关键词： 多发性硬化症   中枢神经系统   神经病学   挪威奥斯陆   呼吸系统   泌尿系统   视神经   癌症风险  

摘要： 在挪威奥斯陆举行的欧洲神经病学大会(2019-07-06)上发表的一份研究显示,挪威一项新的长期研究表明,多发性硬化症(multiple sclerosis,MS)可能会增加患癌风险。总的来说,风险较小,只有12%。然而,患有MS的人患某些癌的风险——如中枢神经系统(脑、脊髓和视神经)癌和泌尿系统癌的风险大约高出50%。研究发现,患有MS的人患上呼吸系统癌的可能性要高出2/3。

关键词： cardiac arrest   induced hypothermia   nutrition   pneumonia   targeted temperature management  

摘要： Background Whether providing nutrition support is beneficial or deleterious during targeted temperature management (TTM) after cardiac arrest is unclear. We therefore performed a retrospective observational study to determine whether early nutrition was beneficial or deleterious during TTM. Methods We retrospectively studied patients admitted to our intensive care unit (ICU) between 2008 and 2014 after successfully resuscitated cardiac arrest. We compared the group given nutrition within 48 hours after ICU admission (E+ group) to the group given nutrition later on or not at all (E- group). Results Of the 203 included patients, 143 were in the E+ group and 60 in the E- group. The E+ group had a significantly higher proportion of patients with a good 3-month neurological outcome (42.7% vs 16.7%, P < 0.001). The difference remained significant after adjustment on a propensity score (odds ratio, 3.47;95% confidence interval, 1.48-8.14;P = 0.004). The cumulative energy deficit for an energy goal of 20 kcal/kg/d from admission to day 7 was significantly lower in the E+ group (3304 +/- 2863 kcal vs 5017 +/- 2655 kcal, P < 0.001). Within the E+ group, the subgroups with nutrition initiation when body temperature was <36 degrees C vs >= 36 degrees C were not significantly different regarding the frequencies of early-onset pneumonia, ventilator-associated pneumonia, vomiting, and prokinetic drug use (all P-values > 0.05). Conclusions Early nutrition after cardiac arrest during TTM appears safe and may be associated with better neurological outcomes. These findings warrant a randomized controlled trial to resolve the remaining issues.

关键词： PLP1   BEX3   TCEAL1   sex limited traits   contiguous gene deletion syndrome   intrachromosomal repeats  

摘要： Xq22 deletions that encompass PLP1 (Xq22-PLP1-DEL) are notable for variable expressivity of neurological disease traits in females ranging from a mild late-onset form of spastic paraplegia type 2 (MIM# 312920), sometimes associated with skewed X-inactivation, to an early-onset neurological disease trait (EONDT) of severe developmental delay, intellectual disability, and behavioral abnormalities. Size and gene content of Xq22-PLP1-DEL vary and were proposed as potential molecular etiologies underlying variable expressivity in carrier females where two smallest regions of overlap (SROs) were suggested to influence disease. We ascertained a cohort of eight unrelated patients harboring Xq22-PLP1-DEL and performed high-density array comparative genomic hybridization and breakpoint-junction sequencing. Molecular characterization of Xq22-PLP1-DEL from 17 cases (eight herein and nine published) revealed an overrepresentation of breakpoints that reside within repeats (11/17, similar to 65%) and the clustering of similar to 47% of proximal breakpoints in a genomic instability hotspot with characteristic non-B DNA density. These findings implicate a potential role for genomic architecture in stimulating the formation of Xq22-PLP1-DEL. The correlation of Xq22-PLP1-DEL gene content with neurological disease trait in female cases enabled refinement of the associated SROs to a single genomic interval containing six genes. Our data support the hypothesis that genes contiguous to PLP1 contribute to EONDT.

关键词： L. monocytogenes meningitis   Adults   Altered consciousness   Seizure   Bacteremia   Hydrocephalus  

摘要： The clinical characteristics and therapeutic outcomes of adult Listeria monocytogenes meningitis are not commonly examined in isolation in the literature. During a study period of 19 years (2000-2018), 366 patients with culture-proven adult bacterial meningitis (ABM) were identified in the author's hospital (264 patients in 2000-2010 and 102 patients in 2011-2018). Of the 366 ABM patients, 330 had monomicrobial infections while the other 36 had mixed infections. L. monocytogenes infection was identified in 11 of the 330 patients with monomicrobial ABM (3 in 2000-2010 and 8 in 2011-2018). These 11 patients included 5 males and 6 females, aged 47 to 76 years (median age = 61.7). None of the 11 patients had a postneurosurgical state as the underlying cause, but 3 of them contracted the infection nosocomially. Common underlying conditions included liver cirrhosis (4), systemic malignancy (3), diabetes mellitus (3), and renal disease (2). The most common clinical manifestations were fever (11), altered consciousness (8), seizure (8), bacteremia (7) and hydrocephalus (5). The therapeutic result revealed a mortality rate of 72.7% (8/11), but no significant prognostic factors were identified. The clinical features of 8 additional Taiwanese L. monocytogenes ABM patients reported in the literature, were also included for analysis. The present study revealed an increase in L. monocytogenes ABM in recent years and most patients presented with severe neurological manifestations. The current study is a preliminary overview of L. monocytogenes meningitis in adults and a further large-scale study is needed for improved delineation of this specific infectious syndrome. (C) 2019 Elsevier Ltd. All rights reserved.

关键词： Epilepsy   PHQ-9   NDDI-E   Depression   Suicidality   Questionnaire   Validation  

摘要： Purpose: We compared the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E) with the Patient Health Questionnaire-9 (PHQ-9) according to different antiepileptic drug (AED) loads in people with epilepsy (PWE). Methods: Depression and suicidality were assessed with the Mini International Neuropsychiatric Interview (MINI), the NDDI-E, and the PHQ-9. A receiver operating characteristic (ROC) curve analysis was used. Sensitivity and specificity of the NDDI-E and PHQ-9 were compared between an AED load < 2 and >= 2. Results: Of 213 participants included, 22.5 % were diagnosed with current depression by the MINI, and 9 % had a moderate to severe risk of suicide. Using the cutoff of 9.5 and 13.5 for the PHQ-9 and NDDI-E, respectively, in the total group, the PHQ had a higher sensitivity (91.7 %) but lower specificity (75.8 %) than the NDDI-E (66.7 % and 87.3 %, respectively) (p < 0.001). In a group with an AED load < 2, the sensitivity and specificity did not differ between the PHQ-9 and NDDI-E. In a group with an AED load 2, however, the PHQ-9 had a higher sensitivity (90.9 %, p < 0.063) but lower specificity (65.2 %, p < 0.001) than the NDDI-E (68.2 % and 81.2 %, respectively). For screening of suicide risk, the sensitivity and specificity were not different between the PHQ-9 and NDDI-E in all tested groups. The negative predictive values of both instruments were above 95 % for suicide risk. Conclusion: In patients with a low AED load, the sensitivity and specificity did not differ between the PHQ-9 and NDDI-E. In contrast, in patients with a high AED load, the PHQ-9 had a poor specificity whereas the NDDI-E had a poor sensitivity.

关键词： Cassava   Linamarin   Acetone cyanohydrin   Neurotoxic   Motor disorder  

摘要： Cassava (Manihot esculenta Crantz) is a tropical plant that is used as fresh food, processed food, or raw material for the preparation of flours with high nutritional value. However, cassava contains cyanogenic glycosides, such as linamarin and lotaustralin, that can trigger severe toxic effects and some neurological disorders, including motor impairment, cognitive deterioration, and symptoms that characterize tropical ataxic neuropathy and spastic epidemic paraparesis (Konzo). These alterations that are associated with the consumption of cassava or its derivatives have been reported in both humans and experimental animals. The present review discusses and integrates preclinical and clinical evidence that indicates the toxic and neurological effects of cassava and its derivatives by affecting metabolic processes and the central nervous system. An exhaustive review of the literature was performed using specialized databases that focused on the toxic and neurological effects of the consumption of cassava and its derivatives. We sought to provide structured information that will contribute to understanding the undesirable effects of some foods and preventing health problems in vulnerable populations who consume these vegetables. Cassava contains cyanogenic glycosides that contribute to the development of neurological disorders when they are ingested inappropriately or for prolonged periods of time. Such high consumption can affect neurochemical and neurophysiological processes in particular brain structures and affect peripheral metabolic processes that impact wellness. Although some vegetables have high nutritional value and ameliorate food deficits in vulnerable populations, they can also predispose individuals to the development of neurological diseases.

关键词： Acute kidney injury   Averrhoa   calcium oxalate   dialysis  

摘要： Star fruit (Averrhoa carambola) is a well-known product in tropical countries. There are few reports published in literature with acute kidney injury due to oxalate induced nephropathy. However, none of them have an important neurological feature. We present a case of a 51-year-old male with paresis and altered mental status. Screening for neurological diseases such as stroke, Guillain-Barre, meningitis and encephalitis were negative. In the evolution, he developed acute kidney failure and was submitted to 4 dialysis sessions. After talking to the family, we discovered he had ingested over 50 star fruits prior to the acute neurologic deficits. He recovered renal function so a renal biopsy was not required. Physicians should actively look for star fruit ingestion history in patients presenting with unexplained acute kidney injury with or without neurological features. Besides, taking star fruit in a large amount, accompanied by an empty stomach and dehydrated state, is a risk factor for neurotoxicity.

摘要： [...]the results of the analysis of the cerebrospinal fluid for infection, cell cytology, protein, and glucose were normal except an elevated white blood cell count, 36 cells/μL. A chest X-ray revealed no abnormal findings. The sensory abnormality identified from the neurologic examination is most likely due to the previous spinal cord trauma. Because the findings of the sensory NCS were normal, gait ataxia was attributed to PCD. [6] Here, we report a case of anti-Hu antibody associated with PCD and motor axonopathy and anti-recoverin antibody associated with retinopathy, which share a common systemic cancer, SCLC. [...]the involvement of more than one system should be considered in a patient with PNS.

关键词： Posterior   post-operative   reversible encephalopathy   syndrome  

摘要： Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiological syndrome that is generally reversible and may lead to permanent neurological damage if left untreated. PRES has been commonly linked with hypertension along with associated vasogenic oedema. Children are more susceptible to these perturbations due to the narrow range of cerebral autoregulation. PRES must be considered in differentials of any neurological dysfunction which is associated with hypertension in the immediate post-operative period. Inadequate pain control in the post-operative period may cause hypertension that may lead to subsequent PRES. We report a case of postoperative PRES in a 12-year-old previously normotensive child posted for splenectomy with an acute rise in blood pressure in the post-operative period.