Clinical evaluation of ALS drugs
Citation Manager Formats
Make Comment
See Comments

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
During the past several years there has been a dramatic increase in the number of clinical trials investigating new treatments for amyotrophic lateral sclerosis (ALS). Among the reasons for this intense activity are new insights into the pathogenesis of the disease; the availability of a new assortment of drugs, a number created by recombinant techniques, that are available for clinical evaluation; newly issued standards for the conduct of clinical trials, making them more reproducible and efficient; and improved assessment and measurement techniques for following the natural history of ALS and drug treatment effects on the course of the disease. [1] Increased determination on the part of physicians, patients, their families, and voluntary health organizations to enroll patients in clinical trials makes the goal of finding an effective treatment for ALS much more attainable. [1]
Pathogenesis of ALS.
The pathogenesis of ALS can be divided into initiating events, propagating events, and terminal events. Oxidative stress is believed to be an initiating event in ALS. [2] The identification of patients with familial ALS who have been found to have mutations in Cu/Zn superoxide dismutase (SOD1) supports this hypothesis. [3] This defect may render motor neurons more susceptible to glutamate-induced toxicity. Propagating events are involved in the spread of the disease process. Glutamate is the principal excitatory neurotransmitter in the brain, mediating many neurologic functions, and there is evidence that glutamate excitotoxicity may be an important factor in the propagation of ALS. [4] Decreased uptake of glutamate may lead to over-stimulation of glutamate receptors which, via increased intracellular calcium, can lead to the activation of a series of enzymes, such as protein kinase C, phospholipases, proteases, protein phosphatases, and nitric oxide synthetase, which contribute to injury of the neuronal cell membrane. [5] Autoimmune mechanisms, such as the formation of anticalcium channel antibodies, are also believed to …
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
For assistance, please contact:
AAN Members (800) 879-1960 or (612) 928-6000 (International)
Non-AAN Member subscribers (800) 638-3030 or (301) 223-2300 option 3, select 1 (international)
Sign Up
Information on how to subscribe to Neurology and Neurology: Clinical Practice can be found here
Purchase
Individual access to articles is available through the Add to Cart option on the article page. Access for 1 day (from the computer you are currently using) is US$ 39.00. Pay-per-view content is for the use of the payee only, and content may not be further distributed by print or electronic means. The payee may view, download, and/or print the article for his/her personal, scholarly, research, and educational use. Distributing copies (electronic or otherwise) of the article is not allowed.
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
You May Also be Interested in
Cutaneous α-Synuclein Signatures in Patients With Multiple System Atrophy and Parkinson Disease
Dr. Rizwan S. Akhtar and Dr. Sarah Brooker
► Watch
Related Articles
- No related articles found.
Alert Me
Recommended articles
-
Articles
New approaches to the treatment of ALSRobert G. Miller, Robert Sufit et al.Neurology, April 01, 1997 -
Articles
Multipoint incremental motor unit number estimation as an outcome measure in ALSJ.M. Shefner, M.L. Watson, L. Simionescu et al.Neurology, June 15, 2011 -
Brief Communications
Relationship of the Tufts Quantitative Neuromuscular Exam (TQNE) and the Sickness Impact Profile (SIP) in measuring progression of ALSD. McGuire, L. Garrison, C. Armon et al.Neurology, May 01, 1996 -
Introduction
ALS standard of care consensus conferenceRobert G. Miller et al.Neurology, April 01, 1997