We have located links that may give you full text access.
Case Reports
Journal Article
Sjögren-Larsson syndrome: postmortem brain abnormalities.
Pediatric Neurology 1998 April
Brain abnormalities were described in a patient who had experienced Sjogren-Larsson syndrome (SLS) with typical clinical signs. Examination of the brain revealed (1) accumulation of peculiar lipoid substances stained lightly with periodic acid-Schiff (PAS) in the subpial, subependymal, and perivascular glial layers, the subpial and perivascular spaces, and the white matter of the cerebrum and brainstem; (2) proliferation of perivascular macrophages containing lipofuscin-like pigments; (3) dense distribution of round or ellipsoid small bodies stained strongly with PAS in the subpial, subependymal, and perivascular glial layers and the white matter; (4) appearance of spheroid bodies in the neuropil of relay nuclei (the lateral geniculate body, pontine nuclei, inferior olivary nucleus, posterior funicular nucleus, and dentate nucleus) and the white matter; and (5) reduction of myelinated nerve fibers in the cerebral and cerebellar white matter. Six-layered neuronal cytoarchitecture was preserved in the cerebral neocortex, except for the insula where pyramidal neurons were arranged at random. The exact mechanisms remain unclear, but it is suggested that peculiar lipoid substances may accumulate in the specific regions of the brain and that neuronal and astrocytic processes may be primarily affected in SLS.
Full text links
Related Resources
Trending Papers
Challenges in Septic Shock: From New Hemodynamics to Blood Purification Therapies.Journal of Personalized Medicine 2024 Februrary 4
Molecular Targets of Novel Therapeutics for Diabetic Kidney Disease: A New Era of Nephroprotection.International Journal of Molecular Sciences 2024 April 4
The 'Ten Commandments' for the 2023 European Society of Cardiology guidelines for the management of endocarditis.European Heart Journal 2024 April 18
A Guide to the Use of Vasopressors and Inotropes for Patients in Shock.Journal of Intensive Care Medicine 2024 April 14
Diagnosis and Management of Cardiac Sarcoidosis: A Scientific Statement From the American Heart Association.Circulation 2024 April 19
Essential thrombocythaemia: A contemporary approach with new drugs on the horizon.British Journal of Haematology 2024 April 9
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app
All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.
By using this service, you agree to our terms of use and privacy policy.
Your Privacy Choices
You can now claim free CME credits for this literature searchClaim now
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app