Total cerebral dysgenesis - A very rare case

Abstract

Introduction: Cerebral dysgenesis is the absence of formation or non-formation of a cerebellar hemisphere(s). Although many authors have described this disorder for over centuries now, no author has reported a case of total cerebral dysgenesis. Case presentation: We present a case of a seven months old male infant who had difficulty feeding immediately after birth. He did not cry immediately after delivery. He could not move his limbs or the body. The mother did not have access to antenatal care. MRI of head revealed bilateral absence of cerebral hemispheres with normal skull vault filled with cerebrospinal fluid (CSF). The brain stem is visible, as well as part of the cerebellum. Electroencephalography (E.E.G) done revealed no electrical impulses over areas responsible for coordination of the limbs as well as the entire trunk. The kid was currently being managed conservatively. He was fed via Nasogastric tube (NG-Tube). Conclusions: This presentation is puzzling as compare to hydranencephaly. If he miraculously advances with age, he will live with complex neurological syndromes. Keywords: Cerebral, Dysgenesis, Agenesis, Cerebellum, Hydranencephaly