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2020, Number 4

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AMC 2020; 24 (4)

Report of an atypical case of open lip schizencephaly

Betharte-Sotomayor Y, Céspedes-Rodríguez RA, Marrero-Abreu AR,o Zayas-Suárez E

Language: Spanish
References: 13
Page: 598-606
PDF size: 532.72 Kb.


ABSTRACT

Background: open-lip schizencephaly is a cerebral malformation of the group of cerebral migration disorders, which is considered a rare disease worldwide and has serious structural lesions, and within the most common clinical manifestations find epileptic seizures that are difficult to manage, alterations in language development, visual defects and microcephaly.
Objective: to present an unusual case of brain malformation, diagnosed as a consequence of an acute complication, where other atypical neurological, psychological and ophthalmological features were found.
Clinical case: 19 years old woman; with a prenatal history of a congenital malformation of the central nervous system due to a spastic hemiparesis from birth, but without seizures or mental retardation. She was admitted to the emergency department for signs of acute intracranial hypertension, and during her stay, computed tomography was performed, which showed an open lip schizencephaly. She underwent surgery and after her recovery, neuropsychological and ophthalmological exams were performed which showed unusual characteristics in the patient.
Conclusions: the presence of a motor defect from birth requires an imaging evaluation for structural estimation of the possible existing malformations, which will allow us to anticipate the appearance of other symptoms or complications. The early neuropsychological stimulation will activate the mechanisms of cerebral plasticity with improvement of the quality of life.


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