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This paper is to investigate the current challenges, trends, relevance and the concept of supply chain performance measurements in developing nation basic metal industries. This paper gives an overview of different performance measurement tools that can be applied to measure and improve the supply chain systems of an organization. Since the study is focuses on supply chain performance measurements practices in the Ethiopian basic metal industries. The paper identifies and discusses the main motives and impact of supply chain performance measurement practice on the performance of basic metal manufacturing industries in Ethiopia and provided solutions to areas that needed improvement. While the study reviews the relevance of the main strategies, models and tools to measure the performance of supply chain in manufacturing industries is done though the research is conducted based on a qualitative methods. Structured questionnaires, field observation and oral interviews research techniques is use to get primary data from the sectors. Performance measure sets is identified through descriptive analysis. This study found that, large number of companies that now accomplish performance measurement systems at local and global level. Even though, most manufacturing firms use traditional performance measurement systems such as financial performance measurement systems. But this traditional performance measurement systems were not properly evaluates the performance and competitiveness of the whole supply chain systems of manufacturing sectors. Also according to this research proposes that the concept of supply chain performance measurement is not fully hold by the Ethiopian basic metal industries and highlights the difficulties associated with its implementation. Therefore, to tackle the challenges and attempt to the current performance measurement limitations, further studies need to involving other sectors and industries needs to be undertaken in order to gain an in-depth understanding of the key factors associated with the accomplishment of supply chain performance measurement practices in Ethiopia.

Alternating hemiplegia of childhood (AHC) is a rare, severe neurodevelopmental syndrome characterized by recurrent hemiplegic episodes and distinct neurological manifestations. AHC is usually a sporadic disorder and has unknown etiology. We used exome sequencing of seven patients with AHC and their unaffected parents to identify de novo nonsynonymous mutations in ATP1A3 in all seven individuals. In a subsequent sequence analysis of ATP1A3 in 98 other patients with AHC, we found that ATP1A3 mutations were likely to be responsible for at least 74% of the cases; we also identified one inherited mutation in a case of familial AHC. Notably, most AHC cases are caused by one of seven recurrent ATP1A3 mutations, one of which was observed in 36 patients. Unlike ATP1A3 mutations that cause rapid-onset dystonia-parkinsonism, AHC-causing mutations in this gene caused consistent reductions in ATPase activity without affecting the level of protein expression. This work identifies de novo ATP1A3 mutations as the primary cause of AHC and offers insight into disease pathophysiology by expanding the spectrum of phenotypes associated with mutations in ATP1A3.

Alternating hemiplegia of childhood is a neurological disorder characterized by episodes of hemiplegia, various non-epileptic paroxysmal events and global neurological impairment. Characterization of the evolution and outcome into adulthood has not been sufficiently investigated. The goal of this study was to elucidate the natural history of alternating hemiplegia within a large cohort of 157 patients, as part of the European Network for Research on Alternating Hemiplegia project. A questionnaire was formulated to determine the severity of both paroxysmal and global neurological impairment and address progression of the disorder by allocating data to specific age epochs up to and over 24 years of age. Patients in early age groups were consistently present in subsequent later age groups and for each patient, data were collected for each corresponding age epoch. The study was based on predominantly retrospective and, for a period of 2 years, prospective data. At inclusion, patients were aged from 9 months to 52 years. The median age at diagnosis was 20 months. All patients experienced hemiplegic attacks; 86.5% reported episodes of bilateral weakness, 88% dystonic attacks, 53% epileptic seizures, 72% developed chorea and/or dystonia and 92% mental retardation. When data over the course of the illness were examined for the whole cohort, the severity of symptoms did not appear to change, with the exception of abnormal ocular movements and hypotonia that regressed, but did not disappear into adulthood (from 86 to 36% and 76 to 36%, respectively). No statistically significant correlation between a history of severe paroxysmal hemiplegic/dystonic episodes and a worse neurological outcome was identified. Seven patients died, some of whom experienced severe plegic attacks or epileptic seizures at the time of death. History of severe plegic/dystonic attacks was not found to be an aggravating factor for deceased patients. Our results provide evidence that the natural history of alternating hemiplegia is highly variable and unpredictable for individual patients. However, we did not find evidence to support a steadily progressive and degenerative course of the disorder when patients were analysed as a group. For a minority of patients, a risk of sudden death was associated with more severe neurological impairment. The European Network for Research on Alternating Hemiplegia Registry, validated by our study, includes all major neurological signs and symptoms of alternating hemiplegia and may thus be used as a precedent for the progressive inclusion and follow-up of patients as well as a reference for genetic studies and treatment trials.

Background Drug administration in children is an error-prone task for nurses and parents because individual dose adjustment is often necessary, and suitable formulations for children are frequently lacking. Hence, in the absence of measures for their prevention, medication errors are likely to occur. Objective To assess the error prevalence in drug administration by mouth or gastric tube before and after implementing a programme for quality improvement for nurses and parents. Design, setting and participants Prospective, two-period cohort intervention study on a paediatric neurology ward of a university hospital where drug administration procedures of nurses and parents were consecutively monitored during the routine drug administration hours. Main outcomes measure Prevalence of administration errors before and after implementing instructions for appropriate drug administration, and a teaching and training programme supported by information pamphlets. Results Altogether, 1164 predefined administration tasks were assessed, 675 before and 489 after the intervention. Of these, 95.7% (after the intervention: 92.6%) were performed by nurses. Errors addressed by the intervention were reduced from 261/646 tasks (40.4%) to 36/453 (7.9%, p

Objective: The filigree adjustment rotor of the Codman-Medos (Medos-P) valve has a relatively soft silicone and plastic housing. Inadequate pumping manoeuvres and shocks are a risk for breaking out or decentering the rotor. There are only a few reports in the literature about breakage of Medos-P [for full text, please go to the a.m. URL], 60. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit den Benelux-Ländern und Bulgarien