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

Granata, Tiziana; Freri, Elena; Caccia, Claudio; Setola, Veronica; Taroni, Franco; Battaglia, Giorgio

doi:10.1177/08830738050200040801

Cited by 88 publications

(62 citation statements)

References 36 publications

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“…Both genetic and acquired causes such as teratogens and infections are implicated in this malformation. There is a correlation (although with significant variability) of motor deficits and mental retardation with the size and www.pmrjournal.org location of clefts; however, the severity of epilepsy does not necessarily correlate well with imaging findings [1,2].…”

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confidence: 91%

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: 91%

Schizencephaly: Dramatic Images in a Normally Functioning Adult

Han

Cheng

Surapaneni

et al. 2015

PM&R

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“…10,[18][19][20] It is still not clear if there is a spectrum of severity regarding epilepsy features. We found no meaningful difference in the frequency of epilepsy and seizure control in open-lip versus closed-lip schizencephaly.…”

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confidence: 99%

Epileptic Features of Patients With Unilateral and Bilateral Schizencephaly

et al. 2006

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The extent of cortical maldevelopment might correlate with the severity of the clinical manifestation, such as cognitive delay or motor dysfunction. The objective of this study was to investigate the clinical features of epilepsy in patients with unilateral and bilateral schizencephaly. We studied 44 consecutive patients with schizencephaly diagnosed by magnetic resonance imaging (MRI). The epileptic features were analyzed in detail: frequency of epilepsy, median age at onset of epilepsy, semiology of seizures, characteristic features of electroencephalographic (EEG) abnormalities, epileptic syndromes, and antiepileptic drug treatment. We also verified the presence of motor disabilities. Data were analyzed according to the presence of unilateral or bilateral clefts and to the presence of open-lip versus closed-lip schizencephaly. We used the chi-square test and Fisher exact test for statistical analysis. Twenty-four patients had a unilateral cleft (group 1) and 20 patients had bilateral clefts (group 2). Ages ranged from 1 to 37 years (mean 10.6 years). Epilepsy was present in 15 (63%) patients in group 1 and in 11 (55%) patients of group 2; a history of status epilepticus occurred in 13% of group 1 and in 27% in group 2; and a history of clusters of seizures occurred in 40% of group 1 and in 45% of group 2. Eight (53%) patients in group 1 and 6 (54%) patients in group 2 were in monotherapy. Ten (67%) patients in group 1 and 7 (64%) patients in group 2 had seizures controlled with antiepileptic drugs. The frequency of EEG abnormalities was similar between groups (75% and 85%, groups 1 and 2, respectively). Statistical analysis showed no difference between the two groups in the variables mentioned above. However, motor disability was significantly more frequent and more severe in group 2. Regarding the type of schizencephaly (open lip versus closed lip), there was no difference in the frequency of patients with epilepsy, and severe motor deficit was more frequently found in bilateral and open-lip schizencephaly. The extent of the cortical maldevelopment in patients with schizencephaly does not correlate with the severity of the clinical and EEG features of epilepsy, unlike the cognitive and motor manifestations. In addition, the type of schizencephaly (open lip versus closed lip) does not correlate with the presence of epilepsy or seizure control, unlike motor deficit.

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“…8 Epilepsy mainly with partial, occasionally generalized seizures is a common complication of schizencephaly, probably because of intrinsic epileptogenicity of dysplastic cortex. 2,3,8,9,11 Two patients with unilateral clefts in this study had well-controlled partial seizures, while a patient with bilateral open lip schizencephaly developed infantile spasms followed by partial and generalized seizures refractory to antiepileptic drugs.…”

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confidence: 96%

Epidemiology and Clinical Spectrum of Schizencephaly in South-Eastern Hungary

et al. 2010

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The epidemiology and clinical spectrum of schizencephaly in south-eastern Hungary have been surveyed in a retrospective population-based study. A total of 10 patients (6 boys and 4 girls) were found with schizencephaly among 185 486 live births in a period of 14 years (July 1, 1992 to June 30, 2006), which means a birth prevalence of 0.54 per 10 000 (95% confidence interval [CI]: 0.20-0.87). The schizencephaly was unilateral in 7 cases (with closed lips in 5 and open lips in 2 patients) and bilateral in 3 children (with closed lips in 2 and open lips in 1). The septum pellucidum was absent in 5 cases; however, optic nerve hypoplasia was not found in these patients. Delayed development and intellectual disability were observed in all patients, except 2 with unilateral closed lip schizencephaly. Epilepsy was diagnosed in 3 patients (2 with unilateral and 1 with bilateral schizencephaly).

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

Cited by 88 publications

References 36 publications

Schizencephaly: Dramatic Images in a Normally Functioning Adult

Schizencephaly: Dramatic Images in a Normally Functioning Adult

Epileptic Features of Patients With Unilateral and Bilateral Schizencephaly

Epidemiology and Clinical Spectrum of Schizencephaly in South-Eastern Hungary

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