Scaling New Heights To Diagnose Hydranencephaly

Abstract

Hydranencephaly is a specific worldwide CNS encephaloclastic disorders characterized by destruction of cerebral hemispheres and the corpus striatum and replacement by a thin membranous sac filled with cerebrospinal fluid, glial tissue and ependymal. The sac represents the relatively intact leptomeninges. In this condition midbrain usually remain safe and intact but ambiguity still persists in mode of transmission. However skull and meninges consistent with normal embryogenesis of the telencephalon show their true identity both in congenital or acquired conditions. In many children at birth impaired vision, infantile spasm or seizures, slow growth ,the head appears to be enlarged associated with spasticity and rigidity of arms and legs, thereby both physical and mental retardation seen with poor intellect or skill. The children reveal poor thermoregulation and experience an increased muscular tone (hypertonia) or exaggeration of muscular reflexes (hyperreflexia). Many children attain mortality even prior to delivery or on the onset of birth. MR imaging of the 6 week old female child displays symptoms of irritability, spontaneous reflexes such as, crying, sucking, swallowing and early onset of diabetes insipidus, retarded motor developments,. Diagnosis may be delayed for several months due to relatively normal behaviour, movement of arms and legs, macrocephaly and even normal head size. I. Introduction Hydranencephaly is a rare congenital disorder and one of the most severe forms of bilateral cerebral cortical anomaly, in which the cerebral hemispheres are absent and represented by membranous sacs with the remnants of the cortex dispersed over the membranes and replaced by sacs filled with cerebrospinal fluid. The midbrain, cerebellum, thalami, basal ganglia, and choroid plexus are usually not involved. The term hydranencephaly was first introduced by Spielmeyer. Incidence: 1/10000 live births world wide.