A rare case of schizencephaly in an adult with late presentation

Abstract

Schizencephaly, i.e., split brain, is a rare, congenital cerebral malformation. Magnetic resonance imaging is the best neuroimaging modality for its diagnosis. In literature, only few cases have been reported causing symptoms in adults. Symptomatic patients present as hemiparesis, developmental deficits to seizures. This condition is usually found at birth and presents during early years. Appropriate diagnosis of the disease is necessary to avoid incorrect treatment. Here, we present a rare case of left, unilateral, open lip schizencephaly in an adult patient presenting with partial seizures but with an asymptomatic childhood.

Traumatic epidural hematoma in a patient with severe schizencephaly

“…However, the growth retardation remained unchanged as no cure for the problem was found. 6 The frequency is about 1.5 per 1,000,000 live births, and the frequency of epilepsy is 1 per 1650. 6,7 There is no clear gender distinction but males are slightly more dominant.…”

“…6 The frequency is about 1.5 per 1,000,000 live births, and the frequency of epilepsy is 1 per 1650. 6,7 There is no clear gender distinction but males are slightly more dominant. 8 The etiology is not well understood but those that cause destruction of brain tissue (eg, vascular insult; ischemia; stroke; autoimmune vasculitis; infections caused by TORCH syndrome, toxoplasmosis, syphilis, varicellazoster, parvovirus B19, rubella, cytomegalovirus, herpes; early prenatal injuries related to drug abuse or abdominal trauma of the mother; the frequent coincidence of other central nervous system malformations such as polymicrogyria and gray matter heterotopia; factors of inhibiting brain development); and disorders of nerve cell migration (eg, disturbed migration of primitive neuroblasts; mutations of Lhx2 or EMX2 gene) lead to schizencephaly.…”

“…In these patients, conservative management should be attempted; surgical treatment is only performed in case the epidural hematoma causing severe symptoms. 3,5,6,10…”

Schizencephaly diagnosed after an episode of Seizure during Labour: A case report

“…[1] Although the exact cause of this disorder is uncertain, diverse theories have been put forward to establish a definite underlying etiology. A defect in neuronal migration [2] or some form of molecular genetic abnormalities such as mutation in EMX2 [1], SHH and SIX 3 gene [3] , intrauterine infections [4] are few of the probable causes advocated in literature. MRI is the best suited imaging modality for diagnostic evaluations owing to its superiority in gray and white matter distinction, and hence distinguishing the entity from porencephaly or any other post infectious, postoperative or post necrotic lesions.…”

“…MRI is the best suited imaging modality for diagnostic evaluations owing to its superiority in gray and white matter distinction, and hence distinguishing the entity from porencephaly or any other post infectious, postoperative or post necrotic lesions. [2][5]…”