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Type I schizencephaly: CT and neuropathologic findings
AJNR. American Journal of Neuroradiology
|May 1, 1987
Summary
Type I schizencephaly, a rare brain malformation, presents unique CT findings distinct from Type II. This condition, linked to early gestation neuronal migration disorders, requires careful differentiation from acquired brain lesions.
Area of Science:
- Neurology
- Radiology
- Developmental Biology
Background:
- Type I schizencephaly is less recognized than Type II, characterized by a fused cleft in the cerebral mantle.
- Its computed tomography (CT) appearance differs significantly from Type II and can be mistaken for acquired lesions.
Purpose of the Study:
- To correlate the CT appearance of Type I schizencephaly with clinical findings and neuropathologic features.
- To differentiate Type I schizencephaly from postdevelopmentally acquired lesions.
Main Methods:
- Retrospective analysis of CT scans from eight patients (seven girls, one boy) with Type I schizencephaly.
- Correlation of CT findings with clinical presentations (developmental delay, seizures, hemiparesis) and neuropathologic data.
Main Results:
- CT revealed a unilateral, parasylvian cleft lined by cortex extending from the surface to the ventricle in all cases.
- Micropolygyria was observed along the cleft cortex in all patients and elsewhere in two.
- Four patients with associated microcephaly exhibited more severe neurologic deficits.
Conclusions:
- CT findings in Type I schizencephaly are characteristic and correlate well with neuropathology.
- The condition results from a neuronal migration disorder in early gestation.
- It should not be misdiagnosed as a postdevelopmentally acquired lesion.