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Daniel L. Silbergeld and John W. Miller

heterotopias. 2, 6 Past reports have stressed that most patients with this disorder have significant neurological impairment and often paresis and subnormal intelligence. 9, 10, 16, 17 With the advent of magnetic resonance (MR) imaging, recognition of schizencephaly in neurologically normal subjects has become common. 7, 8, 10 Furthermore, unilateral clefts seem to occur more frequently than the bilateral form initially described. 9, 16, 17 To investigate the role of resective surgery for epilepsy in these patients and identify the relationship between the region of

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Gregory G. Heuer, Douglas A. Hardesty, Kareem A. Zaghloul, Erin M. Simon Schwartz, A. Reghan Foley and Phillip B. Storm

S chizencephaly is a rare congenital brain malformation characterized by unilateral or bilateral cerebral clefts delineated by gray matter that spans the pial and ependymal surfaces. 22 , 23 The prevalence of schizencephaly is ~ 1.5/100,000 live births. 6 The lesions are often classified according to the relationship of the ventricle to the cleft as open-lip or closed-lip schizencephaly. These malformations can also be associated with hydrocephalus, particularly in the open-lip form; thus, it is not uncommon for patients with schizencephaly to require VP

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R. Shane Tubbs, Cuong J. Bui, Marios Loukas, Mohammadali M. Shoja and W. Jerry Oakes

lowering the patient's seizure threshold. After advising the parents that the results following rhizotomy were uncertain due to the diagnosis of holoprosencephaly, informed consent was obtained. F ig . 1. Magnetic resonance images showing dysgenesis of the forebrain in Case 1 (left) and left-sided schizencephaly in Case 2 (right) . Operation The patient underwent an S-1 laminectomy and L1–4 laminotomy, and electrical stimulation (1.2–1.9 mA for 0.2 msec) was applied to the L2–S2 nerve roots bilaterally. Approximately 50 to 80% of the L2–S2 dorsal roots

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Martin Staudt, Ingeborg Krägeloh-Mann, Hans Holthausen, Christian Gerloff and Wolfgang Grodd

polymicrogyria † lt frontoparietotemporal absent septum rt 2 (−) ED 16.5 msec; 58% − 17.9 msec; 45% pellucidum, bilat optic nerve hypoplasia 2 20 ment ret vpa, cbz complex ‡ rt frontoparietal − lt 2 (−) FDI 21.2 msec ; ∼80% − 22.2 msec; ∼70% 3 20 normal none schizencephaly lt frontoparietal absent septum rt 2 (+) ED − 17.1 msec; 49% 17.7 msec; 50%  (closed-lip) pellucidum 4 21

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Benjamin C. Warf, Sarah Tracy and John Mugamba

) history or findings on imaging or at the time of ventriculoscopy that suggested a possible infectious cause of the hydrocephalus, including scarred choroid plexus; 2) an open aqueduct or an aqueduct obstructed by a membrane or cyst rather than stenosis; 3) severe malformations of the cerebral hemispheres including hydranencephaly, significant segments of undeveloped brain, or schizencephaly; 4) myelomeningocele, encephalocele, Dandy-Walker complex, or tumor; or 5) previous shunt insertion. Prior to 2002, infants underwent cranial ultrasonography for preoperative and

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Bernard S. Patrick

pinching off of glia, or of glia streaming through pial defects, or by direct invasion of the pia; they suggested that these heterotopias often appear at a much later stage of fetal development, and even after maturity. Yakovlev and Wadsworth 9, 10 in discussing schizencephalies (true clefts formed in the brain as the result of a failure of development of the cerebral mantle in the zones of cleavage of the primary cerebral fissures) emphasized the embryonal origin of these symmetrical clefts, heterotopia of cerebral substance in the subarachnoid space, and other

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Eric M. Jackson and Alan R. Cohen

A 3- month-old boy presented to the neurosurgery clinic with an enlarging head circumference, anterior fontanelle fullness, and cranial suture splaying. He was born at 34 weeks’ gestation with corpus callosum agenesis and multiple congenital abnormalities, including Dandy-Walker malformation, vertex and skull base meningoceles, schizencephaly, subependymal heterotopias, and cleft lip and palate. The vertex meningocele was repaired at age 1 week, and a right frontal ventriculoperitoneal shunt was placed at age 3 months, with stabilization of his hydrocephalus

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Paul Jakubiak, Rembrandt H. Dunsmore and Ronald S. Beckett

cases, so that porencephaly is ruled out. Yakovlev, et al., 22, 23 have described cleft-like symmetrical hemispheral lesions which became cyst-like with a concomitant hydrocephalus. They termed this condition “schizencephaly” and ascribed it to a developmental defect of the cerebral mantle. However, they emphasized the presence of micropolygyria adjacent to the lesion as an almost constant finding. The cases that they described at postmortem examination were imbeciles during life. This was not so in our patients. Davison 4 described the case of a 70-year-old man

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Lucio Palma

case associated with multiple nests of neuroglia tissue in the meninges of the spinal cord. J Med Res 16 : 495 – 520 , 1907 Wolbach SB: Congenital rhabdomyoma of the heart. Report of a case associated with multiple nests of neuroglia tissue in the meninges of the spinal cord. J Med Res 16: 495–520, 1907 30. Yakovlev PI , Wadsworth RC : Schizencephalies: a study of congenital clefts in cerebral mantle; clefts with fused lips. J Neuropathol Exp Neurol 5 : 116 – 130 , 1946 Yakovlev PI, Wadsworth RC

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Martin H. Weiss, Harold F. Young and Dee E. McFarland

; Uber Experimentelle Hydranencephalie (Blasenhirn). Dt. Z. Nervenheilk. , 1949, 161: 446–505. 2. Beswick , W. F. Schizencephalies with hydrocephalus; clinical diagnosis of severe cerebral agenesis. N. Y. St. J. Med. , 1948 , 48 : 2364 – 2366 . Beswick , W. F. Schizencephalies with hydrocephalus; clinical diagnosis of severe cerebral agenesis. N. Y. St. J. Med. , 1948, 48: 2364–2366. 3. Crome , L. , and Sylvester , P. E. Hydranencephaly. Archs. Dis. Childh. , 1958 , 33 : 235 – 245 . Crome