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Thimm E Richter-Werkle R Kamp G Molke B Herebian D Klee D Mayatepek E Spiekerkoetter U 《Journal of inherited metabolic disease》2012,35(2):263-268
Objective
The implementation of NTBC into treatment of hypertyrosinemia type I (HT I) greatly improved survival by prevention of acute liver failure and hepatocellular carcinoma. However, there are first reports of cognitive impairment in patients with elevated plasma tyrosine concentrations. 相似文献85.
Hoffmann L Haussmann U Mueller M Spiekerkoetter U 《Journal of inherited metabolic disease》2012,35(2):269-277
Tandem mass spectrometry-based newborn screening correctly identifies individuals with very long-chain acyl-CoA dehydrogenase
deficiency (VLCADD). However, a great number of healthy individuals present with identical acylcarnitine profiles during catabolism
in the first three days of life. We routinely perform an enzyme activity assay as confirmation analysis in newborns identified
by screening. Whereas VLCAD residual activities of less than 10% are clearly diagnostic and indicate patients at risk of clinical
disease, the clinical relevance of higher residual activities is unclear. In this study we assess the molecular basis in 34
individuals with residual activities of 10-50%. We identify two pathogenic mutations in patients that result in residual activities
as high as 22%, while individuals with residual activities of 25-50% either present with a heterozygous or no mutation in
the VLCAD gene. In addition, confirmed heterozygous parents present with residual activities as low as 32%. 相似文献
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Sebastian J. Buss Mostafa Emami Derliz Mereles Grigorios Korosoglou Arnt V. Kristen Andreas Voss Dieter Schellberg Christian Zugck Christian Galuschky Evangelos Giannitsis Ute Hegenbart Anthony D. Ho Hugo A. Katus Stefan O. Schonland Stefan E. Hardt 《Journal of the American College of Cardiology》2012
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Ute Strehl Boris Kotchoubey Simone Martinetz Niels Birbaumer 《Journal of neurotherapy》2013,17(3):200-213
In patients with epilepsy, not only seizures but also cognitive, emotional, and social functioning are of increasing interest in research (Kelley, Jacobs, &; Lowenstein, 2009). As a decrease in cognitive functions over the course of the illness is usually reported, we wanted to explore changes in Intelligence Scores observed after a neurofeedback treatment in patients with drug-resistant epilepsies. In a controlled study that compared the outcome of three different interventions (training to regulate slow cortical potentials, N = 34; training to regulate breath rate and the amount of carbon dioxide in the end tidal volume of the exhaled air, N = 11; modification of drug regime, N = 25), pre- and postmeasurements of a short version of the Wechsler Intelligence Scale were applied. The interval between the two assessments was more than 12 months, with a mean of 61 weeks. Mean age of the patients was 35, with a range from 17 to 57. The highly significant 7-point increment of IQ only after training of slow cortical potentials was not related to clinical (e.g., seizure reduction) or neuropsychological (e.g., attention and memory) variables. Instead, it was related to psychophysiological measures: IQ change was inversely related to the Latency of the P300 component of event-related brain potentials and directly related to the Latency of the P2 component and the increase of N2 Amplitude during training. We conclude that regulation training of slow cortical potentials improves IQ in patients with refractory partial epilepsy, which might be related to an improved ability for controlled allocation of cognitive resources. 相似文献
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Tobias Geis Klaus Marquard Tanja Rödl Christof Reihle Sophie Schirmer Thekla von Kalle Antje Bornemann Ute Hehr Markus Blankenburg 《Neurogenetics》2013,14(3-4):205-213
Defects in dystroglycan post-translational modification result in congenital muscular dystrophy with or without additional eye and brain involvement, are referred to as secondary dystroglycanopathies and have been associated with mutations in 11 different genes encoding glycosyltransferases or associated proteins. However, only one patient with a mutation in the dystroglycan encoding gene DAG1 itself has been described before. We here report a homozygous novel DAG1 missense mutation c.2006G>T predicted to result in the amino acid substitution p.Cys669Phe in the β-subunit of dystroglycan in two Libyan siblings. The affected girls presented with a severe muscle–eye–brain disease-like phenotype with distinct additional findings of macrocephaly and extended bilateral multicystic white matter disease, overlapping with the cerebral findings in patients with megalencephalic leucoencephalopathy with subcortical cysts. This novel clinical phenotype observed in our patients further expands the clinical spectrum of dystroglycanopathies and suggests a role of DAG1 not only for dystroglycanopathies but also for some forms of more extensive and multicystic leucodystrophy. 相似文献