Regression of white matter hypodensities with age in Aicardi–Goutierés syndrome: a case report
- 107 Downloads
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-α 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.
KeywordsBasal ganglia calcification CSF pleocytosis White matter hypodensities
- 2.Tolmie JL, Shillito P, Hughes-Benzie R, Stephenson JB (1995) The Aicardi–Goutierés syndrome (familial, early onset encephalopathy with calcifications of the basal ganglia and chronic cerebrospinal fluid lymphocytosis). J Med Genet 32(11):881–844Google Scholar