Regression of white matter hypodensities with age in Aicardi-Goutierés syndrome: a case report

Aicardi-Goutierés syndrome (AGS) is a severe and progressive familial encephalopathy that is characterized by acquired microcephaly, intracranial calcification, white matter lesions, and chronic lymphocytosis with elevated levels of interferon-alpha in the cerebrospinal fluid. Although the degree of...

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Published inChild's nervous system Vol. 22; no. 11; pp. 1503 - 1506
Main Authors Kothare, Sanjeev V, Pungavkar, Sona A, Patkar, Deepak P, Sainani, Nisha I, Naik, Martand H, Gadani, Sameeer
Format Journal Article
LanguageEnglish
Published Germany 01.11.2006
Subjects
Age Factors
Atrophy - pathology
Basal Ganglia Diseases - pathology
Calcinosis - pathology
Child
Disease Progression
Humans
Magnetic Resonance Imaging
Male
Tomography, X-Ray Computed
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Summary:Aicardi-Goutierés syndrome (AGS) is a severe and progressive familial encephalopathy that is characterized by acquired microcephaly, intracranial calcification, white matter lesions, and chronic lymphocytosis with elevated levels of interferon-alpha in the cerebrospinal fluid. Although the degree of calcification and the severity of brain atrophy are variable, typically, the brain lesions appear to progress on successive examinations. We report a 7-year-old male patient who showed relative regression of white matter lesions with nonprogression of basal ganglia calcification and atrophy on follow-up magnetic resonance imaging and computed tomography scans. Magnetic resonance spectroscopy findings were normal. This, to our knowledge, is the first case report, which describes relative regression of the white matter changes in AGS.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
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ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-006-0112-9