Abstract Archives of the RSNA, 2005
1455BP-e
Neurogenic Arthrogryposis Multiplex Congenita Caused by Bilateral Polymicrogyria: Prenatal and Postnatal Imaging Findings
Education Exhibits
Presented on November 27, 2005
Selected for Possible Publication
Cristina Auger MD, Presenter: Nothing to Disclose
Elida Vazquez MD, Abstract Co-Author: Nothing to Disclose
Joaquim Piqueras MD, Abstract Co-Author: Nothing to Disclose
Manuel Roig MD, Abstract Co-Author: Nothing to Disclose
Elena Carreras MD, Abstract Co-Author: Nothing to Disclose
Goya Enriquez MD, Abstract Co-Author: Nothing to Disclose
To present the neurologic pre- and postnatal imaging findings in the 'Fetal Akinesia-Arthrogryposis Sequence' (FAAS), a multietiologic clinical syndrome caused by muscular inactivity in utero.
For didactic purposes, we present a brief review and correlation of clinical and imaging findings.
We present a retrospective review of children with diagnosis of FADS due to different pathological entities, seen in a tertiary pediatric center during the last fourteen years.
The spectrum of diagnosis found encompasses agyria-pachygyria, neuronal migration abnormalities, agenesis of the corpus callosum, hypoplasia of the brainstem and cerebellum, mitochondrial cytopathies, or hypoxic-ischemic lesions.
A total of 7 newborn patients were found to have neurogenic arthrogryposis multiplex congenita (AMC) with associated neuronal migration abnormalities.
Cortical brain malformation discovered on neonatal US and/or MRI were bilateral polymicrogyria, most often perisilvian polymicrogyria, with occasional associated heterotopias.
Conclusion
FADS is the result of a wide spectrum of diseases that in many instances can be identified by imaging.
Auger, C,
Vazquez, E,
Piqueras, J,
Roig, M,
Carreras, E,
Enriquez, G,
Neurogenic Arthrogryposis Multiplex Congenita Caused by Bilateral Polymicrogyria: Prenatal and Postnatal Imaging Findings. Radiological Society of North America 2005 Scientific Assembly and Annual Meeting, November 27 - December 2, 2005 ,Chicago IL.
http://archive.rsna.org/2005/4416576.html