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1.  ALS Multicenter Cohort Study of Oxidative Stress (ALS COSMOS): The study methodology, recruitment, and baseline demographic and disease characteristics 
Objective
In a multicenter study of newly diagnosed ALS patients without a reported family history of ALS, we are prospectively investigating whether markers of oxidative stress (OS) are associated with disease progression.
Methods
An extensive structured telephone interview ascertained environmental, lifestyle, dietary and psychological risk factors associated with OS. Detailed assessments were performed at baseline and at 3 to 6 month intervals during the ensuing 30 months. Our biorepository includes DNA, plasma, urine, and skin.
Results
355 patients were recruited. Subjects were enrolled over a 36 month-period at 16 sites. To meet the target number of subjects, the recruitment period was prolonged and additional sites were included. Demographic and disease characteristics were similar between 477 eligible/non-enrolled and enrolled patients, with the only difference being type of health insurance among enrolled patients. Sites were divided into 3 groups by the number of enrolled subjects. Comparing these 3 groups, the Columbia site had fewer “definite ALS” diagnoses.
Conclusion
This is the first prospective, interdisciplinary, in-depth, multicenter epidemiological investigation of OS related to ALS progression and was accomplished by an aggressive recruitment process. The baseline demographic and disease features of the study sample are now fully characterized.
doi:10.3109/21678421.2013.864312
PMCID: PMC4310702  PMID: 24564738
ALS; Oxidative Stress; Disease Progression; Survival; Epidemiology
2.  Accuracy of Motor Axon Regeneration Across Autograft, Single Lumen, and Multichannel Poly(lactic-co-glycolic Acid) (PLGA) Nerve Tubes 
Neurosurgery  2008;63(1):144-155.
Objective
Accuracy of motor axon regeneration becomes an important issue in the development of a nerve tube for motor nerve repair. Dispersion of regeneration across the nerve tube may lead to misdirection and polyinnervation. In this study, we present a series of methods to investigate the accuracy of regeneration, which we used to compare regeneration across autografts and single lumen poly(lactic-co-glycolic acid) (PLGA) nerve tubes. We also present the concept of the multichannel nerve tube that may limit dispersion by separately guiding groups of regenerating axons.
Methods
Simultaneous tracing of the tibial and peroneal nerves with fast blue (FB) and diamidino yellow (DY), 8 weeks after repair of a 1-cm nerve gap in the rat sciatic nerve, was performed to determine the percentage of double-projecting motoneurons. Sequential tracing of the peroneal nerve with DY 1 week before and FB 8 weeks after repair was performed to determine the percentage of correctly directed peroneal motoneurons.
Results
In the cases in which there was successful regeneration across single lumen nerve tubes, more motoneurons had double projections to both the tibial and peroneal nerve branches after single lumen nerve tube repair (21.4%) than after autograft repair (5.9%). After multichannel nerve tube repair, this percentage was slightly reduced (16.9%), although not significantly. The direction of regeneration was nonspecific after all types of repair.
Conclusion
Retrograde tracing techniques provide new insights into the process of regeneration across nerve tubes. The methods and data presented in this study can be used as a basis in the development of a nerve tube for motor nerve repair.
doi:10.1227/01.NEU.0000335081.47352.78
PMCID: PMC3463233  PMID: 18728579
misdirection; axon targeting; double labeling; peripheral nerve regeneration; rat sciatic nerve model; retrograde tracing
3.  PATIENTS WHO SURVIVE FIVE YEARS OR MORE WITH ALS IN OLMSTED COUNTY, 1925–2004 
Background
A small proportion of patients with ALS survive more than five years. The frequency of five year or longer survival with ALS in a United States population is unknown but may provide a baseline for studies that employ survival as a primary endpoint of analysis.
Methods
All persons diagnosed with ALS in Olmsted County between 1925 and 2004 were studied for demographic and clinical features. Longer term survivors were defined as patients who lived five years or longer, tracheostomy-free, following symptomatic onset.
Results
94 patients (mean survival from symptomatic onset 2.95 years (95% CI 2.54–3.35), mean survival from diagnosis 1.89 years, (95% CI 1.54–2.24)) were diagnosed with ALS. Five-year or longer survivors accounted for 14% of the population of patients (95% CI 7.9%–22.8%). The frequency of five year or longer survivors did not change over time. Mean survival of these individuals was 7.04 years (95% CI 6.14–7.94 years; range 5.11–9.35 years). They had a significantly longer mean time to diagnosis (1.77 years, 95% CI 0.95–2.58 years) as compared to less than five year survivors (0.94 years, 95% CI 0.75–1.13 years) (p=0.02) but could not be reliably identified at the time of diagnosis by age, sex, clinical presentation, or El Escorial category.
Conclusion
Patients surviving more than five years following the symptomatic onset of ALS account for 14% of the total ALS population. This frequency has not changed over time. Patients with five year or longer survival are clinically similar to the total population ALS population in terms of age, gender, presentation, and site of onset but have a longer time from symptomatic onset to diagnosis.
doi:10.1136/jnnp.2009.201251
PMCID: PMC2946435  PMID: 20627966
amyotrophic lateral sclerosis; natural history; prognosis; survival
4.  Survival Profiles of Patients With Frontotemporal Dementia and Motor Neuron Disease 
Archives of neurology  2009;66(11):1359-1364.
Background
Frontotemporal dementia and amyotrophic lateral sclerosis are neurodegenerative diseases associated with TAR DNA-binding protein 43– and ubiquitin-immunoreactive pathologic lesions.
Objective
To determine whether survival is influenced by symptom of onset in patients with frontotemporal dementia and amyotrophic lateral sclerosis.
Design, Setting, and Patients
Retrospective review of patients with both cognitive impairment and motor neuron disease consecutively evaluated at 4 academic medical centers in 2 countries.
Main Outcome Measures
Clinical phenotypes and survival patterns of patients.
Results
A total of 87 patients were identified, including 60 who developed cognitive symptoms first, 19 who developed motor symptoms first, and 8 who had simultaneous onset of cognitive and motor symptoms. Among the 59 deceased patients, we identified 2 distinct subgroups of patients according to survival. Long-term survivors had cognitive onset and delayed emergence of motor symptoms after a long monosymptomatic phase and had significantly longer survival than the typical survivors (mean, 67.5 months vs 28.2 months, respectively; P<.001). Typical survivors can have simultaneous or discrete onset of cognitive and motor symptoms, and the simultaneous-onset patients had shorter survival (mean, 19.2 months) than those with distinct cognitive or motor onset (mean, 28.6 months) (P=.005).
Conclusions
Distinct patterns of survival profiles exist in patients with frontotemporal dementia and motor neuron disease, and overall survival may depend on the relative timing of the emergence of secondary symptoms.
doi:10.1001/archneurol.2009.253
PMCID: PMC2881327  PMID: 19901167
5.  ASRA Practice Advisory on Neurologic Complications in Regional Anesthesia and Pain Medicine 
Neurologic complications associated with regional anesthesia and pain medicine practice are extremely rare. The ASRA Practice Advisory on Neurologic Complications in Regional Anesthesia and Pain Medicine addresses the etiology, differential diagnosis, prevention, and treatment of these complications. This Advisory does not focus on hemorrhagic and infectious complications, because they have been addressed by other recent ASRA Practice Advisories. The current Practice Advisory offers recommendations to aid in the understanding and potential limitation of neurologic complications that may arise during the practice of regional anesthesia and pain medicine.
doi:10.1016/j.rapm.2008.07.527
PMCID: PMC2869280  PMID: 18774509
Complications of anesthesia; Nerve injury; Spinal anesthesia; Epidural anesthesia; Peripheral nerve block; Regional anesthesia; Pain medicine; Transforaminal block
6.  Beyond Parkinson Disease: Amyotrophic Lateral Sclerosis and the Axon Guidance Pathway 
PLoS ONE  2008;3(1):e1449.
Background
We recently described a genomic pathway approach to study complex diseases. We demonstrated that models constructed using single nucleotide polymorphisms (SNPs) within axon guidance pathway genes were highly predictive of Parkinson disease (PD) susceptibility, survival free of PD, and age at onset of PD within two independent whole-genome association datasets. We also demonstrated that several axon guidance pathway genes represented by SNPs within our final models were differentially expressed in PD.
Methodology/Principal Findings
Here we employed our genomic pathway approach to analyze data from a whole-genome association dataset of amyotrophic lateral sclerosis (ALS); and demonstrated that models constructed using SNPs within axon guidance pathway genes were highly predictive of ALS susceptibility (odds ratio = 1739.73, p = 2.92×10−60), survival free of ALS (hazards ratio = 149.80, p = 1.25×10−74), and age at onset of ALS (R2 = 0.86, p = 5.96×10−66). We also extended our analyses of a whole-genome association dataset of PD, which shared 320,202 genomic SNPs in common with the whole-genome association dataset of ALS. We compared for ALS and PD the genes represented by SNPs in the final models for susceptibility, survival free of disease, and age at onset of disease and noted that 52.2%, 37.8%, and 34.9% of the genes were shared respectively.
Conclusions/Significance
Our findings for the axon guidance pathway and ALS have prior biological plausibility, overlap partially with PD, and may provide important insight into the causes of these and related neurodegenerative disorders.
doi:10.1371/journal.pone.0001449
PMCID: PMC2175528  PMID: 18197259

Results 1-6 (6)