Factors associated with survival after early at-home NIV initiation in ALS patients.

BACKGROUND: The initiation of early non-invasive ventilation (NIV) often involves a careful balance between tolerance and effectiveness. In amyotrophic lateral sclerosis (ALS) patients, the establishment of a strategy, including the decision to focus on adhering to a cut-off, setting specific targets, or correcting all events, is crucial. OBJECTIVE: To identify factors at 1 month after early at-home NIV initiation that are associated with improved survival in ALS patients. We explored the impacts of adherence (ADH), quality of treatment, and NIV parameters at 1 month after early at-home NIV initiation on patient survival. METHODS: We conducted a retrospective study of 184 ALS patients at the Bordeaux ALS Centre for whom NIV was initiated between September 2017 and June 2021, and we collected data for a minimum period of 2 years after the last patient included. The primary outcome was the risk of death according to baseline characteristics of our population and the NIV parameters and monitoring during the early NIV initiation period. The secondary outcomes were association with NIV ADH during the early NIV initiation period on prognosis, and NIV ADH cut-off for good versus poor prognosis. RESULTS: Among the 178 ALS patients analysed, we found that quality of NIV treatment and device settings did not significantly influence prognosis. However, low ADH was significantly associated with a higher risk of death. The use of NIV for > 5 h/day during the early NIV initiation period was linked to a decreased risk of death [hazard ratio = 0.4; 95% confidence interval: 0.27-0.9]. CONCLUSION: The use of NIV for > 5 h/day during the early NIV initiation period was associated with increased survival.

Dropped head syndrome in severe hypotension.

INTRODUCTION: Dropped head syndrome (DHS) is characterized by a chin-on-chest deformity, correctable by passive neck extension. METHODS: Case report. RESULTS: A patient with a heavy nephrological history (dialyzed since 5 years) complained for twenty months about a falling head. The symptomatology was punctuated by dialysis sessions, with aggravation secondary to dialysis. Clinical and paraclinical exploration for neurological, neuromuscular or orthopedic disease was negative. Analysis of the post-dialysis blood pressure showed a slow and gradual decline. From the date the patient became symptomatic, blood pressure was below 80/40mmHg. The correction of blood pressure by increasing midodrine posology resulted in a cure of DHS. DISCUSSION: Considering the negativity of explorations, the cure of symptoms following the correction of arterial hypotension, the rhythmic nature of symptomatology by dialysis, and the recurrence of symptoms concomitantly with drops in blood pressure, we suggested that hypotension was the only etiology explaining this DHS.

Anti-SARS-CoV-2 (COVID-19) vaccination efficacy in patients with severe neuromuscular diseases.

INTRODUCTION: Patients with severe neuromuscular disease (sNMD) are considered at high risk of severe COVID-19. Muscle tissue is often replaced by fibroadipose tissue in these diseases whereas the new mRNA-based vaccines are injected intramuscularly. We aimed at evaluating the efficacy of two injections associated with a booster injection of mRNA vaccine in these patients. METHODS: We performed an observational, prospective, single-centre study to investigate the level of anti-S antibodies (Abs) and their neutralization activity at weeks 6 (W6) and 24 (W24) after two injections of mRNA-1273 vaccine and at weeks 12 (BW12) and 29 (BW29) after a booster injection of BNT162b2 vaccine in patients with sNMD. RESULTS: Thirty-three patients with sNMD were included. At W6, 30 patients (90.1%) showed a protective serum level of specific anti-S Abs with a strong neutralization capacity. We observed a decline over time: only 12 patients (36.3%) retained anti-S Abs levels considered as protective at W24. The neutralization activity remained above the cut off in 23 (69.7%). The booster vaccination restored robust neutralization activity for all analysed 22 patients (100%) at BW12, which was maintained without any significant drop at BW29 (16). No severe adverse event was reported in this cohort and none of the 33 patients developed symptomatic COVID-19 over one year. CONCLUSIONS: This study provides evidence that most sNMD patients receiving two injections of COVID-19 mRNA-based vaccines develop a strong humoral response after vaccination. A decline over time was observed but a single booster injection restores a long-term immunity. Moreover, no safety issues were observed.

Circulation of Extended-Spectrum Beta-Lactamase-Producing Escherichia coli of Pandemic Sequence Types 131, 648, and 410 Among Hospitalized Patients, Caregivers, and the Community in Rwanda.

Multi-drug resistant (MDR), gram-negative Enterobacteriaceae, such as Escherichia coli (E. coli) limit therapeutic options and increase morbidity, mortality, and treatment costs worldwide. They pose a serious burden on healthcare systems, especially in developing countries like Rwanda. Several studies have shown the effects caused by the global spread of extended-spectrum beta-lactamase (ESBL)-producing E. coli. However, limited data is available on transmission dynamics of these pathogens and the mobile elements they carry in the context of clinical and community locations in Sub-Saharan Africa. Here, we examined 120 ESBL-producing E. coli strains from patients hospitalized in the University Teaching Hospital of Butare (Rwanda), their attending caregivers as well as associated community members and livestock. Based on whole-genome analysis, the genetic diversification and phylogenetics were assessed. Moreover, the content of carried plasmids was characterized and investigated for putative transmission among strains, and for their potential role as drivers for the spread of antibiotic resistance. We show that among the 30 different sequence types (ST) detected were the pandemic clonal lineages ST131, ST648 and ST410, which combine high-level antimicrobial resistance with virulence. In addition to the frequently found resistance genes bla CTX-M-15 , tet(34), and aph(6)-Id, we identified csg genes, which are required for curli fiber synthesis and thus biofilm formation. Numerous strains harbored multiple virulence-associated genes (VAGs) including pap (P fimbriae adhesion cluster), fim (type I fimbriae) and chu (Chu heme uptake system). Furthermore, we found phylogenetic relationships among strains from patients and their caregivers or related community members and animals, which indicates transmission of pathogens. Also, we demonstrated the presence and potential transfer of identical/similar ESBL-plasmids in different strains from the Rwandan setting and when compared to an external plasmid. This study highlights the circulation of clinically relevant, pathogenic ESBL-producing E. coli among patients, caregivers and the community in Rwanda. Combining antimicrobial resistance with virulence in addition to the putative exchange of mobile genetic elements among bacterial pathogens poses a significant risk around the world.

Flies from a tertiary hospital in Rwanda carry multidrug-resistant Gram-negative pathogens including extended-spectrum beta-lactamase-producing E. coli sequence type 131.

Multidrug-resistant gram-negative (MRGN) bacteria are a serious threat to global health. We used genomics to study MRGN obtained from houseflies in a tertiary Rwandan hospital. Our analysis revealed a high abundance of different MRGN including E. coli pathogenic lineage ST131 suggesting the important role of flies in disseminating highly virulent pathogens in clinical settings and beyond.

Antibody- and macrophage-mediated segmental demyelination in chronic inflammatory demyelinating polyneuropathy: clinical, electrophysiological, immunological and pathological correlates.

BACKGROUND AND PURPOSE: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogeneous autoimmune disorder critically lacking diagnostic biomarkers. Autoantibodies to nodal and paranodal components have recently been described in a small subset of patients. Here, the diagnostic value of immune reactivity toward the myelin compartment was investigated. METHODS: Ninety-four French CIDP patients were retrospectively studied. The reactivity toward the peripheral nerve was investigated. Sural nerve biopsies were examined by electron microscopy and immunofluorescence. RESULTS: Twenty-one patients (22%) and three patients (3%) presented with a strong immunoglobulin G or immunoglobulin M reactivity respectively against the myelin compartment. The clinical, electrophysiological and morphological features were examined in nine of these patients for whom sural nerve biopsies were available. Seven patients were electrodiagnosed with definite CIDP, one with possible CIDP and one was unclassifiable but sural nerve biopsy argued for CIDP diagnosis. Electron microscopy of sural nerve biopsies demonstrated the presence of macrophage-mediated demyelination restricted to the internode in all nine patients. Immunolabelling for voltage-gated sodium channels, myelin and axonal markers confirmed the presence of segmental demyelination and of remyelination. The nodal and paranodal regions, however, were unaffected in these patients. Nerve conduction studies corroborated the multifocal and segmental profile, and seven patients showed increased duration of proximal (1.5-5.1 times) and/or distal (1.2-3.4 times) compound muscle action potential in at least two nerves. CONCLUSION: Antibody- and macrophage-mediated demyelination appears responsible for conduction alterations in CIDP patients and nerve immunostaining assays may serve as a supportive diagnostic biomarker.

Pre-operative evaluation prior to soft tissue sarcoma excision - Why can't we get it right?

BACKGROUND: To define the association between an appropriate pre-operative workup (pre-operative advanced imaging studies, diagnostic biopsy) and incomplete soft tissue sarcoma (STS) excision. PATIENTS AND METHODS: This was a retrospective review of 397 consecutive patient records (2000-2008), looking at primary site advanced imaging (MRI or CT) and diagnostic biopsy procedures completed prior to the initial attempt at definitive surgical excision. Downstream effects of an inadequate pre-operative workup were also evaluated, including time to referral to a sarcoma multi-disciplinary care team and perceived alteration of surgical care in order to obtain a complete excision of the altered sarcoma bed. RESULTS: Thirty-eight percent (149/397) of soft tissue sarcomas identified underwent an incomplete excision prior to referral. A significant difference in the incidence of pre-operative primary site advanced imaging (91% vs. 42%, p < 0.001) and a pre-operative diagnostic biopsy (85% vs. 16%, p < 0.001) was found between the wide excision group and incomplete excision groups. Pre-operative biopsy (p < 0.001), tumor size >5 cm (p < 0.001), and a referral from an orthopaedic surgeon (p < 0.02) were all associated with reduced risk of incomplete excision in multivariate analysis. Seventy-four percent of patients in the incomplete excision group required an alteration in their definitive wide margin surgical resection, including rotational muscle flap coverage (37%), free flap coverage (11%), or amputation (11%). CONCLUSION: A minority of patients referred following incomplete excision of a STS had undergone an appropriate pre-operative workup prior to referral, leading to increased long-term morbidity following definitive re-excision. Education efforts to heighten awareness of suspicious soft tissue lesions remain vital.

Short-read whole genome sequencing for determination of antimicrobial resistance mechanisms and capsular serotypes of current invasive Streptococcus agalactiae recovered in the USA.

OBJECTIVES: Our objective was to evaluate and exploit a whole genome sequence (WGS) bioinformatics pipeline for predicting antimicrobial resistance and capsular serotypes from invasive group B streptococci (iGBS). METHODS: For 1975 iGBS recovered during 2015 from CDC's Active Bacterial Core surveillance, we compared pipeline predictions with broth dilution testing. Fifty-six isolates from earlier surveillance were included for testing ß-lactams. Conventional serotyping was compared to WGS-based assignments for 302 isolates. RESULTS: All 28 isolates with reduced susceptibility to ß-lactam antibiotics harboured one of 19 rare PBP2x types. Resistances to erythromycin/clindamycin (808/1975 isolates, 41.0%), erythromycin (235/1975, 11.9%) and lincosamide/streptogramin A/pleuromutilins (56/1975, 2.8%) were predicted by the presence of erm-methylase, mef and lsa determinants, respectively (41 of 56 lsa gene-positive isolates also contained lnu, erm and/or mef genes). Presence of both erm and lsa determinants (25 isolates) predicted non-susceptibility to quinupristin/dalfopristin. Most isolates (1680/1975, 85.1%) were tet gene-positive, although 41/1565 (2.6%) tetM-positive isolates were tetracycline-susceptible. All 53 fluoroquinolone-resistant isolates contained ParC and/or GyrA substitutions. Resistances to rifampin (eight isolates), trimethoprim, chloramphenicol and vancomycin (two isolates each) were predicted by the pipeline. Resistance to macrolides/lincosamides without pipeline prediction was rare and correlated to divergent resistance genes or rRNA A2062G substitution. A selection of 267 isolates assigned WGS-based serotypes were also conventionally serotyped. Of these, 246 (92.1%) were in agreement, with the remaining 21 (7.8%) conventionally non-serotypeable. For 32 of 1975 isolates (1.6%), WGS-based serotypes could not be assigned. CONCLUSION: The WGS-based assignment of iGBS resistance features and serotypes is an accurate substitute for phenotypic testing.

Intense pre-admission carriage and further acquisition of ESBL-producing Enterobacteriaceae among patients and their caregivers in a tertiary hospital in Rwanda.

OBJECTIVES: To assess the presence and risk factors of intestinal carriage of extended-spectrum beta-lactamase-producing Enterobacteriaceae (ESBL-PE) among patients admitted to the University Teaching Hospital of Butare and among their attending caregivers, and to analyse the acquisition of ESBL-PE carriage during hospital stay and associated factors. METHODS: We screened 392 patients and their attending caregivers at admission and discharge for ESBL-PE carriage. Bacterial species were determined using the API-20E system, and antimicrobial susceptibility testing was performed by agar disc diffusion. Data on socio-economic status, diet, behaviour, household assets, livestock and hospital procedures were collected. RESULTS: At admission, 50% of the patients showed intestinal ESBL-PE carriage (Escherichia coli, 51%; Klebsiella pneumoniae, 39%; Enterobacter cloacae, 19%) as did 37% of their caregivers. Co-resistance was common but no carbapenem resistance was detected. At discharge, the proportion of ESBL-PE-colonised patients increased to 65% (caregivers, 47%) with almost complete carriage in paediatric patients (93%). The acquisition rate among initially non-colonised patients was 55% (or, 71/1000 patient days). Independent predictors of admission carriage included a colonised caregiver, prior antibiotic intake, egg consumption and neglecting to boil drinking water, whereas being a paediatric patient, undergoing surgery and male gender predicted acquisition during hospitalisation. CONCLUSIONS: Abundant admission carriage of ESBL-PE and a high acquisition rate in a Rwandan university hospital point to potential intrahospital transmission and community dissemination. Caregivers are an additional source of possible spread. Risk factors of colonisation such as diet and water source need to be tackled to prevent the further emergence and spread of ESBL-PE.

[Anesthesia for medical students : A brief guide to practical anesthesia in adults with a web-based video illustration]. / Anästhesie für Medizinstudierende : Kurzanleitung zur praktischen Anästhesie bei Erwachsenen mit webbasierter Videoillustrierung.

In Germany, Austria and Switzerland, anesthesiologists are the second largest group of physicians in hospitals, but this does not correspond to the amount of anesthesiology teaching that medical students receive in medical schools. Accordingly, the chances of medical students recognizing anesthesiology as a promising personal professional career are smaller than in other disciplines with large teaching components. Subsequent difficulties to recruit anesthesiology residents are likely, although many reasons support anesthesiology as a professional career.Traditional strategies to teach medical students in anesthesiology in medical school consist of airway management or cardiopulmonary resuscitation attempts in manikins. Anesthesiology is a complex interaction consisting of anatomy, physiology, pharmacology, clinical evaluation, experience, knowledge, and manual skills. While some medical schools offer teaching in high fidelity simulators, clinical teaching in the operating room is often limited. When medical students opt for a clinical rotation in anesthesiology, there is a chance to demonstrate the fascinating world of anesthesiology, but this chance has to be utilized carefully by anesthesiologists, as young talents have to be discovered, supported, and challenged.We have put together a short guide for medical students for a clinical rotation in anesthesiology in adults in order to generate basic knowledge and interest in anesthesiology as well as a sense of achievement. Basic knowledge about premedication, induction, maintenance and strategies for anesthesia is discussed. Further, the most important anesthesia drugs are discussed and manual skills, such as intravenous cannulation, mask ventilation, intubation, and regional anesthesia are featured with QR-code based video illustrations on a smartphone or personal computer. We did not discuss possible local mannerism and special patient groups (e. g., children, special medical history), local guidelines, or standard operating procedures.Medical students can be inspired by anesthesiology when not simply told about the tools of the trade and strategies, but instead by gaining knowledge and clinical skills that render a sense of achievement, likely during a clinical rotation into anesthesiology. A short theoretical instruction of anesthesiology enables rapid and targeted orientation even before a clinical rotation. Whether this subsequently results in a higher recruiting chance is currently unknown.

Cardiac arrhythmia and late-onset muscle weakness caused by a myofibrillar myopathy with unusual histopathological features due to a novel missense mutation in FLNC.

Myofibrillar myopathies (MFM) are mostly adult-onset diseases characterized by progressive morphological alterations of the muscle fibers beginning in the Z-disk and the presence of protein aggregates in the sarcoplasm. They are mostly caused by mutations in different genes that encode Z-disk proteins, including DES, CRYAB, LDB3, MYOT, FLNC and BAG3. A large family of French origin, presenting an autosomal dominant pattern, characterized by cardiac arrhythmia associated to late-onset muscle weakness, was evaluated to clarify clinical, morphological and genetic diagnosis. Muscle weakness began during adult life (over 30 years of age), and had a proximal distribution. Histology showed clear signs of a myofibrillar myopathy, but with unusual, large inclusions. Subsequently, genetic testing was performed in MFM genes available for screening at the time of clinical/histological diagnosis, and desmin (DES), αB-crystallin (CRYAB), myotilin (MYOT) and ZASP (LDB3), were excluded. LMNA gene screening found the p.R296C variant which did not co-segregate with the disease. Genome wide scan revealed linkage to 7q.32, containing the FLNC gene. FLNC direct sequencing revealed a heterozygous c.3646T>A p.Tyr1216Asn change, co-segregating with the disease, in a highly conserved amino acid of the protein. Normal filamin C levels were detected by Western-blot analysis in patient muscle biopsies and expression of the mutant protein in NIH3T3 showed filamin C aggregates. This is an original FLNC mutation in a MFM family with an atypical clinical and histopathological presentation, given the presence of significantly focal lesions and prominent sarcoplasmic masses in muscle biopsies and the constant heart involvement preceding significantly the onset of the myopathy. Though a rare etiology, FLNC gene should not be excluded in early-onset arrhythmia, even in the absence of myopathy, which occurs later in the disease course.

Classifications of neurogenetic diseases: An increasingly complex problem.

Neurodegenerative disorders represent a wide group of diseases affecting the central and/or peripheral nervous system. Many of these disorders were described in the 19th century, but our genetic knowledge of them is recent (over the past 25 years). However, the continual discovery of disease-causing gene mutations has led to difficulties in the classification of these diseases. For this reason, our present proposals for updating and simplifying the classification of some of these conditions (Charcot-Marie-Tooth diseases, distal hereditary motor neuropathies, hereditary sensory and autonomic neuropathies, hereditary spastic ataxias, hereditary spastic paraplegias and hereditary spastic ataxias) are expounded here.

Effects of a calcium supplement on bone mineral density in male cyclists.

AIM: The purpose of this study was to maintain or improve bone density in male road cyclists through provision of calcium and vitamin D3 supplementation ingested before cycling. METHODS: Participants were male cyclists (N=17), with a mean (±SD) age of 42.7 (9.4) years. Measurements of lumbar spine and hip areal bone mineral density (aBMD) were performed at the start and end of a cycling season. Cyclists were randomized into the calcium supplement (CAL) or the control group (CON) group based on lumbar spine T-scores. The CAL group was instructed to consume 1600 mg calcium and 1000 IU vitamin D3 prior to cycling for the 5-month period. RESULTS: Femoral trochanter aBMD significantly decreased during the 5 month cycling season. There was no difference in aBMD between CAL and CON groups. CONCLUSION: Negative effects of competitive cycling on aBMD in hip structures can be observed within 5 months. Calcium and vitamin D3 ingested prior to cycling does not ameliorate this effect. This proof of concept paper provides evidence that more work is needed to find mechanisms to protect cyclists from destructive bone loss in hip structures.

Motor neuronopathy in Chediak-Higashi syndrome.

Chediak-Higashi syndrome is a rare autosomal recessive disease characterized by partial oculocutaneous albinism, recurrent pyogenic infections and the presence of giant granules in many cells such as leucocytes (hallmark of the disease). Neurological symptoms are rare. We describe two sisters who presented the same phenotype of slowly progressive motor neuronopathy (with Babinski sign in one patient); biopsy of the sural nerve showed an abnormal endoneurial accumulation of lipofuscin granules. We discuss these two observations and compare them with the few case reports of neuropathy in Chediak-Higashi syndrome.

[Modified McPeek score in multiple trauma patients. Prospective evaluation of a points system for recording follow-up factors]. / Modifiziertes McPeek-Punkte-System bei Polytraumapatienten. Prospektive Evaluierung eines Punktesystems zur Erfassung von Verlaufsfaktoren.

BACKGROUND: Many commonly available trauma scores predict mortality, but to evaluate the success of a certain therapy or for difficult scientific and epidemiological purposes this may be insufficient in the face of improved survival rates. For outcome analysis of multiple trauma patients, the extent of medical resources needed could be an additional outcome measurement. McPeek et al. developed a potential scoring system for elective surgery patients, which was recently modified for multiple trauma patients. AIM: The current study investigated if the McPeek score could be prospectively used in multiple trauma patients and whether it could become an additional helpful tool in outcome assessment. Applicability was assessed by practical examples. MATERIAL AND METHODS: In this prospective single-centre study at the University Hospital of Innsbruck, Austria, between December 2008 and November 2010 multiple trauma patients (≥ 18 years) with an injury severity score (ISS) ≥ 17 were enrolled. Besides demographic data, prehospital vital parameters and diagnoses, all diagnoses from the trauma, mortality, length of stay in the intensive care unit and the hospital were recorded. The commonly used trauma scores ISS, revised trauma score (RTS), a severity characterization of trauma (ASCOT) and trauma and injury severity score (TRISS) were applied and an observed McPeek score was allocated following end of hospitalization. The McPeek scoring system was used according to the latest modifications. A correlation between trauma scores and the McPeek score was performed. The McPeek score was then predicted by a common trauma score using ordinal regression with the polytomous universal model (PLUM method). By subtracting the predicted from the observed McPeek scores the residual McPeek value was calculated and used for practical examples of outcome analysis with the McPeek scoring system. RESULTS: Out of 406 identified multiple trauma patients during the study phase, 183 had to be excluded due to missing data (mainly prehospital or following transfer). A total of 223 patients (mean ISS 31.2, mean age 47.2 years) were enrolled and assigned to the population-based observed McPeek score (median 4.0). Correlation coefficients were Glasgow coma scale (GCS) 0.59, ISS 0.62, RTS 0.65, TRISS 0.74 and ASCOT 0.77 (p < 0.0001). The TRISS predicted the McPeek score best in ordinal regression (pseudo-R(2) = 0.944, p < 0.0001). The residual McPeek score (observed minus predicted) was used to illustrate the influence of the blood glucose level on admission and the influence of head injury on outcome of multiple injury patients in detail. CONCLUSION: The modified McPeek score is applicable to multiple trauma patients to assess outcome for scientific or epidemiological purposes. Its main advantage is that it quantifies outcome independently of regional or national circumstances.

Flow cytometric detection of dyserythropoiesis: a sensitive and powerful diagnostic tool for myelodysplastic syndromes.

Several groups have published flow cytometry scores useful for the diagnosis or prognosis of myelodysplastic syndromes (MDS), mainly based on the detection of immunophenotypic abnormalities in the maturation of granulocytic/monocytic and lymphoid lineages. As anemia is the most frequent symptom of early MDS, the aim of this study was to identify markers of dyserythropoiesis relevant for the diagnosis of MDS analyzed by selecting erythroblasts in a whole no-lysis bone marrow strategy by using a nuclear dye. This prospective study included 163 patients, including 126 with cytopenias leading to MDS suspicion and 46 controls without MDS. In a learning cohort of 53 unequivocal MDS with specific markers, there was a significant difference between the coefficients of variation of mean fluorescence intensities of CD71 and CD36 in MDS patients compared with controls. These two parameters and the hemoglobin level were used to build a RED-score strongly suggestive of MDS if ≥ 3. Using the RED-score in the whole cohort, 80% of MDS or non-MDS patients were correctly classified. When combined with the flow score described by Ogata et al., this strategy allowed to reach a very high sensitivity of 88% of patients correctly classified.

POEMS syndrome with prominent acute axonal lesions.

Polyneuropathy is a common presenting component of POEMS syndrome whose symptoms are attributed to an overproduction of vascular endothelial growth factor (VEGF). We report two female patients with POEMS syndrome presenting as a severe predominantly axonal neuropathy. A nerve biopsy was performed for these patients; pathological data confirmed unusual numerous acute axonal lesions associated with other classical signs of POEMS syndrome. POEMS syndrome is usually associated with demyelinating neuropathy (and secondary axonal loss); however, prominent axonal neuropathy (with acute axonal lesions on nerve biopsy) can also be observed in this disease. These observations illustrate the heterogeneity of peripheral nervous system involvement in POEMS syndrome.

Study of accessory deep peroneal nerve motor conduction in a population of healthy subjects.

AIM OF THE STUDY: The short extensor muscle of the toes (extensor digitorum brevis) is innervated by the deep peroneal nerve (DPN) but can also receive innervation from an accessory deep peroneal nerve (ADPN), a branch of the superficial peroneal nerve (SPN). PATIENTS AND METHODS: We conducted a systematic electrophysiological study of the DPN and ADPN in 200 healthy subjects (400 legs). RESULTS: We found the presence of an ADPN in 13.5% of the subjects (8.5% of the legs). On average, ADPN amplitudes and motor potential areas were one-fifth of those for the corresponding DPN. Without this systematic search, most of the ADPN would not have been detected. Comparative study of electrophysiological parameters in patients with and without ADPN showed a significantly higher (P<0.0001) DPN motor potential area ratio (distal/proximal ratio) in subjects without an ADPN. CONCLUSION: Even though electrophysiological identification of the ADPN is generally not easy (in simple DPN motor conduction studies), the nerve can sometimes be clinically symptomatic (ankle pain). Evaluation of the DPN motor potential area ratio (distal/proximal ratio) heightens the detection of ADPN.

[Vitamin D and multiple sclerosis. A prospective survey of patients of Poitou-Charentes area]. / Vitamine D et sclérose en plaques. Étude prospective d'une cohorte de patients de la région Poitou-Charentes.

INTRODUCTION: Recent reports demonstrate the implication of vitamin D in multiple sclerosis (MS). METHODS: In a multicentric regional study (Poitou-Charentes area) during the first trimester 2010, we measured the 25-OH vitamin D serum level in 170 consecutive MS patients, and in 170 controls matched for age (±4 years), sex and date of blood sample analysis. We searched for correlations between 25-OH vitamin D serum levels and the MS form, the disability (EDSS), the relapse rate during the previous year and the presence and number of enhancing lesions on T1-weighted MRI dating less than 12 months in relapsing MS. RESULTS: Hypovitaminosis D was very frequent in MS patients and 25-OH vitamin D serum level was significantly lower (14.5 ± 9.2 mcg/mL) in MS patients than in the control group (16.7 ± 9.6 mcg/mL). This serum level was inversely correlated with the degree of disability measured with EDSS score and was lower in secondary progressive (RR-SP) and primary progressive (PP) MS than in relapsing MS (RR). No correlation was found between 25-OH vit D serum level and relapse rate during the previous year in RR MS and the presence and number of enhancing lesions on T1-weighted MRI dating from less than 12 months. CONCLUSION: 25-OH vit D serum level is very low in MS, mainly in RR-SP and PP MS and is correlated with disability. This suggests MS patients should be screened for vitamin D deficiency and given supplementation systematically when hypovitaminosis D is discovered.