Magnetic Resonance Imaging and Neurobehavioral Correlates in Schizencephaly

Aniskiewicz, Albert S.; Frumkin, Neva L.; Brady, Debbie E.; Moore, James B.; Pera, Abraham

doi:10.1001/archneur.1990.00530080099017

Cited by 44 publications

(18 citation statements)

References 18 publications

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“…A few other similar cases have been reported [3,4]. Our case reinforces the timeless adage to treat the patient and not a test or imaging finding, especially when clinical and functional correlation with a test or imaging finding is variable.…”

supporting

confidence: 77%

Schizencephaly: Dramatic Images in a Normally Functioning Adult

Han

Cheng

Surapaneni

et al. 2015

PM&R

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A 47-year-old man presented after he had a witnessed tonic-clonic seizure while standing on the street. Since childhood he had a history of "cerebral palsy," and after a motor vehicle accident he had leftsided weakness and seizures that began only a month before presentation to us. The patient had been taking levetiracetam but had been noncompliant with this medication for a week prior to the episode of seizure that occurred on the street. After this seizure, he had worsened left-sided weakness. Previous seizures were notable for ensuing extended Todd's paralysis lasting more than a day. Prior to presentation the patient was living alone, independent with activities of daily living and ambulation, and worked as a restaurant cook. Examination revealed that the patient had normal cognition and speech but significant left-side weakness with increased tone and shortened limbs.

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confidence: 77%

Schizencephaly: Dramatic Images in a Normally Functioning Adult

Han

Cheng

Surapaneni

et al. 2015

PM&R

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“…;i0 The gray mat¬ ter lining these clefts is usually abnor¬ mal, in the form of polymicrogyria (small, irregular gyri without interven¬ ing sulci).30 Schizencephaly most com¬ monly involves the parasylvian region and the precentrai and postcentral gyrus. 31 Pathologic studies suggest that schizencephaly results from severe hemispheric injury early in the second trimester.32 The specific mechanism of injury may vary from case to case.32 An association between septo-optic dyspla¬ sia and schizencephaly has been the subject of a number of recent reports.14·3335…”

Section: Resultsmentioning

confidence: 99%

Optic Nerve Hypoplasia

1993

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“…[3][4][5][6] The same applies to language abilities. 14 The two patients with left parasylvian cleft had linguistic deficits that were more pronounced in the left-related syntactic aspect of language production and comprehension, even if speech abilities appeared to be normal in informal conversational language. In unilaterally affected patients, language impairment was related to the presence of open-lip clefts, to the frontoparietal location, and to the extent of the malformation rather than to the side of the cleft.…”

Section: Clinical Manifestationsmentioning

confidence: 96%

“…The term "schizencephaly" was introduced in two articles by Yakovlev and Wadsworth, who reported a detailed neuropathologic analysis of five autopsied patients with a complex brain malformation and severe mental and neurologic deficits. The reported studies include case series that correlated imaging and clinical features [3][4][5][6] and series or single case reports more focused on specific topics, such as imaging, 7,8 functional neuroimaging, [9][10][11][12][13] neuropsychology, 14,15 epilepsy features, 16,17 surgical treatment, 18,19 and etiopathogenesis. 1,2 As for other malformations of cortical development, schizencephaly has been increasingly recognized in vivo owing to the recent refinement of neuroimaging.…”

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confidence: 99%

Topical Review: Schizencephaly: Clinical Spectrum, Epilepsy, and Pathogenesis

et al. 2005

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After almost 60 years since the original description, we have reviewed the results of the more recent studies on schizencephaly in an attempt to delineate its imaging and clinical spectra of presentation and to point out the still unsettled controversies on its pathogenesis. The clinical picture is mainly based on the presence of motor deficits and mental retardation, but the severity of the clinical picture is extremely variable, mainly related to the size and location of the clefts and to the presence of associated cerebral malformations. By contrast, the outcome of epilepsy, which is present in about half of the cases and drug resistant in a third, is not strictly related to the severity of the malformation. Some clinical and functional magnetic resonance imaging studies have suggested that, beside the features of the anatomic damage, the functional reorganization of a malformed and unaffected cortex is most likely crucial in determining the clinical outcome. Review of the genetic studies and the more recent personal data suggests that the role of the EMX2 gene in schizencephaly, if any, is restricted to a minority of cases, leaving the etiopathogenesis of this brain malformation still a matter of study and debate.

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Magnetic Resonance Imaging and Neurobehavioral Correlates in Schizencephaly

Cited by 44 publications

References 18 publications

Schizencephaly: Dramatic Images in a Normally Functioning Adult

Schizencephaly: Dramatic Images in a Normally Functioning Adult

Optic Nerve Hypoplasia

Topical Review: Schizencephaly: Clinical Spectrum, Epilepsy, and Pathogenesis

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