Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain‐Barré syndrome

Kleyweg, R. P.; Meché, F.G.A. van der; Schmitz, Paul

doi:10.1002/mus.880141111

Cited by 787 publications

(585 citation statements)

References 8 publications

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“…Muscle strength can be quantified using the Medical Research Council (MRC) sum score, a validated ordinal scale which grades muscle weakness in six bilateral muscle groups from upper and lower limbs between 0 (no contraction) and 5 (normal strength) (TABLE 3). Adding these subscores results in a maximum sum score of 60 (369). ICUAW was defined as MRC sum score for muscle strength to be Ͻ48, provided no other cause of weakness could be identified (147).…”

Section: B Aspects Of Clinical Evaluation and Diagnosticsmentioning

confidence: 99%

The Sick and the Weak: Neuropathies/Myopathies in the Critically Ill

Friedrich

Reid

Berghe

et al. 2015

Physiological Reviews

300

329

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Critical illness polyneuropathies (CIP) and myopathies (CIM) are common complications of critical illness. Several weakness syndromes are summarized under the term intensive care unit-acquired weakness (ICUAW). We propose a classification of different ICUAW forms (CIM, CIP, sepsis-induced, steroid-denervation myopathy) and pathophysiological mechanisms from clinical and animal model data. Triggers include sepsis, mechanical ventilation, muscle unloading, steroid treatment, or denervation. Some ICUAW forms require stringent diagnostic features; CIM is marked by membrane hypoexcitability, severe atrophy, preferential myosin loss, ultrastructural alterations, and inadequate autophagy activation while myopathies in pure sepsis do not reproduce marked myosin loss. Reduced membrane excitability results from depolarization and ion channel dysfunction. Mitochondrial dysfunction contributes to energy-dependent processes. Ubiquitin proteasome and calpain activation trigger muscle proteolysis and atrophy while protein synthesis is impaired. Myosin loss is more pronounced than actin loss in CIM. Protein quality control is altered by inadequate autophagy. Ca 2ϩ dysregulation is present through altered Ca 2ϩ homeostasis. We highlight clinical hallmarks, trigger factors, and potential mechanisms from human studies and animal models that allow separation of risk factors that may trigger distinct mechanisms contributing to weakness. During critical illness, altered inflammatory (cytokines) and metabolic pathways deteriorate muscle function. ICUAW prevention/treatment is limited, e.g., tight glycemic control, delaying nutrition, and early mobilization. Future challenges include identification of primary/secondary events during the time course of critical illness, the interplay between membrane excitability, bioenergetic failure and differential proteolysis, and finding new therapeutic targets by help of tailored animal models.

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Section: B Aspects Of Clinical Evaluation and Diagnosticsmentioning

confidence: 99%

The Sick and the Weak: Neuropathies/Myopathies in the Critically Ill

Friedrich

Reid

Berghe

et al. 2015

Physiological Reviews

300

329

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“…Information on MMN was collected using the protocol of Cats and colleagues as a template [Cats et al 2010 [Kleyweg et al 1991]. The following muscles were assessed on both sides: upper arm abductors, elbow flexors, wrist extensors, hip flexors, knee extensors and foot dorsal flexors.…”

Section: Patients and Parametersmentioning

confidence: 99%

Treatment of patients with multifocal motor neuropathy with immunoglobulins in clinical practice: the SIGNS registry

Stangel

Gold

Pittrow

et al. 2016

Ther Adv Neurol Disord

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Objectives:The management of patients with multifocal motor neuropathy (MMN) under everyday clinical conditions has been insufficiently studied. We therefore collected comprehensive observational data on patients with MMN who received intravenous (IV) or subcutaneous (SC) immunoglobulins (IGs) as maintenance therapy. Methods: This was a prospective, noninterventional study (registry) in neurological centres (hospitals and offices) throughout Germany. Results: As of 1 December 2015, 80 patients with MMN were included (mean age 55.4 ± 9.8 years, 67% males, mean disease duration 10.7 ± 10.2 years). The affected limb regions were predominantly distal muscle groups of the upper extremities. On the inflammatory neuropathy cause and treatment (INCAT) scale, 94% of the patients had some disability in the arms and 61% in the legs. At inclusion, 98.8% received IVIG and 1.3% SCIG. Substantial variation was observed between IVIG treatment intervals (every 0.7 to 17.3 weeks) and dosage (0.2-2.1 g/kg body weight received during a single administration; mean monthly dosage, 0.9 g/kg body weight). However, the mean monthly dosage was steady over time. At 1-year follow up, improvement was seen in muscle strength, INCAT and quality of life (QoL) scores (SF-36 questionnaire). Conclusions:The management of patients with MMN in everyday clinical practice demonstrates a wide range of absolute dosages and treatment intervals of IG, supporting the recommended practice of determining treatment dose on an individual patient basis. The improvements in muscle strength and reduction in disability, accompanied by increased QoL, strengthen the case for use of IG as a maintenance treatment for MMN.

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“…6 Medical Research Council (MRC) sum score was used for valuing the muscle strength from 0 to 5 in proxi- mal and distal muscles in upper and lower limbs bilaterally; score ranged from 40 (normal) to 0 (quadriplegic) and by Hughes et al disability score for GBS. 7,8 We further subdivided Hughes grade 5 to 5A and 5B based on requirement of ventilator. (Table 1).…”

Section: Methodsmentioning

confidence: 99%

Guillain-Barré syndrome: Clinical profile and Consensus to revise Hughes grade 5

Manorenj¹,

Inturi²,

Bhargava³

et al. 2016

IJMEDPH

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Background: Guillain-Barré Syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy and an important cause of acute flaccid paralysis (AFP) worldwide. Respiratory insufficiency requiring ventilator occurs in 30% of patients that prolong the hospital stay, leading to morbidity and mortality. There had been relatively few studies of Guillain-Barre syndrome in adults from South India. Aim: To evaluate clinical profile, epidemiological, laboratory, and electro diagnostic features of patients with GBS in adults. Settings and design: A prospective study was conducted over a period of 4 years at ESIC Superspeciality Hospital, Hyderabad. Materials and methods: Total 36 patients were identified and data was collected. We studied the epidemiological, clinical, electrophysiological features and their outcome. We subdivided Hughes grade 5 into 5A and 5B based on the requirement of ventilator. Statistical Analysis: Data obtained in the study were subjected to statistical analysis with Statistical Package for Social Sciences (SPSS) version 18. Bivariate analysis was done using chi-square test. Results: Of 36 GBS patients, 21(58.3%) were males; the mean age was 35 years. Antecedent infection was found in 23(63.8%). Majority 12 (33%) were in Hughes grade 4, 10 (27.7%) were in Hughes grade 5A. 97.2% had limb weakness. A significant association was found between low Medical research sum score (MRC) and respiratory failure. Most predominant neurophysiological variant was acute inflammatory demyelinating polyradiculoneuropathy (AIDP) 12 (33.3%). Duration of illness was less than 1week in 19 (52.7%) of cases. Asymmetry was observed in 5 (13.8%) and recurrence of Guillain-Barre Syndrome seen in 2 (5.5%) cases. Complete recovery was noted at 6 months in 34 (94.4%) cases. Conclusion: Early diagnosis of respiratory failure and prompt intervention improves patient outcome. Further large sample studies are required to assess respiratory failure and subdivision of Hughes grade 5.

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Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain‐Barré syndrome

Cited by 787 publications

References 8 publications

The Sick and the Weak: Neuropathies/Myopathies in the Critically Ill

The Sick and the Weak: Neuropathies/Myopathies in the Critically Ill

Treatment of patients with multifocal motor neuropathy with immunoglobulins in clinical practice: the SIGNS registry

Guillain-Barré syndrome: Clinical profile and Consensus to revise Hughes grade 5

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