Language abilities and gestural communication in a girl with bilateral perisylvian syndrome: a clinical and rehabilitative follow-up (original) (raw)
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Speech and language in bilateral perisylvian polymicrogyria: a systematic review
Developmental Medicine & Child Neurology, 2019
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Neuropediatrics, 2008
The aim of this study was to correlate the clinical features, psycholinguistic aspects and neuroradiological data of patients with CBPS. Patients and Methods & Ascertainment of patients and their families We performed a detailed clinical investigation of 31 patients and their families. Family histories were obtained and pedigrees were constructed. Twenty-two patients, belonging to six unrelated kindreds, had a familial recurrence of PMG. The criteria for diagnosis included a clinical presentation of pseudobulbar palsy, epilepsy and / or language disorder without hearing defi cits, mental retardation or oral motor / structural abnormalities, and PMG on imaging studies. We systematically interviewed patients and their family members according to a standard detailed questionnaire, emphasizing the family history of problems with phonation and delayed speech,
Epilepsy & Behavior, 2005
Bilateral perisylvian polymicrogyria (BPP) is a malformation of cortical development, frequently associated with severe dysarthria or anarthria. BPP patients are therefore often labeled as severely retarded, but a detailed neuropsychological profile has not been reported to date. In a series of 14 patients, we demonstrated that only a minority had extremely low intelligence, and that some aspects of cognitive function correlated with the extent of the cortical disorganization. Early age at seizure onset correlated positively with Performance IQ scores (P < 0.05) and negatively with the extent of the lesion (P < 0.01), reflecting that patients with more severe BPP are more likely to have early seizure onset, resulting in greater interference with ongoing cognitive development. Receptive and expressive language skills were found to be equally poor. Frontal lobe function and memory abilities were relatively well preserved, suggesting that the observed cognitive profiles were related, at least in part, to specific areas of cortical dysfunction and not only to global dysfunction.
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.
Brain and Language, 2004
Limited research has been conducted on the structure of the pars triangularis (PT) in dyslexia despite functional neuroimaging research finding it may play a role in phonological processing. Furthermore, research to date has not examined PT size in ADHD even though the right inferior frontal region has been implicated in the disorder. Hence, one of the purposes of this study was to examine the structure of the PT in dyslexia and ADHD. The other purposes included examining the PT in relation to overall expressive language ability and in relation to several specific linguistic functions given language functioning often is affected in both dyslexia and ADHD. Participants included 50 children: 10 with dyslexia, 15 with comorbid dyslexia/ADHD, 15 with ADHD, and 10 controls. Using a 2 (dyslexia or not) X 2 (ADHD or not) MANCOVA, findings revealed PT length and shape were comparable between those with and without dyslexia. However, children with ADHD had smaller right PT lengths than those without ADHD, and right anterior ascending ramus length was related to attention problems in the total sample. In terms of linguistic functioning, presence of an extra sulcus in the left PT was related to poor expressive language ability. In those with adequate expressive language functioning, left PT length was related to phonological awareness, phonological short-term memory and rapid automatic naming (RAN). Right PT length was related to RAN and semantic processing. Further work on PT morphology in relation to ADHD and linguistic functioning is warranted.
Journal of Child Language, 2013
Children with pre/perinatal unilateral brain lesions (PL) show remarkable plasticity for language development. Is this plasticity characterized by the same developmental trajectory that characterizes typically developing (TD) children, with gesture leading the way into speech? We explored this question, comparing 11 children with PL-matched to 30 TD children on expressive vocabulary-in the second year of life. Children with PL showed similarities to TD children for simple but not complex sentence types. Children with PL produced simple sentences across gesture and speech several months before producing them entirely in speech, exhibiting parallel delays in both gesture+speech and speech-alone. However, unlike TD children, children with PL produced complex sentence types first in speech-alone. Overall, the gesture-speech system appears to be a robust feature of language-learning for simple-but not complex-sentence constructions, acting as a harbinger of change in language development even when that language is developing in an injured brain.
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.