Carotid angioplasty stenting revisited: clinical and radiological (MRI) outcome.

International audienceBACKGROUND: Although carotid angioplasty and stenting (CAS) is widely used to treat carotid stenosis, recent studies point to the inferiority of the procedure compared with carotid endarterectomy. METHODS: We present 50 consecutive cases of CAS treated in our unit. Endarterectomy was contraindicated in these patients due to high operative risk. All the patients underwent a diffusion-weighted MRI (DWI) before and after the procedure and had a neurological assessment in a stroke unit. RESULTS: No deaths were recorded until 30 days after the procedure. Six patients [12%, confidence interval at 95% (CI(95)) = 3.7-20.2] had a positive DWI MRI after the procedure but only 2 (4%, CI(95) = 0-9.43) had a worse neurological status. CONCLUSION: This study shows that CAS is feasible with a low morbid-mortality rate in patients with a high surgical risk. DWI is highly sensitive to detect neurological complications after the procedure

Heterogeneity of Polyneuropathy Associated with Anti-MAG Antibodies

Polyneuropathy associated with IgM monoclonal gammopathy and anti-myelin associated glycoprotein (MAG) antibodies is an immune-mediated demyelinating neuropathy. The pathophysiology of this condition is likely to involve anti-MAG antibody deposition on myelin sheaths of the peripheral nerves and it is supposed to be distinct from chronic inflammatory demyelinating neuropathy (CIDP), another immune-mediated demyelinating peripheral neuropathy. In this series, we have retrospectively reviewed clinical and laboratory findings from 60 patients with polyneuropathy, IgM gammopathy, and anti-MAG antibodies. We found that the clinical picture in these patients is highly variable suggesting a direct link between the monoclonal gammopathy and the neuropathy. Conversely, one-third of patients had a CIDP-like phenotype on electrodiagnostic testing and this was correlated with a low titer of anti-MAG antibodies and the absence of widening of myelin lamellae. Our data suggest that polyneuropathy associated with anti-MAG antibodies is less homogeneous than previously said and that the pathophysiology of the condition is likely to be heterogeneous as well with the self-antigen being MAG in most of the patients but possibly being another component of myelin in the others

Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome

Immune-mediated neuromuscular disorders include pathologies of the peripheral nervous system, neuromuscular junction, and muscles. If overlap syndromes (or the association of almost two autoimmune disorders) are recognized, the simultaneous occurrence of several autoimmune neuromuscular disorders is rare. We describe two patients presenting the simultaneous occurrence of inflammatory neuropathy, myositis, and myasthenia gravis (with positive acetylcholine receptor antibodies). For each patient, we carried out a pathological analysis (nerve and muscle) and an electrophysiological study (and follow-up). To our knowledge, this is the first description of such a triple immune-mediated neuromuscular syndrome. We compared our observations with a few other cases of simultaneous diagnosis of two inflammatory neuromuscular disorders

Intranervous immunoglobulin deposits: an underestimated mechanism of neuropathy.

International audienceThere are several pathogenic mechanisms of peripheral nerve involvement in patients with monoclonal dysglobulinemia. Intranervous proliferation of malignant cells, immunoglobulin, or amyloid deposits in the endoneurial space can only be determined by examination of nerve biopsy specimens. We present clinical, electrophysiological, and histological data from seven patients whose polyneuropathy was induced by immunoglobulin deposits in the endoneurial space. As these lesions cannot be demonstrated on clinical and electrophysiological grounds, the indication for nerve biopsy derives from careful analysis of each patient presenting with a polyneuropathy and a monoclonal dysglobulinemia. To visualize and clearly characterize these deposits, electron microscopic examination is indispensable. Immunocytochemical methods using both light and electron microscopy for ultrastructural analysis are of great value. Demonstration of endoneurial immunoglobulin deposits may have major therapeutic consequences. Indeed, identification of these deposits prompted the use of aggressive treatment, which was quite effective in five of our seven patients

A novel pathogenic variant of NEFL responsible for deafness associated with peripheral neuropathy discovered through next‐generation sequencing and review of the literature

International audienceNeurofilaments are neuron-specific intermediate filaments essential for the radial growth of axons during development and the maintenance of axonal diameter. Pathogenic variants of Neurofilament Light (NEFL) are associated with CMT1F, CMT2E, and CMTDIG and have been observed in less than 1% of Charcot-Marie-Tooth (CMT) cases, resulting in the reporting of 35 variants in 173 CMT patients to date. However, only six variants have been reported in 17 patients with impaired hearing. No genotype-phenotype correlations have yet been established. Here, we report an additional case: a 69-year-old female, who originally presented with axonal sensory and motor neuropathy at the age of 45, associated with moderate sensorineural hearing loss, with a slight slope at high frequencies. Next-generation sequencing identified a novel pathogenic variant: c.269A > G, p.(Glu90Gly). Hearing impairment is often linked to CMT due to pathogenic variants of NEFL, especially p.(Glu90Lys) and p.(Asn98Ser), and in our case p.(Glu90Gly). These pathogenic variants are all located at hot spots, in the head domain and the two ends of the rod domain of the protein

Sustainable recycling of poultry eggshell waste for the synthesis of calcium oxide nanoparticles and evaluating its antibacterial potency against food-borne pathogens

Nanoparticles are considered new antibacterial agents with a potential broad range of applications. Recently, the synthesis of bio-nanoparticles (NPs) from natural sources such as coral, Ostrea shell, and eggshell, has attracted considerable attention. Eggshells are organic waste, rich in calcium carbonate (CaCO3), and it is an easy method to reduce it into powder of nano size. Utilization of waste materials as a precursor for NPs synthesis makes the entire process cheaper, greener, and more sustainable. Waste chicken eggshells were collected from the Specific Pathogen Free farm in Egypt. Eggshells were rinsed, dried, and finely ground to powder. The sol-gel chemical method was used for the synthesis of CaO-NPs from eggshell powder. The characteristics of eggshell NPs were visualized using a scanning electron microscope, transmission electron microscope, Fourier transform infrared spectroscopy, and ultraviolet–visible spectroscopy. Additionally, the minimum inhibitory concentration was applied to test the antibacterial efficacy of CaO-NPs at 1.00, 0.50, 0.25, 0.12, and 0.06% concentrations on Methicillin-resistant Staphylococcus aureus (MRSA), Bacillus cereus, Escherichia coli, and Salmonella enteritidis. The results of the characterization confirmed the conversion of CaCO3 to CaO-NPs with an average diameter of 27.7 nm. Zones of inhibition started to appear from 0.25% concentration for B. cereus, 0.50% for MRSA and E. coli, and 1.0% for S. enteritidis. The concentration of CaO-NPs solution strongly correlated with the resulting zone of inhibition (r = 0.86 to 0.90). CaO-NPs showed a potent efficacy against gram-positive bacteria. Hence, eggshell wastes from poultry production could be a feasible organic source for the biosynthesis of CaO NPs with promising efficient antibacterial properties

GM2 gangliosidosis AB variant: first case of late onset and review of the literature

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Some new proposals for the classification of inherited myopathies

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History and current difficulties in classifying inherited myopathies and muscular dystrophies

International audienceThe 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