Inflammatory Neuropathy Consortium base (INCbase): a protocol of a global prospective observational cohort study for the development of a prediction model for treatment response in chronic inflammatory demyelinating polyneuropathy.

Background: INCbase is an international, multicenter prospective observational study using a customizable web-based modular registry to study the clinical, biological and electrophysiological variation and boundaries of chronic inflammatory demyelinating polyneuropathy (CIDP). The primary objective of INCbase is to develop and validate a clinical prediction model for treatment response.

Methods: All patients meeting clinical criteria for CIDP can be included in INCbase.

Standardized Tapering off Subcutaneous Immunoglobulin in Chronic Inflammatory Demyelinating Polyneuropathy.

Background: Attempting discontinuation of treatment in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) is recommended. However, there is no evidence based regimen for tapering off subcutaneous immunoglobulin (SCIG). This trial investigated stepwise tapering off SCIG to detect remission and the lowest effective dosage.

Changes in axonal and clinical function during intravenous and subcutaneous immunoglobulin therapy in chronic inflammatory demyelinating polyneuropathy.

Background And Aims: Intravenous immunoglobulin (IVIg) has a rapid clinical effect which cannot be explained by remyelination during each treatment cycle in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). This study aimed to investigate axonal membrane properties during the IVIg treatment cycle and their potential correlation with clinically relevant functional measurements.

Methods: Motor nerve excitability testing (NET) of the median nerve was performed before and 4 and 18 days after initiation of an IVIg treatment cycle in 13 treatment-naïve (early) CIDP patients and 24 CIDP patients with long term (late) IVIg treatment, 12 CIDP patients treated with subcutaneous immunoglobulin (SCIg) and 55 healthy controls.

Cystamine Treatment Fails to Prevent the Development of Pulmonary Hypertension in Chronic Hypoxic Rats.

Introduction: Pulmonary hypertension is characterized by vasoconstriction and remodeling of pulmonary arteries, leading to right ventricular hypertrophy and failure. We have previously found upregulation of transglutaminase 2 (TG2) in the right ventricle of chronic hypoxic rats. The hypothesis of the present study was that treatment with the transglutaminase inhibitor, cystamine, would inhibit the development of pulmonary arterial remodeling, pulmonary hypertension, and right ventricular hypertrophy.

Quality of life in chronic inflammatory demyelinating polyneuropathy patients treated with subcutaneous immunoglobulin.

Background: Subcutaneous immunoglobulin (SCIG) is effective treatment of chronic inflammatory demyelinating polyneuropathy (CIDP). Quality of life (QoL) increases following switch from intravenous administration to SCIG, but its correlation with clinical functioning is sparsely studied.

Aims Of The Study: The aim of this study is to evaluate the correlation between QoL and clinical functioning in CIDP patients treated with SCIG.

Pediatric Guillain-Barré Syndrome in a 30-Year Nationwide Cohort.

Background: Guillain-Barré syndrome is the most common cause of acute flaccid paresis in childhood. Few validated large-scale population-based data are available concerning pediatric Guillain-Barré syndrome, including incidence, risk factors, and initial clinical characteristics.

Methods: In the Danish National Patient Registry, we identified all children aged below 16 years (N = 212) diagnosed with Guillain-Barré syndrome and admitted to any Danish department of pediatrics between 1987 and 2016.

[Chronic inflammatory demyelinating polyneuropathy].

In this review, we discuss chronic inflammatory demyelinating polyneuropathy (CIDP), which is a disease with proximal and distal weakness and sensory disturbances resulting in impaired daily activity. The diagnosis is based on the clinical presentation and electrophysiology demonstrating demyelination in the peripheral nerves. CIDP can be successfully treated with immunoglobulin, glucocorticoids or plasma exchange, and during the latest decade, immunoglobulin has been administered subcutaneously improving patients' flexibility and autonomy.

Retrospective correlation analysis of plasma Immunoglobulin G and clinical performance in CIDP.

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) can be successfully treated with immunoglobulin either intravenously (IVIG) or subcutaneously (SCIG). Measurement of plasma immunoglobulin G levels (P-IgG) and its correlation to clinical improvement has shown conflicting results. This study aims to clarify whether changes in P-IgG are related to clinical development in patients with CIDP treated with IVIG or SCIG.

Neuromuscular electrical stimulation in early rehabilitation of Guillain-Barré syndrome: A pilot study.

Introduction: In Guillain-Barré syndrome (GBS), patients often develop muscle atrophy from denervation and immobilization. We, therefore, conducted a pilot study of neuromuscular electrical stimulation (NMES) to evaluate feasibility, safety, and effect on muscle wasting in the early phase of GBS.

Methods: Seventeen patients were randomized to receive 20 min of muscle fiber stimulation followed by 40 min of NMES of the right or left quadriceps muscle with the untreated side as control.

[Antibodies in patients with chronic inflammatory demyelinating polyneuropathy].

Recent studies have identified specific immunoglobulin (Ig) G4 antibodies against the paranodal proteins neurofascin 155 and contactin 1 in subgroups of patients with chronic inflammatory demyelinating polyneuropathy (CIDP). These patients present with distinct clinical phenotypes and poor response to first-line therapy with intravenous Ig. Detection of these antibodies in patients with CIDP has diagnostic, prognostic, and therapeutic implications.

Muscle Strength and Aerobic Capacity in Patients with CIDP One Year after Participation in an Exercise Trial.

Background: We have previously shown that patients with chronic inflammatory demyelinating polyneuropathy (CIDP) improve muscle strength and aerobic capacity after resistance and aerobic exercise.

Objective: The purpose of this study was to determine if muscle strength and aerobic capacity are preserved one year after discontinuation of regular exercise.

Methods: All patients in the previous exercise study were eligible for a one-year follow-up with measurement of combined isokinetic muscle strength (cIKS) by dynamometry and maximal oxygen consumption velocity (VO2-max).

Does grip strength reflect isokinetic muscle strength in lower limbs in patients with chronic inflammatory demyelinating polyneuropathy?

Introduction: Grip strength (GS) is a common measure of general muscle strength in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). However, it is important to investigate the correlation and responsiveness of GS compared with isokinetic muscle strength (IKS) and function of the lower limbs.

Methods: Seventy patients with CIDP were evaluated with GS, IKS, and functional measures of the lower limbs.