A rare presentation of systemic complication of hypoxic ischemic encephalopathy in post cochlear implant child

<p class="abstract">Cochlear implant is a small, complex electronic device to restore some hearing in profoundly deaf or severely hard-of hearing people when organ of corti is not developed or destroyed by injury to such an extent that no hearing can be obtained by hearing aids. Many classifications have been proposed for cochlear implant related problems .We report a case of post cochlear implant child who reported to OPD with complaint of progressive weakness of both upper and lower limb which developed 1 year after surgery ,which parents attributed it as a complication of surgery. On retrospective analysis all patient preop investigations were normal. After detailed examination of the child, neurophysician concluded that child is suffering from global developmental delay with dystonic cerebral palsy. MRI brain and cochlea which was done before the surgery which showed T1 hypointense,T2 and FLAIR hyperintense ,bilateral symmetrical foci with high ADC values are seen in bilateral lentiform nuclei .It has been reported that the bithalamic hyperdensities on CT and/or MRI in severely asphyxiated neonates, were suggested of a distinctive pattern of brain injury which might be useful as an early predictor of status marmoratus,afrequent pathological correlate of dyskinetic cerebral palsy. Approximately 1% of infants who survived perinatal asphyxial HIE would develop delayed-onset dystonia in the course of 7 to 13 years (median 10 years) after birth.</p>

Congenital Malformations and Perinatal Diseases

This chapter describes and illustrates the changes of the central nervous system in congenital malformations and perinatal diseases; it also provides insights in the genetic abnormalities associated with these conditions. Congenital malformations include neurulation failure resulting in neural tube closure defects; disorders of development of the prosencephalon; malformations of the cortical plate (neuronal heterotopia, polymicrogyria, lissencephaly, and focal cortical dysplasia [a frequent cause of epilepsy in children]); and disorders of hindbrain development, particularly malformations of the cerebellum. Destructive lesions of developing brain are described in association with a variety of situations generally resulting in “hypoxia-ischemia.” They may affect the neocortex, causing porencephaly or hydranencephaly; the basal ganglia (status marmoratus); or the white matter (perinatal telencephalic leukoencephalopathy and periventricular leukomalacia).

Congenital Malformations and Perinatal Diseases

This chapter describes and illustrates the changes of the CNS in congenital malformations and perinatal diseases; it also provides insights into the genetic abnormalities associated with these conditions. Congenital malformations include neurulation failure resulting in neural tube closure defects, disorders of development of the prosencephalon, malformations of the cortical plate (neuronal heterotopia, polymicrogyria, lissencephaly, and focal cortical dysplasia, a frequent cause of epilepsy in children), and disorders of hindbrain development, particularly malformations of the cerebellum. Destructive lesions of developing brain are described in association with a variety of situations generally resulting in “hypoxia-ischemia.” They may affect the neocortex, causing porencephaly or hydranencephaly, the basal ganglia (status marmoratus), or the white matter (perinatal telencephalic leukoencephalopathy and periventricular leukomalacia).