CNS Malformations

Posted by e-Medical PPT Thursday, October 14, 2010
Arnold–Chiari malformation is a malformation of the brain. It consists of a downward displacement of the cerebellar tonsils through the foramen magnum,sometimes causing hydrocephalus as a result of obstruction of cerebrospinal fluid outflow.The cerebrospinal fluid outflow is caused by phase difference in outflow and influx of blood in the vasculature of the brain. It can cause headaches, fatigue, muscle weakness in the head and face, difficulty swallowing, dizziness, nausea, impaired coordination, and, in severe cases, paralysis.The conditions sometimes associated with Chiari Malformation include hydrocephalus, syringomyelia, spinal curvature, tethered spinal cord syndrome, and connective tissue disorders such as Ehlers-Danlos syndrome and Marfan Syndrome.
Dandy-Walker syndrome is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it.The key features of this syndrome are an enlargement of the fourth ventricle; a partial or complete absence of the cerebellar vermis, the posterior midline area of cerebellar cortex responsible for coordination of the axial musculature; and cyst formation near the internal base of the skull. Symptoms, which often occur in early infancy, include slower motor development and progressive enlargement of the skull. In older children, symptoms of increased intracranial pressure such as irritability, vomiting and convulsions and signs of cerebellar dysfunction such as unsteadiness and lack of muscle coordination or jerky movements of the eyes may occur.

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