scholarly journals A Case of Stiffperson Syndrome

2021 ◽  
Vol 3 (3) ◽  
pp. 52-54
Author(s):  
Mamdouh H. Kalakatawi ◽  
Nusrat Aziz ◽  
Norhan A. K. Mostafa
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Neurological Disorders ◽  
Clinical Manifestations ◽  
Ivig Therapy ◽  
Acid Decarboxylase ◽  
Medical Doctors ◽  
Paraspinal Muscles ◽  
History Of

Stiffperson syndrome (SPS) is one of the rarest neurological disorders. It is an autoimmune disease with antibodies against glutamic acid decarboxylase - enzyme needed to produce neurotransmitter GABA. This disease is very debilitating and fatal if not managed in time. A 32-year-old man presented with stiffness of abdominal and thoracolumbar paraspinal muscles first noticed 10 months back. There was history of jerkiness of hands. After excluding other mimicking conditions like multiple sclerosis, parkinsonism and axial dystonia, a diagnosis of Stiffperson syndrome was made. Treatment with IVIG therapy along with anti-spasticity drugs resulted in improved patient outcome. SPS is potentially treatable though cannot be cured. Not one treatment fits all patients with SPS and may have to be tailored according to the clinical manifestations. Increasing awareness in medical doctors and identifying the disease early can help the patients escape the disabling effects of SPS and improve the quality of life.

The influence of cognitive impairment on health-related quality of life in neurological disease

2010 ◽  
Vol 22 (1) ◽  
pp. 2-13 ◽  
Author(s):  
Alex J. Mitchell ◽  
Steven Kemp ◽  
Julián Benito-León ◽  
Markus Reuber
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Cognitive Impairment ◽  
Cognitive Deficits ◽  
Neurological Disorders ◽  
Neurological Disease ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related

Mitchell AJ, Kemp S, Benito-León J, Reuber M. The influence of cognitive impairment on health-related quality of life in neurological disease.Background:Cognitive impairment is the most consistent neurological complication of acquired and degenerative brain disorders. Historically, most focus was on dementia but now has been broadened to include the important construct of mild cognitive impairment.Methods:Systematic search and review of articles linked quality of life (QoL) and cognitive complications of neurological disorders. We excluded QoL in dementia.Results:Our search identified 249 publications. Most research examined patients with brain tumours, stroke, epilepsy, head injury, Huntington's disease, motor neuron disease, multiple sclerosis and Parkinson's disease. Results suggested that the majority of patients with epilepsy, motor neuron disease, multiple sclerosis, Parkinson's disease, stroke and head injury have subtle cognitive deficits early in their disease course. These cognitive complaints are often overlooked by clinicians. In many cases, the cognitive impairment is progressive but it can also be relapsing-remitting and in some cases reversible. Despite the importance of severe cognitive impairment in the form of dementia, there is now increasing recognition of a broad spectrum of impairment, including those with subclinical or mild cognitive impairment. Even mild cognitive difficulties can have functional and psychiatric consequences–especially when they are persistent and untreated. Specific cognitive deficits such an inattention, dysexecutive function and processing speed may affect a number of quality of life (QoL) domains. For example, cognitive impairment influences return to work, interpersonal relationships and leisure activities. In addition, fear of future cognitive decline may also impact upon QoL.Conclusions:We recommend further development of simple tools to screen for cognitive impairments in each neurological condition. We also recommend that a thorough cognitive assessment should be a part of routine clinical practice in those caring for individuals with neurological disorders.

scholarly journals Systemic mastocytosis: variable manifestations can lead to a challenging diagnostic process

2019 ◽  
Vol 12 (8) ◽  
pp. e229967 ◽  
Author(s):  
Susanna Nallamilli ◽  
Aideen O’Neill ◽  
Andrew Wilson ◽  
Mallika Sekhar ◽  
Jonathan Lambert
Keyword(s):  
Quality Of Life ◽  
Bone Marrow ◽  
Cell Clone ◽  
Systemic Mastocytosis ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Diagnostic Process ◽  
Histamine Antagonists ◽  
History Of

Systemic mastocytosis results from proliferation and activation of an abnormal mast cell clone. It is a heterogeneous disorder with clinical manifestations ranging from skin lesions alone to aggressive multi-organ infiltration and decreased survival. Given these varied manifestations, diagnosis can be difficult. We describe the case of a woman who presented with rash and diarrhoea and had a history of anaphylactic reactions. Over a protracted period, the patient’s symptoms were investigated by a number of specialties including gastroenterology, dermatology, immunology and haematology. Morphological, immunohistochemical and molecular analysis of bone marrow samples ultimately led to a diagnosis of systemic mastocytosis. Management with leukotriene and histamine antagonists resulted in significant improvement in symptoms and quality of life. The case serves to highlight the protean manifestations of systemic mastocytosis, the tests available to diagnose it and the agents available to treat it.

scholarly journals Correlation between quality of life indicators, peculiarities of diet and clinical manifestations of multiple sclerosis

2000 ◽  
Vol XXXII (3-4) ◽  
pp. 17-22
Author(s):  
M. Yu. Tatarinova ◽  
N. F. Smirnova ◽  
A. N. Boyko
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Clinical Manifestations ◽  
Scientific Work ◽  
Dietary Factors ◽  
Common Condition ◽  
Social Functions ◽  
Quality Of Life Indicators ◽  
Physical Functions

Multiple sclerosis is one of the most important problems of neurology. Onset age of disease is young, and young people, which are able to work often become invalids. That is why multiple sclerosis is medical and social problem. Questions of epidemiology and quality of life of our patients with multiple sclerosis are modern questions of multiple sclerosis problem. Our scientific work is devoted to relation between indicators of quality of life and dietary factors, clinical peculiarities of disease. We received interesting results. We found, that among males most important for quality of life are physical functions and common condition of health. And emotial problems, reduction of social functions, reduction of vitality are most important for quality of life among females.

Treatment of Multiple Sclerosis

2021 ◽  
pp. 548-554
Author(s):  
Orhun H. Kantarci
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Complete Recovery ◽  
Disease Course ◽  
Progressive Neurodegeneration ◽  
History Of ◽  
Level 3 ◽  
Active Disease

A comprehensive and ideal approach to treatment of multiple sclerosis (MS) has 5 levels of intervention. They are 1) prevention of active disease (clinical or subclinical relapses) in the first place, to prevent cumulative relapse-associated disability burden; 2) shortening the duration of active inflammatory demyelination associated with relapses, to limit the extent of irreversible central nervous system injury; 3) induction of more complete recovery after a relapse, to prevent or delay an ensuing chronic progressive neurodegeneration; 4) slowing the progressive phase of MS after progression ensues; and 5) treatment of symptoms and complications. The ultimate combined goal of these intervention levels is to alter the natural history of MS and prevent progression responsible for the main quantity of disability in MS, thereby improving the quality of life for patients with MS. Currently, treating physicians can accomplish levels 1, 2, and 5. Level 3 and level 4 interventions are yet to be developed sufficiently to affect the disease course meaningfully.

scholarly journals Fatigue and depression in multiple sclerosis: Correlation with quality of life

2011 ◽  
Vol 63 (3) ◽  
pp. 617-622 ◽  
Author(s):  
Svetlana Miletic ◽  
Gordana Toncev ◽  
Jasna Jevdjic ◽  
B. Jovanovic ◽  
D. Canovic
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Clinical Manifestations ◽  
Life Questionnaire ◽  
Relapsing Remitting ◽  
Related Quality ◽  
Health Related ◽  
Fatigue Severity ◽  
Severity Of Depression

The aim of this work was to examine the relationship between fatigue and depression, common features of multiple sclerosis (MS), and the quality of life (QOL). The study was comprised of 120 patients with clinical manifestations of definite MS. Relapsing-remitting MS was present in 76.7% patients and secondary progressive MS was present in 23.3% patients. Mean disease duration was 8.1 ? 5.6 years and the mean Expanded Disability Status Score (EDSS) was 3.5 ? 1.8 (range 1-8). Fatigue was measured with the Fatigue Severity Scale (FSS), depression was measured by the Beck Depression Inventory (BDI) and QOL was assessed using the health-related quality of life questionnaire SF-36. We observed that the global FSS score was 4.6 ? 1.8 (range 1-7) and BDI was 10.7 ? 10.3 (range 0-39). The FSS significantly and positively correlated with the BDI scores (r = 0.572; p = 0.000). The severity of fatigue had a significant impact on the quality of life (r = -0.743; p = 0.000), in particular on mental health (r = -0.749; p = 0.000). We observed a significant correlation between the severity of depression and impaired quality of life (r = -0.684; p = 0.000). This study shows that fatigue and depression are associated with impaired QOL in MS.

“Seeing” in NARCOMS: a look at vision-related quality of life in the NARCOMS registry

2012 ◽  
Vol 19 (7) ◽  
pp. 953-960 ◽  
Author(s):  
Amber R Salter ◽  
Tuula Tyry ◽  
Timothy Vollmer ◽  
Gary R Cutter ◽  
Ruth Ann Marrie
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Care Providers ◽  
Visual Aids ◽  
Eye Care ◽  
Visual Disability ◽  
Research Committee ◽  
Related Quality ◽  
History Of

Background: Research on vision-related quality of life (QOL) in multiple sclerosis (MS) is still limited. Tools such as the Visual Functioning Questionnaire-25 (VFQ-25) and the Vision Performance Scale (VPS) facilitate assessments of the severity of visual impairment and its impact on daily life. Objective: The objective of this paper is to examine vision-related QOL, comorbid eye conditions, use of visual aids and utilization of eye-care providers in the North American Research Committee on Multiple Sclerosis (NARCOMS) population, and to explore these issues in those with a history of optic neuritis (ON) and diplopia. Methods: In 2008, NARCOMS registrants reported on their use of visual aids, the VFQ-25, VPS, history of ON, diplopia, refractive error conditions (REC) and comorbid eye diseases (CED). We conducted regression analyses and correlations between select variables. Results: The response rate for the survey was 60.4%. Of the 9107 responders, 66.7% reported visual disability measured by VPS. Of respondents, 43.1% had a history of ON and 38.6% reported prior diplopia. Frequencies of myopia (51.8%), hyperopia (26.6%), and uveitis (3.4%) exceeded those expected for the general population. Mean (SD) VFQ-25 composite score was 82.0 (14.2). A history of ON or diplopia accounted for 9.7% of the variance in the VFQ-25; 90.6% of respondents used glasses or contact lenses. Rates of utilizations of eye-care providers were lower than expected. Conclusion: Prior ON, diplopia, REC and CED adversely impact vision-related QOL in MS. Increased utilization of eye-care providers and use of visual aids could improve vision-related QOL in people with MS.

Heroin-Based Treatment

2002 ◽  
Vol 32 (2) ◽  
pp. 523-542 ◽  
Author(s):  
Ingo Ilja Michels
Keyword(s):  
Quality Of Life ◽  
Maintenance Treatment ◽  
Social Problems ◽  
Practical Experience ◽  
Medical Doctors ◽  
The Social ◽  
Long Time ◽  
History Of ◽  
Definition Of

This article describes the history of the heated legal controversy in Germany about prescribing medicinal narcotics. The professional corporations of medical doctors have claimed a right to govern the interpretation and definition of legal terms and for a long time resisted the clinical insights and practical experience about maintenance treatment that had been gathered from abroad. The next logical step was to prescribe heroin – also formerly a licensed medication – in cases where methadone was not accepted or physically tolerated by patients. Slowly the social and political taboos against providing narcotic maintenance to addicts have been removed. The author addresses the question of whether prescribing heroin to addicts can reduce health and social problems and improve the client's quality of life.

scholarly journals Impact of natalizumab on quality of life in a real-world cohort of patients with multiple sclerosis: Results from MS PATHS

2021 ◽  
Vol 7 (2) ◽  
pp. 205521732110046
Author(s):  
Carrie M Hersh ◽  
Bernd Kieseier ◽  
Carl de Moor ◽  
Deborah M Miller ◽  
Denise Campagnolo ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Neurological Disorders ◽  
Well Being ◽  
Social Health ◽  
The Difference ◽  
Expectation Bias ◽  
The Impact ◽  
Multiple Sclerosis Treatment

Background Optimizing multiple sclerosis treatment warrants understanding of changes in physical, mental, and social health. Objective To assess the impact of natalizumab on Quality of Life in Neurological Disorders (Neuro-QoL) scores. Methods Annualized change in T-scores and likelihood of ≥5-point improvement over baseline were calculated for each Neuro-QoL domain after natalizumab initiation. Comparisons with ocrelizumab-treated patients were conducted after propensity score weighting and adjustment for relevant co-medications, year, and drug-year interaction. Results Among 164 natalizumab patients analyzed, 8 of 12 Neuro-QoL domains improved significantly, with greater improvement in patients with abnormal baseline Neuro-QoL. In the subgroup comparison of natalizumab-treated ( n = 145) and ocrelizumab-treated ( n = 520) patients, significant improvement occurred in 9 of 12 and 4 of 12 domains, respectively. The difference between groups was statistically significant for positive affect and well-being ( p = 0.02), sleep ( p = 0.003), and satisfaction with social roles and activities (SRA) ( p = 0.03) in the overall population and for emotional and behavioral dyscontrol ( p = 0.01), participation in SRA ( p = 0.0001), and satisfaction with SRA ( p = 0.02) in patients with abnormal baseline Neuro-QoL. Conclusions Natalizumab can produce clinically meaningful improvements in mental and social health. Such improvements are unlikely to be primarily driven by expectation bias, as their magnitude exceeded improvements with another high-efficacy therapy, ocrelizumab.

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