Advanced Search

Journal Navigation

Journal Home

Subscriptions

Archive

Contact Us

Table of Contents

Click here for FREE ACCESS to this landmark database

Click here to sign up for SAGE Journal Email Alerts today!

Sign In to gain access to subscriptions and/or personal tools.
Journal of Child Neurology
This Article
Right arrow Full Text (PDF)
Right arrow References
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Saved Citations
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Request Reprints
Right arrow Add to My Marked Citations
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Right arrow Citing Articles via Scopus
Google Scholar
Right arrow Articles by Dimova, P. S.
Right arrow Articles by Mikova, O. A.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Dimova, P. S.
Right arrow Articles by Mikova, O. A.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati  
What's this?

Case of Aicardi-Goutières Syndrome With Long-lasting Increase of Cerebrospinal Interferon-{alpha}

Petia S. Dimova, MD

Clinic of Child Neurology St. Naum University Hospital of Neurology and Psychiatry, pdimova{at}cablebg.net

Olya A. Mikova, MD

Laboratory of Neuroimmunology St. Naum University Hospital of Neurology and Psychiatry Sofia, Bulgaria

Aicardi-Goutières syndrome is a rare progressive encephalopathy characterized by a typical clinical picture, bilateral basal ganglia calcifications, leukodystrophy and brain atrophy, lymphocytosis, and elevated interferon-{alpha} in the cerebrospinal fluid. Among the cases described to date, variability in the clinical expression or in the cerebrospinal fluid abnormalities has been reported. We present a case with a delayed diagnosis at the age of 8 years, when brain computed tomography was done because there was no first image from the age of 8 months, when the disease started. Symmetric basal ganglia calcifications were visualized and led to purposeful investigation of the cerebrospinal fluid. It revealed an interferon-{alpha} titer of 103 IU/mL, which, together with the progressive brain damage and disease course, was crucial for the diagnosis. This rare finding of long-term highly elevated interferon-{alpha} in the cerebrospinal fluid is discussed with respect to the clinical course. (J Child Neurol 2005;20:915—920).

Journal of Child Neurology, Vol. 20, No. 11, 915-920 (2005)
DOI: 10.1177/08830738050200111101
Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati    What's this?


This article has been cited by other articles:


Home page
 Br Med BullHome page
S. Orcesi, R. La Piana, and E. Fazzi
Aicardi-Goutieres syndrome
Br. Med. Bull., March 1, 2009; 89(1): 183 - 201.
[Abstract] [Full Text] [PDF]