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Neurology Dec 2023Multiple system atrophy (MSA) is a multisystem neurodegenerative disorder affecting adults older than 30 years and presenting with a constellation of symptoms, including...
Multiple system atrophy (MSA) is a multisystem neurodegenerative disorder affecting adults older than 30 years and presenting with a constellation of symptoms, including parkinsonian features, ataxia, and autonomic disturbances. The pathophysiology of MSA has gradually been unveiled. It is characterized by α-synuclein protein aggregates in neurons and glial cells that are different from those seen in Parkinson disease (PD). MSA is the most common condition, after PD, that has parkinsonism as a cardinal feature, and it is also one of the most common causes of sporadic cerebellar ataxias. Because the clinical presentation is heterogeneous, we as clinicians must always be certain of the probability of an MSA diagnosis for each patient we are facing.
Topics: Adult; Humans; Multiple System Atrophy; Parkinson Disease; Ataxia; Atrophy
PubMed: 37857491
DOI: 10.1212/WNL.0000000000208066 -
JAAPA : Official Journal of the... Aug 2023Complex regional pain syndrome (CRPS), formerly called reflex sympathetic dystrophy (RSD), is a chronic pain phenomenon associated with an alteration in peripheral and...
Complex regional pain syndrome (CRPS), formerly called reflex sympathetic dystrophy (RSD), is a chronic pain phenomenon associated with an alteration in peripheral and central pain perception in a localized body region. Because of the many risk factors associated with this phenomenon, the true nature of the disease risk and clinical course are a challenge to predict. After the diagnosis is confirmed and treatment is provided promptly, clinicians must consider patient health and function holistically to foster improvement in overall quality of life.
Topics: Humans; Quality of Life; Complex Regional Pain Syndromes; Reflex Sympathetic Dystrophy
PubMed: 37493995
DOI: 10.1097/01.JAA.0000931440.10463.f2 -
Revue Neurologique 2024Pure autonomic failure (PAF) is a neurodegenerative disease affecting the sympathetic component of the autonomic nervous system and presenting as orthostatic hypotension... (Review)
Review
Pure autonomic failure (PAF) is a neurodegenerative disease affecting the sympathetic component of the autonomic nervous system and presenting as orthostatic hypotension (OH). It is a rare, sporadic disease of adults. Although OH is the primary symptom, the autonomic dysfunction may be more generalised, leading to genitourinary and intestinal dysfunction and sweating disorders. Autonomic symptoms in PAF may be similar to those observed in other autonomic neuropathies that need to be ruled out. PAF belongs to the group of α synucleinopathies and is characterised by predominant peripheral deposition of α-synuclein in autonomic ganglia and nerves. However, in a significant number of cases, PAF may convert into another synucleinopathy with central nervous system involvement with varying prognosis: Parkinson's disease (PD), multiple system atrophy (MSA), or dementia with Lewy bodies (DLB). The clinical features, the main differential diagnoses, the risk factors for "phenoconversion" to another synucleinopathy as well as an overview of treatment will be discussed.
Topics: Adult; Humans; Pure Autonomic Failure; Synucleinopathies; Parkinson Disease; Multiple System Atrophy; Lewy Body Disease; Autonomic Nervous System Diseases
PubMed: 38129276
DOI: 10.1016/j.neurol.2023.11.003 -
Handbook of Clinical Neurology 2024A number of the well-recognized autoimmune and paraneoplastic neurologic syndromes commonly involve the autonomic nervous system. In some cases, the autonomic nerves or... (Review)
Review
A number of the well-recognized autoimmune and paraneoplastic neurologic syndromes commonly involve the autonomic nervous system. In some cases, the autonomic nerves or ganglia are primary targets of neurologic autoimmunity, as in immune-mediated autonomic ganglionopathies. In other disorders such as encephalitis, autonomic centers in the brain may be affected. The presence of autonomic dysfunction (especially gastrointestinal dysmotility) is sometimes overlooked even though this may contribute significantly to the symptom burden in these paraneoplastic disorders. Additionally, recognition of autonomic features as part of the clinical syndrome can help point the diagnostic evaluation toward autoimmune and paraneoplastic etiologies. As with other paraneoplastic disorders, the clinical syndrome and the presence and type of neurologic autoantibodies help to secure the diagnosis and direct the most appropriate investigation for malignancy. Optimal management for these conditions typically includes aggressive treatment of the neoplasm, immunomodulatory therapy, and symptomatic treatments for orthostatic hypotension and gastrointestinal dysmotility.
Topics: Humans; Autoantibodies; Nervous System Diseases; Autonomic Nervous System Diseases; Paraneoplastic Syndromes, Nervous System; Neoplasms; Autonomic Nervous System
PubMed: 38494282
DOI: 10.1016/B978-0-12-823912-4.00005-0 -
Revue Medicale Suisse Feb 2024
Topics: Humans; Accidental Falls; Hypotension, Orthostatic
PubMed: 38380665
DOI: 10.53738/REVMED.2024.20.862.414 -
The Journal of Spinal Cord Medicine Jul 2024
Topics: Humans; Hypotension, Orthostatic; Autonomic Dysreflexia; Exercise Therapy; Exercise
PubMed: 38647374
DOI: 10.1080/10790268.2024.2340818 -
Progress in Cardiovascular Diseases 2023With expanding commercial space programs, uncertainty remains about the cardiovascular effects of space environmental exposures including microgravity, confinement,... (Review)
Review
BACKGROUND
With expanding commercial space programs, uncertainty remains about the cardiovascular effects of space environmental exposures including microgravity, confinement, isolation, space radiation, and altered bacterial virulence. Current limited data suggests additional health threats compared to Earth.
METHODS
We systematically reviewed PubMed, CENTRAL, Web of Science, EMBASE and Cochrane databases for prospective studies on spaceflight and cardiovascular outcomes. Search terms combined cardiovascular disease topics with spaceflight concepts. No date or language restrictions were imposed.
RESULTS
35 studies representing 2696 space travelers met inclusion criteria. Studies were grouped into spaceflight associations with: atherosclerosis, mortality, cardiac function, orthostatic intolerance, and arrhythmias. Atherosclerosis evidence was limited, with animal studies linking space radiation to endothelial damage, oxidative stress, and inflammation. However, human data showed no significantly increased atherosclerotic disease in astronauts. Mortality studies demonstrated lower cardiovascular mortality in astronauts compared to the general population however there was conflicting data. Cardiac function studies revealed physiologic ventricular atrophy, increased arterial stiffness, and altered blood flow distribution attributed to microgravity exposure. Effects appeared transient and reversible post-flight. Orthostatic intolerance studies found astronauts experienced altered heart rate variability, baroreflex response, and blood pressure changes post-flight. Arrhythmia studies showed increased ventricular ectopy during spaceflight, but limited data on long term flights.
CONCLUSIONS
Environmental space hazards impact the cardiovascular system through multiple mechanisms. Microgravity causes cardiac atrophy and orthostatic intolerance while space radiation may potentially accelerate atherosclerosis. Further research is needed, especially regarding long-term spaceflights.
Topics: Humans; Cardiovascular Diseases; Orthostatic Intolerance; Prospective Studies; Space Flight; Hemodynamics; Arrhythmias, Cardiac; Atherosclerosis; Atrophy
PubMed: 37531984
DOI: 10.1016/j.pcad.2023.07.009 -
Neurological Sciences : Official... Sep 2023Although dysautonomia is a well-recognized complication of acute demyelinating polyradiculoneuropathy, it is rarely reported and evaluated in chronic demyelinating... (Review)
Review
BACKGROUND AND AIMS
Although dysautonomia is a well-recognized complication of acute demyelinating polyradiculoneuropathy, it is rarely reported and evaluated in chronic demyelinating neuropathies. The purpose of this review is to search and synthesize the current literature on the prevalence and type of autonomic dysfunction (AD) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
METHODS
PubMed and Web of Science were searched for studies reporting AD in CIDP.
RESULTS
Twelve studies, including 346 patients with CIDP, were found eligible for the review. Seven studies used autonomic tests only as an additional component of the comprehensive clinical evaluation, and found that dysautonomia in CIDP may indicate the presence of a comorbid disease (e.g., diabetes) and facilitate the differentiation of CIDP from other neuropathies (e.g., amyloid neuropathy). Five studies performed quantitative assessment of autonomic function in CIDP as a primary goal. Two studies have used the Composite Autonomic Severity Score (CASS) to assess severity and distribution of dysautonomia. The reported prevalence of dysautonomia in CIDP during quantitative assessment of autonomic function ranged from 25 to 89%, depending on the battery of tests used, with CASS not exceeding 4 points. The abnormalities in autonomic tests indicated both sympathetic and parasympathetic dysfunction and did not correlate with the duration, severity and variant of CIDP.
CONCLUSIONS
Clinical or subclinical involvement of the ANS has been shown to be common and relatively mild in CIDP. The impact of autonomic impairment on disability and of its possible response to therapy in CIDP needs to be further investigated.
Topics: Humans; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Autonomic Nervous System; Diabetes Mellitus; Primary Dysautonomias
PubMed: 37083958
DOI: 10.1007/s10072-023-06802-z -
Journal of Neurology Nov 2023Multiple system atrophy (MSA) is a sporadic, fatal, and rapidly progressive neurodegenerative disease of unknown etiology that is clinically characterized by autonomic... (Review)
Review
Multiple system atrophy (MSA) is a sporadic, fatal, and rapidly progressive neurodegenerative disease of unknown etiology that is clinically characterized by autonomic failure, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. Early onset and extensive autonomic dysfunction, including cardiovascular dysfunction characterized by orthostatic hypotension (OH) and supine hypertension, urinary dysfunction characterized by overactive bladder and incomplete bladder emptying, sexual dysfunction characterized by sexual desire deficiency and erectile dysfunction, and gastrointestinal dysfunction characterized by delayed gastric emptying and constipation, are the main features of MSA. Autonomic dysfunction greatly reduces quality of life and increases mortality. Therefore, early diagnosis and intervention are urgently needed to benefit MSA patients. In this review, we aim to discuss the systematic treatment of autonomic dysfunction in MSA, and focus on the current methods, starting from non-pharmacological methods, such as patient education, psychotherapy, diet change, surgery, and neuromodulation, to various drug treatments targeting autonomic nerve and its projection fibers. In addition, we also draw attention to the interactions among various treatments, and introduce novel methods proposed in recent years, such as gene therapy, stem cell therapy, and neural prosthesis implantation. Furthermore, we elaborate on the specific targets and mechanisms of action of various drugs. We would like to call for large-scale research to determine the efficacy of these methods in the future. Finally, we point out that studies on the pathogenesis of MSA and pathophysiological mechanisms of various autonomic dysfunction would also contribute to the development of new promising treatments and concepts.
Topics: Male; Humans; Multiple System Atrophy; Quality of Life; Autonomic Nervous System Diseases; Erectile Dysfunction; Parkinsonian Disorders
PubMed: 37477834
DOI: 10.1007/s00415-023-11876-y -
Revista Portuguesa de Cardiologia :... Sep 2023
Topics: Humans; Syncope; Reflex; Syncope, Vasovagal
PubMed: 37023980
DOI: 10.1016/j.repc.2023.04.001