Lower Motoneuron Dysfunction Impacts Spontaneous Motor Recovery in Acute Cervical Spinal Cord Injury.

Paresis after spinal cord injury (SCI) is caused by damage to upper and lower motoneurons (LMNs) and may differentially impact neurological recovery. This prospective monocentric longitudinal observational study investigated the extent and severity of LMN dysfunction and its impact on upper extremity motor recovery after acute cervical SCI. Pathological spontaneous activity at rest and/or increased discharge rates of motor unit action potentials recorded by needle electromyography (EMG) were taken as parameters for LMN dysfunction and its relation to the extent of myelopathy in the first available spine magnetic resonance imaging (MRI) was determined.

The Prevention of Positioning Injuries During Gynecologic Surgery. Guideline of the DGGG, OEGGG and SGGG (S2k Level, AWMF Registry Number 015/077, October 2020).

Positioning injuries are relatively common, forensically highly relevant complications of gynecologic surgery. The aim of this official AWMF S2k-guideline is to provide statements and recommendations on how to prevent positioning injuries using the currently available literature. The literature was evaluated by an interdisciplinary group of experts from professional medical societies.

Modeling and Bioinformatics Identify Responders to G-CSF in Patients With Amyotrophic Lateral Sclerosis.

Developing an integrative approach to early treatment response classification using survival modeling and bioinformatics with various biomarkers for early assessment of filgrastim (granulocyte colony stimulating factor) treatment effects in amyotrophic lateral sclerosis (ALS) patients. Filgrastim, a hematopoietic growth factor with excellent safety, routinely applied in oncology and stem cell mobilization, had shown preliminary efficacy in ALS. We conducted individualized long-term filgrastim treatment in 36 ALS patients.

Chance or challenge, spoilt for choice? New recommendations on diagnostic and therapeutic considerations in hereditary transthyretin amyloidosis with polyneuropathy: the German/Austrian position and review of the literature.

Hereditary transthyretin amyloidosis is caused by pathogenic variants (ATTR) in the TTR gene. Alongside cardiac dysfunction, the disease typically manifests with a severely progressive sensorimotor and autonomic polyneuropathy. Three different drugs, tafamidis, patisiran, and inotersen, are approved in several countries, including the European Union and the United States of America.

Magnetic Measurement of Electrically Evoked Muscle Responses With Optically Pumped Magnetometers.

Objective: Electroneurography has been an essential method for assessing peripheral nerve disorders for decades. During this procedure, a nerve is briefly electrically excited, and nerve conduction properties are identified by indirect means from the behavior of the innervated muscle. The magnetic field of the resulting muscle response can also be recorded by novel, uncooled magnetometers, which have become very attractive for different medical applications over recent years.

Signal Modeling and Simulation of Temporal Dispersion and Conduction Block in Motor Nerves.

Objective: Electroneurography is a well-established diagnostic test for supporting the diagnosis of disorders of myelinated peripheral nerves. Neurophysiological quantities are automatically calculated and are used to determine the pathology of the nerve (axonal damage) or its sheath (myelin damage). Specific differential diagnostic criteria are derived from time-domain normative data, which result primarily from a computer simulation in the early 1990s based on animal data, namely rats.

Biomarker Supervised G-CSF (Filgrastim) Response in ALS Patients.

To evaluate safety, tolerability and feasibility of long-term treatment with Granulocyte-colony stimulating factor (G-CSF), a well-known hematopoietic stem cell factor, guided by assessment of mobilized bone marrow derived stem cells and cytokines in the serum of patients with amyotrophic lateral sclerosis (ALS) treated on a named patient basis. 36 ALS patients were treated with subcutaneous injections of G-CSF on a named patient basis and in an outpatient setting. Drug was dosed by individual application schemes (mean 464 Mio IU/month, range 90-2160 Mio IU/month) over a median of 13.

Combinatory Biomarker Use of Cortical Thickness, MUNIX, and ALSFRS-R at Baseline and in Longitudinal Courses of Individual Patients With Amyotrophic Lateral Sclerosis.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative process affecting upper and lower motor neurons as well as non-motor systems. In this study, precentral and postcentral cortical thinning detected by structural magnetic resonance imaging (MRI) were combined with clinical (ALS-specific functional rating scale revised, ALSFRS-R) and neurophysiological (motor unit number index, MUNIX) biomarkers in both cross-sectional and longitudinal analyses. The unicenter sample included 20 limb-onset classical ALS patients compared to 30 age-related healthy controls.

Misleading bilateral blink reflex loss in a severe tetanus case.

Tetanus is rare and often forgotten in the diagnostic workup. The diagnosis is mainly based on typical clinical symptoms, because of missing sensitive paraclinical test. As described in our case, a missing bilateral blink reflex may occur in severe tetanus, which should not lead to the rejection of the diagnosis.

Neurographic course Of Wallerian degeneration after human peripheral nerve injury.

Introduction: Neurographic data on Wallerian degeneration (WD) after motor nerve injury are available only from animal studies and human case reports of 9 patients altogether. A precise knowledge of neurographic features of WD would be highly relevant for diagnostic, prognostic, therapeutic, and forensic aspects of traumatic lesions.

Methods: We prospectively studied WD in patients with a peripheral nerve injury.

Myopathy in Childhood Muscle-Specific Kinase Myasthenia Gravis.

Background: Adult and pediatric patients suffering from MuSK (muscle-specific kinase) -antibody positive myasthenia gravis exhibit similar features to individuals with acetylcholine receptor (AChR) antibodies, but they differ in several characteristics such as a predominant bulbar, respiratory and neck weakness, a generally worse disease severity and a tendency to develop muscle atrophy. Muscle atrophy is a rare phenomenon that is usually restricted to the facial muscles.

Results: We describe a girl with MuSK-antibody positive myasthenia gravis who developed a myopathy with severe generalized muscular weakness, muscle atrophy, and myopathic changes on electromyography.

Autonomic and peripheral nervous system function in acute tick-borne encephalitis.

Objectives: Tick-borne encephalitis (TBE) is an emerging flaviviral zoonosis in Central and Eastern Europe. TBE can present as meningitis, meningoencephalitis, or meningoencephalomyelitis. Dysfunction of the autonomic (ANS) and peripheral motoric and sensory nervous system (PNS) might contribute to acute and long-term complications.

Stein's Double Cross-Lip Flaps Combined with Johanson's Step Technique for Subtotal Lower Lip Reconstruction.

Unlabelled: In a previous study, a single cross-lip flap (Abbe flap) combined with Johanson's step technique for repair of defects of more than 2/3 of the lower lip was superior, in terms of aesthetic and functional outcome, compared with Bernard Webster-related techniques (cheek advancement). Herewith, a double cross-lip flap (Stein procedure) is proposed for repair of subtotal lower lip defects. A systematic review of the Stein procedure is provided.

Toward the validation of a new method (MUNIX) for motor unit number assessment.

Introduction: This prospectively designed study analyzed the correlation of a new, non-invasive neurophysiological method (Motor Unit Number Index - MUNIX) with two established Motor Unit Number Estimation (MUNE) methods.

Methods: MUNIX and incremental stimulation MUNE (IS-MUNE) were done in the abductor digiti minimi muscle (ADM), while MUNIX and spike-triggered averaging MUNE (STA-MUNE) were tested in the trapezius muscle. Twenty healthy subjects and 17 patients with amyotrophic lateral sclerosis (ALS) were examined.

The Prevention of Positioning Injuries during Gynecologic Operations. Guideline of DGGG (S1-Level, AWMF Registry No. 015/077, February 2015).

Official guideline published and coordinated by the German Society of Gynecology and Obstetrics (DGGG). Positioning injuries after lengthy gynecological procedures are rare, but the associated complications can be potentially serious for patients. Moreover, such injuries often lead to claims of malpractice and negligence requiring detailed medical investigation.

[Common and not so common nerve entrapment syndromes: diagnostics, clinical aspects and therapy].

Altogether, nerve entrapment syndromes have a very high incidence. Neurological deficits attributable to a focal peripheral nerve lesion lead to the clinical diagnosis. Frequently, pain is the dominant symptom but is often not confined to the nerve supply area.

Safety and feasibility of long term administration of recombinant human granulocyte-colony stimulating factor in patients with amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neuronal disease resulting in a loss of the upper and lower motor neurons and subsequent death within three to four years after diagnosis. Mouse models and preliminary human exposure data suggest that the treatment with granulocyte-colony stimulating factor (G-CSF) has neuro-protective effects and may delay ALS progression. As data on long-term administration of G-CSF in patients with normal bone marrow (BM) function are scarce, we initiated a compassionate use program including 6 ALS patients with monthly G-CSF treatment cycles.

Entrapment syndrome of multiple nerves in graft-versus-host disease.

Introduction: Peripheral nerve entrapment syndromes are associated with hereditary neuropathy with liability to pressure palsies and a variety of rheumatic and endocrinological diseases.

Methods: We report a patient with entrapment syndromes of multiple nerves associated with chronic graft-versus-host-disease (GVHD) after allogeneic hematopoietic stem cell transplantation. Nerve ultrasound, histology, and ultrastructural changes were assessed.

Muscle cramps and neuropathies in patients with allogeneic hematopoietic stem cell transplantation and graft-versus-host disease.

Objective: Graft-versus-host disease (GVHD) is an immune-mediated multisystemic disorder and the leading cause of morbidity after allogeneic hematopoietic stem cell transplantation. Peripheral nervous system manifestations of GVHD are rare but often disabling. Whereas immune-mediated neuropathies are an established feature of GVHD, muscle cramps are not well characterized.

Discharge rates in electromyography distinguish early between peripheral and central paresis.

Introduction: Abnormally increased discharge rates (DRs) of motor unit potentials on concentric needle electromyography (CNEMG) indicate a loss of motor units in peripheral neurogenic lesions.

Methods: To determine when increased DRs occur during the course of a peripheral nerve lesion, we retrospectively analyzed CNEMG recordings of 19 patients with acute weakness of peripheral origin.

Results: The initial CNEMG studies took place from 3.

Differentiation between uniform and non-uniform motor nerve conduction slowing.

Objective: Demyelination may cause a uniform reduction of the conduction velocity of all fibres of a peripheral nerve segment, or may affect only certain nerve fibres in a non-uniform way while sparing others. This study was done to improve the detection of non-uniform conduction slowing by using the high-frequency attenuation (HFA) method.

Methods: Nerve conduction data from patients with early inflammatory demyelinating neuropathy (non-uniform demyelination, n=20), hereditary neuropathy (uniform demyelination, n=9), motor neuron disease (axon loss, n=20), and from healthy control subjects (n=20) were analysed.