BILATERAL TROCHLEAR PALSY DUE TO CEREBRAL VASCULITIS RELATED TO COVID-19 INFECTION

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Pregnancy, puerperium and cerebrovascular disease

Six patients who presented cerebrovascular disease during puerperium or pregnancy were studied. One of them presented hemorrhagic stroke caused by intracerebral bleeding due to pre-eclampsia. Three patients presented ischemic stroke, they all had positive serologic reactions for Chagas' disease, but only two of them had clinical cardiopathy; one of these patients had anticardiolipin antibody. The other two patients presented cerebral venous thrombosis of sagittal sinus. Both were smokers and one of them used oral contraceptive. We emphasize the importance of clinical investigation to seek for the common causes of cerebrovascular disease in young people, with special attention to Chagas disease in Brazil.Foram estudadas seis pacientes com doenças cerebrovasculares no período gravídico-puerperal. Uma apresentou hemorragia cerebral, três apresentaram infarto cerebral e duas trombose de seio venoso cerebral. A associação de fatores clínicos ao estado peculiar produzido pela gravidez parece ter resultado nas doenças apresentadas, com especial ênfase à doença de Chagas presente em todos os infartos cerebrais.UNIFESP-EPMUNIFESP, EPMSciEL

Early hematopoietic stem cell transplantation in a patient with severe mucopolysaccharidosis II : 7 years follow-up

Mucopolysaccharidosis type II (MPS II - Hunter syndrome) is an X-linked lysosomal storage disorder caused by a deficiency in the enzyme iduronate-2 sulfatase (I2S), leading to the accumulation of the glycosaminoglycans, affecting multiple organs and systems. Enzyme replacement therapy does not cross the blood brain barrier, limiting results in neurological forms of the disease. Another option of treatment for severe MPS, hematopoietic stem cell transplantation (HSCT) has become the treatment of choice for the severe form of MPS type I, since it can preserve neurocognition when performed early in the course of the disease. To date, only few studies have examined the long-term outcomes of HSCT in patients with MPS II. We describe the seven-year follow-up of a prenatally diagnosed MPS II boy with positive family history of severe MPS form, submitted to HSCT with umbilical cord blood cells at 70 days of age. Engraftment after 30 days revealed mixed chimerism with 79% donor cells; after 7 years engraftment remains at 80%. I2S activity 30 days post-transplant was low in plasma and normal in leukocytes and the same pattern is observed to date. At age 7 years growth charts are normal and he is very healthy, although mild signs of dysostosis multiplex are present, as well as hearing loss. The neuropsychological evaluation (Wechsler Intelligence Scale for Children - Fourth Edition - WISC-IV), disclosed an IQ of 47. Despite this low measured IQ, the patient continues to show improvements in cognitive, language and motor skills, being quite functional. We believe that HSCT is a therapeutic option for MPS II patients with the severe phenotype, as it could preserve neurocognition or even halt neurodegeneration, provided strict selection criteria are followed

Assessment of risk scores to predict mortality of COVID-19 patients admitted to the intensive care unit

ObjectivesTo assess the ABC2-SPH score in predicting COVID-19 in-hospital mortality, during intensive care unit (ICU) admission, and to compare its performance with other scores (SOFA, SAPS-3, NEWS2, 4C Mortality Score, SOARS, CURB-65, modified CHA2DS2-VASc, and a novel severity score).Materials and methodsConsecutive patients (≥ 18 years) with laboratory-confirmed COVID-19 admitted to ICUs of 25 hospitals, located in 17 Brazilian cities, from October 2020 to March 2022, were included. Overall performance of the scores was evaluated using the Brier score. ABC2-SPH was used as the reference score, and comparisons between ABC2-SPH and the other scores were performed by using the Bonferroni method of correction. The primary outcome was in-hospital mortality.ResultsABC2-SPH had an area under the curve of 0.716 (95% CI 0.693–0.738), significantly higher than CURB-65, SOFA, NEWS2, SOARS, and modified CHA2DS2-VASc scores. There was no statistically significant difference between ABC2-SPH and SAPS-3, 4C Mortality Score, and the novel severity score.ConclusionABC2-SPH was superior to other risk scores, but it still did not demonstrate an excellent predictive ability for mortality in critically ill COVID-19 patients. Our results indicate the need to develop a new score, for this subset of patients