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Neurogenic arthrogryposis multiplex congenita: clinical and muscle biopsy findings. 神经源性多发性先天性关节挛缩:临床和肌肉活检结果。
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120370
C Adams, L E Becker, E G Murphy
{"title":"Neurogenic arthrogryposis multiplex congenita: clinical and muscle biopsy findings.","authors":"C Adams,&nbsp;L E Becker,&nbsp;E G Murphy","doi":"10.1159/000120370","DOIUrl":"https://doi.org/10.1159/000120370","url":null,"abstract":"<p><p>Thirteen cases of arthrogryposis multiplex congenita without evidence of spinal muscular atrophy, congenital muscular dystrophy, or structural myopathy were reviewed. Family history, consanguinity, pregnancy, delivery, number and severity of contractures, and outcome were evaluated. Laboratory investigations had been performed and a biopsy of muscle from an affected limb had been examined histochemically and by electron microscopy. Five biopsies showed fiber type 1 predominance and three had type 2 predominance. Patterns of fiber-type predominance may have resulted from altered neural influence leading to impaired maturation of type 1 or 2 motor units. Nine patients had been followed up for 3-8 years. Two were still not walking at age 8 years. For the 6 who were walking, the mean age at ambulation was 4.7 years. There was no deterioration in power. Muscle biopsy is recommended in arthrogryposis multiplex congenita. Identification of a probable neurogenic arthrogryposis multiplex congenita is important because the condition is not progressive and is apparently not transmitted genetically.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 2","pages":"97-102"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120370","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14380283","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 7
Cerebral hemiatrophy--correlation of human with animal experimental data. 脑半球萎缩——人与动物实验数据的相关性。
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120375
L Ford, G M de Courten-Myers, T Mandybur, R E Myers
{"title":"Cerebral hemiatrophy--correlation of human with animal experimental data.","authors":"L Ford,&nbsp;G M de Courten-Myers,&nbsp;T Mandybur,&nbsp;R E Myers","doi":"10.1159/000120375","DOIUrl":"https://doi.org/10.1159/000120375","url":null,"abstract":"<p><p>We report the neuropathologic findings in a 63-year-old white male with a history of birth asphyxia, cerebral palsy, seizures and mild mental retardation in conjunction with similar brain pathologic findings in animal models of perinatal asphyxia. The human case showed a left cerebral hemispheric hemiatrophy associated with an extensive ulegyria involving all cerebral lobes on that side and a single microscopic focus of cortical atrophy in the right hemisphere. Among a large number of experimental perinatal asphyctic exposures only an occasional animal, like the human case described, showed unilateral hemispheric injury with softening and necrosis if examined early and ulegyria with hemispheric hemiatrophy if examined late. The present paper suggests that perinatal asphyxia under specific pathophysiologic conditions may cause unilateral brain injury. Our experimental studies suggest the specific condition of perinatal asphyxia potentially causing unilateral or asymmetrical brain damage is marked hypoxemia combined with substantial reductions in blood pressure but without circulatory collapse. Given these conditions, the asymmetry of the brain damage likely reflects fetal head position within the gravitational field relative to the heart. With disturbed cerebral blood flow autoregulation from asphyxia, the gravitational field likely accentuates the ischemia of those brain areas most elevated above the level of the heart. Thus, we postulate head position may play a pivotal role in defining brain regions that are damaged in hypotensive perinatal asphyxia. This interpretation may affect the intensive care of hypoxemic, hypotensive newborns aimed at minimizing the risk of brain damage.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 3","pages":"114-9"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120375","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14381110","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 7
Rapid deterioration in a three-year-old with left hemiparesis. 三岁左偏瘫患者病情迅速恶化。
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120377
S Ashwal, L B Rorke, M A Epstein, J L Sladky, R A Zimmermann
{"title":"Rapid deterioration in a three-year-old with left hemiparesis.","authors":"S Ashwal,&nbsp;L B Rorke,&nbsp;M A Epstein,&nbsp;J L Sladky,&nbsp;R A Zimmermann","doi":"10.1159/000120377","DOIUrl":"https://doi.org/10.1159/000120377","url":null,"abstract":"","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 3","pages":"124-33"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120377","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14381112","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
Development of the human motor trigeminal nucleus. 人类运动三叉神经核的发展。
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120395
S Hamano, N Goto, T Nara
{"title":"Development of the human motor trigeminal nucleus.","authors":"S Hamano,&nbsp;N Goto,&nbsp;T Nara","doi":"10.1159/000120395","DOIUrl":"https://doi.org/10.1159/000120395","url":null,"abstract":"<p><p>The morphometric features of the motor trigeminal nucleus of the developing human brain were examined using complete serial celloidin sections of 13 brains, including 10 fetal brains. Our results suggest that the developmental processes of the motor trigeminal nucleus should be divided into four stages as follows: (1) primary stage, characterized by the early neuronal differentiation; (2) secondary or preparatory stage; (3) tertiary stage, characterized by massive neuronal death, and (4) postnatal stage, characterized by the maturation of neuropils. There were differences between the neuronal death observed in human and animal brains. Possible implications of these observations for the development of the nervous system are discussed.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 5","pages":"230-5"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120395","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14394200","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 20
Brainstem glioma with multiple intraspinal metastases during life: case report and review of the literature. 脑干胶质瘤伴生命中多发椎管内转移:病例报告及文献回顾。
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120371
D Silbergeld, M Berger, B Griffin, A Bleyer, R Geyer, J Milstein, J Eskridge
{"title":"Brainstem glioma with multiple intraspinal metastases during life: case report and review of the literature.","authors":"D Silbergeld,&nbsp;M Berger,&nbsp;B Griffin,&nbsp;A Bleyer,&nbsp;R Geyer,&nbsp;J Milstein,&nbsp;J Eskridge","doi":"10.1159/000120371","DOIUrl":"https://doi.org/10.1159/000120371","url":null,"abstract":"<p><p>We present a case of a 17-year-old male with an unbiopsied brainstem glioma who developed drop metastases a few months following completion of hyperfractionated radiation therapy to the primary site. The literature is reviewed with reference to intraspinal metastases from brainstem gliomas.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 2","pages":"103-7"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120371","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13992467","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 9
Diastematomyelia associated with ectopic renal tissue. 与异位肾组织有关的肺气肿。
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120372
S J Gaskill, K Kagen-Hallett, A E Marlin
{"title":"Diastematomyelia associated with ectopic renal tissue.","authors":"S J Gaskill,&nbsp;K Kagen-Hallett,&nbsp;A E Marlin","doi":"10.1159/000120372","DOIUrl":"https://doi.org/10.1159/000120372","url":null,"abstract":"<p><p>A case of diastematomyelia associated with ectopic renal tissue is presented. While findings of ectopic renal tissue and Wilms tumor in dysraphic conditions have been reported in the literature, there has been no incorporation of these findings into the theories of the development of neural tube defects. This case supports the hypothesis of neural tube defects secondary to rupture of a previously intact neural tube (secondary neural tube defect). A discussion and review of the literature regarding the hypotheses of primary and secondary neural tube defects is included.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 2","pages":"108-11"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120372","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13992468","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 20
Parietal cephaloceles: radiographic and magnetic resonance imaging evaluation. 顶叶性脑膨出:影像学和磁共振成像评价。
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120366
J T Curnes, W J Oakes
{"title":"Parietal cephaloceles: radiographic and magnetic resonance imaging evaluation.","authors":"J T Curnes,&nbsp;W J Oakes","doi":"10.1159/000120366","DOIUrl":"https://doi.org/10.1159/000120366","url":null,"abstract":"<p><p>Three patients with parietal cephaloceles underwent evaluation and treatment at Duke University Medical Center between 1984 and 1987. All presented within the first 2 years of life with painful swelling near the vertex of the head. All patients had skull films and computed tomography, and two underwent magnetic resonance imaging (MRI). All 3 children had associated hindbrain deformities; two with Dandy-Walker malformation, the third with a Chiari II malformation. Each child eventually developed hydrocephalus. MRI is the procedure of choice to evaluate these patients, providing direct sagittal imaging of the posterior fossa and craniocervical junction, and displaying communication of the cephalocele with intracranial structures as well as associated venous vascular anomalies.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 2","pages":"71-6"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120366","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14380280","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 20
Patient data system for monitoring shunts. 用于监测分流的患者数据系统。
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120401
E Frank, E Su, K Smith
{"title":"Patient data system for monitoring shunts.","authors":"E Frank,&nbsp;E Su,&nbsp;K Smith","doi":"10.1159/000120401","DOIUrl":"https://doi.org/10.1159/000120401","url":null,"abstract":"<p><p>Rapidly locating accurate data on a patient's shunt system is often extremely difficult. We have developed a simple system to fill a perceived need for recording current data on a patients shunt. This system employs an easily updated record in the patient's hospital or clinic chart as well as a wallet-sized data card for the patient or his family to carry. The data in the chart include the configuration of the patient's current shunt system and a graphic record of previous shunt problems. The small patient data card describes the age of the shunt system and its current configuration. We have found that this system provides assistance in the routine follow-up of patients with shunts and plays an extremely necessary role in the emergency evaluation of these patients, particularly when an emergency evaluation is undertaken in facilities distant from the location of regular treatment.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 5","pages":"258-63"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120401","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14394203","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Symptomatic Chiari I malformation in childhood: a report of 7 cases. 儿童症状性I型恰氏体畸形7例报告
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120386
R C Dauser, M A DiPietro, J L Venes
{"title":"Symptomatic Chiari I malformation in childhood: a report of 7 cases.","authors":"R C Dauser,&nbsp;M A DiPietro,&nbsp;J L Venes","doi":"10.1159/000120386","DOIUrl":"https://doi.org/10.1159/000120386","url":null,"abstract":"<p><p>The Chiari I malformation, once thought to be a disorder of clinical importance only in the teen years and beyond, is now recognized as being significant in the pediatric population as well. We have reviewed 7 cases of Chiari I malformation in children less than 12 years of age. Hydrosyringomyelia was often, but not invariably, an accompanying feature, and this in turn was associated with scoliosis and motor weakness. All patients had posterior fossa and cervical decompression with dural grafting procedures, as well as various types of syringosubarachnoid shunts placed. Intraoperative ultrasound was found to be a valuable surgical adjunct. Symptoms and signs were improved or stabilized in all patients. We feel that this disorder is now more commonly reported in young children because of the increased availability of noninvasive neuroimaging techniques, especially magnetic resonance imaging. Questions are raised as to the actual incidence of undiscovered cases of this disorder in children with commonly seen conditions such as scoliosis.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 4","pages":"184-90"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120386","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14396024","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 62
Epithelioid hemangioendothelioma of the central nervous system. Immunohistochemical and ultrastructural observations of a pediatric case. 中枢神经系统上皮样血管内皮瘤。1例小儿病例的免疫组织化学和超微结构观察。
Pediatric neuroscience Pub Date : 1988-01-01 DOI: 10.1159/000120355
A L Taratuto, G Zurbriggen, G Sevlever, M Saccoliti
{"title":"Epithelioid hemangioendothelioma of the central nervous system. Immunohistochemical and ultrastructural observations of a pediatric case.","authors":"A L Taratuto,&nbsp;G Zurbriggen,&nbsp;G Sevlever,&nbsp;M Saccoliti","doi":"10.1159/000120355","DOIUrl":"https://doi.org/10.1159/000120355","url":null,"abstract":"<p><p>In 1982, Weiss and Enzinger described a group of soft tissue tumors in adults that they called epithelioid hemangioendothelioma [Cancer 50: 970-981, 1982]. Such tumors have also been found in heart, bone, liver, lung and lymph nodes, and Kepes and Rubinstein described two intracranial examples in adults in 1986 [J. Neuropath. exp. Neurol. 45:319, 1986]. This report documents clinical and pathological features of an epithelioid hemangioendothelioma in the parietal lobe of a 4-year-old boy.</p>","PeriodicalId":77766,"journal":{"name":"Pediatric neuroscience","volume":"14 1","pages":"11-4"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000120355","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14346224","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 48
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