We examined the clinical effectiveness of rituximab in fourteen patients with refractory myasthenia gravis (MG). Manual muscle testing (MMT) score was recorded at baseline and followed during the course of the study. Steroid dose, frequency of intravenous immunoglobulin (IVIG) infusions, and plasma exchange (PLEX) were also monitored throughout the duration of the study. All patients responded dramatically to rituximab, as measured by a change in MMT score, prednisone dose, or the frequency of IVIG infusions or PLEX. Rituximab appears safe and effective for the treatment of refractory MG. It should be considered as a therapeutic option in refractory patients.

Although evidence has emerged regarding functional neural impairment of all four limbs with a diagnosis of type II diabetes (T2D), there is conflicting evidence regarding impairment in manual function with the disease. The purpose of the current study was to evaluate hand/fingertip function in T2D as compared to healthy age- and gender-matched controls. Ten adults with T2D and ten healthy age- and gender-matched control subjects underwent a battery of clinically validated and laboratory-based evaluations of sensory function, motor function, and quality of life evaluation. The T2D group exhibited sensory dysfunction and altered kinetic output and inconsistent differences in clinically-validated timed performance tasks as compared to age-matched controls. No difference in quality of life was found between the two groups. Sensory dysfunction and some timed evaluations correlated with disease severity. Linear kinetic features did not covary with diminished sensation; however, nonlinear measures did covary with sensation changes. None of the recorded measures were related to clinical diagnosis of peripheral neuropathy. The relationship among exhibited behavioral changes is discussed in terms of small fiber neuropathy, micro-vascular adaptations, and endothelial dysfunction co-occurring with T2D.

Objective-To examine the relations between demographic factors, specific psychosocial factors, and cardiac rehabilitation attendance. Design-Cohort, repeated measures design. Setting-A large tertiary care centre in western Canada Patients-304 consecutive consenting patients discharged following acute myocardial infarction and/or coronary artery bypass graft surgery. Main outcome measures-The Jenkins self-eYcacy expectation scales and activity checklists of behaviour performance for maintaining health and role resumption, modified version of the selfmotivation inventory, and the shortened social support scale. Results-Those who had higher role resumption behaviour performance scores at two weeks after discharge were significantly less likely to attend cardiac rehabilitation programmes. At six months after discharge, those who attended cardiac rehabilitation demonstrated higher health maintenance self-eYcacy expectation and behaviour performance scores. Health maintenance self-eYcacy expectation and behaviour performance improved over time. Women reported less social support but showed greater improvement in health maintenance self-eYcacy expectation. Changes in self-eYcacy scores were unrelated to-but changes in health maintenance behaviour performance scores were strongly associated with-cardiac rehabilitation attendance. Conclusions-Cardiac patients and practitioners may have misconceptions about the mandate and potential benefits of rehabilitation programmes. Patients who resumed role related activities early and more completely apparently did not see the need to "rehabilitate" while those who attended cardiac rehabilitation programmes enhanced their secondary prevention behaviours. (Heart 2001;85:290-294)

Myasthenia gravis is a nerve-muscle junction disease, for which the most specific test is an increase in the anti-acetylcholine receptor antibodies (anti-AChR-Abs) titer. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting both upper and lower motor neurons. Positive AChR-Ab in patients with pure ALS are exceedingly rare. We report the case of a patient with confirmed ALS and very high levels of AChR-Ab and review the literature on this topic.

Background Patients with muscular dystrophy ( MD ) represent a vulnerable patient population with no clearly defined care model in modern‐day clinical practice to manage a high burden of heart disease and comorbidities. We demonstrate the effectiveness of cardiac interventions, namely the initiation and optimization of medical and device therapies, as part of a multidisciplinary care approach to improve clinical outcomes in patients with MD . Methods and Results We conducted a prospective cohort study at the Neuromuscular Multidisciplinary clinic following patients with dystrophinopathies, limb‐girdle MD , type 1 myotonic dystrophy, and facioscapulohumeral MD . A negative control group classified as non‐ MD myopathies without heart disease, was also tracked. Our cohort of 185 patients (median age: 42 years; 79 [42.7%] women), included 145 patients with MD . Cardiomyopathy was present in 65.6% of the patients with dystrophinopathies (21 of 32) and 27.3% of the patients with limb‐girdle MD (9 of 33). Conduction abnormalities were common in type 1 myotonic dystrophy (33.3% [20/60] patients). Cardiac intervention reversed systolic dysfunction, with left ventricular ejection fraction improving from 43% to 50.0% over a 3‐year period. A sustained reduction in healthcare utilization was also observed. The number of outpatient clinic visits decreased from 3.0 to 1.5 visits per year, the duration of hospitalizations was reduced from 14.2 to 0.9 days per year, and the number of cardiac‐related hospitalizations decreased from 0.4 to 0.1 hospitalizations per year associated with low mortality. Conclusions Our study demonstrates that cardiac intervention as part of a comprehensive multidisciplinary care approach to treating patients with MD leads to a sustained improvement in clinical outcomes.