Adrenoleukodystrophy 1례

We describe a 9-year-old boy who showed typical neurologic manifestations i.e., progressive behavioral changes, intellectual impairment, visual disturbances and hearing loss, cerebellar and pyramidal signs with characteristic neuroimaging features, which led us to make a clinical deagnosis of ALD. It was confirmed later by demonstration of increased VLCFA levels in RBC membrane using HPLC. He has no family history of neurologic or endocrine disorder. Prophylactic antiepileptic medicaion could not prevent the development of seizure disorder

Crossed Cerebellar Diaschisis in Cerebral Infarction: Correlation of SPECT and Clinical Features

Patients with supratentorial cerebral infarction frequently show depressed metabolic activity in the contralateral cerebellar hemisphere which is known as crossed cerebellar diaschtsisfCt.D). In order to investigate the relationship between this phenomenon and the characteristics of the supratentorial lesion, we retrospectively evaluated the findings of 99mTc-HMPAO single photon emission computed tomography(SPECT) in 26 patients with a single supratentorial infarction lesion. A cerebellar asymmetry index (AIcbll), percent difference between both cerebellar hemispherestzx'Scbll],SPECT volume deficit (SVD), and magnetic resonance volume deficit (MVD) were quantitated. A CCD, defined as AIcbll >12%, was observed in 12 of the 26 patients (46.2%). No correlation was found between the ~%cbll and duration of disease, SVD, or MVD. SVD and MVD values showed no significant difference between CCD positive and negative groups (71+47ml \IS. 70+68ml and 90+84ml \IS. 67+77ml, respectively). Patients with frontoparietal lobe or deep middle cerebral artery territory infarctions showed a significantly higher incidence of CCD and lower ~%cbll values. Patients with severe hemiparesis had a higher incidence of CCD and lower ~%cbll values than those with milder or no hemiparesis (incidence, 5/5 \IS. 6/18, p=0.008; ~ %cbll,-21. 4+3.8% \IS -8. 3±11. 1%, p=O. 014). None of the 12 patients with CCD showed clinical signs of cerebellar dysfunction. In conclusion, the location rather than the extent of the lesion appears to be the major determinant for the occurrence and magnitude of CCD in stroke patients

A Study on Selective Cerebellar Degeneration Following ,β-Fluorethyl Acetate Poisoning

This is to report clinical and radiological findings in 7 patients with selective cerebellar degeneration following ,B-fluoroethyl acetate intoxication. ,B-Fluoroethyl acetate, an ethyl ester of fluoroacetate which is a metabolic inhibitor of Krebs cycle is used as a rodenticide. Following initial stage of coma from intoxication, the patients woke up to show selective cerebellar dysfunction, often so severe as not to be able to sit or stand unsupported. They improved gradually over several months to years with variable degrees of residual cerebellar dysfunction. Gait disturbance and dysarthria were the most prominent and persistent factors of the cerebellar dysfunction, whereas mild nystagmus was rarely seen. Cognitive function was not impaired. Cerebellar atrophy became noticeable on CT and MRI 4 weeks after poisoning, and progressed over time even with clinical improvement. Cerebellar degeneration contrasts with pallidal degeneration following carbon monoxide poisoning. ,B-Fluoroethyl acetate may be selectively toxic to the cerebellum

Randomized Trial of Stents Versus Bypass Surgery for Left Main Coronary Artery Disease 5-Year Outcomes of the PRECOMBAT Study

AbstractBackgroundIn a previous randomized trial, we found that percutaneous coronary intervention (PCI) was not inferior to coronary artery bypass grafting (CABG) for the treatment of unprotected left main coronary artery stenosis at 1 year.ObjectivesThis study sought to determine the 5-year outcomes of PCI compared with CABG for the treatment of unprotected left main coronary artery stenosis.MethodsWe randomly assigned 600 patients with unprotected left main coronary artery stenosis to undergo PCI with a sirolimus-eluting stent (n = 300) or CABG (n = 300). The primary endpoint was a major adverse cardiac or cerebrovascular event (MACCE: a composite of death from any cause, myocardial infarction, stroke, or ischemia-driven target vessel revascularization) and compared on an intention-to-treat basis.ResultsAt 5 years, MACCE occurred in 52 patients in the PCI group and 42 patients in the CABG group (cumulative event rates of 17.5% and 14.3%, respectively; hazard ratio [HR]: 1.27; 95% confidence interval [CI]: 0.84 to 1.90; p = 0.26). The 2 groups did not differ significantly in terms of death from any cause, myocardial infarction, or stroke as well as their composite (8.4% and 9.6%; HR, 0.89; 95% CI, 0.52 to 1.52; p = 0.66). Ischemia-driven target vessel revascularization occurred more frequently in the PCI group than in the CABG group (11.4% and 5.5%, respectively; HR: 2.11; 95% CI: 1.16 to 3.84; p = 0.012).ConclusionsDuring 5 years of follow-up, our study did not show significant difference regarding the rate of MACCE between patients who underwent PCI with a sirolimus-eluting stent and those who underwent CABG. However, considering the limited power of our study, our results should be interpreted with caution. (Bypass Surgery Versus Angioplasty Using Sirolimus-Eluting Stent in Patients With Left Main Coronary Artery Disease [PRECOMBAT]; NCT00422968

DEVELOPING TIME-BASED CLUSTERING NEURAL NETWORKS TO USE CHANGE-POINT DETECTION: APPLICATION TO FINANCIAL TIME SERIES

This study suggests time-based clustering models integrating change-point detection and neural networks, and applies them to financial time series forecasting. The basic concept of the proposed models is to obtain intervals divided by change points, to identify them as change-point groups, and to involve them in the forecasting model. The proposed models consist of two stages. The first stage, the clustering neural network modeling stage, is to detect successive change points in the dataset, and to forecast change-point groups with backpropagation neural networks (BPNs). In this stage, three change-point detection methods are applied and compared. They are: (1) the parametric approach, (2) the nonparametric approach, and (3) the model-based approach. The next stage is to forecast the final output with BPNs. Through the application to financial time series forecasting, we compare the proposed models with a neural network model alone and, in addition, determine which of three change-point detection methods performs better. Furthermore, we evaluate whether the proposed models play a role in clustering to reflect the time. Finally, this study examines the predictability of the integrated neural network models based on change-point detection.Clustering, change-point detection, backpropagation neural networks, Chow test, Pettitt test, likelihood ratio test

Suture-Tape Augmentation for Chronic Ankle Instability

Category: Ankle Introduction/Purpose: This retrospective study was performed to evaluate the clinical outcomes of internal brace technique using suture tape for chronic ankle instability. Methods: twenty-three patients were followed for more than 2 years after suture tape augmentation for lateral ankle instability. Twenty five patients were followed for more than 2 years after modified Brostrom procedure. The clinical results after using two techniques were evaluated and compared using Foot and Ankle Outcome Score (FAOS), American Orthopaedic Foot and Ankle Society (AOFAS) score, Olarud and Molander score, Hamilton score and Visual Analogue Scale (VAS). Anterior talar translation and talar tilt angle were obtained from stress radiographs to evaluate the mechanical ankle stability. Results: There was no significant difference in VAS at final follow-up (P < .325). There was no significant difference in FAOS at final follow-up (P < .242). There was no significant difference in AOFAS score at final follow-up (P < .418). There was no significant difference in Olarud and Molander score at final follow-up (P < .244). According to the Hamilton score, 14 cases (93%) in internal brace group and 23 cases (92%) achieved satisfactory functional results. Talar tilt angle and anterior talar translation had improved to an average of 2.2 degrees and 4.4 mm in internal brace group and 2.7 degrees and 5.4 mm in modified Brostrom group, respectively, at final follow-up, and there was no significant difference (P < .128, < .226, respectively). There were no complications such as skin irritation and wound infection. Conclusion: Suture tape augmentation has shown the potential for stabilizing the lateral ankle ligmament complex as an alternative to direct repair. Functional, objective, and subjective outcomes were excellent in all cases and no complications were seen postoperatively. To confirm the above further, it is appropriate to conduct a larger study