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International Journal of Molecular... Oct 2023Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, characterized in its typical presentation by a combination of lower and upper motor neuron symptoms,... (Review)
Review
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, characterized in its typical presentation by a combination of lower and upper motor neuron symptoms, with a progressive course and fatal outcome. Due to increased recognition of the non-motor symptoms, it is currently considered a multisystem disorder with great heterogeneity, regarding genetical, clinical, and neuropathological features. Often underestimated, autonomic signs and symptoms have been described in patients with ALS, and various method analyses have been used to assess autonomic nervous system involvement. The aim of this paper is to offer a narrative literature review on autonomic disturbances in ALS, based on the scarce data available to date.
Topics: Humans; Amyotrophic Lateral Sclerosis; Neurodegenerative Diseases; Motor Neurons; Primary Dysautonomias
PubMed: 37834374
DOI: 10.3390/ijms241914927 -
Physical Medicine and Rehabilitation... Aug 2023Persistence of symptoms beyond the initial acute phase of coronavirus disease-2019 (COVID-19) is termed postacute SARS-CoV-2 (PASC) and includes neurologic, autonomic,... (Review)
Review
Persistence of symptoms beyond the initial acute phase of coronavirus disease-2019 (COVID-19) is termed postacute SARS-CoV-2 (PASC) and includes neurologic, autonomic, pulmonary, cardiac, psychiatric, gastrointestinal, and functional impairment. PASC autonomic dysfunction can present with dizziness, tachycardia, sweating, headache, syncope, labile blood pressure, exercise intolerance, and "brain fog." A multidisciplinary team can help manage this complex syndrome with nonpharmacologic and pharmacologic interventions.
Topics: Humans; SARS-CoV-2; COVID-19; Autonomic Nervous System Diseases; Syncope; Syndrome
PubMed: 37419532
DOI: 10.1016/j.pmr.2023.04.003 -
Hypertension (Dallas, Tex. : 1979) Mar 2024Although orthostatic hypotension (OH) has long been recognized as a manifestation of autonomic dysfunction, a growing body of literature has identified OH as a common... (Review)
Review
Although orthostatic hypotension (OH) has long been recognized as a manifestation of autonomic dysfunction, a growing body of literature has identified OH as a common comorbidity of hypertension. This connection is complex, related to pathophysiology in blood pressure regulation and the manner by which OH is derived as the difference between 2 blood pressure measurements. While traditional therapeutic approaches to OH among patients with neurodegenerative disorders focus on increasing upright blood pressure to prevent cerebral hypoperfusion, the management of OH among patients with hypertension is more nuanced; resting hypertension is itself associated with adverse outcomes among these patients. Although there is substantial evidence that intensive blood pressure treatment does not cause OH in the majority of patients with essential hypertension, some classes of antihypertensive agents may unmask OH in patients with an underlying autonomic impairment. Practical steps to manage OH among adults with hypertension start with (1) a thorough characterization of its patterns, triggers, and cause; (2) review and removal of aggravating factors (often pharmacological agents not related to hypertension treatment); (3) optimization of an antihypertensive regimen; and (4) adoption of a tailored treatment strategy that avoids exacerbating hypertension. These strategies include countermaneuvers and short-acting vasoactive agents (midodrine, droxidopa). Ultimately, further research is needed on the epidemiology of OH, the impact of hypertension treatment on OH, approaches to the screening and diagnosis of OH, and OH treatment among adults with hypertension to improve the care of these patients and their complex blood pressure pathophysiology.
Topics: Adult; Humans; Hypotension, Orthostatic; American Heart Association; Hypertension; Midodrine; Blood Pressure; Antihypertensive Agents; Autonomic Nervous System Diseases
PubMed: 38205630
DOI: 10.1161/HYP.0000000000000236 -
Neurological Sciences : Official... Aug 2023A 56-year-old man presented to the clinic with episodic headaches for several years which had been worsening over a few months prior to the presentation. He described...
A 56-year-old man presented to the clinic with episodic headaches for several years which had been worsening over a few months prior to the presentation. He described headache as sharp, stabbing pain around the left eye associated with nausea, vomiting, photophobia, and phonophobia lasting for hours associated with flushing on the left side of the face. The picture of his face during these episodes showed flushing of the left side of the face, ptosis of the right eyelid, and miosis (panel A). Flushing in his face would resolve with the abortion of the headache. At the time of presentation to the clinic, his neurological exam was only significant for mild left eye ptosis and miosis (panels B and C). Extensive workup including MRI brain, cervical spine, thoracic spine, lumbar spine, CTA head and neck, and CT maxillofacial was unremarkable. He had tried several medications in the past including valproic acid, nortriptyline, and verapamil without significant benefit. He was started on erenumab for migraine prophylaxis and was given sumatriptan for abortive therapy following which his headaches improved. The patient was diagnosed with idiopathic left Horner's syndrome and his migraines with autonomic dysfunction would present with unilateral flushing opposite to the site of Horner's presenting as Harlequin syndrome [1, 2].
Topics: Male; Humans; Middle Aged; Autonomic Nervous System Diseases; Horner Syndrome; Miosis; Headache
PubMed: 37199876
DOI: 10.1007/s10072-023-06849-y -
Ugeskrift For Laeger Aug 2023Guillian-Barré syndrome (GBS) is an immune mediated disease which is most commonly caused by infections. Symptoms are rapidly progressive and may include servere...
Guillian-Barré syndrome (GBS) is an immune mediated disease which is most commonly caused by infections. Symptoms are rapidly progressive and may include servere weakness of the extremities, bulbar weakness, autonomic dysfunction and respiratory insufficiens. In rare cases paraneoplastic syndrome may mimic GBS, which is important to know as treatment will fail unless the underlying malignancy is treated. This is a case report of paraneoplastic non-small cell lungcancer mimicking GBS with effect of the checkpoint inhibitor pembrolizumab.
Topics: Humans; Paraneoplastic Syndromes; Autonomic Nervous System Diseases; Extremities
PubMed: 37615229
DOI: No ID Found -
Europace : European Pacing,... Aug 2023Over the last 25 years, the Europace journal has greatly contributed to dissemination of research and knowledge in the field of syncope. More than 400 manuscripts have...
Over the last 25 years, the Europace journal has greatly contributed to dissemination of research and knowledge in the field of syncope. More than 400 manuscripts have been published in the journal. They undoubtedly improved our understanding of syncope. This symptom is now clearly differentiated from other forms of transient loss of consciousness. The critical role of vasodepression and/or cardioinhibition as final mechanisms of reflex syncope is emphasized. Current diagnostic approach sharply separates between cardiac and autonomic pathways. Physiologic insights have been translated, through rigorously designed clinical trials, into non-pharmacological or pharmacological interventions and interventional therapies. The following manuscript is intended to give the reader the current state of the art of knowledge of syncope by highlighting landmark contributions of the Europace journal.
Topics: Humans; Syncope; Syncope, Vasovagal; Heart
PubMed: 37622579
DOI: 10.1093/europace/euad163 -
Primary Care Jun 2024Autonomic disorders can present with hypotension, gastrointestinal, genitourinary symptoms, and heat intolerance. Diabetes is the most common causes of autonomic...
Autonomic disorders can present with hypotension, gastrointestinal, genitourinary symptoms, and heat intolerance. Diabetes is the most common causes of autonomic failure, and management should focus on glucose control to prevent developing autonomic symptoms. The most prevalent cause of dysautonomia, or autonomic dysfunction, is Postural Orthostatic Tachycardia Syndrome (POTS). Autonomic testing characterizes causes for nonspecific symptoms but is not necessary in patients with classic presentations. Treatment for autonomic dysfunction and failure focus on discontinuing offending medications, behavioral modification, and pharmacologic therapy to decrease symptom severity. Autonomic failure has no cure; therefore, the focus remains on improving quality of life.
Topics: Humans; Autonomic Nervous System Diseases; Postural Orthostatic Tachycardia Syndrome; Primary Health Care; Quality of Life
PubMed: 38692780
DOI: 10.1016/j.pop.2024.02.006 -
Scientific Reports Sep 2023Autonomic symptoms (AS) are critical in Parkinson's disease (PD). We aimed to determine the relative significance of clinical factors allowing predictions about...
Autonomic symptoms (AS) are critical in Parkinson's disease (PD). We aimed to determine the relative significance of clinical factors allowing predictions about incidence of AS, and examine AS profiles among PD patients by motor subtype and its relation to AS. The cross-sectional data of a multicentre sample, including 714 PD patients and 194 healthy controls from Parkinson's Progression Marker Initiative study and Pingchan granule study were analyzed, stratified by PD subtypes [postural instability and gait disturbances (PIGD), tremor dominant (TD), and indeterminate] and domain autonomic dysfunction. Compared with healthy controls, PD patients scored higher in the total Scales for Outcomes in Parkinson's Disease-Autonomic dysfunction score and in several domain scores in particular, and there was a significant overlap in domain AS. Risk factors of individual domain autonomic dysfunction were heterogeneous. PIGD and indeterminate were the predominant subtypes in pupillomotor and thermoregulatory symptoms. TD and indeterminate were more likely to suffer from cardiovascular problem. The odd in sexual dysfunction was significant for PIGD. Gastrointestinal and urinary symptoms seemed not to be associated with a specific subtype. Our study demonstrated that AS were highly heterogeneous and 3 subtypes differed in autonomic performance, providing clues to understand mechanisms underlying AS in PD.
Topics: Humans; Parkinson Disease; Cross-Sectional Studies; Tremor; Primary Dysautonomias; Autonomic Nervous System
PubMed: 37666916
DOI: 10.1038/s41598-023-41662-9 -
FP Essentials Oct 2023Complex regional pain syndrome (CRPS) is a chronic pain condition characterized by intense pain, usually in a body region that has experienced trauma, and autonomic and...
Complex regional pain syndrome (CRPS) is a chronic pain condition characterized by intense pain, usually in a body region that has experienced trauma, and autonomic and inflammatory features. It most commonly develops after an arm or leg injury. Patients typically present with extreme hyperalgesia and/or allodynia. The Budapest Criteria are used to make this clinical diagnosis. Prompt diagnosis and aggressive management are critical because long-term outcomes are improved when treatment is initiated soon after symptom onset. The primary management options are rehabilitation and physical therapy, including such approaches as progressive tactile stimulation, normalization of movement to prevent limited range of motion, and others. No drugs are approved by the Food and Drug Administration (FDA) for CRPS management, but some evidence supports the use of drugs used to manage other types of neuropathic pain (eg, gabapentin, tricyclic antidepressants, serotonin-norepinephrine reuptake inhibitors). Nonsteroidal anti-inflammatory drugs also are used, and corticosteroids, bisphosphonates, ketamine, and other drugs can be helpful, particularly for early-stage CRPS. When pain severity prevents patients from participating in rehabilitation, referral to a pain specialist is warranted for consideration of procedural interventions, including sympathetic nerve blockade and spinal cord stimulation.
Topics: Humans; Chronic Pain; Complex Regional Pain Syndromes; Gabapentin; Physical Therapy Modalities; Ketamine
PubMed: 37812530
DOI: No ID Found -
Clinical Autonomic Research : Official... Aug 2023
Topics: Humans; Hypotension, Orthostatic; Droxidopa; Hypertension
PubMed: 37468794
DOI: 10.1007/s10286-023-00966-6