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What's in the Literature?

Silvestri, Nicholas J. MD; Wolfe, Gil I. MD; Bromberg, Mark MD, PhD; Lacomis, David MD

Journal of Clinical Neuromuscular Disease: March 2016 - Volume 17 - Issue 3 - p 146–157
doi: 10.1097/CND.0000000000000111
Literature Review

In this issue, we review clinical features associated with an elevated serum creatine kinase level found in a percentage of patients with amyotrophic lateral sclerosis (ALS). The treatment of ALS remains problematic, and issues with offerings on the internet for unregulated stem cell treatment and the movement for right-to-try experimental drugs are discussed. The last What's in the Literature? discussed information about recording from stimulating electrodes implanted in the diaphragm of ALS patients, and this issue discusses the results of a randomized trial using diaphragm pacing. Chronic inflammatory demyelinating polyradiculoneuropathy is important to diagnose, as it is one of the few treatable chronic neuropathies. However, the diagnosis can be challenging to make, and a review of diagnostic errors is discussed. Once diagnosed, treatment management can be difficult, and endpoint measures are discussed. Updates on the use of mycophenolate mofetil and cyclophosphamide in the treatment of myasthenia gravis are reviewed, as they are the use of cell-based assays for detecting antibodies to clustered acetylcholine receptors. Factors associated with life-threatening events in myasthenia, ethnic differences in the course of the disease, and treatment in very elderly patients are also discussed. Hematopoietic stem cell transplantation as a treatment of mitochondrial neurogastrointestinal encephalomyopathy is reviewed. A natural history study in boys with Duchenne muscular dystrophy looking at the age of loss of ambulation and side effect profiles associated with corticosteroid treatments is discussed, as it is a comprehensive study looking at the clinical features and treatment of necrotizing autoimmune myopathy.

*Department of Neurology, University at Buffalo School of Medicine and Biomedical Sciences, Buffalo, NY;

Department of Neurology, University of Utah School of Medicine, Salt Lake City, UT; and

Departments of Neurology and Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA.


The authors report no conflicts of interest.

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