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Detailed linear, angular and surface area measurements were undertaken on the lateral skull radiographs of 32 patients with proven idiopathic adult Chiari type 1 malformation. Basal angles were larger, the clivus was shorter and Klaus' index was reduced in patients compared to normal controls. The size of the posterior fossa was smaller in patients than in controls. By discriminant analysis of the skull X-ray data it was possible to identify correctly two thirds of patients as belonging to the patient group. The findings indicate that craniocervical bony anomalies in the adult Chiari malformation are commoner than was previously realised, and they support the view that tonsillar herniation in these patients results from bony occipital dysplasia.  相似文献   

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Introduction Rates of neurocognitive risk range from 35–50% of school-aged children with isolated single suture craniosynostosis (SSC). It has been hypothesized that early surgical intervention to release suture fusion reduces risk for increased intracranial pressure (ICP) and the corresponding risk to neurodevelopment. However, studies assessing children with SSC have been inconsistent in finding an association between neurocognitive development, age of surgery, and ICP. Review SSC produces notable distortion of the cranial vault and underlying brain mass. Although a linear relationship between skull distortion, ICP, and neurocognitive deficits has generally been assumed, recent studies have postulated an interactive process between the skull and developing brain that results in neuroanatomical changes that are not limited to areas directly beneath the fused suture. The specific neuropsychological deficits identified in children with SSC including problems with attention and planning, processing speed, visual spatial skills, language, reading, and spelling may be related to the anatomic differences that persist after correction of suture fusion. Conclusions Available literature on neurocognitive development of children with SSC is suggestive of mild but persistent neuropsychological deficits, which become more significant as cognitive demands increase at school age. Anatomical studies of children without SSC are beginning to identify particular groups of brain structures that if disrupted or malformed, may be associated with specific cognitive deficits. Controlled research investigating the relationship between persistent anatomical changes and neurocognitive functioning of school-aged children with SSC is needed.  相似文献   

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Background Previous studies on visual function in craniosynostosis have mainly focused on ocular movements and ophthalmologic findings. More recently, some studies also included the assessment of more functional and electrophysiological aspects of vision, such as acuity and visual evoked potentials. Methods We reviewed all the relevant publications on visual findings in infants and children with both syndromic and nonsyndromic craniosynostosis and reported our own recent experience on the presurgical assessment of visual function in infants with single suture nonsyndromic craniosynostosis. Results Most studies report abnormal ophthalmologic findings, mainly strabismus and refractive deficits. Only few recent studies, including ours, have reported the impairment of more functional aspects of vision, such as visual acuity and visual evoked potentials in relation to the various forms of craniosynostoses. Discussion We suggest a few guidelines for further studies, which may help to better elucidate the mechanisms underlying possible visual impairment in the various types of craniosynostosis. Presented at the Consensus Conference on Pediatric Neurosurgery, Rome, 1–2 December 2006  相似文献   

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This study examined early language acquisition in children with single suture craniosynostosis (SSC) and in children with deformational posterior plagiocephaly. Our purpose was to determine whether infants with SSC have normal language acquisition at the age of 3 years, and whether infants with deformational posterior plagiocephaly demonstrate parallel development when compared with children with SSC.  相似文献   

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目的 探讨Chiari畸形合并颅颈交界畸形的临床特点及治疗方法.方法 回顾性分析56例Chiari畸形合并颅颈交界畸形的临床资料,其中合并颅底陷入35例,寰枕融合25例,颈椎分节不全6例.病人均行颅后窝减压硬脑膜扩大修补术,行小脑扁桃体部分切除术21例.固定方式采用钛缆固定和自体骨移植41例,C<,1>侧块~C<,2>椎弓根螺钉固定和自体骨移植15例.结果 术后发生颅内感染2例,脑脊液切口漏1例.55例随访6~60个月,平均42个月;植骨融合满意52例,钛缆固定和自体骨移植植骨未能融合3例;症状改善51例(92.7%),无变化4例(7.3%);随访期间无死亡病例.结论 Chiari畸形合并颅颈交界畸形应行颅颈交界关节稳定性检查及评估,对有潜在颅颈交界不稳定病人应行后路减压并枕颈植骨固定融合术,单纯后路减压可能加重颅颈交界不稳定,症状难以缓解甚至加重.  相似文献   

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Introduction One third of patients with craniofacial dysostosis syndromes and about 15–20% of children with single suture craniostenosis have a documented increase in intracranial pressure (ICP). The early detection of intracranial hypertension is important in order to reduce the risks for brain development and visual function. However, in children with craniosynostosis, the clinical manifestations of abnormally increased ICP are difficult to detect, as the majority of patients may have neither warning signs nor symptoms for a long period of time.Review Moreover, data from the literature suggest that neither fundoscopic nor radiological findings are necessarily related to intracranial pressure recordings in this type of pathology. In this context prolonged ICP monitoring seems actually to be the most valuable diagnostic tool. Extradural, subdural, and intraparenchymal devices have been used, and different softwares have been employed for PC storage and analysis of the data obtained. Most recent series consider mean ICP combined with plateaux waves as the most sensitive indicators in the final evaluation of the results. Conclusions The analysis of the literature demonstrates a significant difference in the prevalence of raised ICP preoperatively between non-syndromic and syndromic patients. Among the non-syndromic children, a direct relation between the number of sutures involved and raised ICP is documented. The analysis of plateaux waves seems to be particularly important in children with borderline mean ICP values. Persistent postoperative raised ICP has been described in 6–15% of patients with craniofacial dysostosis. It must be related to the multifactorial etiology of increased ICP in these patients, which includes cerebral venous congestion, upper airway obstruction, and hydrocephalus.  相似文献   

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We reported a post-marketing experience of 190 patients affected by relapsing multiple sclerosis on treatment with natalizumab. Clinical findings during pre-treatment period and throughout the study were documented. Magnetic resonance imaging (MRI) scans were performed at baseline and at 6, 12, and 24 months of therapy. Cumulative proportions of patients disease activity free (i.e. absence of relapses, disability progression, MRI activity) were measured as efficacy endpoints. Despite that the baseline characteristics suggested a more severe course of disease in our sample than that of the AFFIRM trial, data on effectiveness of natalizumab were comparable. At 1 year of treatment we found 80 and 70% patients free from relapses and MRI activity, respectively, that is similar to 75 and 62% detected in the AFFIRM trial. Moreover, only 5% of our patients showed a progression of disability after a mean follow-up time of 15 months. Finally, the presence of antibodies anti-Natalizumab was strongly related to the occurrence of relapses (p = 0.002) and MRI activity (p < 0.001) even in the post-marketing experience.  相似文献   

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This report presents three families with Chiari malformation type I that became symptomatic during childhood: a mother and son; a set of monozygotic twins; and two half-siblings and their two maternal cousins. These children presented with various symptoms, including headache, stiff neck, and swallowing difficulty. A review of the relevant literature is presented, with an emphasis on familial examples and proposed inheritance. Less common presentations of Chiari malformation type I are discussed, as well as the possible pathogenesis of Chiari malformation type I and associated syringomyelia.  相似文献   

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Background Both the diagnosis and treatment regimens for the Chiari I malformation (CIM) are varied and controversial. The present paper analyzes the literature regarding this form of hindbrain herniation in regard to definition, anatomy, pathobiology, symptoms, findings, treatment, and outcomes. Discussions Appropriate literature germane to the CIM is reviewed and discussed. There is variation in the reported anatomy, outcome, and treatment for children with CIM. Based on the literature, most patients have preoperative symptoms or findings (e.g., syringomyelia) improve no matter what surgical technique is utilized. However, standardized treatment paradigms based on randomized controlled studies are still necessary to elucidate the optimal selection and treatment criteria.  相似文献   

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Introduction It is the Chiari II malformation and its effects that determine the quality of life of the individual born with spina bifida.Discussion The cause of this malformation has been a source of debate for many years. Understanding the cause enables strategies for the management of problems created by this malformation to be developed. An open neural tube defect allows fluid to escape from the cranial vesicles, altering the intracranial environment and leads to all of the brain changes seen in the Chiari II malformation. Decompression of the intracranial vesicles causes overcrowding, decrease in the size of the third ventricle, and changes in the fetal skull. It also permanently links the intracranial ventricular system to the spinal cord central canal.  相似文献   

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目的探讨ChiariⅠ畸形的外科治疗方法。方法采用后颅窝减压,扩大重建术治疗6例;后颅窝减压,扩大成形术加脊髓空洞分流术治疗10例。结果所有患者症状和体征均明显改善,无死亡和其他并发症。结论后颅窝减压,扩大成形术和(或)脊髓空洞分流术是治疗ChiariⅠ畸形的有效方法。  相似文献   

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Segmental hyperhidrosis was seen in three cases of syringomyelia with Chiari malformation confirmed by magnetic resonance imaging. Using the iodine starch reaction, all three cases showed hyperhidrosis in the areas roughly corresponding to those of sensory disturbances. Two possible modes of pathogenesis are discussed: stimulation of preganglionic neurons and interference to the inhibitory tract.  相似文献   

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Case report We report on a 7-year-old girl with generalized epilepsy and mental retardation. Discussion Neurologic examination was normal and only facial dysmorphic features, compatible with frontonasal dysplasia, were observed. However, magnetic resonance imaging (MRI) revealed Chiari I malformation together with bilateral opercular polymicrogyria. To our knowledge, this is the first report of the association of these developmental disorders. This condition raises questions regarding the ethiopathogenetic classification of Chiari spectrum and as to whether embryologic and genetic causes could be potentially interconnected.  相似文献   

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BACKGROUND: Saccadic adaptation corrects errors in saccadic amplitude. Experimentally-induced saccadic adaptation provides a method for studying motor learning. The cerebellum is a major participant in saccadic adaptation. Chiari type II malformation (CII) is a developmental deformity of the cerebellum and brainstem that is associated with spina bifida. We investigated the effects of CII on saccadic adaptation. METHOD: We measured eye movements using an infrared eye tracker in 21 subjects with CII (CII group) and 39 typically developing children (control group), aged 8-19 years. Saccadic adaptation was induced experimentally using targets that stepped horizontally 120 to the right and then stepped backward 3 degrees during saccades. RESULTS: Saccadic adaptation was achieved at the end of the adaptation phase in participants in each group. Saccadic amplitude gain decreased by 6.9% in the CII group and 9.3% in the control group. The groups did not differ significantly (p = 0.27). Amplitude gain reduction was significantly less in the CII participants who had multiple shunt revisions. Regression analyses revealed no effects of spinal lesion level, presence of nystagmus, or cerebellar vermis dysmorphology on saccadic adaptation. CONCLUSION: The neural circuits involved in saccadic adaptation appear to be functionally intact in CII.  相似文献   

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