Congenital lymphocytic choriomeningitis virus infection: spectrum of disease
Article first published online: 7 JUN 2007
Copyright © 2007 American Neurological Association
Annals of Neurology
Volume 62, Issue 4, pages 347–355, October 2007
How to Cite
Bonthius, D. J., Wright, R., Tseng, B., Barton, L., Marco, E., Karacay, B. and Larsen, P. D. (2007), Congenital lymphocytic choriomeningitis virus infection: spectrum of disease. Ann Neurol., 62: 347–355. doi: 10.1002/ana.21161
- Issue published online: 29 OCT 2007
- Article first published online: 7 JUN 2007
- Manuscript Accepted: 26 APR 2007
- Manuscript Revised: 22 MAR 2007
- Manuscript Received: 15 JAN 2007
- NIH (National Institute of Neurological Disorders and Stroke, Public Health Service). Grant Number: K08 NS02007
- March of Dimes Birth Defects Foundation. Grant Number: 1-01-217
- The John Martin Fund for Neuroanatomical Research
- Children's Miracle Network. Grant Number: 1464
Lymphocytic choriomeningitis virus (LCMV) is a human pathogen and an emerging neuroteratogen. When the infection occurs during pregnancy, the virus can target and damage the fetal brain and retina. We examined the spectrum of clinical presentations, neuroimaging findings, and clinical outcomes of children with congenital LCMV infection.
Twenty children with serologically confirmed congenital LCMV infection were identified. The children underwent neuroimaging studies and were followed prospectively for up to 11 years.
All children with congenital LCMV infection had chorioretinitis and structural brain anomalies. However, the presenting clinical signs, severity of vision disturbance, nature and location of neuropathology, and character and severity of brain dysfunction varied substantially among cases. Neuroimaging abnormalities included microencephaly, periventricular calcifications, ventriculomegaly, pachygyria, cerebellar hypoplasia, porencephalic cysts, periventricular cysts, and hydrocephalus. The combination of microencephaly and periventricular calcifications was the most common neuroimaging abnormality, and all children with this combination had profound mental retardation, epilepsy, and cerebral palsy. However, others had less severe neuroimaging abnormalities and better outcomes. Some children had isolated cerebellar hypoplasia, with jitteriness as their presenting sign and ataxia as their principal long-term neurological dysfunction.
Congenital LCMV infection can have diverse presenting signs, neuroimaging abnormalities, and clinical outcomes. In the companion article to this study, we utilize an animal model to show that the clinical and pathological diversity in congenital LCMV infection is likely due to differences in the gestational timing of infection. Ann Neurol 2007