空气波压力治疗仪在改善老年糖尿病病人周围神经病变中的作用

[目的]探讨空气波压力治疗仪在老年糖尿病病人周围神经病变中的作用。[方法]将80例糖尿病周围神经病变病人随机分为治疗组和对照组,两组各40例,两组采用常规降糖并给予凯时、弥可保治疗。治疗组在此基础上加用空气波压力治疗仪治疗。观察治疗前后感觉减退、麻木、疼痛及肌电图神经传导速度改善情况。[结果]在肢端麻木、感觉减退、疼痛的改善及肌电图传导速度上,治疗组优于对照组（P〈0．05）。[结论]空气波压力治疗仪能明显改善糖尿病周围神经病病人肢体麻木、感觉减退症状,减轻疼痛及改善神经传导速度,提高病人生活质量。     

Peripheral neuropathy associated with the hypereosinophilic syndrome

This case report documents the natural history of the peripheral polyneuropathy associated with persistent hypereosinophilia after treatment with corticosteroids. The neuropathy was documented by nerve conduction studies which revealed reduced amplitude of the sensory and motor evoked responses and slowed conduction velocities that were consistent with axon loss. Sural nerve biopsy revealed mild axonal loss distally without eosinophilic infiltration. The patient was treated with high-dose corticosteroids with a rapid normalization of his eosinophil count. Neurologic examination six months later revealed a slight improvement in his motor strength and reflexes but no change in sensory functions. Follow-up electromyographic evaluation demonstrated an average 89% improvement in evoked potential amplitudes and a slight improvement in distal latencies and conduction velocities. These findings suggest that the neuropathy associated with hypereosinophilia is axonal in nature and that there is a temporal relationship between reduction in the absolute eosinophil count and the improvement of the neuropathy.     

Peripheral neuropathy after cis-platinum (II) (DDP) therapy

A 56-year-old man developed a malignant fibrous histiocytoma in the left antecubital space in THE LEFT ANTECUBITAL SPACE IN September 1974. He underwent local resection, local radiation therapy, and chemotherapy with Adriamycin and Levamisole with resolution of the disease. In January 1977, a recurrent mass developed in the left antecubital space and electrophysiologic studies revealed segmental demyelination of the left median nerve across the tumor site. In July 1977, a Cis-diamminedichloplatinum (II) (DDP) therapy was instituted and 5 months later the patient developed paresthesias in both hands and both feet. Repeat nerve conduction studies revealed abnormalities in all 4 extremities consistent with a mixed sensorimotor peripheral neuropathy. Serial studies at 2-month intervals during DDP therapy showed further deterioration in nerve conduction. Following completion of DDP therapy, the patient's symptoms improved and subsequent nerve conduction studies done over an 11-month period showed improvement in nerve conduction with values approaching normal.     

Peripheral neuropathy after concomitant dimethyl sulfoxide use and sulindac therapy

The case is presented of a 63-year-old man with a long history of degenerative arthritis who took sulindac (Clinoril) 200 mg BID for 6 months with no untoward effects. Then, without physician knowledge, he began using 90% dimethyl sulfoxide (DMSO) topically to his upper and lower extremities. Shortly thereafter, he developed a profound mixed sensorimotor peripheral neuropathy. Serial electromyographic and nerve conducion studies performed at intervals of several months for 1 year suggested both segmental demyelination and axonal neuropathy. The patient experienced initial deterioration followed by gradual but incomplete recovery.     

Dyspnea as the predominant manifestation of bilateral phrenic neuropathy

Phrenic neuropathy associated with brachial neuritis has been well described; however, bilateral phrenic neuropathy with minimal or no involvement of the brachial plexus has not. We review the clinical features, as well as the results of radiographic studies, pulmonary function tests, and electrodiagnostic studies, of 3 patients in whom dyspnea was the presenting manifestation of bilateral phrenic neuropathy. All 3 patients had acute-onset dyspnea, which led to consideration of a pulmonary or cardiac etiology. Phrenic nerve conduction studies showed bilateral axonal degeneration of the phrenic nerves. Diaphragmatic paralysis should be considered in the differential diagnosis of acute-onset dyspnea. Dyspnea increases typically when the patient lies down, and paradoxical respiration may be present. Neck, shoulder, or upper limb pain may occur at onset. Inspiratory pressures may be reduced, but a comparison of the forced vital capacity when the patient is standing and supine is more specific for diaphragmatic weakness. Phrenic nerve conduction studies and diaphragmatic electromyography may provide evidence of bilateral involvement. Subclinical evidence of brachial plexus involvement may be present. The prognosis for patients with bilateral phrenic neuropathy may be more favorable than reported previously.     

干燥综合征伴周围神经病变的临床电生理与病理特点

目的 探究干燥综合征伴周围神经病变的临床电生理与病理特点.方法 以2018年1月至2021年1月收治的50例干燥综合征伴周围神经病变患者为试验组,同时以50例干燥综合征不伴周围神经病变患者为对照组.分析试验组临床电生理以及病理特点,比较两组患者临床表现的差异性.结果 试验组感觉神经传导异常患者中,10.00％为尺神经受...     

Clinical efficacy of fidarestat, a novel aldose reductase inhibitor, for diabetic peripheral neuropathy: a 52-week multicenter placebo-controlled double-blind parallel group study

OBJECTIVE: The purpose of this study was to evaluate the efficacy of fidarestat, a novel aldose reductase (AR) inhibitor, in a double-blind placebo controlled study in patients with type 1 and type 2 diabetes and associated peripheral neuropathy. RESEARCH DESIGN AND METHODS: A total of 279 patients with diabetic neuropathy were treated with placebo or fidarestat at a daily dose of 1 mg for 52 weeks. The efficacy evaluation was based on change in electrophysiological measurements of median and tibial motor nerve conduction velocity, F-wave minimum latency, F-wave conduction velocity (FCV), and median sensory nerve conduction velocity (forearm and distal), as well as an assessment of subjective symptoms. RESULTS: Over the course of the study, five of the eight electrophysiological measures assessed showed significant improvement from baseline in the fidarestat-treated group, whereas no measure showed significant deterioration. In contrast, in the placebo group, no electrophysiological measure was improved, and one measure significantly deteriorated (i.e., median nerve FCV). At the study conclusion, the fidarestat-treated group was significantly improved compared with the placebo group in two electrophysiological measures (i.e., median nerve FCV and minimal latency). Subjective symptoms (including numbness, spontaneous pain, sensation of rigidity, paresthesia in the sole upon walking, heaviness in the foot, and hypesthesia) benefited from fidarestat treatment, and all were significantly improved in the treated versus placebo group at the study conclusion. At the dose used, fidarestat was well tolerated, with an adverse event profile that did not significantly differ from that seen in the placebo group. CONCLUSIONS: The effects of fidarestat-treatment on nerve conduction and the subjective symptoms of diabetic neuropathy provide evidence that this treatment alters the progression of diabetic neuropathy.     

Electrophysiologic recovery after vitamin E-deficient neuropathy.

A case report is presented of an electrophysiologic recovery from vitamin E-deficient neuropathy after treatment with water-soluble vitamin E in a patient with chronic hepatobiliary disease. The patient was a 64-year-old man who had experienced progressive difficulty in ambulation, with ataxia, over the previous 3 years. The symptoms were associated with pain, tingling sensation in the extremities, and reduced fine motor activity. The patient had chronic hepatobiliary disease, with recurrent cholangitis and external drainage of bile acid through a T-tube for more than 20 years. Vitamin E level was barely detectable (<0.5 mg/L). Sensory conduction was absent in both sural nerves. Other sensory and motor conduction studies in the upper and lower extremities showed decreased amplitude. The patient was treated with water-soluble vitamin E. After 4 months of therapy, his ambulation function improved, but pain and tingling sensation in both hands remained. Sensory nerve action potentials appeared in both sural nerves, and amplitudes of other sensory nerves were increased. In a second follow-up study after 9 months, all of the evaluated parameters in the nerve conduction studies, as well as the vitamin E level, were normal. The authors conclude that vitamin E-deficient neuropathy is reversible and electrophysiologic recovery can occur with water-soluble vitamin E therapy.     

Relationship of nervous tissue transketolase to the neuropathy in chronic uremia

Patients with chronic uremia develop neurologic defects which are similar to the demyelinating lesions seen in thiamine deficiency. The present study describes inhibitory effects of uremic material on nervous tissue transketolase, a thiamine-dependent enzyme of the pentose phosphate pathway which has been reported to have functional importance in the metabolism of myelinated nervous structures.Transketolase activity (TKA) of normal human brain and spinal cord was measured by the conversion of ribose-5-phosphate (R5P) to sedoheptulose-7-phosphate (S7P). TKA was significantly inhibited by plasma, cerebrospinal fluid and low molecular weight dialysate fractions obtained from patients with uremic neuropathy, but not by samples from normal subjects. The specific effect on transketolase by uremic material was established by showing suppressed formation of S7P from R5P also in the presence of excess cofactor thiamine pyrophosphate and of the other substrate xylulose-5-phosphate. Uremic plasma likewise inhibited a partially purified transketolase preparation from bakers' yeast.31 of 35 chronic uremic patients with inhibition values between 10 and 84% before or during the early phase of intermittent hemodialysis had evidence of neuropathy. Data of clinical grading of the neurologic deficits and values of motor nerve conduction velocity revealed a correlation between the extent of uremic neuropathy and the degree of nervous tissue transketolase inhibition.Hemodialysis markedly reduced the inhibitory effects of the patients' plasma and the data indicate that uremic patients who received effective long-term dialysis treatment show a parallel decline of transketolase inhibition and uremic neuropathy.The findings demonstrate that in patients with chronic renal failure, low molecular weight factors accumulate and inhibit nervous tissue transketolase. This biochemical defect-uncorrectable by thiamine but reversible by dialysis-may interfere with the metabolism of myelin-supporting cells, and/or of the axonal metabolism of medullated structures, and may thus contribute to the degeneration of myelinated nerves seen with uremic neuropathy.     

Preventive effect of long-term aldose reductase inhibition (ponalrestat) on nerve conduction and sural nerve structure in the spontaneously diabetic Bio-Breeding rat.

To test the hypothesis that aldose reductase inhibition may prevent or delay the development of functional and structural neuropathy in the insulin-deficient diabetic Bio-Breeding rat (BB-rat), hyperglycemic rats were begun on the aldose reductase inhibitor (ARI) ponalrestat 25 mg/kg body wt soon after the onset of diabetes and followed for 4 or 6 mo. Ponalrestat treatment completely prevented the characteristic nerve conduction slowing and structural abnormalities of the node of Ranvier for 4 mo despite only partial preservation of axonal integrity. Ponalrestat treatment for 6 mo achieved a partial but significant prevention of nerve conduction slowing, axoglial dysjunction, and axonal degenerative changes. This incomplete but significant prevention of neuropathy by ponalrestat suggests that additional mechanisms besides polyol-pathway activation may be of importance in the pathogenesis of diabetic neuropathy. Alternatively, the dosage used in the present study may not have been sufficient to achieve a complete prevention. Despite the only partial protective effect of ARI treatment on degenerative peripheral nerve changes in hyperglycemic BB-rats, 6 mo of treatment resulted in a more than threefold increase in regenerating nerve fibers. These data suggest that prophylactic ARI treatment may be efficacious in delaying the development of diabetic neuropathy.     

Multifocal acquired demyelinating sensory and motor neuropathy misdiagnosed as carpal tunnel syndrome: a case report

Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), a subtype of chronic inflammatory demyelinating polyneuropathy, is a non-compressive peripheral nerve disorder. Symptoms of MADSAM include asymmetrical weakness and sensory deficits in the distribution of individual peripheral nerves, which are frequently noted in the distal portion of peripheral nerves. MADSAM can be easily misdiagnosed as any of the various compressive peripheral neuropathies. Here, we present a case of MADSAM misdiagnosed as carpal tunnel syndrome (CTS). A 53-year-old woman had bilateral asymmetrical hand weakness (left hand: significant weakness, right hand: slight motor weakness) and a slight weakness of her bilateral lower extremities. Sensory deficit was found on the volar side of her left hand. She had visited many clinics previously and was diagnosed with CTS. However, an electrodiagnostic study performed in our hospital did not identify CTS but indicated a demyelinating peripheral neuropathy in all limbs. On the basis of the patient’s clinical symptoms and laboratory findings, she was diagnosed with MADSAM. When patients exhibit progressive aggravating motor weakness and sensory deficits in more than one distal limb without a specific finding of compressive neuropathy in electrodiagnostic studies, clinicians should consider the possibility of MADSAM.     

Specific antinociceptive activity of cholest-4-en-3-one, oxime (TRO19622) in experimental models of painful diabetic and chemotherapy-induced neuropathy

Diabetes and cancer chemotherapies are often associated with painful neuropathy. The mechanisms underlying neuropathic pain remain poorly understood, and the current therapies have limited efficacy and are associated with dose-limiting side effects. We recently described the pharmacological characterization of cholest-4-en-3-one, oxime (TRO19622), a cholesterol-like compound, that significantly reduced axonal degeneration and accelerated recovery of motor nerve conduction in a model of peripheral neuropathy induced by crushing the sciatic nerve. These results triggered investigation of efficacy in other preclinical models of peripheral neuropathy. Here, we report evidence that daily oral administration of TRO19622, while similarly improving motor nerve conduction impaired in streptozotocin-induced diabetic rats, also reversed neuropathic pain behavior as early as the first administration. Further exploration of these acute antinociceptive effects demonstrated that TRO19622 was also able to reverse tactile allodynia in vincristine-treated rats, a model of chemotherapy-induced neuropathic pain. It is interesting to note that TRO19622 did not have analgesic activity in animal models of pain produced by formalin injection, noxious thermal or mechanical stimulation, or chronic constriction injury of the sciatic nerve, indicating that painful diabetic or chemotherapy-induced neuropathies share a common mechanism that is distinct from acute, inflammationdriven, or lesion-induced neuropathic pain. These results support the potential use of TRO19622 to treat painful diabetic and chemotherapy-induced neuropathies.     

The sensory symptoms of diabetic polyneuropathy are improved with alpha-lipoic acid: the SYDNEY trial

OBJECTIVE: Because alpha-lipoic acid (ALA), a potent antioxidant, prevents or improves nerve conduction attributes, endoneurial blood flow, and nerve (Na(+) K(+) ATPase activity in experimental diabetes and in humans and may improve positive neuropathic sensory symptoms, in this report we further assess the safety and efficacy of ALA on the Total Symptom Score (TSS), a measure of positive neuropathic sensory symptoms. RESEARCH DESIGN AND METHODS: Metabolically stable diabetic patients with symptomatic (stage 2) diabetic sensorimotor polyneuropathy (DSPN) were randomized to a parallel, double-blind study of ALA (600 mg) (n = 60) or placebo (n = 60) infused daily intravenously for 5 days/week for 14 treatments. The primary end point was change of the sum score of daily assessments of severity and duration of TSS. Secondary end points were sum scores of neuropathy signs (NIS), symptoms (NSC), attributes of nerve conduction, quantitative sensation tests (QSTs), and an autonomic test. RESULTS: At randomization, the groups were not significantly different by the criteria of metabolic control or neuropathic end points. After 14 treatments, the TSS of the ALA group had improved from baseline by an average of 5.7 points and the placebo group by an average of 1.8 points (P < 0.001). Statistically significant improvement from baseline of the ALA, as compared with the placebo group, was also found for each item of the TSS (lancinating and burning pain, asleep numbness and prickling), NIS, one attribute of nerve conduction, and global assessment of efficacy. CONCLUSIONS: Intravenous racemic ALA, a potent antioxidant, rapidly and to a significant and meaningful degree, improved such positive neuropathic sensory symptoms as pain and several other neuropathic end points. This improvement of symptoms was attributed to improved nerve pathophysiology, not to increased nerve fiber degeneration. Because of its safety profile and its effect on positive neuropathic sensory symptoms and other neuropathic end points, this drug appears to be a useful ancillary treatment for the symptoms of diabetic polyneuropathy.     

Continuous delivery of neurotrophin 3 by gene therapy has a neuroprotective effect in experimental models of diabetic and acrylamide neuropathies.

Neurotrophic factors (NFs) are promising agents for the treatment of peripheral neuropathies such as diabetic neuropathy. However, the value of treatment with recombinant NF is limited by the short half-lives of these molecules, which reduces efficiency, and by their potential toxicity. We explored the use of intramuscular injection of a recombinant adenovirus encoding NT-3 (AdNT-3) to deliver sustained low doses of NT-3. We assessed its effect in two rat models: streptozotocin (STZ)-induced diabetes, a model of early diabetic neuropathy characterized by demyelination, and acrylamide experimental neuropathy, a model of diffuse axonal neuropathy which, like late-onset human diabetic neuropathy, results in a diffuse sensorimotor neuropathy with dysautonomy. Treatment of STZ-diabetic rats with AdNT-3 partially prevented the slowing of motor and sensory nerve conduction velocities (p < 0.01 and p < 0.0001, respectively). Treatment with AdNT-3 of acrylamide-intoxicated rats prevented the slowing of motor and nerve conduction velocities (p < 0.001 and p < 0.0001, respectively) and the decrease in amplitude of compound muscle potentials (p < 0.0001), an index of denervation. Acrylamide-intoxicated rats treated with NT-3 had higher than control levels of muscle choline acetyltransferase activity (p < 0.05), suggesting greater muscle innervation. In addition, treatment of acrylamide-intoxicated rats with AdNT-3 significantly improved behavioral test results. Treatment with AdNT-3 was well tolerated with minimal muscle inflammation and no detectable general side effects. Therefore, our results suggest that NT-3 delivery by adenovirus-based gene therapy is a promising strategy for the prevention of both early diabetic neuropathy and axonal neuropathies, especially late axonal diabetic neuropathy.     

Electromyography and ultrasonography in the diagnosis of a rare double-crush ulnar nerve injury

Akyuz M, Yalcin E, Selcuk B, Onder B, Özçakar L. Electromyography and ultrasonography in the diagnosis of a rare double-crush ulnar nerve injury.Reported here is a 46-year-old man who was seen for pain, numbness, and weakness in his left upper limb and hand. Electromyographic studies demonstrated denervation of ulnar-innervated muscle groups except for the flexor carpi ulnaris. A localized nerve conduction block could not be depicted because of severe axonal loss. Ultrasonographic evaluation showed enlargement of the ulnar nerve at 2 sites: at the level of the epicondylar groove and the inside of the flexor carpi ulnaris muscle. Herein, we would like to emphasize the complementary role of an ultrasound in peripheral nerve pathologies, not only does it confirm the entrapment but it also displays the underlying cause(s).     

多发性硬化周围神经损害的临床与神经电生理分析

目的探讨多发性硬化（MS）患者合并周围神经损害的的临床和神经电生理特点。方法分析41例MS患者周围神经损害的临床，同时采用神经传导速度（NCV）技术检测MS患者周围神经的运动传导速度（MCV）、感觉传导速度（SCV）及其潜伏期、波幅；检测正中神经、胫神经F波、H反射的潜伏期和F波的出现率。结果MS合并PNS损害的发生率为41．4％，其临床表现主要为肢体麻木12例（29．2％）、肢体乏力10例（24．3％）、神经根性疼痛2例（4．8％）；体征有末梢／根型感觉障碍9例（21．9%）、腱反射减低5例（12．1％）、肌力减低（〈Ⅳ级）5例（12．1%）、肌萎缩3例（7．3%）。神经电生理改变为F波、H反射潜伏期延长，F波的出现率下降；神经电位波幅降低；不同程度运动感觉神经传导速度减慢及末端潜伏期延长。结论MS是一种以CNS受损为主的脱髓鞘疾病，部分患者可以同时累及PNS，神经电生理检测可对周围神经损害进行定位，同时可定量反映周围神经病变的程度。     

重金属中毒所致周围神经损害的电生理研究

目的：探讨处于重金属环境中的作业工人急、慢性中毒性周围神经损害的电生理改变。方法：应用常规肌电图技术对30例重金属接触者进行肌电图(EMG)、神经传导速度(NCV)检测。结果：30例患者的电生理检查均呈周围神经损害．其中急性起病18例．以轴索损害为主；慢性蓄积性中毒12例，以脱髓鞘损害为主；电生理改变下肢较上肢明显．感觉神经传导异常重于运动神经传导。结论：电生理学检查对研究重金属中毒所致的周围神经损害提供了重要的客观依据。可作为临床诊断、鉴别诊断以及了解病损程度的动态随访指标。     

History,Diagnosis, and Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is probably the best recognized progressive immune-mediated peripheral neuropathy. It is characterized by a symmetrical, motor-predominant peripheral neuropathy that produces both distal and proximal weakness. Large-fiber abnormalities (weakness and ataxia) predominate, whereas small-fiber abnormalities (autonomic and pain) are less common. The pathophysiology of CIDP is inflammatory demyelination that manifests as slowed conduction velocities, temporal dispersion, and conduction block on nerve conduction studies and as segmental demyelination, onion-bulb formation, and endoneurial inflammatory infiltrates on nerve biopsies. Although spinal fluid protein levels are generally elevated, this finding is not specific for the diagnosis of ClDP. Other neuropathies can resemble CIDP, and it is important to identify these to ensure correct treatment of these various conditions. Consequently, metastatic bone surveys (for osteosclerotic myeloma), serum electrophoresis with immunofixation (for monoclonal gammopathies), and human immunodeficiency virus testing should be considered for testing in patients with suspected CIDP. Chronic inflammatory demyelinating polyradiculoneuropathy can present as various subtypes, the most common being the classical symmetrical polyradiculoneuropathy and the next most common being a localized asymmetrical form, multifocal CIDP. There are 3 well-established, first-line treatments of CIDP—corticosteroids, plasma exchange, and intravenous immunoglobulin—with most experts using intravenous immunoglobulin as first-line therapy. Newer immune-modulating drugs can be used in refractory cases. Treatment response in CIDP should be judged by objective measures (improvement in the neurological or electrophysiological examination), and treatment needs to be individualized to each patient.     

Acute painful neuropathy restricted to the abdomen following rapid glycaemic control in type 2 diabetes

A 46-year-old Japanese man with type 2 diabetes mellitus, whose only diabetic complication was simple retinopathy, developed acute painful neuropathy. This presented as paresthesia and hyperesthesia restricted to the abdomen. The patient's haemoglobin A(1c) had dropped from 12% to 7.5% within 5 months, following a rapid improvement in glycaemic control. On investigation, there were no indications of disease in the intraabdominal area. Nerve conduction studies were consistent with mild sensorimotor peripheral and autonomic neuropathy. The patient required medication (mexiletine, sulpiride and imipramine hydrochloride) to control the pain. Four months after presentation, the symptoms showed a dramatic improvement and the treatment for pain relief was discontinued without any recurrence of paresthesia or hyperesthesia in the patient's abdomen. This was a very unusual case of diabetic post-treatment painful neuropathy in which the prominent features were severe pain, paresthesia and hyperesthesia restricted to the abdomen.     

Peripheral neuropathy in patients treated with leflunomide

OBJECTIVE: Our objective was to describe the clinical features, time course, and outcome of new-onset peripheral neuropathy occurring in patients treated with leflunomide. METHODS: Case reports of peripheral neuropathy submitted to the US Food and Drug Administration in association with leflunomide use were reviewed. Data on patient demographics, underlying medical conditions and medications, details of leflunomide therapy, and treatment and outcome of the neuropathy event were abstracted. Time to neuropathy onset and time to improvement or recovery were analyzed by survival analysis. RESULTS: Of 80 reported patients, 61% were women. The patients' mean age was 62 years. Symptoms of peripheral neuropathy began after a mean of 6 months of leflunomide use (range, 3 days to 3 years). Electrodiagnostic testing in 37 patients was consistent with a distal axonal, sensory, or sensorimotor polyneuropathy in most patients. Patients who stopped leflunomide use within 30 days of neuropathy symptom onset were more likely to have improvement or recovery than those who continued taking leflunomide for a longer period (P <.001). CONCLUSION: Leflunomide use is associated with peripheral neuropathy in some patients. This neuropathy is usually axonal in nature, affecting multiple sensory or motor nerves of distal extremities. Patients who stopped leflunomide use within 30 days of symptom onset were more likely to have improvement of symptoms or complete recovery than were patients who continued to use the drug for longer periods of time.