Congenital Bilateral Perisylvian Syndrome: Familial Occurrence, Clinical and Psycholinguistic Aspects Correlated with MRI

Brandão-Almeida, Iara Lêda; Hage, Simone Rocha de Vasconcellos; Oliveira, Ecila Paula dos Mesquita de; Guimarães, Catarina Abraão; Teixeira, Karine Couto Sarmento; Abramides, Dagma Venturini Marques; Montenegro, Maria Augusta; Santos, Nuno M.S. dos; Cendes, Fernando; Lopes-Cendes, Íscia; Guerreiro, Marilisa M.

doi:10.1055/s-0028-1085462

Cited by 21 publications

(27 citation statements)

References 25 publications

(41 reference statements)

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“…Histologic characteristics of these malformations strongly suggested a genesis in prenatal development, since they revealed abnormal placement of neurons within cortical layers (i.e., malformations including ectopias and microgyria). More recently, similar findings have been reported for individuals with developmental language impairment (Oliveira et al, 2007; Brandão-Almeida et al, 2008; Boscariol et al, 2010, 2011). Initially, these findings were thought to implicate a relationship between clinical diagnosis and specific disruption of fronto-temporal regions critical to language processing (since the distribution of anomalies in the affected brains was substantially greater in left perisylvian areas).…”

Section: Auditory Processing Deficits and Language Disability: Human supporting

confidence: 78%

Early neural disruption and auditory processing outcomes in rodent models: implications for developmental language disability

Fitch

Alexander

Threlkeld

2013

Front. Syst. Neurosci.

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Most researchers in the field of neural plasticity are familiar with the “Kennard Principle,” which purports a positive relationship between age at brain injury and severity of subsequent deficits (plateauing in adulthood). As an example, a child with left hemispherectomy can recover seemingly normal language, while an adult with focal injury to sub-regions of left temporal and/or frontal cortex can suffer dramatic and permanent language loss. Here we present data regarding the impact of early brain injury in rat models as a function of type and timing, measuring long-term behavioral outcomes via auditory discrimination tasks varying in temporal demand. These tasks were created to model (in rodents) aspects of human sensory processing that may correlate—both developmentally and functionally—with typical and atypical language. We found that bilateral focal lesions to the cortical plate in rats during active neuronal migration led to worse auditory outcomes than comparable lesions induced after cortical migration was complete. Conversely, unilateral hypoxic-ischemic (HI) injuries (similar to those seen in premature infants and term infants with birth complications) led to permanent auditory processing deficits when induced at a neurodevelopmental point comparable to human “term,” but only transient deficits (undetectable in adulthood) when induced in a “preterm” window. Convergent evidence suggests that regardless of when or how disruption of early neural development occurs, the consequences may be particularly deleterious to rapid auditory processing (RAP) outcomes when they trigger developmental alterations that extend into subcortical structures (i.e., lower sensory processing stations). Collective findings hold implications for the study of behavioral outcomes following early brain injury as well as genetic/environmental disruption, and are relevant to our understanding of the neurologic risk factors underlying developmental language disability in human populations.

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Section: Auditory Processing Deficits and Language Disability: Human supporting

confidence: 78%

Early neural disruption and auditory processing outcomes in rodent models: implications for developmental language disability

Fitch

Alexander

Threlkeld

2013

Front. Syst. Neurosci.

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“…The most consistent findings were ectopy and occasional microgyria, mainly affecting the perisylvian region of the left hemisphere4. Magnetic resonance imaging identified perisylvian polymicrogyria in families with histories of language and learning disabilities5, 6.…”

Section: Introductionmentioning

confidence: 99%

Processamento temporal auditivo: relação com dislexia do desenvolvimento e malformação cortical

Boscariol¹,

Guimarães²,

Hage

et al. 2010

Pró-Fono R. Atual. Cient.

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Background: temporal auditory processing and developmental dyslexia. Aim: to characterize the temporal auditory processing in children with developmental dyslexia and to correlate findings with cortical malformations. Method: twenty school-aged children, ranging in age from 8 to 14 years were evaluated. These children were divided into two groups: the experimental group (EG) was composed by 11 children (eight were male) with developmental dyslexia and the control group (CG) was composed by nine normal children (six were male). After neurological assessment and verification of the intellectual level, language, reading and writing skills in order to determine the diagnosis, children underwent a peripheral audiological evaluation and Random Gap Detection Test and/or Random Gap Detection Test Expanded. Results: a statistically significant difference between children in the EG and CG were observed, with children in the EG presenting worst performances. Most of the children in the EG presented perisylvian polymicrogyria. Conclusion: children with developmental dyslexia may present temporal auditory processing disorders with deficits in phonological processing. Cortical malformations may be the anatomical substrate of these disorders. Key Words: Dyslexia; Hearing; Child. ResumoTema: processamento temporal auditivo e dislexia do desenvolvimento. Objetivo: caracterizar o processamento temporal auditivo em escolares com dislexia do desenvolvimento e correlacionar com malformação cortical. Método: foram avaliados 20 escolares, com idade entre 8 e 14 anos, divididos em grupo experimental (GE) composto por 11 escolares (oito do gênero masculino) com o diagnóstico de dislexia do desenvolvimento e grupo controle (GC) composto por nove escolares (seis do gênero masculino) sem alterações neuropsicolinguísticas. Após avaliações neurológica, neuropsicológica e fonoaudiológica (avaliação de linguagem e leitura e escrita) para obtenção do diagnóstico, os escolares foram submetidos à avaliação audiológica periférica e posteriormente aplicou-se o teste Random Gap Detection Test e/ou Random Gap Detection Test Expanded. Resultados: observou-se diferença estatisticamente significante entre os escolares do GE e GC, com pior desempenho para o GE. A maioria dos escolares do GE apresentou polimicrogiria perisylviana. Conclusão: escolares com dislexia do desenvolvimento podem apresentar alterações no processamento temporal auditivo com prejuízo no processamento fonológico. Malformação do desenvolvimento cortical pode ser o substrato anatômico dos distúrbios.

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“…Retrospective (n=2) and prospective (n=4) cohort studies were identified, as well as two case-control studies. 16 Four studies only included participants with a primary presenting problem of language delay 19,28,29 or diagnosis of specific language impairment, 30 now referred to as developmental language disorder. 23 Four studies limited inclusion to participants with IQ/Performance IQ over 70 19,28 or 80, 29 or to individuals without cognitive impairment but did not define this according to a specific IQ.…”

Section: Characteristics Of Included Studiesmentioning

confidence: 99%

“…11 With advances in neuroimaging, confusion in terminology has arisen due to the multiplicity of terms used to describe what is now defined as BPP based on imaging findings. 16,17 Significant language delays and differences between receptive and expressive language scores have been noted in the WDS and congenital bilateral perisylvian syndrome populations. 5 While the term Foix-Chavany-Marie syndrome is typically associated with opercular infarcts, a developmental form has been described which is BPP.…”

mentioning

confidence: 99%

“…These include diagnoses based on imaging, such as bilateral opercular polymicrogyria 12 and bilateral perisylvian dysplasia, 13,14 and diagnosis based on a combination of clinical and imaging factors, such as congenital bilateral perisylvian syndrome. 10,11,16 Receptive language abilities are within the typical to impaired range; 18,19 while expressive language is significantly impaired, 20 with many individuals non-verbal. 2 Given the overlap in clinical phenotype between imaging positive and imaging negative WDS and evidence for a genetic aetiology, consideration of WDS, congenital bilateral perisylvian syndrome, and BPP as a spectrum of perisylvian abnormalities has been proposed.…”

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confidence: 99%

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Speech and language in bilateral perisylvian polymicrogyria: a systematic review

Braden

Leventer

Jansen

et al. 2019

Develop Med Child Neuro

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BPPBilateral perisylvian polymicrogyria WDS Worster-Drought syndrome AIM We aimed to systematically review the speech production, language, and oral function phenotype of bilateral perisylvian polymicrogyria (BPP), and examine the correlation between the topography of polymicrogyria and the severity of speech, language, and oral functional impairment.METHOD A systematic search of MEDLINE, Embase, and PubMed databases was completed on 26th October 2017 using Medical Subject Heading terms synonymous with BPP and speech, language, or oral motor impairment. In total, 2411 papers were identified and 48 met inclusion criteria.RESULTS Expressive and receptive language impairment and oral structural and functional deficits are frequent in BPP. Expressive deficits are frequently more severe than receptive. Only one study used formal assessments to demonstrate the presence of speech disorder, namely dysarthria. Seven studies reported an association between diffuse BPP and more severe language impairment.INTERPRETATION Findings confirmed that language deficits are common in BPP, though assessment of the specific speech phenotype is limited. The paucity of high quality studies detailing the specific communication phenotype of BPP highlights the need for further investigation. Improving understanding of this phenotype will inform the development of targeted therapies and lead to better long-term outcomes.

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Congenital Bilateral Perisylvian Syndrome: Familial Occurrence, Clinical and Psycholinguistic Aspects Correlated with MRI

Abstract: The severity of clinical manifestations in CBPS is correlated with the extent of cortical involvement. Most patients with CBPS have a history of speech delay or language difficulties and no epilepsy. Dyslexia can be found in patients with PMG.

Cited by 21 publications

References 25 publications

Early neural disruption and auditory processing outcomes in rodent models: implications for developmental language disability

Early neural disruption and auditory processing outcomes in rodent models: implications for developmental language disability

Processamento temporal auditivo: relação com dislexia do desenvolvimento e malformação cortical

Speech and language in bilateral perisylvian polymicrogyria: a systematic review

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