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Inclusion body myositis is the most common idiopathic inflammatory myopathy in elderly individuals. It typically causes proximal and distal limb weakness with forearm flexors and quadriceps being the most severely affected muscles. Axial musculature is infrequently involved. Here, we report an 80-year-old man who presented with an 18-month history of(More)
OBJECTIVE To characterize the clinical features and MRI abnormalities of leucine-rich glioma-inactivated 1 (LGI1)-autoantibody (Ab) faciobrachial dystonic seizures (FBDS). METHODS Forty-eight patients with LGI1-Ab encephalopathy were retrospectively identified by searching our clinical and serologic database from January 1, 2002, to June 1, 2015. Of(More)
BACKGROUND Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death. OBJECTIVE To characterize patients with a neurodegenerative disease and a rapidly progressive course to death. DESIGN, SETTING, AND PATIENTS Using a text(More)
BACKGROUND In humans, Menkes disease is the well-recognized neurological disorder due to inherited copper deficiency. Myelopathy due to acquired copper deficiency is not a well-recognized entity in humans, although myelopathy due to copper deficiency is well documented in some animal species. PATIENTS We describe 3 patients who developed a progressive(More)
Linezolid is the first drug in a new class of synthetic antimicrobials, the oxazolidinones, to be approved by the Food and Drug Administration. Linezolid is active against methicillin- and vancomycin-resistant gram-positive microorganisms. We describe 2 patients who developed peripheral neuropathy after prolonged treatment with linezolid.(More)
A hexanucleotide repeat expansion in the chromosome 9 open reading frame 72 (C9ORF72) gene was recently discovered as the cause underlying frontotemporal degeneration (FTD) and/or amyotrophic lateral sclerosis (ALS) linked to chromosome 9 (c9FTD/ALS). In this atypical case of c9FTD/ALS, the proband presented with amnestic mild cognitive impairment which(More)
BACKGROUND The use of biodegradable synthetic nerve conduits for the reconstruction of segmental nerve defects has been extensively reported in both animal and human studies, with a majority of studies evaluating sensory nerve recovery. However, few studies have compared these nerve conduits for functional motor recovery. The purpose of this study was to(More)
OBJECTIVE In patients with symptomatic diabetic polyneuropathy, is oral alpha-lipoic acid (ALA) effective in improving neuropathic symptoms compared with placebo? METHODS The question was addressed with a structured evidence-based clinical neurologic practice review via videoconferencing between 3 academic institutions. Participants included consultant(More)
Monitoring of peripheral nerve function is important during the surgical treatment of peripheral nerve and plexus lesions, allowing for rapid assessment of the integrity of the roots, plexus, and nerves. The results of this monitoring assist the surgeon in the overall approach to treatment of these lesions. There are, however, many technical challenges to(More)