关键词： Extracranial-intracranial bypass   Leg plegia   Perfusion  

摘要： While intracranial ischemic insults often result in neuronal death and permanent neurological deficits, some patients may develop potentially reversible neurological dysfunction from persistent hypoperfusion, as surviving neurons remain in an idling state. We report a unique series of three patients with long-standing neurological deficits who underwent EC-IC bypass for repeated TIAs and demonstrated unexpected, rapid resolution of long-standing pre-existing neurological deficits. We suggest that these individuals harbored regions of underperfused, idling neurons that responded rapidly to restored cerebral reperfusion.

关键词： Stroke   Pediatrics   Schimke immuno-osseous dysplasia   Transient ischemic attack   Reversible cerebral vasoconstriction syndrome   Treatment  

摘要： Background: Schimke immuno-osseous dysplasia is a rare autosomal recessive disease resulting from biallelic SMARCAL1 mutations. It presents in early childhood and is characterized by short stature, nephropathy, and immunodeficiency. Approximately 50% of those affected have neurological complications including migraines, transient ischemic attacks, and strokes. Methods: We present a six-year-old boy with Schimke immuno-osseous dysplasia without evidence of atherosclerosis with recurrent episodes of severe headache, fluctuating hemiparesis, and aphasia. Results: Magnetic resonance imaging and angiography were normal during the initial episode;multiple areas of reversible restricted diffusion with decreased perfusion and arterial stenosis were seen with subsequent attacks. Conclusions: This constellation of symptoms and imaging findings is suggestive of reversible cerebral vasoconstriction syndrome, which we propose as a mechanism for the transient ischemic attacks and infarcts seen in some patients with Schimke immuno-osseous dysplasia, as opposed to accelerated atherosclerosis alone. This new insight may provide a basis for novel preventative therapy in this rare disorder. (C) 2018 Elsevier Inc. All rights reserved.

关键词： 25-hydroxyvitamin D   early neurological deterioration   ischemic stroke  

摘要： Background and Aims Vitamin D deficiency has been linked to a higher risk of ischemic stroke. We therefore explored the relationship between serum 25-hydroxyvitamin D [25(OH)D] levels and early neurological deterioration (END) after acute ischemic stroke in a hospital-based prospective study. Methods From June 2016 to June 2018, patients with ischemic stroke within 48 hr from symptom onset were consecutively recruited. Serum 25(OH)D levels were measured at admission. END was defined as an increase of >= 1 point in motor power or >= 2 points in the total National Institute of Health Stroke Scale score within 7 days after admission. Multiple logistic regression models were performed to calculate the odds ratio (OR) and confidence intervals (CI) of 25(OH)D levels in predicting END. Results A total of 478 subjects were enrolled, of which 136 (28.5%) patients developed END. The mean 25(OH)D levels were 49.5 +/- 15.8 nmol/L. Univariate logistic regression analysis showed that advanced age, white matter lesions, high level of body mass index, diastolic blood pressure, fasting blood glucose and homocysteine, and low 25(OH)D levels were associated with END. Furthermore, multivariate regression analysis demonstrated that the first quartile of 25(OH)D concentrations [OR, 2.628;95% CI,1.223-5.644;p = 0.013] was independently risk factor for END. Conclusions This study illustrated that lower 25(OH)D levels might be associated with an increasing risk of END in acute ischemic stroke patients.

关键词： postnatal repair   prenatal diagnosis   spina bifida  

摘要： Objective Parents faced with the choice between postnatal management and prenatal surgery for spina bifida need to have up-to-date information on the expected outcomes. The aim of this study was to report the long-term physical and neurological outcomes of infants with prenatally diagnosed isolated spina bifida that underwent postnatal surgical repair and were managed by a multidisciplinary team from a large tertiary center. Methods This was a retrospective cohort study of all cases of fetal spina bifida managed in a tertiary unit between October 1999 and January 2018. All cases of fetal spina bifida from the local health region were routinely referred to the tertiary unit for further perinatal management. Details on surgical procedures and neonatal neurological outcomes were obtained from institutional case records. Ambulatory status, bladder and bowel continence and neurodevelopment were assessed at a minimum of 3 years. Results During the study period, 241 pregnancies with isolated spina bifida were seen in the unit. Of these, 84 (34.9%) women opted to continue with the pregnancy after multidisciplinary counseling by clinicians. Sixty-seven infants underwent postnatal repair of spina bifida aperta and were included in the analysis. After birth, hindbrain herniation was observed in 91.5% of infants with only seven requiring surgical decompression. Ventriculoperitoneal shunt placement was needed in 64.2% of infants, while normal cognitive development or mild impairment was demonstrated in 85.4% of cases with data for this outcome available, at a mean age of 8 years. Cumulatively, 40% of infants were walking independently or using minor support, and normal or mild impairment of bladder and bowel function was reported in 45.5% and 44.4% of infants, respectively. Conclusions Neurodevelopmental and neurological outcomes between prenatal and postnatal repair are similar. As with fetal surgery, conventional postnatal surgery is associated with the reversal of hindbrain h

关键词： Acute disseminated encephalomyelitis   Post-malaria neurological syndrome   Plasmodium falciparum   Magnetic resonance imaging  

摘要： There are four neurological complications that can occur after malaria treatment at a time when the patient is aparasitaemic: delayed cerebellar ataxia, acute inflammatory demyelinating polyneuropathy, post-malaria neurological syndrome and acute disseminated encephalomyelitis (ADEM). The authors describe a case of a 54-year-old male who presented with encephalopathy and generalized seizures forty-three days after complete recovery from acute malaria by Plasmodium falciparum. Diagnosis of post-malaria ADEM was made based on the acute onset of the neurological symptoms, characteristic findings in magnetic resonance imaging of the brain and prompt response to steroid therapy. ADEM is an autoimmune demyelinating disease of the central nervous system that usually arises after an infection or vaccination. Its occurrence after malaria infection is relatively rare, and to the best of our knowledge there are only thirteen cases described in the literature.

摘要： Background: Cardiac arrest (CA) due to pulmonary embolism (PE) is associated with low survival rates and poor neurological outcomes. We examined whether Extracorporeal Cardiopulmonary Resuscitation (ECPR) improves the outcomes of patients who suffer from CA due to massive PE. Methods: We retrospectively included 39 CA patients with proven or strongly suspected PE in two hospitals in the Netherlands, in a 'before/after'design. 20 of these patients were treated with Conventional Cardiopulmonary Resuscitation (CCPR) and 19 patients with ECPR. Results: The main outcomes of this study were ICU survival and favourable neurological outcome, defined as Cerebral Performance Category (CPC) score 1-2. The ICU survival rate in CCPR patients was 5% compared to 26% in ECPR patients (p<0.01). Survival with favourable neurological outcome was present in 0/20 (0%) CCPR patients compared to 4/19 (21%) of the ECPR patients (p<0.05). Conclusion: ECPR seems a promising treatment for cardiac arrest patients due to (suspected) massive pulmonary embolism compared to conventional CPR, though outcomes remain poor.

摘要： This commentary is on the original article by Pinchefsky et al. on pages350-358 of this issue.

关键词： Functional neurological symptoms   Conversion disorder   Children   Adolescents   Treatment  

摘要： Aim: To describe a cognitive-behavioural treatment and clinical outcomes in a series of children with functional neurological symptoms (FNS). Method: Thirty-six children with FNS were assessed and of these twenty-two (13 male, 9 female) with a mean age 14.5 years (SD = 2.6, range 6-17 years) completed treatment with cognitive behaviour therapy embedded in routine child and adolescent clinical/systemic practice. Treatment outcomes were measured at baseline and post-intervention on the Child Global Assessment Scale (CGAS), Strengths and Difficulties Questionnaire (SDQ), Goal Based Outcomes (GBO) and Revised Child Anxiety and Depression Scale (RCADS). Results: Scores on the CGAS improved significantly post-intervention (p < 0.001) with 82% of participants showing reliable change. Individualised goals (GBO) also showed clinically meaningful gains. Standard measures of emotional and behavioural symptoms (SDQ and RCADS) did not correlate well with clinical diagnoses, were usually subthreshold at baseline, and did not show significant improvement post-intervention. Interpretation: The outcome of this pilot study suggests that CBT can be effective in the rehabilitation of young patients with FNS. Detection of common comorbid psychiatric disorders was not assisted by use of standardised measures, although most participants were clinically anxious or depressed. More research is needed to understand why children with FNS and their parents may not endorse mental health symptoms on questionnaires, and to further evaluate interventions within randomised controlled trials. (C) 2018 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

关键词： Autoimmune neurology   Multiple sclerosis   Neuromyelitis optica   Acute disseminated encephalomyelitis   Autoimmune encephalitis   Sarcoidosis  

摘要： Autoimmune disorders of the central nervous system are common and often affect people in the most productive years of their lives. Among primary autoimmune diseases of the central nervous system, multiple sclerosis is most prevalent in the United States. Many other autoantibody-mediated neurologic syndromes have been identified within the past 2 to 3 decades, including neuromyelitis optica and anti-N-methyl-D aspartate receptor encephalitis. Finally, the central nervous system can also be affected by systemic autoimmune diseases such as sarcoidosis. Many of these diseases are treatable when detected early.