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. Cross-sectional area (CSA) of the muscle measured by ultrasound and isometric knee extensor strength were the primary and secondary outcome measures. RESULTS: No treatment related adverse effects were recorded. Change in CSA was -0.25 cm2 (confidence interval [CI], -0.93-0.42) on the stimulated side versus -0.60 cm2 (CI, -1.32-0.11) on the nonstimulated side (P = 0.08). No effect was observed on muscle strength. CONCLUSIONS: NMES seems safe and feasible in the early phase of GBS. Further studies are needed to explore effect on muscle function. Muscle Nerve 59:481-484, 2019.

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. A total of 145 (68%) medical files could be retrieved and reviewed, enabling classification of patients with true Guillain-Barré syndrome. The nationwide Guillain-Barré syndrome incidence rate was calculated and stratified by age, gender, time periods, and season. Risk factors and initial Guillain-Barré syndrome characteristics were assessed by medical record review. RESULTS: The positive predictive value of Guillain-Barré syndrome diagnosis codes was 86%. The crude Guillain-Barré syndrome incidence rate was 0.69 per 100,000 person years and peaked at two years of age. The incidence rate was higher among men (0.80) than women (0.58) and was relatively stable over the 30-year period. No seasonal difference of the incidence rate was found. Of the 125 Guillain-Barré syndrome cases, 63% were preceded by infection, whereas none were preceded by surgery or malignant disease. Medically treated pain was documented in 70%, mainly confined to the lower extremities. CONCLUSIONS: Pediatric Guillain-Barré syndrome diagnoses in the Danish National Patient Registry have high validity, the incidence peaks at age two years, and is preceded by infection in two-thirds of children. Lower extremity pain is a common clinical presentation in the acute setting.

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. METHODS: Patients from five previous studies treated with either IVIG or SCIG with evaluation at baseline and re-evaluation after two or 10/12 weeks, respectively were included. At evaluation and re-evaluation, the following tests were done: combined isokinetic muscle strength (cIKS), grip strength, 9-hole-peg test (9-HPT), 40-meter-walk test (40-MWT), clinical examination of muscle strength score by the Medical Research Council (MRC) and measurement of plasma immunoglobulin G (P-IgG). RESULTS: Fifty-five patients were included in the IVIG group and 41 in the SCIG group. There was no correlation between the changes in P-IgG and cIKS in neither the IVIG group (r = 0.137, p = 0.32) nor the SCIG group (r =  - 0.048, p = 0.77). Similarly, no correlations could be demonstrated between P-IgG and grip strength, 9-HPT, 40-MWT or MRC. CONCLUSIONS: In patients with CIDP receiving SCIG or IVIG, changes in P-IgG during treatment did not correlate with changes in muscle strength or other motor performance skills.

[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. By time, 30% of the patients will remit, and maintenance treatment will no longer be necessary.

[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. This review summarises the current knowledge on clinical characteristics, pathogenesis and treatment of these patients.