Churg-Strauss syndrome (CSS) is a rare systemic vasculitis that affect small and medium-sized blood vessels and is accompanied by asthma, eosinophilia, and peripheral neuropathy. This report describes a case of a 52-year-old man who had a history of sinusitis, asthma, and thymus cancer and who had complained of bilateral lower extremity paresthesia and weakness for a month. Peripheral neuropathy was detected by electrodiagnostic studies. Resection of a mediastinal mass, which was diagnosed as thymic neuroendocrine carcinoma, was performed five months before his visit. After thymectomy, peripheral blood tests revealed a gradual increase in eosinophils. Two months after surgery, he was admitted to the hospital for dyspnea, and nodules of focal consolidation were found in his chest X-ray. One month later, pyoderma occurred in the right shin, and the skin biopsy showed extravascular eosinophilic infiltration. He was diagnosed with CSS after thymectomy, and we report a very rare case of CSS presented with thymic neuroendocrine carcinoma.
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To investigate risk factors for diabetic peripheral polyneuropathy and their correlation with the quantified severity of nerve dysfunction in patients with diabetes mellitus (DM).
A total of 187 diabetic patients with clinically suspected polyneuropathy (PN) were subclassified into 2 groups according to electrodiagnostic testing: a DM-PN group of 153 diabetic patients without electrophysiological abnormality and a DM+PN group of 34 diabetic patients with polyneuropathy. For all patients, age, sex, height, weight, duration of DM, and plasma glycosylated hemoglobin (HbA1c) level were comparatively investigated. A composite score was introduced to quantitatively analyze the results of the nerve conduction studies. Logistic regression analysis and multiple regression analysis were used to evaluate correlations between significant risk factors and severity of diabetic polyneuropathy.
The DM+PN group showed a significantly higher HbA1c level and composite score, as compared with the DM-PN group. Increased HbA1c level and old age were significant predictive factors for polyneuropathy in diabetic patients (odds ratio=5.233 and 4.745, respectively). In the multiple linear regression model, HbA1c and age showed a significant positive association with composite score, in order (β=1.560 and 0.253, respectively).
Increased HbA1c level indicative of a state of chronic hyperglycemia was a risk factor for polyneuropathy in diabetic patients and a quantitative measure of its severity.
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Acute transverse myelitis (ATM) is an upper motor neuron disease of the spinal cord, and concomitant association of peripheral polyneuropathy, particularly the axonal type, is rarely reported in children. Our cases presented with ATM complicated with axonal type polyneuropathy. Axonal type polyneuropathy may be caused by acute motor-sensory axonal neuropathy (AMSAN) or critical illness polyneuropathy and myopathy (CIPNM). These cases emphasize the need for nerve and muscle biopsies to make the differential diagnosis between AMSAN and CIPNM in patients with ATM complicated with axonal polyneuropathy.
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To demonstrate the prevalence and characteristics of subclinical ulnar neuropathy at the elbow in diabetic patients.
One hundred and five patients with diabetes mellitus were recruited for the study of ulnar nerve conduction analysis. Clinical and demographic characteristics were assessed. Electrodiagnosis of ulnar neuropathy at the elbow was based on the criteria of the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM1 and AANEM2). The inching test of the ulnar motor nerve was additionally performed to localize the lesion.
The duration of diabetes, the existence of diabetic polyneuropathy (DPN) symptoms, the duration of symptoms, and HbA1C showed significantly larger values in the DPN group (p<0.05). Ulnar neuropathy at the elbow was more common in the DPN group. There was a statistically significant difference in the number of cases that met the three diagnostic criteria between the no DPN group and the DPN group. The most common location for ulnar mononeuropathy at the elbow was the retrocondylar groove.
Ulnar neuropathy at the elbow is more common in patients with DPN. If the conduction velocities of both the elbow and forearm segments are decreased to less than 50 m/s, it may be useful to apply the AANEM2 criteria and inching test to diagnose ulnar neuropathy.
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To understand the quantitative correlation between the clinical severity and physical examinations along with the electrodiagnostic findings by subjects with carpal tunnel syndrome (CTS) and also the influence of diabetic polyneuropathy (DPN) on physical examinations by subjects with CTS.
Among 200 patients suffering from hand tingling sensations, 68 patients were diagnosed with CTS on at least one hand by nerve conduction tests. Therefore, the Phalen test (PT), hand elevation test (HET), Tinel sign (TS) results were recorded on both hands. The physical examination grades were compared with the electrophysiological CTS grades in 126 hands of 68 patients. Also the comorbidity effect of DPN to CTS was evaluated. For the evaluation of the severity correlations between CTS, PT, HET, and TS, the Spearman analysis was used. An attempt was started to create a formula which could depict the electrophysiological severity of CTS.
Out of the 68 tested subjects, 31 were diagnosed with both DPN and CTS, and 37 with CTS only. Both PT and HET correlated well with the severity of CTS where the correlation of PT was higher than that of HET. The formula were the motor distal latency (MDL)=(72.4-PT)/5.3 and MDL=(76-HET)/7.2. Both PT and HET showed in the presence of DPN a relatively higher relation with CTS without significance.
PT and HET would be useful screening tools for the diagnosis and treatment of CTS as the grade of PT and HET present the severity of CTS well. During this study, a formula was created expecting the severity of nerve conduction study with PT and HET through the time domain value of physical examinations.
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In herpes zoster infection, neurological complications may be overlooked because pain is a more prominent symptom and because peripheral polyneuropathy associated with weakness is rare. A 57-year-old male visited our hospital, complaining of pain and skin eruptions on the right flank. He was diagnosed as having herpes zoster and the symptoms were alleviated by administration of acyclovir for a week. After three weeks, the herpes zoster relapsed. He was re-admitted and diagnosed with chronic myeloid leukemia (CML), and imatinib mesylate was prescribed for five weeks. Ten weeks after the onset of herpes zoster, bilateral foot drops and numbness of the right foot dorsum developed. Through an electrodiagnostic study, he was diagnosed as having peripheral polyneuropathy that was suspected to be caused by neural invasion by varicella zoster virus. After administration of famciclovir, not only the pain but also the neurologic symptoms improved. We herein report a case of peripheral polyneuropathy that was supposed to be related to herpes zoster.
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To evaluate the prevalence and risk factors of peripheral neuropathy in patients with rheumatoid arthritis (RA) treated with leflunomide (LEF) by quantitative sensory testing (QST).
A total of 94 patients were enrolledin this study, out of which 47 patients received LEF. The other 47 patients received alternative disease-modifying antirheumatic drugs and served as the control group. The demographic characteristics, laboratory findings, concomitant diseases, and medication history were evaluated at the time of QST. The cooling (CDT) and vibratory detection threshold (VDT) as the representative components of QST were measured.
Age, gender, RA duration, ESR, and CRP did not show any significant differences between the two groups. VDT did not demonstrate any significant difference in both groups. However, CDT in LEF group was significantly higher than that of the control group (8.6±2.7 in LEF vs. 5.6±3.8 in control). The proportion of RA patients in the LEF group showing abnormally high CDT was over 2 times greater than that of the control group, but these findings were not statistically significant. Age, RA duration (or LEF medication in LEF group), ESR, and CRP did not show significant correlation with CDT in both groups. VDT significantly correlated with age in both groups.
LEF treatment in patients with RA may lead to abnormal CDT in QST. CDT value was not affected by age, RA duration, disease activity, or LEF duration. It remains to be determined whether QST may be a valuable non-invasive instrument to evaluate the early sensory changes in patients with RA taking LEF.
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MethodThe adult patient who were admitted to the ICU and taken ventilator care with endotracheal intubation were included. The time after admission was 48 to 144 hours. In case of axonal neuropathy of peripheral nerve, if affected nerves were in different two limbs or different three nerves were affected, CIP was diagnosed. If some nerves got abnormal results but did not satisfied the above criteria, the patient was classified as peripheral neuropathy group. The days of using neuromuscular blockade, continuous insulin infusion, catecholamine, vasopressor, corticosteroid, benzodiazepine, parenteral nutrition and fact for continuous renal replacement therapy, SOFA (sequential organ failure assessment) score were evaluated to find the risk factors.
ResultsEighteen patients were included. Six patients were CIP and another six were peripheral neuropathy. Risk factors for CIP were age, duration of intensive care, days of neuromuscular blockade and parenteral nutrition (p＜0.05). There was no difference on mortality rate among the three groups.
ConclusionThe result of early electrodiagnosis on ICU patients for CIP diagnosis revealed that risk factors of CIP were age, duration of intensive care, days of neuromuscular blockade and parenteral nutrition.
Method: Nerve conduction studies were performed in 23 patients with chronic renal failure. We not only measured distal latencies, amplitudes, and conduction velocities of median and ulnar motor nerves but also measured same parameters of radial sensory nerves at both upper limbs. In case of pateints with suspected peripheral polyneuropathy, we checked peripheral nerves at one lower limb. The results of nerve conduction studies and the frequency of cubital tunnel syndrome or carpal tunnel syndrome were compared between arteiovenous fistula side and non-arteiovenous fistula side.
Results: The amplitudes of median motor, ulnar motor nerves and radial sensory nerve in arteiovenous fisula side are statistically lower than those in non-arteiovenous fisula side (p＜0.05). In the 14 patients with peripheral polyneuropathy, the difference is also statistically significant between two sides (p＜0.05). Compared arteiovenous fisula side with non-arteiovenous fisula side, the frequency of cubital tunnel syndrome or carpal tunnel syndrome was not different between two sides.
Conclusion: Arteiovenous fisula may damage to the peripheral nerve in patients with chronic renal failure. (J Korean Acad Rehab Med 2003; 27: 85-89)
Method: Prospectively, total 40 patients with non-insulin dependent diabetes mellitus were included in the study. NCS was performed on median, ulnar, posterior tibial, deep peroneal, superficial peroneal, and sural nerves. Distal latency and conduction velocity (CV) of compound muscle action potential (CMAP), distal latency and amplitude of sensory nerve action potential (SNAP) were used as parameters of NCS. Multiple linear regression analysis were used to analyze the relations of HbA1c and parameters of NCS, after adjustment for age, height, weight, and disease duration of diabetes mellitus.
Results: HbA1c level had an inverse relation to CV of median motor nerve (β=1.272, p＜0.01), ulnar motor nerve (β=1.287, p＜0.01), posterior tibial nerve (β=0.982, p＜0.05), and deep peroneal nerve (β=1.449, p＜0.05).
Conclusion: This study indicates that HbA1c level was inversely related to motor nerve CV, and that sustained hyperglycemia may be involved in demyelination of motor nerves. Analysis of motor nerve CV related to HbA1c is expected to be useful in the follow-up or efficacy study of diabetes mellitus neuropathy as baseline data. (J Korean Acad Rehab Med 2003; 27: 80-84)
Objective: To evaluate the usefulness of the sural/superficial radial sensory nerve action potential amplitude ratio (SRAR) in the electrodiagnosis of diabetic polyneuropathy.
Method: Nerve conduction study was performed in 80 diabetic patients and 31 normal adults. Standard nerve conduction studies were performed. Sural nerve response was recorded with the active electrode on the posterosuperior margin of the lateral malleolus and stimulation 14 cm proximally. Superficial radial nerve response was recorded with the active on the snuffbox and stimulation 10 cm proximally. Baseline-to-peak sensory nerve action potential (SNAP) amplitudes were measured, and SRAR were obtained. The diabetic group was subdivided into an electrophysiologically normal group (DMNL, n=32) and neuro-
pathy group (DMPN, n=48). SRAR was compared among the control (NORM, n=31), DMNL and DMPN groups.
Results: SRAR was 0.344 in NORM, 0.314 in DMNL and 0.145 in DMPN, respectively. SRAR showed 77.1% sensitivity and 93.8% specificity. Sural SNAP amplitude sensitivity was 85.4% and specificity, 88.7%.
Conclusion: The relatively high specificity of SRAR indicates its usefulness in the diagnosis of diabetic polyneuropathy. However, as the superficial radial sensory SNAP amplitude decreased significantly in the diabetic groups compared to control, the SRAR is not superior to the sural SNAP amplitude in the diagnosis of diabetic polyneuropathy. (J Korean Acad Rehab Med 2002; 26: 147-151)
Organophosphate is known to damage both the peripheral and central nervous system. We report a case of organophosphate-induced peripheral polyneuropathy with myelopathy. A 46 years old woman who had ingested a large amount of insecticide (organophosphate) was transported to our hospital. Following medical treatment, she was transferred to the Department of Rehabilitation Medicine 1 month later. Upon admission to rehabilitation medicine, the patient was quadriplegic with markedly decreased muscle tone and strength. Electrodiagnostic examination revealed low amplitude of sensory nerve action potential (SNAP), unevokable compound muscle action potential in distal muscles and abnormal spontaneous activities with needle
electromyography, which were compatible with peripheral polyneuropathy. Three months later, motor and sensory function of upper extremities were normalized. The muscle tone of lower extremity increased to Ashworth grade II. Follow-up electrodiagnostic examination revealed normalization of SNAP and disappearance of spontaneous activities, but somatosensory evoked potential which were initially normal, revealed prolonged P40 latencies in the lower extremities. These electrophysiological findings were thought to result from the spinal cord lesion and correlated with clinical findings. We diagnosed the patient as peripheral polyneuropathy with delayed myelopathy induced by organophosphate. (J Korean Acad Rehab Med 2002; 26: 113-116)
Objective: To acknowledge whether flexion or extension of wrist joint produces any changes in median nerve conduction of the diabetes with or without polyneuropathy.
Method: With thirty healthy adults selected as control, 33 diabetes with polyneuropathy (Group I) and 21 diabetes without polyneuropathy (Group II) were studied. Before the study, the wrist joint was positioned in flexion or extension for 5 minutes. The variables used for the statistic analysis were mean changes of latencies and amplitudes in the median motor and sensory responses in neutral, flexed, and extended position.
Results: After wrist flexion or extension, there was no significant difference in the mean change of latencies and trans-carpal conduction velocities between Group I and Group II in the median motor and sensory nerve conduction studies, and in the mean change of amplitudes between the two groups in the median motor nerve study. But, there was significant difference in the mean change of amplitude between Group I and Group II in the median sensory nerve study after wrist extension.
Conclusion: We conclude that the change of amplitude in median nerve conduction study in different wrist position may be helpful to detect carpal tunnel syndrome with diabetic polyneuropathy in its early stage.
Objective: To evaluate the characteristics of peripheral nervous system involvement in patients with mucopolysaccharidoses (MPS).
Method: Electrophysiologic studies were performed in 26 children with MPS confirmed by semiquantitative MPS study, high resolution electrophoresis and enzyme assay. The age distribution of the patients were 2 to 18 year old (mean 8.2 year old).
Results: Of the 26 children, 21 children (80.8%) showed abnormal electrophysiologic finding. Eighteen children had median entrapment neuropathy at wrist level (carpal tunnel syndrome), 3 children had demyelinating peripheral polyneuropathies dominant in motor nerves.
Conclusion: The most prominent features of the peripheral nervous system involvement in MPS patients were entrapment neuropathy at wrist but concomittent peripheral polyneuropathy. Further studies would be necessary to clarify the characteristics of the peripheral polyneuropathy in MPS.
Infiltrative polyneuropathy is an infrequent complication of lymphoma or leukemia. The polyneuropathy can occur in patients with known malignancy, but sometimes it has been initial manifestation of underlying disease. The diagnosis was usually made at autopsy, because more common causes of polyneuropathy were frequently considered during life and many cases showed negative results on sural nerve biopsy. We experienced a case of steroid responsive polyneuropathy due to infiltration of malignant T cell on peripheral nerves prior to lymph node biopsy.
Castleman's disease is a rare clinicopathological entity characterized by multicentric angiofollicular lymph node hyperplasia and sometimes associated with polyneuropathy. We report 4 cases identified with diagnosis of Castleman's disease by lymph node biopsy and peripheral polyneuropathy. They had a hypesthesia of all limbs, gait disturbance, weakness of distal lower limbs and enlargement of lymph nodes. Among them 2 patients were combined with POEMS (polyneuropathy, organomegaly, endocrinopathy, increase of M protein, skin change) syndrome. Motor and sensory nerve conduction velocities were below 70% of lower normal limit, the amplitudes of compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) were reduced. Deterioration of nerve conduction study was more severe in lower limb than the upper. More denervation potentials were found in lower limbs than the upper and in distal limbs than the proximal. As disease getting worse, the slower conduction velocity and lower amplitude of CMAP and SNAP were observed.
Objective: The purpose of this study was to determine whether quantitative sensory test can be used as a screening test of peripheral polyneuropathy in patients with diabetes mellitus, and to evaluate the severity of peripheral polyneuropathy in patients with diabetes mellitus using quantitative sensory test.
Method: We performed nerve conduction study to right upper and left lower extremity of the patients. Quantitative sensory test was performed using TSA-2001 thermal sensory analyser on right thenar and left foot dorsum in both diabetic and control groups.
Results: 1) The warm sense and heat pain threshold were higher, the cold sense and cold pain threshold were lower in diabetic group than age-matched control group (p＜0.05). 2) The warm sense and heat pain threshold were higher, the cold sense and cold pain threshold were lower in diabetic group than young-aged control group (p＜0.05). 3) As nerve conduction study results were severe, the cold sense threshold in right thenar were decreased (p＜0.05).
Conclusion: Quantitative sensory study in patients with diabetes mellitus are sensitive to identify neuropathic change; thus, they would be used as the screening method of diabetic peripheral polyneuropathy.
Objective: The sympathetic skin response (SSR) was measured in patients with chronic renal failure (CRF) for diagnosis of uremic polyneuropathy and its correlations with nerve conduction study (NCS) and clinical autonomic symptoms were investigated.
Method: The SSR was measured in 15 patients with CRF on regular hemodialysis, aged 26 to 67 years. With median nerve stimulation at the wrist using the extremity without arteriovenous fistula, the SSR was recorded from both palm and sole simultaneously. The responses were interpreted as normal (presence) or abnormal (absence). Routine nerve conduction study was also performed in the same extremities and clinical autonomic symptoms were investigated.
Results: Nine of fifteen patients (60.0%) had symptoms suggestive of autonomic dysfunction: the most frequent findings were orthostatic dizziness and sweating problem. The SSR was absent in four of fifteen patients (26.7%). There is no significant relationship between SSR and autonomic symptoms (P＞0.05). The nerve conduction study was abnormal in eight of fifteen patients (53.3%), and the SSR was absent in two of seven patients with normal NCS. There is no significant relationship between NCS and SSR (P＞0.05).
Conclusion: Although the proportion of abnormal SSR was small, it may be a valuable method in the assessment of uremic polyneuropathy in conjunction with routine nerve conduction study in CRF patients.
A 45-year-old man with a long history of nephrotic syndrome presented with low back pain and progressive weakness of both legs for one day. Physical examination showed an acutely ill-appearing patient with a loss of both femoral artery pulses. Immediate digital subtraction angiography of abdominal aorta revealed total occlusion of the distal abdominal aorta. Transvascular embolectomy using urokinase was undertaken 6 hours later. Digital subtraction angiography after embolectomy revealed both common ilicac arteries to be patent with good distal flow. Electrodiagnostic examinations (post embolectomy 5th and 45th day) revealed peripheral polyneuropathy of both lower extremity. Anticoagulation therapy was continued and the patient was discharged several months later. During this period, there was improvement in both lower limbs from power of grade 1 to 4 except for both ankle dorsiflexors which had not recovered.
We report a rare case of peripheral ischemic polyneuropathy of both lower extremities as the result of acute abdominal aortic occlusion.
Objective: To assess the facial and trigeminal nerve involvement in diabetic patients using blink reflex study and direct facial motor conduction study.
Method: The subjects were 397 diabetic patients and 34 normal controls. Diabetic patients were subdivided into two groups based on the findings of nerve conduction studies of limb nerves.: Group I, patients with diabetic polyneuropathy; Group II, patients without diabetic polyneuropathy. The blink reflexes and direct facial motor responses and R1 latency/direct response latency (R/D) ratio were obtained in all the subjects. R1 latency was correlated to the findings of nerve conduction studies of limb nerves.
Results: 1) R1 latencies or R2 latencies were abnormally prolonged in 22.4% of Group I, 3.3% of Group II, and direct facial responses were abnormal in 11.8% of Group I, 2% of Group II. 2) There were no significant differences in R/D ratio between the two groups. 3) These findings suggest that not only the facial nerve, but also the trigeminal nerve or brain stem could be affected in diabetic patients with polyneuropathy.
Conclusion: In diabetic patients, blink reflex can provide useful information in determining the degree and distribution of cranial nerve and brain stem lesions.
Objective: The purpose of this study is to prove protective action of melatonin on the development of neuropathy in diabetic rat.
Method: The experimental rats (Sprague-Dawley) were divided into 3 groups: Group 1, streptozotocin-induced diabetic rats with trial of melatonin; Group 2, streptozotocin-induced diabetic rats without trial of melatonin; Group 3, normal control. Streptozotocin was injected intraperitoneally in group 1 and 2. Melatonin was administered per orally in group 1 from 1 week after the injection of streptozotocin. The melatonin effect on diabetic neuropathy was evaluated by the measurement of conduction velocities and amplitudes of rat tail mixed nerve action potentials. The electrophysiologic examinations were performed before and 2, 4, and 6 weeks after administration of streptozotocin.
Results: The rat tail mixed nerve conduction velocities were decreased at 4 weeks in group 1 and 2, and showed significant improvement at 6 weeks in group 1 as compared with those of group 2 (p＜0.05). The amplitudes of the compound nerve action potentials did not show difference before and after streptozotocin and melatonin trials, neither among groups.
Conclusion: In this experimental study, we observed the inhibitory effect of melatonin on the progression of polyneuropathy in early stage of diabetic rat. For the clinical application to human beings, further study is required.
Objective: To find simple screening method for assessment of patients with diabetic neuropathy by use of brief questionnaire, scored physical examination including vibration perception threshold, and mixed median conduction study.
Method: Subjects were 24 patients with diabetes mellitus. Conventional nerve conduction study was performed in 24 patients and the patients were divided into two groups: 11 patients with polyneuropathy; 13 patients of normal findings. The questionnaires were given for evaluation of sensory function in all the subjects. The questionnaires were followed by physical examination including two point discrimination, vibration perception threshold by using biothesiometer, 10 gram filament test, pin prick test, DTR check, and muscle strength test. Abnormality was determined by the number of positive responses or abnormal clinical findings. Finally, we recorded the conduction velocity and amplitude of median mixed nerve and compared these parameters to values of 20 normal adults.
Results: The questionnaire and physical exam scores were higher in patients with polyneuropathy (p＜0.01). The patients with polyneuropathy showed higher vibration perception threshold values (p＜0.01) and slower conduction velocity and smaller amplitude of median mixed nerve (p＜0.05). In correlation with conventional nerve conduction study, the median mixed nerve conduction velocity had higher kappa value than amplitude in screening for diabetic polyneuropathy.
Conclusion: We suggest the questionnaires, scored physical examination, vibration perception threshold, and median mixed nerve conduction velocity to be a simple screening method for assessment of patients with diabetic plyneuropathy.
Objective: To investigate the prevalance of carpal tunnel syndrome (CTS) and polyneuropathy (PNP) in chronic hemodialysis patients, and to know the relationships between the clinical symptoms and electrophysiological evidence of CTS, the edema of the hand and CTS, and the shunt side and CTS.
Method: We carried out a standardized nerve conduction study on 30 patients who had undergone a chronic dialysis for varying lengths of time. Differential diagnosis between CTS and PNP was done on the basis of difference of the median-ulnar motor and sensory latencies in the patients with a prolonged distal median motor or sensory latency.
Results: Sixteen (54%) had a combined PNP with the CTS; Seven (23%) patients had a PNP only; Two (7%) patients had a CTS only. There was no definite findings of peripheral neuropathy in five (17%) patients. Among eighteen patients with the CTS, sixteen were subclinical and two were overt CTS. Presence of edema and shunt was not crucial for the development of CTS.
Conclusion: Prevalence of CTS in chronic hemodialysis patients was 60%. Subclinical CTS was more frequent compared to the overt CTS. Dialysis patients need a frequent nerve conduction study for the early identification of carpal tunnel syndrome and to avoid the irreversible nerve damage.
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