Morphometric study of motoneurons in congenital nemaline myopathy and werdnig‐Hoffmann disease
Citation Manager Formats
Make Comment
See Comments
Abstract
To test whether congenital nemaline myopathy(NM) might have a neurogenic basis, we evaluated the number and frequency distribution of L5 motoneuron cell bodies and their myelinated ventral root (VSR) axons in a typical case. Results were compared to those from three age-matched controls and those from a child with Werdnig-Hoffmann disease (WHD). Diameter histograms of cell bodies of L5 motoneurons from controls contained three peaks which were identified as large, intermediate, and small cytons (LC, IC, and SC). In previous work from our laboratory, the numbers of LC and IC of the L5 segment was found to correspond closely enough to the numbers of LA and IA (large and intermediate diameter myelinated axons) of L5 ventral spinal root to indicate that the majority of LC are alpha and the majority of IC are gamma motoneurons. The number of LC of L5 reference cords varied from 3508 to 4916, and of IC from 967 to 2204. The diameter histogram of cytons from the NM case differed from controls; the LC and IC peaks were displaced to smaller diameter categories, but their number was normal (4520 and 2023, respectively). In WHD, the numbers of LC and IC were 211 and 946, respectively. Axons were similarly affected. The severe decrease from normal in the number of large motoneurons in WHD, and the reduction in size without loss of motoneurons in NM, indicates a different morphologic basis for NM and WHD. In NM the reduction in size without decrease in number of motoneurons is probably secondary to congenital myopathy and does not provide evidence for a neurogenic etiology.
- © 1978 by the American Academy of Neurology
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
Dr. Dennis Bourdette and Dr. Lindsey Wooliscroft
► Watch
Related Articles
- No related articles found.