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https://www.readbyqxmd.com/read/28628467/juvenile-and-adult-onset-systemic-lupus-erythematosus-a-comparative-study-in-a-large-cohort-from-the-spanish-society-of-rheumatology-lupus-registry-relesser
#1
Vicenç Torrente-Segarra, Tarek Carlos Salman Monte, Iñigo Rúa-Figueroa, Fernando Sánchez-Alonso, Francisco Javier López-Longo, María Galindo-Izquierdo, Jaime Calvo-Alén, Alejandro Olivé-Marqués, Jesús Ibañez-Ruán, Loreto Horcada, Ana Sánchez-Atrio, Carlos Montilla, Rafael Benito Melero González, Elvira Díez-Álvarez, Victor Martinez-Taboada, José Luis Andreu, Olaia Fernández-Berrizbeitia, José Ángel Hernández-Beriain, Marian Gantes, Blanca Hernández-Cruz, Ángela Pecondón-Español, Carlos Marras, Gema Bonilla, José M Pego-Reigosa
OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years...
June 12, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28624716/seizure-related-hospital-admissions-readmissions-and-costs-comparisons-with-asthma-and-diabetes-in-south-australia
#2
Michelle L Bellon, Christopher Barton, Nikki McCaffrey, Denise Parker, Claire Hutchinson
PURPOSE: Seizures are listed as an Ambulatory Care Sensitive Condition (ACSC), where, in some cases, hospitalisation may be avoided with appropriate preventative and early management in primary care. We examined the frequencies, trends and financial costs of first and subsequent seizure-related hospital admissions in the adult and paediatric populations, with comparisons to bronchitis/asthma and diabetes admissions in South Australia between 2012 and 2014. METHODS: De-identified hospital separation data from five major public hospitals in metropolitan South Australia were analysed to determine the number of children and adults admitted for the following Australian Refined Diagnosis Related Groups: seizure related conditions; bronchitis/asthma; and diabetes...
June 10, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28623751/epilepsy-and-stigmatization-in-turkey
#3
Ozge Doganavsargil-Baysal, Buket Cinemre, Yesim Senol, Ebru Barcin, Zehra Gokmen
Patients with epilepsy experience stigmatization quite often. Studies investigating stigmatization perceived by patients with epilepsy in Turkey are limited in number. In this study, we aimed to understand the relationship between stigmatization and psychopathology and also to identify the effects of stigmatization on the quality of life in epilepsy. Patients completed a socio-demographical data form and epilepsy information form. They were evaluated in terms of psychiatric diagnosis using SCID I and they were asked to complete SCL-90 R, Epilepsy Stigmatization Scale and SF 36...
June 14, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28619360/further-delineation-of-cog8-cdg-a-case-with-novel-compound-heterozygous-mutations-diagnosed-by-targeted-exome-sequencing
#4
Aram Yang, Sung Yoon Cho, Ja-Hyun Jang, Jinsup Kim, Sook Za Kim, Beom Hee Lee, Han-Wook Yoo, Dong-Kyu Jin
Congenital disorders of glycosylation (CDG) are a rapidly expanding group of inherited metabolic disorders with highly variable clinical presentations caused by deficient glycosylation of proteins and/or lipids. CDG-IIh is a very rare subgroup of CDG caused by mutations in the conserved oligomeric Golgi (COG) complex gene, COG8, and so far, only two cases have been reported in the medical literature. Here, we describe an 8-year-old Korean boy with psychomotor retardation, hypotonia, failure to thrive, elevated serum liver enzymes, microcephaly, and talipes equinovarus...
June 13, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28605627/impaired-responsibility-dimension-of-self-esteem-of-brazilian-adolescents-with-epilepsy
#5
Nathália F Siqueira, Fernando L B B Oliveira, Elisabete Abib Pedroso de Souza
This study aimed to compare the self-esteem of Brazilian adolescents with epilepsy and Brazilian adolescents without this condition and the correlations between self-esteem of these adolescents with depression and anxiety symptoms. Study participants were 101 adolescents of both sexes, aged 10-19years old, from elementary and high school education. Fifty patients diagnosed with uncomplicated epilepsy attending the pediatric epilepsy clinic of University Hospital composed the case group. The other fifty-one adolescents without this diagnosis were attending public schools in Campinas-SP region...
June 9, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28604555/immune-related-neurological-symptoms-in-an-adolescent-patient-receiving-the-checkpoint-inhibitor-nivolumab
#6
Dmitry Tchapyjnikov, Alexandra J Borst
Immune checkpoint inhibitors are a novel group of immunotherapeutic agents for the treatment of cancer. Immune-related adverse events, including neurological symptoms, have been associated with these agents. We present the case of an adolescent with refractory Hodgkin lymphoma treated with nivolumab, a PD1 inhibitor, who developed Hashimoto thyroiditis, posterior reversible encephalopathy syndrome causing seizures, as well as urinary retention, truncal/appendicular spasticity, dysphagia, and a progressive encephalopathy that was clinically consistent with a diagnosis of progressive encephalopathy with rigidity and myoclonus, a stiff-person-syndrome spectrum disorder...
June 9, 2017: Journal of Immunotherapy
https://www.readbyqxmd.com/read/28575766/increased-odds-and-predictive-rates-of-mmpi-2-rf-scale-elevations-in-patients-with-psychogenic-non-epileptic-seizures-and-observed-sex-differences
#7
Victor A Del Bene, Miguel Arce Rentería, Moshe Maiman, Mitch Slugh, Deana M Gazzola, Siddhartha S Nadkarni, William B Barr
OBJECTIVE: The Minnesota Multiphasic Personality Inventory-2-Restructured Form (MMPI-2-RF) is a self-report instrument, previously shown to differentiate patients with epileptic seizures (ES) and psychogenic non-epileptic seizures (PNES). At present, the odds of MMPI-2-RF scale elevations in PNES patients, as well as the diagnostic predictive value of such scale elevations, remain largely unexplored. This can be of clinical utility, particularly when a diagnosis is uncertain. METHOD: After looking at mean group differences, we applied contingency table derived odds ratios to a sample of ES (n=92) and PNES (n=77) patients from a video EEG (vEEG) monitoring unit...
May 30, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28557061/classification-of-involuntary-movements-in-dogs-myoclonus-and-myotonia
#8
REVIEW
M Lowrie, L Garosi
Myoclonus is a sudden brief, involuntary muscle jerk. Of all the movement disorders, myoclonus is the most difficult to encapsulate into any simple framework. On the one hand, a classification system is required that is clinically useful to aid in guiding diagnosis and treatment. On the other hand, there is need for a system that organizes current knowledge regarding biological mechanisms to guide scientific research. These 2 needs are distinct, making it challenging to develop a robust classification system suitable for all purposes...
May 30, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28464288/electroclinical-findings-of-minor-motor-events-during-sleep-in-temporal-lobe-epilepsy
#9
Loretta Giuliano, Denise Uccello, Daniela Fatuzzo, Greta Mainieri, Mario Zappia, Vito Sofia
OBJECTIVE: It is well known that sleep-related motor seizures can originate from the temporal lobe. However, little is known about the clinical features of minor motor manifestations during sleep in patients with temporal lobe epilepsy. The main objective of our study was to verify the existence of minor motor events during sleep in patients with mesial temporal lobe epilepsy (MTLE) and to define their clinical features and electroencephalography (EEG) correlations. METHODS: We enrolled in the study patients with diagnosis of symptomatic MTLE and a group of healthy controls...
May 2, 2017: Epilepsia
https://www.readbyqxmd.com/read/28412316/differentiating-stroke-and-seizure-in-acute-setting-perfusion-computed-tomography
#10
Katarzyna Kubiak-Balcerewicz, Urszula Fiszer, Ewa Nagańska, Cezary Siemianowski, Aleksander Sobieszek, Agnieszka Witak-Grzybowska, Aldona Kosińska-Szot
BACKGROUND: Perfusion computed tomography (PCT) is part of acute stroke protocol in many hospitals; however, its clinical utility is still being disputed. Beyond its use in core and penumbra estimation, there is also a question about PCT role in stroke mimics diagnosis. Case series or small, retrospective studies showed equivocal results. This is the first published prospective, comparative study on PCT in differentiating stroke and seizure in acute setting. METHODS: Patients with acute focal neurologic deficits and without acute ischemic lesions on routine CT underwent PCT and electroencephalography (EEG) within 12 hours after symptom onset...
April 13, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28332073/aicardi-gouti%C3%A3-res-syndrome-unusual-neuro-radiological-manifestations
#11
Ghada M H Abdel-Salam, Mohamed S Abdel-Hamid, Shaimaa A Mohammad, Sherif F Abdel-Ghafar, Doaa R Soliman, Hala T El-Bassyouni, Laila Effat, Maha S Zaki
Aicardi-Goutières syndrome (AGS) is one of the expanding group of inherited congenital infection like syndromes. Here, we describe the detailed clinical and imaging findings of two sibs with AGS. Each shows scattered periventricular intracranial calcifications, severe global delay, seizures, microcephaly and spasticity. Interestingly, chilblains were observed in the two sisters as well as their parents and a paternal uncle. The brain MRI of the older sister showed marked ventricular dilatation as a result of unusual associated porencephalic cysts...
June 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28284396/pyridoxal-phosphate-supplementation-in-neuropediatric-disorders
#12
Elisenda Cortès-Saladelafont, Marta Molero-Luis, Hsjd Working Group, Rafael Artuch, Àngels García-Cazorla
Pyridoxal phosphate (PLP) is the active form of vitamin B6 and a cofactor in many enzyme reactions including neurotransmitter metabolism. PLP metabolism disturbances may mostly lead to refractory seizures. In this report, we review the main pathophysiological factors related with PLP deficiency and our experience in PLP treatment in pediatric patients with low-normal cerebrospinal fluid PLP values who presented epilepsy. Only one case had a definite diagnosis (Phelan-McDermid syndrome). The results of extensive metabolic workups and targeted genetic studies were normal for all patients...
November 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28273990/neurological-manifestations-in-systemic-lupus-erythematosus-a-single-centre-study-from-north-east-india
#13
Sanjeeb Kakati, Bhupen Barman, Sobur U Ahmed, Masaraf Hussain
INTRODUCTION: Neurological manifestations although common in Systemic Lupus Erythematosus (SLE), are often not recognized due to their diversed and varied presentation. Therefore, the study was planned to highlight the pattern of neurological involvement in SLE to help in early recognition. AIM: To study the pattern of neurological involvement in SLE and its correlation with disease activity and different investigation. MATERIALS AND METHODS: This hospital based prospective observational study was carried out from August 2009 to July 2010...
January 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28173780/protocol-for-the-encode-trial-evaluating-a-novel-online-depression-intervention-for-persons-with-epilepsy
#14
Björn Meyer, Mario Weiss, Martin Holtkamp, Stephan Arnold, Katja Brückner, Johanna Schröder, Franziska Scheibe, Yvonne Nestoriuc
BACKGROUND: Depression is common among persons with epilepsy (PwE), affecting roughly one in three individuals, and its presence is associated with personal suffering, impaired quality of life, and worse prognosis. Despite the availability of effective treatments, depression is often overlooked and treated inadequately in PwE, in part because of assumed concerns over drug interactions or proconvulsant effects of antidepressants. Internet-administered psychological interventions might complement antidepressant medication or psychotherapy, and preliminary evidence suggests that they can be effective...
February 7, 2017: BMC Psychiatry
https://www.readbyqxmd.com/read/28088048/disclosure-management-behaviors-in-korean-adults-with-well-controlled-epilepsy-their-relation-to-perception-of-stigma
#15
Sang-Ahm Lee, Byung-In Lee
PURPOSE: In spite of the fact that epilepsy is a concealable stigmatized identity, there is little evidence pertaining to disclosure management in adults living with epilepsy. We determined the factors contributing to disclosure management strategies in adults living with well-controlled epilepsy. METHODS: This was a cross-sectional multicenter study. Korean adults whose seizures had remitted for at least one year participated in this study. Using statistical analyses, we determined whether disclosure management behaviors measured using the Disclosure Management Scale (DMS) were related to demographic, clinical, and psychosocial variables...
February 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28065191/dissociation-in-patients-with-dissociative-seizures-relationships-with-trauma-and-seizure-symptoms
#16
S Pick, J D C Mellers, L H Goldstein
BACKGROUND: This study aimed to extend the current understanding of dissociative symptoms experienced by patients with dissociative (psychogenic, non-epileptic) seizures (DS), including psychological and somatoform types of symptomatology. An additional aim was to assess possible relationships between dissociation, traumatic experiences, post-traumatic symptoms and seizure manifestations in this group. METHOD: A total of 40 patients with DS were compared with a healthy control group (n = 43), matched on relevant demographic characteristics...
January 9, 2017: Psychological Medicine
https://www.readbyqxmd.com/read/27912112/ethnic-variation-of-genetic-idiopathic-generalized-epilepsy-in-malaysia
#17
Kheng Seang Lim, Ching Ching Ng, Chung Kin Chan, Wee Shean Foo, Joyce Siew Yong Low, Chong Tin Tan
PURPOSE: Ethnic variation in epilepsy classification was reported in the Epilepsy Phenome/Genome Project. This study aimed to determine the ethnic variation in the prevalence of genetic (idiopathic) generalized epilepsy (GGE) and GGE with family history in a multi-ethnic Asian population in Malaysia. METHOD: In this cross-sectional study, 392 patients with a clinical diagnosis of GGE were recruited in the neurology outpatient clinic, University of Malaya Medical Centre (UMMC), from January 2011 till April 2016...
February 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27869305/analysis-of-falls-in-patients-with-epilepsy-enrolled-in-the-perampanel-phase-iii-randomized-double-blind-studies
#18
RANDOMIZED CONTROLLED TRIAL
Ilo E Leppik, Haichen Yang, Betsy Williams, Sharon Zhou, Randi Fain, Anna Patten, Francesco Bibbiani, Antonio Laurenza
OBJECTIVE: To analyze occurrence of falls among patients with partial seizures, with/without secondarily generalized seizures (SGS), and primary generalized tonic-clonic seizures (PGTCS) in the perampanel phase III clinical studies. METHODS: Studies 304, 305, and 306 randomized subjects (≥12 years) with drug-resistant partial seizures (with/without SGS) to perampanel 2, 4, 8, or 12 mg or placebo for double-blind treatment. The adverse event (AE) of falls was analyzed in the Safety Analysis Set (N = 1480)...
January 2017: Epilepsia
https://www.readbyqxmd.com/read/27843467/comparison-of-relation-between-attention-deficit-hyperactivity-disorder-in-children-with-and-without-simple-febrile-seizure-admitted-in-arak-central-iran
#19
Bahman Salehi, Parsa Yousefichaijan, Smira Safi Arian, Somaieh Ebrahimi, Mahdyieh Naziri
OBJECTIVE: Febrile seizure is one of the most prevalent childhood convulsions with the most common age of onset at 14-18 mo old. Fever decreases the brain threshold for seizure. Attention Deficit Hyperactivity Disorder (ADHD) is also a neurologic-behavioral problem defined by attention deficit and hyperactivity according to DSM-IV criteria in which the child must have these signs in two different environments. There is controversy on the possible relation between febrile seizure and ADHD; while some studies approve a strong relation, some exclude any relation and some attribute ADHD to the side effects of other reasons...
2016: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/27789949/managing-drug-resistant-epilepsy-challenges-and-solutions
#20
REVIEW
Linda Dalic, Mark J Cook
Despite the development of new antiepileptic drugs (AEDs), ~20%-30% of people with epilepsy remain refractory to treatment and are said to have drug-resistant epilepsy (DRE). This multifaceted condition comprises intractable seizures, neurobiochemical changes, cognitive decline, and psychosocial dysfunction. An ongoing challenge to both researchers and clinicians alike, DRE management is complicated by the heterogeneity among this patient group. The underlying mechanism of DRE is not completely understood. Many hypotheses exist, and relate to both the intrinsic characteristics of the particular epilepsy (associated syndrome/lesion, initial response to AED, and the number and type of seizures prior to diagnosis) and other pharmacological mechanisms of resistance...
2016: Neuropsychiatric Disease and Treatment
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