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Mammalian lipid droplets (LDs), first described as early as the 1880s, were virtually ignored for more than 100 years. Between 1991 and the early 2000s, however, a series of discoveries and conceptual breakthroughs led to a resurgent interest in obesity as a disease, in the metabolism of intracellular triacylglycerol (TAG), and in the physical locations of LDs as cellular structures with their associated proteins. Insights included the recognition that obesity underlies major chronic diseases, that appetite is hormonally controlled, that hepatic steatosis is not a benign finding, and that diabetes might fundamentally be a disorder of lipid metabolism. In this brief review, I describe the metamorphosis of LDs from overlooked globs of stored fat to dynamic organelles that control insulin resistance, mitochondrial oxidation, and viral replication., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier B.V. All rights reserved.)

This article focuses on the contributions made by Michel Jouvet about the neurons responsible for generating the muscle atonia of paradoxical sleep (REM sleep). He was the first to describe the neurons responsible for muscle atonia during paradoxical sleep using "pontine" cats (in which the forebrain has been removed down to the pons) and localized pontine lesions. Also discussed is the research going on in the 1980s, when Michel Jouvet was hunting for the hypnogenic factor. At that time, he thought that it was secreted by the hypophysis; but this factor finally turned out to be controlled by the hypocretin/orexin and melanin concentrating hormone neurones located in the lateral hypothalamus. Several unforgettable moments with Michel Jouvet are described which occurred between 1983 and his last moments with us., (© Société de Biologie, 2019.)

The wide spectrum of hereditary muscular disorders leads to unavoidable difficulties in their classification, even for specialists. For this reason, new proposals are required that would ultimately replace our current rather complex classifications by a simpler structure. Our proposal will be limited to dystrophic and non-dystrophic myopathies (excluding metabolic disorders, mitochondriopathies, and channelopathies) for which similar proposals would also be relevant. Various genes (encoding structural proteins associated with the sarcolemma, nuclear membrane proteins, and proteins involved in myofiber metabolism have now been sequenced and mutations ascribed to specific forms of inherited muscular disorders. Based on our observations and our recent proposals in other neurogenetic conditions and informal discussions with specialists of neuromuscular disorders, the prerequisite for a simple and sound classification for inherited muscular disorders should encompass the clinical and pathological phenotypes (described in a simple and clear manner), the mode of inheritance, and the mutated gene. We think that the denomination of the different subtypes could be simplified considerably, although any new proposal of classification of muscular disorders will need to be discussed in the neurological and genetic communities., (Copyright © 2017 Elsevier B.V. All rights reserved.)

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.)

The portrait of Ginevra de Benci was painted by Leonardo da Vinci around 1474. This painting is less famous and less known than Mona Lisa but it is extremely captivating and interesting due to its own characteristics. The overall picture gives to the viewer the impression of being in front of an enigmatic, hermetic and introspective woman. However, some details of her facial expression, especially the gaze direction, the upper eyelids' heaviness, the flat mouth and the absence of any defined emotion, give her the appearance of a patient with a muscular disease.

Objective: To examine the neurologic cinematographic contributions of Gheorghe Marinescu., Background: Near the end of the 19th century, cinematography developed and was immediately recognized as a new technique applicable to medical documentation. After studying with several prominent European neurologists and deeply influenced by Jean-Martin Charcot, Marinescu returned to Bucharest in 1897 and applied moving picture techniques to the study of neurologic patients., Methods: The Romanian State Archives were researched for original Marinescu films, and related publications were translated from Romanian and French., Results: Between 1899 and 1902, Marinescu perfected the use of cinematography as a research method in neurosciences and published five articles based on cinematographic documents. He focused his studies particularly on organic gait disorders, locomotor ataxia, and hysteria. He adapted Charcot's method of lining up several patients with the same disorder and showing them together to permit appreciation of archetypes and formes frustes. He decomposed the moving pictures into sequential tracings for publication. He documented treatment results with cases filmed before and after therapy. Processed and digitized excerpts of these films accompany this manuscript., Conclusions: Marinescu's cinematographic studies led to several original contributions in clinical neurology. Remaining film archives include examples of many neurologic diseases, his examination techniques, and the working medical environment of the young founder of the Romanian school of neurology.

Fou rire prodromique (prodrome of crazy laughter) is a rarely described nosological entity. In 1903, Charles Féré, a French neurologist, introduced the term fou rire prodromique to describe pathological laughter heralding an apoplectic event. He was also among the first to describe gelastic epilepsy. His description was influenced by Edouard Brissaud, who supported the existence of a thalamic center for laughter regulation and suggested that spasmodic laughter and crying were due to lesions of the faisceau psychique (anterior internal capsule) or to irritation of the faisceau géniculé (corticobulbar tract). One hundred Years later, we review the evolution of the theories about pathological laughter and crying from Charles Bell in the early XIXth Century, up to the seminal works of Kinnier Wilson and James Papez and the era of modern neuroscience.

At the end of the XIXth century, the first cases of primary progressive ophthalmoplegia reported were related to a primary degeneration of the oculomotor nuclei; and this concept was accepted until there were considered of purely muscular origin in the 1950's. From this time and during twenty years these cases were reported as ocular myopathies. However the coexistence of neurological abnormalities and above all, the presence of mitochondrial abnormalities in the muscles, the brain and the tissues rich in energy, gave ground to the concept of mitochondrial encephalomyopathies. Then the nature of the dysfunctioning was assessed through biochemical studies, mainly the enzyme deficits of the respiratory chain mitochondria. But correlations between clinical and biochemical data were still difficult as these cases corresponded either to muscular damage (myalgia, exercise intolerance), either to various neurological alterations (epilepsy, cerebellar syndrome) or to multisystemic manifestations. Molecular biology technics introduced the notion of mitochondrial inheritance which contributed to underline the identity of certain varieties, mainly the Kearns and Sayre Syndrome. The increasing number of cases with mitochondrial abnormalities leads to the inverse approach: in central or peripheral damage of the nervous system or in very variable muscular alterations, the investigation of a mitochondrial dysfunctioning is justified and shows the way to possible therapies. This evolution of concept is remarkable in the history of medicine. An abnormality which, at first, was considered as an esthetic alteration, became the subject of the study which, through the advances of the clinical data, of the pathological anatomy, biochemistry and genetics, explained the mechanism of several alterations of the muscles and of the nervous system.

Enthesopathies are bony lesions involving the sites of insertion of muscles or ligaments. Those caused by hyperactivity of the relevant muscles may be distinguished clearly from those of metabolic or inflammatory origin. Observations from sporting and occupational medicine indicate that specific enthesopathies are correlated with different activities. Examination of the enthesopathies present on two groups of well-preserved neolithic skeletons from separate regions of the Sahara with different paleoenvironments show that overall 20% of the skeletons presented lesions. Three different forms of enthesopathy involved the arm, principally the elbow, and may be tentatively correlated with javelin throwing, wood cutting, and archery. Two types of lesion involving the foot were observed in skeletons from a hunter-gatherer population and may be correlated with much walking or running over hard ground. I suggest that the analysis of such lesions on ancient skeletons may, in concert with other archaeological data, throw light on the activities of ancient people.