Hageman G, Hoogenraad T U, Prevo R L
Department of Neurology, Medical Spectrum Twente, Enschede, The Netherlands.
Brain Dev. 1994 Nov-Dec;16(6):463-6. doi: 10.1016/0387-7604(94)90009-4.
We describe a 21-year-old woman with neurogenic congenital contractures (arthrogryposis) of the lower limbs, normal intelligence, hyper-reflexia and partial epilepsy. MRI revealed bilateral opercular (perisylvian) cortical dysplasia with infolding of cerebral cortex, a focal neuroblast migrational disorder. This type of migrational disorder is known to have a prenatal onset after the 20th fetal week, whereas the anterior horn cell degeneration responsible of neurogenic arthrogryposis originates at 12-14 weeks of gestation. A prenatal viral infection along the neural axis during both these gestational periods or a genetic defect could be responsible for both lesions in this case.
