Journal of Clinical Neuromuscular Disease 
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This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.What is in the Myopathy Literature?
This update covers the results of a randomized, placebo-controlled study that provides evidence that lamotrigine is effective in treating nondystrophic myotonias. Next, an overview of adverse effects of immune checkpoint inhibitors is provided, and the association of autoimmune myopathy and these monoclonal antibody therapies is discussed in light of recent reports. Last, the utility of electrodiagnostic testing in patients with intensive care unit weakness is addressed with emphasis on the high sensitivity and specificity of prolonged compound muscle action potential amplitudes in diagnosing critical illness myopathy. (So...
Source: Journal of Clinical Neuromuscular Disease - May 26, 2018 Category: Neurology Tags: Review Article Source Type: research
Case of Anti–Single Recognition Particle–Mediated Necrotizing Myopathy After Influenza Vaccination
We reported a 28-year-old healthy woman presented with subacute onset ascending muscle weakness 2 weeks after an annual influenza vaccination. Cerebral Spinal Fluid study showed normal cell counts with elevated protein and nerve conduction study showed reduced diffuse compound muscle action potential amplitudes suggesting a diagnosis of Guillain–Barré syndrome. Despite treatment using intravenous immunoglobulin, her condition continued to get worse with new bulbar and respiratory muscle weakness. Eventually, the diagnosis of anti–single recognition particle–mediated necrotizing myopathy was made based on elevated cr...
Source: Journal of Clinical Neuromuscular Disease - May 26, 2018 Category: Neurology Tags: Case Review Source Type: research
Distinct Clinical and Genetic Findings in Iranian Patients With Glycogen Storage Disease Type 3
Conclusions:
This is the first comprehensive report of patients with GSD-III in Iran with 2 uncommon clinical presentations and 5 novel mutations in the AGL gene. (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - May 26, 2018 Category: Neurology Tags: Original Article Source Type: research
Lumbosacral Radiculoplexopathy as the Initial Presentation of Lymphoma: A Report of 4 Cases
Conclusions:
Lymphomatous lumbosacral radiculoplexopathy should be considered in patients with progressive lumbosacral radicular symptoms. Magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography, but not cerebrospinal fluid, are helpful in achieving early diagnosis. Treatment responses seem favorable. (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - May 26, 2018 Category: Neurology Tags: Original Article Source Type: research
A Retrospective Analysis of the Safety Profile of Intravenous Immunoglobulin in 1176 Patients Receiving Home Infusion Therapy
Objectives:
This analysis assessed the safety of intravenous immunoglobulin (IVIg) in the treatment of patients with neuroimmunological and immunological disorders in a home-based setting.
Methods:
Adverse reactions (ARs) were assessed in a retrospective review of 1176 patients receiving 28,677 home-based IVIg infusions between 1996 and 2013.
Results:
Of 1176 patients, 648 (55.1%) experienced IVIg-related ARs; 536 (45.6%) were mild, 78 (6.6%) moderate, and 34 (2.9%) severe. Thirty-seven (3.1%) patients were hospitalized because of ARs; of these, headache was most common (51.4%). Mean number of ARs per patient incre...
Source: Journal of Clinical Neuromuscular Disease - May 26, 2018 Category: Neurology Tags: Original Article Source Type: research
40th ANNUAL CARRELL-KRUSEN NEUROMUSCULAR SYMPOSIUM, Texas Scottish Rite Hospital for Children, Dallas, Texas, February 22–23, 2018: Meeting Convenor Susan T. Iannaccone, MD, FAAN
The Annual Carrell-Krusen Neuromuscular Symposium is a unique conference attended by more than 200 each year at the Texas Scottish Rite Hospital for Children. Its name honors Dr. Brandon Carrell who retired as physician-in-chief at Texas Scottish Rite Hospital in 1978 and Dr. Ed Krusen who organized the first Muscular Dystrophy Association clinic in Dallas. This Symposium began in 1978 under the leadership of Dr. Jay Cook at a Muscular Dystrophy Clinic Directors' meeting and is now jointly sponsored by the hospital, the Muscular Dystrophy Association, Childrens' Medical Center of Dallas, and the University of Texas Southwe...
Source: Journal of Clinical Neuromuscular Disease - March 1, 2018 Category: Neurology Tags: Abstracts: PDF Only Source Type: research
Coexistence of Ankylosing Spondylitis and Myotonic Dystrophy: Camptocormia or Thoracic Ankylosis
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - February 22, 2018 Category: Neurology Tags: Letters to the Editor Source Type: research
Miller-Fisher Syndrome With Botulism Toxin Ingestion: The Ultimate Descending Paralysis
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - February 22, 2018 Category: Neurology Tags: Letters to the Editor Source Type: research
Case of Charcot–Marie–Tooth Type 2C Due to a TRPV4 Gene Mutation With Isolated Sudomotor Autonomic Dysfunction
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - February 22, 2018 Category: Neurology Tags: Letters to the Editor Source Type: research
Hypoglycemia in a Patient With Facioscapulohumeral Muscular Dystrophy
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - February 22, 2018 Category: Neurology Tags: Letters to the Editor Source Type: research
Asymmetric Muscle Involvement in an Indian Family With Central Core Myopathy
No abstract available (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - February 22, 2018 Category: Neurology Tags: Letters to the Editor Source Type: research
Peripheral Vasculitic Neuropathy Associated With Minocycline Use
We describe 2 patients presenting with multiplex mononeuritis, associated with skin manifestation, secondary to minocycline-induced vasculitis. One of the cases is associated neither with lupus nor polyarteritis nodosa. An extensive laboratory workup ruled out any possible underlying immunologic disorder. Electrodiagnostic studies were conducted to show axonal neuropathy in patchy and multifocal distribution consistent with multiplex mononeuritis. This diagnosis was confirmed with nerve biopsy. Withdrawing from the offending medication, minocycline, improved the patients' clinical condition and the quantitative serological...
Source: Journal of Clinical Neuromuscular Disease - February 22, 2018 Category: Neurology Tags: Short Report Source Type: research
Radiation-Induced Painful Neurogenic Hypertrophy Treated With Botulinum Toxin A
We report a 27-year-old woman with a history of adenoid cystic carcinoma of the submandibular gland presented with painful twitching of her left shoulder. Electrodiagnostic studies were consistent with a diagnosis of radiation-induced spinal accessory nerve hyperactivity. The patient failed conventional medical therapy. She was treated with an injection of botulinum toxin A, and within 1 month experienced significant relief of symptoms. We thus conclude that Botulinum toxin is a therapeutic option for the pain associated with radiation-induced peripheral nerve hyperactivity. (Source: Journal of Clinical Neuromuscular Disease)
Source: Journal of Clinical Neuromuscular Disease - February 22, 2018 Category: Neurology Tags: Short Report Source Type: research
What is in the Myopathy Literature?
This review is focused on recent reports of sporadic inclusion body myositis (sIBM), myopathy in patients with human immunodeficiency virus type 1 (HIV) infection, and necrotizing autoimmune myopathy with antibodies to signal recognition particle. The sIBM articles cover associations with certain genetic polymorphisms, the possible pathogenic role of anti-cytosolic 5′-nucleotidase 1A antibody, and disease-related burden and health care costs. Another article addressed the possible pathogenic role of signal recognition particle antibody in necrotizing myopathy. A series of HIV patients with overlapping features of sIBM an...
Source: Journal of Clinical Neuromuscular Disease - February 22, 2018 Category: Neurology Tags: Review Article Source Type: research
