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1.
Postep Psychiatr Neurol ; 33(1): 26-34, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38948688

ABSTRACT

Purpose: Tolosa-Hunt syndrome (THS) is a rare cause of painful ophtalmoplegia with different clinical manifestations. It is described as a unilateral periorbital headache with concomitant dysfunction of at least one out of the IIIrd, IVth and VIth cranial nerves due to the granulomatous inflammation of periorbital structures, but no underlying cause has been established. Case description: We present six patients referred to the Neurology Department due to a unilateral headache with ipsilateral paresis of at least one cranial nerve responsible for eye movements. The THS diagnostic criteria of the International Headache Disorders Classification (ICHD-3) were applied and analysed. Few patients had atypical clinical manifestations according to these criteria. Comment: Diagnosing THS may prove very challenging. There is a lack of specific markers for the disorder, whereas diagnostic criteria leave a wide area for misdiagnosis. The diagnostic approach should be focused on the exclusion of other pathologies because typical steroid therapy may prove fatal in otherwise benign cases.

2.
Cureus ; 16(6): e61692, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38975518

ABSTRACT

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.

3.
Ann Med Surg (Lond) ; 86(3): 1695-1699, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38463132

ABSTRACT

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.

4.
Article in Russian | MEDLINE | ID: mdl-38529871

ABSTRACT

The article presents a case of idiopathic hypertrophic pachymeningitis of a 61-year-old male patient with severe cephalgia and progressive neuropathy of the oculomotor nerves. The diagnosis was confirmed by MRI with gadolinium, which revealed thickening of the dura mater with accumulation of paramagnetic in the convexital parts of the frontal and temporal regions, as well as on the base of the skull and tentorium. During the use of pulse therapy with glucocorticosteroids (GCS) the symptoms regressed, but when the therapy was stopped, there was a relapse of ptosis and oculomotor abnormalities on the other side followed by a slower effect of GCS therapy. The article also presents a brief review of current knowledge about this pathology.


Subject(s)
Meningitis , Skull , Male , Humans , Middle Aged , Skull/pathology , Meningitis/diagnosis , Meningitis/drug therapy , Meningitis/etiology , Magnetic Resonance Imaging , Hypertrophy
5.
Front Neurol ; 15: 1326867, 2024.
Article in English | MEDLINE | ID: mdl-38419701

ABSTRACT

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy caused by the autoantibody of aquaporin-4 (AQP4). Herein, we report a case of Tolosa-Hunt syndrome presenting with abducens palsy and AQP4 antibodies. This was a rare case of AQP4-immunoglobulin G seropositivity in a patient with Tolosa-Hunt syndrome. Our findings may expand the clinical phenotype of NMOSD and indicate that clinicians should consider testing for AQP4 antibodies in patients with Tolosa-Hunt syndrome.

6.
J Neurol ; 271(2): 935-943, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37853245

ABSTRACT

BACKGROUND AND PURPOSE: Tolosa-Hunt Syndrome (THS) is a rare disorder, and detailed clinical information and treatment outcomes have yet to be fully elucidated. This study aims to investigate the clinical features and factors associated with the treatment outcomes of THS, as defined by the established diagnostic criteria. METHODS: This study retrospectively recruited 91 patients with a diagnosis of THS from 2003 to 2020. We analyzed the clinical features and outcomes, the initial treatment response, recurrences, and the final treatment response. RESULTS: Isolated ocular motor nerve palsy was the most common (82.4%) finding of ophthalmoplegia, involving the oculomotor nerve in more than half of the cases (52.0%). The MRI lesions were mostly observed in the cavernous sinus (94.5%) with an extracavernous extension in about one-third of them. Five patients showed only extracavernous lesions. A total of 25 (27.5%) patients experienced recurrence. Recurrence occurred during steroid tapering as part of the initial treatment in seven, while in 18 patients, it happened after the successful termination of the initial treatment. However, all patients achieved complete remission at the final. Age was associated with a decrease in initial symptom duration (HR = 1.023, CI = 1.004-1.044) as well as an increase in recurrence-free duration (HR = 0.944, CI = 0.911-0.978). High-dose corticosteroid treatment was associated with a decrease in initial symptom duration (HR = 1.642, CI = 1.001-2.695) and total treatment duration (HR = 2.203 CI = 1.302-3.730). CONCLUSIONS: THS can recur frequently especially in younger but have a favorable prognosis. High-dose corticosteroids can be an effective initial treatment and reduce the total treatment duration.


Subject(s)
Cavernous Sinus , Ophthalmoplegia , Tolosa-Hunt Syndrome , Humans , Tolosa-Hunt Syndrome/diagnosis , Tolosa-Hunt Syndrome/drug therapy , Tolosa-Hunt Syndrome/complications , Retrospective Studies , Cavernous Sinus/pathology , Magnetic Resonance Imaging , Adrenal Cortex Hormones/therapeutic use , Paralysis , Recurrence
7.
BMC Neurol ; 23(1): 437, 2023 Dec 11.
Article in English | MEDLINE | ID: mdl-38082244

ABSTRACT

BACKGROUND: Neuromuscular diseases (NMD) emerged as one of the main side effects of the COVID-19 vaccination. We pooled and summarized the evidence on the clinical features and outcomes of NMD associated with COVID-19 vaccination. METHODS: We comprehensively searched three databases, Medline, Embase, and Scopus, using the key terms covering "Neuromuscular disease" AND "COVID-19 vaccine", and pooled the individual patient data extracted from the included studies. RESULTS: A total of 258 NMD cases following COVID-19 have been reported globally, of which 171 cases were Guillain-Barré syndrome (GBS), 40 Parsonage-Turner syndrome (PTS), 22 Myasthenia Gravis (MG), 19 facial nerve palsy (FNP), 5 single fiber neuropathy, and 1 Tolosa-Hunt syndrome. All (100%) SFN patients and 58% of FNP patients were female; in the remaining NMDs, patients were predominantly male, including MG (82%), GBS (63%), and PTS (62.5%). The median time from vaccine to symptom was less than 2 weeks in all groups. Symptoms mainly appeared following the first dose of vector vaccine, but there was no specific pattern for mRNA-based. CONCLUSION: COVID-19 vaccines might induce some NMDs, mainly in adults. The age distribution and gender characteristics of affected patients may differ based on the NMD type. About two-thirds of the cases probably occur less than 2 weeks after vaccination.


Subject(s)
Bell Palsy , COVID-19 , Facial Paralysis , Guillain-Barre Syndrome , Myasthenia Gravis , Neuromuscular Diseases , Adult , Humans , Female , Male , COVID-19 Vaccines/adverse effects , COVID-19/epidemiology , COVID-19/prevention & control , Neuromuscular Diseases/epidemiology , Guillain-Barre Syndrome/epidemiology , Guillain-Barre Syndrome/etiology
8.
Ital J Pediatr ; 49(1): 157, 2023 Nov 27.
Article in English | MEDLINE | ID: mdl-38012680

ABSTRACT

BACKGROUND: Tolosa-Hunt syndrome (THS) and recurrent painful ophthalmoplegic neuropathy (RPON) are rare diseases reported within the "Painful lesions of the cranial nerves" section of the International Classification of Headache Disorders-3rd edition (ICHD-3). In case of a first painful attack, differential diagnosis could be challenging and many pitfalls are due to the rarity of the disorders and the lack of information about correct medical management in youngsters. CASE PRESENTATION: Our main purpose was to report a new case of THS and a new case of RPON describing management and diagnostic investigation at the time of the first episode. In both cases of THS (13 years old) and RPON (14 years old) a unilateral periorbital headache associated with acute onset of ipsilateral third cranial nerve paresis, scarcely responding to non-steroidal anti-inflammatory drugs (NSAID), was present at the beginning of the first attack. Brain MRI with "time-of-flight" (TOF) angiography and the need to administer steroids (after 72 h from onset) in order to stop pain were the most important handles allowing us to adopt the correct management both in THS or RPON since onset and to face recurrences in RPON by avoiding useless therapy during follow-up. CONCLUSION: Unilateral periorbital headache associated with third-fourth or sixth cranial nerve paresis should ideally be investigated with a full work-up, comprehensive of brain MRI with TOF angiography since the first attack. In cases with negative brain MRI spontaneous resolution should be considered and watchful waiting might be advisable before starting steroid therapy.


Subject(s)
Ophthalmoplegia , Tolosa-Hunt Syndrome , Humans , Adolescent , Tolosa-Hunt Syndrome/complications , Tolosa-Hunt Syndrome/diagnosis , Tolosa-Hunt Syndrome/therapy , Pain , Magnetic Resonance Imaging , Headache , Paresis/complications
9.
Cureus ; 15(10): e46635, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37936989

ABSTRACT

Tolosa-Hunt syndrome (THS) is an idiopathic inflammatory condition involving the cavernous sinus and orbital apex with an incidence of 1 case per million per year. We report on a case of a 70-year-old male with atypical MRI findings, vision loss, and painless ophthalmoplegia.  Ophthalmic evaluation revealed his best-corrected visual acuity was 20/40 in the right eye and counting fingers at a 0.5-foot distance in the left eye. External examination of the left eye revealed limited ocular movement, proptosis, and a positive relative afferent pupillary defect. Complete blood count, inflammatory markers, and full biochemistry tests, including thyroid and liver function tests, were within the normal range. A magnetic resonance imaging of the orbits with and without contrast demonstrated a homogenously enhancing lesion at the posterior intraconal compartment of the left orbit, extending to the orbital apex with the involvement of the adjacent extraocular muscles. The patient was started on intravenous methylprednisolone 60 mg daily and later discharged on prednisone 5 mg daily with partial symptom improvement on follow-up. Resection and biopsy revealed a soft tissue lesion with mixed inflammatory infiltrate. The clinical, pathological, and imaging findings favored the diagnosis of THS.

10.
Curr Pain Headache Rep ; 27(12): 843-849, 2023 Dec.
Article in English | MEDLINE | ID: mdl-38032539

ABSTRACT

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.


Subject(s)
Cavernous Sinus , Tolosa-Hunt Syndrome , Humans , Tolosa-Hunt Syndrome/diagnosis , Tolosa-Hunt Syndrome/therapy , Tolosa-Hunt Syndrome/complications , Diagnosis, Differential , Cavernous Sinus/pathology , Magnetic Resonance Imaging , Steroids
11.
Korean J Fam Med ; 44(4): 240-243, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37491988

ABSTRACT

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.

12.
Clin J Gastroenterol ; 16(3): 450-456, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37014540

ABSTRACT

Crystal-storing histiocytosis (CSH) is a rare disease associated with the accumulation of histiocytes containing crystalline matter within their cytoplasm. Herein, we present the case of a female patient who was diagnosed with Tolosa-Hunt syndrome at 45 years of age and idiopathic retroperitoneal fibrosis when she was 48 years. She developed portal hypertension (PH), but did not present with cirrhosis; as such, the cause of PH was not identified. Her PH gradually worsened when she was 54 years, and at the age of 60 years, she died from an acute subdural hematoma. Autopsy revealed retroperitoneal fibrosis with severe fibrosis extending around the hepatic veins and into the porta hepatis. Histologically, the retroperitoneal tissue showed a dense infiltrate of eosinophilic histiocytes with crystal structures in the cytoplasm, which was pathologically diagnosed as CSH. Nodular regenerative hyperplasia was observed in the liver parenchyma, whereas cirrhosis was not. In the present case, CSH caused fibrosis, which was believed to be the cause of PH. In addition, we considered that nodular regenerative hyperplasia caused by the altered hepatic blood flow due to treatment of gastric varices contributed to worsening PH. Hence, CSH should be considered as an underlying disease in noncirrhotic portal hypertension.


Subject(s)
Histiocytosis , Hypertension, Portal , Humans , Female , Middle Aged , Autopsy , Hyperplasia , Rare Diseases/complications , Hypertension, Portal/etiology , Liver Cirrhosis/complications , Histiocytosis/complications , Histiocytosis/pathology
13.
BMC Neurol ; 23(1): 25, 2023 Jan 17.
Article in English | MEDLINE | ID: mdl-36650509

ABSTRACT

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.


Subject(s)
Ophthalmoplegia , Sinusitis , Sphenoid Sinusitis , Humans , Female , Adult , Sphenoid Sinusitis/diagnosis , Sphenoid Sinusitis/diagnostic imaging , Magnetic Resonance Imaging , Sinusitis/complications , Ophthalmoplegia/diagnosis , Steroids/therapeutic use
14.
Radiol Case Rep ; 18(3): 1353-1357, 2023 Mar.
Article in English | MEDLINE | ID: mdl-36712191

ABSTRACT

Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia due to non-specific granulomatous inflammation in the cavernous sinus region. It is diagnosed by the International Classification of Headache Disorders (ICHD)-3 criteria. We report the case of a young lady who presented with a right-sided headache for 2 weeks, followed by right-sided diplopia for 4 days. Clinical examination revealed right trochlear nerve palsy. Magnetic resonance imaging (MRI) of her brain showed abnormal thickening and postcontrast enhancement of the right orbital apex and superior orbital fissure, suggesting THS. Examination of cerebrospinal fluid (CSF) ruled out intracranial infection. The initial presentation satisfied the ICHD-3 criteria. Further imaging revealed cervical, axillary, and intra-abdominal lymphadenopathy with granulomatous lesions in the spleen and right kidney. Ultrasound (US)-guided axillary lymph node biopsy was positive for Mycobacterium tuberculosis. QuantiFERON TB gold plus test from serum was positive. Based on radiological and histopathological findings, a diagnosis of disseminated tuberculosis involving lymph nodes, kidneys, spleen, and lungs was made. THS is a diagnosis of exclusion. This case signifies that patients diagnosed with THS based on ICHD-3 criteria should be extensively evaluated to rule out granulomatous infections such as tuberculosis. Typical THS symptoms with granulomatous inflammation can give false reassurance to clinicians and prevent investigation for more dangerous etiologies. As painful ophthalmoplegia can arise secondary to a myriad of pathologies, diagnostic workups for all possibilities should be exhausted before arriving at a diagnosis of THS. Regardless of MRI findings, workups for tuberculosis and fungal infections should be completed.

15.
Eur J Ophthalmol ; 33(3): NP45-NP48, 2023 May.
Article in English | MEDLINE | ID: mdl-35001696

ABSTRACT

PURPOSE: to describe a rare case of orbital apex syndrome caused by aspergillosis with acute presentation. CASE DESCRIPTION: retrospective case report of a 70-year-old man who developed unilateral ophthalmoplegia overnight. He was initially given the diagnosis of suspect Tolosa-Hunt syndrome, but biopsy of the involved tissue showed aspergillosis. CONCLUSION: orbital apex syndrome caused by fungal disease is a life-threatening condition that should be promptly diagnosed and treated. It may present acutely and should not be misdiagnosed as Tolosa-Hunt syndrome. To our knowledge this is the first such case report in the English ophthalmic language Literature.


Subject(s)
Aspergillosis , Ophthalmoplegia , Tolosa-Hunt Syndrome , Male , Humans , Aged , Tolosa-Hunt Syndrome/diagnosis , Retrospective Studies , Aspergillosis/complications , Aspergillosis/diagnosis , Aspergillosis/drug therapy , Ophthalmoplegia/diagnosis , Ophthalmoplegia/etiology , Diagnosis, Differential , Magnetic Resonance Imaging
16.
J Yeungnam Med Sci ; 40(2): 198-201, 2023 Apr.
Article in English | MEDLINE | ID: mdl-35272433

ABSTRACT

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.

17.
Neurol India ; 70(6): 2422-2426, 2022.
Article in English | MEDLINE | ID: mdl-36537427

ABSTRACT

Background: Hypertrophic pachymeningitis (HPM) is a unique disorder characterized by thickening and fibrosis of the dura mater. Clinically it presents with headache, cranial nerve palsies, and other focal neurological deficits. Two forms exist, one is primary, where all other causes have been excluded and the other is secondary where an identifiable cause exists. It is important to recognize these secondary causes as treatment depends on the etiology. Objective: To elucidate the various characteristics of HPM. To delineate clinical-radiological features that help differentiate secondary from primary causes and to understand treatment response and disease outcomes of HPM. Methods: This retrospective observational study included 33 patients who presented with radiological diagnosis of HPM from January 2014 to July 2019. Spontaneous intracranial hypotension patients were excluded. All patients were extensively evaluated for secondary causes and treatment outcomes were analyzed on follow-up. Results and Conclusions: Secondary causes of HPM were present in 48% cases. The clue for primary causes is an associated Tolosa-Hunt syndrome. Secondary causes in our series are immunological, infection, and malignancy. Clues to differentiate primary from these secondary causes are clinical like myelopathy, seizures, poor response to immunosuppression; radiological like hypertrophic cranial nerves, infarcts, bony erosion, and leptomeningeal involvement. There are case reports in literature but large Indian studies are lacking. This manuscript presents a large cohort of cases with HPM, which helps differentiate primary from secondary causes, as management and prognosis depend on etiology. An algorithm depicting the approach to the management of HPM has been presented.


Subject(s)
Cranial Nerve Diseases , Meningitis , Humans , Magnetic Resonance Imaging , Meningitis/etiology , Cranial Nerve Diseases/etiology , Headache/complications , Treatment Outcome , Hypertrophy/pathology , Dura Mater/pathology
18.
Acta neurol. colomb ; 38(4): 219-223, oct.-dic. 2022. graf
Article in Spanish | LILACS | ID: biblio-1419936

ABSTRACT

RESUMEN INTRODUCCIÓN: El síndrome de Tolosa-Hunt (STH) se caracteriza por una oftalmoplejía dolorosa, de etiología desconocida. De acuerdo con los hallazgos histopatológicos, se ha descrito la formación de un tejido granulomatoso en los senos cavernosos. PRESENTACIÓN DEL CASO: Una mujer de 22 años con cuadro clínico de 3 semanas de evolución caracterizado por cefalea hemicránea derecha, dolor ocular derecho y diplopía. Su examen físico evidenció la existencia de una oftalmoplejía derecha; la resonancia magnética (RM) de silla turca demostró engrosamiento y realce en la región del seno cavernoso derecho. Se presenta el caso clínico de una causa inusual de oftalmoplejía dolorosa. DISCUSIÓN: La oftalmoplejía dolorosa tiene múltiples diagnósticos diferenciales que incluyen causas neoplá-sicas, vasculares, inflamatorias e infecciosas que pueden afectar el seno cavernoso o la fisura orbitaria superior. El STH, que es una causa rara de oftalmoplejía dolorosa, sigue siendo un diagnóstico de exclusión. Por otra parte, se caracteriza por tener una adecuada respuesta al tratamiento con glucocorticoides. CONCLUSIÓN: La negatividad en las investigaciones de las etiologías de oftalmoplejía, los hallazgos imagenológicos en la RM y la adecuada respuesta cínica con el uso de los corticoides permiten confirmar el diagnóstico. No debería ser necesaria la biopsia del seno cavernoso ante la sospecha de STH con adecuada respuesta al manejo corticoide.


ABSTRACT INTRODUCTION: Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia of unknown etiology, the formation of a granulomatous tissue in the cavernous sinuses has been described in histopatho-logical findings. CASE PRESENTATION: A 22-year-old woman presenting with 3 weeks of right sided headache, right eye pain and diplopia. Physical examination revealed the existence of a right ophthalmoplegia; magnetic resonance imaging (MRI) of the sella turcica showed thickening and enhancement of the right cavernous sinus. A clinical case of an unusual cause of painful ophthalmoplegia is presented. DISCUSSION: Painful ophthalmoplegia has multiple differential diagnoses that include neoplastic, vascular, inflammatory and infectious causes that can affect the cavernous sinus or the superior orbital fissure. STH is a rare case of painful ophthalmoplegia that continues to be a diagnosis of exclusion characterized by an adequate response to treatment with glucocorticoids. CONCLUSION: The negativity of the investigations for the causes of ophthalmoplegia, the imaging findings in the MRI and the adequate response to corticosteroids allows the diagnosis to be made. Biopsy should not be necessary when THS is suspected and there is an adequate response to corticosteroid management.


Subject(s)
Ophthalmoplegia , Tolosa-Hunt Syndrome , Pain , Cavernous Sinus , Diplopia
19.
Eur J Neurol ; 29(10): 3127-3129, 2022 10.
Article in English | MEDLINE | ID: mdl-36073199

ABSTRACT

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.


Subject(s)
Actinomycosis , Cavernous Sinus , Ophthalmoplegia , Tolosa-Hunt Syndrome , Actinomycosis/complications , Actinomycosis/pathology , Cavernous Sinus/diagnostic imaging , Cavernous Sinus/pathology , Humans , Male , Middle Aged , Ophthalmoplegia/diagnosis , Ophthalmoplegia/etiology , Ophthalmoplegia/pathology , Tolosa-Hunt Syndrome/complications , Tolosa-Hunt Syndrome/diagnosis , Tolosa-Hunt Syndrome/pathology
20.
Cureus ; 14(6): e26093, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35875300

ABSTRACT

Tolosa-Hunt syndrome (THS) is an extremely rare disorder characterized by painful unilateral ophthalmoplegia triggered by idiopathic inflammation of the cavernous sinus affecting the third, fourth, and/or sixth cranial nerve. Corticosteroid therapy effectively improves THS symptoms; thus, early clinical suspicion and diagnosis are essential. We report the case of a 37-year-old patient who presented with left-sided eye pain and double vision for four days. Physical examination was significant for oculomotor, trochlear and abducent nerves palsies on the left eye with slow light reflex. Contrast-enhanced magnetic resonance imaging of the head displayed mild asymmetry of the cavernous sinus regions with fullness on the left side and focal lateral thickening, confirming the THS diagnosis. The patient's symptoms improved dramatically upon starting oral corticosteroid therapy.

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