Paraproteinaemic neuropathy: MGUS and beyond

2021 ◽  
pp. practneurol-2020-002837
Author(s):  
Antonia S Carroll ◽  
Michael P T Lunn

Paraproteinaemic neuropathies comprise a heterogeneous group of neuro-haematological conditions with some distinct neurological, haematological and systemic phenotypes. The spectrum of disease varies from mild to severe, indolent to rapidly progressive and from small fibre sensory involvement to dramatic sensorimotor deficits. The haematological association may be overlooked, resulting in delayed treatment, disability, impaired quality of life and increased mortality. However, the presence of an irrelevant benign paraprotein can sometimes lead to inappropriate treatment. In this review, we outline our practical approach to paraproteinaemic disorders, discuss the utility and limitations of diagnostic tests and the distinctive clinical phenotypes and touch on the complex multidisciplinary management approaches.

2006 ◽  
Vol 175 (4S) ◽  
pp. 410-411
Author(s):  
Germar M. Pinggera ◽  
Michael Mitterberger ◽  
Leo Pallwein ◽  
Peter Rehder ◽  
Ferdinand Frauscher ◽  
...  

2019 ◽  
Vol 09 (03) ◽  
Author(s):  
Ben Hadj Ali Emna ◽  
Bouker Ahmed ◽  
Guiga Ahmed ◽  
Ben Yahia Wissal ◽  
Atig Amira ◽  
...  

Cancers ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 450
Author(s):  
Peter Baumgarten ◽  
Mana Sarlak ◽  
Daniel Monden ◽  
Andrea Spyrantis ◽  
Simon Bernatz ◽  
...  

Seizures are among the most common symptoms of meningioma. This retrospective study sought to identify risk factors for early and late seizures in meningioma patients and to evaluate a modified STAMPE2 score. In 556 patients who underwent meningioma surgery, we correlated different risk factors with the occurrence of postoperative seizures. A modified STAMPE2 score was applied. Risk factors for preoperative seizures were edema (p = 0.039) and temporal location (p = 0.038). For postoperative seizures preoperative tumor size (p < 0.001), sensomotory deficit (p = 0.004) and sphenoid wing location (p = 0.032) were independent risk factors. In terms of postoperative status epilepticus; sphenoid wing location (p = 0.022), tumor volume (p = 0.045) and preoperative seizures (p < 0.001) were independent risk factors. Postoperative seizures lead to a KPS deterioration and thus an impaired quality of life (p < 0.001). Late seizures occurred in 43% of patients with postoperative seizures. The small sub-cohort of patients (2.7%) with a STAMPE2 score of more than six points had a significantly increased risk for seizures (p < 0.001, total risk 70%). We concluded that besides distinct risk factors, high scores of the modified STAMPE2 score could estimate the risk of postoperative seizures. However, it seems not transferable to our cohort


Children ◽  
2021 ◽  
Vol 8 (7) ◽  
pp. 535
Author(s):  
Seung Kim ◽  
Mireu Park ◽  
Eunyoung Kim ◽  
Ga Eun Kim ◽  
Jae Hwa Jung ◽  
...  

We share our experience on the implementation of a multidisciplinary aerodigestive program comprising an aerodigestive team (ADT) so as to evaluate its feasibility. We performed a retrospective chart review of the patients discussed at the monthly ADT meetings and analyzed the data. A total of 98 children were referred to the ADT during the study period. The number of cases increased steadily from 3.5 cases per month in 2019 to 8.5 cases per month in 2020. The median age of patients was 34.5 months, and 55% were male. Among the chronic comorbidities, neurologic disease was the most common (85%), followed by respiratory (36%) and cardiac (13%) disorders. The common reasons for consultation were suspected aspiration (56%), respiratory difficulty (44%), drooling/stertor (30%), regurgitation/vomiting (18%), and feeding/swallowing difficulty (17%). Following discussions, 58 patients received active interventions, including fundoplication, gastrostomy, laryngomicrosurgery, tracheostomy, and primary dilatation of the airway. According to the questionnaire of the caregiver, the majority agreed that the main symptoms and quality of life of patients had improved (88%), reducing the burden on caregivers (77%). Aerodigestive programs may provide comprehensive and multidisciplinary management for children with complex airway and digestive tract disorders.


2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Isaac Samir Wasfy ◽  
Enayat Mohamed Soltan ◽  
Hassan A. Abdelwahab ◽  
Hend Mikhail Salama

Abstract Background This study aims to assess the severity of lower urinary tract symptoms, and to assess predictors of impaired quality of life among Egyptian adults complain of lower urinary tract symptoms. Methods An observational cross-sectional research was done using an online anonymous poll survey. The survey was implemented through sharing on different social media applications. The survey was posted from June 1, 2020, to June 10, 2020. The overall communities of the Egyptian adults who satisfied the incorporation rules and consented to take an interest in the research were incorporated using convenience and snowball collecting methods (188 adults). A semi-structured questionnaire on socio-demographic characteristics and Arabic Urogenital Distress Inventory (UDI-6) and Incontinence Impact Questionnaire (IIQ-7) short forms were used. Results Approximately 220 Egyptian adults shared in the research but only 188 fulfilled inclusion and exclusion criteria. Approximately 92% of them had at least one symptom of lower urinary tract manifestations. Irritative symptoms presented in 65 (85.5%) of males and 102 (91.1%) of females. Stress symptoms presented in 44 (57.9%) of males and 63 (56.2%) of females with higher statistically significant mean of stress symptoms. Obstruction/discomfort symptoms presented in 51 (67.1%) of males, and 77 (68.8%) of females. Seeking help and duration of the urological problem were statistically significant independent positive predictors of UDI-6-total. The Irritative score, obstruction/discomfort score, and duration of the urological problem were statistically significant independent positive predictors of IIQ-7-total. Conclusions Urological problems are common and have an impact on the quality of life in various domains of physical activity, social relationships, travel, and emotional health.


2021 ◽  
Vol 10 (15) ◽  
pp. 3199
Author(s):  
Masaru Honma ◽  
Hiroyoshi Nozaki

Psoriasis is a chronic inflammatory skin disease induced by multifactorial causes and is characterized by bothersome, scaly reddish plaques, especially on frequently chafed body parts, such as extensor sites of the extremities. The latest advances in molecular-targeted therapies using biologics or small-molecule inhibitors help to sufficiently treat even the most severe psoriatic symptoms and the extra cutaneous comorbidities of psoriatic arthritis. The excellent clinical effects of these therapies provide a deeper understanding of the impaired quality of life caused by this disease and the detailed molecular mechanism in which the interleukin (IL)-23/IL-17 axis plays an essential role. To establish standardized therapeutic strategies, biomarkers that define deep remission are indispensable. Several molecules, such as cytokines, chemokines, antimicrobial peptides, and proteinase inhibitors, have been recognized as potent biomarker candidates. In particular, blood protein markers that are repeatedly measurable can be extremely useful in daily clinical practice. Herein, we summarize the molecular mechanism of psoriasis, and we describe the functions and induction mechanisms of these biomarker candidates.


2016 ◽  
Vol 196 ◽  
pp. 1310-1314 ◽  
Author(s):  
Laura Lorido ◽  
Sonia Ventanas ◽  
Tolga Akcan ◽  
Mario Estévez

2018 ◽  
Vol 44 (4) ◽  
pp. 292-298 ◽  
Author(s):  
Erica Nishida Hasimoto ◽  
Daniele Cristina Cataneo ◽  
Tarcísio Albertin dos Reis ◽  
Antonio José Maria Cataneo

ABSTRACT Objective: To determine the prevalence of primary hyperhidrosis in the city of Botucatu, Brazil, and to evaluate how this disorder affects the quality of life in those suffering from it. Methods: A population survey was conducted in order to identify cases of hyperhidrosis among residents in the urban area of the city, selected by systematic cluster sampling. In accordance with the census maps of the city, the sample size should be at least 4,033 participants. Ten interviewers applied a questionnaire that evaluated the presence of excessive sweating and invited the subjects who reported hyperhidrosis to be evaluated by a physician in order to confirm the diagnosis. Results: A total of 4,133 residents, in 1,351 households, were surveyed. Excessive sweating was reported by 85 residents (prevalence = 2.07%), of whom 51 (60%) were female. Of those 85 respondents, 51 (60%) agreed to undergo medical evaluation to confirm the diagnosis and only 23 (45%) were diagnosed with primary hyperhidrosis (prevalence = 0.93%). Of the 23 subjects diagnosed with primary hyperhidrosis, 11 (48%) reported poor or very poor quality of life. Conclusions: Although the prevalence of self-reported excessive sweating was greater than 2%, the actual prevalence of primary hyperhidrosis in our sample was 0.93% and nearly 50% of the respondents with primary hyperhidrosis reported impaired quality of life.


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