Optimisation of Diesel Engine for Hybrid Military Tracked Vehicles using Matlab-Simulink

The demand in the technology requirements for diesel engines is growing keeping hybrid vehicles in mind. In future the diesel engine no longer drives the wheels directly; as a result the engine can be engaged at a limited number of operating points, thus, offering an opportunity to optimise the fuel efficiency and performance at those operating points. The extent to which this optimisation is possible is limited by practical considerations. Also if the positive and negative power peaks in vehicle during mobility (e.g. acceleration and regenerative braking respectively) can be accommodated by high-power batteries, then the size of the engine can be considerably reduced. The engine’s operating points depend on the power-control strategy. The consequences of modifications to these operating points will have an effect on performance and efficiency. As in series hybrid only a limited number of operating points are involved and dynamic performance requirements are not imposed on the diesel engine, significant improvements can be achieved by the optimisation of the diesel engine at these operating points. The feasibility of optimisation of the engine at these operating points can be done by modification on the injection systems, the valve timings and other such parameters. This kind of approach requires the use of complex and repeated experimental analysis of the engine which is costly, cumbersome and time consuming. An alternative to this kind of experimental approach is to develop a simulation model of the engine with the generator in Matlab- Simulink

Congenital myasthenic syndrome caused by a frameshift insertion mutation in

Objective: Description of a new variant of the glutamine-fructose-6-phosphate transaminase 1 (GFPT1) gene causing congenital myasthenic syndrome (CMS) in 3 children from 2 unrelated families. Methods: Muscle biopsies, EMG, and whole-exome sequencing were performed. Results: All 3 patients presented with congenital hypotonia, muscle weakness, respiratory insufficiency, head lag, areflexia, and gastrointestinal dysfunction. Genetic analysis identified a homozygous frameshift insertion in the GFPT1 gene (NM_001244710.1: c.686dupC; p.Arg230Ter) that was shared by all 3 patients. In one of the patients, inheritance of the variant was through uniparental disomy (UPD) with maternal origin. Repetitive nerve stimulation and single-fiber EMG was consistent with the clinical diagnosis of CMS with a postjunctional defect. Ultrastructural evaluation of the muscle biopsy from one of the patients showed extremely attenuated postsynaptic folds at neuromuscular junctions and extensive autophagic vacuolar pathology. Conclusions: These results expand on the spectrum of known loss-of-function GFPT1 mutations in CMS12 and in one family demonstrate a novel mode of inheritance due to UPD

Lower Extremity Predominant Stiff-Person Syndrome and Limbic Encephalitis With Amphiphysin Antibodies in Breast Cancer

A 54-year-old woman presented with several weeks of psychiatric symptoms, partial-onset seizures, and painful spasms of the lower extremities. On examination, she exhibited severe stiffness and intermittent extensor spasms of the lower extremities. Magnetic resonance imaging of the brain showed T2 hyperintensity in the left temporal lobe with enhancement after gadolinium administration on T1-weighted images. Amphiphysin antibodies were present in the serum. Radiographic screening for malignancy disclosed a metastatic breast cancer. The case is a unique example of amphiphysin autoimmunity, illustrating the possibility of paraneoplastic stiff-person syndrome and limbic encephalitis coexisting in a patient with a \ classical\ presentation of stiff-person syndrome confined to the lower extremities

Charcot-Marie-Tooth disease: extensive cranial nerve involvement on CT and MR imaging

We report a case of genetically verified Charcot-Marie-Tooth disease in which the patient had cranial nerve symptoms. CT and MR imaging demonstrated enlargement of several cranial nerves, as well as their skull-base foramina, with faint contrast material enhancement identified

Single-Amino-Acid Deletion in the RYR1 Gene, Associated with Malignant Hyperthermia Susceptibility and Unusual Contraction Phenotype

Malignant hyperthermia (MH) is an anesthetic-drug–induced, life-threatening hypermetabolic syndrome caused by abnormal calcium regulation in skeletal muscle. Often inherited as an autosomal dominant trait, MH has linkage to 30 different mutations in the RYR1 gene, which encodes a calcium-release–channel protein found in the sarcoplasmic reticulum membrane in skeletal muscle. All published RYR1 mutations exclusively represent single-nucleotide changes. The present report documents, in exon 44 of RYR1 in two unrelated, MH-susceptible families, a 3-bp deletion that results in deletion of a conserved glutamic acid at position 2347. This is the first deletion, in RYR1, found to be associated with MH susceptibility. MH susceptibility was confirmed among some family members by in vitro diagnostic pharmacological contracture testing of biopsied skeletal muscle. Although a single-amino-acid deletion appears to be a subtle change in the protein, the deletion of Glu2347 from RYR1 produces an unusually large electrically evoked contraction tension in MH-positive individuals, suggesting that this deletion produces an alteration in skeletal-muscle calcium regulation, even in the absence of pharmacological agents

Histopathologic Progression and a Novel Mutation in a Child With Nemaline Myopathy

Nemaline myopathy is a clinically heterogeneous congenital myopathy caused by mutations in at least 6 genes related to thin filaments. Histologically, they show a characteristic if not homogeneous picture of nemaline rods, essential for the diagnosis. However, little is known regarding the development and progression of muscle histopathologic changes in nemaline myopathy. Results of muscle biopsies at 7 weeks of age and at 15 months of age from a child with nemaline myopathy due to a novel mutation in the ACTA1 gene are presented. The findings of the biopsies, separated by 13 months, demonstrate progression from vague cytoplasmic bodies in the first biopsy to typical nemaline rods in the second biopsy

Long-term Safety, Tolerability, and Efficacy of Bimagrumab (BYM338) in Sporadic Inclusion Body Myositis: Results of an Open-label Extension Study

Objective: To assess the long-term safety, tolerability and efficacy of multiple doses of bimagrumab in participants with sporadic inclusion body myositis (sIBM) who completed previous core study. Methods: In this multicenter, open-label extension study, 10 adults received bimagrumab 10 mg/kg IV every 4 weeks up to 2 years. Safety (primary endpoint) was assessed by recording adverse events (AEs). Efficacy was assessed by change from baseline in thigh muscle volume (TMV), lean body mass (LBM), 6-minute walk distance (6MWD), hand grip and quadriceps strength. Results: Participants had a mean (standard deviation [SD]) age of 70.1 (10.4) years. All participants (n=10) discontinued the treatment due to early termination of the study (n=7) or AEs (n=3; myocardial infarction, esophageal carcinoma, and dementia, none of which were treatment related). The most common AEs were muscle spasms and falls (both 9/10, 90%), followed by diarrhea (6/10, 60%), acne and skin abrasion (both 5/10, 50%). At Weeks 8 and 16, mean TMV increased from baseline by 4.1% (4.3) and 4.5% (6.3). Mean LBM increased from baseline and was sustained at 6.9% (3.9) at Week 76. Means of 6MWD showed a progressive decline from baseline to Week 104 accompanied by modest numerical increase in hand grip strength and no significant changes in quadriceps strength. Conclusion: Treatment of up to 2 years with bimagrumab had a good safety profile and was well-tolerated in participants with sIBM. Despite an increase in muscle mass and maintenance of strength measures noted on a group level, heterogeneity of treatment responses prevent us from drawing firm conclusion about impact on mobility