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1.
Childs Nerv Syst ; 40(3): 895-903, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37975904

RESUMO

Dystonia represents a significant source of disability in children. Generalized dystonia, which involves multiple body regions, leads to impaired mobility and motor function, resulting in substantial challenges in daily activities. Surgical treatments are used when medical treatments fail. Intrathecal baclofen (ITB) or deep brain stimulations (DBS) are the most employed surgical therapies. When these options are not feasible or ineffective, some authors have explored the use of intraventricular baclofen (IVB). In this report, we present four cases of pediatric patients with generalized dystonia who underwent treatment with IVB, resulting in notable improvements. To further explore the potential of this treatment modality, we conducted a comprehensive literature review. The findings from our study provide a comprehensive overview that can guide palliative management in similar cases.


Assuntos
Distonia , Distúrbios Distônicos , Relaxantes Musculares Centrais , Humanos , Criança , Baclofeno/uso terapêutico , Distonia/tratamento farmacológico , Relaxantes Musculares Centrais/uso terapêutico , Bombas de Infusão Implantáveis , Espasticidade Muscular
2.
Rev Neurol (Paris) ; 2024 Jun 11.
Artigo em Inglês | MEDLINE | ID: mdl-38866657

RESUMO

BACKGROUND: Previous studies showed the efficacy of epilepsy surgery in carefully selected children with epilepsy associated with tuberous sclerosis complex. However, how this selection is conducted, and the characteristics of the patients brought to surgery are still poorly described. By conducting a multicentric retrospective cohort study covering the practice of the last twenty years, we describe the paths leading to epilepsy surgery in children with epilepsy associated with tuberous sclerosis complex. METHODS: We identified 84 children diagnosed with tuberous sclerosis complex and epilepsy by matching two exhaustive registries of genetic diseases and subsequent medical records reviews within two French neuropediatric and epilepsy centers. Demographic, clinical, longitudinal, and diagnostic and surgical procedures data were collected. RESULTS: Forty-six percent of the children were initially drug-resistant and 19% underwent resective surgery, most often before the age of four. Stereotactic electroencephalography was performed prior to surgery in 44% of cases. Fifty-seven and 43% of patients remained seizure-free one and ten years after surgery, respectively. In addition, 52% of initially drug-resistant patients who did not undergo surgery were seizure-free at the last follow-up. The number of anti-seizure medications required decreased in 50% of cases after surgery. Infantile spasms, intellectual disability, autism spectrum disorder or severe behavioral disorders were not contraindications to surgery but were associated with a higher rate of complications and a lower rate of seizure freedom after surgery. CONCLUSION: Despite the assumption of complex multifocal epilepsy and practical difficulties in young children with tuberous sclerosis complex, successful surgery results are comparable with other populations of patients with drug-resistant epilepsy, and a spontaneous evolution to drug-sensitive epilepsy may occur in non-operated patients.

3.
Childs Nerv Syst ; 36(6): 1325-1330, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-31925509

RESUMO

The occurrence of secondary synostosis of coronal sutures at distance from H-craniectomy surgery for scaphocephaly concerns about 10% of children. Intracranial hypertension in these children remains exceptional but generally requires a surgical reoperation. Two children aged 3 and 5- months- old had been operated for scaphocephaly by H-craniectomy in two different hospital centers. Their clinical follow-up described a partial persistence of dolichocephalic deformity and an impression of parietal stenosis. During their growth, chronic headaches appeared with a complaint expressed at the ages of 4 and 5 years. In both cases, ophthalmic examination revealed significant bilateral papillary edema without loss of visual acuity. The imaging assessment (CT-scan and MRI) showed the absence of Chiari malformation and venous abnormality. For both, there was a compression image of the parietal lobes in relation to the persistence of a temporoparietal synostosis. An osteogenic parietal distraction permitted a volumetric brain expansion consecutive to the skull and meninges remodeling in only 6 months, associated with a leap forward acquisition, a normalization of the ophthalmic examination, and a complete loss of headaches. In conclusion, this new approach could be used in the case of chronic intracranial hypertension consecutive to a secondary parietal synostosis after a scaphocephaly surgery.


Assuntos
Craniossinostoses , Hipertensão Intracraniana , Osteogênese por Distração , Pré-Escolar , Suturas Cranianas , Craniossinostoses/diagnóstico por imagem , Craniossinostoses/cirurgia , Humanos , Lactente , Hipertensão Intracraniana/diagnóstico por imagem , Hipertensão Intracraniana/etiologia , Hipertensão Intracraniana/cirurgia , Crânio
4.
Neuropathol Appl Neurobiol ; 39(6): 693-705, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23278243

RESUMO

BACKGROUND: Pilocytic astrocytomas (PAs) are characterized by an excellent prognosis although several factors of adverse outcome have been reported. The mitogen-activated protein kinase pathway plays a major role in their tumorigenesis. AIM: To report a series of 148 PAs in children to define clinicopathological and biological prognostic factors. METHODS: Clinical data were collected from patient files and mail inquiry. Pathological specimens were centrally reviewed. The three major KIAA1549:BRAF fusion subtypes were analysed by reverse transcription - polymerase chain reaction (RT-PCR) in a subset of 47 frozen cases and by fluorescence in situ hybridization on formalin-fixed paraffin-embedded tissue in 23 cases. Tumour location, age at surgery, extent of surgical removal, histological subtype and KIAA1549:BRAF fusion by RT-PCR were searched for prognostic significance. RESULTS: Pilomyxoid astrocytoma (PMA) and the hypothalamo-chiasmatic (H/C) location were associated with a worse prognosis [P < 0.001 for overall survival (OS) and P = 0.001 for progression-free survival (PFS)]. Patients who underwent complete surgical excision had a better OS (P = 0.004) and a longer PFS (P < 0.001) than the others. Age was also a strong prognostic factor for OS but not for PFS. Infants (<1 year) and young children (<3 years) had a much worse outcome than the others (P < 0.001 and P = 0.004 respectively). KIAA1549:BRAF fusion status was not predictive of outcome. CONCLUSION: This study highlights the good prognostic factors of PAs but H/C PA remains a subgroup with dismal prognosis associated with young age, PMA variant and incomplete surgery. Search for KIAA1549:BRAF fusion in tumours with PA pattern is recommended even though the prognostic impact is still unclear.


Assuntos
Astrocitoma/patologia , Neoplasias Encefálicas/patologia , Encéfalo/patologia , Adolescente , Fatores Etários , Astrocitoma/genética , Neoplasias Encefálicas/genética , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Prognóstico , Proteínas Proto-Oncogênicas B-raf/genética , Estudos Retrospectivos , Adulto Jovem
5.
Epilepsy Res ; 191: 107090, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36774667

RESUMO

OBJECTIVE: Temporal lobe epilepsy is the most common type of focal drug-resistant epilepsy. Seizures with predominant involvement of basal temporal regions (BTR) are not well characterized. In this stereo electroencephalography (SEEG) study, we aimed at describing the ictal networks involving BTR and the associated clinical features. METHODS: We studied 24 patients explored with SEEG in our center with BTR sampling. We analyzed their seizures using a quantitative method: the "epileptogenicity index". Then we reported the features of the patients with maximal epileptogenicity within BTR, especially ictal network involved, ictal semiology and post-surgical outcome. RESULTS: We found that rhinal cortex, parahippocampal cortex and posterior fusiform gyrus were the most epileptogenic structures within the BTR (mean EI: 0.57, 0.55, 0.54 respectively). Three main groups of epileptogenic zone organization were found: anterior (23% of total seizures) posterior (30%) and global (47%, both anterior and posterior). Contralateral spread was found in 35% of left seizures and 20% of right seizures. Naming deficit was more prevalent in left BTR (71% vs 29% in right seizures; p = 0.01) whereas automatic speech production was preferentially represented in right seizures (11% vs 54%; p = 0.001). Surgery was proposed for 11 patients (45.8%), leading to seizure freedom in 72% (Engel Class I). One patient presented post-operative permanent functional deficit. CONCLUSION: Basal-temporal lobe epilepsy seems to be a specific entity among the temporal epilepsy spectrum with specific clinical characteristics. Resective surgery can be proposed with good outcomes in a significant proportion of patients and is safe provided that adequate language assessment has been preoperatively made.


Assuntos
Epilepsia do Lobo Temporal , Humanos , Eletroencefalografia , Córtex Cerebral , Convulsões , Lobo Temporal
6.
Neurochirurgie ; 67(6): 564-570, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33915148

RESUMO

INTRODUCTION: Good practice guidelines help clinicians to establish a suspected diagnosis of non-accidental head injury (NAHI) and help forensic experts to establish a level of certainty for the diagnosis. The objective of this study was to assess how the French Health Authority (HAS) guidelines contribute to the process of producing an expert assessment, on causation and certainty in cases of suspected NAHI. METHOD: A retrospective study was conducted of the expert assessments that were conducted by a paediatric surgeon and forensic expert attached to our local court between 2002 and 2018, with the aim of determining the causal mechanism of the lesions and express a degree of certainty regarding the diagnosis. RESULTS: In our study, we found that, despite the HAS guidelines, a number of documents deemed essential for the forensic expert were sometimes missing, and that, by applying these guidelines, the decisions reached in some expert assessments could been reclassified and certain factors formerly described as risk factors for injury could be excluded. A precise dating of the traumatic event was proposed in half of cases. CONCLUSION: Our study highlights the vital role of the HAS guidelines, not only for patient management but also to ensure high-quality expert assessments. Unfortunately, guidelines were not yet being properly adhered to by medical teams.


Assuntos
Maus-Tratos Infantis , Traumatismos Craniocerebrais , Criança , Maus-Tratos Infantis/diagnóstico , Traumatismos Craniocerebrais/diagnóstico , Humanos , Lactente , Estudos Retrospectivos , Fatores de Risco
7.
Neurochirurgie ; 67(6): 618-620, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33529695

RESUMO

BACKGROUND: The diagnosis of extra-axial cavernoma and surgical decision-making can be difficult on children. REPORTED CASE: In this report we present the case of a 33-month-old child for whom the work up done for asthenia, anorexia and psychomotor regression reveals a well-defined occipital extra-axial lesion. A follow up MRI was performed two months later, revealing an increase in the size of the lesion associated with hemorrhagic changes. The surgical decision is performed after an arteriography; it allows the total removal of the lesion contained in a duplication of the dura mater, by placing a vascular clip on an accessory venous sinus draining the lesion, respecting the integrity of the lateral sinus. Histology confirms a cavernoma. No complication resulted from the surgical procedure. This atypical case of pediatric dural cavernoma associated with a well-defined accessory venous sinus in arteriography is to our knowledge the first description in the literature. CONCLUSION: Extra-axial cavernomas have a misleading presentation. The management of these lesions is of twofold interests: to avoid a repercussion on the development of these children, and to obtain histological confirmation.


Assuntos
Seio Cavernoso , Hemangioma Cavernoso , Seio Cavernoso/diagnóstico por imagem , Seio Cavernoso/cirurgia , Criança , Pré-Escolar , Dura-Máter , Humanos , Imageamento por Ressonância Magnética , Veias
8.
Neuropathol Appl Neurobiol ; 36(5): 399-410, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20202125

RESUMO

AIMS: Studying the molecules and signalling pathways regulating glioma invasiveness is a major challenge because these processes determine malignancy, progression, relapse and prognosis. We took advantage of our previous study focused on genes that were critical in tumour invasion to further study here an unknown sequence, referred to as KIAA0510, the chromosomal location of which was 1q25, described as a 5596-bp long mRNA and that we found to be significantly overexpressed in pilocytic astrocytomas compared with glioblastomas. METHODS AND RESULTS: Using in silico analysis as well as Polymerase chain reaction techniques, we decipher the full genomic characterization of the KIAA0510 sequence and demonstrate that KIAA0510 constitutes the 3'-untranslated region of tenascin-R gene. We have clearly confirmed the overexpression of tenascin-R in pilocytic astrocytomas vs. glioblastomas at mRNA and protein levels. We also analysed a large series of various brain tumours and found that in the group of astrocytic tumours, tenascin-R expression decreased with malignancy, whereas oligodendrogliomas sometimes retained a high level of tenascin-R even in high-grade tumours. Gangliogliomas strongly expressed tenascin-R too. In contrast, ependymomas and meningiomas were negative. In normal brain, tenascin-R was exclusively expressed by normal oligodendrocytes and subsets of neurones during post-natal development and in adulthood, where it could differentially affect cellular adhesiveness and/or differentiation. CONCLUSION: KIAA0510, the 3'-untranslated region of the tenascin-R gene, and tenascin-R are overexpressed in pilocytic astrocytomas. Gangliogliomas shared with pilocytic astrocytomas strong tenascin-R expression. Whether tenascin-R overexpression negatively influences brain invasion remains to be determined.


Assuntos
Astrocitoma/genética , Neoplasias Cerebelares/genética , Regulação Neoplásica da Expressão Gênica , Tenascina/genética , Regiões 3' não Traduzidas/genética , Adolescente , Adulto , Idoso , Astrocitoma/fisiopatologia , Neoplasias Cerebelares/fisiopatologia , Criança , Pré-Escolar , Ependimoma/genética , Ependimoma/fisiopatologia , Ganglioglioma/genética , Ganglioglioma/fisiopatologia , Humanos , Lactente , Neoplasias Meníngeas/genética , Neoplasias Meníngeas/fisiopatologia , Meningioma/genética , Meningioma/fisiopatologia , Pessoa de Meia-Idade , Neoplasias Supratentoriais/genética , Neoplasias Supratentoriais/fisiopatologia , Adulto Jovem
9.
Neurochirurgie ; 66(2): 91-96, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31866515

RESUMO

Simple, complex or syndromic craniosynostosis may be responsible for ocular and especially oculomotor pathologies. Among simple craniosynostosis, anterior plagiocephaly is the most frequently associated with oculomotor disorders. Oculomotor disorders encountered in craniosynostosis are specific to this pathology. They may be related to orbital deformities or oculomotor muscle malformations. Early craniofacial surgery reduces the onset and severity of these oculomotor disorders which is very important for ophtalmological patient care. Indeed, these oculomotor disorders are difficult to treat for the ophthalmologist with most of the time several surgeries needed, and lead to amblyopia if neglected.


Assuntos
Craniossinostoses/complicações , Craniossinostoses/cirurgia , Oftalmoplegia/complicações , Oftalmoplegia/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Ambliopia/etiologia , Ambliopia/terapia , Criança , Pré-Escolar , Craniossinostoses/diagnóstico , Humanos , Músculos Oculomotores/anormalidades , Músculos Oculomotores/cirurgia , Oftalmoplegia/diagnóstico , Doenças Orbitárias/cirurgia , Plagiocefalia/complicações , Plagiocefalia/diagnóstico , Plagiocefalia/cirurgia
10.
Eur Ann Otorhinolaryngol Head Neck Dis ; 137(2): 99-103, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31627971

RESUMO

IMPORTANCE: Non-steroidal anti-inflammatory drugs (NSAIDs) are known to inhibit chemotaxis, oxidative burst and phagocytosis, bacterial killing in granulocytes as well as inhibiting neutrophil aggregation or degranulation, thereby interfering with the function of lymphocytes. On the other hand, ibuprofen is widely prescribed in pediatrics for its powerful analgesic and antipyretic effects. To our knowledge, no previous publication outlines the relationship between Ibuprofen therapy and an increased risk of intracranial and/or orbital complications of acute fronto-ethmoidal sinusitis in childhood. OBJECTIVE: To look for a relationship between ibuprofen and occurrence of intra-cranial and/or orbital complications of acute fronto-ethmoidal sinusitis in pediatrics. SETTING AND METHODS: The medical charts of patients younger than 18 years admitted into the E.N.T. departments of 4 academic care centers during 2 consecutive years for fronto ethmoidal sinusitis were reviewed retrospectively. The history of ibuprofen intake, the occurrence of complication (orbital or intracranial) as well as the usual demographic data were noted. A statistical analysis was performed in order to ascertain whether a relationship between taking NSAIDs and the onset of an intracranial and/or orbital complication exists. RESULTS: Intake of ibuprofen appeared to be a risk-factor of intracranial complications or associated orbital and intracranial complications of acute fronto-ethmoidal sinusitis in children. Neither gender nor age nor initial pain intensity were statistically related to the onset of complications. CONCLUSION AND RELEVANCE: This retrospective multicenter cohort study appears to suggest that ibuprofen increases the risk of orbital and/or intracranial complications of acute fronto-ethmoidal sinusitis in childhood. Therefore, we recommend not prescribing ibuprofen if one suspects an acute sinusitis in a child or adolescent.


Assuntos
Anti-Inflamatórios não Esteroides/efeitos adversos , Encefalopatias/induzido quimicamente , Sinusite Etmoidal/complicações , Sinusite Frontal/complicações , Ibuprofeno/efeitos adversos , Doenças Orbitárias/induzido quimicamente , Doença Aguda , Adolescente , Encefalopatias/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Doenças Orbitárias/epidemiologia , Estudos Retrospectivos , Fatores de Risco
11.
Seizure ; 18(1): 34-7, 2009 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-18657451

RESUMO

Vagal nerve stimulation (VNS) has shown promising results in various cohorts of non-surgical refractory epilepsy in adults and children. However studies report a significant delay between implantation and clinical response. We describe a cohort of 28 children and adolescents prospectively followed, classified by epileptic syndromes and treated with VNS using a 6-week rapid ramping protocol between January 2000 and March 2005. Our cohort showed favorable outcome within 6 months which was sustained at 24 months: 68% (19/28) showing >or=50% reduction in seizure frequency, including 14% (4/28) who became seizure-free. VNS was particularly efficacious in children with cryptogenic generalized and partial epilepsies. Although adverse events occurred in 68% (19/28) of patients, most were transient. In conclusion, rapid ramping is associated with an early and lasting response in most children but with a slightly higher side-effect rate.


Assuntos
Terapia por Estimulação Elétrica , Epilepsia/terapia , Pediatria , Nervo Vago/fisiologia , Adolescente , Anticonvulsivantes/uso terapêutico , Criança , Comportamento Infantil , Pré-Escolar , Transtornos Cognitivos/etiologia , Estudos de Coortes , Terapia por Estimulação Elétrica/efeitos adversos , Epilepsia/psicologia , Feminino , Humanos , Masculino , Testes Neuropsicológicos , Qualidade de Vida , Estudos Retrospectivos , Fatores de Tempo
12.
Neurochirurgie ; 65(6): 417-420, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31301388

RESUMO

BACKGROUND: Decision-making is often difficult in odontoid fracture in children. CASE REPORT: We present the case of a 6-year-old boy who sustained cervical trauma on falling out of a tree. Initial cervical X-ray and CT-scan did not find any traumatic lesion. Three-week check-up revealed an unstable C2 fracture in the synchondrosis at the base of the odontoid bone, with anterior displacement (type IC on the classification of Hosalkar et al.), without neurological symptoms except for cervical pain and limitation of head rotation. MRI confirmed the absence of medullary lesion. The Harms technique was used to fix C1 and C2, using adult instrumentation without bone graft. Bone fusion was obtained at 8 months. Hardware was removed at 10 months. No complications were reported. CONCLUSIONS: Posterior internal fixation for unstable C2 fractures in children can be effective and relatively safe.


Assuntos
Vértebras Cervicais/lesões , Fixação Interna de Fraturas/métodos , Processo Odontoide/lesões , Fraturas da Coluna Vertebral/terapia , Acidentes por Quedas , Vértebras Cervicais/cirurgia , Criança , Humanos , Masculino , Lesões do Pescoço/diagnóstico por imagem , Lesões do Pescoço/terapia , Procedimentos Neurocirúrgicos , Processo Odontoide/diagnóstico por imagem , Dor/etiologia , Fraturas da Coluna Vertebral/diagnóstico por imagem , Fusão Vertebral , Tomografia Computadorizada por Raios X
13.
Brain Stimul ; 12(5): 1121-1126, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31196836

RESUMO

BACKGROUND: An ecstatic phenomenon is an altered state of consciousness with a sense of "hyper-reality", and a complete present-moment awareness with a feeling of union with the Universe. A better understanding of the network mechanisms underlying this fascinating subjective experience may help to unravel some mysteries of human consciousness. Insula has been recently proposed to be a key region to elicit these symptoms. OBJECTIVE/HYPOTHESIS: We studied functional connectivity changes in several brain areas during the induction of ecstatic auras by direct electrical stimulation of the dorsal anterior insular cortex in patients with refractory focal epilepsy implanted with intracerebral electrodes (stereotactic-EEG, SEEG) in the context of their pre-surgical evaluation. METHODS: Three patients were selected on the basis of the occurrence of ecstatic symptoms triggered by direct intracerebral electrical stimulation (ES) of the antero-dorsal part of the insula. ES was performed (50 Hz, 1.5-2.1 mA, in a bipolar fashion to each contact in the gray matter during a 3 s period) to map functional cortices and trigger habitual seizures. One stimulation inducing ecstatic changes in each patient was analyzed. Functional connectivity analysis was performed by measuring interdependencies (nonlinear regression analysis based on the h2 coefficient) between SEEG signals before and after stimulations. RESULTS: In all patients, only the stimulation of dorsal anterior insula was able to reproduce an ecstatic aura. We observed a significant increase of functional connectivity values between several brain regions in the immediate period following stimulations. The most commonly implicated region was the dorsal anterior insula. Out-degrees (a measure intended to identify leading structures in a network) identified the dorsal anterior insula as the most common leading region in the induced networks. CONCLUSION(S): Our findings bring additional support in favor of a major role played by the dorsal anterior insula in ecstatic experiences.


Assuntos
Córtex Cerebral/fisiologia , Estimulação Encefálica Profunda/métodos , Epilepsia Resistente a Medicamentos/fisiopatologia , Emoções/fisiologia , Epilepsias Parciais/fisiopatologia , Adolescente , Adulto , Estado de Consciência/fisiologia , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/terapia , Epilepsias Parciais/diagnóstico por imagem , Epilepsias Parciais/terapia , Feminino , Humanos , Masculino , Adulto Jovem
14.
Arch Pediatr ; 26(1): 21-29, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30554853

RESUMO

INTRODUCTION: There are few studies that have investigated the long-term outcome of children who have been victims of shaken baby syndrome (SBS). However, the consequences appear to be significant and the data available from a social point of view are scarce. The main objective of this study was to define the medical and social outcome in 2016 of the infants who were victims of SBS and admitted to one of the Marseille university hospitals. The number of patients followed by a specialized team was evaluated along with their clinical state, living conditions, and whether a social support system such as the Child welfare system had been put into place. METHOD: The study was retrospective and descriptive. Patients under 1 year of age who were hospitalized between January 2005 and December 2013 and manifested clinical and paraclinical characteristics enabling the diagnosis of SBS were included in the study. The diagnosis was certain, probable, or possible according to the definitions given by the consensus of the 2011 shaken baby health authority conference (HAS). RESULTS: Eighty babies qualified for the study, three of whom died in intensive care. Only ten of these patients (12.9%) had regular (annual) neuropediatric check-up during the whole study period. Thirty-seven patients (48%) had an annual neuropediatric check-up during the first 2 years only following the diagnosis. Only 12 of the children (15.6%) were still being followed after the age of 6. The children were followed up on average for 2.5 years (29.6 months). In 24 cases (31%), the last medical visit revealed an abnormal neurological examination including multiple disabilities due to spastic quadriplegia and severe intellectual deficit, which led to total dependency in half of these cases. Forty-four patients (57%) had a normal neurological examination. Concerning the babies' social outcome, 50 patients (64.9%) had returned home, 12 (15.6%) benefited, from educational assistance at the patient's home (AEMO) following the ruling of a children's judge, and 19 (24.7%) were still placed in foster care (ASE). The average foster care placement lasted 34.7 months. DISCUSSION AND CONCLUSION: Long-term medical follow-up for children having sustained serious head injury as a result of abuse is inadequate. Sequelae such as multiple disabilities are less frequent than described in the literature. According to this study, a longer-term follow-up is necessary for children suffering from sequelae such as learning disabilities than what is actually possible in our center.


Assuntos
Maus-Tratos Infantis/estatística & dados numéricos , Síndrome do Bebê Sacudido/diagnóstico , Desenvolvimento Infantil , Proteção da Criança/estatística & dados numéricos , Pré-Escolar , Deficiências do Desenvolvimento/epidemiologia , Deficiências do Desenvolvimento/etiologia , Feminino , Seguimentos , Cuidados no Lar de Adoção/estatística & dados numéricos , França , Hospitais Universitários , Humanos , Lactente , Masculino , Estudos Retrospectivos , Síndrome do Bebê Sacudido/complicações , Síndrome do Bebê Sacudido/terapia
15.
Childs Nerv Syst ; 24(1): 143-7, 2008 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-17968559

RESUMO

CASE STUDY: We report the case of a 7-year-old boy who presented in 1998 a tumour of the left frontal lobe. Initially diagnosed as anaplastic ependymoma, the boy was treated by gross total resection followed by radiotherapy at the operated site. In July 2005, an orbital tumour was discovered and resected. The tumour was composed of sheets of rhabdoid cells which diffusely expressed vimentin and focally epithelial membrane antigen (EMA) and alpha-smooth actin by immunohistochemistry. The first tumour was re-examined. Small foci of rhabdoid cells were found. Immunohistochemistry anti-INI1 performed on both tumours was negative. Molecular techniques performed on frozen specimen of the orbital tumour confirmed the diagnosis of atypical teratoid/rhabdoid tumour (ATRT). DISCUSSION: We discuss the pathological criteria for diagnosis of ATRT and the usefulness of early radiotherapy in the light of the recent literature.


Assuntos
Neoplasias Encefálicas/diagnóstico , Teratoma/diagnóstico , Actinas/análise , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/cirurgia , Criança , Terapia Combinada , Diagnóstico Diferencial , Lobo Frontal/química , Lobo Frontal/efeitos da radiação , Lobo Frontal/cirurgia , Humanos , Imuno-Histoquímica , Masculino , Mucina-1/análise , Músculo Liso/química , Tumor Rabdoide/diagnóstico , Tumor Rabdoide/radioterapia , Tumor Rabdoide/cirurgia , Teratoma/radioterapia , Teratoma/cirurgia , Resultado do Tratamento , Vimentina/análise
16.
J Neuroradiol ; 35(5): 261-7, 2008 Dec.
Artigo em Francês | MEDLINE | ID: mdl-18472164

RESUMO

Few studies exist in the literature on pediatric brain tumors examined with advances MRI techniques. The aim of this review is to try to find out some specific tissular characteristics of the main cerebral tumors encountered in children, especially through diffusion imaging, perfusion imaging and proton magnetic resonance spectroscopy (MRS). However, hemispheric cerebral tumors are not as common as in the adult population.


Assuntos
Neoplasias Encefálicas/diagnóstico , Encéfalo/patologia , Imageamento por Ressonância Magnética/métodos , Encéfalo/metabolismo , Criança , Pré-Escolar , Meios de Contraste , Humanos , Lactente , Espectroscopia de Ressonância Magnética/métodos
17.
Neurochirurgie ; 64(3): 211-215, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29731315

RESUMO

INTRODUCTION: Currarino's syndrome (CS) is an autosomal dominant disorder of embryonic development causing a rare malformating syndrome characterized by a triad of an anorectal malformations, presacral mass (most commonly an anterior sacral meningocele) and sacral bony defects. Mutations of the HLXB9 gene have been identified in most CS cases, but a precise genotype-phenotype correlation has not been described so far. Family screening is obligatory. The diagnosis is usually made during childhood and rarely in adulthood. In this context, imaging, and especially MRI plays a major role in the diagnosis of this syndrome. Surgical management is provided by pediatric surgeons or neurosurgeons. FAMILIAL CASE REPORT: Here, we present a family case report with CS requiring different neurosurgical management. The son, a 3-year-old boy, developed a tethered spinal cord syndrome associated to a lipoma of the filum terminale, a sacro-coccygeal teratoma and an anal adhesion. A combined surgical approach permitted a good evolution on the urinary and digestive functions despite a persistent fecal incontinence. The 2-year-old daughter presented with a cyst of the thyreoglossal tract infected and fistulized to the skin. She was also followed for a very small lipoma of the filum terminale that required a neurosurgical approach. The father, 44-year-old, manifested functional digestive and urinary disorders caused by a giant anterior sacral meningocele. The ligation of the neck of the cyst and aspiration of the liquid inside in full through a posterior partial approach permit a complete collapse of the cyst with an instantly satisfactory clinical outcome. CONCLUSION: In these cases, cooperation between pediatric surgeons and neurosurgeons was crucial. The follow-up of these patients should be done in a spina bifida clinic. A geneticist evaluation must be offered to the patient in the case of a CS as well as a clinical evaluation of the relatives (parents, siblings).


Assuntos
Canal Anal/anormalidades , Anormalidades do Sistema Digestório/complicações , Lipoma/cirurgia , Procedimentos Neurocirúrgicos , Reto/anormalidades , Sacro/anormalidades , Siringomielia/complicações , Pré-Escolar , Feminino , Humanos , Lipoma/complicações , Lipoma/diagnóstico , Masculino , Meningomielocele/diagnóstico , Meningomielocele/cirurgia , Defeitos do Tubo Neural/diagnóstico , Defeitos do Tubo Neural/cirurgia , Papel do Médico
19.
Neurochirurgie ; 53(2-3 Pt 2): 223-37, 2007 Jun.
Artigo em Francês | MEDLINE | ID: mdl-17507057

RESUMO

UNLABELLED: Cavernomas represent 1.7 to 18% of all vascular malformations in children and 25% are observed in children under 18 years of age. Cases observed in neonates and infants have been published, but the mean age varies from 9.1 to 10.2 years. There is no predominance between boys and girls. CLINICAL PRESENTATION: In children, hemorrhage is a common manifestation with an incidence varying from 27.3 to 78% versus 8 to 37% in adult patients. Isolated headaches occur in 2.8% of patients and elevated ICP is observed in 20.1%. Epilepsy is reported in 16 to 60% of children, depending on the series. Neurological deficits are observed in 22.7% of patients and are more severe for deep-seated and brainstem cavernoma. About 14.2% of the cases are discovered fortuitously in asymptomatic patients. Spinal cord deficits are observed in 5% of the cases. LOCATION: Using data in the literature plus our personal series of 57 cases, 79.4% of lesions are in the supratentorial compartment and 20.6% in the posterior fossa, the majority located in the brainstem, most of them in the pons. Spinal cord cavernomas represent 5% and multiples cavernomas (12.6%) of the reviewed cases. MANAGEMENT: Appropriate management of cavernomas has long been a subject of much debate. Today, a consensus has been reached to favor medical management of asymptomatic and non hemorrhagic lesions and surgical management of symptomatic and/or hemorrhagic cavernomas whatever the localization. Progress in neuroimaging, surgical mapping, intraoperative monitoring and microsurgical techniques has greatly contributed to improved approach to those lesions. RESULTS: Results obtained in 217 cases were reviewed. Near 70% of the children are neurologically intact, 19.3% are improved or stable, 2.7% worsened and 1.13% died. Results for epilepsy are very encouraging, surgery is efficient in almost all the children except for temporal lobe cavernomas where invasive presurgical evaluation is recommended. Deep-seated and brainstem cavernomas can safely be removed in most of the cases. Only two children died from recurrent hemorrhage due to residual lesions.


Assuntos
Neoplasias do Sistema Nervoso Central/epidemiologia , Hemangioma Cavernoso do Sistema Nervoso Central/epidemiologia , Adolescente , Neoplasias do Sistema Nervoso Central/patologia , Neoplasias do Sistema Nervoso Central/cirurgia , Hemorragia Cerebral/etiologia , Criança , Pré-Escolar , Epilepsia/etiologia , Feminino , Cefaleia/etiologia , Hemangioma Cavernoso do Sistema Nervoso Central/patologia , Hemangioma Cavernoso do Sistema Nervoso Central/cirurgia , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Exame Neurológico , Procedimentos Neurocirúrgicos , Estudos Retrospectivos , Caracteres Sexuais , Fatores Sexuais , Neoplasias da Medula Espinal/etiologia
20.
Neurochirurgie ; 53(4): 307-11, 2007 Aug.
Artigo em Francês | MEDLINE | ID: mdl-17585955

RESUMO

In spite of the development of endoscopic surgery in hydrocephalus, ventriculoperitoneal shunt remains indicated in many cases particularly for non obstructive hydrocephalus. The peritoneal cavity is always an excellent receptacle for the LCS at the price of an unaesthetic abdominal scar, which may pose a problem, especially for the young adult. The trans-umbilical approach allows us to avoid the unsightly scar. Thus, between May 2004 and September 2006, a ventriculoperitoneal shunt was fashioned in 14 patients using a trans-umbilical access. Average age was 52 years and average post-intervention follow-up was 17 months. No patient developed infection, in particular in the umbilical area. One patient had to undergo a second operation for ventricular repositioning using the same peritoneal drain that could be repositioned via the trans-umbilical access with no particular problem. In one particular case, a full change of the shunt was necessary because of a sore on the shunt chamber, the material was reinstalled via the conventional umbilical access without any particular difficulty. This is the first preliminary study which confirms the feasibility of the trans-umbilical method for adults with no increase in the infectious risk, and with a very satisfactory aesthetic result in children.


Assuntos
Umbigo/cirurgia , Derivação Ventriculoperitoneal , Adolescente , Adulto , Idoso , Cicatriz/prevenção & controle , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/prevenção & controle , Complicações Pós-Operatórias/terapia , Reoperação , Infecção da Ferida Cirúrgica/epidemiologia , Infecção da Ferida Cirúrgica/terapia , Umbigo/anatomia & histologia
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