Search

Background and Purpose Chronic inflammatory demyelinating polyneuropathy ( CIDP) may have variable evolution profiles, which have not been compared between cohorts. The relationship of disease status with motor strength, function and electrophysiology is uncertain. Methods Disease status was studied with a simplified proposed scale in two patient cohorts totalling 72 subjects from Leicester, UK, and Angers, France. Clinical and electrophysiological records were analysed. Results Independent ascertainment of disease status in each cohort revealed similar rates of remission ( P = 0.23), stable/improving disease ( P = 0.34) and unstable/active disease ( P = 1). No correlation was ascertained with strength or function. Median nerve compound muscle action potential was the only independent electrophysiological predictor of disease status ascertained ( P = 0.046). Conclusions Disease status distribution may represent an important comparative indicator for management of CIDP cohorts and could be useful for benchmarking service and treatment provision. Degree of upper limb motor axonal loss may represent a useful electrophysiological marker of disease status in CIDP. [ABSTRACT FROM AUTHOR]

Abstract: To investigate neurofilament (NF) dynamics during the cytoskeleton reorganization in regenerating axons, and their electrophysiological and histological consequences, we used two transgenic lines of mice: neurofilament high (NFH)-LacZ and NFH-green fluorescent protein (GFP). In NFH-LacZ mice, NFs are retained in cell bodies and deficient in axons (Eyer and Peterson, 1994), while in NFH-GFP mice the fluorescent fusion protein is normally transported along axons (Letournel et al., 2006). Following a crush of the sciatic nerve, conduction recovery in NFH-GFP mice is similar to wild-type (wt) mice, but it is reduced in NFH-LacZ mice. Moreover, changes of axonal calibres following regeneration are similar between NFH-GFP and wt mice, but they are systematically reduced in NFH-LacZ mice. Finally, the axonal transport of NFH-GFP fusion protein and NFs is re-initiated after the crush as evidenced by the fluorescent and immunolabelling of axons distal from the crushed point, but NFs and the fusion protein are not transported along axons during regeneration in NFH-LacZ mice. Together, these results argue that the absence of axonal NFs in NFH-LacZ mice compromises the axonal regeneration, and that the NFH-GFP reporter fusion protein represents an efficient model to evaluate the NF dynamics during axonal regeneration. [Copyright &y& Elsevier]

Abstract: Intraventricular meningiomas are infrequent intracranial tumors. Clinical symptoms are mainly due to an increased intracranial pressure or a direct pressure on the surrounding brain structures. Inflammatory syndrome was described in some patients with chordoid meningiomas. Here we report a case of right intraventricular clear cell meningioma in a 50-year-old man who presented with fever, headache, and inflammatory syndrome. Clinical and biological normalization was rapidly obtained after tumor removal. Immunohistochemical examination showed tumor cells and lymphocytes positivity for the pyrogenic cytokine interleukin-6, with a same intensity. To our knowledge, this is the first case described in the literature concerning an adult man with an intraventricular clear cell meningioma associated with a systemic inflammatory syndrome. [Copyright &y& Elsevier]

Abstract: We report a case of a 9-month pregnant woman who presented acute psychiatric and neurological symptoms with extensive involvement of the white matter on MRI and no oligoclonal bands on CSF examination. Despite high doses of intravenous steroids, plasmapheresis and immunosuppressive drugs, a fatal outcome (coma) was noted 8 months later. Neuropathological examination confirmed the diagnosis of Marburg''s type of multiple sclerosis showing sharp-edged lesions of demyelination, giant astrocytes, numerous macrophages and little perivascular inflammation. We discuss the definition and limits of the Marburg entity with reference to acute disseminated encephalomyelitis, impact of pregnancy, unusual MRI features, neuropathology and treatment. [Copyright &y& Elsevier]

La polyradiculonévrite inflammatoire démyélinisante chronique (PIDC) est une pathologie hétérogène, qui peut avoir une évolution sévère et une mauvaise réponse thérapeutique. L'apport de l'électrophysiologie, l'IRM et la découverte d'anticorps spécifiques ont permis de mieux caractériser et diagnostiquer cette pathologie. Présentation d'un cas clinique d'une nodo-paranodopathie d'évolution subaiguë et sévère, résistante aux immunoglobulines intra-veineuses (Ig-iv). Une femme de 57 ans, sans antécédents particuliers, se présente en 2017 pour l'apparition rapidement progressive d'un déficit sensitivomoteur douloureux des quatre membres responsables de troubles de la marche avec une importante ataxie et des difficultés croissantes pour réaliser les activités manuelles. Un ENMG montre des arguments en faveur d'un neuropathie démyélinisante (critères en faveur d'une PIDC), la TDM-TAP est sans particularité et la ponction lombaire montre une hyperprotéinorachie majeure à 4 g/L. Les Ig-iv ont été débutées, mais malgré un traitement bien conduit, la patiente a continué à s'aggraver avec majoration de son ataxie proprioceptive et du déficit moteur ainsi que du tremblement, devenu invalidant. Un traitement par corticoïdes a été débuté, avec une réponse dose-dépendante. Devant la présentation clinique, l'absence de réponse aux Ig-iv et l'amélioration dose-dépendante sous corticoïdes, la recherche des anticorps impliqués dans les nodo-paranodopathies a été entreprise et a mis en évidence la présence d'anticorps anti-Caspr1. Un traitement par Rituximab a été mis en place avec l'amélioration progressive de la symptomatologie. Il convient d'évoquer une nodo-paranodopathie chez les patients présentant une PIDC sévère, d'évolution subaiguë et résistante aux Ig-iv afin de débuter rapidement un traitement par Rituximab, souvent très efficace dans ces sous-types de PIDC. [ABSTRACT FROM AUTHOR]

Background: The electrodiagnostic value of distal compound muscle action potential duration (DCMAPD) has been studied rarely in chronic inflammatory demyelinating polyneuropathy (CIDP). Cut-offs proposed have not been widely evaluated. The influence of low-cut EMG filter settings ≤ 10 Hz as used in Europe is uncertain. Methods: We retrospectively reviewed records of 110 patients with typical, treatment-responsive CIDP, from Leicester, U.K., Paris and Angers, France. All fulfilled revised European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) clinical and electrodiagnostic criteria for typical CIDP (2010), before consideration of DCMAPD prolongation. Results were compared with those of 110 controls with chronic sensory/sensory-motor axonal neuropathy. We constructed receiver operating characteristic (ROC) curves for each nerve and derived cut-offs for DCMAPD prolongation, offering specificity of ≥ 98% vs. controls. Results: DCMAPD was significantly greater in all nerves for CIDP patients, compared with controls ( P < 0.001). ROC curves allowed derivation of cut-offs of sensitivities ranging from 27.1% (ulnar nerve) to 60% (tibial nerve). Using these cut-offs to define DCMAPD prolongation in any studied motor nerve offered a sensitivity of 69.1% for CIDP and specificity of 97.3% vs. controls. Conclusion: Cut-offs for DCMAPD are dependent on EMG filter settings. DCMAPD prolongation in any motor nerve, using our derived cut-offs, represents a sensitive and specific marker of CIDP in patients studied with EMG equipment with low-cut filter settings ≤ 10 Hz. Appropriate use of this parameter appears an essential criterion to consider in assessing suspected CIDP, which may be helpful in limiting extensiveness and duration of electrophysiological testing, thereby reducing patient discomfort. [ABSTRACT FROM AUTHOR]

Aims: Histological analysis of brain tissue samples provides valuable information about the pathological processes leading to common neurodegenerative disorders. In this context, the development of novel high‐resolution imaging approaches is a current challenge in neuroscience. Methods: To this end, we used a recent super‐resolution imaging technique called STochastic Optical Reconstruction Microscopy (STORM) to analyse human brain sections. We combined STORM cell imaging protocols with neuropathological techniques to image cryopreserved brain samples from control subjects and patients with neurodegenerative diseases. Results: This approach allowed us to perform 2D‐, 3D‐ and two‐colour‐STORM in neocortex, white matter and brainstem samples. STORM proved to be particularly effective at visualizing the organization of dense protein inclusions and we imaged with a <50 nm resolution pathological aggregates within the central nervous system of patients with Alzheimer's disease, Parkinson's disease, Lewy body dementia and fronto‐temporal lobar degeneration. Aggregated Aβ branches appeared reticulated and cross‐linked in the extracellular matrix, with widths from 60 to 240 nm. Intraneuronal Tau and TDP‐43 inclusions were denser, with a honeycomb pattern in the soma and a filamentous organization in the axons. Finally, STORM imaging of α‐synuclein pathology revealed the internal organization of Lewy bodies that could not be observed by conventional fluorescence microscopy. Conclusions: STORM imaging of human brain samples opens further gates to a more comprehensive understanding of common neurological disorders. The convenience of this technique should open a straightforward extension of its application for super‐resolution imaging of the human brain, with promising avenues to current challenges in neuroscience. [ABSTRACT FROM AUTHOR]

Background and purpose: Fifteen percent of patients with myasthenia gravis (MG) are refractory to conventional treatment. Case reports and a few studies show probable benefit of rituximab in these cases. Our objective was to assess the efficacy and the safety of rituximab in patients with MG, in a multicentric real‐life study. Method: Inclusion criteria were: age > 18 years; MG with anti‐acetylcholine receptor (AChR) antibodies, anti‐muscle‐specific kinase (MuSk) antibodies or significant decrement after repetitive nerve stimulation; Myasthenia Gravis Foundation of America (MGFA) class >II; refractory or steroid‐dependent MG; and treatment with rituximab. Efficacy was assessed at 6 months using the MGFA‐post‐intervention status (PIS) score, the myasthenic muscle score (MMS) and the number of patients receiving steroids <10 mg/day. Data on adverse events were collected. Results: Twenty‐nine patients were included: 20 with anti‐AChR MG, five with anti‐MuSK MG and four with seronegative MG. MGFA‐PIS score was improved or better (improved, minimal manifestations or remission) in 86.2% of patients after 6 months of treatment (P < 0.0001). The mean MMS increased from 68.8 to 83.1 (P < 0.0001). A decrease in steroid dosage (<10 mg/day) was effective in 57.9% of treated patients. In all, 42.8% of patients experienced adverse events: infections (21.4% of patients); infusion reaction (7%); bradycardia (3.7%); and cytopenia (7%). Conclusion: The present study demonstrates the efficacy and safety of rituximab in patients with MG. Additional studies remain necessary to determine the role of rituximab in the pharmacopeia of MG treatment and to establish precise recommendations for the infusion protocol. [ABSTRACT FROM AUTHOR]

Background and purpose: The frequency of pain and cramps is uncertain in anti-myelin associated glycoprotein antibody (anti-MAG) neuropathy. Whether these symptoms may affect function/quality of life is unknown. Methods: A cross-sectional study of the prevalence, correlates and impact of pain, pain subtypes and cramps, their severity, frequency and anatomical distribution was performed for 55 clinically stable patients with anti-MAG neuropathy. Results: Pain of any type was reported by 80% of subjects. The most common subtype was paraesthesiae and dysaesthesiae (70%). Cramps were reported by >60% of patients, with lower limb cramps in all and upper limb cramps in about 20%. Cramps affected daily activities in >30% of these subjects, sleep in 60%, ability to exercise in >30%. Total pain score correlated with several Short Form 36 health-related quality of life (SF-36 HR-QoL) measures (P < 0.05), with Inflammatory Rasch-built Overall Disability Scale (I-RODS) (P = 0.006) and 10-m timed walk (P = 0.019). An independent association was ascertained with I-RODS (P = 0.002). Different pain subtypes showed multiple associations with SF-36 HR-QoL measures and/or functional scales. Upper limb cramps had multiple SF-36 HR-QoL functional correlates, with an independent association with the Overall Neuropathy Limitation Score (ONLS) (P = 0.004). Cramp severity correlated with ONLS (P = 0.04) and I-RODS (P = 0.028) and inversely with level of physiotherapy input (P = 0.009). Cramp frequency was associated with tremor score (P = 0.004) and multiple SF-36 HR-QoL subsections. Conclusions: Neuropathic pain and cramps may affect function and quality of life in anti-MAG neuropathy. Optimizing treatments of these symptoms, including by adequate levels of physiotherapy, may be beneficial in affected patients and requires further research. [ABSTRACT FROM AUTHOR]

Summary Background Somatosensory evoked potentials (SSEPs) are increasingly performed for the assessment of peripheral neuropathies, but no practical guidelines have yet been established in this specific application. Study aim To determine the relevant indication criteria and optimal technical parameters for SSEP recording in peripheral neuropathy investigation. Methods A survey was conducted among the French-speaking practitioners with experience of SSEP recording in the context of peripheral neuropathies. The results of the survey were analyzed and discussed to provide recommendations for practice. Results SSEPs appear to be a second-line test when electroneuromyographic investigation is not sufficiently conclusive, providing complementary and valuable information on central and proximal peripheral conduction in the somatosensory pathways. Conclusions Guidelines for a standardized recording protocol, including the various parameters to be measured, are proposed. Clinical relevance We hope that these proposals will help to recognize the value of this technique in peripheral neuropathy assessment in clinical practice. [ABSTRACT FROM AUTHOR]

La découverte d'une élévation anormale de la vitamine B12 sanguine (B12) est le plus souvent fortuite. Cela interroge souvent les cliniciens sur l'intérêt d'explorer cette anomalie biologique, parfois sans cause apparente. L'association entre B12 élevée et cancer solide a été documentée par deux études sur registres de santé. Cependant, leur design ne permettait pas un ajustement sur les autres comorbidités associées à une B12 élevée. Les objectifs de notre étude étaient de confirmer cette association après ajustement sur les causes connues d'élévation de la B12 et d'étudier ses variations selon le taux de B12, le type de cancer et la présence de métastases. Nous avons étudié tous les patients avec un dosage sanguin de B12 durant une hospitalisation dans les services de médecine interne, gériatrie ou neurologie du CHU d'Angers entre janvier 2007 et décembre 2016. Nous avons comparé les patients avec une B12 élevée (≥ 1000 ng/L) à des témoins présentant une B12 < 1000 ng/L, appariés en 1/1 sur l'âge, le sexe et le service d'origine. Les analyses étaient ajustées sur les causes connues de B12 élevée : hémopathies myéloïdes, hépatopathies aiguës ou chroniques, néphropathies chroniques, maladies auto-immunes ou inflammatoires, supplémentation excessive en B12 et cancers solides. Notre étude a comparé 785 patients avec une B12 ≥ 1000 ng/L à 785 témoins appariés. Parmi les patients avec une B12 ≥ 1000 ng/L, 252 (32,1 %) patients avaient un cancer solide, contre 97 (12,4 %) dans le groupe témoin (p < 0,001). Après ajustement, toutes les comorbidités connues pour élever la B12 étaient plus fréquentes chez les patients avec une B12 ≥ 1000 ng/L, à l'exception des maladies auto-immunes ou inflammatoires. Une B12 élevée était associée à la présence d'un cancer solide sans (OR 1,96 [IC95 % : 1,18–3,25]) et avec métastase (OR 4,21 [IC95 % : 2,67–6,64]). Les cancers du pancréas, du côlon/rectum, des poumons, de la prostate, de l'urothélium et les métastases hépatiques et osseuses étaient significativement associés à une B12 élevée. La fréquence des cancers solides croissait avec l'importance de l'élévation de la B12, notamment en présence de métastases et au-delà de 1250 ng/L : 13,7 % pour le sous-groupe 1000–1249 ng/L, 21,3 % pour le sous-groupe 1250–1749 ng/L, et 24,1 % pour le sous-groupe ≥ 1750 ng/L. Une B12 élevée doit faire considérer la possibilité d'un cancer solide sous-jacent. L'absence d'association entre cancers hépatiques et B12 élevée dans notre étude, contrairement à celles sur registres, s'explique par l'ajustement sur les comorbidités hépatiques. L'augmentation de la force de l'association en fonction du taux de B12 dans les cancers métastatiques interroge sur un lien entre la B12 et la masse tumorale ou sa dissémination. [ABSTRACT FROM AUTHOR]