[Non-inflammatory muscle pain]. / Nichtentzündliche Muskelschmerzen.

Muscle pain as a common symptom in daily practice frequently occurs as a non-specific accompanying symptom in multiple internal and neurological diseases. Primarily inflammatory or autoimmune muscular diseases are causing muscle pain. However, a number of non-inflammatory causes of pain can also be considered for differential diagnosis. These are presented in this article. In principle, a distinction must be made between focal and diffuse muscle pain. As an invasive diagnostic procedure, a muscle biopsy should only be performed as the last step in the diagnostic alogorithm. If diffuse muscle pain is only associated with slight muscle weakness or is completely absent, there is usually a primary rheumatic cause. Statins (HMG-CoA reductase inhibitors) can lead to rhabdomyolysis, muscle fiber atrophy and muscle necrosis by damaging the muscle fiber membrane. Myotonias are autosomal dominant or autosomal recessive inherited disorders of muscle function. The genetic defect leads to pronounced muscle stiffness. The cause of metabolic myopathies can be disorders of the carbohydrate, fat or purine metabolism. Fibromyalgia syndrome (FMS) is a non-inflammatory disease and, according to the current knowledge, recognized as the result of an exposure to physical, biological and psychosocial factors (biopsychological disease model). To help diagnosing FMS, pain regions and core symptoms (fatigue, sleep disturbances) can be detected using questionnaires (Widespread Pain Index [WPI] and Symptom Severity Scale [SSS]).

[Idiopathic Inflammatory Myopathies]. / Idiopathische inflammatorische Myopathien.

Myositis is a rare and an extremely heterogeneous autoimmune disease, that causes muscle weakness. Currently, "idiopathic inflammatory myopathies (IIM)" is the preferred umbrella-term used to describe the disease complexity within individuals. IIM include dermatomyositis, polymyositis, inclusion body myositis, autoimmune necrotizing myopathy, overlap myositis and antisynthetase syndrome. Research activity concerning myositis was very intense over the past ten years and led to new diagnostic approach as well as to novel therapeutic strategies. Correct classification is the key for successful management. One single treatment regime for every possible organ involvement in all different forms of IIM is still not existing.

McArdle Sign: A Specific Sign of Multiple Sclerosis.

OBJECTIVE: To measure McArdle sign (rapidly reversible weakness induced by neck flexion) both qualitatively and quantitatively and to evaluate its specificity and clinical utility for diagnosis of multiple sclerosis (MS). PATIENTS AND METHODS: In this prospective study, McArdle sign was evaluated by a technician blinded to diagnosis by measuring changes in finger extensor strength in successive trials of neck extension and flexion, first clinically and then with a torque measurement device. We studied 25 healthy controls and 81 patients with finger extensor weakness. Patients were not selected for having McArdle sign. Fifty-two patients had MS, 24 had other myelopathies, and 5 had peripheral nerve lesions accounting for their weakness. The study was conducted between February 1, 2016, and June 30, 2017. RESULTS: The median clinical McArdle sign and the 2 quantitative measures of neck flexion-induced strength reduction were greater in patients with MS than in the other groups (P<.001). Baseline strength did not confound the difference. The area under the receiver operating characteristic curve was 0.84 (95% CI, 0.75-0.93) comparing patients with MS vs healthy controls and 0.84 (95% CI, 0.75-0.93) comparing MS vs patients with other myelopathies. The 2 quantitative and 1 clinical measurement of McArdle sign by the technician who performed the quantitative testing were correlated (r=.57 and r=.58; P<.001), and in turn, the technician's and unblinded referring physician's clinical assessments were correlated (r=.58; P<.001). McArdle sign was evident in some patients who had minor disability and who were in early phases of MS. CONCLUSION: McArdle sign, when defined as greater than 10% neck flexion-induced reduction in strength, is entirely specific and 65% sensitive for a diagnosis of MS when compared with other conditions that mimic MS-associated myelopathy. It may facilitate diagnosis in certain clinical situations. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT03122873.

ICU-acquired weakness: should medical sovereignty belong to any specialist?

ICU-acquired weakness (ICUAW), including critical illness polyneuropathy, critical illness myopathy, and critical illness polyneuropathy and myopathy, is a frequent disabling disorder in ICU subjects. Research has predominantly been performed by intensivists, whose efforts have permitted the diagnosis of ICUAW early during an ICU stay and understanding of several of the pathophysiological and clinical aspects of this disorder. Despite important progress, the therapeutic strategies are unsatisfactory and issues such as functional outcomes and long-term recovery remain unclear. Studies involving multiple specialists should be planned to better differentiate the ICUAW types and provide proper functional outcome measures and follow-up. A more strict collaboration among specialists interested in ICUAW, in particular physiatrists, is desirable to plan proper care pathways after ICU discharge and to better meet the health needs of subjects with ICUAW.

Coding in Muscle Disease.

Accurate coding is critically important for clinical practice and research. Ongoing changes to diagnostic and billing codes require the clinician to stay abreast of coding updates. Payment for health care services, data sets for health services research, and reporting for medical quality improvement all require accurate administrative coding. This article provides an overview of administrative coding for patients with muscle disease and includes a case-based review of diagnostic and Evaluation and Management (E/M) coding principles in patients with myopathy. Procedural coding for electrodiagnostic studies and neuromuscular ultrasound is also reviewed.

Prevalence and definition of sarcopenia in community dwelling older people. Data from the Berlin aging study II (BASE-II).

BACKGROUND: Sarcopenia describes the age-associated loss of muscle mass, strength and function. The aim of this study was to compare the prevalence of sarcopenia in a cohort of community dwelling elderly people living in Berlin, Germany, according to the criteria proposed by current consensus statements and to study the respective impact on self-reported physical performance. MATERIAL AND METHODS: This study included 1405 participants from the Berlin aging study II (BASE-II). The appendicular skeletal muscle mass index (SMI) was assessed with dual energy X-ray absorptiometry (DXA), muscle strength was measured by hand grip strength and the timed up and go" test (TUG) was performed as a functional parameter to reflect mobility. RESULTS: The prevalence of sarcopenia was 24.3 % in terms of reduced SMI only and considerably lower for sarcopenia with reduced grip strength (4.1 %) and sarcopenia with limited mobility (2.4 %). Only 0.6 % of the participants fulfilled all three criteria. Of the subjects with a normal SMI, 8.6 % had reduced grip strength and 5.1 % had limited mobility, whereas 1.3 % subjects fulfilled both criteria. Participants with reduced strength or function reported severe difficulties in performing physical tasks significantly more often than participants with normal or reduced SMI alone (p <0.029-p <0.0001). CONCLUSION: In BASE-II low skeletal muscle mass was much more frequent than reduced grip strength or poor function. Reduced strength and function were found to be associated with a greater impact on physical performance than reduced muscle mass. Low SMI does not seem to be a prerequisite for low strength or limitations in mobility.

A step backward: the 'Rough' facial nerve grading system.

OBJECTIVES: Several modalities currently exist to rate the degree of facial function clinically but even though it has significant limitations, the most widely used scale is the House-Brackmann grading system (HBGS). A simplified scale is introduced here, the 'Rough' Grading System (RGS - Grade I: normal movement; Grade II: slight paralysis; Grade III: frank paralysis with eye closure; Grade IV: frank paralysis without eye closure; Grade V: almost complete paralysis with only slight movements; Grade VI: total paralysis). The aim of the present study was to verify the interrater reliability and the interscale validity of this simplified grading system. STUDY DESIGN: Scale validation study based on a prospective cohort. METHODS: Fifty patients with facial palsy, consecutively referred to our department were filmed while performing some codified facial movements. Then two independent groups (one rating using the HBGS, the other rating using the RGS) assigned a grade after reviewing the videos. The time required for the rating was also noted. RESULTS: The HBGS showed a mean value of interrater agreement of 0.46 while the RGS showed a mean value of 0.59. The concurrent validity between HBGS and RGS ranged from 0.86 to 0.90 (p < 0.001 for every comparison). There was no statistically significant difference between HBGS and RGS in the mean time taken for rating (p = 0.15). CONCLUSIONS: The RGS reached an adequate level of interrater reliability, higher than the HBGS. The correlation between the two scales is high and the times required for rating are similar. The present results may justify the use of the RGS in routine clinical practice. LEVEL OF EVIDENCE: N/A.

Using the Short Physical Performance Battery to screen for frailty in young-old adults with distinct socioeconomic conditions.

AIM: To analyze the Short Physical Performance Battery's (SPPB) ability in screening for frailty in community-dwelling young elderly from cities with distinct socioeconomic conditions. METHODS: Elderly (65-74 years-of-age) from Canada (Saint Bruno; n = 60) and Brazil (Santa Cruz; n = 64) were evaluated with the SPPB to assess physical performance. Frailty was defined as the presence of ≥ 3 of the following criteria: weight loss, exhaustion, weakness, mobility limitation and low physical activity. Linear regression and receiver operating characteristics analyses were carried out. RESULTS: The SPPB correlated with frailty (R(2) = 0.33), with better results for Saint Bruno. A cut-off of 9 in the SPPB had good sensitivity (92%) and specificity (80%) in discriminating frail from non-frail in Saint Bruno (area under the curve [AUC] = 0.81), but showed fair results in Santa Cruz (AUC = 0.61, sensitivity = 81% and specificity = 52%). CONCLUSIONS: The SPPB better discriminated frailty in elderly with higher socioeconomic conditions (Saint Bruno).

Classification of cause of motor weakness in traumatic brain injury using diffusion tensor imaging.

BACKGROUND: Many studies have attempted to elucidate the causes of motor weakness in patients with traumatic brain injury (TBI). Most of these studies have focused on the specific cause of motor weakness. However, little is known about the classification and elucidation of the causes of motor weakness in consecutive patients with TBI. OBJECTIVE: To attempt to classify with diffusion tensor imaging the causes of motor weakness in patients with TBI by conducting an analysis of the injury mechanism of the corticospinal tract (CST). DESIGN: Retrospective study. SETTING: Rehabilitation department of a university hospital. Patients  We recruited 41 consecutive patients who showed motor weakness among patients with TBI admitted for rehabilitation. MAIN OUTCOME MEASURES: We classified the causes of weakness according to the injury mechanism of the CST on diffusion tensor imaging. RESULTS: Injury mechanisms of the CST were classified as follows, in order: diffuse axonal injury, 24 patients (58.5%); traumatic intracerebral hemorrhage, 9 patients (21.9%); transtentorial herniation, 6 patients (14.6%); and focal cortical contusion, 4 patients (9.8%). In patients with diffuse axonal injury, the mean number of lesions composing CST injury was 3.6 (range, 2-6) and CST injury locations were as follows: the pons (61%), the cerebral peduncle (50%), the medulla (40%), the posterior limb of the internal capsule (17%), and the corona radiata (13%). CONCLUSION: We found that diffusion tensor imaging was useful in elucidation and classification of the causes of motor weakness resulting from CST injury in patients with TBI.

Optimal scoring methods of hand-strength tests in patients with stroke.

The purpose of this study was to determine the optimal scoring methods for measuring strength of the more-affected hand in patients with stroke by examining the effect of reducing measurement errors. Three hand-strength tests of grip, palmar pinch, and lateral pinch were administered at two sessions in 56 patients with stroke. Five scoring methods were used to present the strength scores. The smallest real difference was used to provide information on the measurement error. The smallest real difference percentage was used to compare the effect on minimizing the error. Using mean score of tests for nonspastic patients carried out at least twice was found to be advisable to minimize measurement errors in the grip, palmar pinch, and lateral pinch tests. However, the use of hand-strength tests for patients with spasticity is limited because of the relatively high measurement errors.

Guillain-Barre syndrome: a clinical study in King Chulalongkorn Memorial Hospital.

BACKGROUND: Guillain-Barre Syndrome (GBS) is an acute,fatal, but treatable polyradiculopathy. Clinical data concerning this entity is scarce in Thailand The purpose of the present study was to describe clinical profiles and management of GBS as well as to determine prognostic factors in GBS. MATERIAL AND METHOD: Clinical data of GBS in King Chulalongkorn Memorial Hospital during 2002-2007 were searched by using in-patients hospital database. Asbury and Cornblath's criteria were applied for the diagnosis of GBS. Clinical data, electrophysiological data, management, and clinical prognostic factors were collected and analyzed by SPSS version 16. RESULTS: Fifty-five patients with GBS were recruited, 26 wire male and 29 were female. Mean age was 43 +/- 17 years. History of antecedent infection included: respiratory tract 29%, gastrointestinal tract 7%, ear 2%, and non-specific infection 14%. Initial presentations were limb weakness 87%, limb numbness 78%, bulbar weakness 31%, and facial weakness 18%. Electrodiagnostic study revealed demyelinating process in 54% and axonopathy in 46%. Twenty-nine patients received intravenous immunoglobulin while 13 patients underwent plasmapheresis. Clinical outcomes were satisfactory in most of the patients and only two patients died from sepsis and pneumonia. On discharge, the status of the patients were Hughes grade 1-4 in 73%, 14%, 5%, and 4% respectively. Bulbar paresis as the presenting symptom was the only clinical prognostic factor that significantly determined airway compromised and subsequently respiratory failure. CONCLUSION: Clinical features of GBS in the present series were characterized by generalized muscle weakness with mild numbness in combination with facial and bulbar paresis in nearly half of patients. Respiratory failure was encountered in 9% of cases. Clinical outcomes were satisfactory in most of the patients with or without specific treatment. The most significant predictor for adverse clinical course was the bulbar paresis as a presenting symptom and patients who presented with less disability score had a better recovery.

[Congenital and other myopathies]. / Kongenitale und andere Myopathien.

The myopathies presented here fall into two groups: Congenital myopathies and protein aggregate myopathies. These genetic conditions often require all modern diagnostic investigations, including histology, enzyme histochemistry, immunohistochemistry and electron microscopy to pave the way to an adequate individual molecular analysis and diagnosis. This is necessary to provide the patient and his or her family information about disease-characteristics or even disease-specific features. Distal myopathies, although caused by mutations in different genes, and toxic myopathies as acquired neuromuscular conditions largely provide non-specific morphological features a correct nosological interpretation of which only succeeds with additional non-morphological data.

Development of Korean Academy of Medical Sciences Guideline rating the physical impairment: lower extremities.

Lower Extremities Committee of Korean Academy of Medical Sciences Guideline for Impairment Rating develops new guidelines which are based on McBride method, American Medical Association Guides, Disability evaluation by The Korean Orthopaedic Association, The Korean Neurosurgery Society, and Korean Academy of Rehabilitation Medicine. The committee analyzed and discussed to create an ideal method practical in Korea. Our committee endeavors to develop new methods which are easy to use, but are suitable for professional use and also independent from the examinee's intentions. The lower extremities are evaluated on the basis of anatomic change, functional change, and diagnosis based evaluation. Nine methods are used to assess the lower extremities. Anatomic assessment includes leg length discrepancy, ankylosis, amputation, skin loss, peripheral nerve injury, and vascular disease. In functional assessment, range of motion and muscle strength are included. Diagnosis-based assessments are used to evaluate impairment caused by specific fractures, deformities, ligament instability, meniscectomies, post-traumatic arthritis, fusion of the foot, and lower extremity joint replacements.

A framework for diagnosing and classifying intensive care unit-acquired weakness.

Neuromuscular dysfunction is prevalent in critically ill patients, is associated with worse short-term outcomes, and is a determinant of long-term disability in intensive care unit survivors. Diagnosis is made with the help of clinical, electrophysiological, and morphological observations; however, the lack of a consistent nomenclature remains a barrier to research. We propose a simple framework for diagnosing and classifying neuromuscular disorders acquired in critical illness.

Development of Korean Academy of Medical Sciences Guideline Rating the Physical Impairment: Lower Extremities

Lower Extremities Committee of Korean Academy of Medical Sciences Guideline for Impairment Rating develops new guidelines which are based on McBride method, American Medical Association Guides, Disability evaluation by The Korean Orthopaedic Association, The Korean Neurosurgery Society, and Korean Academy of Rehabilitation Medicine. The committee analyzed and discussed to create an ideal method practical in Korea. Our committee endeavors to develop new methods which are easy to use, but are suitable for professional use and also independent from the examinee's intentions. The lower extremities are evaluated on the basis of anatomic change, functional change, and diagnosis based evaluation. Nine methods are used to assess the lower extremities. Anatomic assessment includes leg length discrepancy, ankylosis, amputation, skin loss, peripheral nerve injury, and vascular disease. In functional assessment, range of motion and muscle strength are included. Diagnosis-based assessments are used to evaluate impairment caused by specific fractures, deformities, ligament instability, meniscectomies, post-traumatic arthritis, fusion of the foot, and lower extremity joint replacements.

Weakness is the primary contributor to finger impairment in chronic stroke.

OBJECTIVE: To assess the relative contributions of several neurologic and biomechanic impairment mechanisms to overall finger and hand impairment in chronic hemiparetic stroke survivors. DESIGN: Repeated-measures design. SETTING: Clinical research laboratory. PARTICIPANTS: Thirty stroke survivors with chronic hemiparesis. Fifteen subjects had severe hand motor impairment and 15 had moderate impairment, as measured with the Chedoke-McMaster Stroke Assessment. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: The biomechanic factors stiffness and resting flexion torque, together with the neurologic factors spasticity, strength, and coactivation, were quantified by using a custom hand manipulator, a dynamometer, and electromyographic recordings. Both passive and active rotations of the metacarpophalangeal joints of the fingers were examined. RESULTS: Although subjects in the severely impaired group exhibited statistically greater passive stiffness and resting flexion torque than their moderately impaired counterparts (P<.05), the overall effect of these biomechanic changes appeared small in relation to the deficits attributable to neurologic changes such as spasticity and, especially, weakness. In fact, weakness in grip strength and isometric extension accounted for the greatest portion of the variance between the 2 groups (eta(2)=.40 and eta(2)=.23, respectively). CONCLUSIONS: Thus, deficits in hand motor control after stroke seem to derive mainly from weakness, which may be attributable to the loss of descending corticospinal pathway activation of motoneurons.

Quantifying shoulder rotation weakness in patients with shoulder impingement.

The purpose of this study was to determine whether strength deficits could be detected in individuals with and without shoulder impingement, all of whom had normal shoulder strength bilaterally according to grading of manual muscle testing. Strength of the internal rotators and external rotators was tested isokinetically at 60 degrees /s and 180 degrees /s, as well as manually with a handheld dynamometer (HHD) in 17 patients and 22 control subjects. Testing was performed with the shoulder positioned in the scapular plane and in 90 degrees of shoulder abduction with 90 degrees of elbow flexion (90-90). The peak torque was determined for each movement. The strength deficit between the involved and uninvolved arms (patients) and the dominant and nondominant arms (control subjects) was calculated for each subject. Comparisons were made for the scapular-plane and 90-90 positions between isokinetic and HHD testing. Despite a normal muscle grade, patients had marked weakness (28% deficit, P < .01) in external rotators at the 90-90 position tested with the HHD. In contrast, external rotator weakness was not evident with isokinetic testing at the 90-90 position (60 degrees /s and 180 degrees /s, 0% deficit, P = .99). In control subjects, greater internal rotator strength in the dominant compared with the nondominant arm was evident with the HHD at the 90-90 position (11%, P < .01) and in the scapular plane (7%, P < .05). Using an HHD while performing manual muscle testing can quantify shoulder strength deficits that may not be apparent with isokinetic testing. By using an HHD during shoulder testing, clinicians can identify weakness that may have been presumed normal.