丹参注射液治疗糖尿病周围神经病变

筛选出非胰岛素依赖型糖尿病伴周围神经病变患者５３例，随机分两组。两组患者均接受糖尿病常规治疗，同时其中一组加用丹参注液治疗，一组做为对照，结果显示：与对照组相经治疗组症状改善显著，并且改善症状所需时间也缩短，表明丹参注射液有助于糖尿病周围神经病变的恢复。     

Characterization of the anti-neural antibodies in the Sera of leprosy patients

Sera from 43 leprosy patients were tested for antibodies that bound to normal human nerve. Thirty-eight percent showed positive staining as demonstrated by indirect immunofluorescence. Only 1 out of 30 control sera tested displayed similar staining. Western blots of myelin and neural intermediate filament (IF) proteins were tested with patient sera. Two of the anti-neural antibody (ANeAb)-positive leprosy sera bound to the P0 protein of PNS myelin. All 17 ANeAb-positive leprosy sera displayed 2 or more bands in the molecular weight range of Mr 45 000-55 000, when tested against IF proteins. One explanation for these findings is that leprosy patients produce antibodies to intermediate filament (IF) proteins released subsequent to the bacterial invasion of the peripheral nerves. The importance of these autoantibodies in the pathogenesis of leprosy is discussed.     

大鼠视神经损伤定量模型的建立

目的建立标准化视神经损伤大鼠动物模型，对致伤强度、损伤程度及两者之间的关系进行量化分析。方法在立体定位下，利用微电极毁损视神经颅内段，毁损电压为5V，频率为60kHz，通过改变电毁损的电流强度，造成不同程度视神经损伤。然后进行视网膜切片，计数视神经节细胞层细胞，定量视神经损伤。结果析因方差分析结果显示：视神经节细胞计数存不同刺激时间之间，差异硅著（F=3472，14，P〈0．001）；在不同电流强度组间，差异显著（F=335．83，P〈0．001）；经LSD法多重比较，视神经节细胞计数在刺激电流之间及刺激时间之间差异在α=0．05水平均有显著性意义。刺激强度和刺激时间的单独效应：同一电流组随着刺激时间增加．视神经节细胞计数呈下降趋势；除对照组和90s组外（F=0．79，P=0．548；F=1．54，P=0．242），刺激时间相同时，随电流强度增加．细胞计数也呈下降趋势，刺激电流强度与刺激时间之间交互效廊显著（F=27．30，P〈0．001）：结论立体定向电毁损大鼠颅内段视神经模型可以测定致伤强度和视神经损伤程度，是较理想的视神经损伤模型。     

Mortality in patients with diabetic neuropathic osteoarthropathy (Charcot foot).

OBJECTIVE: To determine the mortality of a population of patients diagnosed with Charcot neuropathic osteoarthropathy managed by a single specialist unit and to compare the results with a control population. METHODS: We have undertaken a retrospective analysis of all cases of Charcot foot on the comprehensive database which has been maintained at the specialist diabetic foot clinic at the City Hospital, Nottingham since 1982. Survival and the incidence of amputation (major and minor) was compared with a control population referred with uncomplicated neuropathic ulceration. Controls were individually matched for gender, age (+/-2 years), disease type, disease duration (+/-2 years) and year of referral (+/-3 years). RESULTS: Forty-seven cases (21 female, 26 male) of Charcot foot were identified, of whom 18 (38.3%) had Type 1 diabetes. Mean age and disease duration at presentation were 59.2 +/- 13.4 (sd) and 16.2 +/- 11.2 years, compared with 59.7 +/- 12.6 and 16.3 +/- 11.2 years, respectively, in the controls. Twenty-one (44.7%) of those with Charcot had died, after a mean interval of 3.7 +/- 2.8 years. This compared with 16 (34.0%) after a mean 3.1 +/- 2.7 years in the control group. Mean duration of follow-up in the survivors was 4.7 +/- 4.9 years (Charcot) and 5.3 +/- 3.9 years (controls). A total of 11 (23.4%) Charcot patients had had a major amputation on the side of the index lesion, compared with five (10.6%) controls. There was no difference between the two groups (P > 0.05, Chi-square). CONCLUSIONS: The mortality in this group of patients with Charcot foot was higher than expected. Nevertheless, there was no difference between those with Charcot and those with uncomplicated neuropathic ulceration. It is possible that it is neuropathy, rather than Charcot osteoarthropathy, which is independently associated with increased mortality in diabetes. The mechanism underlying any such association is not known. There is a need for a formal, prospective, multicentre study to investigate the life expectancy and cardiovascular risk of those with Charcot osteoarthropathy.     

Hypoxia‐inducible factor 1α may be a marker for vasculitic neuropathy

Neuromuscular biopsy is still an essential method for diagnosing vasculitic neuropathy, although its diagnostic sensitivity is at most 60%. Our objective was to examine the expression of hypoxia‐inducible factor 1α (HIF‐1α) in peripheral nerves and to evaluate its usefulness in diagnosing vasculitic neuropathy, especially for discrimination from other axonal neuropathies. Forty‐one patients with vasculitic neuropathy consisting of 20 definite, 14 probable and seven possible diagnoses, 15 patients with metabolic neuropathy, five with motor neuron disease and six with chronic inflammatory demyelinating polyneuropathy were included. Nerve biopsy specimens were immunohistochemically examined for HIF‐1α and various cell markers. Distinct immunoreactivity (IR) was observed in nuclei of endoneurial cells in 54% (22/41) of vasculitic patients, while specimens from metabolic neuropathies showed less nuclear IR and the difference of mean density of HIF‐1α‐positive nuclei was significant. Two patients with possible vasculitis who showed HIF‐1α‐positive nuclei in endoneurium, were later confirmed to have vasculitis by skin biopsies. Most of the cells expressing HIF were demonstrated to be Schwann cells. There was a trend in the vasculitic patients with early phase nerve damage to display higher endoneurial HIF‐1α‐IR. HIF‐1α may be an immunohistochemical marker for vasculitic neuropathy, especially when the observed section contains no vasculitic lesions.     

Novel C59T leader peptide mutation in the MPZ gene associated with late-onset, axonal, sensorimotor polyneuropathy

The objective of this study was to report a novel exon-1 mutation in the myelin protein zero (MPZ) gene, resulting in axonal Charcot–Marie–Tooth neuropathy with recurrent hyper-CK-emia. In a 64-year-old woman slowly progressive distal lower limb weakness, muscle cramps in the lower limb muscles, and stocking-type numbness had developed from the age of 61. Neurologic examination revealed discrete hip flexor weakness, weakness for foot extension, diffuse wasting of the distal lower limb muscles, reduced patella tendon reflexes, and absent Achilles tendon reflexes. There was recurrently elevated creatine kinase with a maximum of 607 U/l ( n , <145 U/l). Stimulation of the peroneal and tibial nerves did not evoke a muscular response. Electromyography was neurogenic. Biopsy of the right sural nerve showed diffuse axonal degeneration and loss of axons of all diameters. Muscle biopsy showed increased fiber-size variability, angulated fibers, internalized nuclei, accumulations of nuclei, grouped atrophic muscle fibers, and fiber splitting. Molecular genetic analysis by PCR and direct nucleotide sequencing revealed the heterozygous C59T exon-1 MPZ gene mutation, resulting in the amino acid exchange S20F of the MPZ signal protein domain (leader peptide). The novel C59T mutation in the leader peptide of the MPZ gene is pathogenic and manifests as severe, late-onset, axonal, symmetric sensorimotor polyneuropathy (CMT2) and hyper-CK-emia.     

Sudden cardiorespiratory arrest after renal transplantation in a patient with diabetic autonomic neuropathy and prolonged QT interval

A 31–yr male with insulin dependent diabetes mellitus for 20 years underwent general anaesthesia for renal transplantation. During transfer from operating theatre to ICU he developed bradycardia advancing to ventricular fibrillation and had to be resuscitated. Bradycardia did not respond to atropine. Postoperative autonomic nervous function tests showed advanced autonomic neuropathy. He was found to have constantly prolonged QTc interval in his pre– and postoperative ECGs (462–503 ms). Prolongation of QTc interval could be used as a valuable predictor of postoperative cardiac complications in diabetic patients with autonomic neuropathy.     

Neuropathy associated with “benign” anti-myelin-associated glycoprotein IgM gammopathy: Clinical,immunological, neurophysiological pathological findings and response to treatment in 33 cases

We studied 33 patients presenting with a peripheral neuropathy associated with non-malignant anti-myelin-associated glycoprotein (MAG) IgM monoclonal gammopathy (MG) in an attempt to delineate their clinical, immunological, electrophysiological and pathological characteristics; we also reviewed our experience concerning long-term follow-up and therapy. Peripheral neuropathy associated with non-malignant anti-MAG IgM MG was observed mostly in males (sex ratio 7.2), and mean age at onset was 67 years (range 46–81). A predominantly sensory pattern was noted in more than 80% of cases, although some patients were affected by a predominantly motor peripheral neuropathy. Although disease progression was slow in most cases, 45% of patients suffered severe disability, and in 2 cases, the patient's death appeared to stem directly from the neuropathy. The electrophysiological findings were indicative of a demyelinating process in 90% of cases, and electron microscopic examination of nerve biopsy specimens demonstrated widening of the myelin lamellae in more than 95% of cases. Most of our patients showed a disappointing response to steroids and chemotherapy or plasma exchanges. Intravenous immune globulin, evaluated in 17 patients, had a transient, mostly subjective effect in 35% and led to a clear-cut improvement in 24% of cases. We did not observe any correlation between the severity of the clinical picture and the anti-sulphoglucuronyl paragloboside antibody titre; in individual cases, clinical improvement occurred without lowering of IgM levels. Although the severity and the rate of progression may greatly vary from patient to patient, the combination of clinical, electrophysiological and pathological features delineates a characteristic pattern in peripheral neuropathy associated with non-malignant anti-MAG IgM MG.     

Approved indications of lipo-PGE1 in Japan

For its peripheral vascular dilating effect and platelet agglutination inhibitory activity, prostaglandin E₁ is used in the treatment of diseases which are likely to cause peripheral circulatory failure or thrombus. In Japan, lipo-PGE₁, which was developed to give it a target-directed nature by modifying the conventional PGE₁, has been used and found to be useful in clinical practice. In this report, we attempt to describe the clinical benefits of lipo-PGE₁ focusing on the diseases which have been approved for its indications.