SARS-CoV-2 and Guillain-Barré syndrome: AIDP variant with a favourable outcome.

BACKGROUND AND PURPOSE: The spectrum of COVID-19, caused by severe acute respiratory syndrome coronavirus 2 infection (SARS-CoV-2), includes different neurologic manifestations of the central and peripheral nervous system. METHODS: From March through April 2020, in two university hospitals located in western Switzerland, we examined three patients with Guillain-Barré syndrome (GBS) following SARS-CoV-2. RESULTS: These cases were characterized by a primary demyelinating electrophysiological pattern (Acute inflammatory demyelinating polyneuropathy or AIDP) and a less severe disease course compared to recently published case series. Clinical improvement was observed in all patients at week five. One patient was discharged from hospital after full recovery with persistence of minor neurological signs (areflexia). Two of the three patients remained hospitalized: one was able to walk and the other could stand up with assistance. CONCLUSIONS: We report three cases of typical GBS (AIDP) occurring after SARS-CoV-2 infection and presenting with a favourable clinical course. Given the interval between COVID-19-related symptoms and neurological manifestations (mean of 15 days) we postulate a secondary immune-mediated mechanism rather than direct viral damage.

What is new in 2015 in dysimmune neuropathies?

This review discusses and summarizes the concept of nodopathies, the diagnostic features, investigations, pathophysiology, and treatment options of chronic inflammatory demyelinating polyradiculoneuropathy, and gives updates on other inflammatory and dysimmune neuropathies such as Guillain-Barré syndrome, sensory neuronopathies, small-fiber-predominant ganglionitis, POEMS syndrome, neuropathies associated with IgM monoclonal gammopathy and multifocal motor neuropathy. This field of research has contributed to the antigenic characterization of the peripheral motor and sensory functional systems, as well as helping to define immune neuropathic syndromes with widely different clinical presentation, prognosis and response to therapy.

Successful long-term ambulatory norepinephrine infusions in a patient with pure autonomic failure.

We present a case study of a patient with pure autonomic failure who was successfully treated with ambulatory norepinephrine (NE) infusions over a 9-year-period of time before death occurred unexpectedly. Given this patient's response to the NE infusion treatment, we discuss the option of ambulatory NE infusions as a treatment for severe orthostatic hypotension that is refractory to common treatments.

[2013: what's new in inflammatory neuropathies]. / Revue de la littérature 2013--les neuropathies inflammatoires.

Several high-quality publications were published in 2013 and some major trials studies were started. In Guillain-Barré syndrome, events included the launch of IGOS and a better understanding of diagnostic limits, the effect of influenza vaccination, and better care, but uncertainty remains about analgesics. A new mouse model was also described. In chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), diagnostic pitfalls can be recalled. Our knowledge of underlying pathophysiological processes has improved, and the value of monitoring with function and deficit scores has been demonstrated. IVIG can sometimes be effective longer than expected, but CIDP remains sensitive to corticosteroids, particularly with the long-term beneficial effects of megadose dexamethasone. The impact of fingolimod remains to be demonstrated in an ongoing trial. Advances concerning multifocal motor neuropathy, inflammatory plexopathy, and neuropathy with anti -MAG activity are discussed but treatments already recognized as effective should not be changed. Imaging of peripheral nerve progresses.

[Neuropathies associated with paraproteinemia (monoclonal gammopathy)]. / Neuropathies associées aux paraprotéinémies (gammapathies monoclonales).

Coexistence of neuropathy and paraproteinemia (monoclonal gammopathy) is a common and complex problem seen in clinical practice and requires the distinction of specific syndromes. The clinical courses of these neuropathies are typically chronic and progressive. A precise distinction of the type of haematologic disorder associated (benign or malignant), investigation of other organs manifestations, and assessment of specific markers are mandatory. These steps are important to initiate an appropriate therapy that may include chemotherapy and/or immunosuppressive treatment targeting the neuropathy and the haematological dysfunction.

[Neuroborreliosis, tick-borne encephalitis and neurosyphilis]. / Neuroborréliose, méningo-encéphalite verno-estivale et neurosyphilis.

Infections affecting frequently the nervous system include Lyme disease, tick-borne encephalitis and syphilis. These three most dreaded neuro-infectious diseases observed in Switzerland are discussed, based on diagnostic criteria, screening testing, and treatments modalities. Neuroborreliosis and neurosyphilis are bacterial infectious diseases treatable by antibiotics, whereas the treatment of tick-borne encephalitis, a viral disease, is only based on preventive vaccination.

[Dysimmune neuromuscular disorders: diagnostic challenges and new ways of management]. / Maladies auto-immunes neuromusculaires: diagnostic et prise en charge.

This review describes some dysimmune neuromuscular disorders and their recent management: syndrome of peripheral nerve hyperexcitability (treatment of cramps, immunosuppressors); Guillain-Barré syndrome (new mechanisms and consensus treatment); chronic inflammatory demyelinating polyradiculoneuropathy (new indication for the use of pulse dexamethasone, new scores of activity); importance of subcutaneous immunoglobulin in multifocal motor neuropathy and of infusions of rituximab in myasthenia gravis; new entities in myositis and their treatment.

[2012: news in neurology]. / Neurologie.

In 2012, intramuscular midazolam appears as effective as intravenous lorezepam for the first line treatment of convulsive status epilepticus. Perampanel, a new anti-epileptic drug, will be soon available. Two oral treatments are now available for stroke prevention in atrial fibrillation setting. The methylphenidate and the Tai Chi could increase the walk capacity of patients suffering from Parkinson disease. A comprehensive cardiac work-up is essential for some congenital myopathy. A new drug against migraine seems free from vasoconstrictive effect. Antioxidants are harmful in Alzheimer disease. Some oral medication will be available for multiple sclerosis.

Effects of sensor ear tags with twin pin fixing system on health and well-being of cattle.

INTRODUCTION: A sensor ear tag (SET) containing Global Positioning System (GPS), accelerometer, Radio-Frequency Identification (RFID), and Bluetooth technologies was tested for wearing comfort and compliance with animal welfare requirements in cattle in a free stall barn and on summer pasture in Switzerland. The SET was equipped with a long-lasting battery via solar panel and used a «twin pin¼ fixing system. Right ears of 12 newborns and 26 adolescent animals were tagged with the SET. While left ears were tagged simultaneously with official ear tags in newborns, the adolescent animals already carried the official ear tags. The newborns stayed in a free stall barn during the entire experiment, while adolescent animals were housed in a free stall barn and on pasture during summer. All animals developed crusts beginning on day 7 after tagging with the SET. Pain reactions were observed occasionally in the first two weeks. Ear growth in newborns during 11 months of observation did not differ between ears with SET and official ear tags. Cortisol concentration in saliva of newborns decreased in the first week after tagging which is physiological for this age group. In older animals cortisol concentrations in saliva were not affected. We registered 19 incidences in 11 animals with the SET, that required veterinary or staff intervention. Two animals lost the SET with ear injury. Scars due to tag migration were observed in ears of all newborns after the 9th month of observation. In conclusion, SET with a weight of 32 g that need a twin pin fixation in cows do not seem to induce systemic or local inflammations more frequently compared to official ear tags; however, the higher risk of accidental injuries and migration in ear cartilage would not meet Swiss welfare standards and the attachment to the ear needs to be improved for general use.

INTRODUCTION: Une marque auriculaire à capteur (SET) contenant les technologies Global Positioning System (GPS), accéléromètre, identification par radiofréquence (RFID) et Bluetooth a été testée en termes de confort et de conformité aux exigences de bien-être animal chez des bovins dans une étable à stabulation libre et sur des pâturages d'estivage en Suisse. Le SET était équipé d'une batterie longue durée via un panneau solaire et utilisait un système de fixation «twin pin¼. Les oreilles droites de 12 veaux et de 26 génisses ont été équipées avec le SET. Les oreilles gauches ont été marquées en même temps avec les marques officielles chez les nouveau-nés alors que les génisses portaient déjà ces marques officielles. Les nouveau-nés ont été détenu dans une étable à stabulation libre, avec accès à une aire de sortie et aux paturages voisins pendant toute la durée de l'expérience tandis que les génisses ont été logés dans une étable à stabulation libre et en pâturage pendant l'été. Tous les animaux ont développé des croûtes à partir du 7e jour après le marquage avec le SET. Des réactions douloureuses ont occasionnellement été observées au cours des deux premières semaines. La croissance des oreilles des nouveau-nés au cours des 11 mois d'observation n'a pas différé entre les oreilles marquées par le SET et les oreilles marquées de manière standard. La concentration de cortisol dans la salive des nouveau-nés a diminué au cours de la première semaine successive au marquage, ce qui est physiologique pour ce groupe d'âge. Chez les animaux plus âgés, les concentrations de cortisol dans la salive n'ont pas été affectées. Nous avons enregistré 19 incidents chez 11 animaux avec le SET, qui ont nécessité l'intervention d'un vétérinaire ou d'un membre du personnel. Deux animaux ont perdu le SET avec blessure à l'oreille. Des cicatrices dues à la migration des marques ont été observées sur les oreilles de tous les nouveau-nés après le 9e mois d'observation. En conclusion, les SET d'un poids de 32 g qui nécessitent une fixation par deux tiges chez les bovins ne semblent pas induire d'inflammations systémiques ou locales plus fréquemment que les marques auriculaires officielles. Cependant le risque plus élevé de blessures accidentelles et de migration dans le cartilage de l'oreille ne correspondrait pas aux normes suisses en matière de bien-être et la fixation à l'oreille doit être améliorée pour une utilisation généralisée.

[2011: What's new in dysimmune neuropathies]. / Revue de la littérature 2011: neuropathies dysimmunitaires.

There are strong research activities in the field of dysimmune neuropathies. In Guillain-Barré syndrome, new pathophysiological mechanisms have been demonstrated with the potential development of new therapies, a clinical prediction model is applicable early in the course of disease, and under investigation are new treatment strategies with adapted intravenous Ig dosages. In chronic inflammatory demyelinating polyneuropathies, current diagnostic tests are discussed but biomarkers are needed, such as histological changes or differential gene expression in nerve or skin biopsies. The exploration of novel therapeutic approaches including monoclonal antibodies and oral immunosuppressants, known from multiple sclerosis studies, suggests new approaches to treatment. Changes of the peripheral nerves on MR imaging are better known and the usefulness of serum antibodies is reviewed.

[Neurology]. / Neurologie.

In 2011, new oral anticoagulants for atrial fibrillation are available and the ABCD3-I score predicting stroke after TIA updates the ABCD2 score. New McDonald criteria allow faster MS diagnosis and the first oral treatment (fingolimod) for MS can be prescribed. A new anti-antiepileptic drug (retigabine) is available and sodium valproate has long term neurological adverse effects after in utero exposure. Among Parkinson disease treatments, deep brain stimulation is extending applications and dopamine agonists with extended release are as efficient and well tolerated as standard forms at long term scale. Monoclonal antibodies and immunosuppressant agents are proposed as good alternatives in the treatment of chronic dysimmune polyneuropathies. Gene therapy for the treatment of genetic myopathies is progressing.

Foodborne botulism, a forgotten yet life-threatening disease: a case report.

OBJECTIVE: Botulism is a very rare disease in Switzerland, with less than one case per year, an incidence of 0.01 cases for 100,000 inhabitants. Indeed, over the past ten years, 9 cases have been reported to Public Health registry. Foodborne botulism (FB) is caused by ingestion of preformed botulinum neurotoxin. Characteristic features should be rapidly recognized, and prompt treatment should be administered to avoid further progression towards respiratory failure and death. CASE REPORT: We report the case of a patient who developed gastrointestinal symptoms just after a sandwich consumption followed by rapidly progressive cranial nerve impairment, truncal muscle weakness in a descending pattern and respiratory failure requiring mechanical ventilation. The diagnosis of foodborne botulism was delayed due to differential diagnosis considerations. Specific antitoxin therapy was administered immediately after firm clinical conviction of botulism, without waiting for serologic results that later confirmed the diagnosis. As expected, muscle weakness recovery was slow, with persistent chronic deficits nine years later. CONCLUSIONS: This case highlights differential diagnosis issues of botulism. These include acute neuromuscular disorders such as myasthenia gravis, Guillain-Barré syndrome, or tick-borne encephalitis. The importance of careful medical history and repeated clinical evaluation to avoid misdiagnosis can be lifesaving. Our case highlights the typical warning signs.

[Therapeutic advances in neurology]. / Neurologie.

This article summarizes the main therapeutic advances of 2010 in the field of neurology. It focuses on aspects that are likely to change the care of patients in clinical practice. Among these, we discuss the new oral treatments that have proved to be effective in multiple sclerosis, the results of two large studies comparing endarterectomy and stenting in carotid stenosis, novel therapeutic approaches for the treatment of non-motor symptoms in Parkinson's disease as well as the results of several pharmacological studies in the field of epilepsy.

Ulnar neuropathy at the elbow: Reappraisal of the wrist-upper arm latency difference between ulnar and median nerves.

OBJECTIVES: To evaluate the sensitivity and specificity of the latency difference (DLat) between ulnar and median nerves of the arm after stimulation at the wrist; one of the easiest techniques proposed for recognizing ulnar neuropathy at the elbow (UNE). As latency difference is not a standardized technique, we set up a multicenter study to recruit large numbers of normal subjects and patients with UNE or generalized neuropathy. METHODS: Six centers participated in the study with data obtained from three groups of participants, controls (CTRLs), patients with UNE and patients with generalized neuropathy (GNP). We first verified the anatomical superposition of the ulnar and median nerves in cadaver examination. The optimal recording site for these two nerves was found to be 10 cm above the medial epicondyle. We then standardized the position of the arm with full extension of the elbow and stimulated first the median and then the ulnar nerves at the wrist. CTRLs were examined on both arms at two consecutive visits. RESULTS: We recorded 32 idiopathic UNE cases, 44 GNP patients and 62 controls. We demonstrated that a DLat cut-off value of 0.69 ms brings a sensitivity of 0.86 and specificity of 0.89 to discriminate CTRLs from UNE. We also validated that intra-examiner reproducibility was good. CONCLUSION: We report a lower normal value for DLat than reported in several non-standardized studies and CTRL and UNE groups have clearly separated DLat values. SIGNIFICANCE: Due to its high sensitivity, our standardized technique could be used as a first-line diagnostic tool when UNE is suspected.

[Neuropathy associated with monoclonal gammopathy (dysglobulinemia)]. / Neuropathies associées aux gammapathies monoclonales (dysglobulinémies).

Neurological complications of monoclonal gammopathy, or dysglobulinemia, are typically affecting the peripheral nerve. The clinical course is often chronic and progressive and requires a precise diagnosis of the type of plasma cell disorder associated with the neuropathy, to investigate other organs manifestations and to assess the presence of specific markers. These steps are required to initiate an appropriate therapy that may include chemotherapy, immunosuppressive or immunomodulatory treatment.

[Therapeutic advances in neurology]. / Neurologie.

The neurology field has been greatly improved in 2008. The therapeutic window of intravenous thrombolysis for acute ischemic stoke is extended to 4 h 30. New studies show that the clinical progression of Parkinson's disease might be slowed by some medication. Deep brain stimulation may be beneficial early in the course of the disease. Tysabri and Fingolimod in multiple sclerosis are discussed. The pharmacopoeia for epilepsy is in constant development with new products recently released in Switzerland. CGRP receptor antagonists are about to be launched as a promising acute migraine treatment. The pharmacological approach in amyotrophic lateral sclerosis patients might be improved according to research results.

Glucocorticoid corticosteroids for Duchenne muscular dystrophy.

BACKGROUND: Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy of childhood. This incurable disease is characterised by muscle wasting and loss of walking ability leading to complete wheelchair dependence by 13 years of age. Prolongation of walking is one of the major aims of treatment. OBJECTIVES: The aim of this review was to assess whether glucocorticoid corticosteroids stabilize or improve muscle strength and walking in boys with DMD. SEARCH STRATEGY: This is an update of the Cochrane systematic review first published in 2004 (Manzur 2004). We searched the Cochrane Neuromuscular Disease Group Trials Register (August 2006) using the term 'Duchenne muscular dystrophy'. We also searched MEDLINE (January 1966 to July 2007), EMBASE (January 1980 to August 2006), CINAHL and LILACS (January 1982 to August 2006). We wrote to authors of published studies and other experts in this disease to help identify other trials, checked the references in the identified trials and hand searched the abstracts of relevant journals. SELECTION CRITERIA: Types of studies: randomised or quasi-randomised trials. TYPES OF PARTICIPANTS: all patients with a definite diagnosis of Duchenne muscular dystrophy. Types of interventions: glucocorticoids such as prednisone, prednisolone, deflazacort or others, with a minimum treatment period of three months. PRIMARY OUTCOME MEASURE: prolongation of walking (independent walking without long leg calipers). SECONDARY OUTCOME MEASURES: strength outcome measures, manual muscle strength testing using Medical Research Council strength scores, functional outcome measures and adverse events. DATA COLLECTION AND ANALYSIS: We identified six randomised controlled trials that met the inclusion criteria for our review, and one of these (Beenakker 2005) is a new addition to this update, as it was published subsequent to our first review (Manzur 2004). Two review authors independently selected the trials for the review and assessed methodological quality. Data extraction and inputting were double-checked. PRIMARY OUTCOME MEASURE: data from one small study used prolongation of walking as an outcome measure and did not show significant benefit. SECONDARY OUTCOME MEASURES: The meta-analysis of the results from four randomised controlled trials with altogether 249 participants showed that glucocorticoid corticosteroids improved muscle strength and function over six months. Improvements were seen in time taken to rise from the floor (Gowers' time), nine metres walking time, four-stair climbing time, ability to lift weights, leg function grade and forced vital capacity. One randomised controlled trial with altogether 28 participants showed that glucocorticoid corticosteroids stabilize muscle strength and function for up to two years. The most effective prednisolone regime appears to be 0.75 mg/kg/day, given in a daily dose regime. Not enough data were available to compare efficacy of prednisone with deflazacort. Adverse effects: Excessive weight gain, behavioural abnormalities, cushingoid appearance and excessive hair growth were all more common with glucocorticoid corticosteroids than placebo. Long-term adverse effects of glucocorticoid therapy could not be evaluated because of the short-term duration of the randomised studies.Non-randomised studies: A number of non-randomised studies with important efficacy and adverse effects data are tabulated and discussed. AUTHORS' CONCLUSIONS: There is evidence from randomised controlled studies that glucocorticoid corticosteroid therapy in Duchenne muscular dystrophy improves muscle strength and function in the short-term (six months to two years). The most effective prednisolone regime appears to be 0.75 mg/kg/day, given daily. In the short term, adverse effects were significantly more common but not clinically severe. Long-term benefits and hazards of glucocorticoid treatment cannot be evaluated from the currently published randomised studies. Non-randomised studies support the conclusions of functional benefits but also identify clinically significant adverse effects of long-term treatment. These benefits and adverse effects have implications for future research studies and clinical practice.

Stimulus-response curve of human motor nerves: multicenter assessment of various indexes.

The value of various indexes to characterize the stimulus-response curve of human motor nerves was assessed in 40 healthy subjects recruited from four European centers of investigation (Créteil, Lausanne, Liège, Marseille). Stimulus-response curves were established by stimulating the right median and ulnar motor nerves at the wrist, with stimulus durations of 0.05 and 0.5 ms. The following parameters were studied: the threshold intensity of stimulation to obtain 10% (I 10), 50% (I 50), and 90% (I 90) of the maximal compound muscle action potential, the ratios I 10/I 50, I 90/I 50, (I 90 - I 10)/I 10, (I 90-I 50)/I 50, and (I 50 - I 10)/I 10, and the slopes of the stimulus-response curves with or without normalization to I 50. For each parameter, within-center variability and reproducibility (in a test-retest study) were assessed and between-center comparisons were made. For most of the parameters, the results varied significantly within and between the centers. Within the centers, only the ratios I 10/I 50 and I 90/I 50 were found constant and reproducible. Between the centers, the absolute intensity thresholds (I 10, I 50, I 90) and the ratio I 90/I 50 did not show significant differences at stimulus duration of 0.5 ms, whatever the stimulated nerve. The reduced variability and good reproducibility of the ratios I 10/I 50 and I 90/I 50 open perspectives in neurophysiological practice for the use of these indexes of the stimulus-response curve, a rapid and noninvasive test.

Autosomal dominant nemaline myopathy: a new phenotype unlinked to previously known genetic loci.

We report a large family with a mild form of autosomal dominant nemaline myopathy and a new phenotype. Onset of symptoms was in infancy with hypotonia and motor delay. Weakness involved neck flexors, abdominal and proximal limb muscles. There was no bulbar, respiratory or foot dorsiflexion weakness and no slowness in movement. Patients had remarkably good physical endurance and no limitation in daily activities, but were slow runners since childhood. Nemaline rods were seen in less than 5% of muscle fibres. No linkage to the five known nemaline myopathy genes (alpha-tropomyosin-3, nebulin, alpha-actin, troponin T1 and beta-tropomyosin), to the ryanodine receptor gene (associated with core-rod myopathy) or to the 15q21-23 locus was found.