Table is card and pk = 250 Result =
Submode=
Alexander Disease, Rosenthal Fiber Encephalopathy, MedPix™ : 250 - Medical Image Database and Atlas
Welcome! It's Thursday, September 02, 2010 :: :: :: RSS Link

Location and Category

Location:
More Like This ? Brain and Neuro
Sublocation:
none selected
Category:
More Like This ? Congenital, genetic
Find Related Topics: Click on the Location, Sublocation, or Category Links - (above)

TOPIC and DISCUSSION :: Slide Sorter :: Print Topic :: Slide Sorter ::

More Like This ? Alexander Disease, Rosenthal Fiber Encephalopathy
Topic 250 - Created: 1988-01-01 00:00:00-05 - Modified: 2008-09-11 12:36:59-04
ACR Index: 1.6

Alexander's disease (AD), also called "Rosenthal Fiber Encephalopathy" and "fibrinoid leukodystrophy" is an uncommon neurodegenerative disease. Both childhood and adult types have been described. It is caused by a mutation in the GFAP gene (Glial Fibrillary Acidic Protein). [PubMed REF] href="http://www.neurology.org/cgi/content/abstract/66/4/494" target='newind' onclick='return confirm_leave("WARNING MESSAGE: \n Following this link may leave the MedPix website. \n \n Do you want to continue ?", "http://www.neurology.org/cgi/content/abstract/66/4/494");' >Click for HyperLink

Early presentation (first weeks of age), macrocephaly, failure to achieve milestones, progressive spastic quadriparesis, intellectual failure, early death (infancy, childhood), low density in frontal white matter => extends posteriorly and into internal capsule, enhancement of tips of frontal horns, prolonged T1 and T2 in frontal regions => progresses posteriorly to parietal and the internal and external capsules, frankly cystic changes occur late with marked atrophy of the corpus callosum.

The adult-onset disease (AOAD) also affects the medulla oblongata and the upper cervical spinal cord.

=====================================================
Can MRI diagnose adult-onset Alexander Disease?

"Atrophy and changes in signal intensity in the medulla oblongata and upper cervical spinal cord were present in 11 of 11 cases and were the diagnostic features." [PubMed REF] Click for HyperLink


Click for HyperLink

=====================================================
Click for HyperLink

=====================================================
Click for HyperLink
Click for HyperLink

Contributor Credits

Submitted by: James George Smirniotopoulos, M.D. - Author Info
Affiliation: Uniformed Services University
Approved By: Alice Boyd Smith - Editor Info
Affiliation: Uniformed Services University


Text and Images may be Copyrighted © 1999 - 2009 by the Original Content Contributors.
Copyrighted materials are reproduced here with their Permission.

MedPix® is a Registered Trademark of USUHS :: The MedPix™ Database Engine is Patented - USPTO No. 7,080,098
Portions of MedPix™ are Copyright © 1999 - 2009 by J.G. Smirniotopoulos, M.D. & H. Irvine, M.D.
The MedPix™ Classification Schema Copyright © 1999 - 2009 by J.G.Smirniotopoulos,M.D.
MedPix™ has displayed more than   324,659,079   pages since 3 September 2000.
... Google Analytics Active ...