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The metabolic roles of carnitine have been greatly clarified over the past 50 years, and it is now well established that carnitine is a key player in mitochondrial generation of energy and metabolism of acetyl coenzyme A. A therapeutic role for carnitine in treatment of nutritional deficiencies in infants and children was first demonstrated in 1958, and since that time it has been used to treat a number of inborn errors of metabolism. Carnitine was approved by the US Food and Drug Administration in 1985 for treatment of 'primary carnitine deficiency', and later in 1992 for treatment of 'secondary carnitine deficiency', a definition that included the majority of relevant metabolic disorders associated with low or abnormal plasma carnitine levels. Today, carnitine treatment of inborn errors of metabolism is a safe and integral part of many treatment protocols, and a growing interest in carnitine has resulted in greater recognition of many causes of carnitine depletion. Notwithstanding, there is still a lack of data from randomized clinical trials, even on the use of carnitine in inborn errors of metabolism, although ethical issues may be a contributing factor in this regard., (© 2016 S. Karger AG, Basel.)

Background: Between 1880 and 1883, the famous German neurologist Wilhelm Erb was appointed Professor of Special Pathology at Leipzig University and Head of the Medical Outpatient Department., Summary: Besides the favourable clinical conditions, it was first and foremost the access to large numbers of patients that enabled him to both establish a new, juvenile form of progressive muscular atrophy and to classify various forms of muscular atrophies already discovered into a new clinical entity which he called dystrophia muscularis progressiva., Key Messages: His summarising these different forms of muscular atrophies into one group is the most long-lasting outcome of Erb's years in Leipzig. The access to large numbers of different patients at the Medical Outpatient Department, on the one hand, and the dynamics of Leipzig's neurosciences, in particular the so-called 'Leipzig Nervous Circle', on the other, had a lasting impact on Erb and definitely gave him other valuable insights, such as the thought that tabes dorsalis must have been caused by a syphilitic infection., (Copyright © 2013 S. Karger AG, Basel.)