MR imaging was used to evaluate six patients who had schizencephaly, a disorder of cell migration characterized by holohemispheric, gray-matter-lined clefts. Clinically, these patients presented with intractable seizures and variable developmental delay. Although three of these patients had previous CT scans, the diagnosis was made only by MR. MR was more sensitive than CT in detecting the clef...

BACKGROUND Schizencephaly is one of the most severe forms of neuronal migration defects and is an extremely rare cause of seizure disorder. CASE DETAILS We report a case of bilateral schizencephaly (Type II) presenting as seizure disorder since birth. DISCUSSION This case is rare because of the relatively benign features compared to other reported cases. CONCLUSION Compared to other cases...

We report on two Somalian sibs with severe developmental retardation and spastic cerebral paresis. Both children have bilateral cerebral clefts in the Sylvian region with dilatation of the ventricles, absence of the septum pellucidum, and heterotopia. The diagnosis of familial schizencephaly was made. The occurrence of schizencephaly in two affected sibs supports a genetic basis for schizenceph...

BACKGROUND Schizencephaly is a rare developmental malformation of the central nervous system associated with cell migration disturbances. Schizencephaly can be uni- or bilateral and is divided into two morphological types. The cleft is defined as type I ("closed lips") if there are fused clefts in cerebral mantle. In type II ("open lips") the clefts are separated and filled with cerebrospinal f...

OBJECTIVE To present 16 patients with schizencephaly and neurological involvement, and analyse their characteristics and neuroimages. MATERIAL AND METHODS The study included 16 patients, 8 males and 8 females, all of whom were diagnosed with schizencephaly at less than 3 years of age; 2 patients were diagnosed prenatally. Schizencephaly was identified by computerized tomography (CT) in 1 pati...

Schizencephaly is a rare disorder of brain development resulting in the formation of abnormal unilateral or bilateral clefts in the cerebral hemispheres. It is often accompanied by partial seizures, mental retardation, and hemiparesis. Two patients are described with clear psychotic symptoms with either unilateral or bilateral schizencephaly. The implications of the association between schizenc...

The refinements of neuroimaging have brought to the fo reground a wealth of conditions that were previously the restricted domain of clinical neuropathology. This fascinating (and often bewildering) harvest, includes primary malformations and intrauterine disruptions of the brain. To the first category belong chromosomal abnormalities and genetic or cryptogenic malformations. The latter categor...

45 melanin, but also slow nail growth rate resulting in larger accumulation of the pigment. The presence of HHN neither correlates with degree of renal impairment nor with blood urea nitrogen or creatinine levels. HHN can be an important clue in making the diagnosis of renal disease. 3 0 year old male presented with history of generalised tonic clonic seizures since the age of 16 poorly control...

Schizencephaly is a rare malformation of the central nervous system. Both genetic and non-genetic etiologies like prenatal infections or ischemia have been postulated. Clinical manifestations most often include varying degrees of developmental delay, motor impairment and seizures. It can be associated with septo-optic dysplasia (SOD), optic nerve hypoplasia and absence of septum pellucidum, pac...

The case reports of 17-year-old female dizygotic twins are presented. One of the twins had bilateral closed-lip schizencephaly, and the other had focal cortical dysplasia. Septum pellucidum was absent in both cases. The cortical dysplasia in case 2 corresponded to the same hemispheric location with the right schizencephalic cleft in case 1. The combination of schizencephaly and focal cortical d...