Eric Sorenson

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The TAR DNA-binding protein 43 (TDP-43) has been identified as the major disease protein in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin inclusions (FTLD-U), defining a novel class of neurodegenerative conditions: the TDP-43 proteinopathies. The first pathogenic mutations in the gene encoding TDP-43 (TARDBP) were(More)
BACKGROUND The progranulin gene (GRN) is mutated in 5-10% of patients with frontotemporal lobar degeneration (FTLD) and in about 20% of patients with familial FTLD. The most common mutation in GRN is Arg493X. We aimed to establish the contribution of this mutation to FTLD and related disorders. METHODS We measured the frequency of Arg493X in 3405(More)
BACKGROUND Mitochondrial dysfunction occurs early in the course of ALS, and the mitochondria may be an important site for therapeutic intervention. Creatine stabilizes the mitochondrial transition pore, and is important in mitochondrial ATP production. In a transgenic mouse model of ALS, administration of creatine prolongs survival and preserves motor(More)
INTRODUCTION Real time ultrasound imaging of the diaphragm is an under-used tool in the evaluation of patients with unexplained dyspnea or respiratory failure. METHODS We measured diaphragm thickness and the change in thickness that occurs with maximal inspiration in 150 normal subjects, with results stratified for age, gender, body mass index, and(More)
OBJECTIVE To study the frequency, demographics, clinical characteristics, and outcomes of patients with an antemortem diagnosis of fibrocartilaginous embolism (FCE), a rare cause of spinal cord and cerebral infarction because of the presumed embolization of nucleus pulposus material into the vascular circulation. METHODS We retrospectively reviewed the(More)
OBJECTIVE To determine whether clinical and demographic features are associated with prognosis in patients with frontotemporal dementia and motor neuron disease (FTD-MND). METHODS This was a case series of FTD-MND categorized according to behavioral- or language-dominant symptoms at presentation and throughout the disease course. Demographic, clinical,(More)
OBJECTIVE Intraneural ganglia (IG) are an underappreciated but treatable cause of common peroneal neuropathy (CPN). This study was designed to determine if there are clinical measures that distinguish CPN caused by IG from CPN without a clear proximate cause. METHODS Clinical and electrodiagnostic features of 22 cases of IG were compared in a case-control(More)
The authors sought to determine trends in the incidence of ALS in Olmsted County from 1925 to 1998. Seventy-seven cases of ALS were identified during the period studied. The incidence rate remained stable at 1.7 cases per 100,000 people per year. Mean age at onset was 63 years. Mean survival was 23 months from diagnosis. Mean survival for patients <60 years(More)
BACKGROUND Preclinical and clinical studies of gabapentin in patients with ALS led the authors to undertake a phase III randomized clinical trial. METHODS Patients were randomly assigned, in a double-blinded fashion, to receive oral gabapentin 3,600 mg or placebo daily for 9 months. The primary outcome measure was the average rate of decline in isometric(More)