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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

medicine.medical_specialty, Neurology, Lumbar vertebrae, Thoracic Vertebrae, 03 medical and health sciences, 0302 clinical medicine, Medicine, Humans, Ganglioneuroma, Retroperitoneal Neoplasms, Child, Lumbar Vertebrae, Spinal Neoplasms, business.industry, Medical school, Laminectomy, General Medicine, Anatomy, medicine.disease, Magnetic Resonance Imaging, Abdominal Pain, medicine.anatomical_structure, 030220 oncology & carcinogenesis, Pediatrics, Perinatology and Child Health, Thoracic vertebrae, Surgery, Female, Neurology (clinical), Dumbbell, business, and 030217 neurology & neurosurgery

Department of Neurology Psychology and Psychiatry Botucatu Medical School UNESP Universidade Estadual Paulista Campus de Botucatu, Distrito de Rubiao Jr s/n

Adult, Male, Decompressive Craniectomy, medicine.medical_specialty, Pediatrics, medicine.medical_treatment, lcsh:Medicine, Brain Ischemia, law.invention, Sepsis, 03 medical and health sciences, 0302 clinical medicine, Randomized controlled trial, law, Intensive care, medicine, Humans, 030212 general & internal medicine, lcsh:Science, Stroke, Aged, Aged, 80 and over, Multidisciplinary, business.industry, Medical record, Incidence (epidemiology), lcsh:R, Retrospective cohort study, Middle Aged, South America, medicine.disease, Surgery, lcsh:Q, Female, Decompressive craniectomy, business, 030217 neurology & neurosurgery, and Research Article

Made available in DSpace on 2018-11-26T16:19:18Z (GMT). No. of bitstreams: 0 Previous issue date: 2016-01-14 Background Malignant cerebral artery strokes have a poor prognosis, with nearly 80% of mortality in some series despite intensive care. After a large randomized trial, decompressive hemicraniectomy has been performed more often in stroke patients. Here, we describe patients in a tertiary teaching hospital in Brazil, emphasizing the impact of age on outcomes. Methods A retrospective cohort of patients, with malignant strokes which received a decompressive hemicraniectomy, from paper and electronic medical records, from January 2010 to December 2013 was divided into two groups according to age. Results The final analysis included 60 patients. The overall mortality was higher among patients older than 60 yrs (67% vs. 41%; p = 0.039), whose group also had a worse outcome (76% with mRS 5 or 6) at 90 days (OR 3.91 CI95% 1.30-11.74), whereas only 24% had mRS of 0-4 (p = 0.015). All patients who presented with sepsis died (p = 0.003). The incidence of pulmonary infection was very high in the elderly group (76%) with significant intergroup differences (p = 0.027, OR 8.32 CI95% 0.70-98.48). Conclusions Older patients present more commonly with infections, more disabilities and a higher mortality, highlighting very poor results in elderly population. These results should be proved with a South American trial, and if confirmed, it can impact on future decisions regarding decompressive craniectomy for acute ischemic stroke in our region. Univ Estadual Paulista, Botucatu Med Sch, Dept Neurol Psychol & Psychiat, Botucatu, SP, Brazil Univ Estadual Paulista, Botucatu Med Sch, Dept Publ Hlth Med, Botucatu, SP, Brazil Univ Estadual Paulista, Botucatu Med Sch, Dept Anesthesiol, Botucatu, SP, Brazil Univ Estadual Paulista, Botucatu Med Sch, Dept Neurol Psychol & Psychiat, Botucatu, SP, Brazil Univ Estadual Paulista, Botucatu Med Sch, Dept Publ Hlth Med, Botucatu, SP, Brazil Univ Estadual Paulista, Botucatu Med Sch, Dept Anesthesiol, Botucatu, SP, Brazil

medicine.medical_specialty, Subarachnoid hemorrhage, biology, business.industry, Glasgow Outcome Scale, C-reactive protein, Glasgow Coma Scale, Vasospasm, medicine.disease, Transcranial Doppler, Cerebral vasospasm, Modified Rankin Scale, Anesthesia, Internal medicine, medicine, biology.protein, Cardiology, and business

Aim: The interest of inflammatory marker increased in the last years, even in preventing clinical outcome after subarachnoid hemorrhage (SAH). Our objective was to study the relationships between C-reactive protein levels and clinical outcome and the development of cerebral vasospasm after aneurismal SAH. Methods: One hundred adult patients with aneurismal SAH were prospectively evaluated. Glasgow Coma Scale (GCS) score, Hunt and Hess grade, Fisher grade, CT scans, digital subtraction angiography studies, transcranial doppler (TCD) and daily neurological examinations were recorded. Serial serum CRP measurements were obtained on daily between admission and 10th days. Glasgow Outcome Scale (GOS) and the modified Rankin Scale (mRS) were used to predict outcome. Results: A progressive increase in the CRP levels from the admission to the 3rd postictal day was observed, followed by a slow decrease until the 9th day. Hemodynamic changes in TCD were associated with higher serum CRP levels. Patients with lower GCS scores presented with increased CRP levels. Patients with higher Hunt and Hess grades on admission developed significantly higher CRP serum levels. Patients with higher admission Fisher grades showed increased levels of CRP. A statistically significant inverse correlation was established in our series between CRP serum levels and GOS and mRS scores on discharge and CRP levels. Conclusion: Increased CRP levels were strongly associated with poor clinical outcome. CRP levels can predict cerebral vasospasm and delayed ischemic deficits with higher statistic significance. There are relationships between hemodynamic chances in TCD and higher CRP levels.

Male, Time Factors, Escala visual analógica, Ablative case, Medicine, Prospective Studies, Prospective cohort study, numeric rating scale, Pain Measurement, Sacroiliac joint, Middle Aged, Denervation, sacroiliac joint pain, medicine.anatomical_structure, Treatment Outcome, Neurology, Anesthesia, Secondary Outcome Measure, Catheter Ablation, Female, Adult, musculoskeletal diseases, medicine.medical_specialty, sacroileíte, Long term follow up, Sacroiliac joint pain, patient global impression of change scale, lcsh:RC321-571, Lumbar, radiofrequency, Sacroileíte, escala de impressão global de mudança do paciente, Humans, Pain Management, Sacroiliitis, lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry, Aged, Pain score, business.industry, Sacrococcygeal Region, Reproducibility of Results, Radiofrequência, Sacroiliac Joint, Surgery, Radiography, escala visual analógica, Escala de impressão global de mudança do paciente, Neurology (clinical), business, Low Back Pain, radiofrequência, and Follow-Up Studies

Made available in DSpace on 2015-08-26T19:19:05Z (GMT). No. of bitstreams: 0 Previous issue date: 2015-06-01. Added 1 bitstream(s) on 2015-08-31T13:05:03Z : No. of bitstreams: 1 S0004-282X2015000600476.pdf: 200466 bytes, checksum: a41831d49e9d8c85a3cfa6b2f335275b (MD5) A Sacroileíte pode ser responsável por até 40% dos casos de dor lombar crônica. Objetivo Análise da eficácia da denervação por radiofrequência na articulação sacro-ilíaca em seis, doze e dezoito meses. Método Trinta e dois pacientes com diagnóstico de sacroileíte foram incluídos em estudo prospectivo. O prognóstico primário foi avaliado pela escala visual analógico (NRS). O prognóstico secundário foi avaliado pela escala de impressão global de mudança pelo paciente (PGIC). Resultados Melhora a curto prazo da dor foi observada, com redução media na NRS de 7,7 ± 1,8 para 2,8 ± 1,2 após 1 mês e para 3,1 ± 1,9 em 6 meses do procedimento (p < 0,001). Após 12 e 18 meses, o NRS manteve-se 3,4 ± 2,1 e 4,0 ± 2,7, respectivamente. Conclusão A denervação da articulação sacro-ilíaca por radiofrequência pode reduzir significativamente a dor em pacientes com sacroileíte. Sacroiliac joint (SIJ) pain is responsible for up to 40% of all cases of lumbar back pain. Objective Report the long-term efficacy of radiofrequency denervation for sacroiliac joint pain at six, twelve and eighteen months. Method Third-two adults’ patients with sacroiliac join pain diagnosis were included for a prospective study. Primary outcome measure was pain intensity on the Numeric Rating Scale (NRS). Secondary outcome measure was Patient Global Impression of Change Scale (PGIC). Results Short-term pain relief was observed, with the mean NRS pain score decreasing from 7.7 ± 1.8 at baseline to 2.8 ± 1.2 at one month and to 3.1 ± 1.9 at six months post-procedure (p < 0.001). Long-term pain relief was sustained at twelve and eighteen months post-procedure, with NRS pain remaining at 3.4 ± 2.1 and 4.0 ± 2.7, respectively. Conclusion Radiofrequency denervation of the SIJ can significantly reduce pain in selected patients with sacroiliac syndrome. Universidade Estadual Paulista Hospital de Clínicas Faculdade de Medicina de Botucatu Universidade Estadual Paulista, Hospital de Clínicas, Faculdade de Medicina de Botucatu, Divisão de Neurocirurgia, Botucatu SP, Brazil

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.

medicine.medical_specialty, Myelopathy, Spine surgery, business.industry, Radiological weapon, medicine.medical_treatment, medicine, Cervical spondylosis, business, medicine.disease, Laminoplasty, and Surgery

Multilevel Cervical Myelopathy Treatment - Open-Door Laminoplasty vs. Multiple Cervical Arcocristectomies Cervical myelopathy due to posterior multiple level compression remains a surgical challenger. Many surgical options have been used in the last years, but the best choice is unclear. We compared open-door laminoplasty and multiple level arcocristectomies on clinical and radiological findings.

medicine.medical_specialty, Severe headache, integumentary system, business.industry, Skin flap, Bone defect, Article, Surgery, Dysautonomic syndrome, Trefinated, medicine, Sinking skin flap syndrome, Presentation (obstetrics), Complication, and business

Submitted by Vitor Silverio Rodrigues (vitorsrodrigues@reitoria.unesp.br) on 2014-05-27T11:30:51Z No. of bitstreams: 0Bitstream added on 2014-05-27T14:43:38Z : No. of bitstreams: 1 2-s2.0-84885149210.pdf: 1239639 bytes, checksum: ac94b1af3a62329ebaf40775f9c0f499 (MD5) Made available in DSpace on 2014-05-27T11:30:51Z (GMT). No. of bitstreams: 0 Previous issue date: 2013-10-14 INTRODUCTION Sinking skin flap syndrome or syndrome of the trephined is a rare complication after a large craniectomy, with a sunken skin above the bone defect with neurological symptoms such as severe headache, mental changes, focal deficits, or seizures. PRESENTATION OF CASE We report a case of 21 years old man with trefinated syndrome showing delayed dysautonomic changes. DISCUSSION Our patient had a large bone flap defect and a VP shunt that constitute risk factors to develop this syndrome. Also, there is reabsorption of bone tissue while it is placed in subcutaneous tissue. The principal symptoms of sinking skin flap syndrome are severe headache, mental changes, focal deficits, or seizures. Our patient presented with a delayed dysautonomic syndrome, with signs and symptoms very characteristics. Only few cases of this syndrome were related in literature and none were presented with dysautonomic syndrome. CONCLUSION We reported here a very uncommon case of sinking skill flap syndrome that causes a severe dysautonomic syndrome and worsening the patient condition. © 2013 The Authors. Division of Neurosurgery Botucatu Medical School São Paulo State University, Botucatu Hospital das Clínicas UNESP, Botucatu Division of Neurosurgery Botucatu Medical School São Paulo State University, Botucatu Hospital das Clínicas UNESP, Botucatu

Pathology, gait disorder, Case Report, postoperative period, proton nuclear magnetic resonance, neurologic examination, diplopia, neurosurgery, nuclear magnetic resonance imaging, Pilomyxoid astrocytoma, Pediatric, child, Astrocytoma, lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens, brain stem tumor, female, Oncology, glial fibrillary acidic protein, histopathology, Neurosurgery, Brainstem, brain tumor, medicine.medical_specialty, Histology, brain stem pilomyxoid astrocytoma, Brain tumor, reoperation, lcsh:RC254-282, World health, abducens nerve paralysis, medicine, case report, human, Karnofsky Performance Status, astrocytoma, neoplasms, business.industry, ataxia, medicine.disease, school child, pilomyxoid astrocytoma, pediatric, brain tumor, Brain stem tumor, human tissue, nervous system diseases, pediatric, nervous system, pilomyxoid astrocytoma, Histopathology, and business

Submitted by Vitor Silverio Rodrigues (vitorsrodrigues@reitoria.unesp.br) on 2014-05-27T11:28:55Z No. of bitstreams: 0Bitstream added on 2014-05-27T14:37:27Z : No. of bitstreams: 1 2-s2.0-84878331515.pdf: 704870 bytes, checksum: d34dfd002f248ae7e934d16d4d76d872 (MD5) Made available in DSpace on 2014-05-27T11:28:55Z (GMT). No. of bitstreams: 0 Previous issue date: 2013-04-15 A pilomyxoid astrocytoma is a recently described tumor that occurs predominantly in the hypothalamic-chiasmatic region and is rarely found elsewhere. It has similar features as pilocytic astrocytomas, but has distinct histological characteristics and a poorer prognosis. A pilomyxoid astrocytoma is an aggressive tumor, and increased awareness is necessary with a suspect case. We present the first case of a pilomyxoid astrocytoma of the brainstem described after the newest World Health Organization classification of central nervous system tumors. © F.O. Pereira et al., 2013. Licensee PAGEPress, Italy. Division of Neurosurgery of Botucatu Medical School São Paulo State University, Botucatu Division of Pathology of Botucatu Medical School São Paulo State University, Botucatu Division of Neurosurgery of Botucatu Medical School São Paulo State University, Botucatu Division of Pathology of Botucatu Medical School São Paulo State University, Botucatu

musculoskeletal diseases, medicine.medical_specialty, business.industry, Cauda equina syndrome, Case Report, Anatomy, Lumbar vertebrae, medicine.disease, Nerve compression syndrome, medicine.anatomical_structure, Intervertebral Disc Displacement, medicine, Orthopedics and Sports Medicine, Surgery, Radiology, Lumbar disc herniation, Neurosurgery, Surgical treatment, business, and Diskectomy

Introduction Intradural lumbar disc herniations are uncommon presentations of a relatively frequent pathology, representing less than 1% of all lumbar disc hernias. They show specific features concerning their clinical diagnosis, with a higher incidence of cauda equina syndrome, and their surgical treatment requires a transdural approach.

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