Interictal EEG findings in two cases with ‘double cortex’ syndrome

Parmeggiani, Antonia; Santucci, Margherita; Ambrosetto, Paolo; Amadi, A.; Baioni, Elisabetta; Rossi, P.

doi:10.1016/0387-7604(94)90031-0

Cited by 13 publications

(16 citation statements)

References 8 publications

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“…About one third of patients with SBH have an association of tonicclonic and myoclonic seizures with atypical absences and drop attacks. [10][11][12][13] However, focal clinical signs such as head deviation or clonic movements of one limb at seizure onset observed in some individuals suggest the diagnosis of focal partial epilepsy. [10][11][12] Infantile spasms and Lennox-Gastaut syndrome also have been reported.…”

Section: Introductionmentioning

confidence: 99%

“…[10][11][12][13] However, focal clinical signs such as head deviation or clonic movements of one limb at seizure onset observed in some individuals suggest the diagnosis of focal partial epilepsy. [10][11][12] Infantile spasms and Lennox-Gastaut syndrome also have been reported. [14] About 60% of the patients have focal lobar or multifocal epileptic abnormalities, [10,11,14] but EEG findings are usually characterized by generalized slow spike-and-wave or polyspike and wave, and multifocal spiking.…”

Section: Introductionmentioning

confidence: 99%

“…[14] About 60% of the patients have focal lobar or multifocal epileptic abnormalities, [10,11,14] but EEG findings are usually characterized by generalized slow spike-and-wave or polyspike and wave, and multifocal spiking. [11][12][13] In individuals with SBH, cognitive abilities range from normal to learning disabilities and/or severe intellectual disability. Behavior problems may also be observed.…”

Section: Introductionmentioning

confidence: 99%

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Novel Mutation chrX:110644366 C>A of the DCX Gene in 4-year-old Girl with Sporadic Double Cortex Syndrome

Shnayder¹,

Artyukhov²,

Егорова³

et al. 2017

Int J Biomed

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Subcortical band heterotopia (SBH), also known as double cortex syndrome (DC), is listed as a "rare disease" by the Genetic and Rare Diseases Information Center of the National Institutes of Health with an incidence of 1 to 200,000 people in the population. The cause of the disease is mutation in the DCX gene (also known as DBCN, XLIS) on chromosome Xq22.3-q23. SBH is an X-linked dominant disorder. Traditionally, genetic testing for SBH has been done in the order of the probability of detection of mutation according to the radiologic features, but the success rate could be variable with this time-consuming approach.In this study, novel mutation chrX: 110644366C>A in the DCX gene was identified in a 4-year-old Russian girl with sporadic SBH. The present report demonstrates that whole exome sequencing may be a useful tool for the identification of previously known and de novo mutations in children with SBH as well as malformations of cortical development. (Int J Biomed. 2017;7(1):67-70.)

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Novel Mutation chrX:110644366 C>A of the DCX Gene in 4-year-old Girl with Sporadic Double Cortex Syndrome

Shnayder¹,

Artyukhov²,

Егорова³

et al. 2017

Int J Biomed

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“…Interictal EEG findings correlate with seizure types and severity, and usually include multifocal and generalized epileptiform discharges as well as generalized slowing (2,4,5). BH can result in Lennox-Gastaut syndrome (3,5,10), and most often produces the clinical picture of a symptomatic generalized epilepsy.…”

mentioning

confidence: 99%

“…Like other NMDs, BH is associated with seizures and mental retardation. Intractable seizures and severe cognitive impairment are most common (1)(2)(3)(4)(5), but clinically milder cases occur (6)(7)(8)(9).…”

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confidence: 99%

Ictal EEG Patterns in Band Heterotopia

Grant

Rho

2002

Epilepsia

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Summary: Band heterotopia (BH) or "double cortex" syndrome is a neuronal migration disorder resulting in a diffuse band of subcortical grey matter and variable abnormality of the overlying cortex. Patients with BH have a spectrum of psychomotor delay and seizures. Associated epileptic syndromes and interictal EEG findings have been described, but ictal EEG patterns are lacking.Methods: We describe the clinical, interictal, and ictal EEG findings in two girls with BH and intractable seizures.Results: Ictal EEG patterns correlated well with clinical seizure types, and did not have features unique to BH. Similarly, seizure behaviors and interictal EEG findings were typical of those seen in symptomatic generalized epilepsies.Conclusions: Despite evidence implicating the ectopic grey matter in seizure discharges, we conclude that seizure semiology and associated ictal EEG patterns in BH are no different from those seen in other causes of symptomatic generalized epilepsies.

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ILAE neuroimaging task force highlight: Subcortical laminar heterotopia

Kasper,

Archer,

Bernhardt

et al. 2024

Epileptic Disorders

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The ILAE Neuroimaging Task Force publishes educational case reports that highlight basic aspects of neuroimaging in epilepsy consistent with the ILAE's educational mission. Subcortical laminar heterotopia, also known as subcortical band heterotopia (SBH) or “double cortex,” is an intriguing and rare congenital malformation of cortical development. SBH lesions are part of a continuum best designated as agyria‐pachygyria‐band‐spectrum. The malformation is associated with epilepsy that is often refractory, as well as variable degrees of developmental delay. Moreover, in an increasing proportion of cases, a distinct molecular‐genetic background can be found. Diagnosing SBH can be a major challenge for many reasons, including more subtle lesions, and “non‐classic” or unusual MRI‐appearances. By presenting an illustrative case, we address the challenges and needs of diagnosing and treating SBH patients in epilepsy, especially the value of high‐resolution imaging and specialized MRI‐protocols.

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Interictal EEG findings in two cases with ‘double cortex’ syndrome

Cited by 13 publications

References 8 publications

Novel Mutation chrX:110644366 C>A of the DCX Gene in 4-year-old Girl with Sporadic Double Cortex Syndrome

Novel Mutation chrX:110644366 C>A of the DCX Gene in 4-year-old Girl with Sporadic Double Cortex Syndrome

Ictal EEG Patterns in Band Heterotopia

ILAE neuroimaging task force highlight: Subcortical laminar heterotopia

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