Neuromyelitis optica spectrum: novel concept of pathogenesis, diagnosis and treatment of Devic’s disease

2009 ◽  
Vol 150 (46) ◽  
pp. 2101-2109 ◽  
Author(s):  
Péter Csécsei ◽  
Anita Trauninger ◽  
Sámuel Komoly ◽  
Zsolt Illés

The identification of autoantibodies generated against the brain isoform water channel aquaporin4 in the sera of patients, changed the current diagnostic guidelines and concept of neuromyelitis optica (NMO). In a number of cases, clinical manifestation is spatially limited to myelitis or relapsing optic neuritis creating a diverse. NMO spectrum. Since prevention of relapses provides the only possibility to reduce permanent disability, early diagnosis and treatment is mandatory. In the present study, we discuss the potential role of neuroimaging and laboratory tests in differentiating the NMO spectrum from other diseases, as well as the diagnostic procedures and therapeutic options. We also present clinical cases, to provide examples of different clinical settings, diagnostic procedures and therapeutic decisions.

2021 ◽  
Vol 153 (8) ◽  
Author(s):  
Alex J. Smith ◽  
Gokhan Akdemir ◽  
Meetu Wadhwa ◽  
Dan Song ◽  
Alan S. Verkman

Extracellular solutes in the central nervous system are exchanged between the interstitial fluid, the perivascular compartment, and the cerebrospinal fluid (CSF). The “glymphatic” mechanism proposes that the astrocyte water channel aquaporin-4 (AQP4) is a major determinant of solute transport between the CSF and the interstitial space; however, this is controversial in part because of wide variance in experimental data on interstitial uptake of cisternally injected solutes. Here, we investigated the determinants of solute uptake in brain parenchyma following cisternal injection and reexamined the role of AQP4 using a novel constant-pressure method. In mice, increased cisternal injection rate, which modestly increased intracranial pressure, remarkably increased solute dispersion in the subarachnoid space and uptake in the cortical perivascular compartment. To investigate the role of AQP4 in the absence of confounding variations in pressure and CSF solute concentration over time and space, solutes were applied directly onto the brain surface after durotomy under constant external pressure. Pressure elevation increased solute penetration into the perivascular compartment but had little effect on parenchymal solute uptake. Solute penetration and uptake did not differ significantly between wild-type and AQP4 knockout mice. Our results offer an explanation for the variability in cisternal injection studies and indicate AQP4-independent solute transfer from the CSF to the interstitial space in mouse brain.


Life ◽  
2021 ◽  
Vol 11 (11) ◽  
pp. 1200
Author(s):  
Francesca Tinti ◽  
Ilaria Umbro ◽  
Mariadomenica D’Alessandro ◽  
Silvia Lai ◽  
Manuela Merli ◽  
...  

Cholemic nephropathy (CN) is a recognized cause of acute kidney injury (AKI) in patients with severe hyperbilirubinemia (sHyb) and jaundice. Pathophysiological mechanisms of CN are not completely understood, but it seems caused both by direct toxicity of cholephiles and bile casts formation in nephrons enhanced by prolonged exposure to sHyb, particularly in the presence of promoting factors, as highlighted by a literature reviewed and by personal experience. The aim of our update is to retrace CN in its pathophysiology, risk factors, diagnosis and treatment, underlining the role of sHyb, promoting factors, and CN-AKI diagnostic criteria in the different clinical settings associated with this often-concealed disease. Our purpose is to focus on clinical manifestation of CN, exploring the possible transition to CKD. Cholemic nephropathy is an overlooked clinical entity that enters differential diagnosis with other causes of AKI. Early diagnosis and treatment are essential because renal injury could be fully reversible as rapidly as bilirubin levels are reduced. In conclusion, our proposal is to introduce an alert for considering CN in diagnostic and prognostic scores that include bilirubin and/or creatinine with acute renal involvement, with the aim of early diagnosis and treatment of sHyb to reduce the burden on renal outcome.


2018 ◽  
pp. 290-294
Author(s):  
Jennifer Bellis

This chapter reviews the evaluation, workup, diagnosis and treatment of chest pain in a child. Additionally, it reviews common causes of pediatric chest pain as well as important components of the history and physical that may indicate a more serious cause of pediatric chest pain. These patients may require further diagnostic procedures or referral to a subspecialist for further evaluation. Finally, it discusses the role of electrocardiogram and chest X-rays in the workup of pediatric chest pain. In general, most adolescent occurrence of chest pain has a benign etiology, commonly with a musculoskeletal cause. Absent other abnormal findings, these patients often require only a minimal workup.


2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Leandro Castañeyra-Ruiz ◽  
Ibrahim González-Marrero ◽  
Agustín Castañeyra-Ruiz ◽  
Juan M. González-Toledo ◽  
María Castañeyra-Ruiz ◽  
...  

Neuromyelitis optica is an inflammatory disease characterized by neuritis and myelitis of the optic nerve. Its physiopathology is connected with the aquaporin-4 water channel, since antibodies against aquaporin-4 have been found in the cerebrospinal fluid and blood of neuromyelitis optica patients. The seropositivity for aquaporin-4 antibodies is used for the diagnosis of neuromyelitis optica or neuromyelitis optica spectrum disease. On the other hand, aquaporin-4 is expressed in astrocyte feet in the brain-blood barrier and subventricular zones of the brain ventricles. Aquaporin-4 expression is high in cerebrospinal fluid in hydrocephalus. Furthermore, neuroepithelial denudation precedes noncommunicating hydrocephalus and this neuroepithelial disruption could allow aquaporin-4 to reach anomalous brain areas where it is unrecognized and induce the generation of aquaporin-4 antibodies which could cause the neuromyelitis optica and certain types of hydrocephalus.


Cells ◽  
2019 ◽  
Vol 8 (2) ◽  
pp. 90 ◽  
Author(s):  
Simone Mader ◽  
Lior Brimberg

Aquaporin-4 (AQP4) is a water channel expressed on astrocytic endfeet in the brain. The role of AQP4 has been studied in health and in a range of pathological conditions. Interest in AQP4 has increased since it was discovered to be the target antigen in the inflammatory autoimmune disease neuromyelitis optica spectrum disorder (NMOSD). Emerging data suggest that AQP4 may also be implicated in the glymphatic system and may be involved in the clearance of beta-amyloid in Alzheimer’s disease (AD). In this review, we will describe the role of AQP4 in the adult and developing brain as well as its implication for disease.


2011 ◽  
Vol 26 (S2) ◽  
pp. 180-180
Author(s):  
R. Softic

IntroductionSecondary gain was defined in terms of interpersonal, and social advantage achieved by patient as outcome of the illness. It helps the Ego to incorporate the symptom(s). Secundary gain is unconcious mechanism. It shouldn’t be mixed up with malingering or factitious disorder.Case report45 years old male patient was hospitalized at a Clinic of Psychiatry in Tuzla complaining the memory inpairment, confusion, and weakness of right arm and leg. The patient had been hospitalized at the Clinic of Neurology few months prior to actual hospitalisation. Diagnostic procedures, including MRI of the brain were done. Despite of negative tests results the diagnosis of ischemic stroke was made. During clicical observation the diagnosis of Dissociative motor disorder was postulated.ConclusionThe dissociative disorders is common in clinical practice. Existence of strong secondary gain may lead to chronicity and working and social dysfunction.


2021 ◽  
Vol 15 ◽  
Author(s):  
Rushi Zou ◽  
Linling Li ◽  
Li Zhang ◽  
Gan Huang ◽  
Zhen Liang ◽  
...  

Pain sensitivity is highly variable among individuals, and it is clinically important to predict an individual’s pain sensitivity for individualized diagnosis and management of pain. Literature has shown that pain sensitivity is associated with regional structural features of the brain, but it remains unclear whether pain sensitivity is also related to structural brain connectivity. In the present study, we investigated the relationship between pain thresholds and morphological connectivity (MC) inferred from structural MRI based on data of 221 healthy participants. We found that MC was highly predictive of an individual’s pain thresholds and, importantly, it had a better prediction performance than regional structural features. We also identified a number of most predictive MC features and confirmed the crucial role of the prefrontal cortex in the determination of pain sensitivity. These results suggest the potential of using structural MRI-based MC to predict an individual’s pain sensitivity in clinical settings, and hence this study has important implications for diagnosis and treatment of pain.


2021 ◽  
Vol 12 ◽  
Author(s):  
Tingjun Chen ◽  
Dale B. Bosco ◽  
Yanlu Ying ◽  
Dai-Shi Tian ◽  
Long-Jun Wu

Neuromyelitis optica (NMO) is an autoantibody-triggered neuro-inflammatory disease which preferentially attacks the spinal cord and optic nerve. Its defining autoantibody is specific for the water channel protein, aquaporin‐4 (AQP4), which primarily is localized at the end-feet of astrocytes. Histopathology studies of early NMO lesions demonstrated prominent activation of microglia, the resident immune sentinels of the central nervous system (CNS). Significant microglial reactivity is also observed in NMO animal models induced by introducing AQP4-IgG into the CNS. Here we review the potential roles for microglial activation in human NMO patients as well as different animal models of NMO. We will focus primarily on the molecular mechanisms underlying microglial function and microglia-astrocyte interaction in NMO pathogenesis. Understanding the role of microglia in NMO pathology may yield novel therapeutic approaches for this disease.


2016 ◽  
Vol 9 (3) ◽  
Author(s):  
Munir Saeed ◽  
Mahmood Ayyaz ◽  
Muhammad Arshad Cheema

Patients with an obscure and unrelievable abdominal condition may be forced to receive open laparotomy for diagnosis. Diagnostic Laparoscopy has been suggested as an alternative to diagnostic laparotomy in selected cases. It is a prospective descriptive study in which, thirty one patients under-going diagnostic Laparoscopy using conventional pneumoperitoneal techniques from January 2000 to June 2002 were included in this study. The correlation between preoperative diagnosis. laparoscopic diagnosis and pathologic diagnosis as well as the outcome of laparoscopic diagnosis and treatment have been assessed. Major indications for diagnostic Laparoscopy included acute abdominal pain (n=9). chronic abdominal pain (n=13), different intraabdominal tumor (n=7). All patients with acute abdominal conditions. 8 of the 13 patients with chronic abdominal pain and 4 of the 7 patients with abdominal tumors had no reasons for a further exploratory procedure, thus preventing the morbidity or mortality which might occur after unnecessary laparotomy. Diagnostic Laparoscopy benefits patients by avoiding unnecessary surgery. avoiding unnecessary delay in diagnosis and treatment and shortening the operative and hospitalized period. However. it provides only an alternative not a substitute for traditional diagnostic procedures and will never lessen the importance of conventional laparotomy.


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