Schizencephaly is severe but rare cortical malformation. It is a disorder characterized by a cleft in cerebral mantle, which communicates between the subarachnoid space laterally, and ventricular system medially. Diagnostic modalities are CT scan & MRI. Here we report a case of middle aged female presenting with distinct clinical features & characteristic CT Brain findings. Though the case is rare is a good example for reviewing the literature and coming to diagnosis of schizencephaly.

1.      Yakovlev PI, Wadsworth RC. Schiz-encephalies: A study of the congenital clefts in the cerebral mantle. I. Clefts with fused lips. J Neuropathol Exp Neuro 1946; 5: 116-130.

2.      Yakovlev PI, Wadsworth RC. Schiz-encephalies: A study of the congenital clefts in the cerebral mantle. II Clefts with hydro-cephalus and lips separated. J Neuropathol Exp Neurol 1946: 5: 169-206.

3.      Klingensmith WC, Coiffi-Ragan DT. Schiz-encephaly. Diagnosis and progresssion in utero. Radiology 1986; 159: 617-618.

4.      Komarnski CA, Cyr DR, Mack LA, Wein-berger E. Prenatal diagnosis of Schizence-phaly. J Ultrasound Med 1990; 9: 305-307.

5.      Brunelli S, Faiella A, Capra V, Nigro V, Simeone A, Cama A. Germline mutations in the homebox gene EMX2 in patients with severe schizencephaly. Nat Genet 1996; 12: 94-96

6.      Granata T, Farina L, Faiella A, Cardini R, D’Incerti L, Boncinelli E. Familial schiz-ence-phaly associated with EMX2 mutation. Neuro-logy 1997; 48: 1403-1406.

Dr Harshal Bhitkar

MBBS, MD Medicine

Asst Professor Dept of Medicine, BJGMC Pune