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1.
Medicine (Baltimore) ; 103(9): e37339, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38428898

RESUMO

Autonomic imbalance predicts worse clinical outcomes in patients with heart failure (HF). Managing the variables affecting heart rate variability (HRV) might improve the clinical outcomes of patients with HF. This study aimed to investigate variables affecting HRV. We assessed autonomic nervous system activity (low-frequency [Lf], high-frequency [Hf], and Lf/Hf ratio) in 60 patients with HF, employing standard measures to capture short-term HRV. To estimate the independent effects of variables such as well-known cardiac risk factors and psychosocial conditions on HRV, multivariate analyses were conducted. For psychosocial variables, we assessed depression and quality of life in patients and their family caregivers. We also assessed the self-care behavior of patients and their caregivers' burden. Depression in family caregivers and self-care behavior of patients were independently associated with a decreased Hf (ß-coefficient = 0.309, P = .039 and ß-coefficient = -0.029, P = .047, respectively). Depression of family caregivers and self-care behavior of patients may affect HRV in patients with HF.


Assuntos
Doenças do Sistema Nervoso Autônomo , Insuficiência Cardíaca , Humanos , Qualidade de Vida , Sistema Nervoso Autônomo , Frequência Cardíaca/fisiologia
2.
Sci Transl Med ; 16(738): eadg3665, 2024 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-38478631

RESUMO

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease, characterized by the death of upper (UMN) and lower motor neurons (LMN) in the motor cortex, brainstem, and spinal cord. Despite decades of research, ALS remains incurable, challenging to diagnose, and of extremely rapid progression. A unifying feature of sporadic and familial forms of ALS is cortical hyperexcitability, which precedes symptom onset, negatively correlates with survival, and is sufficient to trigger neurodegeneration in rodents. Using electrocorticography in the Sod1G86R and FusΔNLS/+ ALS mouse models and standard electroencephalography recordings in patients with sporadic ALS, we demonstrate a deficit in theta-gamma phase-amplitude coupling (PAC) in ALS. In mice, PAC deficits started before symptom onset, and in patients, PAC deficits correlated with the rate of disease progression. Using mass spectrometry analyses of CNS neuropeptides, we identified a presymptomatic reduction of noradrenaline (NA) in the motor cortex of ALS mouse models, further validated by in vivo two-photon imaging in behaving SOD1G93A and FusΔNLS/+ mice, that revealed pronounced reduction of locomotion-associated NA release. NA deficits were also detected in postmortem tissues from patients with ALS, along with transcriptomic alterations of noradrenergic signaling pathways. Pharmacological ablation of noradrenergic neurons with DSP-4 reduced theta-gamma PAC in wild-type mice and administration of a synthetic precursor of NA augmented theta-gamma PAC in ALS mice. Our findings suggest theta-gamma PAC as means to assess and monitor cortical dysfunction in ALS and warrant further investigation of the NA system as a potential therapeutic target.


Assuntos
Esclerose Amiotrófica Lateral , Doenças do Sistema Nervoso Autônomo , Dopamina beta-Hidroxilase/deficiência , Doenças Neurodegenerativas , Norepinefrina/deficiência , Humanos , Camundongos , Animais , Esclerose Amiotrófica Lateral/metabolismo , Superóxido Dismutase-1/genética , Superóxido Dismutase-1/metabolismo , Doenças Neurodegenerativas/metabolismo , Medula Espinal/metabolismo , Modelos Animais de Doenças , Camundongos Transgênicos , Superóxido Dismutase/metabolismo
3.
Int J Mol Sci ; 25(5)2024 Feb 29.
Artigo em Inglês | MEDLINE | ID: mdl-38474067

RESUMO

Severe obesity in young children prompts for a differential diagnosis that includes syndromic conditions. Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) syndrome is a potentially fatal disorder characterized by rapid-onset obesity associated with hypoventilation, neural crest tumors, and endocrine and behavioral abnormalities. The etiology of ROHHAD syndrome remains to be established, but recent research has been focusing on autoimmunity. We report on a 2-year-old girl with rapid-onset obesity during the first year of life who progressed to hypoventilation and encephalitis in less than four months since the start of accelerated weight gain. The patient had a high titer of anti-ZSCAN1 antibodies (348; reference range < 40), and the increased values did not decline after acute phase treatment. Other encephalitis-related antibodies, such as the anti-NDMA antibody, were not detected. The rapid progression from obesity onset to central hypoventilation with encephalitis warns about the severe consequences of early-onset ROHHAD syndrome. These data indicate that serial measurements of anti-ZSCAN1 antibodies might be useful for the diagnosis and estimation of disease severity. Further research is needed to determine whether it can predict the clinical course of ROHHAD syndrome and whether there is any difference in antibody production between patients with and without tumors.


Assuntos
Neoplasias das Glândulas Suprarrenais , Doenças do Sistema Nervoso Autônomo , Encefalite , Doenças Hipotalâmicas , Obesidade Pediátrica , Feminino , Humanos , Pré-Escolar , Hipoventilação/complicações , Hipoventilação/diagnóstico , Obesidade Pediátrica/complicações , Neoplasias das Glândulas Suprarrenais/complicações , Síndrome , Encefalite/complicações
4.
CNS Neurosci Ther ; 30(3): e14651, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38432692

RESUMO

AIMS: To investigate the risk factors for early-onset psychosis in Parkinson's disease (PD) in a cohort of patients from the Parkinson's Progression Markers Initiative. METHODS: Longitudinal data on motor and non-motor features, dopamine transporter (DAT) imaging, and cerebrospinal fluid (CSF) measurements were collected. The survival probability of psychotic symptoms, potential risk factors for psychosis development over a 5-year follow-up period, and the performance of the prediction model were evaluated. RESULTS: Among the 338 newly diagnosed patients with PD, 83 developed psychotic symptoms. Gastrointestinal autonomic dysfunction, presence of probable rapid-eye-movement sleep behavior disorder, and the ratio Aß42: total-tau could independently predict onset of psychosis in PD (hazard ratio (HR) = 1.157, 95% confidence interval (CI) 1.022-1.309, p = 0.021, HR = 2.596, 95% CI 1.287-5.237, p = 0.008, and HR = 0.842, 95% CI 0.723-0.980, p = 0.027, respectively). The combined model integrating baseline clinical predictors, DAT imaging, and CSF measurements achieved better sensitivity than the clinical predictors alone (area under the curve = 0.770 [95% CI 0.672-0.868] vs. 0.714 [95% CI 0.625-0.802], p = 0.098). CONCLUSION: We identified clinical and CSF predictors of early-onset psychosis in patients with PD. Our study provides evidence and implications for prognostic stratification and therapeutic approaches for PD psychosis.


Assuntos
Doenças do Sistema Nervoso Autônomo , Doença de Parkinson , Transtornos Psicóticos , Humanos , Doença de Parkinson/diagnóstico , Doença de Parkinson/diagnóstico por imagem , Estudos de Coortes , Transtornos Psicóticos/diagnóstico por imagem , Transtornos Psicóticos/etiologia , Fatores de Risco
5.
J Int Med Res ; 52(3): 3000605241233159, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38436278

RESUMO

Neuronal intranuclear inclusion disease (NIID) is a rare progressive neurodegenerative disease that mainly manifests as dementia, muscle weakness, sensory disturbances, and autonomic nervous dysfunction. Herein, we report a 68-year-old Chinese woman who was hospitalized because of resting tremor and bradykinesia that had been present for 7 years. Five years prior, bradykinesia and hypermyotonia had become apparent. She had urinary incontinence and rapid eye movement sleep behavior disorder. She was diagnosed with Parkinson's disease (PD) and received levodopa and pramipexole, which relieved her motor symptoms. During hospitalization, diffusion-weighted imaging revealed a high-intensity signal along the cortical medullary junction. Moreover, a skin biopsy revealed the presence of intranuclear inclusions in adipocytes, fibroblasts, and sweat gland cells. NIID was diagnosed by testing the Notch 2 N-terminal-like C (NOTCH2NLC) gene. We report this case to remind doctors to consider NIID when diagnosing patients with symptoms indicative of Parkinson's disease. Moreover, we note that further research is needed on the mechanism by which levodopa is effective for NIID.


Assuntos
Doenças do Sistema Nervoso Autônomo , Doenças Neurodegenerativas , Doença de Parkinson , Humanos , Feminino , Idoso , Doenças Neurodegenerativas/diagnóstico , Doença de Parkinson/diagnóstico , Doença de Parkinson/tratamento farmacológico , Corpos de Inclusão Intranuclear , Levodopa/uso terapêutico , Hipocinesia , Erros de Diagnóstico
6.
Physiol Rep ; 12(5): e15963, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38439737

RESUMO

Hypoxia is mainly caused by cardiopulmonary disease or high-altitude exposure. We used a driving simulator to investigate whether breathing hypoxic gas influences driving behaviors in healthy subjects. Fifty-two healthy subjects were recruited in this study, approved by the Science and Engineering Ethical Committee. During simulated driving experiments, driving behaviors, breathing frequency, oxygen saturation (SpO2 ), and heart rate variability (HRV) were analyzed. Each subject had four driving sessions; a 10-min practice and three 20-min randomized interventions: normoxic room air (21% FIO2 ) and medical air (21% FIO2 ) and hypoxic air (equal to 15% FIO2 ), analyzed by repeated measures ANOVA. Driving behaviors and HRV frequency domains showed no significant change. Heart rate (HR; p < 0.0001), standard deviation of the RR interval (SDRR; p = 0.03), short-term HRV (SD1; p < 0.0001), breathing rate (p = 0.01), and SpO2 (p < 0.0001) were all significantly different over the three gas interventions. Pairwise comparisons showed HR increased during hypoxic gas exposure compared to both normoxic interventions, while SDRR, SD1, breathing rate, and SpO2 were lower. Breathing hypoxic gas (15% FiO2 , equivalent to 2710 m altitude) may not have a significant impact on driving behavior in healthy subjects. Furthermore, HRV was negatively affected by hypoxic gas exposure while driving suggesting further research to investigate the impact of breathing hypoxic gas on driving performance for patients with autonomic dysfunction.


Assuntos
Altitude , Doenças do Sistema Nervoso Autônomo , Sindactilia , Humanos , Voluntários Saudáveis , Hipóxia
7.
Int J Rheum Dis ; 27(3): e15088, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38454192

RESUMO

BACKGROUND: Sjögren's Syndrome (SS), mainly affecting women in their midlife, is characterized by persistent inflammation in glands producing tears and saliva, often leading to significant complications. This study investigates the differences in autonomic system functioning between individuals with SS and healthy controls. METHODS: From April 2019 to December 2022, 329 diagnosed primary SS (pSS) patients and 30 healthy controls were enrolled at Taipei Veterans General Hospital, Taipei, Taiwan. The study assessed autonomic nervous system functioning using various HRV metrics. Participants were divided based on age and AECG criteria, including salivary gland biopsy and autoantibody status. RESULTS: Significant differences in Heart Rate Variability (HRV) were observed between pSS patients and healthy controls. The total power index was notably lower in pSS patients (4.98 ± 1.29) than in controls (5.54 ± 1.21, p = .022). Additionally, Vagal (VAG) activity was significantly reduced in the pSS group (4.95 ± 1.33) compared to the healthy control group (5.47 ± 1.19, p = .041). Age-stratified analysis highlighted that the ≤50 years pSS group had a higher heart rate (77.74 ± 10.42) compared to the >50 years group (73.86 ± 10.35, p = .005). This group also showed a higher total power index (5.78 ± 1.30) versus the >50 years group (4.68 ± 1.19, p < .001), and significantly lower VAG activity (4.70 ± 1.26, p = .007) compared to healthy controls. Furthermore, the Standard Deviation of Normal-to-Normal Intervals (SDNN) was greater in the ≤50 years SS group (44.45 ± 37.12) than in the >50 years group (33.51 ± 26.18, p = .007). In pSS patients, those positive for both salivary gland biopsy and autoantibodies demonstrated a lower Total Power (4.25 ± 1.32) and R-wave validity (93.50 ± 4.79, p < .05) than other groups, suggesting more severe autonomic imbalance. The R-R interval variation (RRIV) was also significantly higher in this dual-positive group (696.10 ± 975.41, p < .05). Additionally, the ESSPRI for dryness was markedly higher in the dual-positive group (8.10 ± 1.45, p < .05), indicating more severe symptoms. These findings reveal significant variations in autonomic function in SS patients, especially in those with dual-positive biopsy and autoantibody status. CONCLUSION: This study demonstrates significant autonomic dysfunction in pSS patients compared to healthy controls, particularly in those positive for both salivary gland biopsy and autoantibodies. The age-stratified analysis further emphasizes the impact of aging on autonomic system functioning in pSS, suggesting a need for age-specific management approaches in pSS patient care.


Assuntos
Doenças do Sistema Nervoso Autônomo , Síndrome de Sjogren , Humanos , Feminino , Pessoa de Meia-Idade , Síndrome de Sjogren/complicações , Frequência Cardíaca , Saliva , Lágrimas , Autoanticorpos
8.
Handb Clin Neurol ; 200: 275-282, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38494282

RESUMO

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.


Assuntos
Doenças do Sistema Nervoso Autônomo , Neoplasias , Doenças do Sistema Nervoso , Síndromes Paraneoplásicas do Sistema Nervoso , Humanos , Autoanticorpos , Doenças do Sistema Nervoso Autônomo/diagnóstico , Doenças do Sistema Nervoso Autônomo/etiologia , Síndromes Paraneoplásicas do Sistema Nervoso/diagnóstico , Síndromes Paraneoplásicas do Sistema Nervoso/terapia , Neoplasias/complicações , Sistema Nervoso Autônomo
9.
Handb Clin Neurol ; 200: 307-325, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38494285

RESUMO

Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disease characterized by proximal muscle weakness, loss of tendon reflexes, and autonomic dysfunction. Muscle weakness usually starts in the upper legs and can progress to oculobulbar and in severe cases respiratory muscles. P/Q-type voltage-gated calcium channels (VGCCs) localized in the presynaptic motor nerve terminal and in the autonomic nervous system are targeted by antibodies in LEMS patients. These antibodies can be detected in about 90% of patients, and the presence of decrement and increment upon repetitive nerve stimulation is also a highly sensitive diagnostic test. Rapid diagnosis is important because of the association with SCLC in 50%-60% of patients, which stresses the need for vigorous tumor screening after diagnosis. Clinical parameters can predict tumor probability and guide frequency of tumor screening. Treatment of the tumor as well as symptomatic treatment and immunosuppression can effectively control symptoms in the majority of patients.


Assuntos
Doenças do Sistema Nervoso Autônomo , Síndrome Miastênica de Lambert-Eaton , Humanos , Síndrome Miastênica de Lambert-Eaton/diagnóstico , Síndrome Miastênica de Lambert-Eaton/terapia , Autoanticorpos , Sistema Nervoso Autônomo , Debilidade Muscular/complicações
11.
Eur Rev Med Pharmacol Sci ; 28(5): 1931-1936, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38497876

RESUMO

OBJECTIVE: The autonomic nervous system (ANS) plays an important role in maintaining physiological regulation. It regulates the body's response to many variable situations. Orthostatic intolerance (OI) is one of the most important signs of autonomic dysfunction. Autonomic dysfunction is known to cause premature ejaculation (PE) by disturbing the balance in erection and ejaculation cycles. Considering that OI may develop due to autonomic dysfunction in patients with PE, we hypothesized that OI symptoms would increase in these patients. The aim of our study was to investigate the relationship between orthostatic intolerance and PE. PATIENTS AND METHODS: This case-control study included a total of 39 patients with PE and 47 volunteers without PE. All subjects were assessed using the self-reported Orthostatic Grading Scale (OGS). In addition, the validated five-item Turkish version of the Premature Ejaculation Diagnostic Tool (PEDT) was used to evaluate PE. The PE group included patients with a PEDT score ≥ 11. RESULTS: The mean ages of the PE and control groups were 38.2 ± 7.8 and 40.5 ± 9.1 years, respectively (p = 0.137). The mean PEDT scores of the PE and control groups were 13.9 ± 3.6 and 6.6 ± 2.9, respectively (p < 0.0001), and their mean OGS scores were 5.6 ± 2.4 and 1.6 ± 1.3, respectively (p < 0.0001). A statistically significant correlation was found between the PEDT and OGS scores (r: 0.686, p < 0.0001). CONCLUSIONS: The orthostatic intolerance symptoms of patients with PE were higher than those of the control group. There was a correlation between the severity of PE and the severity of orthostatic intolerance. This is the first study in the literature to reveal a relationship between orthostatic intolerance and PE.


Assuntos
Doenças do Sistema Nervoso Autônomo , Intolerância Ortostática , Ejaculação Precoce , Masculino , Humanos , Adulto , Pessoa de Meia-Idade , Ejaculação Precoce/diagnóstico , Estudos de Casos e Controles , Intolerância Ortostática/diagnóstico , Sistema Nervoso Autônomo
12.
PLoS One ; 19(3): e0299745, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38498440

RESUMO

BACKGROUND: Globally, hypertension is among the leading causes of premature mortality. It is a noncommunicable disease characterized by a persistent state of raised blood pressure that increases the risk of cardiovascular diseases and medical conditions affecting the brain and kidneys. There is a paucity of thorough hypertension knowledge assessment among hypertensive patients in the Jazan region of Saudi Arabia. Thus, this study aimed to assess overall and specific knowledge about hypertension and to identify predictors of inadequate knowledge. METHODS: A cross-sectional study was conducted in the Jazan region of Saudi Arabia between February and April 2023. Data were collected using an online, self-administered questionnaire divided into two sections. In the first section, the characteristics of the participants were collected. In the second section, the Hypertension Knowledge-Level Scale was used to measure overall and specific knowledge areas (subdimensions). The overall and subdimensional means were tested using Mann-Whitney U and Kruskal-Wallis H tests. Furthermore, the binary logistic regression was conducted to determine inadequate knowledge predictors. RESULTS: In all 253 hypertensive patients were eligible for participation; almost 70% of whom were male. The mean age of the participants was 45 years (±14.7), and their mean overall knowledge score was 17.60 (±5.09), which was equivalent to 67.7% of the maximum score. In addition, 40.7% of participants had an adequate level of hypertension knowledge. The complications subdimension level of knowledge was borderline optimal. At the same time, an adequate knowledge level was detected only in the lifestyle subdimension. CONCLUSION: Most patients showed inadequate levels of knowledge related to hypertension management. Diet, medical treatment, disease definition, drug compliance, and complications were subsequently the least knowledgeable subdimensions among the study population. Therefore, these subdimensions should be prioritized when planning hypertension educational interventions and during follow-up sessions, especially for patients of younger age groups and those with lower educational levels.


Assuntos
Doenças do Sistema Nervoso Autônomo , Hipertensão , Humanos , Masculino , Pessoa de Meia-Idade , Feminino , Estudos Transversais , Arábia Saudita/epidemiologia , Hipertensão/epidemiologia , Hipertensão/tratamento farmacológico , Inquéritos e Questionários
13.
Clin Auton Res ; 34(1): 125-135, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38446362

RESUMO

PURPOSE: Orthostasis increases the variability of continuously recorded blood pressure (BP). Low-frequency (LF) BP oscillations (Mayer waves) in this setting are related to the vascular-sympathetic baroreflex. Mechanisms of increased high-frequency (HF) BP oscillations at the periodicity of respiration during orthostasis have received less research attention. A previously reported patient with post-neurosurgical orthostatic hypotension (OH) and vascular-sympathetic baroreflex failure had large tilt-evoked, breathing-driven BP oscillations, suggesting that such oscillations can occur independently of vascular-sympathetic baroreflex modulation. In the present study we assessed effects of orthostasis on BP variability in the frequency domain in patient cohorts with or without OH. METHODS: Power spectral analysis of systolic BP variability was conducted on recordings from 73 research participants, 42 with neurogenic OH [13 pure autonomic failure, 14 Parkinson's disease (PD) with OH, 12 parkinsonian multiple system atrophy, and 3 status post-brainstem neurosurgery] and 31 without OH (control group of 16 healthy volunteers and 15 patients with PD lacking OH), before, during, and after 5' of head-up tilt at 90 degrees from horizontal. The data were log transformed for statistical testing. RESULTS: Across all subjects, head-up tilting increased HF power of systolic BP variability (p = 0.001), without a difference between the neurogenic OH and control groups. LF power during orthostasis was higher in the control than in the OH groups (p = 0.009). CONCLUSIONS: The results of this observational cohort study confirm those based on our case report and lead us to propose that even in the setting of vascular-sympathetic baroreflex failure orthostasis increases HF power of BP variability.


Assuntos
Doenças do Sistema Nervoso Autônomo , Hipotensão Ortostática , Humanos , Pressão Sanguínea/fisiologia , Barorreflexo/fisiologia , Tontura , Frequência Cardíaca/fisiologia , Respiração
14.
J Integr Neurosci ; 23(2): 37, 2024 Feb 19.
Artigo em Inglês | MEDLINE | ID: mdl-38419450

RESUMO

BACKGROUND: The purpose of this study was to determine the detailed characteristics of dizziness in patients with de novo Parkinson's disease (PD) and the clinical implications of dizziness. METHODS: Ninety-three people with de novo PD were enrolled between July 2017 and August 2022 for this retrospective study. Using each representative scale, various motor and non-motor symptoms were assessed. In addition, clinical manifestations of dizziness in those patients, including its presence, type, frequency, and duration of occurrence, were investigated. RESULTS: Thirty-nine patients with de novo PD reported dizziness, with presyncope being the most common (38%). The most common frequency was several times a week (51%). The most common duration was a few seconds (67%). Multivariable logistic regression analysis showed that dizziness was more common in women than in men {odds ratio (OR): 3.3601, 95% confidence interval (CI): 1.0820-10.4351, p = 0.0361}. Dizziness was significantly related to non-motor symptoms of low global cognition (OR: 0.8372, 95% CI: 0.7285-0.9622, p = 0.0123) and severe autonomic dysfunction (OR: 1.1112, 95% CI: 1.0297-1.1991, p = 0.0067). A post-hoc analysis revealed that dizziness was only associated with cardiovascular dysautonomia (adjusted OR: 10.2377, 95% CI: 3.3053-31.7098, p < 0.0001) among several domains of dysautonomia. CONCLUSIONS: About 42% of patients with de novo PD complained of dizziness. The occurrence of dizziness in those people was highly associated with female gender women, cognitive impairment, and cardiovascular dysautonomia. These results suggest that clinicians should pay close attention when patients with PD complain of dizziness.


Assuntos
Doenças do Sistema Nervoso Autônomo , Doença de Parkinson , Masculino , Humanos , Feminino , Tontura/epidemiologia , Tontura/etiologia , Estudos Retrospectivos , Doenças do Sistema Nervoso Autônomo/complicações , Vertigem
15.
CNS Neurosci Ther ; 30(2): e14544, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38372446

RESUMO

AIMS: Autonomic dysfunction with central autonomic network (CAN) damage occurs frequently after intracerebral hemorrhage (ICH) and contributes to a series of adverse outcomes. This review aims to provide insight and convenience for future clinical practice and research on autonomic dysfunction in ICH patients. DISCUSSION: We summarize the autonomic dysfunction in ICH from the aspects of potential mechanisms, clinical significance, assessment, and treatment strategies. The CAN structures mainly include insular cortex, anterior cingulate cortex, amygdala, hypothalamus, nucleus of the solitary tract, ventrolateral medulla, dorsal motor nucleus of the vagus, nucleus ambiguus, parabrachial nucleus, and periaqueductal gray. Autonomic dysfunction after ICH is closely associated with neurological functional outcomes, cardiac complications, blood pressure fluctuation, immunosuppression and infection, thermoregulatory dysfunction, hyperglycemia, digestive dysfunction, and urogenital disturbances. Heart rate variability, baroreflex sensitivity, skin sympathetic nerve activity, sympathetic skin response, and plasma catecholamine concentration can be used to assess the autonomic functional activities after ICH. Risk stratification of patients according to autonomic functional activities, and development of intervention approaches based on the restoration of sympathetic-parasympathetic balance, would potentially improve clinical outcomes in ICH patients. CONCLUSION: The review systematically summarizes the evidence of autonomic dysfunction and its association with clinical outcomes in ICH patients, proposing that targeting autonomic dysfunction could be potentially investigated to improve the clinical outcomes.


Assuntos
Doenças do Sistema Nervoso Autônomo , Sistema Nervoso Autônomo , Humanos , Sistema Nervoso Autônomo/fisiologia , Sistema Nervoso Simpático/fisiologia , Doenças do Sistema Nervoso Autônomo/etiologia , Doenças do Sistema Nervoso Autônomo/terapia , Nervo Vago/fisiologia , Hemorragia Cerebral/complicações , Hemorragia Cerebral/terapia , Frequência Cardíaca/fisiologia
16.
Continuum (Minneap Minn) ; 30(1): 224-242, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38330480

RESUMO

OBJECTIVE: This article discusses the effects of myelopathy on multiple organ systems and reviews the treatment and management of some of these effects. LATEST DEVELOPMENTS: Recent advances in functional electrical stimulation, epidural spinal cord stimulation, robotics, and surgical techniques such as nerve transfer show promise in improving function in patients with myelopathy. Ongoing research in stem cell therapy and neurotherapeutic drugs may provide further therapeutic avenues in the future. ESSENTIAL POINTS: Treatment for symptoms of spinal cord injury should be targeted toward patient goals. If nerve transfer for upper extremity function is considered, the patient should be evaluated at around 6 months from injury to assess for lower motor neuron involvement and possible time limitations of surgery. A patient with injury at or above the T6 level is at risk for autonomic dysreflexia, a life-threatening condition that presents with elevated blood pressure and can lead to emergent hypertensive crisis. Baclofen withdrawal due to baclofen pump failure or programming errors may also be life-threatening. Proper management of symptoms may help avoid complications such as autonomic dysreflexia, renal failure, heterotopic ossification, and fractures.


Assuntos
Disreflexia Autonômica , Doenças do Sistema Nervoso Autônomo , Hipertensão , Traumatismos da Medula Espinal , Humanos , Disreflexia Autonômica/diagnóstico , Disreflexia Autonômica/etiologia , Disreflexia Autonômica/terapia , Baclofeno/uso terapêutico , Traumatismos da Medula Espinal/complicações , Doenças do Sistema Nervoso Autônomo/complicações , Hipertensão/complicações
17.
Sci Rep ; 14(1): 3588, 2024 02 13.
Artigo em Inglês | MEDLINE | ID: mdl-38351168

RESUMO

Data on the impact of arterial stiffness on autonomic function are limited. We sought to investigate whether heart rate recovery (HRR), a predictor of autonomic function, is impaired in patients with increased arterial stiffness. A total of 475 participants (mean age 55.8 ± 11.1 years, 34.3% women) who underwent a treadmill exercise test (TET) for the evaluation of chest pain were retrospectively analyzed. All patients underwent brachial-ankle pulse wave velocity (baPWV) measurement on the same day. HRR was defined as the difference in heart rate from maximal exercise to 1 min of recovery. Participants with the lowest HRR tertile were older and had more cardiovascular risk factors than those with the highest HRR tertile. Simple correlation analysis showed that baPWV was negatively correlated with HRR (r = - 0.327, P < 0.001). In multiple linear regression analysis, there was a significant association between baPWV and HRR, even after adjusting for potential confounders (ß = - 0.181, P < 0.001). In participants who underwent TET, baPWV was negatively correlated with HRR. The results of our study indicate a potential relationship between arterial stiffness and the autonomic nervous system.


Assuntos
Doenças do Sistema Nervoso Autônomo , Rigidez Vascular , Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Masculino , Rigidez Vascular/fisiologia , Estudos Transversais , Índice Tornozelo-Braço , Teste de Esforço , Estudos Retrospectivos , Análise de Onda de Pulso , Fatores de Risco
18.
Int J Mol Sci ; 25(4)2024 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-38396973

RESUMO

Autoimmune autonomic ganglionopathy (AAG) is a disease of autonomic failure caused by ganglionic acetylcholine receptor (gAChR) autoantibodies. Although the detection of autoantibodies is important for distinguishing the disease from other neuropathies that present with autonomic dysfunction, other factors are important for accurate diagnosis. Here, we provide a comprehensive review of the clinical features of AAG, highlighting differences in clinical course, clinical presentation, and laboratory findings from other neuropathies presenting with autonomic symptoms. The first step in diagnosing AAG is careful history taking, which should reveal whether the mode of onset is acute or chronic, followed by an examination of the time course of disease progression, including the presentation of autonomic and extra-autonomic symptoms. AAG is a neuropathy that should be differentiated from other neuropathies when the patient presents with autonomic dysfunction. Immune-mediated neuropathies, such as acute autonomic sensory neuropathy, are sometimes difficult to differentiate, and therefore, differences in clinical and laboratory findings should be well understood. Other non-neuropathic conditions, such as postural orthostatic tachycardia syndrome, chronic fatigue syndrome, and long COVID, also present with symptoms similar to those of AAG. Although often challenging, efforts should be made to differentiate among the disease candidates.


Assuntos
Doenças Autoimunes do Sistema Nervoso , Doenças Autoimunes , Doenças do Sistema Nervoso Autônomo , Doenças do Sistema Nervoso Periférico , Humanos , Gânglios Autônomos , Síndrome Pós-COVID-19 Aguda , Sistema Nervoso Autônomo , Doenças do Sistema Nervoso Autônomo/diagnóstico , Doenças do Sistema Nervoso Autônomo/etiologia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/patologia , Doenças do Sistema Nervoso Periférico/patologia , Autoanticorpos
19.
J Parkinsons Dis ; 14(2): 335-346, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38306061

RESUMO

Background: Increased prevalence of cardiovascular autonomic failure might play a key role on Parkinson's disease (PD) progression of glucocerebrosidase gene (GBA)-mutated patients, determining a malignant phenotype of disease in these patients. Objective: To objectively characterize, for the first time, the cardiovascular autonomic profile of GBA-mutated patients compared to idiopathic PD patients by means of cardiovascular reflex tests (CRTs). Methods: This is a case-control (1 : 2) study on PD patients belonging to well-characterized prospective cohorts. For each PD patient carrying GBA variants, two idiopathic PD patients, matched for sex and disease duration at CRTs, were selected. Patients recruited in these cohorts underwent a complete clinical and instrumental evaluation including specific autonomic questionnaires, CRTs and extensive genetic analysis. Results: A total of 23 GBA-PD patients (19 males, disease duration 7.7 years) were included and matched with 46 non-mutated PD controls. GBA-mutated patients were younger than controls (59.9±8.1 vs. 64.3±7.2 years, p = 0.0257) and showed a more severe phenotype. Despite GBA-mutated patients reported more frequently symptoms suggestive of orthostatic hypotension (OH) than non-mutated patients (39.1% vs 6.5%, p = 0.001), the degree of cardiovascular autonomic dysfunction, when instrumentally assessed, did not differ between the two groups, showing the same prevalence of neurogenic OH, delayed OH and cardiovascular reflex impairment (pathological Valsalva maneuver). Conclusion: GBA-PD patients did not show different instrumental cardiovascular autonomic pattern than non-mutated PD. Our findings suggested that symptoms suggestive of OH should be promptly investigated by clinicians to confirm their nature and improve patient care and management.


Assuntos
Doenças do Sistema Nervoso Autônomo , Hipotensão Ortostática , Doença de Parkinson , Humanos , Masculino , Doenças do Sistema Nervoso Autônomo/diagnóstico , Doenças do Sistema Nervoso Autônomo/etiologia , Estudos de Casos e Controles , Glucosilceramidase/genética , Mutação , Doença de Parkinson/complicações , Doença de Parkinson/genética , Estudos Prospectivos
20.
J Med Case Rep ; 18(1): 102, 2024 Feb 20.
Artigo em Inglês | MEDLINE | ID: mdl-38374205

RESUMO

BACKGROUND: Cardiac autonomic neuropathy is a highly prevalent pathology in the diabetic population, and is the leading cause of death in this population. Orthostatic hypotension is the main clinical manifestation of the disease. In some patients, this orthostatic hypotension is associated with supine hypertension, posing a therapeutic challenge since treatment of one entity may aggravate the other. The challenge is to manage each of these two hemodynamic opposites without exposing the patient to a life-threatening risk of severe hypotension or hypertension. CASE PRESENTATION: We report a case of a 62-year-old ethnic Moroccan woman who has cardiovascular risk factors such as type 2 diabetes, arterial hypertension, and dyslipidemia. The patient's symptoms included dizziness, tremors, morning sickness, palpitations, and intolerance to exertion. Given her symptomatology, the patient benefited from an exploration of the autonomic nervous system through cardiovascular reactivity tests (Ewing tests), which confirmed the diagnosis of cardiac autonomic neuropathy. In addition to orthostatic hypotension, our patient had supine arterial hypertension, complicating management. To treat orthostatic hypotension, we advised the patient to avoid the supine position during the day, to raise the head of the bed during the night, and to have a sufficient fluid intake, with a gradual transition from decubitus to orthostatism and venous restraint of the lower limbs. Supine hypertension was treated with transdermal nitrates placed at bedtime and removed 1 hour before getting up. One week after the introduction of treatment, the patient reported a clear regression of functional symptoms, with an improvement in her quality of life. Improvement in symptomatology was maintained during quarterly follow-up consultations. CONCLUSIONS: Cardiac autonomic neuropathy is a very common pathology in diabetic patients. It is a serious condition with a life-threatening prognosis. Its management must be individualized according to the symptomatology and profile of each patient. The treatment of patients with orthostatic hypotension and supine hypertension requires special attention to ensure that each entity is treated without aggravating the other.


Assuntos
Doenças do Sistema Nervoso Autônomo , Diabetes Mellitus Tipo 2 , Hipertensão , Hipotensão Ortostática , Feminino , Humanos , Pessoa de Meia-Idade , Hipotensão Ortostática/complicações , Hipotensão Ortostática/terapia , Diabetes Mellitus Tipo 2/complicações , Qualidade de Vida , Hipertensão/complicações , Hipertensão/tratamento farmacológico , Doenças do Sistema Nervoso Autônomo/complicações , Doenças do Sistema Nervoso Autônomo/tratamento farmacológico , Sistema Nervoso Autônomo
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