Oftalmoplejía internuclear bilateral (WEBINO) en un paciente pediátrico con Lupus Eritematoso Sistémico / Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) in a pediatric patient with Systemic Lupus Erythematosus

Resumen El síndrome de WEBINO (wall-eyed bilateral internuclear ophthalmoplegia), se presenta por una lesión del tegmento pontino (incluye área pontina paramediana, fascículo longitudinal medial y núcleo del abducens). Presenta limitación bilateral en la aducción y exotropía en la posición de la mirada primaria, nistagmo del ojo que abduce e incapacidad para la convergencia. Reporte de caso: Presentamos el caso de una paciente de 14 años con antecedente de Lupus Eritematoso Sistémico que debutó con diplopía horizontal de inicio súbito. El diagnóstico de WEBINO fue clínico y asociado con hallazgos de lesión isquémico pontomesencefálica en Resonancia Nuclear Magnética y angioresonancia cerebral. Se administró tratamiento con Metilprednisolona y presentó resolución gradual de los síntomas, sin embargo una semana después falleció por criptococosis sistémica. Conclusiones: Hacer el diagnostico de WEBINO se hace desafiante por su rareza y por la precisión de su localización neuroanatómica. Se debe realizar una exploración detallada para definir la causa probable y establecer el tratamiento oportuno que favorezca el pronóstico neurológico.

Background: Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is presented by a lesion of the pontine tegment (includes paramedian pontine area, medial longitudinal fascicle and nuclei of the abducens). It presents bilateral limitation in adduction and exotropia in the position of the primary gaze, abducting eye nystagmus and inability to converge. Case report: We present the case of a 14-year-old patient with a history of Systemic Lupus Erythematosus who debuted with sudden onset horizontal diplopia. WEBINO's diagnosis was clinical and associated with findings of ponto-mesencephalic ischemic injury in magnetic resonance imaging and magnetic resonance angiography. Treatment with Methylprednisolone was administered and she presented gradual resolution of the symptoms, however, one week later she died of systemic cryptococcosis. Conclusions: Making the WEBINO diagnosis is challenging due to its rarity and the precision of its neuroanatomical location. A detailed examination should be performed to define the probable cause and establish the appropriate treatment that favors the neurological prognosis.

Botulinum toxin in the management of acquired motor fusion deficiency.

Acquired disruption of motor fusion is a rare condition characterized by intractable diplopia. Management of these patients is extremely difficult. Prisms in any combination or even surgery may not help relieve their symptoms. We describe a longstanding case of acquired motor fusion disruption which was managed successfully with botulinum toxin injection.

Factors associated with insensitivity to pyridostigmine therapy in Thai patients with ocular myasthenia gravis.

The objective of this study was to determine factors associated with pyridostigmine therapy in patients with ocular myasthenia gravis (OMG). This retrospective study included eighty-five patients with OMG who have been treated with pyridostigmine. Patients were excluded if they were diagnosed as generalized myasthenia gravis within a month after diagnosis or were treated with other medications. Forty-two patients responded to pyridostigmine and 43 patients did not. There were no significant differences in gender, age, the duration of symptoms before treatment, the dosage of pyridostigmine, and the initial presentations of ptosis or diplopia between the two groups. However, an initial presentation of concurrent ptosis and diplopia and the presence of systemic involvement after follow up were significant factors associated with an insensitivity to pyridostigmine in patients with OMG (p = 0.001 and p = 0.01, respectively). Determining these factors could help predict the pyridostigmine response in patients with OMG.

Síndrome de Parinaud asociado a otros estrabismos / Parinaud syndrome associated with other strabismus

Objetivo: Describir los estrabismo asociados al Síndrome de Parinaud (SP). Métodos: Se estudiaron pacientes con SP de 1998 a 2004 asociados a otros estrabismos. Resultados: Total 18 pacientes, 6 mujeres y 12 hombres. Edad: 28 años DE 22 (10; 1C 95 por ciento). Lesión al III nervio craneal en 15 pacientes (79 por ciento), II nervio: 8 pacientes (44.4 por ciento), VII nervio: 3 pacientes, hemiparesia: 3, lesión a IV nervio 2, parálisis bilateral de III nervio 2, nistagmus 2, Síndrome de foville 2, y síndrome de uno y medio 1 paciente. Causas: Accidente vascular cerebral 4, uno de ellos asociado a infarto agudo de miocardio, traumatismo cráneo-encefálico 3, tumor cerebral 6, neurocisticercosis 2. Se obtuvo ortoposición con toxina botulínica en 1.72 +/- 1.1 (0.65 IC 95) y cirugía en 9, mejoría espontánea 1. Conclusiones: Identificar el diagnóstico topográfico y pronóstico de la lesión cerebral asociada a SP es muy importante en el control y vigilancia del paciente así como para ofrecer una rehabilitación mas dirigida.

Objective: To describe the causes of Parinaud syndrome (PS) with diplopia to determine a topographic diagnosis and prognosis. Method: We studied patients with SP from January 1980 to January 2004; causes and treatment. We identified all strabismus associated to PS. Results: We included 18 patients with diplopia, 6 female and 12 male. Age: 28 years old, SD 22 (10; CI 95 percent). Related strabismus: III nerve palsy in 15 patients (79 percent), optical nerve damage in 8 (44.4 percent), VII nerve palsy in 3, IV nerve palsy 2, bilateral III nerve palsy 2, nystagmus 2, Foville syndrome 2, One and half syndrome 1. Causes: Tumor 6, brain stroke 4, cranial trauma 3, neurocisticercosis 2. Ortoposition with botulinum toxin treatment was obtained with 1.72 +/-1.1 (0.65; IC 95 percent) inyections. Surgery was done in 9 patients, only one patient had spontaneous resolution of diplopia and SP. Conclusions: To identify the topographic diagnosis and prognosis of brain diseases in PS is very important to complete the study of strabismus. In this way we can know more about of damage extension and we can identify recurrences quickly to offer a better control.

Toxina botulínica: aplicaçöes clínicas / Botulinum toxin: clinic aplication

A toxina botulínica (TOXBA) produz paralisia muscularpela inibiçäo pré-sináptica da liberaçäo da acetilcolina. A eliminaçäo de sua toxicidade natural através de sofisticadas técnicas laboratoriais tornou possível clínica no tratamento de uma diversidade de desordens de movimentos até entäo de difícil controle. A filosofia de seu uso repousa na identificaçäo dos principais músculos envolvidos no movimento anormal e na paralisaçäo dos mesmos pela injeçäo tópica de TOXBA, de modo a bloquear o movimento anormal em questäo. O procedimento, em mäos experimentadas, é até simples e tem a vantagem de ser um método poco invasivo e de poder ser realizado ambulatorialmente. Além de seguro, tem elevada eficácia. A experiência pessoal do autor e a literatura mostram que melhora significativa é obtidaem estrabismo, 85 por cento; blefaroespasmo, 70-90 por cento; distonia oromadibular, 70 por cento; distonia cervical (torcicolo espasmódico), 50-90 por cento; distonia laríngea, 80-100 por cento; distonia de mäo, 40-80 por cento; e espasmo hemifacial, 90 por cento...

Opsoclonus in malaria: the first report in the literature.

The first report in the literature of opsoclonus in malaria was presented. A 24-year-old woman had a two week history of high fever and lassitude. Physical examination revealed no specific neurological sign on admission. Ring form and gametocytes of Plasmodium falciparum were found in blood smear. Combined treatment of quinine and tetracycline were prescribed. She developed classical abnormal eye movement of opsoclonus and hand tremor on the following day, which gradually subsided on the tenth hospital day. Computed brain tomography and cerebrospinal fluid study were normal. She was discharged home after 3 weeks of admission with some occasional minimal opsoclonus without hand tremor.