Double Cortex

Ricci, Stefano; Cusmai, Raffaella; Fariello, G.; Fusco, Lucia; Vigevano, Federico

doi:10.1001/archneur.1992.00530250065017

Arch Neurol

1992

DOI: 10.1001/archneur.1992.00530250065017

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Double Cortex

Stefano Ricci

Raffaella Cusmai²,

G. Fariello

et al.

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Cited by 50 publications

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“…While there has been little research investigating cognitive outcome in patients with SBH, the available evidence suggests that intellectual outcome is variable (Ricci et al 1992, Battaglia 1993, Iannetti 1993, Miura et al 1993, Palmini et al 1993, Barkovich et al 1994, Ketonen et al 1994. This variability has been associated with the severity of the disorder (as measured by the thickness of the cortical band), age at onset of seizures, frequency and type of seizures (generalized versus focal seizure activity), and the degree of ventricular enlargement (Barkovich et al 1994, Dobyns et al 1996.…”

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Neuropsychological profile of a 9‐year‐old child with subcortical band heterotopia or‘double cortex’

Jacobs

Anderson

Harvey

2001

Develop Med Child Neuro

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Subcortical band heterotopia (SBH) or‘double cortex’, is a congenital brain abnormality that results from aberrant migration of neurons during development of the cortex. MRI shows a continuous band of heterotopic grey matter located between the cortex and ventricular walls, separated from them by a thin layer of white matter. The condition is quite rare, found predominantly in females, and is occasionally familial with an X‐linked dominant inheritance. Current research has focused on genetic and neurological correlates, with cognitive assessment restricted to a global measure of general intellectual functioning. This paper describes in detail the results of a neuropsychological assessment of a 9‐year‐old female recently diagnosed with SBH. Predominant features were a significantly reduced speed of processing for visuomotor and oral output and reduced immediate registration of information. This difficulty has functional implications affecting skill acquisition, learning in the classroom, and social interaction.

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mentioning

confidence: 99%

Neuropsychological profile of a 9‐year‐old child with subcortical band heterotopia or‘double cortex’

Jacobs

Anderson

Harvey

2001

Develop Med Child Neuro

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Subcortical connections of normotopic and heterotopic neurons in sensory and motor cortices of thetish mutant rat

et al. 1998

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Orthograde and retrograde tracers were used to examine subcortical connections of neurons in the neurological mutant tish rat. This animal exhibits bilateral heterotopia similar to those observed in epileptic humans with subcortical band heterotopia. Terminal varicosities were labeled in the striatum, thalamus, brainstem, and spinal cord following injections of the anterograde tracer biotinylated dextran amine (BDA) into the heterotopic cortex. The general topography of corticothalamic projections was evaluated by injecting the retrograde tracer Fluoro-Gold (FG) into ventral thalamic nuclei. Retrograde labeling of small-to-medium sized neurons was observed in layer VI of topographically restricted portions of the normotopic cortex. Similar appearing cells were labeled in the neighboring portions of the underlying heterotopia; however, these neurons did not display characteristic lamination or radial orientation. Thalamocortical terminals labeled by injecting BDA into the ventroposterolateral nucleus (VPL) were observed primarily in layer IV of the medial aspect of the normotopic somatosensory cortex. In contrast, a radial column of terminals was present in the underlying heterotopia. Typical barrel labeling was found in the lateral aspect of the normotopic somatosensory cortex after injecting the ventroposteromedial nucleus (VPM), whereas more diffuse patches of labeling were observed in the underlying heterotopia. Heterotopic neurons in the tish cortex, thus, exhibit characteristic features of subcortical connectivity. Both normotopic and heterotopic neurons in the tish brain project to appropriate subcortical sites and establish bidirectional topographic connections with the thalamus. These results suggest that primary sensory-motor information is represented in a parallel manner in the normotopic and heterotopic cortices of the tish rat.

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Epilepsy and genetic malformations of the cerebral cortex

Guerrini

Carrozzo²

2001

Am. J. Med. Genet.

188

181

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Malformations of the cerebral cortex are an important cause of developmental disabilities and epilepsy. Here we review those malformations for which a genetic basis has been elucidated or is suspected and the types of associated epilepsy. Schizencephaly (cleft brain) has a wide anatomo-clinical spectrum, including partial epilepsy in most patients. Familial occurrence is rare. Heterozygous mutations in the EMX2 gene were reported in 13 patients. X-linked bilateral periventricular nodular heterotopia (BPNH) consists of typical BPNH with epilepsy in females and prenatal lethality in males. About 88% of patients have partial epilepsy. Filamin A mutations, all leading to a truncated protein, have been reported in three families and in sporadic patients. The most frequent forms of lissencephaly (agyria-pachygyria) are caused by mutations of LIS1. XLIS mutations cause classical lissencephaly in hemizygous males and subcortical band heterotopia (SBH) in heterozygous females. The thickness of the heterotopic band and the degree of pachygyria correlate with the likelihood of developing Lennox-Gastaut syndrome. Mutations of the coding region of XLIS were found in all reported pedigrees and in 38-91% of sporadic female patients with SBH. With few exceptions, children with LIS1 mutations have isolated lissencephaly, with severe developmental delay and infantile spasms. Autosomal recessive lissencephaly with cerebellar hypoplasia, accompanied by severe developmental delay, seizures, and hypotonia has been associated with mutations of the reelin gene. Fukuyama congenital muscular dystrophy is due to mutations of the fukutin gene and is accompanied by polymicrogyria. Febrile seizures and epilepsy with generalized tonic-convulsions appear in about 50% of children but are usually not severe. Tuberous sclerosis (TS) is caused by mutations in at least two genes, TSC1 and TSC2; 75% of cases are sporadic; 60% of patients have epilepsy, manifested in 50% of them as infantile spasms. TSC1 mutations seem to cause a milder disease with fewer cortical tubers and lower frequency of seizures. Among several syndromes featuring polymicrogyria, bilateral perisylvian polymicrogyria had familial occurrence on several occasions. Genetic heterogeneity is likely, including autosomal recessive, X-linked dominant, X-linked recessive inheritance, and association with 22q11.2 deletions. About 65% of patients have severe epilepsy, often Lennox-Gastaut syndrome.

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Double Cortex

Cited by 50 publications

References 10 publications

Neuropsychological profile of a 9‐year‐old child with subcortical band heterotopia or‘double cortex’

Neuropsychological profile of a 9‐year‐old child with subcortical band heterotopia or‘double cortex’

Subcortical connections of normotopic and heterotopic neurons in sensory and motor cortices of thetish mutant rat

Epilepsy and genetic malformations of the cerebral cortex

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