Abstract
Ultrastructural and biochemical studies were performed on a biopsy from the cerebral cortex of a child with late infantile amaurotic idiocy. The disease began clinically at 26 months of age with intellectual and behavioral deterioration and progressive blinijness resulting from pigmentary degeneration of the retina. The cytoplasm olf neurons was distended by material that stained intensely in periodic acid-Schiff preparations, and ultrastructural studies showed many abnormal membrane-bound cytosomes. Although concentrations of lipid-bound sialic acid were normal, showing only minor changes in the ganglioside pattern, riondialyzable sialoglycoproteins were strikingly increased. Biochemical characterization of some forms of amaurotic familial idiocy may be dependent on increasing knowledge of metabolic interrelations between sialoglycopiroteins and gangliosides.
- © 1974 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
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