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https://www.readbyqxmd.com/read/29327071/diseases-of-complement-dysregulation-an-overview
#1
REVIEW
Edwin K S Wong, David Kavanagh
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease...
January 11, 2018: Seminars in Immunopathology
https://www.readbyqxmd.com/read/29323198/paroxysmal-nocturnal-hemoglobinuria-pnh-brain-mri-ischemic-lesions-in-neurologically-asymtomatic-patients
#2
Wilma Barcellini, Elisa Scola, Silvia Lanfranconi, Marika Grottaroli, Francesca Binda, Bruno Fattizzo, Anna Zaninoni, Gloria Valcamonica, Claudia Maria Cinnante, Carla Boschetti, Massimiliano Buoli, Carlo Alfredo Altamura, Nereo Bresolin, Fabio Triulzi, Alberto Zanella, Agostino Cortelezzi
This study investigated for the first time brain ischemic involvement in 19 consecutive neurologically asymptomatic PNH patients by non-enhanced cerebral MRI, and by intracranial arterial and venous angio-MRI. Eleven cases (58%, 7 aged <65) showed pathological findings: 9 white matter (WM) abnormalities related to chronic ischemic small vessel disease, 2 a focal abnormality >5 mm, and 5 cases a score >4 by the age-related white matter changes (ARWMC) scale. Compared with age and sex-matched controls (1:2 ratio), patients showed an increased frequency of periventricular WM vascular degeneration (32% versus 5...
January 11, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29301160/-pharmacotherapy-of-vestibular-disorders-nystagmus-and-cerebellar-disorders
#3
K Feil, N Böttcher, O Kremmyda, C Muth, J Teufel, A Zwergal, T Brandt, M Strupp
There are currently different groups of drugs for the pharmacotherapy of vertigo, nystagmus and cerebellar disorders: antiemetics; anti-inflammatories, antimenieres, and antimigraineous medications and antidepressants, anticonvulsants, aminopyridines as well as acetyl-DL-leucine. In acute unilateral vestibulopathy, corticosteroids improve the recovery of peripheral vestibular function, but currently there is not sufficient evidence for a general recommendation. There is insufficient evidence to support the view that 16 mg t...
January 2018: Laryngo- Rhino- Otologie
https://www.readbyqxmd.com/read/29295578/devices-for-ambulatory-monitoring-of-sleep-associated-disorders-in-children-with-neurological-diseases
#4
Adriana Ulate-Campos, Melissa Tsuboyama, Tobias Loddenkemper
Good sleep quality is essential for a child's wellbeing. Early sleep problems have been linked to the later development of emotional and behavioral disorders and can negatively impact the quality of life of the child and his or her family. Sleep-associated conditions are frequent in the pediatric population, and even more so in children with neurological problems. Monitoring devices can help to better characterize sleep efficiency and sleep quality. They can also be helpful to better characterize paroxysmal nocturnal events and differentiate between nocturnal seizures, parasomnias, and obstructive sleep apnea, each of which has a different management...
December 25, 2017: Children
https://www.readbyqxmd.com/read/29287036/benign-paroxysmal-positional-vertigo-bppv-in-children-and-adolescents-clinical-features-and-response-to-therapy-in-110-pediatric-patients
#5
Jacob R Brodsky, Sophie Lipson, Jared Wilber, Guangwei Zhou
OBJECTIVE: This study aimed to characterize the clinical features and outcomes of benign paroxysmal positional vertigo (BPPV) in the pediatric population. STUDY DESIGN: Retrospective case review. SETTING: Tertiary care center. PATIENTS: One hundred ten patients, aged 5 to 19 years old, diagnosed with BPPV. MAIN OUTCOME MEASURES: Patient demographics, comorbidities, canal involvement, response to treatment, and incidence of recurrence...
December 28, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/29282340/-from-involuntary-movements-to-movement-disorders
#6
Toshio Fukutake
Apart from the term 'involuntary movements', the term 'movement disorders' encompasses not only classical hyperkinesias but also hypokinesias including catalepsy or apraxia. It enables us to understand abnormal movements by their phenomena instead of by their localization. To advance the grasping of movement disorders, we discuss the clinical and pathophysiological features of abnormal movements in catatonia/catalepsy, anti-NMDAR encephalitis, paroxysmal dyskinesias, stiff person/leg syndrome, corticobasal degeneration/syndrome, and hysteria...
December 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29279710/stroke-in-a-young-man-secondary-to-paroxysmal-atrial-fibrillation-and-thyrotoxicosis-a-case-report
#7
Rodrigo Bazan, Thiago Dias Fernandes, Gláucia Maria Ferreira da Silva Mazeto, Mariângela Esther Alencar Marques, Gabriel Pereira Braga, Gustavo José Luvizutto, Silméia Garcia Zanati Bazan
We report a case of a male patient with stroke caused by atrial fibrillation (AF) due to thyrotoxicosis. At hospital admission, he presented hypertension and AF. Magnetic resonance imaging confirmed a right-side ischemic area. The thyrotoxicosis was confirmed by thyroid function and thyroid scintigraphy that showed goiter with diffuse hypercaptation. The patient was treated with tapazole and total thyroidectomy, and pathological findings suggested Graves' disease. Hyperthyroidism is associated with increased supraventricular ectopic activity in patients with a normal heart, and may be an important causal link between hyperthyroidism and AF...
September 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/29250756/sporadic-nocturnal-frontal-lobe-epilepsy-report-on-two-cases-and-review-of-the-first-taiwanese-series-of-10-cases
#8
Shih-Bin Yeh, Carlos H Schenck
PURPOSE: To report two additional cases of sporadic (i.e. non-familial) Nocturnal Frontal Lobe Epilepsy (NFLE) and integrate these two cases within the first series of 10 cases of sporadic NFLE reported in Taiwanese patients, and compare the findings with familial NFLE and with findings from Caucasian NFLE patients. METHODS: Clinical interviews, neurological examinations, EEG, brain MRI, and overnight videopolysomnographic (vPSG) monitoring with EEG seizure montage, and treatment outcome...
June 15, 2017: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/29246924/the-prothrombotic-state-in-paroxysmal-nocturnal-haemoglobinuria-a-multifaceted-source
#9
Barnaby Peacock-Young, Fraser L Macrae, Darren J Newton, Anita Hill, Robert A S Ariëns
Paroxysmal nocturnal haemoglobinuria is a rare acquired haematological disorder, the most serious complication of which is thrombosis. The increased incidence of thrombosis in paroxysmal nocturnal haemoglobinuria is still poorly understood, but unlike many other thrombotic disorders, predominantly involves complement mediated mechanisms. This review article discusses the different factors that contribute to the increased risk of thrombosis in paroxysmal nocturnal haemoglobinuria. Paroxysmal nocturnal haemoglobinuria leads to a complex and multifaceted prothrombotic state due to the pathological effects of platelet activation, intravascular haemolysis and neutrophil/monocyte activation...
December 15, 2017: Haematologica
https://www.readbyqxmd.com/read/29238716/pediatric-multiple-sclerosis-in-tunisia-a-retrospective-study-over-11-years
#10
Nedia Ben Achour, Ibtihel Rebai, Sarra Raddadi, Hanene Benrhouma, Hedia Klaa, Aida Rouissi, Ichraf Kraoua, Ilhem Ben Youssef Turki
Introduction: Pediatric multiple sclerosis (pMS) is a rare demyelinating disorder with an onset before the age of 18 years. In this study, we aimed to investigate the characteristics of pMS in Tunisian children. Patients and Methods: We conducted a retrospective study over 11 years (2005-2016) including all patients diagnosed with pMS according to the International Pediatric Multiple Sclerosis Study Group (IPMSSG) criteria of 2012 and followed up in a tertiary care research center...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29236352/iccs-escca-consensus-guidelines-for-the-clinical-utility-of-testing-for-gpi-anchor-deficient-clones-in-paroxysmal-nocturnal-hemoglobinuria-pnh-and-other-bone-marrow-disorders-part-1
#11
Amy E Dezern, Michael J Borowitz
Paroxysmal nocturnal hemoglobinuria (PNH) arises as a consequence of the non-malignant clonal expansion of one or more hematopoietic stem cells with an acquired somatic mutation of the PIGA gene.(1) Progeny of affected stem cells are deficient in glycosyl phosphatidylinositol-anchored proteins (GPI-APs). This deficiency is readily detected by flow cytometry. Though this seems straightforward, the clinical utility of this testing requires that the ordering clinician understand not only the characteristics of the test, but also the biology of the underlying disease, and the clinical and laboratory manifestations in the individual patient...
December 13, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29236350/iccs-escca-consensus-guidelines-to-detect-gpi-deficient-cells-in-paroxysmal-nocturnal-hemoglobinuria-pnh-and-related-disorders-part-3-data-analysis-reporting-and-case-studies
#12
Andrea Illingworth, Iuri Marinov, D Robert Sutherland, Orianne Wagner Ballon, Luigi DelVecchio
Over the past several years, a diverse group of physicians and other laboratory scientists have developed various recommendations and guidelines regarding best practices for PNH testing. This manuscript is based on these previous recommendations as well as various other relevant publications of experts in the area of PNH testing. The goal is to provide flow cytometry laboratories with an updated consensus approach to analysis and reporting of PNH results and to address the most common analytical challenges for accurate reporting of this rare disease...
December 13, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29225584/prevalence-rates-of-the-incubus-phenomenon-a-systematic-review-and-meta-analysis
#13
Marc L Molendijk, Harriët Montagne, Ouarda Bouachmir, Zeynep Alper, Jan-Pieter Bervoets, Jan Dirk Blom
Background: The incubus phenomenon is a paroxysmal sleep-related disorder characterized by compound hallucinations experienced during brief phases of (apparent) wakefulness. The condition has an almost stereotypical presentation, characterized by a hallucinated being that exerts pressure on the thorax, meanwhile carrying out aggressive and/or sexual acts. It tends to be accompanied by sleep paralysis, anxiety, vegetative symptoms, and feelings of suffocation. Its prevalence rate is unknown since, in prior analyses, cases of recurrent isolated sleep paralysis with/without an incubus phenomenon have been pooled together...
2017: Frontiers in Psychiatry
https://www.readbyqxmd.com/read/29214126/pharmacologic-complement-inhibition-in-clinical-transplantation
#14
REVIEW
Vasishta S Tatapudi, Robert A Montgomery
Purpose of Review: Over the past two decades, significant strides made in our understanding of the etiology of antibody-mediated rejection (AMR) in transplantation have put the complement system in the spotlight. Here, we review recent progress made in the field of pharmacologic complement inhibition in clinical transplantation and aim to understand the impact of this therapeutic approach on outcomes in transplant recipients. Recent Findings: Encouraged by the success of agents targeting the complement cascade in disorders of unrestrained complement activation like paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), investigators are testing the safety and efficacy of pharmacologic complement blockade in mitigating allograft injury in conditions ranging from AMR to recurrent post-transplant aHUS, C3 glomerulopathies and antiphospholipid anti-body syndrome (APS)...
2017: Current Transplantation Reports
https://www.readbyqxmd.com/read/29207275/clinical-features-of-otolith-organ-specific-vestibular-dysfunction
#15
Chisato Fujimoto, Sayaka Suzuki, Makoto Kinoshita, Naoya Egami, Keiko Sugasawa, Shinichi Iwasaki
OBJECTIVE: To elucidate the clinical features and vestibular symptoms of patients with otolith organ dysfunction in the presence of normal function of the semicircular canals. METHODS: We reviewed the clinical records of 277 consecutive new patients with balance disorders who underwent testing of cervical and ocular vestibular evoked myogenic potentials (cVEMPs and oVEMPs) as well as caloric testing and video head impulse testing (vHIT). RESULTS: We identified 76 patients who showed normal caloric responses and normal vHIT findings in each SCC plane, but abnormal responses in cVEMP and/or oVEMP testing...
January 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29205326/clinical-features-familial-history-and-migraine-precursors-in-patients-with-definite-vestibular-migraine-the-vm-phenotypes-projects
#16
Roberto Teggi, Bruno Colombo, Roberto Albera, Giacinto Asprella Libonati, Cristiano Balzanelli, Angel Batuecas Caletrio, Augusto Casani, Juan Manuel Espinoza-Sanchez, Paolo Gamba, Jose A Lopez-Escamez, Sergio Lucisano, Marco Mandalà, Giampiero Neri, Daniele Nuti, Rudy Pecci, Antonio Russo, Eduardo Martin-Sanz, Ricardo Sanz, Gioacchino Tedeschi, Paola Torelli, Paolo Vannucchi, Giancarlo Comi, Mario Bussi
OBJECTIVE: The aim of this work was to assess through a questionnaire the features of vertiginous episodes, accompanying symptoms, familial history, and migraine precursors in a sample of 252 subjects with a diagnosis of definite vestibular migraine. BACKGROUND: Migraine is a common neurological disorder characterized by episodic headaches with specific features. About two-thirds of cases run in families, and patients may refer symptoms occurring in infancy and childhood, defined as episodic syndromes that may be associated with migraine...
December 4, 2017: Headache
https://www.readbyqxmd.com/read/29193418/causes-and-predictors-of-30-day-readmission-after-cardiovascular-implantable-electronic-devices-implantation-insights-from-nationwide-readmissions-database
#17
Shahzad Ahmad, Muhammad Bilal Munir, Michael S Sharbaugh, Andrew D Althouse, Deepak Kumar Pasupula, Samir Saba
INTRODUCTION: Readmissions are a burden on health care resources and have negative impact on patients. Cardiovascular Implantable Electronic Devices (CIED) are frequently used in the management of rhythm disorders and advanced heart failure. We assessed 30-day readmissions in patients admitted for CIED implantation in a sample of United States patients. METHODS: Data were extracted from Nationwide Readmissions Database for calendar year 2013. Patients admitted for CIED implantation were identified using ICD-9 codes...
November 28, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/29167286/a-novel-prrt2-pathogenic-variant-in-a-family-with-paroxysmal-kinesigenic-dyskinesia-and-benign-familial-infantile-seizures
#18
Jacqueline G Lu, Juliet Bishop, Sarah Cheyette, Igor B Zhulin, Su Guo, Nara Sobreira, Steven E Brenner
Paroxysmal Kinesigenic Dyskinesia (PKD) is a rare neurological disorder characterized by recurrent attacks of dyskinetic movements without alteration of consciousness that are often triggered by the initiation of voluntary movements. Whole exome sequencing has revealed a cluster of pathogenic variants in PRRT2 (proline-rich transmembrane protein), a gene with a function in synaptic regulation that remains poorly understood. Here, we report the discovery of a novel PRRT2 pathogenic variant inherited in an autosomal dominant pattern in a family with PKD and Benign Familial Infantile Seizures (BFIS)...
November 22, 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29151509/in-paroxysmal-atrial-fibrillation-patients-the-neutrophil-to-lymphocyte-ratio-is-related-to-thrombogenesis-and-more-closely-associated-with-left-atrial-appendage-contraction-than-with-the-left-atrial-body-function
#19
Yukihiro Fukuda, Mitsunori Okamoto, Shunsuke Tomomori, Hiroya Matsumura, Takehito Tokuyama, Yukiko Nakano, Yasuki Kihara
Objective The neutrophil-to-lymphocyte ratio (NLR) is an inflammation marker that can be used to detect atrial inflammatory changes, which may contribute to a reduced left atrial (LA) function and thrombosis. Our study aimed to determine whether or not the association of NLR with the LA appendage (LAA) function in relation to thrombogenesis differs from the association with the LA body function in paroxysmal atrial fibrillation (PAF) patients. Methods A total of 183 PAF patients were studied. The LA volume index, mitral flow velocity (A), and mitral annular motion velocity (A') were examined using transthoracic echocardiography...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29151098/the-alice-in-wonderland-syndrome
#20
Douglas J Lanska, John R Lanska
In 1955, English psychiatrist John Todd defined the Alice-in-Wonderland syndrome (AIWS) as self-experienced paroxysmal body-image illusions involving distortions of the size, mass, or shape of the patient's own body or its position in space, often accompanied by depersonalization and/or derealization. AIWS had been described by American Neurologist Caro Lippman in 1952, but Todd's report was the most influential. Todd named the syndrome for the perceptual disorder of altered body image experienced by the protagonist in Alice's Adventures in Wonderland (1865) by Lewis Carroll (Charles Lutwidge Dodgson)...
2018: Frontiers of Neurology and Neuroscience
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