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

PURPOSE: 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. METHODS: 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. RESULTS: 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 -10 mmHg 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, ß = -28.61), whereas age showed a positive relationship (age: P = 0.027, ß = 0.35). CONCLUSION: 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.

Sympathetic dysfunction dermatitis in a revascularised upper extremity after near-total amputation - A case report and review of literature.

Commonly reported complications after revascularisation or replantation of extremity are vascular thrombosis leading to complete or partial failure, bony non-union or malunion, joint stiffness and incomplete or abnormal sensory recovery. Sympathetic dysfunction dermatitis is an unreported complication after revascularisation or replantation surgery which results due to denervation of the extremity. We report a case of a young adult who developed eczematous dermatitis over the revascularised upper limb and discuss the role of sympathetic dysfunction in the development of these skin lesions. The patient was successfully treated with a short course of oral and topical steroids. Sympathetic dysfunction dermatitis is a rare form of skin eruptions occurring in the revascularised or replanted part of an extremity due to abnormal sympathetic function in the affected part.

123-Iodine MIBG in the Assessment of Sympathetic Denervation in Ogilvie's Syndrome.

123-Iodine metaiodobenzylguanidine (I-123 MIBG) imaging is frequently used in the assessment of sympathetic innervation and autonomic dysfunction in patients with cardiac failure, neurodegenerative Parkinson's syndrome, multiple system atrophy, myotonic dystrophy, and diabetic mellitus. The etiology of pseudo-obstruction remains unknown with likely imbalance between sympathetic and parasympathetic innervation proposed as a hypothesis. We present a case demonstrating the utility of I-123 MIBG scintigraphy for evaluating a case of pseudo-obstruction requiring frequent hospitalization due to progressive complex autoimmune neurological disorder.

A comparative study between preoperative and postoperative conventional autonomic functions in congenital craniovertebral junction anomalies.

Background: Autonomic nervous system (ANS) is invariably affected by craniovertebral junction (CVJ) anomalies. The usual presentation is sudden after trivial trauma. When symptomatic, most of this autonomic dysfunction is clearly elicited clinically with bedside tests. Nonetheless, ANS functionality in relatively less symptomatic or asymptomatic patients is not known as no studies exist. Methodology: We performed a longitudinal prospective study of 40 less symptomatic patients who underwent surgery with conventional autonomic function tests (AFT) in pre- and post-operative periods. Correlation of its association with such anomalies is studied. Results: All 40 had both pre- and post-operative clinical follow-up, pre-operative AFT, whereas only 22 patients had follow-up AFT. The mean age for the group was 32 years and male: female ratio was 2.3:1. Mean Nurick's grade was 1.8, whereas Barthel's index was 83.75%. Clinical improvement was seen in almost 98% at follow-up. Orthostatic test showed a significant association with Nurick's grade. Barthel's index was significantly associated with degree of compression. The mean follow-up was 17.4 months. Most conventional AFTs were significantly decreased in the preoperative period (P ≤ 0.01). Both parasympathetic and sympathetic tone improved on follow-up with better improvement later. Overall clinical involvement of ANS was seen in 22.5% whereas subclinical involvement in the form of AFT impairment was seen in 100%. Conclusion: There is a definite involvement of subclinical ANS in all patients of CVJ anomalies irrespective of their symptomatology. Knowing the extent of involvement in the preoperative period can help prognosticate, prioritize regarding surgery as well as correlate with the extent of improvement.

Combining Stellate ganglion block and intravenous regional anesthesia to treat complex regional pain syndrome: A 19-years' experience in a tertiary center.

OBJECTIVES: To study reduction in pain score after treatment with intravenous regional anesthesia (IVRA) and Stellate ganglion block (SGB) combination on complex regional pain syndrome (CPRS) patients and to quantify patients' satisfaction with treatment and occurrence of complications. METHODS: This is a record-based retrospective review carried out in 2020, targeting patients treated in the University of Jordan Hospital, Amman, Jordan, over the years 2002-2020. RESULTS: Among 99 patients, a significant drop in pain scores occurred in 88% of the patients' sample. Gender, age, type of CRPS, and duration of symptoms didn't affect statistical results. An average of 8.6 sessions needed to achieve 50% drop in pain score, and 2-3 sessions for first clinical improvement. Patients with previous application of plaster of Paris had increased success rates. CONCLUSION: We find it practical, inexpensive, safe, and straightforward to combine SGB with IVRA for CRPS patients.

Impact of dissociative experiences in migraine and its close relationship with osmophobia.

OBJECTIVE: Migraine is a complex episodic disease manifested by dysfunction of the sympathetic nervous system along with numerous neuropsychiatric symptoms. The aim of this study was to identify the dissociative symptoms with neurobiological similarities in episodic and chronic migraine patients and to evaluate their correlation with migraine frequency and severity of attacks. METHODS: The study included 61 episodic, 45 chronic migraine patients diagnosed using the criteria of the International Headache Society and 54 healthy control subjects. Dissociative Experiences Scale, Beck Anxiety Scale and Beck Depression Inventory were filled with the interviews. Demographic, clinical and headache characteristics of the patients were recorded according to migraine types. Results were analyzed by Kruskal-Wallis method and Spearman's correlation tests. RESULTS: Dissociative symptoms were more common in the patients with chronic migraine, and there was a statistically significant difference between the chronic migraine group and the episodic migraine and control groups (p = 0.001, p < 0.001). Dissociative experiences were correlated with depression and anxiety findings, and in both groups, there was a significant correlation between clinical characteristics of migraine and osmophobia in the controlled partial correlation analysis (p < 0.05). CONCLUSION: This study revealed that dissociative symptoms are more common especially in patients with chronic migraine and there is a significant association with osmophobia in both migraine groups. According to these data, we think that dissociative symptoms in chronic migraine patients will be questioned and osmophobia may be a guide in this regard.

Transient anisocoria after a traumatic cervical spinal cord injury: A case report.

Context: Autonomic dysreflexia and dysautonomia can be a common complication following spinal cord injury, especially at levels of T6 and above and can lead to complications associated with the pupil. In this case report, we review autonomic dysreflexia, the anatomy and physiology of the sympathetic nervous system of the eye, as well as review the clinical relevance in understanding autonomic and pupillary changes in response to autonomic dysfunction.Findings: We present a patient with an incomplete C4 level injury who was found to have isolated anisocoria on two separate occasions during his acute inpatient rehabilitation stay.Conclusion/Clinical Relevance: Anisocoria associated with abnormal sympathetic activation may be a clinical sign of dysautonomia.

The Goosebump Sign.

Nerve injury may occur following shoulder trauma. However, joint or bony damage can often dominate the clinical picture such that signs of nerve injury are overlooked. We describe the "goosebump sign" as a hitherto undescribed objective clinical finding of sympathetic dysfunction in peripheral nerve palsy, which can be a useful complementary sign to the standard assessment of muscle power and sensibility.

Cardiac Findings in Amyotrophic Lateral Sclerosis: A Magnetic Resonance Imaging Study.

The objective of this study was to investigate the potential involvement of cardiac structure and function by cardiac magnetic resonance (CMR) imaging in amyotrophic lateral sclerosis (ALS) patients. Our study included 35 patients with ALS without a history of cardiac disease and an age- and gender-matched healthy control group (n = 34). All subjects received a CMR in a 1.5-T whole-body scanner. Patients were also screened with Holter monitoring, echocardiography, and a blood test of cardiac markers. Myocardial mass in ALS hearts was reduced compared to the control group, and ejection volumes in the left and right heart were severely decreased in ALS patients, as shown by echocardiography and CMR. The myocardium showed increased T1 enhancement in 77% of the patients compared to 27% of controls (p = 0.0001). A trend toward late gadolinium enhancement patterns consistent with myocardial fibrosis was observed in 23.5% of the patients (9.1% of controls). Holter monitoring was normal in all patients as well as troponin T. Cardiac involvement seems to be present in ALS patients without clinical cardiac symptoms and with a normal cardiac routine assessment. Structural myocardial defects in CMR may be due to sympathetic dysfunction and may account for reported cardiac deaths in late-stage ALS patients.

Salivary alpha amylase not chromogranin A reflects sympathetic activity: exercise responses in elite male wheelchair athletes with or without cervical spinal cord injury.

BACKGROUND: Salivary alpha amylase (sAA) and chromogranin A (sCgA) have both been suggested as non-invasive markers for sympathetic nervous system (SNS) activity. A complete cervical spinal cord injury leading to tetraplegia is accompanied with sympathetic dysfunction; the aim of this study was to establish the exercise response of these markers in this in vivo model. METHODS: Twenty-six elite male wheelchair athletes (C6-C7 tetraplegia: N = 8, T6-L1 paraplegia: N = 10 and non-spinal cord injured controls: N = 8) performed treadmill exercise to exhaustion. Saliva and blood samples were taken pre, post and 30 min post exercise and analysed for sAA, sCgA and plasma adrenaline concentration, respectively. RESULTS: In all three subgroups, sAA and sCgA were elevated post exercise (P < 0.05). Whilst sCgA was not different between subgroups, a group × time interaction for sAA explained the reduced post-exercise sAA activity in tetraplegia (162 ± 127 vs 313 ± 99 (paraplegia) and 328 ± 131 U mL-1 (controls), P = 0.005). The post-exercise increase in adrenaline was not apparent in tetraplegia (P = 0.74). A significant correlation was found between adrenaline and sAA (r = 0.60, P = 0.01), but not between adrenaline and sCgA (r = 0.06, P = 0.79). CONCLUSIONS: The blunted post-exercise rise in sAA and adrenaline in tetraplegia implies that both reflect SNS activity to some degree. It is questionable whether sCgA should be used as a marker for SNS activity, both due to the exercise response which is not different between the subgroups and its non-significant relationship with adrenaline.

Autonomic dysreflexia: a cardiovascular disorder following spinal cord injury.

Autonomic dysreflexia (AD) is a serious cardiovascular disorder in patients with spinal cord injury (SCI). The primary underlying cause of AD is loss of supraspinal control over sympathetic preganglionic neurons (SPNs) caudal to the injury, which renders the SPNs hyper-responsive to stimulation. Central maladaptive plasticity, including C-fiber sprouting and propriospinal fiber proliferation exaggerates noxious afferent transmission to the SPNs, causing them to release massive sympathetic discharges that result in severe hypertensive episodes. In parallel, upregulated peripheral vascular sensitivity following SCI exacerbates the hypertensive response by augmenting gastric and pelvic vasoconstriction. Currently, the majority of clinically employed treatments for AD involve anti-hypertensive medications and Botox injections to the bladder. Although these approaches mitigate the severity of AD, they only yield transient effects and target the effector organs, rather than addressing the primary issue of central sympathetic dysregulation. As such, strategies that aim to restore supraspinal reinnervation of SPNs to improve cardiovascular sympathetic regulation are likely more effective for AD. Recent pre-clinical investigations show that cell transplantation therapy is efficacious in reestablishing spinal sympathetic connections and improving hemodynamic performance, which holds promise as a potential therapeutic approach.

Botulinum toxin for treatment of Harlequin syndrome.

We described a patient with Harlequin syndrome, a rare neurological condition, characterized by unilateral facial sweating and flushing, who had a good response to botulinum toxin application. She had been submitted to sympathectomy a few years, however she still complained of excessive sweating in the regions mentioned and intense flushing.

Arterial Compliance and Autonomic Functions in Adult Male Smokers.

INTRODUCTION: Smoking is known to augment sympathetic activity and may lead to increased arterial stiffness. Several studies have reported association of increased sympathetic activity and arterial stiffness to cardiovascular risks among smokers. Pulse Wave Velocity (PWV) of peripheral arteries, instead of aorta can be used as a non-invasive indicator of arterial stiffness. AIM: To measure non-invasively, the autonomic functions and peripheral arterial stiffness in smokers, and to find out whether the aforementioned factors are modified by the level of physical activity in these smokers. MATERIALS AND METHODS: This cross-sectional analytical study was conducted in the Department of Physiology, HIMS, Dehradun, over a period of 12 months (2013-2014) on 100 adult males (20-40 years); 50 smokers and 50 non-smokers. The parameters analysed include relevant anthropometric and cardiovascular parameters, Pulse Wave Velocity (PWV), sustained Hand Grip Test (HGT) and Heart Rate Variability (HRV) domains. Data interpretation and analysis was carried out using SPSS 17.0. Comparison of the above mentioned parameters amongst groups was done with unpaired t-test. The relationship of pack-years & physical activity with vascular functions was assessed by Pearson's correlation. Interaction of various grades of smoking and physical activity with Cardiovascular System (CVS) parameters was assessed by one-way ANOVA. RESULTS: Smokers had higher values of PWV (5.7±0.5m/s) as compared to non-smokers (4.8±0.4m/s) (p<0.001). ΔDBP during HGT was lower (7±3.18mmHg) among smokers as compared to non-smokers (19.4±3.5mmHg) (p<0.001). Smoking (pack-years) was positively related to PWV (r= .03) but showed a weak negative relationship with change in Diastolic Blood Pressure (ΔDBP) (r= -0.084, p=0.56) showing that, more the frequency of smoking, the more was arterial stiffening and the lesser was the sympathetic response to the HGT. The smokers had significantly higher sympathetic activity; Low Frequency (LF) & Low Frequency: High Frequency ratio (LF: HF) (p<.001) whereas High Frequency (HF) was significantly lower (p<.001) showing a decline in parasympathetic activity. CONCLUSION: Smokers demonstrated higher peripheral PWV and higher intrinsic sympathetic activity and this increase in intrinsic sympathetic activity may lead to increased arterial stiffness. Interaction of autonomic function and PWV with levels of physical activity and grades of smoking showed no significant differences, suggesting the fact that increased physical activity or reduced smoking may not have any effect on the endothelial dysfunction or CVS morbidity caused by smoking.

Circadian Homeostasis of Liver Metabolism Suppresses Hepatocarcinogenesis.

Chronic jet lag induces spontaneous hepatocellular carcinoma (HCC) in wild-type mice following a mechanism very similar to that observed in obese humans. The process initiates with non-alcoholic fatty liver disease (NAFLD) that progresses to steatohepatitis and fibrosis before HCC detection. This pathophysiological pathway is driven by jet-lag-induced genome-wide gene deregulation and global liver metabolic dysfunction, with nuclear receptor-controlled cholesterol/bile acid and xenobiotic metabolism among the top deregulated pathways. Ablation of farnesoid X receptor dramatically increases enterohepatic bile acid levels and jet-lag-induced HCC, while loss of constitutive androstane receptor (CAR), a well-known liver tumor promoter that mediates toxic bile acid signaling, inhibits NAFLD-induced hepatocarcinogenesis. Circadian disruption activates CAR by promoting cholestasis, peripheral clock disruption, and sympathetic dysfunction.

Successful treatment of severe sympathetically maintained pain following anterior spine surgery.

Sympathetic dysfunction is one of the possible complications of anterior spine surgery; however, it has been underestimated as a cause of complications. We report two successful experiences of treating severe dysesthetic pain occurring after anterior spine surgery, by performing a sympathetic block. The first patient experienced a burning and stabbing pain in the contralateral upper extremity of approach side used in anterior cervical discectomy and fusion, and underwent a stellate ganglion block with a significant relief of his pain. The second patient complained of a cold sensation and severe unexpected pain in the lower extremity of the contralateral side after anterior lumbar interbody fusion and was treated with lumbar sympathetic block. We aimed to describe sympathetically maintained pain as one of the important causes of early postoperative pain and the treatment option chosen for these cases in detail.

Increase of angiotensin-converting enzyme activity and peripheral sympathetic dysfunction could contribute to hypertension development in streptozotocin-induced diabetic rats.

Diabetes augments the risk of hypertension. Although several factors have been implicated in the development of such hypertensive state, we designed this study to investigate blood pressure development, the activity of angiotensin-converting enzyme (ACE) in blood as well as sympathetic neurotransmission in the vas deferens of diabetic rats. We used streptozotocin (STZ)-induced diabetic rats (60 mg/kg) in order to evaluate the systolic blood pressure (SBP), ACE activity and peripheral sympathetic neurotransmission. We observed the following changes of parameters: increase of SBP, decrease of heart rate, augmentation of plasma ACE activity, enhancement of phasic and tonic vas deferens contractions elicited by electrical stimulation at 5 Hz, increase of maximal response to noradrenaline (NA) and decrease of adenosine triphosphate (ATP)-elicited contraction of vasa deferentia. The results reveal that in the development of hypertension in diabetic rats, augmentation of circulating ACE activity precedes the sympathetic dysfunction. Additionally, it seems that the purinergic and noradrenergic neurotransmission is compromised.

An achondroplasic case with foramen magnum stenosis, hydrocephaly, cortical atrophy, respiratory failure and sympathetic dysfunction.

BACKGROUND: Achondroplasia is a relatively frequent genetic disorder that may lead to limb weakness, motor-mental retardation, hydrocephaly, and respiratory disorders. In this pathology, foramen magnum stenosis and accompanying disorders like respiratory depression is well documented. CASE PRESENTATION: A 2.5 year-old child with the diagnosis of achondroplasia admitted to our clinic with severe respiratory depression, limb weakness, and motor mental retardation as well as sympathetic dysfunction. In radiologic evaluations, severe foramen magnum stenosis was detected. The patient was operated and posterior fossa decompression was accomplished to prevent compression of respiratory centers and neurons. CONCLUSION: This case is unique with the narrowest foramen magnum reported up to date and the sympathetic dysfunction which is not reported as a complication in achondroplasic patients. The authors review the relevant literature, focusing on the indications for cervicomedullary decompression in infants with achondroplasia.

Successful Treatment of Severe Sympathetically Maintained Pain Following Anterior Spine Surgery

Sympathetic dysfunction is one of the possible complications of anterior spine surgery; however, it has been underestimated as a cause of complications. We report two successful experiences of treating severe dysesthetic pain occurring after anterior spine surgery, by performing a sympathetic block. The first patient experienced a burning and stabbing pain in the contralateral upper extremity of approach side used in anterior cervical discectomy and fusion, and underwent a stellate ganglion block with a significant relief of his pain. The second patient complained of a cold sensation and severe unexpected pain in the lower extremity of the contralateral side after anterior lumbar interbody fusion and was treated with lumbar sympathetic block. We aimed to describe sympathetically maintained pain as one of the important causes of early postoperative pain and the treatment option chosen for these cases in detail.

MMPI Analysis of Patients with Essential Hyperhisrosis / 대한통증학회지

BACKGROUND: Although the cause of hyperhidrosis has not been the subject of close investigation, there are cases for which excessive sweating happens at the hands, feet and armpits due to hyperactivity of the sympathetic nervous system. This usually occurs in people less than 25 years old and it often causes difficulties for their social and occupational life and there is a decrease in the quality of life. Therefore, this should also be examined according to the mental state of the patient who suffers from hyperhidrosis. METHODS: The Minnesota Multiple Personality Inventory was administered to 59 patients in the Hyperhidrosis Center from March, 2006 to March, 2007. The MMPI's validity and 10 clinic standards were analyzed. The results were compared according to gender and age. RESULTS: Of the standard clinical items, psychopathy and conversion hysteria were 21.1% and 17.5%, respectively. On comparison between males (47.5%) and females (52.5%), the females had a higher score for the hypochondriasis item. The patients above 18 years old had a significantly higher level of hypochondriasis and hypomania compared to the patients below 18 years old. CONCLUSIONS: When analyzing the personality of the patients with essential hyperhidrosis with using the MMPI, it was difficult to look for relations with the mental factor. Therefore, it is necessary to develop diagnostic tests for younger people with considering the relations with the period of morbidity.

Hemodynamic Study of Foot in Diabetic Patient Using Portable Doppler Machine

The importance of neuropathy in the pathogenesis of foot lesions has been well recognized in diabetes. Blood flow in ischemic limbs has been extensively investigated but the circulation of limbs affected by peripheral neu ropathy has received little attention. Some studies on blood flow in peripheral neuropathy have shown a remarkable increase in resting flow, transcutaneous venous oxygen tension, and vascularity, along with loss of the spontaneous variations which occur normally with sympathetic activity of the foot in patients with diabetes. The aim of present study is to find out the effects of somatic and autonomic nervous function in early change of blood flow of foot in diabetic patients, We have studied fifty-one patients of non-insulin-dependent(type II) diabetes with no history of hypertension of diabetic foot ulcers. The evidence of neuropathy was screened by nerve conduction studies and sympathetic skin response of both lower extremities. Blood flow of dorsalis pedls and posterior tibial arteries was measured by portable doppler machine and presented as pressure index(ankle-to-arm systolic pressure ratio). The patients with sympathetic dysfunction showed significant decrease in pressure index compared to normal control and diabetic patients with normal sympathetic function, suggesting that changes of the blood flow occur in diabetic patients with sympathetic dysfunction.