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Amyotrophic Lateral Sclerosis
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1. Hyperimmune goat serum for amyotrophic lateral sclerosis.
Match Strength: 13.471

The authors report a patient with amyotrophic lateral sclerosis (ALS) who showed a lessening of deterioration in respiratory muscle strength during treatment with hyperimmune goat serum (HGS) (Aimspro). Respiratory function tests (RFTs) were measured by established protocols, and all measurements were expressed as a percentage of normal predicted values. The rate of decline was calculated by linear regression analysis. Respiratory muscle strength decline was less during 13 months of treatment with HGS (mean 1.3% per month, range 0.8-1.7%) compared to the preceding 13 months (mean 2.3% per ... Read More »
» Published in J Clin Neurosci. 2006 Dec;13(10):1033-6. Epub 2006 Sep 22.

2. Preventing familial amyotrophic lateral sclerosis: is a clinical trial feasible?
Match Strength: 10.737

OBJECTIVE: To evaluate the feasibility of a clinical trial designed to delay or prevent the onset of disease amongst subjects at risk for familial amyotrophic lateral sclerosis (fALS). BACKGROUND: The success of many agents in prolonging survival in the SOD1 model of ALS has not been translated into effective therapies for patients with ALS. It is our hypothesis that a trial in fALS may reproduce the positive effects seen in fALS animals. METHODS: Pedigrees with at least two affected family members were constructed. Unaffected family members were assigned a risk status based on their ... Read More »
» Published in J Neurol Sci. 2006 Dec 21;251(1-2):3-9. Epub 2006 Sep 26.

3. Pyrrolidine dithiocarbamate inhibits induction of immunoproteasome and decreases survival in a rat model of amyotrophic lateral sclerosis.
Match Strength: 9.496

Pyrrolidine dithiocarbamate (PDTC), an inhibitor of nuclear transcription factor kappa-B (NF-kappaB) and an antioxidant, has beneficial effects in animal models of various diseases, including arthritis, brain ischemia, spinal cord injury, Alzheimer's disease, and Duchenne muscular dystrophy. Because inflammation and oxidative damage are also hallmarks of amyotrophic lateral sclerosis (ALS), we studied the effect of oral PDTC treatment on G93A-superoxide dismutase 1 (SOD1) transgenic (TG) rat model of human ALS and observed that PDTC treatment significantly decreases the survival. PDTC ... Read More »
» Published in Mol Pharmacol. 2007 Jan;71(1):30-7. Epub 2006 Sep 28.

4. Acute perfusion of BMAA in the rat's striatum by in vivo microdialysis.
Match Strength: 7.236

The present study is concerned with the hypothetical toxicity of beta-N-methylamino-L-alanine (BMAA), a compound that has been hypothesized to produce amyotrophic lateral sclerosis/Parkinson-dementia complex. We have used the microdialysis technique to perfused different concentrations of BMAA in the rat's striatum 24h after the implantation of a microdialysis probe (day 1). BMAA perfusion produced a dose-response increase in the extracellular output of dopamine. Forty-eight hours after implantation of the probe (day 2), we have perfused MPP+ 1 mM to check the integrity of the dopaminergic ... Read More »
» Published in Toxicol Lett. 2006 Nov 1;167(1):34-9. Epub 2006 Aug 25.

5. Repetitive transcranial magnetic stimulation for ALS. A preliminary controlled study.
Match Strength: 6.524

Repetitive transcranial magnetic stimulation (rTMS) of brain can modulate cortical neurotransmission, a novel paradigm of repetitive stimulation termed continuous theta-burst stimulation (cTBS) produces a pronounced and prolonged suppression of motor cortex excitability. The aim of this preliminary study was to investigate whether cTBS of motor cortex could have any beneficial effect in patients with amyotrophic lateral sclerosis (ALS). We performed a double-blind, placebo-controlled trial. Twenty patients with definite ALS were randomly allocated to blinded active or placebo stimulation. ... Read More »
» Published in Neurosci Lett. 2006 Nov 13;408(2):135-40. Epub 2006 Sep 18.

6. High-frequency chest wall oscillation in ALS: an exploratory randomized, controlled trial.
Match Strength: 6.088

OBJECTIVES: To evaluate changes in respiratory function in patients with ALS after using high-frequency chest wall oscillation (HFCWO). METHODS: This was a 12-week randomized, controlled trial of HFCWO in patients with probable or definite ALS, an Amyotrophic Lateral Sclerosis Functional Rating Scale respiratory subscale score < or = 11 and > or = 5, and forced vital capacity (FVC) > or = 40% predicted. RESULTS: We enrolled 46 patients (58.0 +/- 9.8 years; 21 men, 25 women); 22 used HFCWO and 24 were untreated. Thirty-five completed the trial: 19 used HFCWO and 16 untreated. HFCWO ... Read More »
» Published in Neurology. 2006 Sep 26;67(6):991-7.

7. Evaluation of treatment effects in Alzheimer's and other neurodegenerative diseases by MRI and MRS.
Match Strength: 5.934

Neurodegeneration refers to a large clinically and pathologically heterogeneous disease entity associated with slowly progressive neuronal loss in different anatomical and functional systems of the brain. Neurodegenerative diseases often affect cognition, e.g. Alzheimer's disease (AD), dementia with Lewy bodies and vascular dementia, or different aspects of the motor system, e.g., amyotrophic lateral sclerosis, Parkinson's disease and ataxic disorders. Owing to increasing knowledge about the mechanisms leading to neurodegeneration, the development of treatments able to modify the ... Read More »
» Published in NMR Biomed. 2006 Oct;19(6):655-68.

8. Increased nodal persistent Na+ currents in human neuropathy and motor neuron disease estimated by latent addition.
Match Strength: 5.775

OBJECTIVE: To investigate the changes in nodal persistent Na(+) currents in human neuropathy and motor neuron disease. In human motor axons, approximately 1.0% of total Na(+) channels are active at rest, termed "persistent" Na(+) channels, and the conductance can be non-invasively estimated by the technique of latent addition in vivo. METHODS: Latent addition was performed in median motor axons of 93 patients with axonal neuropathy (n=38), lower motor neuron disorder (LMND; n=19) or amyotrophic lateral sclerosis (ALS; n=36) and in 27 age-matched normal subjects. Brief hyperpolarizing ... Read More »
» Published in Clin Neurophysiol. 2006 Nov;117(11):2451-8. Epub 2006 Sep 25.

9. Modulation of p75-dependent motor neuron death by a small non-peptidyl mimetic of the neurotrophin loop 1 domain.
Match Strength: 5.632

The p75 neurotrophin receptor (p75NTR) is expressed by degenerating spinal motor neurons in amyotrophic lateral sclerosis (ALS). The mature and pro-form of nerve growth factor (NGF) activate p75NTR to trigger motor neuron apoptosis. However, attempts to modulate p75NTR-mediated neuronal death in ALS models by downregulating or antagonizing p75NTR with synthetic peptides have led to only modest results. Recently, a novel ligand of p75NTR, compound LM11A-24, has been identified. It is a non-peptidyl mimetic of the neurotrophin loop 1 domain that promotes hippocampal neuron survival through ... Read More »
» Published in Eur J Neurosci. 2006 Sep;24(6):1575-80.

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