ABSTRACT
Abstract Tolosa-Hunt syndrome is a painful ophthalmoplegia caused by non-specific granulomatous inflammation, corticoid-sensitive, of the cavernous sinus. The etiology is unknown. Recurrences are common. The diagnosis is made by exclusion, and a variety of other diseases involving the orbital apex, superior orbital fissure and cavernous sinus should be ruled out. This study reports a case of a 29-year-old woman, diagnosed with Tolosa-Hunt Syndrome, who presented ophthalmoparesis and orbital pain. She had poor response to corticotherapy and developed colateral effects, so she was treated with single infliximab dose immunosuppression, evolving total remission of the disease.
Resumo A Síndrome de Tolosa-Hunt é uma oftalmoplegia dolorosa causada por uma inflamação granulomatosa não específica, sensível a corticoides, do seio cavernoso. A etiologia é desconhecida. Recorrências são comuns. O diagnóstico é feito por exclusão, devendo ser descartada uma variedade de outras doenças que envolvem o ápice orbitário, fissura orbitária superior e seio cavernoso. O presente estudo trata-se de um relato de caso de uma paciente de 29 anos, diagnosticada com Síndrome de Tolosa-Hunt, que apresentou paresia e dor em região orbital. Obteve resposta pouco efetiva a corticoterapia e desenvolveu efeitos colaterais, por isso foi tratada com dose única de infliximabe, evoluindo com remissão total da doença.
Subject(s)
Humans , Female , Adult , Tolosa-Hunt Syndrome/drug therapy , Infliximab/administration & dosage , Infliximab/therapeutic use , Pain/drug therapy , Remission Induction , Prednisolone/adverse effects , Prednisone/adverse effects , Single Dose , Ophthalmoplegia/drug therapy , Tolosa-Hunt Syndrome/diagnosisABSTRACT
El síndrome de Guillain-Barré se define como una polirradiculoneuropatía aguda, de inicio súbito y cuyo origen es, en la mayor parte de los casos, autoinmune. Se manifiesta como un cuadro de parálisis motora fláccida, de tipo ascendente, acompañada de arreflexia, con alteraciones sensitivas o sin ellas. Es la causa más frecuente de parálisis fláccida aguda en niños previamente sanos. Presenta distintas variantes que forman parte de un mismo espectro. Una de ellas es el síndrome de Bickerstaff, caracterizado por ataxia, oftalmoplejía externa asociada a encefalopatía o hiperreflexia. Es importante el diagnóstico precoz a fin de poder instaurar rápidamente medidas de sostén y tratamiento que beneficiarán a aquellos pacientes que progresan hacia un cuadro de mayor gravedad. Presentamos el caso de un niño de 4 años de edad, previamente sano, que presenta cuadro compatible con síndrome de Bickerstaff.
Guillain-Barré syndrome is defined as an acute polyradiculoneuropathy, with sudden onset and its origin being mostly autoimmune. It is characterized by flaccid paralysis, symmetrical and ascending, together with areflexia, with or without sensory disturbances. It is the primary cause of acute flaccid paralysis in previously healthy children. Guillain-Barré syndrome presents different variants as part of the same spectrum. One of this is the Bickerstaff syndrome, characterized by ataxia, encephalopathy, hyperreflexia and external ophthalmoplegia. Early diagnosis is important with the view to establishing an early treatment that will be beneficial for those patients that progress to a more serious illness. We report the case of a 4-year-old boy who was previously healthy, and then presented symptoms that are compatible with Bickerstaff syndrome.
Subject(s)
Humans , Male , Child, Preschool , Ataxia/diagnosis , Ataxia/drug therapy , Ophthalmoplegia/diagnosis , Ophthalmoplegia/drug therapy , Reflex, Abnormal , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/drug therapyABSTRACT
Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bickerstaff`s brain stem encephalitis (BBE) and Acute Ophtamoplegia (AO). We report four patients aged 16 to 76 years, with anti-GQ1b syndrome. All presented with MFS, one of them evolved to GBS pharyngeal-cervical-brachial variant and other to GBS with BBE. All had a previous history of diarrhea or upper respiratory tract infection. All had positive anti-GQ1b serum antibodies. Both brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Electrophysiology studies were compatible with a demyelinating disease. Two patients needed airway protection with an orotracheal tube and developed dysautonomia. All four patients were treated with immunomodulation. On the sixth month follow-up, patients had only minimal alterations in the neurological examination.
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Antibodies, Anti-Idiotypic/blood , Encephalitis/diagnosis , Gangliosides/blood , Guillain-Barre Syndrome/diagnosis , Miller Fisher Syndrome/diagnosis , Ophthalmoplegia/diagnosis , Brain Stem , Encephalitis/drug therapy , Gangliosides/immunology , Guillain-Barre Syndrome/drug therapy , Immunoglobulins, Intravenous/therapeutic use , Magnetic Resonance Imaging , Miller Fisher Syndrome/drug therapy , Ophthalmoplegia/drug therapyABSTRACT
PURPOSE: Crotoxin is the main neurotoxin of South American rattlesnake Crotalus durissus terrificus. The neurotoxic action is characterized by a presynaptic blockade. The purpose of this research is to assess the ability of crotoxin to induce temporary paralysis of extraocular and facial muscles in humans. METHODS: Doses of crotoxin used ranged from 2 to 5 units (U), each unit corresponding to one LD50. We first applied 2U of crotoxin in one of the extraocular muscles of 3 amaurotic individuals to be submitted to ocular evisceration. In the second stage, we applied crotoxin in 12 extraocular muscles of 9 patients with strabismic amblyopia. In the last stage, crotoxin was used in the treatment of blepharospasm in another 3 patients. RESULTS: No patient showed any systemic side effect or change in vision or any eye structure problem after the procedure. The only local side effects observed were slight conjunctival hyperemia, which recovered spontaneously. In 2 patients there was no change in ocular deviation after 2U crotoxin application. Limitation of the muscle action was observed in 8 of the 12 applications. The change in ocular deviation after application of 2U of crotoxin (9 injections) was in average 15.7 prism diopters (PD). When the dose was 4U (2 applications) the change was in average 37.5 PD and a single application of 5U produced a change of 16 PD in ocular deviation. This effect lasted from 1 to 3 months. Two of the 3 patients with blepharospasm had the hemifacial spasm improved with crotoxin, which returned after 2 months. CONCLUSIONS: This study provides data suggesting that crotoxin may be a useful new therapeutic option for the treatment of strabismus and blepharospasm. We expect that with further studies crotoxin could be an option for many other medical areas.
OBJETIVO: A crotoxina é a principal neurotoxina da cascavel sul-americana Crotalus durissus terrificus e sua ação neurotóxica caracteriza-se por um bloqueio pré-sináptico. O objetivo da pesquisa é avaliar a capacidade da crotoxina em induzir paralisia transitória de músculos extraoculares e faciais em seres humanos. MÉTODOS: As doses utilizadas de crotoxina foram de 2 a 5 unidades (U), sendo que cada unidade correspondia a uma DL-50. Na primeira etapa, aplicou-se 2U de crotoxina em músculos extraoculares de 3 indivíduos amauróticos, candidatos à evisceração. Na segunda etapa, realizaram-se 12 aplicações de crotoxina em músculos extraoculares de 9 indivíduos estrábicos e amblíopes. Na terceira e última etapa, utilizou-se a crotoxina para o tratamento do blefaroespasmo essencial em 3 indivíduos. RESULTADOS: Nenhum paciente demonstrou qualquer efeito sistêmico ou alteração da visão ou de qualquer estrutura ocular. O único efeito local adverso foi hiperemia conjuntival, que melhorou espontaneamente. Em 2 pacientes não houve alteração do desvio ocular após a aplicação de 2U de crotoxina. Observou-se em 8 das 12 aplicações, limitação do movimento ocular no campo de ação do músculo aplicado. A diminuição do desvio ocular com 2U crotoxina (9 aplicações) foi em média de 15,7 dioptrias prismáticas (DP); na dosagem de 4U (2 aplicações) foi em média de 37,5 DP e na única aplicação de 5U, obteve-se redução de 16 DP no desvio ocular. A alteração do alinhamento ocular manteve-se por 1 a 3 meses. Dois dos 3 pacientes portadores de blefaroespasmo apresentaram melhora dos espasmos hemifacias, os quais voltaram após 2 meses. CONCLUSÕES: Através dos resultados observados neste estudo, acreditamos que a crotoxina possa ser útil no tratamento do estrabismo e do blefaroespasmo. Novos estudos precisam ser realizados para confirmar a eficácia e a segurança da crotoxina como opção terapêutica para diversas áreas da medicina que atualmente utilizam a toxina botulínica.
Subject(s)
Adolescent , Adult , Aged , Animals , Female , Humans , Male , Mice , Middle Aged , Young Adult , Crotoxin/administration & dosage , Facial Muscles/drug effects , Neuromuscular Blocking Agents/administration & dosage , Oculomotor Muscles/drug effects , Ophthalmoplegia/drug therapy , Blepharospasm/drug therapy , Crotoxin/adverse effects , Injections, Intraocular , Neuromuscular Blocking Agents/adverse effects , Strabismus/drug therapyABSTRACT
An eight-year-old male child presented with drooping of the left eyelid with a history of penetrating injury of hard palate by an iron spoon seven days ago, which had already been removed by the neurosurgeon as the computed tomography scan revealed a spoon in the left posterior ethmoid and sphenoid bone penetrating into the middle cranial fossa. On examination, visual acuity was 20/20 in each eye and left eye showed total ophthalmoplegia. Oral cavity revealed a hole in the left lateral part of the hard palate. We managed the case with tapering dose of systemic prednisolone. The total ophthalmoplegia was markedly improved in one month. Cases of foreign bodies in the orbit with intracranial extension are not unusual, but the path this foreign body traveled through the hard palate without affecting the optic nerve, internal carotid artery or cavernous sinus makes an interesting variation.
Subject(s)
Child , Diagnosis, Differential , Dose-Response Relationship, Drug , Follow-Up Studies , Glucocorticoids/administration & dosage , Humans , Male , Ophthalmoplegia/diagnosis , Ophthalmoplegia/drug therapy , Ophthalmoplegia/etiology , Palate, Hard/injuries , Prednisolone/administration & dosage , Tomography, X-Ray Computed , Wounds, Penetrating/complications , Wounds, Penetrating/diagnosisABSTRACT
We report a 40-year old woman with bilateral partial ptosis, complete external ophthalmoplegia, and weakness and fatiguability of upper limbs. She was on treatment for hypertension for 5 months at the time of admission. She was found to have generalised myasthenia gravis and membranous nephropathy with end-stage renal disease. Her symptoms and signs improved within 2 months on treatment with neostigmine and prednisolone. It is postulated that either thymic hyperplasia or the subclinical stage of a thymoma may be the underlying aetiological factor in this patient.
Subject(s)
Adult , Blepharoptosis/drug therapy , Female , Glomerulonephritis, Membranous/diagnosis , Humans , Kidney Failure, Chronic/etiology , Myasthenia Gravis/diagnosis , Neostigmine/therapeutic use , Ophthalmoplegia/drug therapy , Prednisolone/therapeutic use , Thymoma/complications , Thymus Hyperplasia/complications , Thymus Neoplasms/complications , Treatment OutcomeABSTRACT
El síndrome Tolosa-Hunt se caracteriza por dolor retroocular unilateral agudo recurrente con parálisis extraocular, que generalmente afecta a los nervios craneanos tercero, cuarto, quinto y sexto. Puede haber también proptosis y trastornos de la sensibilidad en la frente, escasa reacción de la pupila a la luz, y disminución de la sensibilidad corneal con visión borrosa o ceguera completa. Se la atribuye a una infiltración granulomatosa del apex orbitario o en el seno cavernoso, que responde a la corticoideoterapia. Hombres y mujeres son afectados por igual, generalmente en la quinta década. La enfermedad generalmente dura desde varias semanas hasta varios años. La resonancia magnética es el método de elección para el diagnóstico diferencial.
Subject(s)
Humans , Female , Middle Aged , Magnetic Resonance Imaging , Tolosa-Hunt Syndrome/diagnosis , Diagnostic Imaging , Ophthalmoplegia/diagnosis , Ophthalmoplegia/drug therapy , Prednisone/administration & dosage , Cavernous SinusABSTRACT
Objetivo: reconocer el mejor tratamiento para la pronto recuperación del nervio parético. Material y método: es un ensayo en comunidad, prospectivo y experimental. Se evaluaron 4 grupos: grupo 1: con tóxina botulínic, grupo 2: con carbamazepina oral, grupo 3: con complejo B oral y grupo 4: control metabólico. A todos los pacientes se les realizó exploración oftalmológica completa y estrabológica con prueba de ducción forzad, puntos de Worth y prismas, así como tiras reactivas para la determinación de glucosa en sangre cada 2 semanas y hemoglobina glucosilada al inicio y final del tratamiento, para su control. Resultados: del total de 80 pacientes estudiados (39 por ciento femeninos y 61 por ciento masculinos) con edad promedio de 60 años; el grupo 1 presentó un tiempo promedio de resolución más rápido (12.2 semanas), en comparación a los otros grupos. Conclusión: la resolución del cuadro parético es más rápida en los grupos tratados con toxina butolínica y carbamazepina (12 semanas), y más tardada en el grupo con complejo B (14 semanas).
Purpose: to recognize the best treatment for recovery of paretic craneal nerve. Material and methods: prospective, experimental and community trial, divided in four groups: group 1: with botulinum toxin, group 2: with oral carbamazepine, group 2: with oral carbamazepine, group 3: with oral vitamin B and group 4: with metabolic control. All pacients were evaluated with complete opthalmological and strabological examination with Worth, prism and force duction text; metabolic control was ascertained with glucosilade hemoglobine and a reactive blood test every two weeks. Results: A total of 80 patients were included (39 percent female and 61 percent male) with a mean age a 60 years old; group 1 showed the shortest mean time of resolution of 12.2 weeks compared to others groups. Conclusions: paretic resolution is faster with botulinum toxin and carbamazepine (12 weeks), and slower with vitamin B (14 weeks).
Subject(s)
Female , Middle Aged , Strabismus/rehabilitation , Ophthalmoplegia/rehabilitation , Diabetic Retinopathy/rehabilitation , Administration, Oral , Carbamazepine/administration & dosage , Vitamin B Complex/administration & dosage , Diabetes Mellitus , Strabismus/drug therapy , Ophthalmoplegia/drug therapy , Prospective Studies , Diabetic Retinopathy/drug therapy , Time Factors , Treatment Outcome , Botulinum Toxins/administration & dosageABSTRACT
A enxaqueca oftalmoplégica é síndrome rara em que a cefaléia se associa à oftalmoplegia por paralisia do terceiro, quarto ou sexto nervos cranianos. Ocorre mais freqüentemente em crianças e adolescentes. Ao exame de ressonância magnética (RM), com uso de gadolínio, é observado realce transitório do nervo afetado. Apresentamos o caso de um adolescente, 16 anos, masculino, com história típica e hipersinal nas seqüências ponderadas em T1 pós-infusão venosa do gadolínio na porção cisternal do nervo oculomotor esquerdo. No exame de controle, realizado 18 meses depois, houve remissão completa das alterações na RM. O hipersinal do oculomotor à RM é sempre patológico; entre os diagnósticos diferenciais devemos incluir neoplasias (linfoma, leucemia), infecções (SIDA, sífilis), doenças inflamatórias não-infecciosas (sarcoidose, síndrome de Tolosa-Hunt) e vasculares (aneurisma da artéria comunicante posterior).
Subject(s)
Adolescent , Humans , Male , Migraine Disorders/complications , Ophthalmoplegia/complications , Anti-Inflammatory Agents/therapeutic use , Gadolinium DTPA , Magnetic Resonance Angiography , Migraine Disorders/drug therapy , Ophthalmoplegia/diagnosis , Ophthalmoplegia/drug therapy , Prednisone/therapeutic useABSTRACT
PURPOSE: To report a case with complete ophthalmoplegia after herpes zoster ophthalmicus. METHODS: A 70-year-old male patient visited a clinic because of vesicular eruptions over the left side of his face with severe pain. Drooping and severe swelling of the left eyelid were present, along with keratitis and uveitis. While the lid swelling and uveitis were improving, external ophthalmoplegia and exophthalmos were discovered. Intramuscular injections of dexamethasone 5 mg were given for 10 days, followed by oral administration of prednisolone at a dosage of 15 mg for two weeks and 10 mg for two weeks. RESULTS: The patient was fully recovered from the complete ophthalmoplegia and exophthalmos six months after the onset of the cutaneous lesion. CONCLUSIONS: Complete ophthalmoplegia is a rare ophthalmic complication of herpes zoster infection. Therefore, an evaluation of extraocular muscle and lid function should be performed during the examination of herpes zoster patients in order to screen for ophthalmoplegia.
Subject(s)
Male , Humans , Aged , Severity of Illness Index , Prednisolone/therapeutic use , Ophthalmoplegia/drug therapy , Herpes Zoster Ophthalmicus/complications , Glucocorticoids/therapeutic use , Follow-Up Studies , Exophthalmos/drug therapyABSTRACT
Although ophthalmoplegia following snake bites is not indicative of a serious neurotoxic complication, symptoms of diplopia, dizziness and ocular discomfort can be emotionally devastating for patients. The authors experienced two cases of ophthalmoplegia following snake bites in Korea. The patients complained of diplopia that had developed several hours after the snake bites. The diplopia did not improve with antivenom treatment, but resolved completely after several injections of neostigmine.
Subject(s)
Adolescent , Animals , Child , Female , Humans , Male , Cholinesterase Inhibitors/therapeutic use , Diplopia/drug therapy , Neostigmine/therapeutic use , Ophthalmoplegia/drug therapy , Snake Bites/complicationsABSTRACT
A sindrome de Tolosa-Hunt (STH) consiste de oftalmoplegiadolorosa relacionada a processo inflamatorio granulomatoso no seio cavernoso. Seu diagnostico so pode ser firmado quando outras causas potenciais de oftalmoplegia dolorosa forem descartadas. Resposta satisfatoria e padronizada a corticosteroides, associada a evolucao benigna, pode reforcar essa possibilidade, embora pseudotumor orbitario e neoplasias do tipo linfoma e ate meningioma possam suscitar duvidas no diagnostico diferencial, pois tambem podem apresentar boa resposta a essa medicacao. Faz-se necessario, portanto, ampla investigacao complementar. Apresentamos estudo clinico-radiologico-laboratorial de cinco pacientes com STH Prednisona foi utilizada em doses entre 40 e 80 mg/dia. Houve resposta analgesica excelente, com remissao da dor me menos de 48 horas em 4 pacientes. Houve melhora mais lenta da oftalmoplegia, que regrediu completamente entre 4 e 45 dias emtodos os pacientes.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Anti-Inflammatory Agents/therapeutic use , Ophthalmoplegia/diagnosis , Ophthalmoplegia/drug therapy , Prednisone/therapeutic use , Prednisone/administration & dosageABSTRACT
Twelve patients who satisfied the clinical criteria of Tolosa-Hunt syndrome (THS), underwent axial computerised tomography (CT) scan of the head. Two patients had abnormalities detected in the cavernous sinus on the affected side which supported the clinical diagnosis. This coupled with steroid responsiveness and long-term asymptomatic follow-up firmly established the diagnosis of THS. We discuss the computerised tomographic findings seen in THS and the relevance of using accurate imaging techniques as the first line of investigation in these cases.