Band Brain Heterotopia. Case Report and Literature Review

Franzoni, Emilio; Bernardi, Bruno; Marchiani, Valentina; Crisanti, Angela Francesca; Marchi, R. De; Fonda, Claudio

doi:10.1055/s-2007-979717

Cited by 14 publications

(7 citation statements)

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“…Previous reports have implicated anterior theta activity (4), or intermittent rhythmic delta activity (9) as characteristic components of the interictal EEG in BH. Neither of our patients had such findings, nor are they described in the majority of earlier reports for which detailed EEG data are provided (2,3,5,7,8,13). It also seems unlikely that BH would result in a specific EEG pattern, given the spectrum of anatomic and clinical abnormalities encountered, particularly the variety and severity of seizures.…”

Section: Figmentioning

confidence: 54%

“…In patients with BH, the severity of the associated epileptic syndrome and cognitive impairment often correlate with the degree of abnormal gyration in the outer cortical mantle (5,6,(8)(9)(10). In turn, the severity of gyral abnormality reflects, at least in part, the fraction of neuroblasts arrested during migration and composing the inner band of heterotopic grey matter (1,5,10).…”

Section: Discussionmentioning

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%

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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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Section: Figmentioning

confidence: 54%

Section: Discussionmentioning

confidence: 99%

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Ictal EEG Patterns in Band Heterotopia

Grant

Rho

2002

Epilepsia

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“…Until recently the pathogenesis of heterotopias has been ascnbed to drugs and to environmental, vascular, and genetic causes (4, 7). The majority cases with SCLH concern females (20, 21) and affected males have been described in a minority of cases (1,12,14,19). Des Portes, Pinard and co-worker5 have described mutations in the doublecortin @CX) gene to be the underlying cause of an X-linked SCLH/LIS syndrome (8, 9,21).…”

Section: Pathogenesismentioning

confidence: 99%

Subcortical Laminar Heterotopia in Two Sisters and Their Mother: MRI, Clinical Findings and Pathogenesis

Valk¹,

Snoeck²,

Meiners³

et al. 1999

Neuropediatrics

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MR imaging, clinical data and underlying pathogenesis of subcortical laminar heterotopia (SCLH), also known as band heterotopia, in two sisters and their mother are presented. On MR imaging a different degree of SCLH was found in all three affected family-members. The inversion recovery sequence was considered most useful in the demonstration of the heterotopic band of gray matter and the assessment of cortical thickness. The younger sister presented with epileptic seizures at the age of five months and a delayed achievement of developmental milestones. The older sister of seven years had epileptic seizures since the age of one year, and developmental delay. Their mother has only had one seizure-like episode at the age of 39. Her psychomotor development had been normal. Investigation of DNA samples of the three female family-members revealed a mutation in the X-linked doublecortin gene. Within families with band heterotopia, this gene has also been related to male family members with lissencephaly.

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“…These disorders are characterized by a high heterogeneity of genetic anomalies and brain anatomical defects. Heterotopia can be isolated or co-exist with other brain malformation, including lissencephaly, agyria, microgyria, pachygyria, polymicrogyria, agenesis of the corpus callosum, porencephaly, schizencephaly, and agenesis of the cranial nerves (3)(4)(5). The routine clinical use of magnetic resonance imaging (MRI) of the brain has significantly increased the sensitivity of detection and the accuracy of GMH morphological description (1,6).…”

Section: Introductionmentioning

confidence: 99%

Clinical and genomic findings in brain heterotopia: Report of a pediatric patient cohort from Romania

et al. 2021

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Brain heterotopia is a group of rare malformations with a heterogeneous phenotype, ranging from asymptomatic to a severe clinical picture (drug-resistant epilepsy, severe developmental delay). The etiology is multifactorial, including both genetic and environmental factors. In the present study, a cohort of 15 pediatric patients with brain heterotopia were investigated by clinical examination, electroencephalographic studies, brain imaging, and genomic tests. Most of the patients had epileptic seizures, often difficult to control with one antiepileptic drug; another frequent characteristic in the cohort was developmental delay or intellectual disability, in some cases associated with behavioral problems. The genomic studies revealed an interstitial 22q11.2 microduplication, an anomaly not reported previously in heterotopia patients. Comparing the cohort of the present study with that of a previous series of heterotopia patients, both adult and pediatric, similar aspects, such as the high frequency of drug-resistant epilepsy were observed as well as some differences, such as no systemic malformations and no cases with fatal evolution. The current findings add new data to existing knowledge on a rare heterogeneous disorder. The detailed clinical description, including the epilepsy phenotypes, and genomic profiles bring new insights into a group of disorders, yet to be fully understood.

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Band Brain Heterotopia. Case Report and Literature Review

Cited by 14 publications

References 0 publications

Ictal EEG Patterns in Band Heterotopia

Ictal EEG Patterns in Band Heterotopia

Subcortical Laminar Heterotopia in Two Sisters and Their Mother: MRI, Clinical Findings and Pathogenesis

Clinical and genomic findings in brain heterotopia: Report of a pediatric patient cohort from Romania

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