RESUMEN
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.
Asunto(s)
Seno Cavernoso , Síndrome de Tolosa-Hunt , Humanos , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/terapia , Síndrome de Tolosa-Hunt/complicaciones , Diagnóstico Diferencial , Seno Cavernoso/patología , Imagen por Resonancia Magnética , EsteroidesRESUMEN
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.
Asunto(s)
Oftalmoplejía , Síndrome de Tolosa-Hunt , Humanos , Adolescente , Síndrome de Tolosa-Hunt/complicaciones , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/terapia , Dolor , Imagen por Resonancia Magnética , Cefalea , Paresia/complicacionesRESUMEN
Resumo A síndrome de Tolosa-Hunt (STH) é uma doença rara caracterizada por oftalmoplegia dolorosa unilateral de início súbito causada por uma inflamação granulomatosa inespecífica no seio cavernoso ou fissura orbital superior (ou ambos). A oftalmoparesia ocorre quando os nervos cranianos III, IV e VI são acometidos pela inflamação. Disfunções pupilares podem estar presentes e está relacionado com acometimento das fibras simpáticas que passam pelo seio cavernoso na porção da artéria carótida interna ou fibras parassimpáticas ao redor do nervo oculomotor. O acometimento do primeiro ramo do trigêmeo pode provocar parestesia território correspondente à distribuição desde ramo (testa). Raramente, pode haver extensão da inflamação para além do seio cavernoso ou fissura orbital superior podendo acometer também o nervo óptico. Há uma boa resposta com o uso de corticoides e pode haver remissões espontâneas. Recidivas ocorrem em 40% dos casos. A doença é mais comum após a segunda década de vida. Afeta ambos os gêneros de forma igualitária. O presente estudo trata-se de um relato de caso de um paciente que se apresentou com oftalmoplegia dolorosa de início súbito à direita com 4 dias de evolução seguido de amaurose ipslateral após um dia do início da dor.
Abstract Tolosa-Hunt syndrome (STH) is a rare disease characterized by sudden onset unilateral painful ophthalmoplegia caused by non-specific granulomatous inflammation in the cavernous sinus or superior orbital fissure (or both). Ophthalmoparesis occurs when the cranial nerves III, IV and VI are affected by inflammation. Pupillary dysfunctions may be present and is related to involvement of the sympathetic fibers that pass through the cavernous sinus in the portion of the internal carotid artery or parasympathetic fibers around the oculomotor nerve. The involvement of the first branch of the trigeminal can cause paresthesia corresponding to the distribution from the first branch (forehead). Rarely, there may be extension of inflammation beyond the cavernous sinus or superior orbital fissure and may also affect the optic nerve. There is a good response with the use of corticosteroids and there may be spontaneous remissions. Relapses occur in 40% of cases. The disease is most common after the second decade of life. It affects both genders equally. The present study is a case report of a patient who presented with painful ophthalmoplegia of sudden onset on the right with 4 days of evolution followed by ipsilateral amaurosis after one day of onset of pain.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Dolor , Oftalmoplejía/diagnóstico , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/terapia , Prednisona/uso terapéutico , Imagen por Resonancia Magnética , Seno Cavernoso/patología , Seno Cavernoso/diagnóstico por imagen , Clasificación Internacional de Enfermedades , Nervios Craneales/diagnóstico por imagen , Síndrome de Tolosa-Hunt/clasificación , Diagnóstico Diferencial , CefaleaRESUMEN
The authors report a rare case of bilateral Tolosa-Hunt syndrome, which occurred in a 80-year-old female and remitted spontaneously. Inflammatory lesions were found not only in typical locations, i.e. superior orbital fissures and cavernous sinuses, but also in the pituitary; these imitated gland's macroadenoma in imaging studies.
Asunto(s)
Neoplasias Hipofisarias/diagnóstico , Síndrome de Tolosa-Hunt/diagnóstico , Anciano de 80 o más Años , Diagnóstico Diferencial , Párpados/diagnóstico por imagen , Párpados/patología , Femenino , Hormonas/sangre , Humanos , Imagen por Resonancia Magnética , Órbita/diagnóstico por imagen , Silla Turca/diagnóstico por imagen , Síndrome de Tolosa-Hunt/diagnóstico por imagen , Síndrome de Tolosa-Hunt/terapiaRESUMEN
BACKGROUND: Tolosa-Hunt syndrome is a painful ophthalmoplegia caused by an inflammatory process of unknown etiology in the region of the cavernous sinus, orbital apex, or superior orbital fissure. This disease is rare in the pediatric population. The objective of this study was to provide a clinical framework for the evaluation and treatment of children with this disorder. A systematic approach to the diagnosis of painful ophthalmoplegia in children is proposed. METHODS: We present a 15-year-old girl whose clinical presentation and neuroradiological findings support a diagnosis of Tolosa-Hunt syndrome as defined by the 2013 International Classification of Headache Disorders (Third Edition, ICHD-3 beta) diagnostic criteria. An exhaustive systematic literature search based on these criteria yielded 15 additional cases of Tolosa-Hunt syndrome in children. Clinical, demographic, and radiological features were retrospectively analyzed. The results and statistical analyses are reported. RESULTS: A total of 16 individuals were included in the final analysis. This review summarizes the current knowledge and recommendations for the diagnosis and management of pediatric Tolosa-Hunt syndrome. It highlights demographic, clinical, and radiological features of this disease in children and underscores areas of the literature where evidence is still lacking. CONCLUSIONS: Overall, Tolosa-Hunt syndrome seems to follow a similar course in children compared to adults. The diagnostic approach and treatment require specific considerations. New observations and possible features of pediatric Tolosa-Hunt syndrome are discussed. Further research is needed to optimize clinical detection and medical management of this disease.
Asunto(s)
Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/terapia , Adolescente , Diagnóstico Diferencial , Manejo de la Enfermedad , Femenino , HumanosRESUMEN
BACKGROUND: Painful ophthalmoplegia with normal cranial imaging is rare and confined to limited etiologies. In this study, we aimed to elucidate these causes by evaluating clinical presentations and treatment responses. METHODS: Cases of painful ophthalmoplegia with normal cranial MRI at a single center between January 2001 and June 2011 were retrospectively reviewed. Diagnoses of painful ophthalmoplegia were made according to the recommendations of the International Headache Society. RESULTS: Of the 58 painful ophthalmoplegia cases (53 patients), 26 (44.8%) were diagnosed as ocular diabetic neuropathy, 27 (46.6%) as benign Tolosa-Hunt syndrome (THS), and 5 (8.6%) as ophthalmoplegic migraine (OM). Patients with ocular diabetic neuropathy were significantly older (62.8 ± 7.8 years) than those with benign THS (56.3 ± 12.0 years) or OM (45.8 ± 23.0 years) (p < 0.05). Cranial nerve involvement was similar among groups. Pupil sparing was dominant in each group. Patients with benign THS and OM responded exquisitely to glucocorticoid treatment with resolved diplopia, whereas patients with ocular diabetic neuropathy didn't (p < 0.05). Patients with OM recovered more rapidly than the other groups did (p < 0.05). Overall, most patients (94.8%) recovered completely during the follow-up period. CONCLUSIONS: Ocular diabetic neuropathy and benign THS accounted for most of the painful ophthalmoplegias in patients with normal cranial imaging. Patient outcomes were generally good.
Asunto(s)
Imagen por Resonancia Magnética , Dimensión del Dolor/métodos , Síndrome de Tolosa-Hunt/diagnóstico , Adulto , Anciano , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Síndrome de Tolosa-Hunt/terapia , Adulto JovenAsunto(s)
Humanos , Masculino , Anciano , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/terapia , Cefalea/complicaciones , Paresia/complicaciones , Paresia/diagnóstico , Granuloma de Células Gigantes/complicaciones , Granuloma de Células Gigantes/diagnóstico , Prednisona/uso terapéutico , Síndrome de Tolosa-Hunt/fisiopatología , Síndrome de Tolosa-Hunt , Granuloma de Células Gigantes/fisiopatología , Granuloma de Células GigantesRESUMEN
El clínico frente a una oftalmoplejia dolorosa, debe encarar diferentes alternativas diagnósticas, donde la disciplina semiológica y los estudios imagenológicos, son de capital importancia para arribar al diagnóstico. El síndrome de Tolosa-Hunt es una probable etiología, donde el uso de critérios diagnósticos, el estudio por resonancia magnética y la remisión con tratamiento corticoideo, son fundamentales para llegar al mismo, sabiendo que el seguimento evolutivo es de suma importancia para descartar los diagnósticos diferenciales.
Asunto(s)
Humanos , Adulto , Femenino , Oftalmoplejía/diagnóstico , Oftalmoplejía/etiología , Oftalmoplejía/terapia , Síndrome de Tolosa-Hunt/complicaciones , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/terapia , Síndrome de HornerRESUMEN
We report on a 7-year-old immunocompetent boy initially presenting with right-sided frontal cephalalgia, painful ophthalmoplegia, and ptosis for 1 month. Initial cerebrospinal fluid analysis produced normal results. Magnetic resonance imaging revealed an inflammatory pseudotumor of the right cavernous sinus after intravenous gadolinium administration, indicating a rare idiopathic inflammatory disorder of the cavernous sinus, i.e., Tolosa-Hunt syndrome. Ptosis and cephalalgia resolved after steroid treatment, although right-sided ophthalmoplegia remained. Breakthrough headache, associated with signs of meningeal irritation, developed 6 weeks later. Follow-up contrast-enhanced computed tomography revealed no enhancing cavernous soft tissue mass. A further lumbar puncture disclosed central nervous system infection with Staphylococcus saprophyticus. After 6 weeks of vancomycin, the headache resolved completely, and neuroimaging produced normal results. A diagnosis of Tolosa-Hunt syndrome should be rendered cautiously, because the etiology may involve a rare but not "idiopathic" infection. Moreover, if clinical signs are not fully responsive to steroid treatment, the underlying problems should receive careful investigation.
Asunto(s)
Meningitis Bacterianas/diagnóstico , Infecciones Estafilocócicas/diagnóstico , Staphylococcus saprophyticus , Síndrome de Tolosa-Hunt/diagnóstico , Niño , Humanos , Masculino , Meningitis Bacterianas/microbiología , Meningitis Bacterianas/terapia , Infecciones Estafilocócicas/microbiología , Infecciones Estafilocócicas/terapia , Síndrome de Tolosa-Hunt/microbiología , Síndrome de Tolosa-Hunt/terapiaRESUMEN
Tolosa-Hunt syndrome is a nonspecific granulomatous inflammation of the cavernous sinus, superior orbital fissure and apex of the orbit. It involves episodes of unilateral orbital pain which may last several weeks, lesions of cranial nerve III, IV or VI, and rarely II, V, VII and VIII. It is characterized by remissions and exacerbations, and can cause permanent neurological disorder of the affected nerves. We present the course of the disease in a patient whose initial symptom was gradual visual loss in the right eye, followed by neuralgic pain in the right orbit and face. The diagnosis was confirmed by biopsy and corticosteroid therapy was administered. Exacerbation of the disease required repeated surgery, excision of the fibrous tissue of the cavernous sinus, as well as prolonged corticosteroid therapy.
Asunto(s)
Síndrome de Tolosa-Hunt , Anciano , Humanos , Imagen por Resonancia Magnética , Masculino , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/patología , Síndrome de Tolosa-Hunt/terapiaAsunto(s)
Guías de Práctica Clínica como Asunto , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/terapia , Enfermedades del Nervio Abducens/diagnóstico , Enfermedades del Nervio Abducens/patología , Enfermedades del Nervio Abducens/terapia , Adolescente , Encéfalo/efectos de los fármacos , Encéfalo/patología , Femenino , Humanos , Imagen por Resonancia Magnética , Enfermedades del Nervio Oculomotor/diagnóstico , Enfermedades del Nervio Oculomotor/patología , Enfermedades del Nervio Oculomotor/terapia , Esteroides/efectos adversos , Esteroides/uso terapéutico , Síndrome de Tolosa-Hunt/patologíaRESUMEN
A 45-year-old female developed mild dysesthesia and swelling, followed by ptosisand trigeminal pain, in the right side of the face. Her past medical history was unremarkable, and she had not been aware of any infectious sign. A local otolaryngologist administered glucocorticoid therapy that resolved the face pain, but the ptosis persisted. Neurological examination found complete right oculomotor nerve paresis and mild sensory loss in the first and second segments of the right trigeminal nerve. Blood examination found no abnormalities. Neuroimaging revealed a saccular aneurysm at the branching site of the posterior communicating artery, projecting posteriorly and adjacent to the dorsum sellae, without other intracranial abnormalities. Cerebral angiography demonstrated poor opacification of the superior ophthalmic vein and cavernous sinus on the right side. The patient underwent coil embolization under a diagnosis of symptomatic aneurysm, but her oculomotor neuropathy was only partially improved. We thought that the impairment of the oculomotor function by inflammatory reaction in the cavernous sinus and mechanical compression by the aneurysm had already persisted for too long for post-treatment recovery. We think that the simultaneous occurrence of Tolosa-Hunt syndrome and oculomotor nerve palsy may have resulted because trigeminal neuralgia had increased the blood pressure to induce rapid growth of the preexisting aneurysm, or the inflammatory reaction in the cavernous sinus had promoted the growth of the aneurysm, or that the association was by chance.
Asunto(s)
Enfermedades de las Arterias Carótidas/complicaciones , Arteria Carótida Interna , Aneurisma Intracraneal/complicaciones , Enfermedades del Nervio Oculomotor/etiología , Síndrome de Tolosa-Hunt/etiología , Enfermedades de las Arterias Carótidas/diagnóstico , Enfermedades de las Arterias Carótidas/terapia , Diagnóstico por Imagen , Femenino , Humanos , Aneurisma Intracraneal/diagnóstico , Aneurisma Intracraneal/terapia , Persona de Mediana Edad , Enfermedades del Nervio Oculomotor/terapia , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/terapiaRESUMEN
The differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies, including neoplasms (ie, primary intracranial tumors, local or distant metastases), vascular (eg, aneurysm, carotid dissection, and carotid-cavernous fistula), inflammatory (ie, orbital pseudotumor, giant cell arteritis, sarcoidosis, and Tolosa-Hunt syndrome), and infectious etiologies (ie, fungal and mycobacterial), as well as other miscellaneous conditions (ie, ophthalmoplegic migraine and microvascular infarcts secondary to diabetes). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that can be associated with significant morbidity or mortality if left untreated. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids but should be diagnoses of exclusion.
Asunto(s)
Oftalmoplejía/etiología , Oftalmoplejía/terapia , Manejo del Dolor , Dolor/etiología , Síndrome de Tolosa-Hunt/complicaciones , Adulto , Humanos , Masculino , Oftalmoplejía/diagnóstico , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/terapiaAsunto(s)
Enfermedades Cerebelosas/patología , Ángulo Pontocerebeloso/patología , Síndrome de Tolosa-Hunt/patología , Corticoesteroides/uso terapéutico , Anciano , Seno Cavernoso/diagnóstico por imagen , Seno Cavernoso/patología , Enfermedades Cerebelosas/diagnóstico por imagen , Ángulo Pontocerebeloso/diagnóstico por imagen , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética , Síndrome de Tolosa-Hunt/fisiopatología , Síndrome de Tolosa-Hunt/terapia , Tomografía Computarizada por Rayos XRESUMEN
Painful ophthalmoplegia is an important presenting complaint to emergency departments, ophthalmologists, and neurologists. The etiological differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies including vascular (eg, aneurysm, carotid dissection, carotid-cavernous fistula), neoplasms (eg, primary intracranial tumors, local or distant metastases), inflammatory conditions (eg, orbital pseudotumor, sarcoidosis, Tolosa-Hunt syndrome), infectious etiologies (eg, fungal, mycobacterial), and other conditions (eg, microvascular infarcts secondary to diabetes, ophthalmoplegic migraine, giant cell arteritis). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that if left untreated, can be associated with significant morbidity or mortality. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids, but should be diagnoses of exclusion.
Asunto(s)
Oftalmoplejía/diagnóstico , Dolor/diagnóstico , Síndrome de Tolosa-Hunt/diagnóstico , Adulto , Diagnóstico Diferencial , Humanos , Masculino , Oftalmoplejía/etiología , Oftalmoplejía/terapia , Dolor/etiología , Manejo del Dolor , Síndrome de Tolosa-Hunt/terapiaRESUMEN
El síndrome de Tolosa-Hunt es una entidad neurológica que consiste en una oftalmoplejia dolorosa causada por una inflamación granulomatosa del seno cavernoso anterior. Se reporta un caso clínico de una paciente embarazada con esta rara enfermedad.
Asunto(s)
Humanos , Femenino , Embarazo , Adulto , Complicaciones del Embarazo/diagnóstico , Complicaciones del Embarazo/tratamiento farmacológico , Complicaciones del Embarazo/terapia , Síndrome de Tolosa-Hunt/diagnóstico , Síndrome de Tolosa-Hunt/epidemiología , Síndrome de Tolosa-Hunt/tratamiento farmacológico , Síndrome de Tolosa-Hunt/terapia , Trabajo de PartoRESUMEN
CASE REPORT: We present a case of metastatic larynx cancer in the cavernous sinus. It presented itself as a right sixth cranial nerve palsy which progressed into a painful ophthalmoplegia with normal CT and RNM in its early stages. DISCUSSION: We must suspect a cavernous sinus invasion in patients with cranial nerve palsies and with a history of malignancy, despite negative radiologic findings. Tumoral invasion of the skull base has been described in pharyngeal neoplasms but it is exceptional in larynx carcinomas.
Asunto(s)
Carcinoma de Células Escamosas/secundario , Seno Cavernoso/patología , Neoplasias de los Nervios Craneales/secundario , Neoplasias Laríngeas/patología , Oftalmoplejía/etiología , Enfermedades del Nervio Abducens/etiología , Enfermedades del Nervio Abducens/terapia , Anciano , Carcinoma de Células Escamosas/radioterapia , Terapia Combinada , Neoplasias de los Nervios Craneales/radioterapia , Humanos , Neoplasias Laríngeas/terapia , Imagen por Resonancia Magnética , Masculino , Invasividad Neoplásica , Oftalmoplejía/terapia , Cuidados Paliativos , Síndrome de Tolosa-Hunt/etiología , Síndrome de Tolosa-Hunt/terapiaRESUMEN
En 1954 Eduardo Tolosa y posteriormente Hunt describieron una entidad definida por un intenso dolor orbitario unilateral, disfunción de los nervios oculomotores y de la rama oftálmica del trigémino. Este cuadro se caracterizaba por tener una respuesta espectacular a los corticoides y los escasos estudios histopatológicos mostraron una reacción granulomatosa inespecífica del seno cavernoso. Presentamos 2 casos del síndrome de Tolosa-Hunt. El primero corresponde a una mujer de 24 años, con dolor orbitario izquierdo, compromiso de todos los oculomotores y de la rama oftálmica de trigémino. En los exámenes complementarios destacó la RNM donde se vio un engrosamiento isointenso en T1 e impregnación con el uso de contraste del seno cavernoso izquierdo. El segundo caso correspondió a una mujer de 50 años, con un cuadro clínico de características similares a la primera enferma. Todos los exámenes complementarios resultaron normales, incluyendo la RNM. Las 2 pacientes tuvieron una excelente respuesta al uso de corticoides, permaneciendo asintomáticas a más de 2 años de seguimiento. Destacamos la utilidad de la RNM en el diagnóstico diferencial. El uso empírico de corticoides y el seguimiento clínico de las pacientes, permitieron plantear la hipótesis de un síndrome de Tolosa-Hunt. Esta conducta, evita la realización de procedimientos invasivos de alto riesgo, como una biopsia del seno cavernoso, única manera de llegar a la confirmación histopatológica de esta enfermedad.