Microanatomical Nerve Architecture of 6 Mammalian Species: Is Trans-Species Translational Anatomic Extrapolation Valid?

Background and Objectives Various animal models have historically been used to study iatrogenic nerve injury during performance of conduction nerve blocks. Our aims were to compare the microstructures of nerves in commonly used species to those of humans and to explore the validity of the extrapolating these findings to humans. Methods High-resolution, light-microscopic images were obtained from cross sections of sciatic nerves at their bifurcation from fresh rat, rabbit, pig, sheep, dog, and human cadavers. Various microanatomical characteristics were measured and compared between the species. P

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Pediatric Collagenous Gastritis and Colitis: A Case Series and Review of the Literature

Introduction: Collagenous gastritis is a rare disease characterized by the subepithelial deposition of collagen bands. Two phenotypes of the disease have been described: a pediatric-onset and an adult-onset type. The adult-onset form is associated with collagenous colitis and autoimmune disorders. No effective treatment has been identified to date. Objective: We aim to describe the clinical features and outcomes of patients in our cohort and provide a summary of published pediatric cases with collagenous gastritis and colitis reported to date to gather information that will contribute to improved knowledge of this rare condition. Methods: A retrospective chart review of all patients with collagenous gastritis and/or colitis who were treated at the Royal Children's Hospital, Melbourne, was performed. A literature review was also conducted. Results: A total of 12 cases of collagenous gastritis were reviewed. Three of 12 (25%) patients had associated collagenous colitis. The most common clinical presentation was iron deficiency anemia. Nine (75%) patients were followed up, and repeat endoscopies were performed in 8 (67%). Iron deficiency anemia resolved in all patients on oral iron supplementation. Histologic improvement was only identified in one patient with the adult phenotype who had been treated with oral corticosteroids and azathioprine. Conclusions: Collagenous gastritis is a rare condition in children. A small proportion of children develop features of the "'adult" phenotype at a very young age. Patients with collagenous gastritis require long-term follow-up and monitoring of their disease. Further randomized clinical trials are needed to establish an effective therapeutic strategy. Address correspondence and reprint requests to Dr Judy Matta, MD, Department of Gastroenterology and Clinical Nutrition, Saint George Hospital University Medical Center, P.O. Box 166 378, Achrafieh, Beirut 11 00 2807, Lebanon (e-mail: judymatta@gmail.com). Received 21 June, 2017 Accepted 24 February, 2018 Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). The other authors report no conflicts of interest. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Multivariate Model for the Prediction of Severity of Acute Pancreatitis in Children

This study aimed to develop a severity prediction system for pediatric patients with acute pancreatitis (AP) based on clinical and laboratory parameters recorded at disease onset. A retrospective cohort study including 130 patients with AP, aged 0 to 18 years, was conducted. Correlations between severe AP (SAP) and clinical and laboratory data were established. Parameters with a significant statistical correlation (P ≤ 0.05) were incorporated in logistic regression models, and receiver operating characteristic curves were generated. The best-performance cutoff points were calculated to propose a severity prediction score, for which sensitivity and specificity were determined. Thirty-eight cases (29.2%) were consistent with SAP. A value of ≥1 point yielded a sensitivity of 81.5% and specificity of 64.1% for SAP prediction, when using a score including blood urea nitrogen ≥12.5 mg/dL (1 point) or hemoglobin

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Gavage Feed Volume Determines the Gastric Emptying Rate in Preterm Infants

Objective: Feeding intolerance, manifesting as increased gastric residual, is a common finding in preterm neonates. Little is known about the regulation of gastric emptying early in life and the extent to which this plays a role in the preterm infants' feeding tolerance. The goal of this study was to evaluate clinically stable 28- to 32-week gestation neonates during the first 4 weeks of life and noninvasively determine their gastric emptying rate. Study design: Ultrasound measurements of gastric milk content volume were obtained from 25 neonates immediately after, 30 and/or 60 minutes following routine gavage feeds. The content emptying rate was calculated from the gastric volume data. Results: Gastric emptying rate was not postnatal age-dependent, was significantly higher at 30 minutes, whenever compared with 60-minute postfeed and directly proportional to the feed volume. At any postnatal age, the gastric emptying rate was at least 6-fold greater, when comparing the lowest and highest average stomach content volumes. Conclusions: The gastric emptying rate of preterm infants is content volume-dependent and unrelated to the postnatal age. Given the present findings, further investigation on the gastric residual of preterm infants receiving larger than currently administered feed volumes at the initiation of enteral nutrition, is warranted. Address correspondence and reprint requests to Jaques Belik, MD, Physiology & Experimental Medicine Program, Department of Paediatrics and Physiology, The Hospital for Sick Children Research Institute, Peter Gilgan Centre for Research and Learning, University of Toronto, 686 Bay Street, 9th Floor, Room 09.9713, Toronto, ON M5G 0A4, Canada (e-mail: jaques.belik@sickkids.ca) Received 1 September, 2017 Accepted 6 March, 2018 Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). Funding sources: This study was supported by a grant from the Canadian Institutes of Health Research to J.B. (MOP 133664) and Brazilian Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq; SWE 202940/2015-2) for C.H.F.F. No honorarium, grant, or other form of payment was given to anyone to produce the manuscript. The authors report no conflicts of interest. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Detection of Cytomegalovirus in Colonic Mucosa of Children With Inflammatory Bowel Disease

The aim of this study was to determine the frequency and management of cytomegalovirus (CMV) detection in colonic mucosa of children with inflammatory bowel disease (IBD). Children with moderate-to-severe colitis who had sigmoid biopsy specimens evaluated for CMV by hematoxylin and eosin (H&E) staining, immunohistochemistry (IHC), and polymerase chain reaction (PCR) were included. A total of 90 sigmoid biopsies were collected from 67 patient encounters from 58 patients with colitis including biopsy samples from colectomy specimens of 8 patients who had colectomy during the study period. Four of 61 patient encounters (6.6%) with ulcerative colitis/IBD-unclassified, 2 with corticosteroid refractory disease, had positive biopsies for CMV by PCR but negative H&E and IHC. They responded to escalated medical therapy, without needing anti-viral therapy or colectomy over a median duration of follow-up of 1.1 year. CMV presence is uncommon in colonic mucosa of children with inflammatory bowel disease. Address correspondence and reprint requests to Wael El-Matary, MD, MSc, FRCPCH, FRCPC, Associate Professor and Section Head, Section of Pediatric Gastroenterology, Departments of Pediatrics, Faculty of Medicine, University of Manitoba AE 408, Health Sciences Centre, 840 Sherbrook St., Winnipeg, Manitoba R3A 1S1, Canada (e-mail: welmatary@hsc.mb.ca). Received 3 September, 2017 Accepted 27 February, 2018 Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). Source of funding: W.E.-M. is supported with a grant from the University of Manitoba and the Children's Hospital Research Institute of Manitoba. The authors report no conflicts of interest. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Proton Pump Inhibitors and Infant Pneumonia/Other Lower Respiratory Tract Infections: National Nested Case-control Study

Objective: The possible association between the use of proton pump inhibitors (PPIs) and community-acquired pneumonia (CAP) or any lower respiratory tract infection (LRTI) remains uncertain. We conducted a nested case-control study using routinely collected national health and drug dispensing data in New Zealand to examine the risk of CAP or any LRTI resulting in hospitalization or death in infants dispensed a PPI. Study Design: The cohort included 21,991 patients without a history of CAP or any LRTI who were born between 1 January 2005 and 31 December 2012 and were dispensed omeprazole, lansoprazole, or pantoprazole (the PPIs available in New Zealand during the study period) on at least one occasion during the first year of life. Cases had a first diagnosis after cohort entry (first PPI dispensing) of CAP (n = 65) validated by hospital discharge letter or death record, and chest radiography; or LRTI (including CAP) (n = 566) validated by hospital discharge letter or death record, with or without chest radiography. Up to 10 controls, matched by sex and date of birth, were randomly selected for each case. We conducted complete case analyses for the fully adjusted models. Results: In the adjusted analysis based on CAP cases and their controls, the matched odds ratio for current versus past use of PPIs was 0.88 (95% confidence interval 0.36–2.16). For all LRTI cases and their controls, the matched odds ratio was 1.13 (0.87–1.48). Conclusion: In otherwise healthy community-dwelling infants, current use of a PPI does not appear to increase the risk of CAP or other LRTIs. Address correspondence and reprint requests to Mei-Ling Blank, MPH, Department of Preventive and Social Medicine, University of Otago, PO Box 56, Dunedin 9054, New Zealand (e-mail: meiling.blank@otago.ac.nz) Received 7 November, 2017 Accepted 6 March, 2018 Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). Contributors' Statement The New Zealand Pharmacovigilance Centre and Medsafe, and a Strategic Research Grant from the Department of Preventive and Social Medicine, Dunedin School of Medicine, University of Otago, New Zealand. None of the funders had a role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. The authors report no conflicts of interest. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Neurodevelopmental Outcomes in Children With Liver Diseases: a Systematic Review

Objective: The aim of the study was to determine the neurodevelopmental outcomes of children with liver diseases based on a systematical review of the literature. Method: A literature search according to the PRISMA statement was conducted using predefined search terms in PubMed, Cochrane Library, and PsycINFO. The inclusion criterion was studies published from 2000 onwards that reported on the neurodevelopmental outcomes of term-born children with liver diseases. A narrative synthesis was done to appraise the studies. Results: Twenty-five studies were included (1913 children), 19 of which described children after liver transplantation (LTx; 1372 children). Sixty-seven percentage of the studies on children with liver diseases who survived with their native livers showed low-average or abnormal scores on specific subscales of cognitive and behavioral measures. In studies on children after LTx, this was 82%. After LTx, 83% of studies demonstrated impaired outcomes on behavior, whereas 42% of children received special education. Motor development was impaired in 82% of studies in children with native liver and after LTx. Limitations: Studies were heterogenic because of sample sizes, etiology of liver disease and type of assessment tools used. Conclusions: More than two-third of included studies showed neurodevelopmental deficits in children with liver diseases, affecting all neurodevelopmental areas. Knowledge on risk factors for impaired neurodevelopment is limited and lack of long-term follow-up is worrying, especially considering the increasing survival rates, resulting in more at-risk patients. Studying early predictors and risk factors of abnormal developmental trajectories of children with liver diseases is indicated to assess strategies to improve their long-term neurodevelopmental outcomes. Address correspondence and reprint requests to Janneke L.M. Bruggink, MD, PhD, Division of Pediatric Surgery, Department of Surgery, University Medical Center Groningen, P.O. Box 30.001, 9700 RB Groningen, the Netherlands (e-mail: j.l.m.bruggink@umcg.nl). Received 22 November, 2017 Accepted 11 March, 2018 The authors report no conflicts of interest. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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A Celiac Diasease Associated lncRNA Named HCG14 Regulates NOD1 Expression in Intestinal Cells

Objective: To identify additional celiac disease associated loci in the Major Histocompatibility Complex independent from classical HLA risk alleles (HLA-DR3-DQ2) and to characterize their potential functional impact in celiac disease pathogenesis at the intestinal level. Methods: We performed a high resolution SNP genotyping of the MHC region, comparing HLA-DR3 homozygous celiac patients and non-celiac controls carrying a single copy of the B8-DR3-DQ2 conserved extended haplotype. Expression level of potential novel risk genes was determined by RT-PCR in intestinal biopsies and in intestinal and immune cells isolated from control and celiac individuals. Small interfering RNA-driven silencing of selected genes was performed in the intestinal cell line T84. Results: MHC genotyping revealed two associated SNPs, one located in TRIM27 gene and another in the non-coding gene HCG14. After stratification analysis, only HCG14 showed significant association independent from HLA-DR-DQ loci Expression of HCG14 was slightly downregulated in epithelial cells isolated from duodenal biopsies of celiac patients, and eQTL analysis revealed that polymorphisms in HCG14 region were associated with decreased NOD1 expression in duodenal intestinal cells. Conclusions: We have sucessfully employed a conserved extended haplotype-matching strategy and identified a novel additional celiac disease risk variant in the lncRNA HGC14. This lncRNA seems to regulate the expression of NOD1 in an allele-specific manner. Further functional studies are needed to clarify the role of HCG14 in the regulation of gene expression and to determine the molecular mechanisms by which the risk variant in HCG14 contributes to celiac disease pathogenesis. Address correspondence and reprint requests to Dr. Izortze Santin, Endocrinology and Diabetes Research Group, BioCruces Health Research Institute, UPV-EHU, E48903-Barakaldo, Bizkaia, Basque Country, Spain (e-mail: izortze.santingomez@osakidetza.eus). Received 11 December, 2017 Accepted 11 March, 2018 Conflicts of interest: None declared. Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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The Membrane-bound O-Acyltransferase7 rs641738 Variant in Pediatric Nonalcoholic Fatty Liver Disease

Background: The rs641738 polymorphism in the membrane-bound O-acyltransferase domain containing protein 7 (MBOAT7) gene has been associated with increased risk of nonalcoholic fatty liver disease (NAFLD). Objectives: To investigate the association between the MBOAT7 rs641738 polymorphism and both hepatic steatosis and biochemical markers of liver damage and to evaluate the potential additive effect of this variant and the I148M patatin-like phospholipase domain-containing 3 (PNPLA3) and the rs58542926 transmembrane 6 superfamily member 2 (TM6SF2) polymorphisms. Methods: One thousand and 2 obese children were genotyped for MBOAT7, PNPLA3, and TM6SF2 polymorphisms and underwent anthropometrical, ultrasonographic, and biochemical evaluation. Indirect measurement of liver fibrosis (Pediatric NAFLD Fibrosis Index [PNFI]) and a genetic risk score from these polymorphisms were calculated. Results: Carriers of the MBOAT7 T allele showed both higher alanine transaminase (ALT) (P = 0.004) and PNFI values (P = 0.04) than noncarriers. These findings were confirmed also for the carriers of the MBOAT7 T allele polymorphism with hepatic steatosis compared with noncarriers. A higher genetic risk score was associated with higher ALT (P = 0.011) and with an odds ratio (OR) to show elevated ALT of 3.4 (95% CI 1.3–5.5, P = 0.003). Patients belonging to genetic risk score 3 group had an OR to present steatosis of 2.6 (95% CI 1.43–4.83, P = 0.0018) compared with those belonging to lower genetic risk score group. Conclusions: We first demonstrated in childhood obesity the role of the MBOAT7 rs641738 variant on serum ALT and the combined effect of the MBOAT7, PNPLA3, and TM6SF2 variants on NAFLD risk. We also provided the first pediatric association of the MBOAT7 polymorphism with indirect markers of liver fibrosis. Address correspondence and reprint requests to Anna Di Sessa, MD, Department of Woman, Child and General and Specialized Surgery, University of Campania "Luigi Vanvitelli," Via L. De Crecchio n° 2, 80138 Naples, Italy (e-mail: anna.disessa@libero.it) Received 13 December, 2017 Accepted 14 March, 2018 Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). The authors report no conflicts of interest. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Early Administration of N-acetylcysteine in the Treatment of Clove Oil Ingestion

No abstract available

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Yield and Examiner Dependence of Digital Rectal Examination in Detecting Impaction in Pediatric Functional Constipation

Objectives: Guidelines on functional constipation recommend digital rectal examination (DRE) when constipation is doubtful or with a suspicion of organic etiology. The guidelines do not clarify if DRE is mandatory to diagnose impaction. This study aims to determine the frequency of impaction detectable only on DRE among children satisfying Rome III criteria without requiring DRE and also the inter-observer influences on impaction detection by DRE. Methods: Children between 6 months to 13 years of age, presenting with history suggestive of constipation were assessed. After excluding those with suspicion of organic etiology, those who needed DRE for diagnosis of constipation and those who do not satisfy Rome III criteria without DRE, the rest who satisfied Rome III criteria were assessed for impaction by palpable fecoliths or constipation-associated fecal incontinence. Those without such impaction were randomized to 2 examiners for DRE to diagnose impaction, in the absence of contraindications. Results: Two hundred and thirty-three children were assessed. One hundred and sixty-nine satisfied Rome III without needing DRE. Forty-eight (28.4%) had impaction detectable without DRE. Among the rest, 28 (30.1%) had impaction by DRE. There was no difference between the frequency of impaction detected by the 2 examiners. Clinical characteristics were similar (P > 0.05) between those with impaction detectable by DRE and those without. Conclusions: DRE does detect cases of impaction not discernible by other means. Such a finding may be comparable between examiners. These children may be identified by other clinical characteristics. The clinical significance of such a finding needs more understanding from the standpoint of therapeutic choices. Address correspondence and reprint requests to Dr Barath Jagadisan, MD, PDCC, Additional Professor, Pediatric gastroenterology, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvantri Nagar, Pondicherry 605006, India (e-mail: barathjag@yahoo.com) Received 17 January, 2018 Accepted 12 March, 2018 Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). Ethical clearance: The study had been approved by the institute ethic committee that complies with international standards and is registered with the government of India (JIP/IEC/2015/23/830). The authors report no conflicts of interest. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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Thiopurine Optimization Through Combination With Allopurinol in Children With Inflammatory Bowel Diseases

Objectives: Thiopurines are commonly used in the maintenance of remission for children with inflammatory bowel diseases (IBD). Variation in drug metabolism may impact hepatotoxicity or therapeutic effect. We aimed to describe our center's experience with thiopurine optimization through the use of reduced thiopurine dosing in combination with allopurinol upon hepatotoxicity, drug metabolite levels and clinical outcomes in children with IBD. Methods: Patients aged 2–21 years with IBD treated with the combination of thiopurines/allopurinol between 2008–2015 were retrospectively reviewed. Patients previously treated with anti-tumor necrosis factor (TNF) therapy were excluded. Demographic data, transaminase levels (AST, ALT), drug metabolites levels (6-TG, 6-MMP), physician global assessment (PGA), and corticosteroid use were recorded at baseline, 6 and 12 months. Results: Fifty-two patients (29 female, 56%) met inclusion criteria. Thirty-two of 52 (62%) remained on the combination for 12 months. In those remaining on the thiopurine/allopurinol combination, median ALT and AST levels were reduced (p 

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Oral bisphosphonate use and lung cancer incidence among postmenopausal women

Abstract

Background

Bisphosphonates are common medications for the treatment of osteoporosis in older populations. Several studies, including the Women's Health Initiative (WHI), have found inverse associations of bisphosphonate use with risk of breast and endometrial cancer, but little is known about its association with other common malignancies. The objective of this study was to evaluate the association of bisphosphonate use on the incidence of lung cancer in the WHI.

Patients and methods

The association between oral bisphosphonate use and lung cancer risk was examined in 151,432 postmenopausal women enrolled into the WHI in 1993-1998. At baseline and during follow-up, participants completed an inventory of regularly used medications including bisphosphonates.

Results

After a mean follow-up of 13.3 years, 2,511 women were diagnosed with incident lung cancer. There was no evidence of a difference in lung cancer incidence between oral bisphosphonate users and never users (adjusted hazard ratio (HR), 0.91; 95% confidence intervals (CI), 0.80-1.04; P = 0.16). However, an inverse association was observed among those who were never smokers (HR = 0.57, 95% CI, 0.39-0.84; P < 0.01).

Conclusion

In this large prospective cohort of postmenopausal women, oral bisphosphonate use was associated with significantly lower lung cancer risk among never smokers, suggesting bisphosphonates may have a protective effect against lung cancer. Additional studies are needed to confirm our findings.

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Application of precision medicine to the treatment of anaphylaxis

Purpose of review Recognize the presentation of anaphylaxis for prompt management and treatment and to provide tools for the diagnosis of the underlying cause(s) and set up a long-term treatment to prevent recurrence of anaphylaxis. Recent findings The recent description of phenotypes provides new insight and understanding into the mechanisms and causes of anaphylaxis through a better understanding of endotypes and biomarkers for broad clinical use. Summary Anaphylaxis is the most severe hypersensitivity reaction and can lead to death. Epinephrine is the first-line treatment of anaphylaxis and it is life-saving. Patients with first-line therapy-induced anaphylaxis are candidates for desensitization to increase their quality of life and life expectancy. Desensitization is a breakthrough novel treatment for patients with anaphylaxis in need of first-line therapy, including chemotherapy, mAbs, aspirin and others. Ultrarush with venom immunotherapy should be considered in patients who present with life-threatening anaphylaxis after Hymenoptera sting with evidence of IgE-mediated mechanisms. Food desensitization is currently being expanded to provide increased safety to adults and children with food-induced anaphylaxis. Correspondence to Mariana Castells, MD, PhD, Division of Rheumatology, Immunology and Allergy, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, The Building for Transformative Medicine, 5th floor, Room 5002N, 60 Fenwood Road, Boston, MA 02115, USA. Tel: +1 617 525 1265; fax: +1 617 525 1310; e-mail: mcastells@bwh.harvard.edu Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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Avenues for research in food allergy prevention: unheeded ideas from the epidemiology

No abstract available

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How to diagnose food allergy

Purpose of review To assess the recent studies that focus on specific immunoglobulin E (sIgE) testing and basophil activation test (BAT) for diagnosing IgE-mediated food allergies. Recent findings The sIgE to allergen extract or component can predict reactivity to food. The cutoff value based on the positive predictive value (PPV) of sIgE can be considered whenever deciding whether oral food challenge (OFC) is required to diagnose hen's egg, cow's milk, wheat, peanut, and cashew nut allergy. However, PPV varies depending on the patients' background, OFC methodology, challenge foods, and assay methodology. Component-resolved diagnostics (CRD) has been used for food allergy diagnosis. Ovomucoid and omega-5 gliadin are good diagnostic markers for heated egg and wheat allergy. More recently, CRD of peanut, tree nuts, and seed have been investigated. Ara h 2 showed the best diagnostic accuracy for peanut allergy; other storage proteins, such as Jug r 1 for walnut, Ana o 3 for cashew nut, Ses i 1 for sesame, and Fag e 3 for buckwheat, are also better markers than allergen extracts. Some studies suggested that BAT has superior specificity than skin prick test and sIgE testing. Summary The sIgE testing and BAT can improve diagnostic accuracy. CRD provides additional information that can help determine whether OFCs should be performed to diagnose food allergy. Correspondence to Sakura Sato, MD, Department of Allergy, Clinical Research Center for Allergology and Rheumatology, Sagamihara National Hospital, 18-1 Sakuradai, Minami-ku, Sagamihara, Kanagawa 252-0392, Japan. Tel: +81 42 742 8311; fax: +81 42 742 5314; e-mail: s-satou@sagamihara-hosp.gr.jp Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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How to predict and improve prognosis of food allergy

Purpose of review The prevalence of food allergy is increasing. More children are being diagnosed with food allergies, and it is taking longer to outgrow them, among those who develop tolerance. The aim of this review is to draw the profile of the persistent food allergic, so that prevention strategies can be developed and active treatment set up. Recent findings Many determinants are involved in food allergy prognosis: ethnicity and sex, type of food, innate immune system, eliciting dose, sensitization status and other biomarkers determination, gut microbiome composition, and the presence of comorbidities. Once identified, a persistent food allergy could be conveyed to active treatments, such as oral immunotherapy or the use of biologics, always taking into account their experimental nature. Summary A better understanding of prognostic factors and phenotypes of food allergy is crucial in decision-making when it comes to food allergy prevention and management. A good classification of the allergic patient allows to determine the degree of exclusion diets and the timing of the reintroduction of avoided food when possible. In the cases of persistent and severe food allergy, many promising interventions are emerging which could improve prognosis and quality of care. Correspondence to Lamia Dahdah, MD, University Department of Pediatrics, Division of Allergy, Pediatric Hospital Bambino Gesù, Piazza di Sant'Onofrio 4, Rome 00100, Vatican City, Italy. Tel: +39 046 6859 4777; fax: +39 046 6859 2020; e-mail: lamiaantanios.dahdah@opbg.net Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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Phenotypes/endotypes-driven treatment in asthma

Purpose of review Target therapy is the necessary step towards personalized medicine. The definition of asthma phenotypes and underlying mechanisms (endotypes) represent a key point in the development of new asthma treatments. Big data analysis, biomarker research and the availability of monoclonal antibodies, targeting specific cytokines is leading to the rapid evolution of knowledge. In this review, we sought to outline many of the recent advances in the field. Recent findings Several attempts have been made to identify asthma phenotypes, sometimes with contrasting results. More success has been obtained concerning the pathogenetic mechanism of specific asthma patterns with the consequent identification of biomarkers and development of effective ad hoc treatment. Summary We are in the middle of an extraordinary revolution of our mode of thinking about and approaching asthma. All the effort in the identification of clusters of patients with different disease clinical patterns, prognosis and response to treatment is closely linked to the identification of endotypes (Th2-low and Th2-high). This approach has allowed the development of the specific treatments (anti IgE, Anti IL5 and IL5R) that are now available and is leading to new ones. Correspondence to Angelica Tiotiu, MD, PhD, Pulmonology Department, CHRU Nancy, EA 3450 DevAH – Development, Adaptation and Disadvantage, Cardiorespiratory Regulations and Motor Control, University of Lorraine, 9 Avenue de la Forêt de Haye, F- 54505 – Vandoeuvre-les-Nancy, France. Tel: +33 3 83 15 43 72; fax: +33 3 83 15 35 41; e-mail: a.tiotiu@chru-nancy.fr Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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How to prevent food allergy during infancy: what has changed since 2013?

Purpose of review The purpose of this review is to summarize recent studies and emerging consensus guidelines regarding food allergy prevention in infants of the past 5 years. Recent findings Prior to 2013, the general consensus regarding prevention of food allergy in infants was to recommend delayed introduction or complete avoidance of commonly allergenic foods, such as milk, egg and peanut. However, in the past 5 years, several landmark studies have been conducted, particularly with peanut. The results of these studies have led to a paradigm shift from recommending delayed introduction to early introduction and frequent feeding of highly allergenic foods such as peanut, with hopes of achieving primary and secondary prevention of food allergy in infants. Summary Recent clinical trials have demonstrated that early introduction and frequent feeding, rather than delayed introduction or complete avoidance, of commonly allergenic foods plays a critical role in preventing food allergy in infants. More studies are required to risk-stratify infants by personal and family atopic history to tailor guidelines for groups with inherently different risks. The universal acceptance of the guidelines and their application outcome are still to be determined. Correspondence to Amal Assa'ad, MD, Professor of Pediatrics, Division of Allergy and Immunology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Mail Location 2000, Cincinnati, OH 45229, USA. Tel: +1 513 636 6771; fax: +1 513 636 5835; e-mail: amal.assaad@cchmc.org Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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Current opinion in allergy and clinical immunology: a change in leadership

No abstract available

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Partial Recovery of Proprioception in Rats with Dorsal Root Injury after Human Olfactory Bulb Cell Transplantation

Journal of Neurotrauma, Ahead of Print.


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Diffusion Tensor Imaging of Scarring, Necrosis, and Cavitation Based on Histopathological Findings in Dogs with Chronic Spinal Cord Injury: Evaluation of Multiple Diffusion Parameters and Their Correlations with Histopathological Findings

Journal of Neurotrauma, Ahead of Print.


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Diffusion Tensor Imaging of Scarring, Necrosis, and Cavitation Based on Histopathological Findings in Dogs with Chronic Spinal Cord Injury: Evaluation of Multiple Diffusion Parameters and Their Correlations with Histopathological Findings

Journal of Neurotrauma, Ahead of Print.


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O-3-32. Relationship between brain activity and mental state during emotional olfactory stimulation

The aim of this study was to investigate the relationship between mental state and brain activity in response to odors in the environment. The mental state of 20 healthy adults was estimated using the Profile of Mood States (POMS) scale. The subjects were placed in a room, which was individually filled with vanillin, Mainichi-Koh (Japanese incense), skatol, and distilled water (control) odors, and their electroencephalograms (EEGs) were recorded while performing an auditory oddball task in the room.

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SP-2. What dose TMS stimulate?

Transcranial magnetic stimulation (TMS) is a technique to stimulate the nervous system non-invasively through the intact scalp and skin. The TMS machine delivers a short pulse of electric current into a TMS coil to generate a quick changing magnetic field surrounding the coil as an example of Ampere's law. TMS stimulates the neuronal circuits with the eddy current induced by the changing magnetic field, not the magnetic field its self, based on Faraday's law. To generate enough power for stimulating human's brain, a TMS machine is generally built to generate a current of around 8000 A for a 2.5–4 Tesla output, although it may vary between machine types and manufacturers.

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P1-2-07. Relationship between Saccadic Eye Movements and Higher Brain Function in the elderly

Saccadic eye movements (SEM) are used as a biomarker for psychiatric disorders. However, the relationship between SEM and higher brain function has not been fully elucidated. Therefore, we examined SEM during the Trail Making Test part A (TMT-A), a neuropsychological test of visual attention and processing speed, and assessed the correlation between the frequency of SEM during TMT-A and neuropsychological test results. The eye movements of 43 elderly subjects (>65 y/o, mean 72.4 y/o), with no history of neuropsychiatric disorders, were measured using eye tracking glasses (SensoMotoric Instruments, Germany).

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Shimazono-Lecture-1. What I have done using TMS techniques

CMCT, CTX-BST CT, BST-Cv CT, CCCT, CECT: Using brainstem and conus medullaris stimulation in combination with the motor cortical and spinal nerve stimulation, we estimated conduction delays at several segments in CSTs.Cerebellar inhibition of the motor cortex (CBI): Purkinje cell activation by TMS induced a transient motor cortical inhibition. Mechanisms underlying ataxia was studied with this method.Shor interval intracortical inhibition (SICI): We showed the dependency of SICI on the motor cortical TMS pulse directions, which gave us a hint about the mechanisms of SICI.

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P2-3-8. Change of cross frequency coupling by symptom provocation in Obsessive Compulsive Disorder (OCD) based on sLORETA

We investigated the changes in directional cross frequency interactions between theta and alpha oscillations, across six cortical regions, induced by a symptom provocation procedure, in patients with OCD, and in normal controls. Nine OCD outpatients and nine controls participated in this study. Eyes closed EEG was recorded before and under the instruction to imagine that the towel placed on their hands is contaminated (symptom provocation, SP). Cortical electric neuronal activity were calculated with sLORETA at medial-prefrontal, precuneus, inferior-parietal, and dorsolateral-prefrontal cortices.

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A-19. Visual evoked potentials: Basic technology and clinical applications

Visual information is processed simultaneously via multiple parallel channels and each channel constitutes a set of sequential processes. The visual evoked potentials (VEPs) are the evoked electrophysiological potential that can be extracted, using signal averaging, from the electroencephalographic activity recorded at the scalp. VEPs can provide important diagnostic information regarding the functional integrity of the visual pathways and the visual cortex. Commonly used visual stimuli are flashing lights or pattern reversal (checkerboards or gratings) on the TV monitor or video screen.

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S11-3. Three points to remember when you conduct research using event-related potentials

Event-related potentials (ERP) are a part of the electroencephalogram (EEG) that is time-locked to a certain event, such as stimulus presentation or movement onset. As recent advances in electronics bring inexpensive and easy-to-use EEG amplifiers to the market, more and more people are interested in also recording ERPs. However, easy-to-use does not mean easy-to-succeed. In this talk, I will make three practical recommendations for conducting ERP research. First, make sure that the timings of events are precisely registered with the EEG data.

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B-2. Clinical application of patterned DC stimulation

In addition to conventional surgical and pharmacological methods, electromagnetic interventions to the human brain have been developed for testing and treating neuropsychiatric disorders. Considering that the central principle of brain coding is based on neural firings and cell membrane potentials, that is electrical phenomena, electromagnetic intervention to the brain can be useful and effective for modulating brain functions.Among various electromagnetic methods, the most advanced one is the transcranial magnetic stimulation (TMS) which was put into practical use in 1985.

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B-3. Electroencephalography and seizures learned from ictal recording

Experience on ictal video electroencephalography (EEG) recording enriches knowledge on EEG and seizures. Following five fundamentals are important for correct interpretation of EEG during focal epileptic seizures.Epileptic seizures never happen without EEG changes.Epileptic seizure propagates.EEG changes are not necessarily visible.EEG changes occur as a remote effect.EEG seizure explains clinical symptoms.Focal epileptic seizure starts with several patterns such as low voltage fast activity or repetitive spikes, followed by ictal rhythm that evolves spatially from focal to diffuse area, and temporally from low amplitude fast to high amplitude slow waves.

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P3-4-8. Utility of diaphragm ultrasound and phrenic nerve conduction in myopathy

Respiratory dysfunction in myopathy such as muscular dystrophy is extremely important because it is directly related to life prognosis, and appropriate evaluation and management is necessary. For objective assessment of respiratory muscle strength in myopathy, we study the usefulness of diaphragm ultrasonography and phrenic nerve study. Nineteen myopathy patients who were confirmed by genetic testing or muscle biopsy during our hospital visit were included. In both cases, respiratory function test, phrenic nerve conduction test, diaphragm ultrasonic examination were performed.

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B-7. Electrodiagnostic criteria for ALD: Current state and perspectives

The diagnosis of amyotrophic lateral sclerosis (ALS) requires (1) evidence for extensive upper and lower motor neuron dysfunction, (2) progressive course, and (3) exclusion of other causes of motor neuronal loss. So far, a number of diagnostic criteria for ALS have been proposed, such as El Escorial (1990), revised El Escorial (1998), Awaji (2008), and updated Awaji (2015). According to the frequent revision/proposal criteria, electrodiagnostic criteria for ALS are somewhat confusing. In these criteria, the body motor system are divided into 4 regions; cranial, cervical, thoracic, and lumbosacral, and evidence for upper and lower motor neuron signs in the two or more regions are required for the diagnosis of "probable" ALS.

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P1-5-02. Evaluation of facial muscle elasticity with real-time tissue elastography – Examination in cases with synkinesis following peripheral facial nerve paresis

The study was performed to clarify the relationship between facial muscle elasticity measured by real-time tissue elastography (RTE) and the clinical phenotypes of peripheral facial nerve paresis (PFP). We examined the orbicularis oculi muscles of nine PFP patients with synkinesis by RTE. The strain ratio (SR) of the muscle to an acoustic coupler was calculated as an index of muscle elasticity. We also recorded and integrated electromyograms of left and right orbicularis oculi muscles, respectively.

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Cancers, Vol. 10, Pages 97: Nanopulse Stimulation (NPS) Induces Tumor Ablation and Immunity in Orthotopic 4T1 Mouse Breast Cancer: A Review

Cancers, Vol. 10, Pages 97: Nanopulse Stimulation (NPS) Induces Tumor Ablation and Immunity in Orthotopic 4T1 Mouse Breast Cancer: A Review

Cancers doi: 10.3390/cancers10040097

Authors: Stephen Beebe Brittany Lassiter Siqi Guo

Nanopulse Stimulation (NPS) eliminates mouse and rat tumor types in several different animal models. NPS induces protective, vaccine-like effects after ablation of orthotopic rat N1-S1 hepatocellular carcinoma. Here we review some general concepts of NPS in the context of studies with mouse metastatic 4T1 mammary cancer showing that the postablation, vaccine-like effect is initiated by dynamic, multilayered immune mechanisms. NPS eliminates primary 4T1 tumors by inducing immunogenic, caspase-independent programmed cell death (PCD). With lower electric fields, like those peripheral to the primary treatment zone, NPS can activate dendritic cells (DCs). The activation of DCs by dead/dying cells leads to increases in memory effector and central memory T-lymphocytes in the blood and spleen. NPS also eliminates immunosuppressive cells in the tumor microenvironment and blood. Finally, NPS treatment of 4T1 breast cancer exhibits an abscopal effect and largely prevents spontaneous metastases to distant organs. NPS with fast rise–fall times and pulse durations near the plasma membrane charging time constant, which exhibits transient, high-frequency components (1/time = Hz), induce responses from mitochondria, endoplasmic reticulum, and nucleus. Such effects may be responsible for release of danger-associated molecular patterns, including ATP, calreticulin, and high mobility group box 1 (HMBG1) from 4T1-Luc cells to induce immunogenic cell death (ICD). This likely leads to immunity and the vaccine-like response. In this way, NPS acts as a unique onco-immunotherapy providing distinct therapeutic advantages showing possible clinical utility for breast cancers as well as for other malignancies.

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The impact of palliative care training for oncologists and integrative palliative service in a public-funded hospital cluster—a retrospective cohort study

Abstract

Purpose

Oncological care of advanced cancer patients was provided by multiple departments in Hong Kong. One of these departments, the clinical oncology department (COD), introduced systematic palliative care training for its oncologists since 2002. The COD was recognized as a European Society for Medical Oncology (ESMO) Designated Centre of Integrated Oncology and Palliative Care since 2009. This retrospective cohort study aims to review the impact of integrative training and service on palliative care coverage and outcome.

Methods

Clinical information, palliative service provision, and end-of-life outcomes of patients who passed away from lung, colorectal, liver, stomach, or breast cancer in the Hong Kong West public hospital network during July 2015 to December 2015 were collected.

Results

A total of 307 patients were analyzed. Around half (49.2%) were attended primarily by COD, and 68.9% received palliative service. There are significantly fewer patients referred to palliative care from other departments (p < 0.001), with only 19.9% of this patient group receiving palliative referral. COD patients had longer palliative coverage before death (median 65 days versus 24 days, p < 0.001), higher chance of receiving end-of-life care at hospice units (36.4 versus 21.2%, p = 0.003), lower ICU admission (0.66 versus 5.1%, p = 0.02), and higher percentage of receiving strong opioid in the last 30 days of life (51.0 versus 28.9%, p < 0.001) compared to other departments. In multivariable analysis, COD being the primary care team (odds ratio 12.2, p < 0.001) was associated with higher palliative care coverage.

Conclusion

The study results suggested that systematic palliative care training of oncologists and integrative palliative service model was associated with higher palliative service coverage and improved palliative care outcomes.

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Evaluation of quality of life in caregivers who are providing home care to cancer patients

Abstract

Purpose

The author aimed to evaluate the quality of life and the factors affecting the caregivers of cancer patients receiving home care.

Methods

This cross-sectional descriptive study was performed in 48 cancer patients who were served from home care unit and 48 caregivers between 01 and 28 February 2014. Patients' functional status was evaluated with Katz Index for Activities of Daily Living and the Lawton Scale for Instrumental Activities of Daily Living. The levels of quality of life of caregivers of patients with cancer were determined with Caregiver Quality of Life Index-Cancer (CQOLC).

Results

The mean age of forty-eight patients was 69.79 ± 16.09 years; 62.5% of them were female. The mean duration of home care was 5.99 ± 5.26 years; 25% of patients were fully dependent on the bed. 83.3% of caregivers were female, mean age of caregivers was 50.75 ± 14.89 years, and 77.1% of them were family members. The mean CQOLC score was 74.43 ± 24.45. The highest score was detected in the financial distress and the lowest score was detected in the positive adaptation. The quality of life is increasing as the length of care is reduced and income status increased.

Conclusion

The quality of life of caregivers is very low. Each characteristic of the caregiver will affect the care he/she gives. From this point of view, it is important to consider the characteristics of caregivers in improving the care given to cancer patients. In this respect, there is a need to support caregivers both materially and spiritually.

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The impact of remission status on patients’ experiences with acute myeloid leukemia (AML): an exploratory analysis of longitudinal patient-reported outcomes data

Abstract

Purpose

Shared decision-making in acute myeloid leukemia (AML) requires understanding patients' longitudinal experiences of illness, but little is known about the impact of remission status on patient-reported outcomes (PROs). We aimed to explore the association between remission status and PROs 6–12 months following induction chemotherapy.

Methods

Forty-two patients completed three validated instruments characterizing symptom burden (Patient Care Monitor v2.0), distress (NCCN Distress Thermometer), and QOL (FACT-Leu), as part of a longitudinal observational study. We used regression models to explore the relationship between remission status and PROs, and explore differences by initial disease type (de novo versus secondary/relapsed AML).

Results

Those with secondary or relapsed AML at study onset had marked impairments in all measures compared to de novo AML patients. After 6 months, their mean distress score was 4.8 (> 4.0 warrants intervention), they reported a mean of 14.1 moderate/severe symptoms and had a mean QOL score of 113.6, compared to 1.0, 1.7, and 155.2, respectively, for those with de novo AML (p < .0001). Similarly, patients in relapse had a mean distress score of 5.3, a mean of 12.8 moderate/severe symptoms, and a mean QOL score of 113.4, compared to 1.8, 5.7, and 143.8, respectively, among those in remission (p < .005). These patterns persisted after adjusting for baseline differences (p < .0001).

Conclusion

Remission is associated with markedly better patient well-being in AML. Patients with secondary or relapsed AML face more severe symptom burden, distress, and QOL issues after induction. Interventions are needed to improve AML patients' experiences of illness.

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Efficacy and safety of reinfusion of concentrated ascitic fluid for malignant ascites: a concept-proof study

Abstract

Purpose

Malignant ascites is one of the symptoms causing discomfort in advanced cancer patients. Cell-free and concentrated ascites reinfusion therapy (CART) is one treatment modality, but controlled trials are limited. The primary aim of this study was to explore the efficacy and safety of CART, as well as their predictors, to obtain data for planning a further controlled trial.

Methods

This was a single center retrospective cohort study in patients with refractory malignant ascites. Consecutive adult patients who underwent CART were enrolled. The primary endpoints were the time to next paracentesis and seven patient-reported symptoms (e.g., abdominal pain and distension). The secondary endpoints were adverse events, laboratory findings, and physical findings.

Results

A total of 104 CART procedures for 51 patients were analyzed. The median time to next paracentesis was 27 days (95% CI, 21–35). Intensities of all seven symptoms were significantly improved after CART (p < 0.0001 for all symptoms). Grade 3 hypotension occurred during one procedure, and mild fever occurred in 5%. Total protein, albumin, and estimated glomerular filtration rate were significantly increased. Hemorrhagic ascites, ascites white blood cell count, serum total protein, and lymphocyte percentages were the independent predictors of the time to next paracentesis.

Conclusion

The effects of reinfusion of concentrated ascitic fluid may be maintained for 1 month, being potentially longer than that of total paracentesis alone. This study had no comparison groups and examined the short-term effect. A randomized controlled study to compare the long-term effects of total paracentesis alone vs. CART is necessary.

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Levofloxacin for febrile neutropenia prophylaxis in acute myeloid leukemia patients associated with reduction in hospital admissions

Abstract

Purpose

The purpose of this study is to evaluate the efficacy and safety of prophylactic oral levofloxacin in acute myeloid leukemia (AML) patients after receiving consolidation chemotherapy to prevent febrile neutropenia.

Methods

We conducted a retrospective chart review of 50 AML patients who were prescribed levofloxacin and 50 AML patients who were not prescribed levofloxacin post-consolidation chemotherapy between June 2006 and August 2013 at a tertiary academic medical center. The primary outcome of this study was to evaluate the effectiveness of levofloxacin in preventing hospital readmission due to febrile neutropenia. Secondary outcomes evaluated the safety of this therapy, including the rate of Clostridium difficile-associated diarrhea (CDAD) within 30 days from discharge of receiving consolidation chemotherapy and rate of fluoroquinolone resistance in positive bacterial cultures.

Results

Hospital readmission due to febrile neutropenia after the first consolidation cycle occurred in 42% of patients prescribed levofloxacin, as compared to 72% that were not prescribed levofloxacin (p = 0.002). This was also significantly reduced when levofloxacin was prescribed after all consolidation cycles (51.4 vs. 67%, p = 0.023). CDAD did not occur in any patient prescribed levofloxacin after the first cycle, compared to one case in those not prescribed levofloxacin. Evaluation of the impact on fluoroquinolone resistance was limited due to a paucity of fluoroquinolone susceptibilities reported.

Conclusions

Prescribing oral levofloxacin post-consolidation chemotherapy in AML patients is associated with a reduction in febrile neutropenia. Further research is required to identify the impact on fluoroquinolone resistance and risk of CDAD.

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Demographic, clinical, lifestyle-related, and social-cognitive correlates of physical activity in head and neck cancer survivors

Abstract

Purpose

The purpose of the study is to identify demographic, clinical, lifestyle-related, and social-cognitive correlates of physical activity (PA) intention and behavior in head and neck cancer (HNC) survivors using the theory of planned behavior (TPB).

Methods

Data from two cross-sectional studies on correlates of PA in HNC survivors were pooled. Both studies used self-reports to assess PA and social-cognitive correlates. Potential correlates were collected via self-report or medical records. Univariable and multivariable multilevel linear mixed-effects models were built to identify correlates of PA intention and PA behavior (Z scores). Structural equation model analyses were conducted to study the full TPB model in one analysis, taking into account relevant covariates.

Results

In total, 416 HNC survivors were surveyed. Their mean (SD) age was 66.6 (9.4) years; 64% were men, and 78% were diagnosed with laryngeal cancer. The structural equation model showed that PA intention was significantly higher in HNC survivors with a history of exercising, who had a more positive attitude, subjective norm, and perceived behavioral control. Patients with higher PA intention, higher PBC, a lower age, and without unintentional weight loss or comorbidities had higher PA behavior. The model explained 22.9% of the variance in PA intention and 16.1% of the variance in PA behavior.

Conclusions

Despite significant pathways of the TPB model, the large proportion variance in PA intention and behavior remaining unexplained suggests the need for better PA behavior (change) models to guide the development of PA promotion programs, particularly for the elderly. Such programs should be tailored to comorbidities and nutritional status.

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Tunneled pleural catheter use for pleural palliation does not increase infection rate in patients with treatment-related immunosuppression

Abstract

Purpose

Concerns for infections resulting from antineoplastic therapy-associated immunosuppression may deter referral for symptom palliation with a tunneled pleural catheter (TPC) in patients with malignant/para-malignant pleural effusions (MPE/PMPE). While rates of TPC-related infections range from 1 to 21%, those in patients receiving antineoplastic therapy with correlation to immune status has not been established. We aimed to assess TPC-related infection rates in patients on antineoplastic therapy, determine relation to immune system competency, and assess impact on the patient.

Methods

Patients with a MPE/PMPE undergoing TPC management associated with antineoplastic therapy, from 2008 to 2016, were reviewed and categorized into those with an immunocompromised versus immunocompetent immune status.

Results

Of the 150 patients, a TPC-related infection developed in 13 (9%): pleural space in 11 (7%) and superficial in 2 (1%). Ninety-three percent (139/150) were identified to be immunocompromised during their antineoplastic therapy. No difference in TPC-related infections was seen in patients with an immunocompromised (9%, 12/139) versus immunocompetent status (9%, 1/11); p = 0.614. The presence of a catheter-related infection did not negatively impact overall survival over a median follow-up of 144 days (interquartile range 41–341); p = 0.740.

Conclusions

These results suggest that antineoplastic therapy may not significantly increase the overall risk of TPC-related infections, as the rate remains low and comparable to rates in patients not undergoing antineoplastic therapy. Regardless of immune status, the presence of a catheter-related infection did not negatively impact overall survival. These results should reassure clinicians that the need to initiate antineoplastic therapy should not delay definitive pleural palliation with a TPC.

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Comparison of the MASCC and CISNE scores for identifying low-risk neutropenic fever patients: analysis of data from three emergency departments of cancer centers in three continents

Abstract

Purpose

Patients with febrile neutropenia are a heterogeneous group with a minority developing serious medical complications. Outpatient management of low-risk febrile neutropenia has been shown to be safe and cost-effective. Scoring systems, such as the Multinational Association for Supportive Care in Cancer (MASCC) score and Clinical Index of Stable Febrile Neutropenia (CISNE), have been developed and validated to identify low-risk patients. We aimed to compare the performance of these two scores in identifying low-risk febrile neutropenic patients.

Methods

We performed a pooled analysis of patients presenting with febrile neutropenia to three tertiary cancer emergency centers in the USA, UK, and South Korea in 2015. The primary outcome measures were the occurrence of serious complications. Admission to an intensive care unit (ICU) and 30-day mortality were secondary outcomes. The predictive performance of each score was analyzed.

Results

Five hundred seventy-one patients presented with febrile neutropenia. With MASCC risk index, 508 (89.1%) were classified as low-risk febrile neutropenia, compared to 60 (10.5%) with CISNE classification. Overall, the MASCC score had a greater discriminatory power in the detection of low-risk patients than the CISNE score (AUC 0.772, 95% CI 0.726–0.819 vs. 0.681, 95% CI 0.626–0.737, p = 0.0024).

Conclusion

Both MASCC and CISNE scores have reasonable discriminatory value in predicting patients with low-risk febrile neutropenia. Risk scores should be used in conjunction with clinical judgment for the identification of patients suitable for outpatient management of neutropenic fever. Developing more accurate scores, validated in prospective settings, will be useful in facilitating more patients being managed in an outpatient setting.

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Extended nursing for the recovery of urinary functions and quality of life after robot-assisted laparoscopic radical prostatectomy: a randomized controlled trial

Abstract

Purpose

The purpose of this work is to explore the effects of continuing nursing care intervention on postoperative urinary control and quality of life among patients with prostate cancer.

Methods

This was a single-center, parallel, and randomized controlled trial that was carried out at the Department of Urology, the First Affiliated Hospital of Anhui Medical University, China. The participants underwent robot-assisted laparoscopic radical prostatectomy (RARP) between October 2014 and April 2016. The patients were randomized to the experimental and control groups (n=37/group). Patients in the control group received routine nursing care, while patients in the experimental group received continuing nursing care. During the 6-month follow-up, each patient was invited at the hospital discharge and at 1, 3, and 6 months to fill the ICI-Q-SF and SF-36 questionnaires.

Results

The scores of urinary incontinence were improved in the intervention group compared with controls at 3 and 6 months after discharge (both P < 0.01). The scores of quality of life in the experimental group were significantly higher than control group at 1, 3, and 6 months (all P < 0.01). Adverse events were mild or moderate in intensity and were resolved in all patients. All adverse events were related to RARP.

Conclusions

Continuing nursing care intervention had significant beneficial effects on urinary functions and quality of life in patients with prostate cancer after RARP. This approach warrants to be promoted in the clinical setting.

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Photodynamic therapy for periungual pyogenic granuloma-like during chemotherapy: our preliminary results

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Phase II study of palonosetron, aprepitant and dexamethasone to prevent nausea and vomiting induced by multiple-day emetogenic chemotherapy

Abstract

Purpose

This study aimed to determine the antiemetic efficacy and safety of palonosetron, aprepitant and dexamethasone in patients with testicular germ cell tumours (TGCTs) receiving 5-day cisplatin-based combination chemotherapy.

Methods

In this open-label, single-arm, single-centre study, the antiemetic therapy consisted of palonosetron 0.75 mg on day 1, aprepitant 125 mg on day 1 and 80 mg on days 2–7 and dexamethasone 6.6 mg on days 1–7. The primary endpoint was complete response (CR; no vomiting/retching or rescue medication) in the overall period (0–240 h), and secondary endpoints included complete protection (CP; defined as CR and no more than mild nausea) and total control (TC; defined as CR and no nausea). The incidence and severity of nausea were assessed on the basis of the Common Terminology Criteria for Adverse Events v4.0 and a subjective rating scale completed by patients.

Results

Twenty-five patients were enrolled and evaluated for safety, and 24 patients were evaluated for efficacy. CR was achieved in 62.5% of patients (95% confidence interval [CI] = 40.6–81.2, p = 0.043) in the overall period. CP and TC were achieved in 62.5% (95% CI = 40.6–81.2) and 25.0% of patients (95% CI = 9.8–46.7), respectively, in the overall period. The primary adverse drug reaction was hiccups (48.0%). The events were expected, and none was grade 3 or 4.

Conclusions

The examined combination antiemetic therapy was effective and well-tolerated in patients with TGCTs receiving 5-day cisplatin-based combination chemotherapy.

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Burden of oral mucositis in stem cell transplant patients—the patients’ perspective

Abstract

Purpose

Purpose of this study was to determine the impact of Oral Mucositis (OM) on health-related quality of life (HRQoL) and quality of life associated symptoms and functions in patients undergoing hematopoietic stem cell transplantation (HSCT).

Methods

Prospective, non-interventional single-center observational study at a German tertiary teaching hospital. Inpatient allogenic and autologous stem cell transplant patients ≥18-year-old with high-dose chemotherapy. OM was assessed with the WHO Oral Toxicity Scale, pain according to the Numeric Rating Scale (NRS) and the performance status using the ECOG Score. QOL was captured with the EORTC QLQ-C30 and the QLQ-OH15 questionnaires.

Results

Forty-five stem cell transplant patients (20 autologous, 25 allogenic) were enrolled between August 2016 and February 2017. Twenty-six (58%, 95% CI: 42% - 72%) patients developed OM (10 grade I, 4 grade II, 8 grade III, 4 grade IV). OM affected patients suffered more from pain, sore mouth and sensitive mouth. A lower physical functioning (34.5 vs 7.5, p = 0.003) and a lower oral health-related quality of life (24.3 vs 7.7, p = 0.006) was found in patients with OM development. There was found a positive correlation between the grade of OM and the NRS-value (r = 0.93, 95% CI: 0.89–0.96, p < 0.001).

Conclusion

OM is associated with health-related quality of life and quality of life associated functions and symptoms. More research should be performed to find ways to prevent OM and to stabilize patients' quality of life during HSCT.

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Fractures frequently occur in older cancer patients: the MD Anderson Cancer Center experience

Abstract

Purpose and Introduction

A growing number of cancer patients are older adults aged 65 years and older. Patients with cancer are at increased risk for developing osteoporosis, falls, and fractures. We sought to identify the incidence of fractures in older adults who underwent cancer care between January 2013 and December 2015.

Methods

A comprehensive geriatric assessment was performed, and bone densitometry was measured at baseline, with a 2-year follow-up.

Results

In this study, among 304 patients with gastrointestinal, urologic, breast, lung, and gynecologic cancers we evaluated, and who completed the bone density testing (n = 199), 80% had osteoporosis or low bone mass (osteopenia). There was a higher prevalence of osteoporosis in cancer patients (40 vs. 16%, p = 0.05) than in population studies. Vitamin D insufficiency (< 30 ng/ml) was identified in 49% of tested cases (n = 245). Risk factors for low bone mass or osteoporosis were advanced age (p = 0.05), malnutrition (p = 0.04), and frailty (p = 0.01). Over the following 2 years (median follow-up 18 months), there was an incidence of fractures of 110 per 1000 person-years, or 2.8 times higher than reported in individuals without cancer. Risk factors for fractures included advanced age (70–79 vs. 60–69 years, p = 0.05) and frailty (p = 0.03).

Conclusion

Most older cancer patients studied have osteoporosis or low bone mass, resulting in an almost 3-fold increase in fracture risk as compared to epidemiologic studies. Bone health issues are commonly seen in older cancer patients, we recommend universal bone density testing. The initiation of antiresorptive treatment when findings are of osteopenia or osteoporosis will reduce the risk of fractures.

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Antimicrobial therapy in palliative care: an overview

Abstract

In the advanced stages of illness, patients often face challenging decisions regarding their treatment and overall medical care. Terminal ill patients are commonly affected by infections. However, in palliative care, the use of antimicrobials can be an ethical dilemma. Deciding whether to treat, withhold, or withdraw the antimicrobial treatment for an infection can be difficult. Antimicrobial administration can lead to adverse outcomes but the two main benefits, longer survival and symptom relief, are the main reasons why physicians prescribe antimicrobial when treating terminally ill patients. For the patient who has an irreversible advanced heart or lung disease, or an advanced dementia, or a metastatic cancer, it is easier the decision of withholding mechanical ventilation, tube feeding, and dialysis than antibiotherapy. To characterize infections, agents, and their treatments in palliative care, we conducted a review of the literature. We also included some tips to help health professionals to guide their clinical approach.

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Physical activity among cancer survivors—what is their perception and experience?

Abstract

Purpose

Physical activity (PA) plays an important role relating to cancer. The aim of this study was to investigate the attitude to and experience with the subject of PA in cancer in a large group of tumour patients.

Methods

A standardised questionnaire was carried out and distributed to patients online and in printed form.

Results

Nine hundred five patients answered the questionnaire. Most tumour patients (60%) received information about PA after their cancer therapy. The Internet was often rated to be inadequate as a source of information. One in two tumour patients were recommended PA by a therapist. During the acute phase, the majority (57% of the 776) did not receive a sport-therapeutic exercise programme. Two thirds (68%) of the 898 patients indicated regularly engaging in PA at least 3 or 5 days per week. In most cases (30% of the 787), 2 to 4 h per week were dedicated to PA. In addition to a desire to increase well-being, enjoyment played a large role. Weakness and lack of willpower are among the most common barriers. Most tumour patients confirmed that PA improved their body awareness (58%) or gave them the feeling that they could do something to better cope with the disease (61%) or feel better (68%).

Conclusion

On the one hand, the information requirements of tumour patients with respect to PA have not been adequately taken into account by practitioners. On the other hand, there are still subjective inhibitions on the part of the patients, which keep them from engaging in PA.

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Incongruence in treatment decision making is associated with lower health-related quality of life among prostate cancer survivors: results from the PiCTure study

Abstract

Purpose

We investigated associations between treatment decision making (TDM) and global health-related-quality-of-life (gHRQoL) among prostate cancer (PCa) survivors.

Methods

Postal questionnaires were sent to 6559 PCa survivors 2–18 years post-diagnosis, identified through population-based cancer registries in Ireland. The Control Preference Scale was used to investigate respondents' 'actual' and 'preferred' role in TDM. The TDM experience was considered 'congruent' when actual and preferred roles matched and 'incongruent' otherwise. The EORTC QLQ-C30 was used to measure gHRQoL. Multivariate linear regression was employed to investigate associations between (i) actual role in TDM, (ii) congruence in TDM, and gHRQoL.

Results

The response rate was 54% (n = 3348). The percentages of men whose actual role in TDM was active, shared or passive were 36, 33 and 31%, respectively. Congruence between actual and preferred roles in TDM was 58%. Actual role in TDM was not associated with gHRQoL. In multivariate analysis, after adjusting for socio-demographic and clinical factors, survivors whose TDM experience was incongruent had significantly lower gHRQoL than those who had a congruent experience (− 2.25 95%CI − 4.09, − 0.42; p = 0.008). This effect was most pronounced among survivors who had more involvement in the TDM than they preferred (− 2.69 95%CI − 4.74, − 0.63; p = 0.010).

Conclusions

Less than 6 in 10 PCa survivors experienced congruence between their actual and preferred roles in TDM. Having an incongruent TDM experience was associated with lower gHRQoL among survivors. These findings suggest that involving patients in TDM to the degree to which they want to be involved may help improve PCa survivors' gHRQoL.

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Financial toxicity and symptom burden: what is the big deal?

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Effect of Epley, Semont Maneuvers and Brandt–Daroff Exercise on Quality of Life in Patients with Posterior Semicircular Canal Benign Paroxysmal Positional Vertigo (PSCBPPV)

Abstract

Benign Paroxysmal Positional Vertigo (BPPV) is the most common cause in patients with vertigo (Pereira et al. in Braz J Otorhinolaryngol (Impr) 76(6):704–708, 2010; Dix and Hallpike in Ann Otol Rhinol Laryngol 6:987–1016, 1952). Posterior Semicircular Canal BPPV (PSCBPPV) has more incidence and prevalence then Lateral, and Anterior Semicircular Canal BPPV (Alghwiri et al. in Arch Phys Med Rehabil 93:1822–1831, 2012). Quality of life (QoL) is significantly impaired by vertigo (Sargent et al. in Otol Neurotol 22:205–209, 2001; World Health Organization in International classification of functioning, disability and health, World Health Organization, Geneva, 2001). To study the effect and compare Epley, Semont maneuvers and Brandt–Daroff Exercise on QoL in patients with PSCBPPV. 90 individuals with unilateral PSCBPPV were selected based on positive Dix–Hallpike test. 3 groups Epley, Semont, and Brandt–Daroff were formed and 30 individuals were selected in each group randomly. Dix–Hallpike test and Vestibular Activities and Participation (VAP) Scale based on International Classification of Functioning were administered before and after Epley, Semont maneuvers, and Brandt–Daroff Exercise to fulfill the aim. VAP Scale results revealed significant difference between pre and post treatment score in all 3 groups, suggestive of positive effect on QoL in patients with PSCBPPV. Improvements in VAP Score between 3 groups were compared and significant difference was observed. Dix–Hallpike test results revealed that 90, 73.33, and 50% patients improved in Epely, Semont, and Brandt–Daroff group respectively. Epely maneuver found to be the best choice and then Semont and Brandt–Daroff should be least preferred in treatment of patients with PSCBPPV.

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Accelerating drug development in pediatric cancer: a novel Phase I study design of venetoclax in relapsed/refractory malignancies

Future Oncology, Ahead of Print.


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Accelerating drug development in pediatric cancer: a novel Phase I study design of venetoclax in relapsed/refractory malignancies

Future Oncology, Ahead of Print.


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Use of the Ion AmpliSeq Cancer Hotspot Panel in clinical molecular pathology laboratories for analysis of solid tumours: with emphasis on validation with relevant single molecular pathology tests and the Oncomine Focus Assay

Publication date: Available online 29 March 2018
Source:Pathology - Research and Practice
Author(s): Ahwon Lee, Sung-Hak Lee, Chan Kwon Jung, Gyungsin Park, Kyo Young Lee, Hyun Joo Choi, Ki Ouk Min, Tae Jung Kim, Eun Jung Lee, Youn Soo Lee
Targeted application of next-generation sequencing (NGS) technology allows detection of specific mutations that can provide treatment opportunities for cancer patients. We evaluated the applicability of the Ion AmpliSeq Cancer Hotspot Panel V2 (CHV2) using formalin-fixed, paraffin-embedded (FFPE) tissue of clinical specimens.Thirty-five FFPE tumour samples with known mutational status were collected from four different hospitals and sequenced with CHV2 using an Ion Chef System and Ion S5 XL system. Out of 35 cases, seven were sequenced with Oncomine focus Assay Panel for comparison. For the limit of detection test, we used an FFPE reference standard, a cell line that included an engineered 50% EGFR T790 M in an RKO cell line background. Coverage analysis results including number of mapped reads, on target percent, mean depth, and uniformity were not different according to hospitals. Sensitivity for mutation detection down to 3% was demonstrated. NGS results showed 100% concordance with the results from single molecular pathology tests Assay in 30 cases with 24 known positive mutations and 14 known negative mutations, and another NGS panel of the Oncomine focus in seven cases.The CHV2 NGS test for solid tumours using Ion chef system and S5 XL system in clinical molecular pathology laboratories for analysis of solid tumours could be routinely used and could replace some single molecular pathology tests after a stringent and thorough validation process.



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Effect of Epley, Semont Maneuvers and Brandt–Daroff Exercise on Quality of Life in Patients with Posterior Semicircular Canal Benign Paroxysmal Positional Vertigo (PSCBPPV)

Abstract

Benign Paroxysmal Positional Vertigo (BPPV) is the most common cause in patients with vertigo (Pereira et al. in Braz J Otorhinolaryngol (Impr) 76(6):704–708, 2010; Dix and Hallpike in Ann Otol Rhinol Laryngol 6:987–1016, 1952). Posterior Semicircular Canal BPPV (PSCBPPV) has more incidence and prevalence then Lateral, and Anterior Semicircular Canal BPPV (Alghwiri et al. in Arch Phys Med Rehabil 93:1822–1831, 2012). Quality of life (QoL) is significantly impaired by vertigo (Sargent et al. in Otol Neurotol 22:205–209, 2001; World Health Organization in International classification of functioning, disability and health, World Health Organization, Geneva, 2001). To study the effect and compare Epley, Semont maneuvers and Brandt–Daroff Exercise on QoL in patients with PSCBPPV. 90 individuals with unilateral PSCBPPV were selected based on positive Dix–Hallpike test. 3 groups Epley, Semont, and Brandt–Daroff were formed and 30 individuals were selected in each group randomly. Dix–Hallpike test and Vestibular Activities and Participation (VAP) Scale based on International Classification of Functioning were administered before and after Epley, Semont maneuvers, and Brandt–Daroff Exercise to fulfill the aim. VAP Scale results revealed significant difference between pre and post treatment score in all 3 groups, suggestive of positive effect on QoL in patients with PSCBPPV. Improvements in VAP Score between 3 groups were compared and significant difference was observed. Dix–Hallpike test results revealed that 90, 73.33, and 50% patients improved in Epely, Semont, and Brandt–Daroff group respectively. Epely maneuver found to be the best choice and then Semont and Brandt–Daroff should be least preferred in treatment of patients with PSCBPPV.

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Cervical myelopathy after high-voltage electrical burn of the head: Report of an unusual case

Shri Ram Sharma, Masaraf Hussain, Hibo Hibong

Annals of Indian Academy of Neurology 2018 21(1):76-79

High-voltage electrical injuries are uncommonly reported and may predispose to both immediate and delayed neurologic complications. We present a case of 27-year-old male who experienced a high-voltage electrical burn of the head resulting in quadriparesis. High-voltage electrocution injuries are a serious problem with potential for immediate, delayed, and long-term neurologic sequelae. The existing literature regarding effective treatment of neurologic complications is limited. Multidisciplinary management and long-term follow up are required.

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Brain at risk

Subhash Kaul

Annals of Indian Academy of Neurology 2018 21(1):2-2



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As I Sign in…

Vinay Goyal

Annals of Indian Academy of Neurology 2018 21(1):1-1



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Recent advances in antisense oligonucleotide therapy in genetic neuromuscular diseases

Ashok Verma

Annals of Indian Academy of Neurology 2018 21(1):3-8

Genetic neuromuscular diseases are caused by defective expression of nuclear or mitochondrial genes. Mutant genes may reduce expression of wild-type proteins, and strategies to activate expression of the wild-type proteins might provide therapeutic benefits. Also, a toxic mutant protein may cause cell death, and strategies that reduce mutant gene expression may provide therapeutic benefit. Synthetic antisense oligonucleotide (ASO) can recognize cellular RNA and control gene expression. In recent years, advances in ASO chemistry, creation of designer ASO molecules to enhance their safety and target delivery, and scientific controlled clinical trials to ascertain their therapeutic safety and efficacy have led to an era of plausible application of ASO technology to treat currently incurable neuromuscular diseases. Over the past 1 year, for the first time, the United States Food and Drug Administration has approved two ASO therapies in genetic neuromuscular diseases. This overview summarizes the recent advances in ASO technology, evolution and use of synthetic ASOs as a therapeutic platform, and the mechanism of ASO action by exon-skipping in Duchenne muscular dystrophy and exon-inclusion in spinal muscular atrophy, with comments on their advantages and limitations.

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Chronic lymphocytic leukemia involvement of central nervous system: Clinical diversity, diagnostic algorithm and therapeutic challenges

Biljana Mihaljevic, Mihailo Smiljanic, Darko Antic, Nada Kraguljac Kurtovic, Milena Todorovic Balint

Annals of Indian Academy of Neurology 2018 21(1):85-87



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Preventing “neurophobia”: Remodeling neurology education for 21st-century medical students through effective pedagogical strategies for “neurophilia”

Bhaskara P Shelley, Thomas V Chacko, Balakrishnan R Nair

Annals of Indian Academy of Neurology 2018 21(1):9-18

Neurology has a reputation, particularly as a complex "head-to-toe" discipline for undergraduate medical students. Neurophobia syndrome, a global phenomenon, fundamentally stems from pedagogical deficiencies during the undergraduate curriculum, the lack of vertical integration between basic neurosciences and clinical bedside neurology, the lack of clinical reasoning exercises, cognitive heuristics, and clinical problem-solving, errors in diagnostic competence, and hyposkilia. This ultimately results in poor clinical competence and proficiency in clinical neurology and causes attrition in nurturing a passion for learning the neurology discipline. This article explores plausible factors that contribute to the genesis of neurophobia and multifaceted strategies to nurture interest in neurosciences and provide possible solutions to demystify neurology education, especially the need for evidence-based educational interventions. Remodeling neurology education through effective pedagogical strategies and remedial measures, and using the Miller's pyramid, would provide a framework for assessing clinical competence in clinical bedside neurology. Technology-enhanced education and digital classrooms would undoubtedly stamp out neurophobia in medical students of the 21st century. It will not frighten off another generation of nonneurologist physicians to empower them to hone expertise in order to tackle the increasing burden of neurological disorders in India. Furthermore, promoting neurophilia would facilitate the next generation of medical students in pursuing career options in neurology which would be quintessential not only in closing India's looming neurologist workforce gap but also in fostering interest in research imperatives in the next generation of medical students.

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A viral polymyositis masquerade: Life-threatening case of juvenile dermatomyositis complicated by systemic capillary leak syndrome

Bhaskara P Shelley, Shrijeet Chakraborti

Annals of Indian Academy of Neurology 2018 21(1):70-74

This is a case report of an 8-year-old boy who developed an atypical, rare subphenotype of autoimmune inflammatory acute juvenile dermatomyositis (JDM), initially masquerading as viral polymyositis (PM)-like presentation, that was complicated by a hitherto unreported fulminant, life-threatening pediatric systemic capillary leak syndrome (SCLS). We highlight the close differential between viral PM and JDM, the baffling clinical syndromic constellation of hypotension with hemoconcentration – a "shock"-like syndrome, hypoalbuminemia without albuminuria, and generalized edema with the atypical JDM presentation, and stress crucial need to implement early aggressive, multipronged immunomodulatory treatment along with intensive fluid resuscitation which saved the life, this patient from a stormy, and turbulent 4-week clinical illness. This is the first published case description in the current literature of the association of an aggressive subphenotype of JDM and life-threatening pediatric SCLS. This report opens the Pandora's Box to explore the genetic and pathomechanisms of both disorders.

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Edgar adrian and patrick merton: Names blurred with the passage of time

Kalyan B Bhattacharyya

Annals of Indian Academy of Neurology 2018 21(1):19-23

Edgar Douglas Adrian and Patrick Anthony Merton are two supreme neurophysiologists from England in the last century whose names are almost forgotten these days. Adrian's work on all-or-none phenomenon in nerve and muscle excitability ushered in a new era and Merton's servo theory of muscular movement and muscle fatigue added a new dimension to the understanding of stretch reflex and deep tendon reflexes. Both of them trained and worked at Trinity College, Cambridge and both were elected as Fellow of the Royal Society and Adrian in addition, was awarded the Nobel Prize in 1932 along with Charles Scott Sherrington.

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Cysts in White Matter: A Novel Neuroimaging Finding in Infantile GM1 Gangliosidosis

Mahesh Kamate

Annals of Indian Academy of Neurology 2018 21(1):82-83



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Evaluation of various movement disorders in patients of genetically proven spinocerebellar ataxia: A study from a Tertiary Care Center in Northern India

Divya M Radhakrishnan, Vinay Goyal, Achal Kumar Srivastava, Garima Shukla, Madhuri Behari

Annals of Indian Academy of Neurology 2018 21(1):24-28

Background: Movement disorders are one of the prominent nonataxic symptoms in patients of spinocerebellar ataxia (SCA). The type of movement disorder may provide clinical clue to the type of SCA. Objective: The objective of this study is to evaluate various movement disorders in patients of genetically proven SCAs and to establish a probable clinico-genetic correlation. Methods: Ninety-Five patients of genetically proven SCAs were assessed for the presence of various movement disorders. Results: Patients with SCA (75.8% males) with at least one movement disorder contributed 43.16%. Age for onset of movement disorder was 43.39 ± 13.43 years. SCA-12 (38.95%) was the most common subtype. Among the patients with at least one movement disorder, action tremor of hands contributed majority (90.2%). Dystonia and parkinsonism were present in 17.07% and 12.2% of patients (with movement disorder), respectively. Action tremor of hands was present in 34 patients with SCA-12 (91.89%), and 20 patients (54.05%) had onset of hand tremor preceding the onset of ataxia. Majority of patients with SCA-12 (81%) were of the same ethnic origin belonging to Agrawal community. Patients with movement disorder had a later onset (45 ± 13.88 years) of ataxic symptoms compared to those without a movement disorder (32.8 ± 11.92) (P = <0.0005). There was no significant association between severity of ataxia and presence of movement disorder. Conclusion: Movement disorders are present in about 43% of patients with SCA and can precede or succeed the onset of ataxia. Tremor onset SCA predicted SCA-12, especially in Agrawal community.

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Recovery of visual scotomas by vortioxetine in a patient with symptomatic occipital lobe epilepsy

Halil Onder, Akin Coskun, M Tugba Goksungur

Annals of Indian Academy of Neurology 2018 21(1):88-90



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Determinants of remission in medically treated carpal tunnel syndrome: Study from Central India

Ajoy Sodani, Raunak Dani, Mukesh Dube, Dinesh Choukesey, Sunil Athale

Annals of Indian Academy of Neurology 2018 21(1):29-34

Background: The factors associated with the spontaneous remission (SR) of symptoms in carpal tunnel syndrome (CTS) are not well known. Objectives: To look for determinants of SR in medically treated, electrophysiologically proven patients of CTS. Methods: We revisited the medical records and nerve conduction study data of 130 hands with CTS and divided them into two groups as per the absence or persistence of the symptoms when contacted after a median time lapse of 3 years following the diagnosis. Results: SR occurred in 46.1% of the hands. Higher odds of SR were linked with female gender, symptoms restriction to lateral fingers, symptom duration <10 months, mildly delayed median motor and sensory distal latencies, and median sensory amplitude >20 μV. We developed a seven-point scale, on which a score of ≥4 had a strong association (odds ratio 4.31) with SR. Discussion and Conclusion: No single risk factor, standalone, can predict SR in patients with CTS, which could lead to an invasive treatment (Surgery or local injection) to them. We propose that patients scoring ≥4 on our 7 point scale should be treated medically for the initial 10 months after the symptom onset.

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Spinocerebellar ataxia-21 in a Turkish child

Faruk Incecik, Ozlem M Herguner, Patrick Willems, Neslihan O Mungan

Annals of Indian Academy of Neurology 2018 21(1):68-70

Hereditary cerebellar ataxias are genetically heterogeneous disorders. Autosomal recessive spinocerebellar ataxia-21 (SCAR21) is a neurologic disorder characterized by the onset of cerebellar ataxia, recurrent episodes of liver failure, peripheral neuropathy, and learning disabilities. Herein, we reported a case presented with gait and balance problems, swallowing difficulties, mild delayed motor development, and mild learning disability with SCAR21 that confirmed by mutation analysis in a Turkish child. To the best of our knowledge, this is the first case of SCAR21 from Turkey.

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Value of motor nerve conduction studies in the diagnosis of idiopathic tarsal tunnel syndrome: A single-center prospective observational study from India

Ajoy Sodani, Mukesh Dube, Rahul Jain

Annals of Indian Academy of Neurology 2018 21(1):35-41

Background: Nerve conduction studies are considered to be the gold standard for diagnosing secondary tarsal tunnel syndrome (s TTS), but their utility in the diagnosis of idiopathic tarsal tunnel syndrome (i TTS) is largely unknown. Objective: We sought to investigate the value of motor nerve conductions studies (MNCS) in the diagnosis of clinically suspected i TTS. Materials and Methods: Twenty-six (52 limbs) adult patients of clinically suspected i TTS were subjected to motor nerve conductions of posterior tibial nerve, and its branches and motor conduction parameters were compared with those of 45 healthy controls. Results: Symptoms were bilateral in 70% (P = 0.02), with heel pain in 95% of symptomatic limbs. MNCS was abnormal in 32 (80%) of symptomatic limbs and 8 (66.6%) of asymptomatic limbs (P = 0.004). Out of electrophysiologically abnormal nerves (n = 67), the pathological process could be identified in all the nerves with abnormal MNCS (P = 0.02). Probable demyelination was seen in 58.2% of the electrophysiologically abnormal nerves. Discussion: The present study shows that i TTS are gender and Body Mass Index neutral with bilateral symptoms being common. Tinel's sign was inconsistent. Heel pain did not correlate with abnormal inferior calcaneal nerve conductions. Motor nerve conduction study was abnormal in a significant number of symptomatic limbs. "Probable demyelination" was more frequent in symptomatic limbs. Conclusion: MNCS is significantly abnormal in symptomatic limbs of subjects with i TTS. Demyelination is slightly more common than axonopathy in i TTS. With a sensitivity of 80% and specificity of 33.3%, MNCS seems to be useful as a screening tool in clinically suspected i TTS. This study is Level II: Lesser quality randomized controlled trial or prospective comparative study.

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Unilateral limb thinning –Thinking out of the box

Chandramouleeswaran Venkatraman, Shubha Subramanian, Daniel Sweetson Abraham, Kannan Vellaichamy

Annals of Indian Academy of Neurology 2018 21(1):74-76

We report an unusual presentation in a 9-year-old girl with unilateral circumferential thinning of the entire right upper limb without any other neurological deficit, with normal nerve conduction and electromyography initially thought of as a neurodegenerative disorder based on clinical presentation. Magnetic resonance imaging of the upper limb showed partial lipoatrophy with normal glucose metabolism and lipid profile and negativity for HIV and autoimmune disease (panniculitis) with no family history of similar disorder. Remember to think out of box before labeling neurodegenerative disease.

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The Spectrum of Autonomic Dysfunction in Myasthenic Crisis

Rohit Ninan Benjamin, Sanjith Aaron, Ajith Sivadasan, Suresh Devasahayam, Amalan Sebastin, Mathew Alexander

Annals of Indian Academy of Neurology 2018 21(1):42-48

Background: Autoimmune autonomic dysfunction is described in Myasthenia Gravis. In myasthenic crisis, the spectrum of autonomic dysfunction is hitherto uncharacterized. Objective: The objective of this study is to describe the spectrum of autonomic dysfunction in myasthenic crises using the composite autonomic symptom scale 31 (COMPASS 31) autonomic symptom questionnaire and power spectral analysis of heart rate variability (HRV), which is a simple way of estimating general autonomic dysfunction. Methods: Adult patients with myasthenic crisis from January 1, 2014 to March 15, 2015, were prospectively included in this study. The COMPASS 31 questionnaire for symptoms of autonomic dysfunction and power spectral analysis of HRV were assessed. These were compared with the patient's demographic and clinical parameters and with previous literature. IRB approval was obtained. Results: Sixteen patients were included (M:F 3:1). 15/16 patents (93%) had autonomic dysfunction on COMPASS 31 questionnaire. The domains of involvement were gastrointestinal (80%), orthostatic (67.7%), pupillomotor (67.7%); sudomotor (33.3%), and vasomotor (13.3%). Parasympathetic dysfunction predominance was suggested by the symptom profile. HRV analysis showed a low frequency (LF) spectral shift suggesting slowed parasympathetic responsiveness (LF normalized unit (nu): high frequency [HF] nu mean 8.35, standard deviation ± 5.4, 95% confidence interval 2.2–12.5), which significantly exceeded the mean LF nu: HF nu ratios of the majority of previously reported noncrises myasthenic populations. Conclusions: Myasthenic crisis has autonomic dysfunction involving multiple organ systems. Increased latency of parasympathetic reflexes is suggested. A comprehensive management protocol addressing different autonomic domains is required for holistic patient care.

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Uncommon anatomical variant – Types artery of percheron infarcts: Clinical-radiological correlations

T Harisuthan, Anirudh Vilas Kulkarni, Gigy Varkey Kuruttukulam

Annals of Indian Academy of Neurology 2018 21(1):80-81



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