Evaluation of autonomic nervous system in children with spastic cerebral palsy: clinical and electophysiological study

Background Cerebral palsy (CP) is the most prevalent severe motor disability among children. The aim of this work was to assess autonomic dysfunction in children with cerebral palsy clinically and electrophysiologically. Results Age of the studied children ranged from 4 to 12 years. Quadriplegic type of spastic cerebral palsy constituted 82.5% of CP children while diplegic type constituted 17.5%. Based on Gross Motor Function Classification System (GMFCS), the majority of children were in levels 4 and 5. The prevalence of autonomic dysfunction symptoms were 80% for thermoregulatory abnormalities (cold extremities), 65% for chronic constipation, 52.5% for sleep disturbance, 47.5% for loss of appetite, 40% for sweating abnormalities, 25% for recurrent nausea and/or vomiting, 22.5% for increased sensitivity to light or dark and 15% for bloating. As regards sympathetic skin response, 19 CP children had unobtainable response in both upper and lower limbs while 5 children had unobtainable response in lower limbs only. All of them were in levels 4 and 5 of GMFCS. Postural hypotension was present in 20% of CP children. Mean Heart rate of CP children was significantly increased more than healthy children upon head tilt test. Conclusions Autonomic dysfunction has been objectively proven in CP children through absent sympathetic skin response, presence of orthostatic tachycardia and postural hypotension.

Interictal autonomic dysfunction in patients with epilepsy

Background Autonomic nervous system (ANS) symptoms are frequently present in people with epilepsy (PwE). They are generally more prominent when they originate from the temporal lobe. We aim to investigate the alterations of autonomic functions during the interictal period in patient with temporal lobe epilepsy (TLE) and idiopathic generalized epilepsy (IGE) using heart-based tests, blood pressure (BP)-based tests and sympathetic skin response (SSR). Forty-eight PwE with disease duration ranging from 2 to 15 years and 51 healthy individuals were studied. Long-term electroencephalography (EEG) monitoring, the heart rate variability (HRV) during normal breathing, deep breathing, Valsalva maneuver and standing, BP responses during standing, to isometric hand grip and to mental arithmetic, and the SSR was recorded for all participants. Results 31 patients with TLE and 17 with IGE showed lower RR-IV values during deep breathing, Valsalva maneuver and standing, but not during rest, impaired BP responses during standing, isometric hand grip, and mental arithmetic. Also, prolonged SSR latencies. Within PwE group, no difference was noticed between males and females, nor between the left and right temporal lobes. Conclusion Abnormal autonomic (sympathetic and parasympathetic) regulatory functions suggest that epilepsy may alter the autonomic function and this is not only in TLE but rather in IGE too. These autonomic changes are irrespective of the localization of epilepsy between the two hemispheres. The ANS changes in epileptic patients, particularly those with autonomic symptoms, confirm that electrophysiologic measures of autonomic function may be of value in preventing sudden unexpected death in epilepsy.

High rate of autonomic neuropathy in Cornelia de Lange Syndrome

Background Cornelia de Lange Syndrome (CdLS) is a rare congenital disorder characterized by typical facial features, growth failure, limb abnormalities, and gastroesophageal dysfunction that may be caused by mutations in several genes that disrupt gene regulation early in development. Symptoms in individuals with CdLS suggest that the peripheral nervous system (PNS) is involved, yet there is little direct evidence. Method Somatic nervous system was evaluated by conventional motor and sensory nerve conduction studies and autonomic nervous system by heart rate variability, sympathetic skin response and sudomotor testing. CdLS Clinical Score and genetic studies were also obtained. Results Sympathetic skin response and sudomotor test were pathological in 35% and 34% of the individuals with CdLS, respectively. Nevertheless, normal values in large fiber nerve function studies. Conclusions Autonomic nervous system (ANS) dysfunction is found in many individuals with Cornelia de Lange Syndrome, and could be related to premature aging.

Neurophysiological features in patients with migraine at risk for epilepsy

Objective: to study the electrophysiological parameters of brain bioelectric activity and features of the autonomous nervous system assessing heart rate variability, sympathetic skin response, clinical and physiological tests depending on the lateralization of migraine pain syndrome in the right or left brain hemisphere in patients with epileptic electroencephalogram (EEG) signs and migraine.Material and methods. Thirty six patients with aura-free episodic migraine at risk of developing epilepsy and 9 age-matched healthy subjects were examined. All participants underwent EEG, clinical and physiological tests, assessment of heart rate variability and sympathetic skin response.Results. Patients with right-hemisphere migraine headache had signs of activated sympathetic nervous system at baseline level and during exercise, lower baseline EEG epileptiform activity and in provocative tests. In contrast to the subjects of this group, patients of other group featured with a more stable migraine pain syndrome in the left hemisphere tended to dominate with functional activity of the parasympathetic system such as increased trophotropic support during exercise, as well as greater magnitude of baseline epileptiform bioelectric activity and during load tests.Conclusion. Values of heart rate variability in combination with objective results of clinical and physiological assessment of the autonomous nervous system and electrophysiological parameters of brain bioelectric activity are reliable prognostic indicators for varying functional conditions in patients with episodic migraine.

Clinical and electrophysiological characteristics of neuropathic pain in leprosy patients: A prospective cross-sectional study

Introduction: Neuropathic pain is a common and disabling late complication of leprosy. We investigated the clinical and electrophysiological characteristics of neuropathic pain in leprosy patients by evaluating nerve conduction, sympathetic skin response (SSR) and A-waves. Methods: Twenty one leprosy patients with neuropathic pain validated by the Douleur Neuropathique en 4 (DN4)Questionnaire were selected for study. Pain intensity was measured by the visual analog scale. Demographic and clinical data were collected for all patients. Clinical data included appraisal of the median, ulnar, radial, tibial and common peroneal nerves, assessment of the sympathetic skin response and conventional electrophysiological recordings. Results: Among all electroneuromyographic presentations, multifocal mononeuropathy was still the most prevalent. Sensory loss was observed more frequently than motor deficits. As most patients presented advanced clinical forms of leprosy and were under treatment, this high mean was found and the ulnar nerve was most frequently affected. The sympathetic skin response was absent in 16 patients. Higher DN4 Questionnaire scores were observed in women and in those receiving corticosteroid therapy. These inferences are possible to be made, but our study's limitations don't allow us to be certain about it. The statistical significance found only permits us to evidence what we related on the textual part of the study. Limitations: The small number of patients studied, the lack of sophisticated diagnostic methods for leprosy, as well as the difficulties in assessing nerve conduction were the main limitations of this study. Conclusion: The neurophysiological and clinical findings in leprous neuropathy were modest despite the conspicuous neuropathic pain. Although electrophysiological studies are a vital tool to verify nerve damage, variations in the clinical presentation of leprosy neuropathic pain render the diagnosis challenging. Further studies are needed to describe the neurophysiological evolution of this disease.

Peripheral Nerve Conduction And Sympathetic Skin Response Are Reliable Methods to Detect Diabetic Cardiac Autonomic Neuropathy

AimThis study aimed to investigate the role of nerve conduction studies (NCS) and sympathetic skin response (SSR) in evaluating diabetic cardiac autonomic neuropathy (DCAN).MethodsDCAN was diagnosed using the Ewing test combined with heart rate variability analysis. NCS and SSR were assessed by electrophysiological methods. The association between NCS/SSR and DCAN was assessed via multivariate regression and receiver-operating characteristic analyses.ResultsThe amplitude and conduction velocity of the motor/sensory nerve were found to be significantly lower in the DCAN+ group (all P < 0.05). A lower amplitude of peroneal nerve motor fiber was found to be associated with increased odds for DCAN (OR 2.77, P < 0.05). The SSR amplitude was lower while the SSR latency was longer in the DCAN+ group than in the DCAN– group. The receiver-operating characteristic analysis revealed that the optimal cutoff points of upper/lower limb amplitude of SSR to indicate DCAN were 1.40 mV (sensitivity, 61.9%; specificity, 66.3%, P < 0.001) and 0.85 mV (sensitivity, 66.7%; specificity, 68.5%, P < 0.001), respectively. The optimal cutoff points of upper/lower limb latency to indicate DCAN were 1.40 s (sensitivity, 61.9%; specificity, 62%, P < 0.05) and 1.81 s (sensitivity, 69.0%; specificity, 52.2%, P < 0.05), respectively.ConclusionsNCS and SSR are reliable methods to detect DCAN. Abnormality in the peroneal nerve (motor nerve) is crucial in predicting DCAN. SSR may help predict DCAN.

Investigation of autonomic dysfunction in primary Raynaud’s phenomenon with sympathetic skin response (SSR), R-R interval variation (RRIV) and composite autonomic symptom score (COMPASS)-31

Background: In this study, we aimed to investigate the autonomic dysfunction in patients with primary Raynaud’s phenomenon with using sympathetic skin response (SSR) as a neurophysiologic test, R-R interval variation analysis and composite autonomic symptom score (COMPASS)-31 questionnaire. Methods: Palmar SSR to median nerve electrical stimulation was recorded in 38 patients with 36 healthy age and sex-matched control subjects. The SSR was recorded from the palmar surface of both left and right hands for patients and control groups. The amplitudes and latencies formed as a result of electrical stimulation were calculated and compared between the two groups. Additionally, R-R interval variability was examined during normal breathing, deep breathing, standing up and Valsalva maneuver in both groups. Furthermore, we asked to complete the COMPASS-31 questionnaire, a validated tool to assess symptoms of autonomic dysfunction. And by calculating total COMPASS-31 scores, the relationship between the two groups was investigated. Results: The Raynaud’s phenomenon and control groups were similar in age (37.4 ± 11.6 vs. 34.9 ± 13.0 years), had identical gender ratios and similar body mass index (24.5 ± 6.1 vs. 25.7 ± 4.6%). Palmar SSR to median nerve stimulation of RP patients shows significantly delayed latency (1890 ± 146) (p=0.03). And no difference between amplitudes in comparison to the control group. In the patient and control groups, R-R interval measurements were evaluated during rest and deep breathing, standing up and Valsalva maneuver. When the R-R interval measurements of the patient and control groups at rest and deep breathing were compared, there was no statistically significant difference between the groups. In addition, COMPASS-31 questionnaire scoring system was applied to both groups. The mean COMPASS-31 score was higher in patient group (22.8 ± 13.8), than from healthy controls (8.9 ± 7.8) (p=0.02) Conclusions: Autonomic dysfunction plays a role in the etiology of Raynaud’s phenomenon, due to latency prolongation in the sympathetic skin response and significant difference between COMPASS-31 tests, and these tests can be used in the diagnosis stage of this disease.

Comparison of Sympathetic Skin Response (SSR) between Electrical and Acoustic Stimuli in a Healthy Pediatric Population

Data in the literature report that latency and morphology in the cutaneous sympathetic skin response (SSR) do not change according to the type of stimulus delivered, unlike the amplitude which shows greater values in relation to the intensity of the physical impact caused in patient. Since the acoustic stimulus represents a method better tolerated by the pediatric patient, the aim of this study is to evaluate the presence or absence of significant differences in SSR between electrical and acoustic stimuli. The SSR was performed for each child of 18 recruited in this study, deriving from the palm of the hand and the sole of the foot and initially delivering an electrical stimulus at the level of the median nerve at the wrist. Two acoustic stimuli were subsequently delivered with the aid of audiometric headphones. Our results show no significant differences for the amplitude values obtained (p values > 0.05). For the latency there was a statistically significant difference (p-value = 0.001) for the left hand, subsequently not confirmed by the comparison performed between the two sides (p-values = 0.28 and 0.56). If these preliminary data are confirmed by a larger sample, the acoustic stimulus could be introduced in a standardized protocol for performing SSR in pediatric patients.