Aumento inusual de casos de mielitis en un hospital pediátrico en Argentina / Unusual increase of cases of myelitis in a pediatric hospital in Argentina

Introducción: La asociación entre enterovirus D68 y cuadros de mielitis aguda fláccida ha sido descrita en Estados Unidos, en 2014. Desde ese año, se han reportado casos esporádicamente en Canadá y Europa. Se describe, en este estudio, una serie de casos con mielitis aguda fláccida en el Hospital de Pediatría "Prof. Dr. Juan P. Garrahan" en Buenos Aires, Argentina, en 2016. Métodos: Estudio descriptivo, retrospectivo. Se incluyeron todos los pacientes internados desde el 1/04/2016 al 1/07/2016 con mielitis fláccida aguda con lesiones en la médula espinal que comprometieran la sustancia gris en la resonancia magnética nuclear. Se procesaron, para la búsqueda etiológica, muestras de secreciones nasofaríngeas, hisopados de materia fecal y líquido cefalorraquídeo. Resultados: Se incluyeron 10 pacientes. La mediana de edad fue 4 años (rango de 3 meses a 5 años). Ocho pacientes tuvieron una enfermedad febril autolimitada antes del inicio de los síntomas neurológicos. Los hallazgos neurológicos fueron debilidad fláccida de, al menos, un miembro, cervicoplejia (n= 2) y parálisis facial (n= 2). Todos los pacientes presentaron lesiones longitudinales en la médula espinal, con compromiso de sustancia gris, predominantemente, en el asta anterior. En todos los casos, se realizó una punción lumbar. En 7 pacientes, se observó pleocitosis. En cuatro niños, se identificó enterovirus D68 en secreciones nasofaríngeas y, en uno, se identificó el enterovirusD68 en el líquido cefalorraquídeo. Todos los pacientes persistieron con déficits neurológicos al momento del alta. Conclusiones: Se reporta el primer brote de mielitis aguda fláccida asociada a enterovirusD68 en Argentina. La vigilancia epidemiológica activa permitirá conocer la verdadera incidencia, epidemiología y etiología de esta enfermedad.

Introduction: The association between enterovirus D68 and acute flaccid myelitis was first described in the United States in 2014. Since then, sporadic cases have been reported in Canada and Europe. This study describes a series of cases of acute flaccid myelitis at Hospital de Pediatría "Prof. Dr. Juan P. Garrahan," in Buenos Aires, Argentina, during 2016. Methods: Descriptive, retrospective study. All patients with acute flaccid myelitis and lesions in the spinal cord involving the gray matter, as observed in the magnetic resonance imaging (MRI) scan, hospitalized from 04/01/2016 to 07/01/2016, were included in the study. Samples of nasopharyngeal secretions, fecal swabs and cerebrospinal fluid were collected and processed to look for the causative agent. Results: Ten patients were included. The median age was 4 years old (range from 3 months to 5 years old). Eight patients had a self-limiting febrile condition before the onset of neurological symptoms. Neurological findings were flaccid weakness in, at least, one limb, cervical paralysis (n= 2) and facial paralysis (n= 2). All patients had longitudinal lesions in the spinal cord, with gray matter involvement, mainly in the anterior horn. In all cases, a lumbar puncture (spinal tap) was performed. Pleocytosis was observed in 7 patients. In four children, enterovirus D68 was identified in nasopharyngeal secretions, and in one, it was detected in the cerebrospinal fluid. Neurological deficit persisted in all patients at the time of discharge. Conclusions: The first outbreak of acute flaccid myelitis associated to enterovirus D68 is reported in Argentina. Active epidemiological surveillance will help to determine the true incidence, epidemiology and etiology of this disease.

A case of tumor-like inflammatory demyelinating disease with progressive brain and spinal cord involvement / Um caso de doença desmielinizante inflamatória semelhante a tumor com envolvimento progressivo de cérebro e medula espinhal

CONTEXT: Tumor-like inflammatory demyelinating disease (TIDD) usually occurs in the brain and rarely occurs in the spinal cord. TIDD appears to be very similar to tumors such as gliomas on imaging, which may lead to incorrect or delayed diagnosis and treatment. CASE REPORT: Because of headache and incoherent speech, a 24-year-old Chinese male presented to our hospital with a two-week history of respiratory infections. After dexamethasone treatment, his symptoms still got worse and surgery was performed for diagnostic purposes. Histological examination revealed that the lesion was inflammatory. Further lesions appeared in the spine (T3 and T4 levels) after two months and in the right occipital lobe after three months. After intravenous immunoglobulin (IVIG) and methylprednisolone treatment, his symptoms improved. CONCLUSION: Progressive lesions may damage the brain and spinal cord, and long-term prednisolone and IVIG therapy are beneficial in TIDD patients.

CONTEXTO: A doença desmielinizante inflamatória tumoral (DDIT) geralmente ocorre no cérebro e raramente na medula espinhal. A DDIT é muito semelhante a tumores tais como gliomas em exames de imagem, o que pode conduzir a diagnóstico e tratamento tardios e incorretos. RELATO DO CASO: Por causa de dor de cabeça e discurso incoerente, um homem chinês de 24 anos de idade foi ao hospital com história de duas semanas de infecções respiratórias. Após o tratamento com dexametasona, seus sintomas ficaram ainda piores e a cirurgia foi realizada para fins de diagnóstico. O exame histológico revelou que a lesão era inflamatória. Mais lesões apareceram na coluna vertebral (níveis T3 e T4) após dois meses, e no lobo occipital direito depois de três meses. Depois de tratamento com imunoglobulina intravenosa (IGIV) e metilprednisolona, seus sintomas melhoraram. CONCLUSÃO: Lesões progressivas podem danificar o cérebro e a medula espinhal, e prednisolona a longo prazo e terapia de IGIV são benéficas em pacientes DDIT.

Acute Longitudinal Myelitis As The First Presentation in Child with Systemic Lupus Erythematosus Concurrent with Positive Antiphospholipid Antibody.

Systemic lupus erythematosus (SLE) is a multi-system auto-immune disorder that is characterized by widespread immune deregulation, formation of auto–antibodies, and immune complexes, resulting in infl ammation and potential damage to variety of organs. 25-95% it is complicated by neurological or neuropsychiatric symptoms, which is referred to as neuropsychiatric SLE (NPSLE). NPSLE contain both central and peripheral nervous systems, which includes transverse myelitis. We report our experience of concurrent manifestation of transverse myelitis as an initial presentation of SLE, which suggests the common immune-mediated mechanisms of diseases. We here report the case of a 7-year-old girl with SLE who fi rst presented with features of TM. Th e patient developed ascending weakness starting from low extremities, experienced diffi culty in voiding. An initial diagnosis of TM was made on the basis of clinical fi ndings and MRI spine, which displayed T2 weighted high signal intensities at thoracic level. She partially respond to intravenous immunoglobulin therapy, and serological analysis revealed the presence of anti-dsDNA, anti nuclear antibody with decreased level of complements. Th e diagnosis was revised to acute transverse myelitis resulting from SLE. Additional methylprednisolone pulse therapy led to rapid clinical improvement. Th is was followed by oral prednisolone and cyclophosphamide pulse therapy. Th e crossreactivity of auto-antibodies and increased susceptibility to infection owing to immunologic changes associated with lupus may form the basis of the association. Systemic Lupus Erytheromyitis should consider as an etiology of transverse myelitis. Aggressive treatment may alter the course and lead to a better outcome.

Encefalitis del tallo cerebral y mielitis por Listeria monocytogenes / Brainstem encephalitis and myelitis due to Listeria monocytogenes : a case report and literature review

La romboencefalitis por Listeria monocytogenes es una presentación poco común de la listeriosis del sistema nervioso central; sin embargo, es la presentación más común en personas inmunocompetentes. Aun más rara es la combinación de romboencefalitis con mielitis causada por L. monocytogenes ; no obstante, en este artículo se reporta un caso de encefalitis del tallo y mielitis grave en un paciente sin compromiso del sistema inmunitario. Se presenta un paciente de 21 años de edad, sin deficiencias del sistema inmunitario, que consumió productos lácteos no pasteurizados y, posteriormente, presentó un cuadro de cefalea, vómito, deterioro de su estado general y, finalmente, alteración del estado de conciencia y muerte. Consultó al Instituto Neurológico de Colombia y se hizo diagnóstico de encefalitis del tallo y mielitis por L. monocytogenes . Se discuten las diferencias entre el caso presentado y los reportados en la literatura científica. Ante un paciente con signos de compromiso del tallo cerebral, de posible origen infeccioso, es prudente iniciar tratamiento antibiótico para L. monocytogenes y, en caso de poca respuesta, escalar rápidamente en dicho tratamiento. También lo es extender el estudio radiológico hacia la columna vertebral, con el fin de descartar compromiso de la médula espinal.

Brainstem encephalitis caused by Listeria monocytogenes is an uncommon form of central nervous system listeriosis; however, it is the most common presentation in immunocompetent individuals. Here, we describe an even more rare combination of rhombencephalitis with severe myelitis caused by L. monocytogenes in an immunocompetent patient. We report the case of a 21-year-old immunocompetent patient who consumed unpasteurized dairy products and experienced headache and vomiting that progressed to an impaired general condition, altered consciousness and ultimately death. The patient had presented to the Neurological Institute of Colombia (INDEC in Spanish) for consultation and was diagnosed with brainstem encephalitis and myelitis caused by Listeria monocytogenes . The differences between this particular case and those reported in the literature will be discussed. It is advisable to initiate antibiotic treatment for Listeria monocytogenes if a patient shows signs of brainstem compromise of possible infectious origin and quickly intensify treatment if there is no or minimal response. It is also necessary to extend radiological assessment to include the spinal column to rule out spinal cord involvement.

Dorsal disc prolapse with myelopathy: physical therapy influence

The purpose of this study was to identify the effect of postero-anterior spinal manipulation on dorsal disc prolapse with mild myelopathy; regarding back pain, ROM of lower dorsal trunk flexion and cadence. Ten volunteer's patients participated in the study. Their age ranged from 34 to 48 years, with duration of illness from one to six months. Patients were diagnosed clinically and by MRI as a dorsal disc prolapse. All patients complained of myelopathy with paraparesis with grade 1+ or 2 spasticity according to a modified Ashworth scale. VAS was used to measure local pain intensity. Functional plain X-ray was used to measure ROM of lower dorsal trunk flexion. Cadence was used as a kinematic parameter of gait for those patients. All these evaluation procedures were performed before and at the end of physiotherapy program. Physiotherapy consisted of ultrasound, hot packs and postero-anterior central spinal manipulation. This program was applied three times per week; for six weeks. Results of this study revealed a significant decrease of local back pain intensity at the end of the treatment program. Results also showed a significant increase of both ROM of lower dorsal trunk flexion and cadence at the end of treatment program. Results presented a strong correlation between clinical and kinematic parameters. In conclusion, postero-anterior central spinal manipulation was an efficient and non risky maneuver in the treatment of dorsal disc prolapse with mild myelopathy. Both local and cord manifestations can be improved in these patients

Algunas complicaciones neurológicas de la brucelosis / Some neurological complications of the brucellosis

Se presenta tres casos de Brucelosis con alteraciones neurológicas, caracterizadas por meningoencefalitis, meningomielitis y poliradiculoneuritis. El diagnóstico se comprobó por títulos altos para Brucella en el suero para los tres casos y hemocultivo positivo en los dos primeros. Todos los pacientes presentaron LCR de carácter inflamatorio y evolución favorable cuando el tratamiento antibiótico fue el adecuado. En los tres casos el diagnóstico inicial fue rectificado posteriormente por las pruebas de laboratorio. Se revisa la literatura acerca del diagnóstico de las complicaciones neurológicas y se dan recomendaciones actualizadas para el tratamiento de las diversas formas de Neurobrucelosis