Involvement Of Autonomic Nervous System Research Articles

Autonomic Dysfunction in Amyotrophic Lateral Sclerosis – A Case-Control Study

Introduction. This study aimed to explore autonomic nervous system involvement in amyotrophic lateral sclerosis (ALS) pa- tients by evaluating sympathetic skin response (SSR). Materials and Methods. The study included 35 sporadic (ALS) patients (cases), and 35 healthy age and sex-matched participants (controls) aged <60 years. SSR was recorded in the electrophysiology lab of the Neurology Department of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. Patients with diseases associated with peripheral or autonomic neuropathy were excluded. Prolonged latency (delayed SSR) or an ab- sent response was considered abnormal SSR. Results. SSR was found to be abnormal in 17 (48.6 %) ALS cases, with an absent response in the upper limbs of six cases (17.1%). Abnormal SSR was more prevalent in the lower limbs, with 33 (94.3%) and 20 (57.1%) cases having a delayed or absent response, respectively. In comparison, SSR was normal in all control participants (P-value <0.05). Abnormal SSR was significantly more common in the lower limbs of ALS cases with bulbar palsy than those without bulbar palsy (P-value=0.04). There was no association of SSR with disease severity and duration. Conclusion. ALS is significantly associated with abnormal SSR, indicating autonomic nervous system involvement. There could also be an associa- tion between bulbar palsy and abnormal SSR among ALS patients. Further studies should be carried out to determine the as- sociation of abnormal SSR with disease severity, duration, and type.

Sympathetic dysfunction as an early indicator of autonomic involvement in Parkinson's disease.

The specific characteristics of autonomic involvement in patients with early Parkinson's disease (PD) are unclear. This study aimed to evaluate the characteristics of autonomic dysfunction in drug-naïve patients with early-stage PD without orthostatic hypotension (OH) by analyzing Valsalva maneuver (VM) parameters. We retrospectively analyzed drug-naïve patients without orthostatic hypotension (n = 61) and controls (n = 20). The patients were subcategorized into early PD (n = 35) and mid-PD (n = 26) groups on the basis of the Hoehn and Yahr staging. VM parameters, including changes in systolic blood pressure at late phase 2 (∆SBPVM2), ∆HRVM3, Valsalva ratio (VR), pressure recovery time, adrenergic baroreflex sensitivity, and vagal baroreflex sensitivity, were assessed. In the early PD group, ∆SBPVM2, a marker of sympathetic function, was significantly lower compared with that in controls (risk ratio = 0.95, P = 0.027). Receiver operating characteristic (ROC) curve analysis showed an optimal cut-off value of -10mmHg for ∆SBPVM2 [P = 0.002, area under the curve (AUC): 0.737]. VR exhibited an inverse relationship with Unified Parkinson's Disease Rating Scale Part 3 scores in the multivariable regression analysis (VR: P = 0.038, β = -28.61), whereas age showed a positive relationship (age: P = 0.027, β = 0.35). The ∆BPVM2 parameter of the VM may help detect autonomic nervous system involvement in early-PD without OH. Our results suggest that sympathetic dysfunction is an early manifestation of autonomic dysfunction in patients with PD.

The role of the autonomic nervous system in the development of hypotonic dysphonia

Background. Hypotonic dysphonia occupies a special place in the structure of functional dysphonia in terms of prevalence. At the same time, the autonomic nervous system has a significant impact on the course and outcome of the disease. However, data on the neurovegetative status of patients with hypotonic dysphonia in the scientific literature are extremely rare and do not fully reflect its features.Aim. To determine the neurovegetative status of laryngological patients with hypotonic dysphonia.Materials and methods. 26 patients (6 men and 20 women) with hypotonic dysphonia and hypersensitivity of the larynx (main group) and 45 patients (13 men and 32 women) with hypersensitivity of the larynx without signs of dysphonia (comparison group) were examined as a control, data on the functional state of the autonomic nervous system in 20 practically healthy people (5 men and 15 women) aged 18 to 25 years were used. The state of vegetative parameters – vegetative tone, vegetative reactivity and vegetative activity support – were evaluated using the medical diagnostic complex “Valenta”. The examination of patients included anamnestic analysis, objective examination of lororgans according to generally accepted methods and video stroboscopy of the larynx.Results. The differences between healthy and sick people regarding neurovegetative indicators are obvious, unidirectional and indicate the existence of autonomic disorders in both groups of patients. The ratios of vegetative parameters recorded during the study indicate the involvement of autonomic nervous system not only in the development of laryngeal hypersensitivity, but also in the pathogenesis of functional dysphonia of the hypotonic type.Conclusion. Thus, neurovegetative disorders – vegetative dystonia and autonomic dysfunction – are one of the factors of the pathogenesis of hypotonic dysphonia.

Dysautonomia in Amyotrophic Lateral Sclerosis.

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.

Autonomic dysfunction in systemic sclerosis: A scoping review

IntroductionOver the years several lines of evidence have implied a pathological involvement of autonomic nervous system (ANS) in systemic sclerosis (SSc). However, the relationship between autonomic dysfunction and SSc is not yet fully understood. The aims of this scoping review were to map the research done in this field and inform future research to investigate pathogenic hypotheses of ANS involvement. MethodsWe performed a scoping review of publications collected through a literature search of MEDLINE and Web of Science databases, looking for dysautonomia in SSc. We included original data from papers that addressed ANS involvement in SSc regarding pathogenesis, clinical presentation and diagnostic tools. Results467 papers were identified, 109 studies were selected to be included in the present review, reporting data from a total of 2742 SSc patients. Cardiovascular system was the most extensively investigated, assessing heart rate variability with 24 h HolterECG or Ewing's autonomic tests. Important signs of dysautonomia were also found in digital vasculopathy, gastrointestinal system and SSc skin, assessed both with non-invasive techniques and histologically. Research hypotheses mainly regarding the relationship between sympathetic system – ischemia and the role of neurotrophins were then developed and discussed. ConclusionWe described the currently available evidence on pathogenesis, clinical presentation and diagnostic assessment of dysautonomia in SSc patients. A strong influence of ANS deregulation on SSc clearly emerges from the literature. Future research is warranted to clarify the mechanisms and timing of autonomic dysfunction in SSc.

Idiopathic cervical dystonia and non-motor symptoms: a pilot case-control study on autonomic nervous system

PurposeNon-motor symptoms, such as sleep disturbances, fatigue, neuropsychiatric manifestations, cognitive impairment, and sensory abnormalities, have been widely reported in patients with idiopathic cervical dystonia (ICD). This study aimed to clarify the autonomic nervous system (ANS) involvement in ICD patients, which is still unclear in the literature.MethodsWe conducted a pilot case-control study to investigate ANS in twenty ICD patients and twenty age-sex-matched controls. The Composite Autonomic System Scale 31 was used for ANS clinical assessment. The laser Doppler flowmetry quantitative spectral analysis, applied to the skin and recorded from indices, was used to measure at rest, after a parasympathetic activation (six deep breathing) and two sympathetic stimuli (isometric handgrip and mental calculation), the power of high-frequency and low-frequency oscillations, and the low-frequency/high-frequency ratio.ResultsICD patients manifested higher clinical dysautonomic symptoms than controls (p < 0.05). At rest, a lower high-frequency power band was detected among ICD patients than controls, reaching a statistically significant difference in the age group of ≥ 57-year-olds (p < 0.05). In the latter age group, ICD patients showed a lower low-frequency/high-frequency ratio than controls at rest (p < 0.05) and after mental calculation (p < 0.05). Regardless of age, during handgrip, ICD patients showed (i) lower low-frequency/high-frequency ratio (p < 0.05), (ii) similar increase of the low-frequency oscillatory component compared to controls, and (iii) stable high-frequency oscillatory component, which conversely decreased in controls. No differences between the two groups were detected during deep breathing.ConclusionICD patients showed ANS dysfunction at clinical and neurophysiological levels, reflecting an abnormal parasympathetic-sympathetic interaction likely related to abnormal neck posture and neurotransmitter alterations.

Heart-rate variability as a new marker for freezing predisposition in Parkinson's disease

IntroductionFreezing of gait (FoG) is a debilitating symptom of advanced Parkinson's disease (PD) characterized by a sudden, episodic stepping arrest despite the intention to continue walking. The etiology of FoG is still unknown, but accumulating evidence unraveled physiological signatures of the autonomic nervous system (ANS) around FoG episodes. Here we aim to investigate for the first time whether detecting a predisposition for upcoming FoG events from ANS activity measured at rest is possible. MethodsWe recorded heart-rate for 1-min while standing in 28 persons with PD with FoG (PD + FoG), while OFF, and in 21 elderly controls (EC). Then, PD + FoG participants performed walking trials containing FoG-triggering events (e.g., turns). During these trials, n = 15 did experience FoG (PD + FoG+), while n = 13 did not (PD + FoG-). Most PD participants (n = 20: 10 PD + FoG+ and 10 PD + FoG-) repeated the experiment 2–3 weeks later, while ON, and none experienced FoG. We then analyzed heart-rate variability (HRV), i.e., the fluctuations in time intervals between adjacent heartbeats, mainly generated by brain-heart interactions. ResultsDuring OFF, HRV was significantly lower in PD + FoG + participants, reflecting imbalanced sympathetic/parasympathetic activity and disrupted self-regulatory capacity. PD + FoG- and EC participants showed comparable (higher) HRV. During ON, HRV did not differ among groups. HRV values did not correlate with age, PD duration, levodopa consumption, nor motor -symptoms severity scores. ConclusionsOverall, these results document for the first time a relation between HRV at rest and FoG presence/absence during gait trials, expanding previous evidence regarding the involvement of ANS in FoG.

Autonomic nervous system involvement in chronic inflammatory demyelinating polyradiculoneuropathy: a literature review.

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). PubMed and Web of Science were searched for studies reporting AD in CIDP. 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. 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.

CARDIOVASCULAR AUTONOMIC DYSFUNCTION IN HIV-POSITIVE PATIENTS AND ITS CORRELATION WITH CD4 CELL COUNT

According to Global human immunodeciency virus and acquired immunodeciency disease syndrome statistics, 38.4 million(33.9 million-43.8 million) people live globally with HIV in 2021, and 1.5 million people became newly infected with HIV in 2021(1). As per India HIV estimation report 2020, national adult (15–49 years) HIV prevalence was estimated at 0.22% (0.17%–0.29%) in 2020, 0.23% (0.18%–0.31%) among males and 0.20% (0.15%– 0.26%) among females(2). HIV is a condition which can progressively affect almost all body organs and systems due to immune system failure. Infection with HIV is well known to cause peripheral neuropathy and other neurological complication thus affecting the quality of life(3). Autonomic nervous system involvement can affect heart rate, vasomotor tone, sexual function, gastrointestinal motility, urination, thermoregulation and production of tears and saliva. This can lead to diverse symptoms like postural syncope, impotence, sweating, diarrhea/constipation, dry eyes and mouth, urinary incontinence and changes in skin temperature. Abnormalities in cardiac autonomic function occur at all CD4 levels(4,5). The aim of our study is to assess autonomic dysfunction in HIV-positive patients and to show the correlation between decreasing CD4 + T cell counts with increasing severity of disease in the form of autonomic dysfunction.

Decreased expression of S100A8/A9 in V30M related ATTRv amyloidosis

Introduction Hereditary Transthyretin Amyloidosis is a rare, progressive and life-threatening systemic disease with predominant peripheral and autonomic nervous system involvement caused by mutation of the transthyretin protein. The most common TTR mutation regarding to ATTRv is a substitution of a Methionine for a Valine at position 30 that predisposes TTR to form aggregates and fibrils. Methods S100A8 protein levels were measured in plasma samples from ATTRV30M patients and healthy donors. Additionally, S100A8/9 levels were measured in Schwann cells after incubation with human WT or V30M TTR. Moreover, bone marrow derived macrophages of either genetic background were generated and the expression of S100A8/9 was measured in response to toll like receptors agonists. Results S100A8/A9 mRNA levels are decreased in HSF V30M mice as compared with the WT. Moreover, S100A8 protein levels were found downregulated in plasma samples from ATTRV30M patients. Furthermore, we provide evidence for a dysregulated S100 expression by Schwann cells in response to TTRV30M and by mutated macrophages in response to toll like receptors agonists. Conclusion The presence of TTRV30M impacts S100 expression, possibly contributing to the impaired immune activation of Schwann cells in nerves from ATTRV30M patients. This may be linked to the diminished immune cellular infiltration in these nerves, contributing in this way for the neuronal dysfunction present in the disease.

Sympathetic Denervation and Pharmacological Stimulation of Parasympathetic Nervous System Prevent Pulmonary Vascular Bed Remodeling in Rat Model of Chronic Thromboembolic Pulmonary Hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) develops in 1.5-2.0% of patients experiencing pulmonary embolism (PE) and is characterized by stable pulmonary artery obstruction, heart failure, and poor prognosis. Little is known about involvement of autonomic nervous system (ANS) in the mechanisms of CTEPH. This study was aimed at evaluation of the effect of vagal and sympathetic denervation, as well as stimulation of the parasympathetic nervous system, on the outcomes of CTEPH in rats. CTEPH was induced by multiple intravenous injections of alginate microspheres. Sympathetic and vagal denervation was performed using unilateral surgical ablation of the stellate ganglion and vagotomy, respectively. Stimulation of the parasympathetic nervous system was carried out by administering pyridostigmine. The effect of neuromodulatory effects was assessed in terms of hemodynamics, histology, and gene expression. The results demonstrated the key role of ANS in the development of CTEPH. Sympathetic denervation as well as parasympathetic stimulation resulted in attenuated pulmonary vascular remodeling. These salutary changes were associated with altered MMP2 and TIMP1 expression in the lung and decreased FGFb level in the blood. Unilateral vagotomy had no effect on physiological and morphological outcomes of the study. The data obtained contribute to the identification of new therapeutic targets for CTEPH treatment.

Effect of Age on Heart Rate Variability in Patients with Mitral Valve Prolapse: An Observational Study

Age is an important determinant of heart rate variability (HRV) in healthy individuals. The incidence of arrhythmia is high in patients with mitral valve prolapse (MVP). However, the correlation of HRV in patients with MVP in different age groups is not well established. We presumed that increasing age would be prospectively associated with declining HRV measurement in MVP. Sixty patients with MVP and 120 control individuals were included and underwent 24 h HRV analysis. No significant difference was found in all parameters calculated in the time domain or in the frequency domain between the two groups. However, as patients' age increased, a significant time domain (SDNN, RMSSD, NN50, and pNN50) decline was found in the MVP group, but not in the control group. This suggests that patients with MVP may have autonomic nervous system involvement that increases the risk of arrhythmia and heart disease with increasing age.

Quantitative gastrointestinal function and corresponding symptom profiles in autonomic neuropathy.

Peripheral neuropathies with autonomic nervous system involvement are a recognized cause of gastrointestinal dysmotility for a wide spectrum of diseases. Recent advances in wireless motility capsule testing allow improved sampling of regional and whole gut motility to aid in the diagnosis of gastrointestinal motility disorders and may provide additional insight into segment-specific enteric involvement of peripheral neuropathies affecting autonomic nervous system function. We utilized standardized autonomic nervous system (ANS) reflex assessment and wireless motility capsule testing to evaluate 20 individuals with idiopathic autonomic neuropathy and unexplained gastrointestinal symptoms. Additionally, we examined the relationship between quantifiable autonomic neuropathy and gastrointestinal dysmotility at specific neuroanatomical levels. Symptom profiles were evaluated using the 31-item Composite Autonomic Symptom Score questionnaire (COMPASS-31) and compared to wireless motility capsule data. We found that transit times were predominately abnormal (delayed) in the foregut (10 of 20; 50%), while contractility abnormalities were far more prominent in the hindgut (17 of 20; 85%), and that motility and symptom patterns, as assessed by the COMPASS-31 GI domain items, generally corresponded. Finally, we also found that there was neuroanatomical overlap in the presence of autonomic reflex abnormalities and WMC-based transit and/or contractility abnormalities. We found that transit times were predominately abnormal in the foregut and midgut, while contractility abnormalities were far more prominent in the hindgut in individuals with idiopathic autonomic neuropathy. There was a high rate of agreement in segmental wireless motility capsule data with neuroanatomically corresponding standardized ANS function measures (e.g., cardiovagal, sudomotor, adrenergic). Expanded sudomotor testing, including additional neuroanatomical segments, could provide additional indirect assessment of visceral involvement in ANS dysfunction.

Autonomic nervous system dysfunction in Prader-Willi syndrome.

Prader-Willi syndrome is a complex neurodevelopmental genetic disorder due to lack of paternal expression of critical imprinted genes in the 15q11.2-q13.1 chromosomal region, generally from a paternal deletion. Predominant features include infantile hypotonia, a poor suck with failure to thrive, craniofacial features, and developmental and behavioral problems including self-injury and childhood onset of obesity. In addition to severe obesity, patients with PWS present with other symptoms of autonomic nervous system dysfunction. We examined the features seen in Prader-Willi syndrome and searched the literature for evidence of autonomic nervous system involvement in this rare obesity-related disorder and illustrative findings possibly due to autonomic nervous system dysfunction. Additionally, we reviewed the literature in relation to childhood obesity syndromes and compared those syndromes to the syndromic obesity found in Prader-Willi syndrome. We report autonomic nervous system-related symptoms associated with childhood obesity impacting features seen in Prader-Willi syndrome and possibly other obesity-related genetic syndromes. We compiled evidence of both an autonomic route for the obesity seen in PWS and other autonomic nervous system-related dysfunctions. These include decreased salvation, sleep disordered breathing, increased pain and thermal threshold instability, delayed gastric emptying, altered blood pressure readings, and pupillary constriction responses as evidence of autonomic nervous system involvement. We summarized and illustrated findings of autonomic nervous system dysfunction in Prader-Willi syndrome and other obesity-related syndromes and genetic factors that may play a causative role in development.

Brain monoamine vesicular transport disease caused by homozygous SLC18A2 variants: A study in 42 affected individuals

PurposeBrain monoamine vesicular transport disease is an infantile-onset movement disorder that mimics cerebral palsy. In 2013, the homozygous SLC18A2 variant, p.Pro387Leu, was first reported as a cause of this rare disorder, and dopamine agonists were efficient for treating affected individuals from a single large family. To date, only 6 variants have been reported. In this study, we evaluated genotype–phenotype correlations in individuals with biallelic SLC18A2 variants. MethodsA total of 42 affected individuals with homozygous SLC18A2 variant alleles were identified. We evaluated genotype–phenotype correlations and the missense variants in the affected individuals based on the structural modeling of rat VMAT2 encoded by Slc18a2, with cytoplasm- and lumen-facing conformations. A Caenorhabditis elegans model was created for functional studies. ResultsA total of 19 homozygous SLC18A2 variants, including 3 recurrent variants, were identified using exome sequencing. The affected individuals typically showed global developmental delay, hypotonia, dystonia, oculogyric crisis, and autonomic nervous system involvement (temperature dysregulation/sweating, hypersalivation, and gastrointestinal dysmotility). Among the 58 affected individuals described to date, 16 (28%) died before the age of 13 years. Of the 17 patients with p.Pro237His, 9 died, whereas all 14 patients with p.Pro387Leu survived. Although a dopamine agonist mildly improved the disease symptoms in 18 of 21 patients (86%), some affected individuals with p.Ile43Phe and p.Pro387Leu showed milder phenotypes and presented prolonged survival even without treatment. The C. elegans model showed behavioral abnormalities. ConclusionThese data expand the phenotypic and genotypic spectra of SLC18A2-related disorders.

Study of sympathetic skin response in patients with COVID-19 infection.

ObjectivesMany articles hypothesized the potential role of autonomic nervous system in the pathogenesis and outcome of COVID-19 infection. Several studies reported both central and peripheral nervous system involvement in COVID-19 as well. Up to our knowledge, there is no study evaluating whether this virus could invade the autonomic nervous system affecting its function adversely. Sympathetic skin response (SSR) has long been used as a method of evaluating the autonomic nervous system. Regarding the importance of the autonomic nervous system in hemostasis and wide consequences of COVID-19 infection, we designed this study to evaluate the autonomic nervous system function in patients recovered from COVID-19 compared with normal population who are not yet infected by this virus by the means of SSR. MethodsThis case–control study included 70 patients surviving COVID-19 who met the inclusion and exclusion criteria that went under SSR. The data gathered were compared with those without the history of any symptoms attributable to COVID-19 during the pandemic.ResultsThere was a correlation between COVID-19 infection and abnormal SSR (p value < 0.0001) with the most effect on the latency prolongation of the action potential recorded from the median nerve at palms (effect size: right: 3.90, left: 3.69). Moreover, the greater severity of the disease correlated with more abnormality of parameters recorded by SSR technique.ConclusionsAbnormal SSR parameters could be a good indicator of autonomic nervous system involvement in patients with COVID-19 infection. It might be a predictor of disease severity, clinical outcomes and prognosis as well.

Autonomic Dysfunction during Acute SARS-CoV-2 Infection: A Systematic Review

Although autonomic dysfunction (AD) after the recovery from Coronavirus disease 2019 (COVID-19) has been thoroughly described, few data are available regarding the involvement of the autonomic nervous system (ANS) during the acute phase of SARS-CoV-2 infection. The primary aim of this review was to summarize current knowledge regarding the AD occurring during acute COVID-19. Secondarily, we aimed to clarify the prognostic value of ANS involvement and the role of autonomic parameters in predicting SARS-CoV-2 infection. According to the PRISMA guidelines, we performed a systematic review across Scopus and PubMed databases, resulting in 1585 records. The records check and the analysis of included reports’ references allowed us to include 22 articles. The studies were widely heterogeneous for study population, dysautonomia assessment, and COVID-19 severity. Heart rate variability was the tool most frequently chosen to analyze autonomic parameters, followed by automated pupillometry. Most studies found ANS involvement during acute COVID-19, and AD was often related to a worse outcome. Further studies are needed to clarify the role of autonomic parameters in predicting SARS-CoV-2 infection. The evidence emerging from this review suggests that a complex autonomic nervous system imbalance is a prominent feature of acute COVID-19, often leading to a poor prognosis.

The omnipresence of autonomic modulation in health and disease.

The Autonomic Nervous System (ANS) is a critical part of the homeostatic machinery with both central and peripheral components. However, little is known about the integration of these components and their joint role in the maintenance of health and in allostatic derailments leading to somatic and/or neuropsychiatric (co)morbidity. Based on a comprehensive literature search on the ANS neuroanatomy we dissect the complex integration of the ANS: (1) First we summarize Stress and Homeostatic Equilibrium - elucidating the responsivity of the ANS to stressors; (2) Second we describe the overall process of how the ANS is involved in Adaptation and Maladaptation to Stress; (3) In the third section the ANS is hierarchically partitioned into the peripheral/spinal, brainstem, subcortical and cortical components of the nervous system. We utilize this anatomical basis to define a model of autonomic integration. (4) Finally, we deploy the model to describe human ANS involvement in (a) Hypofunctional and (b) Hyperfunctional states providing examples in the healthy state and in clinical conditions.

Sympathetic skin responses and electromyographic reaction times in chronic autoimmune thyroiditis: An overlooked electrodiagnostic study.

Objectives This study aims to evaluate peripheral nerve functions, sympathetic skin responses (SSRs), and electromyographic (EMG) reaction times in hypothyroid patients and to compare them to healthy individuals. Patients and methods Between January 2007 and September 2007, a total of 54 patients with Hashimoto’s thyroiditis including 35 euthyroid (3 males, 32 females; mean age: 45.2±10.2 years; range, 35 to 60 years) and 19 with subclinical hypothyroidism (2 males, 17 females; mean age: 43.2±12.6 years; range, 40 to 65 years) were included in the study. The control group consisted of 35 healthy individuals (5 males, 30 females; mean age: 39.1±9.3 years; range, 29 to 52 years). Nerve conduction studies (NCSs), SSRs of the hand and foot obtained by stimulation of the contralateral median nerve, and EMG reaction times of the extensor indicis proprius muscle were performed in all subjects. Results There was no significant difference in peripheral NCSs and SSRs between patients and the control group. However, reaction times were longer in the AIT patients compared to the healthy individuals suggesting alterations in cognitive function related to the primary disease process in AIT. Conclusion Electrodiagnosis of autonomic nervous system involvement and cognitive impairment can be challenging in AIT. However, EMG reaction times and SSRs are practical and useful tools that are often overlooked. On the other hand, SSRs may be combined with more quantitative tests, such as sudomotor axon reflex testing, to allow us to better determine the extent of involvement of the autonomic nervous system in AIT.

Heart Rate Variability during Wake and Sleep in Huntington’s Disease Patients: An Observational, Cross-Sectional, Cohort Study

Introduction: Autonomic dysfunction has been reported as one of nonmotor manifestations of both presymptomatic and manifest Huntington’s disease (HD). The aim of our study was to evaluate heart rate variability (HRV) during wake and sleep in a cohort of patients with manifest HD. Methods: Thirty consecutive patients with manifest HD were enrolled, 14 men and 16 women, mean age 57.3 ± 12.2 years. All patients underwent full-night attended video polysomnography. HRV was analyzed during wake, NREM sleep, and REM sleep, in time and frequency domain. Results were compared with a control group of healthy volunteers matched for age and sex. Results: During wake, HD patients presented significantly higher mean heart rate than controls (72.4 ± 9.6 vs. 58.1 ± 7.3 bpm; p < 0.001). During NREM sleep, HD patients showed higher mean heart rate (65.6 ± 11.1 vs. 48.8 ± 4.6 bpm; p < 0.001) and greater low frequency (LF) component of HRV (52.9 ± 22.6 vs. 35.5 ± 17.3 n.u.; p = 0.004). During REM sleep, we observed lower standard deviation of the RR interval in HD subjects (3.4 ± 2.2 vs. 3.7 ± 1.3 ms; p = 0.015). Conclusion: Our results show that HD patients have higher heart rate than controls, during wake and NREM, but not during REM sleep. Among HRV parameters, the most relevant difference regarded the LF component, which reflects, at least partially, the ortho-sympathetic output. Our results confirm the involvement of autonomic nervous system in HD and demonstrate that it is evident during both wake and sleep.