We report a 6-month-old boy with diffuse hypertonia and developmental delay who had unilateral separated-lip schizencephaly and contralateral polymicrogyria. The contralateral polymicrogyria was associated with an incomplete clefting in that hemisphere. An umbilical cord hamartoma is presumed to have caused hypoperfusion to the early developing brain, resulting in bilateral lesions. (J Child Neurol 2003; 18: 232—234).
Get full access to this article
View all access options for this article.
References
1.
Hayashi N., Tsutsumi Y., Barkovich AJ: Morphological features and associated anomalies of schizencephaly in the clinical population: Detailed analysis of MR images. Neuroradiology2002;44:418—427.
2.
Barkovich AJ : Pediatric Neuroimaging, 3rd ed. Philadelphia, Lippincott Williams & Wilkins, 2000.
3.
Haverkamp F. , Zerres K., Ostertun B., et al: Familial schizencephaly: Further delineation of a rare disorder. J Med Genet1995;32:242—244.
4.
Hilburger AC , Willis JK, Bouldin E., Henderson-Tilton A.: Familial schizencephaly . Brain Dev1993;15:234—236.
5.
Hosley MA, Abroms IF, Ragland RL: Schizencephaly: Case report of familial incidence. Pediatr Neurol1992; 8: 148—150.
6.
Robinson RO: Familial schizencephaly. Dev Med Child Neurol1991;33:1010—1012.
7.
Granata T., Farina L., Faiella A., et al: Familial schizencephaly associated with EMX2 mutation. Neurology1997; 48:1403-1406.
8.
Faiella A., Brunelli S., Granata T., et al: A number of schizencephaly patients including 2 brothers are heterozygous for germline mutations in the homeobox gene EMX2. Eur J Hum Genet1997;5:186—190.
9.
Brunelli S., Faiella A., Capra V., et al: Germline mutations in the homeobox gene EMX2 in patients with severe schizencephaly. Nat Genet1996;12:94—96.
10.
Hayashi N., Tsutsumi Y., Barkovich AJ: Polymicrogyria without porencephaly/schizencephaly. MRI analysis of the spectrum and the preval e n c e o f m a c r o s c o p i c f i n d i n g s i n t h e c l i n i c a l population. Neuroradiology2002; 44:647-655.
11.
Barkovich AJ , Kjos BO: Schizencephaly: Correlation of clinical findings with MR characteristics. AJNR Am J Neuroradiol1992;13:85—94.
12.
Craven C., Ward K.: Embryology, fetus, and placenta: normal and abnormal, in Scott JR, DiSaia PJ, Hammond CB, Spellacy WN (eds): Danforth's Obstetrics and Gynecology, 8th ed. Philadelphia, Lippincott Williams & Wilkins, 1999, 29—46.