Congenital Bilateral Perisylvian Syndrome: Familial Occurrence, Clinical and Psycholinguistic Aspects Correlated with MRI (original) (raw)
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Epilepsia, 2001
Purpose: The main features of congenital bilateral perisylvian syndrome (CBPS) are pseudobulbar palsy, cognitive deficits, epilepsy, and perisylvian abnormalities on imaging studies, however, the clinical spectrum of this syndrome is much wider than previously believed and may vary from minor speech difficulties to severely disabled patients. The objective of this study was to present the different imaging and clinical findings of 17 patients with CBPS, their genetic background, and the occurrence of prenatal injury during their pregnancies.
Neurological Sciences, 2010
We present the neuropsychological and linguistic follow-up of a girl with bilateral perisylvian polymicrogyria during 4 years of gestural and verbal speech therapy. Some researchers have suggested that children with bilateral perisylvian polymicrogyria mentally fail to reach the syntactic phase and do not acquire a productive morphology. This patient achieved a mean length of utterance in signs/gestures of 3.4, a syntactic phase of completion of the nuclear sentence and the use of morphological modifications. We discuss the link between gesture and language and formulate hypotheses on the role of gestural input on the reorganization of compensatory synaptic circuits.
Caracterização das manifestações lingüísticas de uma família com Síndrome Perisylviana
Pró-Fono Revista de Atualização Científica, 2005
Background: perisylvian syndrome refers to a variety of clinical manifestations associated to lesions in the perisylvian or opercular regions. Polymicrogyria is the most common structural malformation found. The syndrome may be inherited and the clinical spectrum includes subtle language disturbances on one end and more severe characteristics such as prominent pseudobulbar signs and refractory epilepsy on the other end. Other studies have already associated perisylvian polymicrogyria with developmental language disorders or specific language impairment. Aim: to describe the language deficits of four members of a family with Perisylvian Syndrome, and to correlate these deficits to neuroimaging data Method: the patients underwent neuroimaging investigation, psychological assessment using the Weschler Intelligence Scales, and specific speech-language evaluation. The following tests were used for the assessment of vocabulary, phonology, syntax, pragmatics, reading and writing: Thematical Pictures of Yavas, ABFW -Child Language Test, Peabody Picture Vocabulary Test (PPVT), and other specific protocols. Results: magnetic resonance imaging revealed perisylvian polymicrogyria in all of the subjects, with varied locations and extensions. Speech-language assessment indicated significant oral and written language deficits in all of the subjects. Conclusion: the obtained data indicate that language impairment can co-exist with reading deficits in members of the same family. Neuroimaging findings reveal cortical alterations that are associated to specific language impairments within the spectrum of the Perisylvian Syndrome. Another important aspect evidenced by this study is the similarities in the language profiles of siblings and mother, suggesting that a variety of linguistic manifestations exist within the spectrum of the syndrome. Perisylvian polymicrogyria can be one of the neurobiological malformations involved in the manifestation of these deficits. OLIVEIRA, E. P. M. de.; GUERREIRO, M. M.; GUIMARÃES, C. A.; BRANDÃO-ALMEIDA, I. L.; MONTENEGRO, M. A.; CENDES, F.; HAGE, S. R. V. Characterization of the linguistic profile of a family with Perisylvian Syndrome (original title: Caracterização das manifestações lingüísticas de uma família com Síndrome Perisylviana). Pró-Fono Revista de Atualização Científica, Barueri (SP), v. 17, n. 3, p. 393-402, set.-dez. 2005.
Speech and language in bilateral perisylvian polymicrogyria: a systematic review
Developmental Medicine & Child Neurology, 2019
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Journal of Neurology, 2014
Congenital bilateral perisylvian syndrome (CBPS) presents with heterogeneous clinical manifestations such as pseudobulbar palsy, language disorder, variable cognitive deficits, epilepsy, and perisylvian abnormalities (most frequently polymicrogyria) on imaging studies. We investigated the relationship between seizures and extent of gray matter (GM) and white matter (WM) abnormalities using voxel-based morphometry (VBM) of brain magnetic resonance imaging (MRI) as well the association between seizures, structural abnormalities and cognitive ability. In this cross-sectional study, we evaluated 51 healthy volunteers and 18 patients with CBPS with epilepsy (seizure group, n = 7) and without (non-seizure group, n = 11). We used VBM (SPM8/DARTEL) to investigate areas with excess and atrophy of both gray and white matter, comparing groups of patients with controls. Intellectual ability of patients was assessed by the WISC-III or WAIS-III. Both groups with CBPS and the control group were homogeneous with respect to gender (p = 0.07) and age (p = 0.065). Besides perisylvian polymicrogyria, the seizure group exhibited areas with GM and WM reduction including temporal, frontal, parietal and occipital lobes. In contrast, we identified fewer areas with GM and WM reduction in the non-seizure group. The seizure group presented worse intellectual performance (performance IQ and global IQ) than the non-seizure group. The seizure group presented with a more widespread pattern of cortical and sub-cortical abnormalities, as well as worse cognition. Our results suggest that patients with CBPS and epilepsy appear to have widespread neuronal damage that goes beyond the areas with MRI-visible perisylvian polymicrogyria.
Auditory processing disorder in perisylvian syndrome
Brain and Development, 2010
We hypothesized that the processing of auditory information by the perisylvian polymicrogyric cortex may be different from the normal cortex. To characterize the auditory processing in bilateral perisylvian syndrome, we examined ten patients with perisylvian polymicrogyria (Group I) and seven control children (Group II). Group I was composed by four children with bilateral perisylvian polymicrogyria and six children with bilateral posterior perisylvian polymicrogyria. The evaluation included neurological and neuroimaging investigation, intellectual quotient and audiological assessment (audiometry and behavior auditory tests). The results revealed a statistically significant difference between the groups in the behavioral auditory tests, such as, digits dichotic test, nonverbal dichotic test (specifically in right attention), and random gap detection/random gap detection expanded tests. Our data showed abnormalities in the auditory processing of children with perisylvian polymicrogyria, suggesting that perisylvian polymicrogyric cortex is functionally abnormal. We also found a correlation between the severity of our auditory findings and the extent of the cortical abnormality.
Congenital unilateral perisylvian syndrome: radiological basis and clinical correlations
Journal of Neurology Neurosurgery and Psychiatry, 1996
DESIGN--Advances in neuroimaging have allowed correlations between radiological patterns and clinical features of brain malformations. This paper reports clinical, prognosis, and electroencephalographic features of six children with a previously unrecognised neuroimaging picture of unilateral widening and verticalisation of the sylvian fossa associated with an abnormal ipsilateral perisylvian cortex. RESULTS--All children had reduced hemisphere size and thalamostriatal hypoplasia ipsilateral to the
Congenital Bilateral Perisylvian Syndrome: A Case Report
Journal of the Turkish Epilepsi Society, 2014
Konjenital bilateral perisilviyan sendrom (KBPS) epilepsi, psödobulber paralizi, kognitif hasar ve beyin görüntülemesinde iki taraflı perisilviyan anormalliklerle karakterize bir nöronal migrasyon bozukluğudur. Bu yazıda tedaviye dirençli nöbetler ve düşme atakları ile başvuran ve detaylı öykü, nörolojik muayene ve manyetik rezonans görüntüleme (MRG) bulguları ile KBPS tanısı almış 44 yaşında bir erkek olgu sunuldu. On üç yaşından beri nöbet şikayeti olan hastanın antiepileptik tedaviye rağmen ayda bir olan jeneralize tonik klonik nöbetleri ve her gün olan tonik/atonik düşme atakları mevcuttu. Nörolojik muayenesinde sağ tarafta daha belirgin olan piramidal bulgular, psödobulber paralizi ve hafif kognitif bozulma mevcuttu. İnteriktal elektroensefalografisinde iki taraflı frontotemporal keskin yavaş dalga deşarları mevcuttu. MRG'sinde iki taraflı perisilviyan local kortikal kalınlaşma ve polimikrogri mevcuttu. Tedaviye dirençli nöbetleri olan hastalar mutlaka spesifik bir sendrom olasılığı açısından beyin görüntülemesini de içeren detaylı bir inceleme yapılarak değerlendirilmelidir. Tedaviye dirençli nöbetler, psödobulber bulgular ve mental retardasyon KBPS tanısını destekler. Beyin MRG incelemesi bu sendromun tanısında önemli role sahiptir.
A case of Bilateral Perisylvian Syndrome with reading disability
Cortex, 2016
Bilateral Perisylvian Syndrome (BPS) often presents with epilepsy and significant behavioral impairments that can include mental retardation, dysarthria, delayed speech development, and delayed fine motor development (Graff-Radford et al., 1986; Kuzniecky et al., 1993). While a small subset of BPS cases have been described as having relatively isolated language delays (Leventer et al., 2010), BPS is not expected in children with dyslexia. As part of a Medical University of South Carolina IRB approved multi-site study involving retrospective and de-identified dyslexia data, we unexpectedly identified a 14.05 year old male with evidence of BPS whose father had been diagnosed with dyslexia and dysgraphia. This child had been recruited for a neuroimaging study on dyslexia from a school specializing in educating children with dyslexia. The T1-weighted MRI scan from this child demonstrated a highly unusual perisylvian sulcal/gyral patterning that is a defining feature of BPS (Figure 1). BPS cases exhibit bilateral dysgenesis of the Sylvian fissure and surrounding gyri, which appears to occur because of a limited or absent arcuate fasciculus (Kilinc et al., 2015). This BPS case also had a relatively enlarged atrium of the lateral ventricle that is consistent with the BPS anatomical presentation and reduction of parietal white matter (Graff-Radford et al., 1986; Kilinc et al., 2015; Toldo et al, 2011). An automated analysis of SPM8 segmented T1-weighted MRI scans was used to determine the degree to which the BPS native space white matter image (rigidly aligned and nonnormalized) exhibited gross morphological dis-similarity to white matter images from age and gender matched cases. This image covariance approach provided an average similarity measure for the BPS case and images from 10 control and 19 reading disabled cases across 7 research sites who were selected from 172 males enrolled in reading disability studies, had