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General Medicine

The COVID-19 is an acute respiratory syndrome with different reported non-respiratory symptoms, neurological manifestations included. The nervous system impairment during the infection occurs in adults and children, yet the pediatric population has particularities in the clinical course of the disease. We discuss the neurological manifestations of COVID-19 in children, developing cases to be applied in disciplines within a “problem-based” learning methodology. The proceedings of the research were developed with undergraduate medicine students in mind: a literature review on the topic was performed; then observed by the selection of 16 articles reviewed, summarized and discussed. This paper discusses proceedings, challenges and outcomes, of each stage of the research’s agenda. In the literature review, we found many neurological manifestations in the pediatric population. Other aspects relevant to the topic were also found, such as: association of comorbidities, alterations in complementary exams, prognoses and pathophysiological hypotheses. Based on the findings, we created three clinical cases for application in undergraduate studies. We concluded that the topic has great clinical and learning importance, yet more research is needed on the subject.

Adult, Male, Pediatrics, medicine.medical_specialty, Adolescent, craniofacial dysostosis, Acrocephalosyndactylia, neuropsychology, Apert syndrome, Neuropsychological Tests, Language Development, lcsh:RC321-571, Young Adult, 03 medical and health sciences, 0302 clinical medicine, acrocephalosyndactylia, Peabody Picture Vocabulary Test, Magnetic resonance imaging of the brain, medicine, Humans, Child, 030223 otorhinolaryngology, HABILIDADES NA COMUNICAÇÃO, lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry, Wechsler Intelligence Scale for Children, Language Tests, language, medicine.diagnostic_test, business.industry, Craniofacial Dysostosis, Neuropsychology, Crouzon syndrome, Wechsler Adult Intelligence Scale, central nervous system, medicine.disease, Neurology, Child, Preschool, Female, Neurology (clinical), business, and 030217 neurology & neurosurgery

Made available in DSpace on 2018-11-26T17:42:45Z (GMT). No. of bitstreams: 0 Previous issue date: 2017-12-01. Added 1 bitstream(s) on 2019-10-09T18:27:09Z : No. of bitstreams: 1 S0004-282X2017001200862.pdf: 171004 bytes, checksum: e28d62f3f2ac1e95c629831cc3cf7ac6 (MD5) Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) Objective: To characterize patients with syndromic craniosynostosis with respect to their neuropsycholinguistic abilities and to present these findings together with the brain abnormalities. Methods: Eighteen patients with a diagnosis of syndromic craniosynostosis were studied. Eight patients had Apert syndrome and 10 had Crouzon syndrome. They were submitted to phonological evaluation, neuropsychological evaluation and magnetic resonance imaging of the brain. The phonological evaluation was done by behavioral observation of the language, the Peabody test, Token test and a school achievement test. The neuropsychological evaluation included the WISC III and WAIS tests. Results: Abnormalities in language abilities were observed and the school achievement test showed abnormalities in 66.67% of the patients. A normal intelligence quotient was observed in 39.3% of the patients, and congenital abnormalities of the central nervous system were observed in 46.4% of the patients. Conclusion: Abnormalities of language abilities were observed in the majority of patients with syndromic craniosynostosis, and low cognitive performance was also observed. Univ Sao Paulo, Fac Odontol Bauru, Dept Fonoaudiol & Audiol, Bauru, SP, Brazil Univ Sao Paulo, Hosp Reabil Anomalias Craniofaciais, Bauru, SP, Brazil Univ Estadual Paulista, Fac Med Botucatu, Dept Oftalmot Otorrinilaringol & Cirurgia Cabeca, Botucatu, SP, Brazil Univ Estadual Paulista, Fac Med Botucatu, Dept Neurol Psicol & Psiquiat, Botucatu, SP, Brazil Univ Estadual Paulista, Fac Med Botucatu, Dept Oftalmot Otorrinilaringol & Cirurgia Cabeca, Botucatu, SP, Brazil Univ Estadual Paulista, Fac Med Botucatu, Dept Neurol Psicol & Psiquiat, Botucatu, SP, Brazil FAPESP: 2000/080803 CNPq: 307043/2008-8

Adult, Male, medicine.medical_specialty, Bipolar Disorder, Neuropsychological Tests, law.invention, Depressive Disorder, Treatment-Resistant, 03 medical and health sciences, Cognition, 0302 clinical medicine, Physical medicine and rehabilitation, Double-Blind Method, Randomized controlled trial, law, Humans, Medicine, Bipolar disorder, business.industry, Working memory, Neuropsychology, Hamilton Rating Scale for Depression, Middle Aged, medicine.disease, Transcranial Magnetic Stimulation, 030227 psychiatry, Dorsolateral prefrontal cortex, Psychiatry and Mental health, Clinical Psychology, Treatment Outcome, medicine.anatomical_structure, TRANSTORNOS COGNITIVOS, Female, Verbal memory, Cognition Disorders, business, and 030217 neurology & neurosurgery

Background Bipolar depression (BD) is a highly prevalent condition associated with marked cognitive deficits that persist even in the euthymic phase of the illness. Pharmacological treatments for BD might further aggravate cognitive impairment, highlighting the need of developing interventions that present cognitive safety. In this study, we evaluated the cognitive effects of H1-coil (deep) transcranial magnetic stimulation (TMS) in patients with treatment-resistant bipolar depression. Methods Fourty-three patients were randomized to receive 20 sessions of active (55 trains, 18 Hz, 120% resting motor threshold intensity) or sham rTMS within a double-blind, sham-controlled trial. A battery of 20 neuropsychological assessments, grouped in 6 domains (attention and processing speed, working memory and executive function, inhibitory control, language, immediate verbal memory, and long-term verbal memory) was performed at baseline and after 4 and 8 weeks of trial onset. Depressive symptoms were assessed with the 17-item Hamilton Rating Scale for Depression. Results Cognitive improvement was shown for all cognitive domains. It occurred regardless of intervention group and depression improvement. For the language domain, greater improvement was observed in the sham group over time. No correlations between depression (at baseline or during treatment) and cognitive improvement were found. Limitations Absence of healthy control group. Conclusion The results of this exploratory study provide evidence on the cognitive safety of H1-coil TMS for BD patients. Putative pro-cognitive effects of rTMS in BD were not observed and thus should be further investigated.

medicine.medical_specialty, Pineal region, Pineal Gland, Neurosurgical Procedures, Colloid Cysts, Arachnoid cyst, Cerebellum, medicine, Humans, Postoperative Period, Quadrigeminal cistern, Aged, Third Ventricle, Surgical approach, Third ventricle, QUÍMICA COLOIDAL, Colloid cyst, business.industry, Medical school, Endoscopy, General Medicine, medicine.disease, Magnetic Resonance Imaging, Surgery, Arachnoid Cysts, Treatment Outcome, medicine.anatomical_structure, Drainage, Female, Neurology (clinical), Nervous System Diseases, Brain third ventricle, business, and Hydrocephalus

Department of Neurology Botucatu Medical School Sao Paulo State University (UNESP), Rubiao Jr S/N Caixa Postal: 54018618-970, Botucatu, SP

musculoskeletal diseases, 0301 basic medicine, Adult, Male, congenital, hereditary, and neonatal diseases and abnormalities, Pediatrics, medicine.medical_specialty, Adolescent, Genotype, medicine.medical_treatment, Apert syndrome, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Borderline intellectual functioning, Cognition, Genetics, medicine, Humans, Craniofacial, Receptor, Fibroblast Growth Factor, Type 2, Child, Genetics (clinical), Craniofacial surgery, Alleles, Genetic Association Studies, DISOSTOSE CRÂNIOFACIAL, Retrospective Studies, Rehabilitation, medicine.diagnostic_test, business.industry, Fibroblast growth factor receptor 2, Crouzon syndrome, Brain, Magnetic resonance imaging, Acrocephalosyndactylia, medicine.disease, Magnetic Resonance Imaging, 030104 developmental biology, Phenotype, Amino Acid Substitution, Mutation, Female, business, and 030217 neurology & neurosurgery

Apert and Crouzon are the most common craniosynostosis syndromes associated with mutations in the fibroblast growth factor receptor 2 (FGFR2) gene. We conducted a study to examine the molecular biology, brain abnormalities, and cognitive development of individuals with these syndromes. A retrospective longitudinal review of 14 patients with Apert and Crouzon syndromes seen at the outpatient Craniofacial Surgery Hospital for Rehabilitation of Craniofacial Anomalies in Brazil from January 1999 through August 2010 was performed. Patients between 11 and 36 years of age (mean 18.29 ± 5.80), received cognitive evaluations, cerebral magnetic resonance imaging, and molecular DNA analyses. Eight patients with Apert syndrome (AS) had full scale intelligence quotients (FSIQs) that ranged from 47 to 108 (mean 76.9 ± 20.2), and structural brain abnormalities were identified in five of eight patients. Six patients presented with a gain-of-function mutation (p.Ser252Trp) in FGFR2 and FSIQs in those patients ranged from 47 to78 (mean 67.2 ± 10.7). One patient with a gain-of-function mutation (p.Pro253Arg) had a FSIQ of 108 and another patient with an atypical splice mutation (940-2A →G) had a FSIQ of 104. Six patients with Crouzon syndrome had with mutations in exons IIIa and IIIc of FGFR2 and their FSIQs ranged from 82 to 102 (mean 93.5 ± 6.7). These reveal that molecular aspects are another factor that can be considered in studies of global and cognitive development of patients with Apert and Crouzon syndrome (CS). © 2016 Wiley Periodicals, Inc.

Pediatrics, medicine.medical_specialty, Neurological injury, business.industry, Acquired aphasia, medicine.disease, TERAPÊUTICA FONOAUDIOLÓGICA, Speech therapy, School performance, El Niño, Hospital discharge, Medicine, Early childhood, business, and Stroke

O Acidente Vascular Cerebral é menos frequente em crianças do que em adultos, porém possui um poder devastador para o futuro destes indivíduos, mesmo com indícios de melhor recuperação das alterações. O objetivo deste estudo é descrever os achados fonoaudiológicos de um caso de Acidente Vascular Cerebral infantil, evidenciando a evolução após 12 meses de terapia fonoaudiológica, iniciada precocemente, após lesão neurológica adquirida. Trata-se de um indivíduo de 11 anos, gênero masculino, com normalidade de desenvolvimento neuropsicomotor e independência para as atividades funcionais durante a primeira infância. Em março de 2015 sentiu um mal súbito, foi encaminhado para o hospital de urgência e diagnosticado com Acidente Vascular Cerebral, do tipo hemorrágico, em região fronto-parietotemporal esquerda. Foram realizados procedimentos cirúrgicos, e a internação durou 25 dias. No momento da alta hospitalar houve a orientação sobre a necessidade de atendimento fonoaudiológico. A primeira avaliação de linguagem evidenciou afasia adquirida do tipo emissiva, conforme as classificações propostas para crianças. Foram realizadas 91 sessões de terapia fonoaudiológica, com duração de 50 minutos, pelo período de 12 meses. Ao analisar as habilidades formais de linguagem que permaneceram alteradas, observa-se que as relacionadas à leitura e escrita são as de maior significância, interferindo no desempenho escolar e desenvolvimento comunicativo. Assim, além do atendimento fonoaudiológico até que seja possível uma comunicação funcional, é importante que haja o apoio escolar.

Adult, Male, Microsurgery, Cancer Research, medicine.medical_specialty, von Hippel-Lindau Disease, medicine.medical_treatment, Single Center, Neurosurgical Procedures, Lesion, Hemangioblastoma, medicine, Humans, Cyst, Spinal Cord Neoplasms, Retrospective Studies, Medulla Oblongata, Brain Neoplasms, business.industry, Cauda equina, Recovery of Function, medicine.disease, Spinal cord, Magnetic Resonance Imaging, Spinal hemangioblastoma, Surgery, Treatment Outcome, medicine.anatomical_structure, Neurology, Oncology, Female, Neurology (clinical), medicine.symptom, business, and Magnetic Resonance Angiography

Purpose To analyze the surgical outcome of a consecutive and single center series of medulla oblongata (MO) and spinal cord hemangioblastomas (HB). Patient and methods We retrospectively reviewed the medical charts of all MO and spinal HB patients operated on in our institution between 1985 and 2002. All patients had pre- and at least one post-operative MRI. McCormick classification was used to assess neurological status and functional outcome. Results Forty surgical procedures have been performed on 34 patients (19 females and 15 males, mean age of 41 years). Twenty-five (73%) patients had Von Hippel Lindau (VHL) disease confirmed by genetic screening, and nine patients had sporadic disease. Complete clinical, radiological, and genetic studies were done in all cases. The most frequent clinical symptom was pain (28 patient, 85%) followed by motor (42%) and sensitive deficits (42%). Fourteen lesions (19%) were located in the MO, 28 (38%) in the cervical spine, 25 (34%) in the thoracic spine, 4 (5%) in the lumbar spine and 3 (4%) in the Cauda Equina. In the VHL group, 15 patients (60%) presented multiple lesions and 10 a single neurological lesion (40%). A cyst was present in 23% of VHL patients and in 55% of the non-VHL group. A complete removal was achieved in 85% of all cases. No deaths related to surgery occurred. At the end of the follow-up period (mean 60 months) 50% of patients were stabilized, while the condition of 32.35% was improved and of 17.65% worsened. Comparing the clinical evolution considering the presence or not of VHL we have seen that there are no differences in terms of functional outcome between VHL and non-VHL groups. Conclusion Our results confirmed that surgery remains a safe and effective treatment for medulla oblongata and spinal hemangioblastoma. Only symptomatic lesion required surgical treatment. In other cases, especially in VHL patients, a close and regular follow-up (clinical and MRI) is necessary.

Male, Language Tests, Meningomyelocele, Psycholinguistics, Infant, Language Development, Child Development, Reading, Case-Control Studies, Humans, Female, Language Development Disorders, Educational Measurement, and Child

To describe the performance of individuals with myelomeningocele regarding psycholinguistic and scholastic abilities.Participants were five individuals with myelomeningocele and lumbar sacral abnormalities, and chronological age between 9 years and 10 months and 11 years and 7 months (Group 1 - G1); five subjects with typical development (Group 2 - G2), matched to G1 for age, gender, and educational level. The evaluation consisted of interview with parents/caregivers, and application of the following tests: Illinois Test of Psycholinguistic Abilities (ITPA); School Performance Test; Speed Reading Test; and Rapid Automatized Naming Test.The between-groups comparison in the ITPA subtests showed that the maximum values obtained by G1 corresponded approximately to the minimum values obtained by G2, confirming the difference between the groups, except for the auditory closure subtest. In the Scholastic Performance Test, significant alterations were observed on the performance of G1 in all tasks. In the Speed Reading and Rapid Automatized Naming tests, individuals in G1 also presented considerable deficits, making more mistakes and spending more time than G2 to perform the same tasks.Individuals with myelomeningocele present deficits in psycholinguistic abilities, school performance, reading speed, and rapid automatized naming.

Physics, Meningomielocele, Meningomyelocele, Transtornos da percepção, APRENDIZAGEM, Learning disorders, Linguagem, Perceptual disorders, Speech and Hearing, Reading, Otorhinolaryngology, Leitura, Learning, Transtornos de aprendizagem, Humanities, and Language

OBJETIVO: Descrever o desempenho de indivíduos com mielomeningocele quanto às habilidades psicolinguísticas e escolares. MÉTODOS: Participaram cinco indivíduos, com idade cronológica entre 9 anos e 10 meses e 11 anos e 7 meses, com diagnóstico de mielomeningocele e lesão lombo-sacral (Grupo 1 - G1), e cinco indivíduos com desenvolvimento típico (Grupo 2 - G2), que foram pareados ao G1 quanto a idade, gênero e grau de escolaridade. A avaliação constou de entrevista com os responsáveis e aplicação dos seguintes testes: Teste de Illinois de Habilidades Psicolinguísticas; Teste de Desempenho Escolar; Teste de Velocidade de Leitura; e Teste de Nomeação Automática Rápida. RESULTADOS: A comparação entre os grupos nos subtestes do Teste de Illinois de Habilidades Psicolinguísticas indicou que os valores máximos obtido pelo G1 corresponderam aproximadamente aos valores mínimos obtidos pelo G2, confirmando a diferença entre eles, exceto para o subteste closura auditiva. No Teste de Desempenho Escolar foram observadas alterações significativas no desempenho do G1 em todas as provas. Nos testes de Velocidade de Leitura e de Nomeação Rápida, os indivíduos do G1 também apresentaram prejuízos consideráveis, cometendo mais erros nas tarefas e realizando a atividade de modo mais lento. CONCLUSÃO: Indivíduos com mielomeningocele apresentam alterações nas habilidades psicolinguísticas, no desempenho escolar, na velocidade de leitura e na nomeação automática rápida. PURPOSE: To describe the performance of individuals with myelomeningocele regarding psycholinguistic and scholastic abilities. METHODS: Participants were five individuals with myelomeningocele and lumbar sacral abnormalities, and chronological age between 9 years and 10 months and 11 years and 7 months (Group 1 - G1); five subjects with typical development (Group 2 - G2), matched to G1 for age, gender, and educational level. The evaluation consisted of interview with parents/caregivers, and application of the following tests: Illinois Test of Psycholinguistic Abilities (ITPA); School Performance Test; Speed Reading Test; and Rapid Automatized Naming Test. RESULTS: The between-groups comparison in the ITPA subtests showed that the maximum values obtained by G1 corresponded approximately to the minimum values obtained by G2, confirming the difference between the groups, except for the auditory closure subtest. In the Scholastic Performance Test, significant alterations were observed on the performance of G1 in all tasks. In the Speed Reading and Rapid Automatized Naming tests, individuals in G1 also presented considerable deficits, making more mistakes and spending more time than G2 to perform the same tasks. CONCLUSION: Individuals with myelomeningocele present deficits in psycholinguistic abilities, school performance, reading speed, and rapid automatized naming.

medicine.medical_specialty, medicine.diagnostic_test, Neuropsychology, CRANIOSSINOSTOSE, Crouzon syndrome, Wechsler Adult Intelligence Scale, Magnetic resonance imaging, Audiology, medicine.disease, Conductive hearing loss, Language assessment, medicine, General Earth and Planetary Sciences, Written language, Neuropsychological assessment, Psychology, and General Environmental Science

O objetivo do trabalho foi relatar um caso clínico sobre a síndrome de Crouzon, englobando as habilidades neuropsicolinguisticas. O estudo foi realizado com uma criança de oito anos e dois meses, do gênero masculino, com diagnóstico clínico da síndrome de Crouzon. Para a caracterização das habilidades foi realizada avaliação interdisciplinar com fonoaudiólogo, psicólogo e neurologista. A avaliação fonoaudiológica incluiu a avaliação das habilidades comunicativas, da linguagem oral, considerando as habilidades fonológicas, sintáticas, semânticas e pragmáticas, e da linguagem escrita. Para a avaliação neuropsicológica utilizou-se a escala Wechsler Scale for Children. A avaliação neurológica foi centrada no exame de ressonância magnética de encéfalo. O paciente também realizou avaliação audiológica, e apresentou perda auditiva condutiva de grau leve. Na avaliação clínica e formal da linguagem oral foi possível observar alterações nos aspectos fonológico e semântico da linguagem. Já com relação à linguagem escrita, observou-se desempenho aquém do esperado para a idade, caracterizado principalmente por alterações na escrita e na aritmética. Os resultados da avaliação neuropsicológica evidenciaram valores de Quociente Intelectual dentro dos padrões da normalidade. O resultado da ressonância magnética do encéfalo demonstrou alteração estrutural do sistema nervoso central. Pode-se concluir que os achados evidenciaram alterações nas habilidades de linguagem oral e escrita, além da presença de alteração estrutural do sistema nervoso central.

Mental development, Cognitive evaluation theory, Pediatrics, medicine.medical_specialty, Intelligence quotient, brain, Neuropsychology, Crouzon syndrome, medicine.disease, síndrome de Crouzon, Developmental psychology, Neurology, Quality of life, quality of life, qualidade de vida, medicine, cérebro, Neurology (clinical), Psychology, and Social evaluation

Submitted by Guilherme Lemeszenski (guilherme@nead.unesp.br) on 2013-08-22T18:48:25Z No. of bitstreams: 1 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) Made available in DSpace on 2013-08-22T18:48:25Z (GMT). No. of bitstreams: 1 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) Previous issue date: 2007-06-01 Made available in DSpace on 2013-09-30T19:38:15Z (GMT). No. of bitstreams: 2 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) S0004-282X2007000300020.pdf.txt: 23956 bytes, checksum: 323426ac4fe836f350cf3ce924373237 (MD5) Previous issue date: 2007-06-01 Submitted by Vitor Silverio Rodrigues (vitorsrodrigues@reitoria.unesp.br) on 2014-05-20T13:36:00Z No. of bitstreams: 2 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) S0004-282X2007000300020.pdf.txt: 23956 bytes, checksum: 323426ac4fe836f350cf3ce924373237 (MD5) Made available in DSpace on 2014-05-20T13:36:00Z (GMT). No. of bitstreams: 2 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) S0004-282X2007000300020.pdf.txt: 23956 bytes, checksum: 323426ac4fe836f350cf3ce924373237 (MD5) Previous issue date: 2007-06-01 A síndrome de Crouzon é caracterizada por deformidade craniana, alterações faciais e exoftalmia. O retardo no desenvolvimento neuropsicomotor é observado em alguns casos. Este estudo tem como objetivo analisar a influência do momento da cirurgia, da classe sócio-econômica associada ao nível educacional dos pais e da ocorrência de malformações do sistema nervoso central no desenvolvimento cognitivo destes pacientes correlacionando estes achados à qualidade de vida deles e de suas famílias. Foram estudados 11 pacientes com diagnóstico de síndrome de Crouzon com idade entre um ano e quatro meses e treze anos. A avaliação multidisciplinar dos pacientes incluiu, avaliação social, avaliação cognitiva, estudo do encéfalo por ressonância magnética e avaliação da qualidade de vida. O quociente de inteligência variou de 46 a 102 (m=84,2) e foi correlacionado de forma inversa com o Fator 4 do Questionário de Recursos e Estresse Simplificado (incapacidade da criança); não se correlacionou com as alterações encefálicas, com a condição sócio-econômica dos pais e nem com o momento do tratamento neurocirúrgico. Crouzon syndrome is characterized by cranial and facial abnormalities and exophtalmos. Mental retardation is sometimes observed. The objective of this study was to correlate brain malformations, timing for surgery and also social classification of families and parents education to the neuropsychological evaluation and to the quality of life of these families. Eleven patients with Crouzon syndrome were studied, whose ages were between 16 and 132 months. The multidisciplinary evaluation included : social evaluation, cognitive evaluation, brain studies by magnetic ressonance imaging and quality of life evaluation. The intelligence quotient values observed were between 46 and 102 (m=84.2) and was correlated (inverted correlation) to the factor IV of the short-form of the Questionnaire on Resources and Stress. Mental development was not correlated to brain malformation, neither to the age at time of operation or to the level of family environment and parents education. Universidade de São Paulo Hospital de Reabilitação de Anomalias Craniofaciais Departamento de Cirurgia Craniofacial Hospital Alemão Oswaldo Cruz Departamento de Neuroradiologia USP Hospital das Clínicas Departamento de Neurocirurgia Universidade Estadual Paulista Hospital das Clínicas Departamento de Neurologia e Psiquiatria UNESP Hospital das Clínicas Departamento de Cirurgia e Ortopedia USP Faculdade de Odontologia Departamento de Fonoaudiologia Universidade Estadual Paulista Hospital das Clínicas Departamento de Neurologia e Psiquiatria UNESP Hospital das Clínicas Departamento de Cirurgia e Ortopedia

Male, Adolescent, Craniofacial Dysostosis, Infant, Neuropsychological Tests, Magnetic Resonance Imaging, Socioeconomic Factors, Child, Preschool, Surveys and Questionnaires, Quality of Life, Educational Status, Humans, Female, Child, and Cognition Disorders

Crouzon syndrome is characterized by cranial and facial abnormalities and exophtalmos. Mental retardation is sometimes observed. The objective of this study was to correlate brain malformations, timing for surgery and also social classification of families and parents education to the neuropsychological evaluation and to the quality of life of these families. Eleven patients with Crouzon syndrome were studied, whose ages were between 16 and 132 months. The multidisciplinary evaluation included : social evaluation, cognitive evaluation, brain studies by magnetic resonance imaging and quality of life evaluation. The intelligence quotient values observed were between 46 and 102 (m=84.2) and was correlated (inverted correlation) to the factor IV of the short-form of the Questionnaire on Resources and Stress. Mental development was not correlated to brain malformation, neither to the age at time of operation or to the level of family environment and parents education.