ABSTRACT
RESUMEN Introducción: El manejo diagnóstico y terapéutico en los pacientes con lupus eritematoso sistémico que desarrollan una afectación neuropsiquiátrica representa un reto, debido a la heterogeneidad de las formas en que puede presentarse y la ausencia de criterios diagnósticos. Objetivo: Reconocer las formas clínicas de presentación de los síndromes neuroftalmológicos que traducen afectación pontina. Presentación del caso: Hombre de 71 años con antecedente de lupus eritematoso sistémico con afectación neuopsiquiátrica, que de forma aguda desarrolla un cuadro emético en el curso de una emergencia hipertensiva seguido de una parálisis de la mirada horizontal hacia la izquierda, una oftalmoplejía internuclear posterior derecha y una parálisis facial izquierda. En la neuroimagen se constata una afectación multifocal con marcado daño pontino. Conclusiones: Reconocer las formas clínicas de presentación de estos trastornos neuroftalmológicos raros que generalmente se presentan de forma aguda/subaguda permite al neurólogo realizar el diagnóstico topográfico de la lesión a nivel protuberancial con elevada precisión desde la Sala de Urgencias, así como reducir los posibles diagnósticos diferenciales a una etiología vascular, desmielinizante u ocupativa de espacio(AU)
ABSTRACT Introduction: The diagnostic and therapeutic management of patients with systemic lupus erythematosus who develop a neuropsychiatric involvement represents one of the biggest challenges due to the heterogeneity of the ways in which it can occur and the absence of diagnostic criteria. Objective: To recognize the clinical forms of presentation of neurophthalmological syndromes that express pontine involvement. Case presentation: Seventy-one-year-old man with history of systemic lupus erythematosus with neuropsychiatric involvement who acutely develops an emetic episode in the course of a hypertensive emergency followed by a paralysis of the horizontal gaze to the left, a right-sided posterior internuclear ophthalmoplegia and a left facial palsy. In the neuroimaging, a multifocal involvement with marked pontine damage is observed. Conclusions: Recognizing the clinical forms of presentation of these rare neurophthalmological disorders that generally occur in an acute or subacute form allows the neurologist to perform the topographic diagnosis of the lesion at a protuberancial level with high precision from the time when the patient attends the Emergency Department and reduces the possible differential diagnoses to a vascular, demyelinating or occupational etiology of space(AU)
Subject(s)
Humans , Male , Aged , Lupus Vasculitis, Central Nervous System/complications , Lupus Erythematosus, Systemic/complications , Lupus Vasculitis, Central Nervous System/diagnosis , Lupus Vasculitis, Central Nervous System/drug therapy , Diagnosis, DifferentialABSTRACT
The neuropsychiatric get involved in sistemic lupus erythematosus (SLE) is complex and is the major cause of morbidity and mortality. The incidence in children ranges from 20 percent to 95 percent. The recognition and treatment remain a major diagnostic and therapeutic challenge. The activity for the treatment remains empirical and based on clinical experience. The choice of treatment depends on an accurate diagnosis and identification of the underlying pathogenic mechanism. Symptomatic treatments, immunosuppressive and anti-coagulants are the main therapeutic strategies. You have searched other therapeutic measures in patients with severe disease refractory to standard therapy or the use of cyclophosphamide, immunoglobulin, rituximab, methotrexate + intrathecal dexamethasone, among others. We report one patient with neurolupus (NLES), refractory methylprednisolone and treatment with cyclophosphamide, continuing neurological activity is applied intrathecal MTX 10 mg + dexamethasone 10 mg intrathecal, in a total of 4 doses, finding satisfactory improvement, and controlling activity.
El involucro neuropsiquiátrico en el lupus eritematoso sistémico (LES) es complejo y es causa importante de morbimortalidad. La incidencia en la edad pediátrica va de 20 por ciento al 95 por ciento. Su reconocimiento y tratamiento siguen representando un importante reto diagnóstico y terapéutico. La actividad para el tratamiento sigue siendo empírica y basada en la experiencia clínica; la elección terapéutica depende de un diagnóstico preciso y la identificación del mecanismo patogénico subyacente. Los tratamientos asintomáticos, inmunosupresores y anticoagulantes representan las principales estrategias terapéuticas. Se han buscado otras medidas terapéuticas en pacientes con afección severa o refractarios al tratamiento habitual, como el uso de ciclofosfamida, inmunoglobulina, rituximab, metotrexato + dexametasona intratecal, entre otros. Presentamos el caso de un paciente con neurolupus (NLES), refractario al tratamiento con metilprednisolona y ciclofosfamida, continuando con actividad neurológica; se aplica MTX 10 mg intratecal + dexametasona 10 mg IT, en un total de 4 dosis, encontrando mejoría satisfactoria y control de la actividad.
Subject(s)
Humans , Female , Child , Immunosuppressive Agents/therapeutic use , Methotrexate/therapeutic use , Lupus Vasculitis, Central Nervous System/drug therapyABSTRACT
Neuropsychiatric abnormalities frequently occur in patients with systemic lupus erythematosus, affecting as many as 14-75% of people with this disease. High-dose steroid with or without anticoagulation is the mainstay of treatment in neuropsychiatric systemic lupus erythematosus (NPSLE). Use of mycophenolate as a steroid sparing drug may be a potential alternative agent in the therapy of NPLE, but lack of randomized trials and cost prohibit its widespread use. Its safety profile is higher than that of cyclophosphamide and azathioprine. We report a successfully treated case of neuropsychiatric systemic lupus erythematosus, presenting as psychosis, whose long-term remission was maintained on treatment with mycophenolate mofetil.