NMO in pediatric patients: brain involvement and clinical expression / NMO en pacientes pediátricos: afectación del cerebro y expresión clínica

OBJECTIVE: To analyze the clinical, neuroimaging characteristics and positivity of the acquaporin water channel (NMO-IgG) in pediatric patients with neuromyelitis optica (NMO). This disorder could have a variable clinical expression. To address such variability, the term NMO spectrum has been suggested. METHOD: We evaluated six pediatric patients, with a median age of 11 years at the time of the study, with the diagnosis of NMO by the Wingerchuck criteria. RESULTS: All the cases exhibited bilateral optic neuritis (ON). Four patients had abnormalities on brain MRI from the onset,although only three of them developed symptoms correlated to those lesions during the course of their disorder. NMO-IgG was positive in 80 percent. CONCLUSION: Optic neuropathy is the most impaired feature in NMO patients. Brain MRI lesions are not compatible with multiple sclerosis and positivity of the NMO-IgG are also present in NMO pediatric patients, confirming the heterogeneity in the expression of this disorder.

OBJETIVO: Analizar las características clínicas y de neuroimagen, y la positividad del canal de agua acuaporin (NMO-IgG) en pacientes pediátricos con neuromielitis óptica (NMO). Este trastorno puede tener una expresión clínica variable. El término espectro de NMO ha sido propuesto para poder incluir la variabilidad. METODO: Evaluamos seis pacientes pediátricos, con una mediana de edad de 11 años al momento del estudio, con el diagnóstico de NMO de acuerdo a los criterios de Wingerchuck. RESULTADOS: Todos los casos exhibían neuritis óptica bilateral (ON). Cuatro pacientes tuvieron anormalidades en la RM cerebral desde el inicio, aunque solo tres de ellos desarrollaron síntomas relacionados con las lesiones durante el curso de la enfermedad. NMO-IgG fue positiva en el 80 por ciento. CONCLUSIÓN: Neuropatía óptica es la característica mayormente observada en los pacientes con NMO. Lesiones cerebrales en la RM cerebral no compatibles con esclerosis multiple y positividad de la NMO-IgG están también presentes en los pacientes pediátricos con NMO; lo cual confirma la heterogeneidad en la expresión clínica de este trastorno.

Optical coherence tomography evaluation of retinal nerve fiber layer in longitudinally extensive transverse myelitis / Avaliação da camada de fibras nervosas da retina na mielite transversal longitudinalmente extensa usando tomografia de coerência óptica

OBJECTIVE: To compare optical coherence tomography (OCT) measurements on the retinal nerve fiber layer (RNFL) of healthy controls and patients with longitudinally extensive transverse myelitis (LETM) without previous optic neuritis. METHOD: Twenty-six eyes from 26 patients with LETM and 26 control eyes were subjected to automated perimetry and OCT for comparison of RNFL measurements. RESULTS: The mean deviation values from perimetry were significantly lower in patients with LETM than in controls (p<0.0001). RNFL measurements in the nasal quadrant and in the 3-o'clock segment were significantly smaller in LETM eyes than in controls. (p=0.04 and p=0.006, respectively). No significantly differences in other RNFL measurements were found. CONCLUSION: Patients with LETM may present localized RNFL loss, particularly on the nasal side of the optic disc, associated with slight visual field defects, even in the absence of previous episodes of optic neuritis. These findings emphasize the fact that patients with LETM may experience attacks of subclinical optic nerve damage.

OBJETIVO: Comparar as medidas da camada de fibras nervosas da retina (CFNR) usando a tomografia de coerência óptica (TCO) em indivíduos normais e pacientes com mielite transversal longitudinalmente extensa (MTLE) sem episódio prévio de neurite óptica. MÉTODO: Vinte e seis olhos de 26 pacientes com MTLE e 26 olhos normais foram submetidos à campimetria computadorizada e TCO para comparação das medidas da CFNR. RESULTADOS: Valores do parâmetro desvio médio da campimetria computadorizada foram significativamente menores nos pacientes com MTLE do que nos controles (p<0,001). Medidas da CFNR no quadrante nasal e no segmento 3 horas foram significativamente menores nos olhos dos pacientes com MTLE do que nos olhos normais (p=0,04 e p=0,006, respectivamente). Não foi encontrada diferença significante nas outras medidas da CFNR avaliadas. CONCLUSÃO: Pacientes com MTLE podem apresentar perda localizada da CFNR, particularmente na região nasal do disco óptico, associada a defeitos discretos de campo visual, mesmo na ausência de episódio prévio de neurite óptica. Estes achados sugerem que pacientes com MTLE podem apresentar acometimento subclínico do nervo óptico.

Transverse myelitis and chronic urticaria in systemic lupus erythematosus: Case report

Se relata un caso de urticaria crónica y mielitis transversal aguda en asociación con lupus eritematoso sistémico en una mujer de 40 años. La urticaria se inició en su adolescencia y, después de 26 años, presentó fotosensibilidad, poliartritis, meningitis aséptica y mielitis transversal aguda, con anticuerpos antifosfolípidos y antinucleares positivos. Se ha descrito urticaria crónica y mielitis transversal en asociación, o como la primera manifestación del lupus eritematoso sistémico. La mielitis transversal es una condición rara y poco comprendida, diagnosticada en cerca de 2 por ciento de los pacientes con lupus eritematoso sistémico y, muy frecuentemente asociada con anticuerpos antifosfolípidos. Los autores describen un caso de esta rara asociación y resaltan la necesidad de evaluaciones sistemáticas del diagnóstico en pacientes con urticaria crónica, porque esta condición suele ser una manifestación cutánea de enfermedades sistémicas, la puede anteceder por mucho tiempo y dificultar la correcta caracterización del lupus eritematoso sistémico, correlacionado a la usual corticoterapia prolongada.

Mielite transversa como manifestaçäo clínica inicial de linfoma näo Hodgkin disseminado e mielopatia vacuolar associada ao HIV: relato de caso / Transverse myelitis as initial symptom of disseminated non-Hodgkin lymphoma and HIV-associated vacuolar myelopathy: case report

Linfomas näo Hodgkin de alto grau sao comumente relatados em pacientes com a síndrome da imunodeficiência adquirida (AIDS). Comprometendo com grande frequência o sistema nervoso central, particularmente as leptomeninges e os hemisférios cerebrais. O acometimento epidural é pouco frequente, variando de 3,5 por cento a 8,3 por cento de acordo com os registros da literatura. Os autores relatam o caso de um paciente de 27 anos de idade com AIDS, cuja manifestaçao clínica inicial da doença linfomatosa disseminada foi a mielite transversa associada à mielopatia vacuolar. Destaca-se a importância do diagnóstico diferencial precoce das mielopatias na AIDS, em virtude da alta malignidade da neoplasia e da evoluçao extremamente rápida nesses pacientes.

Herpes group viruses diagnosis by polymerase chain reaction in Guillain bare syndrome and transverse myelitis

This study included 35 patients, their ages ranged from 11 months to 7 years. 20 cases presented with clinical manifestation of Guillain Barre syndrome [GBS] and 15 cases with clinical manifestations of transverse myelitis [TM]. All cases were subjected to general and neurological examinations and CSF analysis, computed tomography of dorsolumbar region for 15 cases with transverse myelitis. Polymerase chain reaction [PCR] was done for both CSF and blood of all cases for detection of cytomegalovirus-DNA, herpes simplex DNA and Epstein-Barr- DNA. Preceding illness in the form of upper respiratory infection, gastroenteritis and exanthem were detected in 65% and 39.99% among cases of GBS and TM, respectively. Total outcome among all 35 patients [8 weeks after onset of neurological manifestations] showed complete recovery in 45.71%, recovery with residual squelae in 40% and death in 14.29%. Cytomegalo-virus DNA PCR positive signal was the most commonly detected marker of viral infection in this study

Acute necrotizing myelopathy with complete myelographic block.

Acute necrotizing myelopathy is a rare disorder. A case showing complete myelographic block extending from D3 to D6 level is described. The suspected aetiological factors for acute necrotizing myelopathy are discussed.