Schizencephaly

  • Etiology: Abnormality of neuronal migration
  • Imaging MRI:
    — Gray matter lined clefts from ependyma of lateral ventricle to cortical surface – a pial-ependymal seam, with or without abnormal adjacent cortex or gyri
    — Usually dimple at ventricular surface
    — If cleft walls are in apposition = closed lip (Type I)
    — If cleft walls are separated by cerebrospinal fluid = open lip (Type II)
    — Unilateral or bilateral
    — Often near pre or post central gyri
    — Associated with optic nerve hypoplasia and absent septum pellucidum and septo-optic dysplasia
  • DDX:
  • Complications:
  • Treatment:
  • Clinical:
    — Often have seizures or hemiparesis or developmental delay
    — Open lip is more common than closed lip
    — Open lip more commonly bilateral, closed lip more commonly unilateral

Radiology Cases of Schizencephaly

MRI of perisylvian polymicrogyria and open lip schizencephaly
Coronal and axial T1 (above) and axial T2 (below) MRI without contrast of the brain shows small haphazard appearing gyri and too few sulci present bilaterally, left greater than right, primarily in the sylvian fissures. There is also a cleft of gray matter with cerebrospinal fluid within extending from the cortex to the posterior aspect of the left lateral ventricle.
CT of right open lip schizencephaly
Axial CT without contrast of the brain shows a cerebrospinal fluid tract communicating between the right occipital cortex and the posterior horn of the right lateral ventricle that was felt to be lined by gray matter on both sides.