R-R interval variation and the sympathetic skin response in the assessment of the autonomic nervous system in leprosy patients

OBJECTIVES: The aim of this study was to evaluate possible autonomic nervous system (ANS) dysfunction in leprosy patients with the sympathetic skin response (SSR) and the heart rate (R-R) interval variation (RRIV) measurements which are easy and reliable methods for evaluation of autonomic functions. MATERIAL AND METHODS: We studied 37 lepromatous leprosy patients (mean age: 38 +/- 17 years, range 23-62 years, 20 females and 17 males) and 35 age-matched healthy subjects (mean age: 34.19 +/- 12.74 years, range 24-48 years, 20 females and 15 males). Non-invasive bedside tests (orthostatic test, Valsalva ratio), R-R interval variation (RRIV) during at rest and deep breathing, the SSR latency and amplitude from both palms, and nerve conduction parameters were studied in all the subjects. RESULTS: The mean values of RRIV in leprosy patients during at rest [mean RRIV in patients, 17.42 +/- 8.64% vs controls, 22.71 +/- 3.77% (P < 0.05)] and during deep breathing [mean RRIV in patients, 21.64 +/- 9.08% vs controls, 30.70 +/- 5.99% (P < 0.005)] was significantly lower compared with the controls. The mean latency of SSR in leprosy patients [mean SSR latency in patients, 1.72 +/- 1.13 ms vs controls, 1.30 +/- 0.41 ms (P < 0.05)] was significantly prolonged compared with the controls. The mean amplitude of SSR in leprosy patients [mean SSR amplitude in patients, 0.54 +/- 0.57 microV vs controls, 1.02 +/- 0.56 microV (P > 0.05)] was smaller compared with the controls, but this difference was not significant. The mean Valsalva ratio in leprosy patients [mean in patients, 1.11 +/- 0.13 vs controls, 1.16 +/- 0.07 (P > 0.05)] was smaller compared with the controls, but not statistically significant. The mean difference of systolic and diastolic blood pressure between supine rest and during standing in leprosy patients were higher compared with the controls [mean systolic pressure in patients, 7 +/- 6 mmHg vs controls, 6 +/- 8 mmHg (P > 0.05) and mean diastolic pressure in patients, 3 +/- 3 mmHg vs controls, 3 +/- 2 mmHg (P > 0.05)], but they did not reach statistical significance. Furthermore, lower RRIV and the prolonged SSR latencies in leprosy patients were closely correlated to some parameters of sensorimotor nerve conduction and each other [median nerve distal latency and RRIV, r = -0.67 (P < 0.05), ulnar nerve distal latency and RRIV, r = -0.59 (P < 0.05), RRIV and SSR latency, r = -0.33 (P < 0.02)]. These data indicate that leprosy patients have the functional abnormalities of ANS. CONCLUSION: We conclude that combined use of these two tests, both of which can be easily and rapidly performed in the electromyogram (EMG) laboratory using standard equipment, allows separate testing of parasympathetic and sympathetic function, and are very sensitive methods in assessing of ANS function in peripheral neuropathy in leprosy patients.     

Comparative neurophysiological study for the diagnosis of mild polyneuropathy in patients with diabetes mellitus and glucose intolerance

This article evaluates diagnostic sensitivity of minimal F-wave latency, sural/radial amplitude ratio (SRAR), dorsal sural/radial amplitude ratio (DSRAR), sympathetic skin response (SSR), and R-R interval variability (RRIV) for detecting early polyneuropathy in patients with glucose intolerance and diabetic patients. F-wave latencies were more prolonged in diabetic patients with normal and abnormal nerve conduction studies than control subjects (p < .001). SRAR was lower, SSR latency was more prolonged, and RRIV was lower in diabetic patients with abnormal nerve conduction studies than healty controls (p < .001). SSR latency was more prolonged and RRIV was lower in diabetic patients with normal nerve conduction studies than healty controls (p < .01, p < .05, respectively). DSRAR was lower in diabetic patients with normal and abnormal nerve conduction studies than control subjects (p < .001). DSRAR was also lower in patients with glucose intolerance than control subjects (p < .01). DSRAR was the most sensitive and specific test in either of diabetic patients with normal nerve conduction studies (sensitivity 66%, specificity 90%) and diabetic patients with abnormal nerve conduction studies (sensitivity 100%, specificity 90%). DSRAR is the most reliable method for detection of early nerve pathology. Patients with glucose intolerance might have subclinical neuropathy that can be demonstrated with DSRAR analysis.     

Autonomic dysfunction in patients with essential tremor

The aim of this study was to evaluate the autonomic function in patients with essential tremor (ET). Thirty-one adult patients with ET and 26 healthy controls were enrolled in the study. The electrophysiological evaluations of the autonomic nervous system function were performed by sympathetic skin response (SSR) and R–R interval variation (RRIV) tests. The mean latency of SSR in ET patients was significantly delayed compared with the controls (P = 0.01). The mean amplitude of sympathetic skin response was significantly lower in ET patients in comparison to the controls (P = 0.001). No differences were found in mean RRIV values in both group subjects. Sympathetic dysfunction may occur in patients with ET. This may be easily demonstrated by SSR tests.     

Effects of epilepsy on autonomic nervous system and respiratory function tests

We have investigated autonomic nervous system function during the interictal period in epileptic patients and the possible effects of autonomic dysfunction on respiratory functions. A total of 32 epileptic patients (23 generalized, 9 partial epilepsy) and 32 healthy volunteers were involved. Sympathetic skin response (SSR), for evaluating the sympathetic nervous system, and RR interval variation (RRIV) were measured at the beginning and third month of antiepileptic treatment, and respiratory function tests (RFTs) were performed. In patients with partial epilepsy, SSR latency in the upper extremity (1.3+/-0.2 s) was longer than that of controls (1.2+/-0.3 s) at baseline (P=0.05), and was significantly reduced (1.1+/-0.3 s) after treatment (P<0.05). RRIV values of patients with generalized epilepsy were statistically significantly lower than those of controls (P<0.01). However, deep breathing RRIV values (32.6+/-15.3%) of patients were lower than those (43.0+/-18.2%) of controls (P<0.05). Sympathetic dysfunction was determined in patients with partial epilepsy and parasympathetic dysfunction in patients with generalized epilepsy. No abnormality was observed on RFTs for both patients with partial epilepsy and patients with generalized epilepsy.     

Are electrophysiological autonomic tests useful in the assessment of dysautonomia in Parkinson's disease?

To assess the autonomic system in Parkinson's disease (PD), the sympathetic skin response (SSR) and the R-R interval variation (RRIV) tests were studied in 26 PD patients and in 24 healthy controls. The aim of the study was to evaluate the sympathetic and parasympathetic system function in PD, to define the pattern of autonomic abnormalities found in SSR and RRIV in parkinsonian patients as well as to analyze the usefulness of both tests in paraclinical assessment of the dysautonomia, compared with clinical symptoms and signs of the autonomic nervous system involvement. The corrrelations between both autonomic tests results were also studied. In PD patients SSR test was abnormal in about 35% and RRIV was abnormal in about 54% of patients. SSR and RRIV were both abnormal in about 27% of PD patients whereas at least one of electrophysiological autonomic tests was abnormal in about 62% of PD patients. Clinical and paraclinical signs of dysautonomia occurred in a similar proportion of patients (i.e. in about 62%). A weak correlation was found between the latency of SSR from upper limbs and the value of RRIV during deep breathing (p=0.063). Our results show that SSR and RRIV are non-invasive paraclinical electrophysiological tests that confirm clinical dysautonomia in PD and can supplement the clinical differentiation of Parkinsonian syndromes.     

COMPARATIVE NEUROPHYSIOLOGICAL STUDY FOR THE DIAGNOSIS OF MILD POLYNEUROPATHY IN PATIENTS WITH DIABETES MELLITUS AND GLUCOSE INTOLERANCE

This article evaluates diagnostic sensitivity of minimal F-wave latency, sural/radial amplitude ratio (SRAR), dorsal sural/radial amplitude ratio (DSRAR), sympathetic skin response (SSR), and R-R interval variability (RRIV) for detecting early polyneuropathy in patients with glucose intolerance and diabetic patients. F-wave latencies were more prolonged in diabetic patients with normal and abnormal nerve conduction studies than control subjects (p < .001). SRAR was lower, SSR latency was more prolonged, and RRIV was lower in diabetic patients with abnormal nerve conduction studies than healty controls (p < .001). SSR latency was more prolonged and RRIV was lower in diabetic patients with normal nerve conduction studies than healty controls (p < .01, p < .05, respectively). DSRAR was lower in diabetic patients with normal and abnormal nerve conduction studies than control subjects (p < .001). DSRAR was also lower in patients with glucose intolerance than control subjects (p < .01). DSRAR was the most sensitive and specific test in either of diabetic patients with normal nerve conduction studies (sensitivity 66%, specificity 90%) and diabetic patients with abnormal nerve conduction studies (sensitivity 100%, specificity 90%). DSRAR is the most reliable method for detection of early nerve pathology. Patients with glucose intolerance might have subclinical neuropathy that can be demonstrated with DSRAR analysis.     

Voluntary motor function in patients with chronic fatigue syndrome

INTRODUCTION: The pathogenesis of chronic fatigue syndrome (CFS) remains unknown. In particular, little is known of the involvement of the motor cortex and corticospinal system. METHODS: Transcranial magnetic stimulation (TMS) was used to assess corticospinal function in terms of latency and threshold of motor-evoked potentials (MEPs) in thenar muscles. Reaction times and speed of movement were assessed using button presses in response to auditory tones. RESULTS: Patients had higher (P<.05) self-assessed indices of fatigue (7/10) than for pain (5/10), anxiety (4/10) or depression (3/10). Mean (+/-S.E.M.) simple reaction times (SRTs) were longer (P<.05) in the patients (275+/-19 ms) than in the controls (219+/-9 ms); choice reaction times (CRTs) were not significantly longer in the patients. Movement times, once a reaction task had been initiated, were longer (P<.05) in the patients in both SRTs (patients, 248+/-13 ms; controls, 174+/-9 ms) and CRTs (patients, 269+/-13 ms; controls, 206+/-12 ms). There was no difference (P>.05) in threshold or latency of MEPs in hand muscles between the patients (threshold, 54.5+/-2.2% maximum stimulator output [% MSO]; latency 22+/-0.3 ms) and controls (threshold 54.6+/-3.6% MSO; latency 22.9+/-0.5 ms). Regression analysis showed no correlation (P>.05) of SRTs with either threshold for MEPs or fatigue index. CONCLUSION: Corticospinal conduction times and excitability were within the normal range despite a slower performance time for motor tasks and an increased feeling of fatigue. This suggests that the feeling of fatigue and the slowness of movement seen in CFS are manifest outside the corticospinal system.     

Sympathetic skin response and RR interval variation in manganism and a comparison with Parkinson's disease

Autonomic dysfunction in five patients with manganism was investigated by sympathetic skin response (SSR) and RR interval variation (RRIV). A comparison was made with 10 patients with Parkinson's disease (PD) and 10 normal controls. The subjects were agematched and in PD disease stage-matched. Autonomic symptoms were more common in PD than in manganism. In SSR, the latency was prolonged in PD and manganism, while the amplitude was reduced only in PD. The RRIV was decreased in PD and manganism, but the reduction in RRIV was more severe in PD than in manganism. The present data indicate that autonomic disturbance may occur in manganism, but is less frequent and less severe when compared with PD.     

Clinical versus electrophysiological assessment of dysautonomia in obstructive sleep apnea syndrome.

To assess the autonomic system in obstructive sleep apnea syndrome (OSAS), the sympathetic skin response (SSR) and the R-R interval variation (RRIV) tests were studied in 34 OSAS patients and in 32 healthy controls. The aim of the study was to evaluate the sympathetic and parasympathetic system function in OSAS, to define the pattern of autonomic abnormalities found in SSR and RRIV in patients, and to analyze the usefulness of both tests in paraclinical assessment of the dysautonomia, compared with clinical symptoms and signs of autonomic nervous system involvement. The correlations between both autonomic tests results were also studied. In OSAS patients, SSR test results were abnormal in about 44% and RRIV results were abnormal in about 21% of patients. The mean values of parameters studied in SSR were significantly different in OSAS patients and controls (P < 0.05), whereas the differences between RRIV results were less important. The SSR and RRIV results in patients with mild apnea (Apnea/ Hypopnea Index (AHI) < 15) were more frequently within normal limits if compared with those of patients with severe apnea, but without reaching statistical significance. The clinical studies results (according to the Autonomic Symptoms Questionnaire) were related to the SSR results (p < 0.05 on chi and Fisher exact test). According to these results, SSR and RRIV are simple paraclinical electrophysiologic tests that confirm clinical dysautonomia. They may be useful as screening tests for assessment of dysautonomia in OSAS.     

Supression of cardiac parasympathetic functions in patients with right hemispheric stroke

OBJECTIVES: The aim of this study was to investigate the relationship between sympathetic and cardiac parasympathetic function and the side of the lesion in stroke patients. METHODS: Thirty-two patients with stroke and 29 healthy age-matched control subjects were studied. Sympathetic skin responses (SSR) and RR interval variations (RRIV) during rest and deep breathing were recorded for the assessment of sympathetic and vagal parasympathetic function, respectively. RESULTS: The mean SSR amplitude values in patients compared with controls were 337 +/- 244 versus 1897 +/- 848 (P < 0.0001) for right hemispheric lesions and 466 +/- 398 versus 1873 +/- 843 (P < 0.0001) for left hemispheric lesions. The mean SSR latencies in patients compared with controls were 1526 +/- 163 versus 1395 +/- 109 (P < 0.05) for right hemispheric lesions and 1490 +/- 125 versus 1423 +/- 112 (P < 0.05) for left hemispheric lesions. RRIV (during deep breathing)/RRIV (at rest) ratios in patients compared with controls were 1.20 +/- 0.25 versus 1.84 +/- 0. 52 (P < 0.0001), and 1.55 +/- 0.88 versus 1.84 +/- 0.52 (P < 0.05) for right and left hemispheric lesions, respectively. CONCLUSION: Supression of vagal parasympathetic activity was more apparent in stroke patients with right hemispheric lesions in our series. Therefore, the right hemisphere seems to have a greater effect upon parasympathetic activity.     

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. © 1994 John Wiley & Sons, Inc.     

精神分裂症患者事件相关电位P300与临床症状关系的研究

目的　探讨精神分裂症P3 0 0 主要指标尤峰间潜伏期的变化及其与临床症状的关系。方法　检测 4 0例精神分裂症患者 (患者组 )和 36名正常人 (对照组 )CZ、C3 、C4电极位置的P3 0 0 ,并用阳性和阴性症状量表 (PANSS)评定患者临床症状。结果　与正常对照组相比 ,在CZ、C3 、C4三点 ,患者组P3波幅低 (P <0 0 1) ,并与阴性症状分呈负相关 (r=- 0 4 0 3,- 0 4 30 ,- 0 35 7) ;N2 潜伏期长 (P <0 0 1) ;P2 N2 峰间潜伏期长 (P <0 0 5和P <0 0 1) ,并与PANSS量表总分、阳性症状、思维障碍、激活性等多种症状分呈负相关 (P <0 0 1～ 0 0 5 ) ;N2 P3 峰间潜伏期短 (P <0 0 1) ,在CZ 点与PANSS量表总分(r=- 0 36 3)和一般症状分 (r=- 0 392 )呈负相关。结论　精神分裂症患者的P3 波幅、N2 潜伏期、P2 N2 、N2 P3 峰间潜伏期有明显异常 ,其中P3 波幅、P2 N2 、N2 P3 峰间潜伏期与多种临床症状呈相关性     

Tests of autonomic dysfunction in patients with multiple sclerosis

Autonomic dysfunction is frequent in patients with multiple sclerosis (MS). The sympathetic skin response (SSR) and the R-R interval variation (RRIV) are simple electrophysiologic tests for the assessment of central and peripheral autonomic disturbances. Both tests were performed in 60 patients with clinically definite MS and 30 controls. The SSR was recorded simultaneously from both upper and both lower limbs. In all volunteers normal responses were recorded from the four limbs, but 39 patients (65%) showed abnormal responses in at least one limb. The reduction in amplitude of the response was correlated with patients' EDSS. In individual limbs, the SSR amplitude correlated with weakness, spasticity and cerebellar dysfunction, but was not sufficiently related to the deep sensory loss. The RRIV was abnormal in 48 MS patients (80%), as compared to the controls, but showed no significant relationship either to the EDSS or to the SSR. The sensitivity of SSR and RRIV is high and comparable with that of visual and somatosensory evoked potentials.     

Diagnostic role of deep tendon reflex latency measurement in small-fiber neuropathy

Small-fiber neuropathy (SFN) is diagnosed on the basis of clinical features and specialized tests of small-fiber function because standard nerve conduction studies are normal. Thus, the objective of this study was to determine the value of deep tendon reflex (DTR) latency measurement in the diagnosis of SFN in patients with preserved DTR on clinical examination. We prospectively examined electromyographic reflexes from the biceps brachii [biceps brachii reflex (BR)], patellar [patellar reflex (PR)], and ankle [ankle reflex (AR)] using a manually operated electronic reflex hammer attached to electromyography machine and recorded by means of surface electrodes in 18 patients with SFN and 38 controls. Intra- and inter-evaluator reliability was good (intraclass correlation coefficient: 0.80-0.91, p < 0.01). In controls, the latencies at all sites were correlated to the height (R= 0.6, p < 0.01). Compared with controls, in patients with SFN, the mean latency in milliseconds was prolonged at all sites (BR: 12.8 +/- 1.6 vs. 8.9 +/- 1.9, p < 0.01; PR: 23.0 +/- 5.8 vs. 17.4 +/- 2.4, p < 0.01; and AR: 34.5 +/- 4.8 vs. 30.0 +/- 2.4, p < 0.01). The sensitivity [61.1% (95% CI: 51-94.9)] and specificity [92% (95% CI: 73-97.3)] of BR latency were roughly equal to those of PR and AR. We conclude that DTR latencies were significantly abnormal in the majority of the patients with SFN, suggestive of subclinical involvement of large myelinated fibers. DTR latency measurement is a reproducible, valuable, sensitive tool in the evaluation of mild subclinical involvement of large fibers.     

交感神经皮肤反应诊断慢性乙醇中毒自主神经病变

目的 :早期发现慢性乙醇 (酒精 )中毒者自主神经病变。方法 :对 48例慢性乙醇中毒患者进行交感神经皮肤反应 (SSR)的检测 ,并与 50例正常人进行对比分析。结果 :慢性乙醇中毒组SSR潜伏期显著延长 (P <0 0 1 ) ,下肢波幅明显减低 (P <0 0 5) ,患者的饮酒年限、饮酒量与SSR呈正相关。结论 :交感神经皮肤反应可作为评价乙醇中毒自主神经功能障碍的客观电生理指标     

Normative data of sympathetic skin response and RR interval variation in Turkish children

Sympathetic skin response (SSR) and RR interval variation (RRIV) are used commonly for the assessment of sympathetic and parasympathetic nervous system function, respectively. We determined the normal values of SSR and RRIV in 23 (14 females, nine males) Turkish children aged 5 to 14 (mean 9.86, SD 2.48) years. SSR was recorded on the hands and feet during the electrical stimulation of both median and posterior tibial nerves, respectively. Similar response was elicited on both feet during the stimulation of the right median nerve. RRIV testing was performed during rest on the supine position and deep inspiration at a frequency of 6 times/min. The SSR was elicited in all children. The mean SSR latencies recorded on the feet during the stimulation of median or posterior tibial nerve were significantly more prolonged than those recorded at the hands (P < 0.001). There was no significant difference between the mean latencies of SSR recorded at the ipsilateral and contralateral palms or soles. The mean latencies recorded at the sole during stimulation of the median nerve were not significantly different compared to those that recorded at the sole during the posterior tibial nerve (P > 0.05). The SSR amplitudes were not assessed because of great variability and rapid habituation. The mean RRIV (46.54+/-11.29%) during deep breathing was significantly increased as compared to that (35.90+/-10.63%) during rest (P < 0.003). As a result, SSR and RRIV are preferred non-invasive tests for evaluation of autonomic nervous system in children. The SSR is useful and reliable if it is obtained in the optimum technical conditions. Further research is necessary to establish strict criteria for abnormality.     

Acute stroke increases QT dispersion in patients without known cardiac diseases

BACKGROUND: Electrocardiographic changes are well known to appear with acute cerebrovascular events. OBJECTIVE: To investigate if QT dispersion (QTd) is increased in patients who have an acute stroke and if this increase could be related to lesion extent and/or localization. DESIGN: The study group consisted of 36 patients who had an acute stroke and no history or signs of cardiovascular disease. An age-matched control group (n = 19) free of cardiovascular disease was also included. Simultaneous 12-lead electrocardiograms (ECGs) were recorded within the first 24 hours (24h-ECG) and after 72 hours (72h-ECG) from stroke onset. QT dispersion was assessed both manually and automatically with assessors blinded to the clinical data. RESULTS: QT dispersion, corrected QTd, and automated QTd were significantly increased in the 24h-ECG compared with the 72h-ECG (60 [range, 20-80] milliseconds vs 40 [range, 0-80] milliseconds, P<.005; mean [SD], 56 [19] vs 36 [21] milliseconds, P<.001; and 50 [range, 14-94] vs 34 [range, 0-84] milliseconds, P<.005, respectively). However, QTd in the 72h-ECG was similar to QTd in the control group. While in the 24h-ECG corrected QTd was significantly greater in patients with large infarcts and large hemorrhages (mean [SD], 70 [20] vs 51 [20] milliseconds, P<.05), in the 72h-ECG corrected QTd was greater in patients with right vs left-sided lesions (mean [SD], 39 [18] vs 24 [18] milliseconds, P<.05). CONCLUSIONS: QT dispersion is increased in the first 24 hours in patients with acute stroke and no cardiovascular disease compared with the control group. Although this finding seems to be related to the size of the lesion rather than to the localization or type of stroke, after 72 hours specific lesion localization could also influence the QTd.     

Proton magnetic resonance spectroscopy of the motor cortex in 70 patients with amyotrophic lateral sclerosis

OBJECTIVE: To evaluate proton magnetic resonance spectroscopy for detection and monitoring of upper motoneuron degeneration in patients with amyotrophic lateral sclerosis. METHODS: Seventy patients with amyotrophic lateral sclerosis according to the El Escorial criteria were compared with 48 healthy control subjects. Single-volume proton magnetic resonance spectroscopy (echo time, 272 milliseconds; repetition time, 2000 milliseconds) was performed in both motor cortices for detection of N-acetylaspartate (NAA), phosphocreatine + creatine ([P]Cr), and choline-containing compounds (Cho) to calculate the metabolite ratios NAA/Cho, NAA/(P)Cr, and Cho/(P)Cr. In addition, absolute metabolite concentrations of NAA, (P)Cr, and Cho were obtained in 30 patients and 15 controls with the unsuppressed water signal used as an internal reference. RESULTS: Absolute concentrations of NAA (P<.001) and (P)Cr (P<.05) were reduced in motor cortices of patients, whereas Cho concentrations remained unchanged. The NAA/Cho and NAA/(P)Cr ratios were reduced in all El Escorial subgroups (P<.001). The Cho/(P)Cr ratio was elevated in patients with definite amyotrophic lateral sclerosis (P<.05). Metabolite ratio changes corresponded to the lateralization of clinical symptoms and were weakly correlated with disease duration and disease severity. In follow-up observations of 16 patients during a mean (+/-SD) of 12.1 +/- 8.7 months, NAA/Cho dropped by 9.1% (P<.01), and Cho/(P)Cr increased by 7.0% (P<.01). Changes of metabolite ratios were significantly correlated with progression of disease severity. CONCLUSIONS: Measurement of NAA concentrations and NAA/Cho ratios appear to be most suitable for detection of motor cortex degeneration by single-volume proton magnetic resonance spectroscopy. Reduced NAA/Cho ratios correspond to aspects of the clinical presentation and reflect disease progression in follow-up measurements.     

Case-control study of anxiety symptoms in hemifacial spasm.

In a case-control study, we evaluated symptoms in nine different psychological domains in hemifacial spasm (HFS; using the Symptom Checklist-90R [SCL-90R]) and found the anxiety score to be significantly greater in HFS compared to healthy controls in both the univariate (P = 0.004) and multivariate analysis (adjusted for sex, age, marital status, and educational level; P = 0.002). Similar findings were obtained when comparison was made with an independent group of outpatient controls. Compared to outpatient controls, the HFS patients had a higher mean Hamilton Anxiety Rating Score (HAM-A; 10.0 +/- 8.0 [range, 0 to 28] vs. 5.0 +/- 5.0 [range, 0 to 25]; P = 0.004), and 19.5% had HAM-A score of 18 or above compared to 3.8% in controls (P = 0.02). Among the HFS patients, the mean anxiety score in SCL-90R was significantly higher in those defined with mild to severe anxiety under HAM-A compared to those without anxiety (74.0 +/- 6.0 vs. 48.0 +/- 13.0) (P < 0.0005). There was good correlation of the anxiety score with the HAM-A in HFS patients (r = 0.915; P < 0.0001). HFS patients with anxiety reported significant improvement of their symptoms (mean HAM-A score 19.0 +/- 5.0 vs. 11.0 +/- 6.0; P = 0.001) following appropriate management. As stress and anxiety can aggravate HFS, diagnosis and early management of anxiety symptoms can improve quality of life in these patients.     

Sympathetic skin response in monomelic amyotrophy

OBJECTIVES: Monomelic amyotrophy (MMA) a variant of motor neuron disease, has the characteristic features of wasting and weakness usually confined to a single upper or lower limb occurring predominantly in young males and a benign outcome. Symptoms of increased sweating, coldness and cyanosis have been observed in a few patients. The objective was to evaluate the involvement of the sympathetic nervous system in MMA by measuring sympathetic skin response. METHODS: Electromyography, motor and sensory nerve conduction studies were done in all the four limbs of 9 patients with atrophy of one upper limb. Stimulation at Erb's point, and above and below elbow was done to look for evidence of conduction block. The sympathetic skin response (SSR) was recorded in all the limbs of these patients. Wasting and weakness of right upper limb in 7 patients and left upper limb in 2 patients was seen. The mean age was 28.3+/-10.1 years. Twenty-five age matched (24.8+/-4.8 years) healthy subjects served as controls. RESULTS: The mean SSR latency in the affected upper limbs of 9 patients was prolonged compared to the 25 control subjects (1.51+/-0.07 s vs 1.42+/-0.19 s, P=0.03). The mean value of SSR latency in 18 upper limbs of the 9 patients which included atrophied and unatrophied limbs was also prolonged compared to the controls (1.50+/-0.08 s vs 1.42+/-0.19 s, P=0.05). There was no significant difference of the mean latency of SSR between the atrophied upper limbs and the clinically normal upper limbs (1.51+/-0.07 s vs 1.49+/-0.09 s, P=0.51). The mean SSR latency in the lower limbs of the patients (2.09+/-0.09 s) did not significantly differ from the control subjects (1.97+/-0.28 s, P=0.09). Motor and sensory nerve conduction was normal and there was no evidence of conduction block. CONCLUSION: In MMA the sympathetic nervous system is involved in the atrophic upper limb and also in the clinically unaffected upper limb but not in the lower limbs.