Absence Of Sympathetic Skin Response Research Articles

Sympathetic and electrochemical skin responses in the assessment of sudomotor function: a comparative study

ObjectivesThe sympathetic skin response (SSR) is a well-established test, whereas the electrochemical skin conductance (ESC) is still under evaluation. Our aim was therefore to assess the diagnostic accuracy of ESC to detect abnormal sudomotor function, using SSR as a reference test. MethodsA cross sectional observational study was performed of 61 neurological patients assessed for possible sudomotor dysfunction and 50 age-matched healthy controls (HC). Patients with diagnoses of vasovagal syncope (VVS, n=25), Parkinson's disease (PD, n=15), multiple system atrophy (MSA, n=11) and peripheral neuropathies (PN, n=10) were included. Sudomotor function was assessed with SSR and ESC tests in all participants. The absence of SSR in the palms or soles indicates abnormal sudomotor function. Receiver operating characteristic (ROC) analysis was used to assess the diagnostic value of the ESC. Cardiovascular autonomic (CV-Aut) function was evaluated through the Ewing score, based on the following tests: Heart rate change with deep breathing, Valsalva ratio, 30:15 ratio, blood pressure changes on standing and during isometric exercise. A Ewing score ≥ 2 indicates the presence of CV-Aut dysfunction. ResultsMean SSR amplitudes and ESC values showed differences between HC and patients with MSA or PN (p < 0.05), but not in patients with VVS or PD. Absence of SSR was associated with abnormal ESC (p < 0.05). Patients with abnormal CV-Aut dysfunction had lower ESC (p< 0.05). Palm ESC (P-ESC) and sole ESC (S-ESC) assessment had a sensitivity of 0.91 and 0.95 to predict sudomotor dysfunction, with a specificity of 0.78 and 0.85, respectively. The area under ROC curve was 0.905 and 0.98, respectively. ConclusionsESC in palms and soles has a high diagnostic accuracy for sudomotor dysfunction as detected by absent SSR in patients with MSA and PN.

66. Autonomic-sensory neuropathy onset in a patient with acute brainstem impairment – A case report

Here we report on a 64-year-old Caucasian man admitted to the Neurology department due to a history of severe hypoacusia, nausea, stipsis, cerebellar ataxia and progressing consciousness impairment, which raised up the clinical suspicion of acute cerebellitis and suggested the introduction of oral corticosteroids. At day 7, a mild improvement of cerebellar symptoms was followed by the onset of orthostatic hypotension, four limb sensory impairment and areflexia. The spinal tap displayed a mild protein increase (78 mg/dl) and high-titer of anti-Hu (ANNA-1) antibodies in CSF. The same antibodies were also found after blood testing. Nerve conduction velocities (NCV) of the four limbs showed a picture of severe peripheral neuropathy with predominant sensory and axonal features, absence of sympathetic skin response. No sensory evoked potentials were detectable either at upper and lower limbs, while cranial district reflexes and NCV were not affected. Autonomic tests confirmed severe orthostatic hypotension with mild parasympathetic involvement. A PET study highlighted a hypercaptation in the right paratracheal region, which a bronchoscopy with transbronchial biopsy identified as a possible lung cancer. Therefore, the patient was diagnosed with dysimmune rhombencephalitis and polyneuropathy in a likely paraneoplastic syndrome.

Réponses cutanées sympathiques et sclérose en plaques : étude descriptive, prospective et association aux troubles neuropérinéaux

Describe the sympathetic skin responses (SSRs), reflecting the sweat activity mediated by the sympathetic nervous system, in patients with multiple sclerosis (MS) followed for urinary disorders and associate SSRs abnormalities with neuroperineal disorders. Prospective monocentric study of a population suffering from MS, who received symptom scores (Expanded Disability Status Scale [EDSS], Urinary Symptoms Profile [USP], Neurogenic Bowel Dysfunction [NBD], International Index of Erectile Function [IIEF] and Female Sexual Function Index [FSFI]) and whose SSRs were collected from the hand palm, the foot sole and the genital skin after mechanical stimulation, before and after anticholinergic drug. Twenty-eight patients were included. Among them, 18 had an abolition of SSR, respectively 10, 14 and 17 at palm, perineum and foot sole. The absence of SSR was not related (P>0.05) with the duration of disease, the EDSS score, USP, IIEF15 or NBD scores. There was a statistically significant relationship between the abolition of SSR at perineum and women genitosexual disorders (P=0.01). Anticholinergic drugs remove 40 % of SSRs at hand and 67% at foot. The abolition of SSR at perineum was associated with the abolition of the SSR at sole (P=4 × 10(-5)). In these MS patients with neuroperineal disorders, more than half showed abnormal SSR. These abnormalities did not seem associated with urinary or rectal disorders but with genitosexual disorders in women. Under anticholinergic treatment, SSR frequently disappeared at palm and foot sole. 4.

Impact of sympathetic dysfunction in the etiology of overactive bladder in women: A preliminary study

We investigated the effects of autonomic sympathetic dysfunction in the etiology of overactive bladder (OAB) in women, by assessing the sympathetic skin response (SSR). In total, 40 women with OAB and 15 volunteers were enrolled. Group 1 (n = 20) consisted of patients who benefitted from oral anticholinergics, Group 2 (n = 20) consisted of patients who were refractory to oral anticholinergics, and the volunteers (n = 15) were the control group. All patients were asked to complete OAB-SF questionnaires. The absence of SSR was accepted as significant. Detailed physical examinations including neurological examinations were performed. All patients underwent SSR tests on the skin of the hands, feet, and genital area in our neurology department using electromyography, and SSR responses were recorded. Statistical significance was accepted at P < 0.05. The mean age was 47.2 ± 12.4 years. In total, 55 women were evaluated (20 in Group 1, 20 in Group 2, and 15 in the control group). Mean OAB scores were similar between groups 1 and 2, although urgency was significantly higher in Group 2 than 1. The absence of SSR was significantly higher in Group 2 than 1 (P = 0.01). Impaired SSR was observed more commonly in Group 2 than 1. All controls had normal SSRs. Regional dysfunction in the autonomic nervous system may be part of OAB's etiology. SSR may be a candidate test for determining early OAB, and may also be useful for predicting antimuscarinic-refractory patients.

Skin Denervation and Its Clinical Significance in Late-Stage Chronic Kidney Disease

To investigate the skin innervation and its clinical significance in late-stage chronic kidney disease (CKD). Case series. National Taiwan University Hospital, Taipei, Taiwan. Forty consecutive nondiabetic patients with late-stage CKD (14 female and 26 male; mean [SD] age, 60.7 [12.3] years), including 2 cases with stage 3 CKD, 6 with stage 4 CKD, and 32 with stage 5 CKD, ie, end-stage kidney disease. Clinical evaluation of neurological deficits, nerve conduction study, autonomic function tests, and a 3-mm-diameter skin biopsy specimen taken from the distal leg. Quantitation of epidermal innervation, parameters of nerve conduction study, R-R interval variability, and sympathetic skin response. Clinically, 21 patients (52.5%) were symptomatic with paresthesia over the limbs or autonomic symptoms. The intraepidermal nerve fiber (IENF) density was markedly reduced in patients with CKD compared with age- and sex-matched controls (mean [SD], 2.8 [2.0] vs 8.6 [2.8] fibers/mm; P < .001). Skin denervation was observed in 27 patients (67.5%). Fifteen patients (37.5%) had abnormalities on nerve conduction studies, and 29 patients (72.5%) had abnormal results on autonomic function tests. By analysis with multiple regression models, the IENF density was negatively correlated with the duration of renal disease (P = .02). Additionally, the R-R interval variability at rest was linearly correlated with the IENF density (P = .02) and the absence of sympathetic skin responses at the soles was associated with reduced IENF density (P = .03). Small-fiber sensory and autonomic neuropathies constitute the major form of neuropathy in late-stage CKD. Furthermore, skin denervation was associated with the duration of renal disease.

The significance of intact sympathetic skin responses in carpal tunnel syndrome

The current study aimed to investigate the impact of carpal tunnel syndrome (CTS) on sympathetic skin response (SSR) recorded from the median and ulnar territory. Thirty patients were studied and idiopathic CTS was documented in a total of 46 hands. These were classified, according to electrophysiological criteria, into two groups; a group of 31 hands with severe CTS and a group of 15 hands with mild/moderate CTS, and were compared with a group of 30 hands of age-matched controls. SSR was recorded simultaneously from the median and ulnar side of the palm following electrical stimulation at the wrist, in a mid-point between median and ulnar nerve. Latency, amplitude, habituation and the median-to-ulnar ratio were estimated. In all controls clear recordings of SSR were obtained. In the patient groups, absence of SSR was never observed either in mild/moderate or in the severe CTS hands. The mean SSR latency and amplitude values recorded from both the median and ulnar nerves did not significantly differ between mild/moderate or severe CTS hands and controls. Likewise, the median-to-ulnar ratio and habituation of SSR latencies and amplitudes did not significantly differ between groups. SSR does not seem to be a sensitive method for evidence of autonomic involvement in CTS, even in patients manifesting sudomotor or other autonomic symptoms. In the present setting, SSR appeared to be independent of somatic afferent function and the corresponding sensory action potentials.

Electrophysiological assessment of sensations arising from the bladder: are there objective criteria for subjective perceptions?

Initial bladder filling sensation, first and strong desire to void are subjective perceptions that occur periodically during the urine storage mode of bladder function, representing sensory input from the lower urinary tract. To our knowledge methods for evaluating sensory bladder function are not available. We studied a simple electrophysiological procedure for the objective assessment of bladder sensations using sympathetic skin responses and surface pelvic floor electromyography. Informed consent was provided by 8 healthy male subjects, who were administered 20 mg. furosemide and 1 l. fluid to drink. Palmar and plantar sympathetic skin responses, and surface pelvic floor electromyogram were continuously recorded during bladder filling, voluntary pelvic floor contraction and voiding. First desire to void evoked simultaneous sympathetic skin responses and pelvic floor contractions. This pattern appeared periodically with the desire to void sensation as well as with strong desire to void at maximum bladder capacity and it correlated well with the subjective sensation of the subjects. Voluntary pelvic floor contraction decreased the subjective intensity of the desire to void sensation as well as sympathetic skin response activity for the same short period. During voiding sympathetic skin responses almost complete absence of sympathetic skin responses was observed. Sensations arising from the bladder induce combined activation of sympathetic skin responses and pelvic floor activity. This coherence indicates synchronized activation and inactivation of the autonomic and somatic pathways necessary for appropriate urine storage and coordinated voiding. Our observations may introduce a new approach for objectively assessing subjective sensations arising from the urinary tract.

Autonomic Dysfunction in Machado-Joseph Disease

Machado-Joseph disease is an autosomal dominant spinocerebellar ataxia with expanded trinucleotide repeats. Although autonomic nervous system degeneration was documented in postmortem reports, the autonomic dysfunction in patients with Machado-Joseph disease, either in clinical analysis or electrophysiological investigations, has not yet been studied in detail. Fifteen patients with genetically confirmed Machado-Joseph disease and 34 healthy subjects were studied. The study design included a detailed questionnaire, R-R interval variation on deep breathing or Valsalva maneuver, and sympathetic skin response evoked by electrical stimulation of the median nerve or magnetic stimulation of the neck. Sixty-seven percent of patients had at least 3 symptoms involving different aspects of autonomic functions. Voiding problems and thermoregulatory disturbance were the most common symptoms. Ten (71%) of 14 patients had abnormal R-R interval variation with a significant reduction of the mean ratio. Prolonged latency or absence of sympathetic skin response to electrical stimulation was identified in 73% of patients and to magnetic stimulation, in 53%. R-R interval variation and sympathetic skin response showed good correlation with the functional stage. Electrical stimulation revealed the highest sensitivity, specificity, and positive predictive value compared with other tests. The present investigation documents that autonomic dysfunction is not uncommon in patients with Machado-Joseph disease and might be related to the clinical progression.

Electrophysiological Assessment of Sensations Arising from the Bladder: Are there Objective Criteria for Subjective Perceptions?

Initial bladder filling sensation, first and strong desire to void are subjective perceptions that occur periodically during the urine storage mode of bladder function, representing sensory input from the lower urinary tract. To our knowledge methods for evaluating sensory bladder function are not available. We studied a simple electrophysiological procedure for the objective assessment of bladder sensations using sympathetic skin responses and surface pelvic floor electromyography. Informed consent was provided by 8 healthy male subjects, who were administered 20 mg. furosemide and 1 l. fluid to drink. Palmar and plantar sympathetic skin responses, and surface pelvic floor electromyogram were continuously recorded during bladder filling, voluntary pelvic floor contraction and voiding. First desire to void evoked simultaneous sympathetic skin responses and pelvic floor contractions. This pattern appeared periodically with the desire to void sensation as well as with strong desire to void at maximum bladder capacity and it correlated well with the subjective sensation of the subjects. Voluntary pelvic floor contraction decreased the subjective intensity of the desire to void sensation as well as sympathetic skin response activity for the same short period. During voiding sympathetic skin responses almost complete absence of sympathetic skin responses was observed. Sensations arising from the bladder induce combined activation of sympathetic skin responses and pelvic floor activity. This coherence indicates synchronized activation and inactivation of the autonomic and somatic pathways necessary for appropriate urine storage and coordinated voiding. Our observations may introduce a new approach for objectively assessing subjective sensations arising from the urinary tract.

Subtle involvement of the sympathetic nervous system in amyotrophic lateral sclerosis.

The literature on the involvement of the autonomic nervous system (ANS) in amyotrophic lateral sclerosis (ALS) is conflicting. We therefore investigated several aspects of autonomic function, namely muscle sympathetic nerve activity (MSNA), blood pressure, cardiac function (electrocardiogram; ECG), and respiration in 16 patients with sporadic ALS and in 12 age-matched healthy volunteers, both at rest and during sympathoexcitatory stimulation. We measured MSNA by provoking venous pooling during short-lasting lower body negative pressure (LBNP) and during the cold pressor test (CPT). To assess the vagal (baroreflex) control of heart rate (HR), we measured spontaneous baroreflex sensitivity (BRS). To assess the involvement of the ANS beyond the cardiovascular system, we measured the sympathetic skin response (SSR). The stand-up test showed that none of the subjects had orthostatic intolerance. In comparison with the control group, the ALS patients had an increased HR and a decreased BRS at rest, and a reduced MSNA response to LBNP. The CPT response was normal and the total MSNA at rest did not differ significantly from that of controls. The latencies of the palmar and plantar SSR were prolonged, and in 3 ALS patients there was no plantar SSR. The results indicate that the sympathetic nervous system shows subtle abnormalities in ALS, predominantly sympathetic overactivity. They also point to the involvement of the preganglionic sympathetic column as the cause of the higher sympathetic activity and the absence of SSR. The higher sympathetic activity is postulated to be due to changes in modulation of the sympathetic system, whereas the absence of the SSR is probably caused by disruption of the reflex pathway.

Neurophysiologic studies in congenital insensitivity to pain with anhidrosis

Thirteen patients with congenital insensitivity to pain and anhidrosis, carrying a mutation at the TRK-A gene, were studied. Neurologic examination revealed vestigial pain sensitivity, suggesting an incomplete involvement of the affected nerves. All 13 patients manifested normal electrophysiologic studies but striking absence of sympathetic skin responses. We suggest the use of the sympathetic skin response test in the clinical evaluation of patients suspected of having congenital insensitivity to pain and anhidrosis.

Sympathetic skin response in acute sensory ataxic neuropathy

Sympathetic skin response (SSR) is a recently described objective method of studying sudomotor sympathetic nerve function and has been studied in a variety of peripheral neuropathies. We report SSR changes in nine patients with acute sensory ataxic neuropathy (ASAN). All had severe sensory and mild motor nerve conduction abnormalities; five had dysautonomia. SSR, elicited by electric shock and cough stimuli, was absent in three patients. Latency was normal in all when SSR was present. Two patients had SSR amplitude of 0.2 mV or less. Absence of SSR did not correlate with dysautonomia, absence of sensory nerve action potential or motor nerve conduction abnormalities. Follow up SSR studies revealed return of absent SSR in one patient over a period of 3 months, despite persistence of ataxia. To our knowledge this is the first report of SSR changes in ASAN.

Sympathetic skin response and R–R interval variation in chronic uremic patients

Sympathetic skin response (SSR) and R-R interval variation (RRIV) were studied in 36 chronic, nondiabetic uremics to compare with their nerve conduction studies (NCS) and clinical dysautonomia. Abnormal SSR was noted in 5 (13.9%) patients, abnormal RRIV in 14 (38.9%), and abnormal NCS in 26 (72.2%). The patients were classified into three groups: group (GP) 1: "normal," n = 21 (58.3%), normal RRIV and SSR; GP 2: "isolated parasympathetic dysfunction," n = 10 (27.8%), abnormal RRIV and normal SSR; and GP 3: "sympathetic sudomotor dysfunction," n = 5 (13.9%), abnormal SSR. A significant difference in age was found among the three groups (GP 3 > GP 2 > GP 1; P < 0.0001, ANOVA). After controlling the age factor, we still noted a tendency toward increasing NCS disturbances (distal latency and nerve conduction velocity of peroneal nerve; P < 0.05, multiple regression analysis) and frequencies of clinical autonomic symptoms (postural dizziness and impotence; P < 0.05, Mantel-Hanszel test) from GP 1 to GP 3. Patients with abnormal SSR (GP 3) displayed significantly higher frequencies of postural dizziness and impotence, indicating the relationship between an absence of SSR and clinical dysautonomia.

Two patients with distal muscular dystrophy and autonomic nerve dysfunction

Two female patients with distal muscular dystrophy (Miyoshi) are reported. Neurological examination revealed marked weakness and a low skin temperature of both lower legs, especially over the gastrocnemius muscle. Biopsy specimens of the anterior tibial muscle exhibited myopathological features compatible with a dystrophic change and showed positive dystrophin reactions with all six antibodies used. Autonomic nerve studies in both patients, including laser Doppler flowmetry (LDF), component analysis of the cardiographic R-R interval and a sympathetic skin response (SSR) test, demonstrated marked abnormalities, such as sensitive vasoconstrictive responses, a suppressed peak of low frequency components and an absence of SSR, respectively, compared with findings in healthy controls. Sympathetic nerve blocking by means of epidural anesthesia produced clinical improvement and a marked decrease in the serum CK level in one patient. Although the etiology of autonomic nerve disturbances in these cases remains to be elucidated, a positive immunoreactivity of nerve growth factor receptor (NGFR) implies the possibility of some unknown sympathetic neurovascular disorder involving muscle degeneration.