Quality of life in patients with polyneuropathy associated with different types of monoclonal gammopathy of undetermined significance

AbstractPolyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS-PNP) has a chronic and slowly progressive course but can lead to significant disability and reduced quality of life (QoL). The aim of this study was to analyze QoL in MGUS-PNP patients and to determine its predictors. Our study included 51 patients diagnosed with MGUS-PNP (23.5% with IgM, 66.7% IgG or IgA, 7.8% undetermined paraprotein, 2.0% light chains). QoL was assessed using the SF-36 questionnaire. The Medical Research Council Sum Score (MRC-SS), INCAT disability and sensory scores, ataxia score, Krupp ’s Fatigue Severity Scale and Beck’s Depression Inventory were also used. Total SF-36 score was 50.0 ± 21.4 and no difference was observed between IgM and IgG/IgA MGUS-PNP. Physical composite score was worse than mental (44.4 ± 21.4 vs. 54.5 ± 20.9). Following factors showed corre lation with SF-36 total score in univariate analysis: INCAT disability score, MRC-SS, INCAT sensory score, level of ataxia, fatigue and depression (p 
Source: Acta Neurologica Belgica - Category: Neurology Source Type: research

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Albert Sanfeliu1, Karsten Hokamp2, Michael Gill1 and Daniela Tropea1,3*1Neuropsychiatric Genetics, Department of Psychiatry, School of Medicine, Trinity Translational Medicine Institute, St James Hospital, Dublin, Ireland2Department of Genetics, School of Genetics and Microbiology, Smurfit Institute of Genetics, Trinity College Dublin, Dublin, Ireland3Department of Psychiatry, School of Medicine, Trinity College Institute for Neuroscience, Trinity College Dublin, Dublin, IrelandRett syndrome is a rare neuropsychiatric disorder with a wide symptomatology including impaired communication and movement, cardio-respiratory abno...
Source: Frontiers in Psychiatry - Category: Psychiatry Source Type: research
ConclusionsDifferent patterns of sleep disturbances are observed in HD patients: insomnia, difficulties in falling asleep, frequent nocturnal awakenings, and excessive daytime sleepiness are the most common sleep problems reported by patients with HD. In several HD studies, specific changes in sleep architecture and in circadian melatonin secretion were identified in laboratory testing.Sleep disorders in HD have diverse and complex determinants, the most significant of which includes damage to brain areas that are responsible for the proper sleep pattern and circadian rhythm regulation. Sleep and circadian rhythm disorders...
Source: Frontiers in Psychiatry - Category: Psychiatry Source Type: research
The results indicate that MSA ‐P patients have more severe motor impairment, hyposmia, depression, anxiety, cognitive impairment, and lower HrQoL than MSA‐C patients. Depression, anxiety, degree of disability, and disease severity are predictors of poor HrQoL among Chinese patients with MSA. AbstractObjectivesMultiple system atrophy (MSA) is a progressive neurodegenerative disorder that causes early sustained disability and poor health ‐related quality of life (HrQoL). The clinical features and their effects on the HrQoL of patients in China have received little attention in the research literature. We evaluated the ...
Source: Brain and Behavior - Category: Neurology Authors: Tags: ORIGINAL RESEARCH Source Type: research
Conclusions: The extremely common depression results from motor disability caused by ataxia; it also affects the disease severity of SCA3. These findings suggested that depression was a part of neurodegeneration in SCA3 and necessitated intensive focus and interventions while caring for SCA3 patients.Eur Neurol 2018;79:266 –271
Source: European Neurology - Category: Neurology Source Type: research
Abstract A decade after the designation of MED13L as a gene and its link to intellectual disability (ID) and dextro-looped transposition of great arteries in 2003, we previously described a recognizable syndrome due to MED13L haploinsufficiency. Subsequent reports of 22 further patients diagnosed by genome-wide testing further delineated the syndrome with expansion of the phenotypic spectrum and showed reduced penetrance for congenital heart defects. We now report two novel patients identified by whole exome sequencing, one with a de novo MED13L truncating mutation and the other with a de novo missense mutation. T...
Source: European Journal of Medical Genetics - Category: Genetics & Stem Cells Authors: Tags: Eur J Med Genet Source Type: research
Objective:To investigate predictors of adherence to wearing the Fitbit activity monitor in patients with Parkinson disease (PD) and stroke.Background:Exercise and activity improve symptoms of PD and stroke, and aid in primary and secondary stroke prevention. However, adherence to physical activity programs is low and little is known about adherence to daily activity monitoring.Design/Methods:Patients (n=19, age=58.8+/–11, range 37–83Y) with PD (n=12) and stroke (n=8) participated in a pilot study to investigate feasibility and adherence of Fitbit monitoring to track activity between clinical office visits.Resul...
Source: Neurology - Category: Neurology Authors: Tags: Movement Disorders: Tics, Tremor & amp;amp; Ataxia II Source Type: research
Discussion Concussion as defined by the International Conference on Concussion in Sport in 2012 is “Concussion is a brain injury and is defined as a complex pathophysiological process affecting the brain, induced by biomechanical forces.” It results in quick onset of signs and symptoms of physical and cognitive impairment. Concussion is sometimes referred to as mild traumatic brain injury (TBI) as mild TBI refers to “…concussions that are generally not life threatening despite the potential for short-term disability and serious ongoing sequelae.” Concussion symptoms are usually categorized as...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
CONCLUSIONS: Distinct profiles in the cognitive and motor domains between these movement disorders may guide practitioners in the differential diagnosis process and ultimately lead to better medical management of FXTAS patients. PMID: 27414076 [PubMed - in process]
Source: The Clinical Neuropsychologist - Category: Psychiatry & Psychology Authors: Tags: Clin Neuropsychol Source Type: research
Abstract Introduction Alpha-1,3-glucosyltransferase congenital disorder of glycosylation (ALG6-CDG) is a congenital disorder of glycosylation. The original patients were described with hypotonia, developmental disability, epilepsy, and increased bleeding tendency. Methods Based on Euroglycan database registration, we approached referring clinicians and collected comprehensive data on 41 patients. Results We found hypotonia and developmental delay in all ALG6-CDG patients and epilepsy, ataxia, proximal muscle weakne...
Source: Journal of Inherited Metabolic Disease - Category: Internal Medicine Source Type: research
Conclusions: This cohort illustrates the expanding phenotypic and genotypic variability of ATP1A3 mutations even within the RDP diagnosis suggesting that systematic testing of a wider clinical spectrum of patients is warranted.Disclosure: Dr. Meijer has nothing to disclose. Dr. Lubarr has nothing to disclose. Dr. Greene has nothing to disclose. Dr. Frucht has received personal compensation for activities with Merz Pharmaceuticals as a consultant. Dr. Raymond has nothing to disclose. Dr. Severt has received personal compensation for activities with Teva, Allergan, Impax, and Lundbeck. Dr. Shanker has nothing to disclose. Dr...
Source: Neurology - Category: Neurology Authors: Tags: Movement Disorders: Ataxia and Dystonia Source Type: research
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