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Cureus Apr 2024Morvan syndrome is a rare condition distinguished by hyperactivity within the central, autonomic, and peripheral nervous systems. Due to the limited number of cases,...
Morvan syndrome is a rare condition distinguished by hyperactivity within the central, autonomic, and peripheral nervous systems. Due to the limited number of cases, this presents clinical challenges stemming from the scarcity of published literature. We present a successful anesthetic approach for a patient diagnosed with Morvan syndrome scheduled for elective major intra-thoracic surgery to remove metastases from a thymoma. The patient had previously undergone thymectomy, with the syndrome being diagnosed only one year after the surgery. Additionally, we conducted a literature review on the anesthetic management of this condition.
PubMed: 38779241
DOI: 10.7759/cureus.58762 -
The Journal of Physiology May 2024This paper updates and builds on a previous White Paper in this journal that some of us contributed to concerning the molecular and cellular basis of cardiac...
This paper updates and builds on a previous White Paper in this journal that some of us contributed to concerning the molecular and cellular basis of cardiac neurobiology of heart disease. Here we focus on recent findings that underpin cardiac autonomic development, novel intracellular pathways and neuroplasticity. Throughout we highlight unanswered questions and areas of controversy. Whilst some neurochemical pathways are already demonstrating prognostic viability in patients with heart failure, we also discuss the opportunity to better understand sympathetic impairment by using patient specific stem cells that provides pathophysiological contextualization to study 'disease in a dish'. Novel imaging techniques and spatial transcriptomics are also facilitating a road map for target discovery of molecular pathways that may form a therapeutic opportunity to treat cardiac dysautonomia.
PubMed: 38778747
DOI: 10.1113/JP284739 -
Neurogastroenterology and Motility May 2024An increasing number of studies have explored the clinical features, epidemiology, pathophysiology, and management of cyclic vomiting syndrome (CVS). CVS is common in... (Review)
Review
BACKGROUND
An increasing number of studies have explored the clinical features, epidemiology, pathophysiology, and management of cyclic vomiting syndrome (CVS). CVS is common in adults and children and negatively impacts patients, families, and the healthcare system. A related condition, cannabinoid hyperemesis syndrome (CHS), has been a focus of interest in the lay press and published literature.
PURPOSE
Clinical presentations of CVS have been defined by small series and expert opinion, but recent prospective studies are refining our understanding of the spectrum of emetic episodes and the breadth of comorbid conditions. Large cross-sectional population analyses are clarifying CVS prevalence and factors related to age, ethnicity, and geographic region. CVS pathophysiology is multifactorial with contributions from migraines, dysautonomia, endogenous cannabinoids, mitochondrial dysfunction, genetic abnormalities, and rapid gastric emptying. CVS treatment relies on antiemetics and antimigraine therapies to abort acute episodes coupled with prophylactic regimens employing neuromodulators and antiepileptics. CHS represents a challenge partly because of difficulties in achieving sustained cannabis abstinence. Benefits of other therapies in CHS remain poorly defined. Several areas warrant further scrutiny including better identification of CVS triggers and characterization of different CVS subsets including those with frequent severe episodes, refined description of epidemiology to allow targeting of populations predisposed to CVS development, rigorous definition of pathogenic factors to provide a foundation for exploratory studies of novel therapies, and conduct of controlled trials by multicenter collaborations to confirm benefits of existing and new therapies in development. Progress in these areas will be facilitated by generous governmental and industry support.
PubMed: 38775195
DOI: 10.1111/nmo.14825 -
Veterinary Anaesthesia and Analgesia 2024
Topics: Horner Syndrome; Dogs; Animals; Bupivacaine; Dog Diseases; Nerve Block; Anesthetics, Local; Thoracotomy; Male; Female
PubMed: 38772849
DOI: 10.1016/j.vaa.2024.03.008 -
Experimental Gerontology Aug 2024Orthostatic hypotension (OH) is common in older adults with hypertension. Antihypertensive treatment (AHT) prevents cardio- and cerebrovascular events. However,... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Orthostatic hypotension (OH) is common in older adults with hypertension. Antihypertensive treatment (AHT) prevents cardio- and cerebrovascular events. However, physicians are concerned to cause OH, making them hesitant to initiate or augment AHT in older adults with hypertension.
METHODS
We systematically researched electronic databases for trials with older participants (≥65 years) with hypertension and OH assessment after initiating, discontinuing, or augmenting AHT. Study quality was assessed using the ROBINS-I tool. Meta-analyses on OH prevalence and postural blood pressure (BP) drop were performed.
RESULTS
Twenty-five studies (26,695 participants) met inclusion criteria, of which fifteen could be included in the meta-analyses. OH prevalence decreased after AHT initiation or augmentation (risk ratio 0.39 (95 % CI = 0.21-0.72; I = 47 %; p < 0.01), n = 6 studies), but also after AHT discontinuation (risk ratio 0.39 (95 % CI = 0.28-0.55; I = 0 %; p < 0.01), n = 2 studies). Postural BP drop did not change after initiation or augmentation of AHT (mean difference 1.07 (95 % CI = -0.49-2.64; I = 92 %; p = 0.18), n = 11 studies). The main reason for ten studies not to be included in the meta-analyses was absence of baseline OH data. Most of these studies reported OH incidences between 0 and 2 %. Studies were heterogeneous in OH assessment methods (postural change, timing of BP measurements, and OH definition). Risk of bias was moderate to serious in twenty studies.
CONCLUSION
Results suggest that AHT initiation or augmentation decreases OH prevalence, implying that the risk of inducing OH may be overestimated in current AHT decision-making in older adults. However, the overall low level of evidence and the finding that AHT discontinuation reduces OH prevalence limit firm conclusions at present and highlight an important research gap. Future AHT trials in older adults should measure OH in a standardized protocol, adhering to consensus guidelines to overcome these limitations.
Topics: Aged; Aged, 80 and over; Humans; Antihypertensive Agents; Blood Pressure; Hypertension; Hypotension, Orthostatic; Prevalence
PubMed: 38772447
DOI: 10.1016/j.exger.2024.112461 -
Hypertension (Dallas, Tex. : 1979) Jul 2024The NET (norepinephrine transporter) is situated in the prejunctional plasma membrane of noradrenergic neurons. It is responsible for >90% of the norepinephrine uptake... (Review)
Review
The NET (norepinephrine transporter) is situated in the prejunctional plasma membrane of noradrenergic neurons. It is responsible for >90% of the norepinephrine uptake that is released in the autonomic neuroeffector junction. Inhibitors of this cell membrane transporter, known as norepinephrine reuptake inhibitors (NRIs), are commercially available for the treatment of depression and attention deficit hyperactivity disorder. These agents increase norepinephrine levels, potentiating its action in preganglionic and postganglionic adrenergic neurons, the latter through activation of α-1 adrenoreceptors. Previous studies found that patients with neurogenic orthostatic hypotension can improve standing blood pressure and reduce symptoms of neurogenic orthostatic hypotension after a single administration of the selective NRI atomoxetine. This effect was primarily observed in patients with impaired central autonomic pathways with otherwise normal postganglionic sympathetic fibers, known as multiple system atrophy. Likewise, patients with normal or high norepinephrine levels may benefit from NRIs. The long-term efficacy of NRIs for the treatment of neurogenic orthostatic hypotension-related symptoms is currently under investigation. In summary, an in-depth understanding of the pathophysiology of neurogenic orthostatic hypotension resulted in the discovery of a new therapeutic pathway targeted by NRI.
Topics: Humans; Hypotension, Orthostatic; Adrenergic Uptake Inhibitors; Norepinephrine; Atomoxetine Hydrochloride; Norepinephrine Plasma Membrane Transport Proteins; Blood Pressure
PubMed: 38766862
DOI: 10.1161/HYPERTENSIONAHA.124.22069 -
European Review For Medical and... May 2024Coronavirus disease (COVID-19) is a respiratory disease caused by SARS-CoV-2, which complicates the functioning of multiple systems, including the autonomic nervous... (Observational Study)
Observational Study
OBJECTIVE
Coronavirus disease (COVID-19) is a respiratory disease caused by SARS-CoV-2, which complicates the functioning of multiple systems, including the autonomic nervous system (ANS), causing dysautonomia. Investigation of dysautonomia and its association with exposure to COVID-19 is limited in healthy people. Therefore, the study aimed to investigate the relationship between ANS dysautonomia and coronavirus exposure and compare the ANS function between exposed and non-exposed to COVID-19.
SUBJECTS AND METHODS
The study involved 141 participants, with a mean age of 18-24.5 years, 83% male (49.6% exposed to COVID-19). The ANS was measured using a composite autonomic symptom scale (COMPASS-31) questionnaire and heart rate variability (HRV) using photoplethysmography. Exposure to COVID-19 was investigated using two national health-status tracking and COVID-19 exposure applications, "Sehhaty" and "Twakkalna".
RESULTS
A significantly inverse weak correlation between COMPASS-31 scores and COVID-19 exposure (r=-0.2, p=0.04). No significant association was found between HRV and COVID-19 exposure. COMPASS-31 scores for the exposed group (median=15, n=70) were significantly higher than those for the non-exposed group (median=12, n=71), U=1,913.5, p=0.03. Height (r=-0.4, p=0.002) and gender (r=0.3, p=0.001) were moderately correlated with COMPASS-31 among the exposed group.
CONCLUSIONS
These findings indicated that exposure to COVID-19 was associated with poorer ANS scores measured via COMPASS-31. Additionally, exposure to COVID-19 resulted in higher dysautonomia symptoms than non-exposed. Height and gender differences contribute to the severity of dysautonomia among exposed people.
Topics: Humans; COVID-19; Male; Female; Adolescent; Young Adult; Autonomic Nervous System; Heart Rate; SARS-CoV-2; Adult; Autonomic Nervous System Diseases; Primary Dysautonomias; Surveys and Questionnaires
PubMed: 38766799
DOI: 10.26355/eurrev_202405_36187 -
Cureus Apr 2024Biermer's disease (BD) or pernicious anemia (PA) is an autoimmune atrophic gastritis characterized by the absence of intrinsic factor (IF) secretion, leading to...
Biermer's disease (BD) or pernicious anemia (PA) is an autoimmune atrophic gastritis characterized by the absence of intrinsic factor (IF) secretion, leading to malabsorption of vitamin B12 in the ileum. Its clinical manifestations are primarily hematological, with neuropsychiatric and cardiovascular manifestations being less common. We present the case of a patient with PA diagnosed based on neurological and cardiovascular complications. The patient, a 56-year-old man with no specific medical history, presented with an episode of melena without other associated digestive symptoms. He also complained of memory and gait disturbances. Clinical examination revealed a cerebellar ataxia with impaired proprioceptive and vibratory sensitivity, and a swollen and red right lower limb with a positive Homan sign. The blood count showed macrocytic anemia. Gastroscopy revealed flattened fundic folds resembling a fundus appearance, and histopathological examination confirmed fundic atrophic gastritis with pseudopyloric metaplasia and lymphoplasmacytic infiltration. Anti-intrinsic factor antibodies were positive, while anti-parietal cell antibodies were negative. Vitamin B12 levels were severely low, and vitamin B9 levels were normal. TSH and HbA1c levels were within normal ranges. The abdominal CT scan showed no abnormalities. Lower limb Doppler ultrasound confirmed the diagnosis of deep vein thrombosis (DVT). Cardiac evaluation revealed sinus bradycardia suggestive of secondary dysautonomia. Therapeutically, the patient was started on vitamin B12 supplementation and anticoagulant therapy for DVT, resulting in a good clinical and biological outcome.
PubMed: 38765343
DOI: 10.7759/cureus.58601 -
Neurobiology of Disease Jul 2024Multiple system atrophy (MSA) is a primary oligodendroglial synucleinopathy, characterized by elevated iron burden in early-affected subcortical nuclei. Although...
BACKGROUND
Multiple system atrophy (MSA) is a primary oligodendroglial synucleinopathy, characterized by elevated iron burden in early-affected subcortical nuclei. Although neurotoxic effects of brain iron deposition and its relationship with α-synuclein pathology have been demonstrated, the exact role of iron dysregulation in MSA pathogenesis is unknown. Therefore, advancing the understanding of iron dysregulation at the cellular level is critical, especially in relation to α-synuclein cytopathology.
METHODS
Iron burden in subcortical and brainstem regions were histologically mapped in human post-mortem brains of 4 MSA-parkinsonian (MSA-P), 4 MSA-cerebellar (MSA-C), and 1 MSA case with both parkinsonian and cerebellar features. We then performed the first cell type-specific evaluation of pathological iron deposition in α-synuclein-affected and -unaffected cells of the globus pallidus, putamen, and the substantia nigra, regions of highest iron concentration, using a combination of iron staining with immunolabelling. Selective regional and cellular vulnerability patterns of iron deposition were compared between disease subtypes. In 7 MSA cases, expression of key iron- and closely related oxygen-homeostatic genes were examined.
RESULTS
MSA-P and MSA-C showed different patterns of regional iron burden across the pathology-related systems. We identified subcortical microglia to predominantly accumulate iron, which was more distinct in MSA-P. MSA-C showed relatively heterogenous iron accumulation, with greater or similar deposition in astroglia. Iron deposition was also found outside cellular bodies. Cellular iron burden associated with oligodendrocytic, and not neuronal, α-synuclein cytopathology. Gene expression analysis revealed dysregulation of oxygen homeostatic genes, rather than of cellular iron. Importantly, hierarchal cluster analysis revealed the pattern of cellular vulnerability to iron accumulation, distinctly to α-synuclein pathology load in the subtype-related systems, to distinguish MSA subtypes.
CONCLUSIONS
Our comprehensive evaluation of iron deposition in MSA brains identified distinct regional, and for the first time, cellular distribution of iron deposition in MSA-P and MSA-C and revealed cellular vulnerability patterns to iron deposition as a novel neuropathological characteristic that predicts MSA clinical subtypes. Our findings suggest distinct iron-related pathomechanisms in MSA clinical subtypes that are therefore not a consequence of a uniform down-stream pathway to α-synuclein pathology, and inform current efforts in iron chelation therapies at the disease and cellular-specific levels.
Topics: Humans; Multiple System Atrophy; Iron; Male; Aged; Female; Middle Aged; alpha-Synuclein; Brain; Aged, 80 and over; Oligodendroglia
PubMed: 38761956
DOI: 10.1016/j.nbd.2024.106535 -
Journal of Cellular and Molecular... May 2024Fermented foods play a significant role in the human diet for their natural, highly nutritious and healthy attributes. Our aim was to study the effect of yeast extract,...
Fermented foods play a significant role in the human diet for their natural, highly nutritious and healthy attributes. Our aim was to study the effect of yeast extract, a fermented substance extracted from natural yeast, on colonic motility to better understand its potential therapeutic role. A yeast extract was given to rats by gavage for 3 days, and myogenic and neurogenic components of colonic motility were studied using spatiotemporal maps made from video recordings of the whole colon ex vivo. A control group received saline gavages. The yeast extract caused excitation of the musculature by increasing the propagation length and duration of long-distance contractions, the major propulsive activity of the rat colon. The yeast extract also evoked rhythmic propulsive motor complexes (RPMCs) which were antegrade in the proximal and mid-colon and retrograde in the distal colon. RPMC activity was evoked by distention-induced neural activity, but it was myogenic in nature since we showed it to be generated by bethanechol in the presence of tetrodotoxin. In conclusion, ingestion of yeast extract stimulates rat colon motility by exciting neurogenic and myogenic control mechanisms.
Topics: Animals; Colon; Gastrointestinal Motility; Rats; Male; Yeasts; Rats, Sprague-Dawley; Tetrodotoxin
PubMed: 38760903
DOI: 10.1111/jcmm.18343