短潜伏期SEPs预测严重外伤性脑损伤致持续急性昏迷觉醒和预后的可靠性研究

Objective: To evaluate the prognostic value of somatosensory evoked potentials (SEPs) in severe traumatic brain injury (TBI) considering both ‘awakening’and disability. Methods: SEPs were recorded in 60 severe TBI with duration of acute coma > 7 days. N20-P25 amplitudes, their side-to-side asymmetry and CCT were measured. SEPs on each hemisphere were classified as normal (N), pathological ( P) or absent (A). ‘Awakening’and disability were assessed after at least 12 mo nths using Glasgow Outcome Scale (GOS). SEP predictive value was compared with G CS and EEG reactivity. Results: Seventy-five percent regained consciousness. 29 /60 had a good outcome (GOS 4-5) and 31/60 had a bad outcome (GOS 1-3). Accord ing to the ROC curve, SEP findings were classified in 3 grades. Grade I (NN, NP) had PPV of 93.1%for ‘awakening’and 86.2%for good outcome. Grade III (AA) ha d PPV of 100%for bad outcome and 72.7%for ‘awakening’. Grade II (PP, NA, PA) was associated with the wider range of outcome. A multivariate analysis includi ng SEP grading, GCS and EEG reactivity did not increase the percentage of cases prognosticated by SEP alone. Conclusions: We confirm the high predictive value o f SEPs in TBI, which is greater than GCS and EEG reactivity.Indeed, SEP grades I and III were able to predict the correct prognosis in more than 80%of severe T BI. Therefore, SEPs should be used more widely in the prognosis of severe TBI.Si gnificance: Differently from post-anoxic, in post-traumaticcoma the presence o f normal SEPs has a favourable predictive value both for ‘awakening’and disabi lity. We think that in literature enough attention has still not been paid to th is finding.

Lewis-Sumner综合征和多灶性运动神经元病

We compared the clinical, electrophysiological, laboratory, and pathological features of 13 patients with Lewis-Sumner syndrome (LSS) with those of 20 pati ents with multifocal motor neuropathy (MMN). LSS and MMN patients have several c ommon clinical features: age at onset, weakness in the distribution of individua l peripheral nerves, mild wasting, cramps and fasciculations, partial areflexia, and frequent stepwise disease course. Cerebrospinal fluid protein level was nor mal or slightly elevated, but always less than 100 mg/dl. Conduction blocks are the electrophysiological hallmarks of these two neuropathies, and no differences in distribution and number of blocks were found. Contrary to MMN, lower-limb involvement at onset was frequent in LSS but extension to the upper limbs was a frequent later feature of the disease. Cranial nerve involvement was noted in 4 LSS patients during relapses and absent in all MMN patients. The major distingui shing features were the clinical and electrophysiological sensory involvement in LSS, and the lack of anti-GM1 antibodies in LSS, whereas IgM anti-GM1 were found in 40% of MMN patients. Some LSS patients responded to steroid therapy, whereas this was ineffective in MMN. From these features, LSS can be considered an entity distinct from MMN, with its own clinical, laboratory, and electrophysi ological characteristics, and as an intermediate link between chronic inflammato ry demyelinating polyneuropathy and MMN.

ECG和心电遥测异常可预测卒中患者3个月时的预后

Background: ECG is a useful tool in monitoring vital functions in patients wi th acute stroke; however, fairly little evidence is available concerning the pre valence and the prognostic impact of ECG findings in patients with acute cerebra l infarction and acute intracerebral haemorrhage (ICH). Methods: This analysis w as based on data from 692 patients with acute cerebral infarction, 155 patients with intracerebral haemorrhage (ICH), and 223 patients with transient ischaemic attack (TIA), who were admitted to hospital within 6 h of symptom onset. A 12 le ad ECG was obtained on admission, and the patient was on telemetry for the first 12- 24 h of hospitalisation. Results: ECG abnormalities were observed in 60% of patients with cerebral infarction, 50% of patients with ICH, and 44% of patients with TIA. In multivariate analyses 3- month mortality in patients with ischaemic stroke was predicted by atrial fibrillation OR 2.0 (95% CI 1.3- 3. 1), atrio- ventricular block OR 1.9 (95% CI 1.2- 3.9), ST- elevation OR (2. 8, 95% CI 1.3- 6.3), ST- depression OR 2.5 (95% CI 1.5- 4.3), and inverte d T- waves OR 2.7 (95% CI 1.6- 4.6). This was independent of stroke severity , pre- stroke disability and age. In patients with ICH, sinus tachycardia OR 4. 8 (95% CI 1.7- 14.0), ST- depression OR 5.2 (95% CI 1.1- 24.9), and inver ted T- wave 5.2 (95% CI 1.2- 22.5) predicted poor outcome. None of the chang es reached significance in patients with TIA. In patients with severe cerebral i nfarction or ICH, heart rate did not decrease within the first 12 h after admiss ion, which was the case in patients with mild to moderate stroke. Rapid heart ra te predicted 3- month mortality in multivariate testing OR 1.7 (95% CI 1.02- 2.7). Conclusions: ECG abnormalities are frequent in acute stroke and may predi ct 3- month mortality.

Leber遗传性视神经病变患者氧化应激诱导的细胞凋亡反应

Objectives: Leber’s hereditary optic neuropathy (LHON)is a maternally inher ited disease in which acute or subacute bilateral visual loss occurs preferentia lly in young men. Over 95%of LHON cases are associated with one of three mitoch ondrial DNA (mtDNA) point mutations, but only 50%of men and 10%of women who ha rbour a pathogenetic mtDNA mutation develop optic neuropathy. This incomplete pe netrance and preference for men suggests that additional genetic (nuclear or mit ochondrial) and/or environmental factors must modulate phenotype expression in L HON. A role for reactive oxygen species (ROS) in mitochondrial diseases, seconda ry to mtDNA mutations, or as a result of the direct effect of ROS cytotoxicity, has been implicated in many mitochondrial disorders, including LHON. The purpo se of this study was toinvestigate the role of oxidative stressinduced apoptosis in LHON. Methods: The 2-deoxyDribose induced apoptotic response of periphe ral blood lymphocytes from six patients with LHON and six healthy subjects was i nvestigated using light microscopy, flow cytometry, agarose gel electrophoresis, and the measurement of mitochondrial membrane potential. Results: Cells of pati ents with LHON had a higher rate of apoptosis than those of controls and there w as evidence of mitochondrial involvement in the activation of the apoptotic casc ade. Conclusions: These differences in oxidative stress induced apoptosis are in line with the hypothesis that redox homeostasis could play a role in the expres sion of genetic mutations in different individuals and could represent a potenti al target in the development of new therapeutic strategies.

散发MM2型克-雅病的临床诊断

Background: No method for the clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease (sCJD) has been established exce pt for pathologic examination. Objective: To identify a reliable marker for the clinical diagnosis of MM2-type sCJD.Methods: CSF, EEG, and neuro imaging studie s were performed in eight patients with MM2-type sCJD confirmed by neuropatholo gic,genetic, and western blot analyses. Results: The eight cases were pathologic ally classified into the cortical (n= 2), thalamic (n = 5), and combined (cortic othalamic) (n =1) forms. The cortical form was characterized by late-onset,slow ly progressive dementia, cortical hyperintensity signals on diffusion-weighted imaging (DWI) of brain, and elevated levels of CSF 14-3-3 protein. The thalami c form showed various neurologic manifestations including dementia, ataxia, and pyramidal and extrapyramidal signs with onset at various ages and relatively lon g disease duration. Characteristic EEG and MRI abnormalities were almost absent. However, all four patients examined with cerebral blood flow (CBF) study using SPECT showed reduction of the CBF in the thalamus as well as the cerebral cortex . The combined form had features of both the cortical and the thalamic forms, sh owing cortical hyperintensity signals on DWI and hypometabolism of the thalamus on [18F]2-fluoro-2-deoxy-D-glucose PET. Conclusion: For the clinical diagno sis of MM2-type sporadic Creutzfeldt-Jakob disease,cortical hyperintensity sig nals on diffusion-weighted MRI are useful for the cortical form and thalamic hy poperfusion or hypometabolism on cerebral blood flow SPECT or [18F]2-fluoro-2 -deoxy-D-glucose PET for the thalamic form.

依那西普治疗皮质类固醇依赖性重症肌无力

The authors report a prospective pilot trial of etanercept in corticosteroid -dependent autoimmune myasthenia gravis. Eleven patients were enrolled, with e ight completing the 6-month trial. Two patients were withdrawn owing to diseas e worsening, and one patient was withdrawn because of an erythematous skin rash. Six of the eight patients who completed the trial improved, based on quantitati ve measures of muscle strength and lowering of corticosteroid requirement.

Improvement of gait of freezing in patients with Parkinson’s disease by electrical stimulation to common peroneal nerve delivered by wearable stimulator

Objective To explore whether the proprioceptive sensory cueing delivered by electrical stimulator to common peroneal nerve can improve the freezing of gait of parkinsonian patients.Methods Thirty patients with Parkinson’s disease experiencing freezing of gait(FOG)admitted to the First Affiliated Hospital of Anhui Medical University from January to December 2018 were included in the trial.