Neuritis óptica recurrente posterior a encefalomielitis diseminada aguda en pediatría. Reporte de un caso / Recurrent optic neuritis after acute disseminated encephalomyelitis in pediatrics. A case report

Abstract: Optic Neuritis (ON) is a condition caused by the inflammation of the optic nerve, causing diminished visual acuity and ocular pain. It is tightly related to Multiple Sclerosis (MS), often being the first demyelinating event. There is a 31% risk of recurrence during the first 10 years after the diagnosis, and 48% of the patients end up being diagnosed with Multiple Sclerosis1. Imaging studies like brain MRI (Magnetic Resonance Imaging) have a critical role in the diagnosis and prognosis of ON, as well as in the recognition of MS.2 The patient is a 11-year-old girl with recurrent ON and past medical history of Acute Disseminated Encephalomyelitis (ADEM) when she was 5 years old. ON diagnosis was based on clinical findings as well as on ophtalmologic, electrophysiologic and imaging studies. The recurrent episodes of ON improved after the use of high dose steroids. Recurrences were observed after titration of the dose, but remission was achieved after adjustment of treatment. During one of the recurrent episodes, blood work was performed to evaluate possible underlying infectious, demyelinating or autoinmmune process. Anti-MOG antibodies were found positive. The patient at the moment is not presenting with any other criteria suggesting MS or Optic Neuromyelitis, but long term follow up is adviced. Key words: Optic neuritis (ON), multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), magnetic resonance imaging (MRI), cerebrospinal fluid (CSF), right eye (RE); visual acuity (VA), optic neuromyelitis (ONM)

Encefalomielitis diseminada aguda posinfecciosa en un paciente con sospecha de infección por virus dengue / Postinfectious acute disseminated encephalomyelitis in a patient with suspected dengue virus infection

Introducción: La infección por virus dengue es considerada una de las arbovirosis de mayor prevalencia en los países tropicales. La encefalomielitis diseminada aguda es un trastorno inflamatorio desmielinizante y multifocal que afecta al sistema nervioso central, de inicio agudo y curso clínico monofásico. El proceso inflamatorio se encuentra mediado por mecanismos inmunológicos y su relación con infecciones por el virus dengue aún no se establece con claridad. Objetivo: Describir un caso clínico con manifestaciones del sistema nervioso central después de una probable infección por el virus dengue. Presentación del caso: Paciente femenina de 50 años, con antecedentes de hipertensión arterial controlada. Quince días después de un cuadro de fiebre de 4 días de duración, que posiblemente fue por una infección por el virus dengue, comienza con síntomas y signos de afectación neurológica caracterizadas por ligera irritabilidad, dificultad para la concentración en una actividad específica de la vida cotidiana. Progresivamente se nota dificultad motora en el hemicuerpo izquierdo además de encontrarse agitada y distraída, motivo por el cual se decide su ingreso hospitalario. Se realiza el diagnóstico a través de los hallazgos en el examen físico, los estudios positivos de resonancia magnética nuclear y el resultado positivo de la IgM de dengue en sangre. Tanto la evolución clínica como la respuesta al tratamiento con esteroides fueron favorables. Conclusiones: El evento ocurrido en este caso sugiere que los facultativos deben tener presente el diagnóstico de encefalomielitis diseminada aguda en pacientes que han tenido infección previa o alta sospecha de esta por el virus dengue(AU)

Introduction: Dengue virus infection is one of the most prevalent arboviruses in tropical countries. Acute disseminated encephalomyelitis is an inflammatory demyelinating multifocal disorder affecting the central nervous system. Its onset is acute and its clinical course monophasic. The inflammatory process is mediated by immunological mechanisms, and its relationship to dengue virus infections is still not clear. Objective: Describe a clinical case of central nervous system manifestations after probable dengue virus infection. Case presentation: Female 50-year-old patient with a history of controlled hypertension. Fifteen days after a 4-day fever episode, possibly due to dengue virus infection, the patient starts presenting neurological signs and symptoms, such as slight irritability and difficulty to concentrate on a specific activity of daily living. The patient notices progressive motor difficulty in her left hemibody and she feels agitated and distracted. It is therefore decided for her to be hospitalized. A diagnosis is made based on physical examination findings, positive nuclear magnetic resonance studies, and the positive result of the dengue IgM blood test. Both the patient's clinical evolution and her response to treatment with steroids were favorable. Conclusions: The event herein described suggests that physicians should consider the diagnosis of acute disseminated encephalomyelitis in patients with previous infection or high suspicion of infection with dengue virus(AU)

Acute disseminated encephalomyelitis (ADEM) presenting with bilateral optic neuritis

A 43-year old lady presented with progressive loss of vision in both eyes followed by rapid deterioration of consciousness within the next few days. This was preceded by a viral infection one week before her presentation. At presentation she had evidence of meningism and signs of bilateral upper motor neuron lesions and was managed initially as acute meningoencephalitis with antibiotics. The brain CT was within normal limits but subsequent MRI of the brain revealed multiple foci of hyperintense lesions on T2-weighted and FLAIR images. The cerebrospinal fluid examination revealed lymphocytosis, and normal protein and glucose levels. Cultures of the CSF were negative. She was managed as acute disseminated encephalomyelitis (ADEM) with high-dose of intravenous methlyprednisolone one gram/day for three consecutive days followed by oral prednisolone 60 mg/day. Despite the management she lapsed into coma and succumbed to her illness nine days after admission.

Optic Neuritis in Acute Disseminated Encephalomyelitis

PURPOSE: This case is the first report of optic neuritis following acute disseminated encephalomyelitis in Korean literature and we report this case with a successful result of conservative treatment. METHODS: We examined a 7-year and-7-month-old female patient who visited our ophthalmology clinic complaining of a headache and visual disturbance in both eyes. At the time of visit, light perception was negative in both eyes, response to light reflex was nil in the right eye and weak in the left eye. Relative afferent papillary defect (RAPD) was noted in both eyes, and the disc swelling was found in both eyes on fundus examination. MRI brain scans revealed asymmetrical multifocal lesions involving the cortex and subcortex in the bilateral brain hemispheres, and contrast enhanced in T2-weighted images. Cerebrospinal fluid (CSF) examination showed results of 45 WBC, 10 polynuclear lymphocyte, and 88 monocyte cells/mm3. RESULTS: As patient was diagnosed with acute disseminated encephalomyelitis, she was prescribed intravenous steroid pulse therapy with methylprednisolone sodium succinate (Solu-medrol(R), Pharmacia, U.S.A), and immunoglobulin (Liv gamma(R), Green Cross, Korea). Seven days of steroid therapy was followed by tapering with oral steroid. Uncorrected vision was 0.9 in the right eye and 0.9 in the left. RAPD was resolved in both eyes. Visual evoked potentials showed both eyes being normal.

Acute Disseminated Encephalomyelitis(ADEM) Presenting as Multiple Cystic Lesions

Acute disseminated encephalomyelitis(ADEM) is an uncommon immune-mediated inflammatory demyelinating disorder that typically affects the white matter of the central nervous system. Radiologic findings of acute disseminated encephalomyelitis are not pathognomomic. The differential diagnosis is always difficult. Occasionally, the clinical features, radiological and histopathological findings of patients with acute disseminated encephalomyelitis mimic the brain tumor or other space occupying lesions. The authors report a 6-year-old girl who presented with right hemiparesis two days after nausea and vomiting. Brain MRI of the patient revealed non-enhanced multiple cystic lesions in subcortical white matter of both cerebral hemisphere with prominent edema. One of the cystic lesions was resected to differentiate with metastatic tumor or inflammatory disease such as abscess and confirmed as the acute disseminated encephalomyelitis via various immunohistochemical stains. Pertinent literature is reviewed with discussion on this uncommon ADEM associated with multiple cystic lesions.

2 cases of acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is an acute inflammatory demyelinating disease of the central nervous system. It is thought to be an autoimmune mediated disorder which occurs usually after viral or bacterial infections, or after immunization. The clinical manifestations reflect sudden onset of the diffuse involvement of the brain, spinal cord and the meninges. We experienced two cases of acute disseminated encephalomyelitis. The diagnoses were made by history, neurologic examination, CSF analysis and brain MRI. The symptoms were improved with steroid treatment. A brief reveiw of literatures also was made.

A case of acute disseminated encephalomyelitis

No abstract available.