Anestesia e Manejo da Dor Pós-Operatória Associada à Técnica de Monitorização Neurofisiológica em Oncopediatria: Relato de Caso / Anesthesia and Postoperative Pain Management Associated with the Neurophysiological Monitoring Technique in Pediatric Oncology: Case Repor / Anestesia y Manejo del Dolor Postoperatorio Asociado a la Técnica de Monitoreo Neurofisiológico en Oncología Pediátrica: Informe de Caso

neuroblastoma é o tumor maligno sólido extracraniano mais comum na infância. Entre seis e dez milhões de crianças são identificadas com essa doença por ano. O tratamento cirúrgico, que envolve a ressecabilidade tumoral, é fator decisivo na estratégia terapêutica desses pacientes, com evidente influência no prognóstico da doença. Relato do caso: Lactente submetido à anestesia geral e a bloqueio de nervo periférico para ressecção de neuroblastoma com monitorização neurofisiológica intraoperatória (MNIO). Como estratégia anestésica, foi realizado o bloqueio do plano do eretor da espinha (ESP Block) associado à técnica de analgesia multimodal visando ao controle da dor. A dor pós-operatória foi avaliada por meio da Children's and Infants' Postoperative Pain Scale (CHIPPS). Conclusão:A MNIO, apesar de restringir as técnicas anestésicas, tem se firmado como uma importante ferramenta para monitorar as funções neurológicas durante as abordagens cirúrgicas com envolvimento do sistema nervoso central, com a finalidade de evitar danos permanentes de importantes estruturas. A interação e a comunicação de toda a equipe são de suma importância nos resultados pós-operatórios

Neuroblastoma is the most common non-central nervous system in childhood. From six to ten million children are identified with this disease annually. Surgical treatment, which involves tumor resectability, is a decisive factor in the therapeutic strategy of these patients, with clear influence on disease's prognosis. Case report: Nursling submitted to general anesthesia and peripheral nerve block for neuroblastoma surgical resection with intraoperative neurophysiological monitoring (IONM) and spinal erector plane block(ESP), associated with the multimodal analgesia technique for pain control. The postoperative pain was evaluated using the Children's and Infants' Postoperative Pain Scale (CHIPPS). Conclusion: IONM has established itself as an important tool to monitor neurological functions during surgical approaches with involvement of the central nervous system, in order to avoid permanent damage to important structures. The interaction and communication of the entire team are of paramount importance in the postoperative results.

Neuroblastoma es el tumor maligno sólido extracraneano más común en la infancia. Entre seis y diez millones de niños son identificados con esta enfermedad por año. El tratamiento quirúrgico, que implica la resecabilidad tumoral, es un factor decisivo en la estrategia terapéutica de estos pacientes, con una influencia evidente en el pronóstico. Informe del caso: Lactante sometido a anestesia general y bloqueo nervioso periférico para resección de neuroblastoma con monitoreo neurofisiológico intraoperatorio (MNIO). Como estrategia anestésica, para resección del neuroblastoma, se realizó bloqueo del plano erector espinal (Bloqueo ESP), asociado a la técnica de analgesia multimodal para controlar el dolor. El dolor postoperatorio se evaluó mediante la Children's and Infants' Postoperative Pain Scale (CHIPPS). Conclusión: La MNIO, a pesar de restringir las técnicas anestésicas, se ha posicionado como una importante herramienta para monitorear las funciones neurológicas durante los abordajes quirúrgicos que involucran al sistema nervioso central, con el fin de evitar daños permanentes a estructuras importantes. La interacción y la comunicación de todo el equipo son de suma importancia en los resultados posoperatorios

Is Asian type MS an MS phenotype, an NMO spectrum disorder, or a MOG-IgG related disease?

BACKGROUND: A specific particularity of neurological diseases in Asia is the relative commonality of neuromyelitis optica (NMO) and Asian type MS (OSMS). Both conditions also occur in South American patients. The Brazilian population differs from the European and the Asian populations due to the mixture of ancestralities between European colonizers and African slaves. To better know the clinical characteristics of Brazilian patients with Asian type MS this study aimed to analyze the clinical, radiological and serological data that would help to distinguish between OSMS and NMO and clarify, in a Non-Asian population, if OSMS is an MS phenotype, an NMO spectrum disorder by 2015 classification, or a complement activating antibody to myelin oligodendrocyte glycoprotein (MOG-IgG) antibody-related disease. METHODS: We selected cases retrospectively with NMO and OSMS in the medical registry of patients with idiopathic inflammatory demyelinating diseases under follow-up since 1997 in Federal Hospital da Lagoa, the principal reference center for MS treatment in Rio de Janeiro, Brazil. OSMS has selective involvement of the optic nerve and spinal cord with no cerebral or cerebellar symptoms associated with small spinal cord lesions and negativity for the aquaporin-4 antibody (AQP4-IgG). NMO full-filled the revised criteria (2006) associated with longitudinally extensive transverse myelitis (LETM). We recorded the following data: ethnicity/skin color, neurologic impairment "at nadir" and "at recovery" of the index events (optic neuritis and transverse myelitis), long term disability, mortality, health quality of life scores by the SF-36 questionnaire, CSF IgG oligoclonal bands and serological AQP4-IgG and MOG-IgG antibodies tested by Cell-based assay. The last brain MRIs were classified as either satisfying or not satisfying MAGNIMS radiologic criteria for MS or typical or not typical for NMOSD. The new classification of NMO spectrum disorders (2015) was applied. RESULTS: Forty-one OSMS and 122 NMO cases were analyzed. OSMS affected mainly young white women, causing unilateral optic neuritis and partial myelitis with excellent recovery. After a mean disease duration of 20 years, 90% of the patients had free ambulation, and 70% had a mild disability or no disability. Only 7.2% presented a secondary progressive course, and no deaths occurred. All cases had negativity to AQP4-IgG and MOG-IgG biomarkers. 95% had resonance criteria for MS. OSMS differed from NMO by ethnicity, morbidity, and mortality: most were African descendants, with severe motor and visual dysfunction, and one third died. Only NMO cases full-filled the new NMOSD classification (52 AQP4-IgG positive, 29 AQP4-IgG negative, and 41 AQP4-IgG unknown). CONCLUSION: In Brazilian patients, OSMS and NMO are different immune-mediated diseases. OSMS is a milder MS phenotype.

Case Report: Acute Transverse Myelitis after Zika Virus Infection.

We report here one case of Zika virus (ZIKV) infection associated with auto-immunity directed against the central nervous system in a Brazilian woman who developed acute transverse myelitis 9 days after recovery from an acute episode of fever with generalized erythema. Imaging of the spinal cord showed an elongated area on the T1-T10 level with gadolinium uptake. The diagnostic of the ZIKV infection was confirmed by cerebrospinal fluid and serum analysis. This patient had serum positivity for autoantibodies against myelin oligodendrocyte glycoprotein (MOG), a specific antibody against the myelin sheath. We propose that a direct central nervous system infection by ZIKV could lead to a specific auto-immunity against MOG protein.

Lower frequency of antibodies to MOG in Brazilian patients with demyelinating diseases: An ethnicity influence?

OBJECTIVE: Antibodies against Myelin Oligodendrocyte glycoprotein (MOG-Ab) have been investigated as potential biological marker for neuromyelitis optica (NMO) and high-risk syndromes (HR) negative for AQP4-Ab in populations with different ethnic background. We tested AQP4 and MOG antibodies in a Brazilian population with high African ethnic background. METHOD: The study population was composed of adult patients from Rio de Janeiro with inflammatory demyelinating diseases (new and old cases). Blood samples were sent blindly to test the AQP4 and MOG antibodies by CBA. The frequency of positive MOG-Ab was estimated in the NMOHR and the NMO spectrum disorders (NMOSD). A systematic review with meta-analysis assessed the frequency of MOG-Ab in Caucasians and non-Caucasians. RESULTS: 200 adult patients (52% Afro-Brazilian) 115 of them with NMOHR were tested. MOG antibodies were found in 5/68 negative cases of AQP4-Ab negative (7%). The criteria for NMOSD were fulfilled by 70 patients with NMOHR and none of them was positive for MOG-Ab. A low prevalence of MOG antibodies and a predominant phenotype of bilateral Optic Neuritis were found in most non-Caucasian patients. CONCLUSION: The low frequency of MOG Ab in patients from Rio de Janeiro and in other non-Caucasian populations suggests a racial/ancestral influence.

Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study.

The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%), increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS prevalence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients' demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%), ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs). Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect immunofluorescence (IIF) with a composite substrate of mouse tissues in 200 NMOSD cases was positive in people with NMO (95/162; 58.6%), longitudinally extensive transverse myelitis (10/30; 33.3%) and bilateral or recurrent optic neuritis (8/8; 100%). No association of NMO-IgG antibody positivity was found with gender, age at onset, ethnicity, early or late onset forms, disease course, or long-term severe disability. The relative frequency of NMO among relapsing-remitting MS (RRMS) + NMO cases in SA was 14.0%. Despite the high degree of miscegenation found in SA, MS affects three quarters of all patients with IIDD, mainly white young women who share similar clinical characteristics to those in Western populations in the northern hemisphere, with the exception of ethnicity; approximately one-third of all cases occur among non-white individuals. At the last assessment, the majority of RRMS patients showed mild disability, and the risk for secondary progression was significantly superior among those of African ethnicity. NMO comprises 11.8% of all IIDD cases in SA, affecting mostly young African-Brazilian women, evolving with a recurrent course and causing moderate or severe disability in both ethnic groups. The South-North gradient with increasing NMO and non-white individuals from Argentina, Paraguay, Brazil and Venezuela confirmed previous studies showing a higher frequency of NMO among non-white populations.

Acute encephalopathy with bilateral thalamotegmental involvement and a benign course: a case report from Brazil.

This rare encephalopathy that generally affects children is preceded by a respiratory infection and fever associated with convulsions and may progress to coma. Outcome is catastrophic in most cases. This case report describes a Brazilian child of African descent with fever, cephalea and bilateral amaurosis, who evolved to coma with pyramidal signs and associated convulsions. MRI showed diffuse, symmetrical lesions in the thalamotegmental region and brainstem. Following administration of methylprednisolone, the clinical condition of the patient improved and the brain lesions regressed, leaving the child with no current neurological deficits. This was a case of acute postinfectious encephalopathy, involving various brain structures. Outcome was favourable with no sequelae following therapy. This case was atypical due to the bilateral visual involvement and extensive encephalic lesions in a child of African descent with no neurological sequelae following therapy. No other similar cases have been reported in the literature.