49.5 years, Males constituted 23/44 (52%). The first symptom was pain in 90%. Ptosis with ophthalmoplegia was the most common deficit 29/44 (66%). Lesions confined to cavernous sinus 27/44 (61%) was the most frequent magnetic resonance imaging finding. All patients received steroids as the initial treatment and 15/44 (34%) received steroid-sparing agents. Follow-up ranged from 6 to 120 months (Mean 39 months). Two patients had alternative diagnosis of leptomeningeal malignancy and hypertrophic pachymeningitis on follow-up. Recurrences occurred in 18/37 (48.6%). Time for recurrence varied from 8 months to 7 years. (Mean 18 months). No clinical or radiological predictors for recurrence were identified. Patients who received steroid-sparing agents had a significantly lower recurrence 3/15 (20%) versus 14/26 (53.8%)P < 0.034. CONCLUSIONS: Around 50% of patients with THS can have recurrence. Steroid-sparing agents appear to prevent recurrence. A prospective multicenter randomized controlled trial may help to evaluate the risk and benefits of steroid-sparing therapy and to identify any possible predictors for recurrence.

Painful ophthalmoplegia syndrome, beyond Tolosa-Hunt syndrome. A case series. / Síndrome de oftalmoplejía dolorosa, más allá del síndrome de Tolosa Hunt. Una serie de casos.

Painful ophthalmoplejía syndrome is characterised by the presence of peri-orbital or peri-cranial pain that is accompanied by paralysis of the oculomotor nerves. The differential diagnosis is broad, and requires a rigorous study. Three clinical cases of patients with painful ophthalmoplejía syndrome are described, in which multiple extension studies were required to obtain an aetiological diagnosis. Painful ophthalmoplejía syndrome is a complex disorder that has multiple causes, including infections, inflammatory, and tumour processes. Biopsy should be considered in those cases in which non-invasive studies are inconclusive.

A Case of Tolosa-Hunt Syndrome With Discoid Lupus Erythematosus.

The occurrence of Tolosa-Hunt syndrome (THS) in the setting of discoid lupus erythematosus (DLE) has not been previously reported. We report a case of a 55-year-old Chinese man with established cutaneous lupus who presented with 1 week of worsening blurry vision and ptosis of the left eye with severe headache 2 weeks prior to presentation. His cranial nerve examination was significant for left afferent pupillary defect, red desaturation, ptosis, and oculomotor nerve palsy. He also presented with active DLE lesions. Magnetic resonance imaging brain demonstrated asymmetric thickening and enhancement of the left cavernous sinus consistent with THS. After a 4-week gradual steroid taper his ophthalmoplegia resolved. The unusual occurrence of THS and DLE prompts consideration of nonsystemic autoimmune disorders in diagnosis of THS.

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.