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.

Tolosa-Hunt syndrome relapse during pregnancy following chronic intake of combined oral contraceptives.

Tolosa-Hunt syndrome (THS) is a rare syndrome of painful ophthalmoplegia secondary to an idiopathic granulomatous inflammation affecting the cavernous sinus, superior orbital fissure or orbit. Pregnancy and pregnancy-related hormones have been identified as potential triggers. A 39-year-old gravida-2 para-1 woman with prior chronic intake of combined oral contraceptives (COC) suffered two episodes of painful ophthalmoplegia-the first event with spontaneous remission and the relapse occurring during pregnancy and with complete resolution following steroid treatment. MRI revealed a postinflammatory mass at the junction of the left orbital apex and anterior cavernous sinus, supporting the diagnosis of THS. To our knowledge, this is the first report of a THS relapse occurring during pregnancy following a chronic history of COC intake. This case adds to the growing evidence supporting the relationship between immune and hormonal factors that may be present during pregnancy and the disease pathogenesis of THS.

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

INTRODUCCIÓN: La miositis orbitaria (MO) es un proceso inflamatorio grave de etiología desconocida que compro mete los músculos extraoculares. La presentación en edad pediátrica es rara y con frecuencia afecta a más de un individuo de una familia, lo que sugiere algún grado de predisposición genética. OBJETIVO: Describir un caso de miositis orbitaria de presentación en edad pediátrica, sus características clínicas, y la utilidad de la imagen por resonancia magnética para la confirmación del diagnóstico. CASO CLÍNICO: Paciente femenina de 13 años que presenta cefalea aguda, dolor periorbitario derecho, exacerbado con los movimientos oculares y visión borrosa a quien se le realizaron estudios para miopatía tiroidea, enfermedades infecciosas, autoinmunidad y cáncer que fueron negativos. En la imagen por resonancia magnética se evidenció miositis del músculo recto medio derecho, sin evi dencia de neuritis óptica. Recibió tratamiento con glucocorticoides sistêmicos intravenosos seguido de esteroides orales con mejoría clínica completa. CONCLUSIONES: La MO tiene etiología desconocida, y puede tener un curso maligno. Dada su presentación clínica inespecífica, el estudio diagnóstico diferencial debe ser amplio, y su estudio debe considerar realizar resonanacia magnética. El inicio temprano del tratamiento con esteroides evita el daño permanente de los músculos extraoculares.

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.

[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.

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.

[Cavernous sinus syndrome as the initial symptom of Burkitt's lymphoma: a case report and literature review]. / Sindrome del seno cavernoso como inicio de un linfoma de Burkitt: a proposito de un caso y revision de la bibliografia.

INTRODUCTION: The cavernous sinus is a structure in the base of the skull that houses several nerve and vascular structures. Its compromise leads to cavernous sinus syndrome, which is a combination of oculomotor disorders and others affecting the first two trigeminal branches, often accompanied by pain or proptosis. Infiltration due to Burkitt's lymphoma is a rare cause of this syndrome. CASE REPORT: A 43-year-old male, carrier of human immunodeficiency virus, with good control of the disease, who developed a clinical picture consisting of progressive painful ophthalmoplegia in the presence of a laterocervical adenopathy. Complementary tests allowed a diagnosis of Burkitt's lymphoma with extranodal extension to the cavernous sinus. A review of the cases published in Medline was also carried out: a total of 15 cases were detected and their epidemiological characteristics, form of presentation, extracranial involvement at the time of diagnosis and clinical progression were described. CONCLUSIONS: Burkitt's lymphoma is a high-grade lymphoproliferative syndrome. Its form associated with immunodeficiency is an important cause of morbidity and mortality in this subgroup of patients. In the cases analysed in the literature, the age of presentation varied and the form of onset was a progressive painful ophthalmoplegia or numb chin syndrome. Exclusive involvement of the cavernous sinus was infrequent, but in that case it entailed a poor prognosis. It is important to rule out a primary extracranial origin and not to confuse it with an idiopathic Tolosa-Hunt syndrome that would delay the beginning of antitumour treatment.

TITLE: Sindrome del seno cavernoso como inicio de un linfoma de Burkitt: a proposito de un caso y revision de la bibliografia.Introduccion. El seno cavernoso es una estructura de la base craneal que alberga varias estructuras nerviosas y vasculares. De su afectacion se deriva el sindrome del seno cavernoso, una combinacion de alteraciones oculomotoras y de las dos primeras ramas del trigemino, frecuentemente acompañadas de dolor o proptosis. La infiltracion por un linfoma de Burkitt es una causa infrecuente de este sindrome. Caso clinico. Varon de 43 años, portador del virus de la inmunodeficiencia humana, con buen control de la enfermedad, que desarrolla un cuadro consistente en oftalmoplejia dolorosa progresiva en presencia de adenopatia laterocervical. Las pruebas complementarias permiten el diagnostico de linfoma de Burkitt con extension extranodal al seno cavernoso. Tambien se realiza una revision de los casos publicados en Medline: se recogen un total de 15 casos y se describen sus caracteristicas epidemiologicas, forma de presentacion, afectacion extracraneal en el momento del diagnostico y evolucion clinica. Conclusiones. El linfoma de Burkitt es un sindrome linfoproliferativo de alto grado. Su forma asociada a inmunodeficiencia es una importante causa de morbimortalidad en este subgrupo de pacientes. En los casos analizados en la bibliografia, la edad de presentacion fue variable y la forma de inicio fue una oftalmoplejia dolorosa progresiva o un sindrome de numb chin. Fue infrecuente una afectacion exclusiva del seno cavernoso, pero en ese caso conllevaba un mal pronostico. Es importante descartar un origen primario extracraneal y no confundirlo con un sindrome de Tolosa-Hunt idiopatico que retrasaria el inicio del tratamiento antitumoral.

Hipofisitis tuberculosa que mimetiza un síndrome de Tolosa-Hunt / Tuberculous hypophysitis mimicking Tolosa-Hunt syndrome

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Validation of ICHD-3 beta diagnostic criteria for 13.7 Tolosa-Hunt syndrome: Analysis of 77 cases of painful ophthalmoplegia.

BACKGROUND: Three editions of International Classification of Headache Disorders (ICHD) diagnostic criteria for Tolosa-Hunt syndrome (THS) have been published in 1988, 2004 and 2013, in ICHD-3 beta, there have been considerable changes [corrected]. The validity of these new diagnostic criteria remains to be established. METHODS: We retrospectively identified 77 patients with non-traumatic painful ophthalmoplegia (PO) admitted between 2003 and 2013. We reviewed patients' age at onset and gender, time courses between onset of pain and development of cranial nerve palsy, the cranial nerves involved, imaging findings, therapeutic efficacy of steroid treatment and recurrence of attacks. RESULTS: THS was the most frequent type of PO (46/77). In THS patients, the third cranial nerve was most commonly involved (76.3%). The median time interval between pain and cranial nerve palsy was two days, although in five patients (10.9%) the interval ranged from 16 to 30 days. Definitely abnormal MRI findings were found in 24 patients (52.2%). CONCLUSIONS: It is essential to rule out other causes of PO in diagnosing THS, with MRI playing a crucial role in differential diagnosis. It may be helpful to understand and master the entity of THS for researchers and clinicians to adjust the gradation and ranking of the diagnostic criteria.

Tolosa-hunt syndrome associated with cytomegalovirus infection.

We herein present the case of a 38-year-old woman with left-sided oculomotor paralysis with ocular pain that developed after a respiratory infection. Her serum was positive for IgM against GM2 and GalNAc-GD1a gangliosides and cytomegalovirus. Thin-slice magnetic resonance imaging revealed enhanced abnormal tissue located primarily in the superolateral part of the left-sided cavernous sinus, which corticosteroids subsequently obscured with immediate resolution of the patient's ocular symptoms. These clinical features were consistent with those of Tolosa-Hunt syndrome (THS). Our findings in the present patient suggest that cytomegalovirus may provoke granuloma formation in the cavernous sinus, as reported in other various organs, thereby leading to the development of THS.

Painful ophthalmoplegia with simultaneous orbital myositis, optic and oculomotor nerve inflammation and trigeminal nucleus involvement in a patient with herpes zoster ophthalmicus.

Viral infection is a rare cause of painful ophthalmoplegia. We report on a 67-year-old patient who developed painful double vision after a vesicular skin rash on the left forehead. MRI disclosed simultaneous inflammatory lesions in all extraocular muscles, the second and third cranial nerve, as well as pathological signal intensity along the spinal trigeminal tract and nucleus within the medulla oblongata and the pons. Cerebrospinal fluid and serum tests for varicella zoster were positive. The patient was treated effectively with intravenous acyclovir and methylprednisolone. Simultaneous lesions in various neighbouring neural structures may be characteristic for the highly neurotropic behaviour of the herpesviridae and should be considered as a cause of painful ophthalmoplegia that can be depicted by appropriate imaging.

Tolosa-Hunt syndrome--diagnostic problem of painful ophthalmoplegia.

BACKGROUND: Tolosa-Hunt syndrome (THS) is an uncommon disease caused by non-specific inflammation of the cavernous sinus, superior orbital fissure and the apex of the orbit. The disease is characterized by periorbital pain, paresis of the bulbomotor and quick response to steroid treatment. The orbital process may lead to optic nerve atrophy. According to the International Headache Society Classification of 2004, the diagnostic protocol includes magnetic resonance imaging (MRI) and biopsy. CASE REPORTS: We presented 46-year old male patient, with THS. The patient had unilateral periorbital pain, inflammatory process in the cavernous sinus, the apex of the orbit and the paranasal sinuses. Inflammatory process had spread into the fascia of the bulbomotor and performed compression to the optic nerve, causing paresis of the bulbomotor, protrusion of the eyeball and atrophy of the optic nerve. Pulse doses of corticosteroids were effective. Regarding the presented patient, diagnostic dilemmas arose from nonspecific sinusitis. The initial ophthalmological diagnosis, based on periorbital pain, drop in visual acuity and the narrow chamber angle was angular glaucoma, which resulted in a delayed diagnosis of THS and the beginning of the treatment. MRI and positive response to the treatment with corticosteroids were relevant for making the diagnosis. CONCLUSION: According to the International Headache Society Classification of 2004, THS is an entity that occurs rarely, its etiopathogenesis is unknown, it is manifested clinically by unilateral orbital pain associated with simple or multiple oculomotor paralyses, which resolves spontaneously but may recur. MRI orbital phlebography and biopsy are the recommended methods for making diagniosis. In our patient MRI findings and positive response to the corticosteroide treatment were relevant for making the diagnosis.

[Tolosa-Hunt syndrome following traumatic eye injury]. / Sindrome de Tolosa-Hunt tras un traumatismo ocular.

INTRODUCTION: Tolosa-Hunt syndrome (THS) is an idiopathic condition involving unilateral eye pain with involvement of oculomotor nerves which responds well to treatment with steroids. It is produced by idiopathic granulomatous inflammation of the cavernous sinus or the orbital apex. CASE REPORT: A 37-year-old male who was admitted to hospital due to a six-week history of blurred vision and pain in the left eye, which was later accompanied by full ipsilateral ophthalmoplegia. Some days prior to the onset of the clinical features, he suffered an accident which resulted in traumatic injury to the left eye. An examination showed data pointing to optic neuropathy in the left eye with complete extrinsic ocular motor palsy. Results of general analyses and lumbar puncture were normal. Magnetic resonance imaging (MRI) of the head revealed a thickening of the wall of the left cavernous sinus and of the ipsilateral orbital apex, which enhanced with gadolinium. Evoked potential studies showed axonal and demyelinating optic neuropathy on the left-hand side. Suspecting this to be a case of THS, treatment was established with high doses of corticoids, which brought about an improvement in the pain and eye movement but not in the blurred vision. A MRI control scan showed a clear improvement in comparison to the one carried out initially. CONCLUSIONS: In cases of painful ophthalmoplegia, the professional should suspect the existence of THS. If the apex of the orbit is affected through the superior orbital fissure, the optic nerve may be damaged. Traumatic injury can be one of the situations that trigger THS.

Síndrome de Tolosa-Hunt tras un traumatismo ocular / Tolosa-Hunt syndrome following traumatic eye injury

Introducción. El síndrome de Tolosa-Hunt (STH) es un cuadro idiopático de dolor orbital unilateral con afectación de nervios oculomotores con buena respuesta al tratamiento esteroideo. Se produce por la inflamación granulomatosa idiopática del seno cavernoso o del ápex orbitario. Caso clínico. Varón de 37 años que ingresó por un cuadro de seis semanas de evolución de visión borrosa y dolor ocular izquierdo al que posteriormente se le sumó una oftalmoplejía completa ipsilateral. Días antes de comenzar la clínica sufrió un traumatismo ocular accidental en el ojo izquierdo. La exploración mostró datos de neuropatía óptica izquierda junto con plejía completa de motores oculares extrínsecos. La analítica general y la punción lumbar fueron normales. La resonancia magnética (RM) craneal evidenció un engrosamiento de la pared del seno cavernoso izquierdo y del ápex orbitario ipsilateral, que realzaba con gadolinio. El estudio de potenciales evocados mostró una neuropatía óptica izquierda axonal y desmielinizante. Ante la sospecha de STH se inició tratamiento con alta dosis de corticoides, tras lo cual se produjo una mejoría del dolor y de la motilidad ocular, pero no de la visión borrosa. La RM de control reveló una evidente mejoría respecto a la inicial. Conclusiones. Ante una oftalmoplejía dolorosa debe sospecharse la existencia de un STH. Si se afecta el ápex de la órbita a través de la fisura orbitaria superior puede verse perjudicado el nervio óptico. Uno de los desencadenantes del STH puede ser un traumatismo (AU)

Introduction. Tolosa-Hunt syndrome (THS) is an idiopathic condition involving unilateral eye pain with involvement of oculomotor nerves which responds well to treatment with steroids. It is produced by idiopathic granulomatous inflammation of the cavernous sinus or the orbital apex. Case report. A 37-year-old male who was admitted to hospital due to a six-week history of blurred vision and pain in the left eye, which was later accompanied by full ipsilateral ophthalmoplegia. Some days prior to the onset of the clinical features, he suffered an accident which resulted in traumatic injury to the left eye. An examination showed data pointing to optic neuropathy in the left eye with complete extrinsic ocular motor palsy. Results of general analyses and lumbar puncture were normal. Magnetic resonance imaging (MRI) of the head revealed a thickening of the wall of the left cavernous sinus and of the ipsilateral orbital apex, which enhanced with gadolinium. Evoked potential studies showed axonal and demyelinating optic neuropathy on the left-hand side. Suspecting this to be a case of THS, treatment was established with high doses of corticoids, which brought about an improvement in the pain and eye movement but not in the blurred vision. A MRI control scan showed a clear improvement in comparison to the one carried out initially. Conclusions. In cases of painful ophthalmoplegia, the professional should suspect the existence of THS. If the apex of the orbit is affected through the superior orbital fissure, the optic nerve may be damaged. Traumatic injury can be one of the situations that trigger THS (AU)

[Recurrent painful ophthalmoplegia secondary to polyostotic fibrous dysplasia of the maxillary sinuses with involvement of the superior orbital fissure]. / Oftalmoplejia dolorosa recurrente secundaria a displasia fibrosa poliostotica del seno maxilar con afectacion de la hendidura esfenoidal.

INTRODUCTION: Fibrous dysplasia is a bone disease that is usually accompanied by asymptomatic lesions but which may sometimes display neurological manifestations due to the involvement of the craniofacial bones. CASE REPORT: A 25-year-old female, with a history of migraines, who visited at the age of 18 due to pain in the right retro-ocular and maxillary region, although with characteristics that were unlike those of her usual migraines, and which was associated with ipsilateral ophthalmoparesis. The condition had a self-limiting course and responded well to corticoids, although it was recurring. Examination revealed intense pain on palpation of the right-hand maxillary sinus and incomplete paralysis of the ipsilateral oculomotor nerve with palpebral ptosis. Results of complementary studies were normal, except for magnetic resonance imaging of the head and computerised axial tomography of the face, which revealed an expansive lesion with involvement of the right superior maxillary sinus and the greater wing of the sphenoid bone, with probable compromise of the superior orbital fissure, consistent with the diagnosis of fibrous dysplasia, which was confirmed by means of a pathology study. CONCLUSIONS: Fibrous dysplasia is a benign bone disorder, of unknown causation, in which normal bone tissue is replaced by amorphous conjunctive tissue. There is sometimes craniofacial involvement and a hypertrophic bone mass is formed which can fill the paranasal sinuses and the orbit, resulting in exophthalmus and visual disorders. To date the scientific literature does not include any reports of this disease manifesting as bouts of recurrent painful ophthalmoparesis which responds to corticoids, as happened in the case of our patient.