Methotrexate osteopathy in infants with tumors of the central nervous system

Methotrexate osteopathy, previously reported as a complication of maintenance-therapy for acute lymphoblastic leukemia, is characterized by osteopenia, particularly involving the lower extremities, thick, dense provisional zones of calcification, growth arrest lines, and corner fractures resembling scurvy. In attempts to postpone radiotherapy in infants under three years of age, the multicentric German therapy protocol for childhood central nervous system tumors (HIT-89 protocol) has employed high cumulative methotrexate doses. Here we describe osteopathy in three patients as a toxic side effect after administration of cumulative methotrexate doses of 20 g/m², 80 g/m² and 135 g/m². The high prevalence of this adverse effect in infants with tumors of the central nervous system may be attributed to the long-term therapy with high cumulative methotrexate-doses. Both factors may favor intracellular accumulation of methotrexate and formation of methotrexate-polyglutamates and may be responsible for bone toxicity. Apparently the susceptibility of the rapidly growing skeletal structures of infants under three years of age to this toxic side effect of methotrexate is remarkably high. © 1994 Wiley-Liss, Inc.     

Atypical clinical features of children with central nervous system tumor: Delayed diagnosis and switch in handedness

Herein is described the cases of three children with central nervous system (CNS) tumor, who had switch in handedness occurring before diagnostic confirmation. Although the onset, age, tumor location, and histology were heterogeneous, the diagnosis of CNS tumor was delayed in all three patients. The present experience indicates that switch in handedness should be recognized as a sign of CNS tumor in pediatric patients, and which might prevent delay in diagnosis. Pediatricians should carefully examine such patients who present with some suggestive symptoms of CNS tumor, even when they are unusual, in order to make a timely and appropriate diagnosis.     

儿童狼疮中枢神经系统的急症表现

目的　 探讨儿童系统性红斑狼疮中枢神经系统急症的特征。 方法 　将狼疮脑病患儿与继发中枢神经系统损害狼疮患儿的ANA、dsDNA滴度、Sm阳性率及补体C3下降的阳性率进行比较 ,并对两组患儿的临床特征进行分析。 结果 　ANA、ds DNA滴度及Sm阳性率、补体C3下降阳性率与狼疮脑病并不相关 ,脑电图有助于狼疮脑病的诊断。 结论 　儿童红斑狼疮中枢急症原发及继发性损害的鉴别诊断需结合临床及有关辅助检查综合分析。     

以肥胖为首发症状的复发性中枢神经系统白血病

目的分析罕见的以肥胖为首发症状的中枢神经系统白血病(CNSL)的诊断。方法 1例6岁4个月男孩,近3个月来进行性肥胖伴性格改变,以往患急性淋巴细胞白血病(ALL,普通B细胞,BCR-ABL阴性,中危),经化疗后持续缓解超过2年。根据临床表现做一系列的实验室和影像学检查进行诊断和鉴别诊断,并进行相关中英文文献复习。结果脑脊液(CSF)白细胞中度升高,以单个核细胞为主,伴少量可疑的幼稚淋巴细胞,葡萄糖和氯化物低,蛋白稍高,MRI示脑膜、大脑皮质、下丘脑和垂体柄以及桥脑等中线结构、颅神经根等信号异常。临床检查分析排除单纯肥胖、Cushing综合征等继发性肥胖和结核性脑膜炎;CSF流式细胞术检查发现65%的有核细胞符合ALL细胞的特征而诊断为CNSL复发,经化疗和头颅放疗后缓解。目前中、英文文献只检索到3篇相似病例的个案报道。结论肥胖和性格改变可以是CNSL的首发症状,但由于罕见,需注意与其他疾病鉴别,传统的CSF细胞形态学结合流式细胞术检查将提高CNSL诊断的可靠性。     

Epidemiology of central nervous system tumors in children and young adults (0-29 years), Yorkshire, United Kingdom

The authors describe the incidence and survival of 480 patients diagnosed under 30 years with a CNS tumor in Yorkshire, UK between 1990 and 2001. The effect on survival from deprivation and other prognostic factors was examined. Young adults (aged 15-29) were significantly less likely to develop CNS tumors than children (p = .001), largely because of an excess of medulloblastoma and ependymoma in the pediatric age range. No significant temporal trends in incidence were present apart from young adults with “other CNS” tumors showing an average annual increase of 10.7% (95% CI 1.3-21.0%; p = .03). Young adults had significantly lower survival rates than children (hazard ratio = 1.52, 95%CI 1.10-2.10). The highest risk of death was observed for patients from the most affluent areas. The overall burden of CNS tumors appears to be relatively constant, but the significantly poorer survival for young people needs further rapid investigation.     

Proteoglycans and injury of the central nervous system

Proteoglycan is a family of glycoproteins which carry covalently-linked glycosaminoglycan chains, such as chondroitin sulfate and heparan sulfate. Proteoglycans are believed to play important roles in morphogenesis and maintenance of various tissues including the central nervous system (CNS) through interactions with cell adhesion molecules and growth factors. In the CNS, a significant amount of evidence has been accumulated to show that proteoglycans function as modulators in various cellular events not only in the development, but also in the pathogenesis of neuronal diseases and lesions. When the CNS is injured, several chondroitin sulfate proteoglycans (CSPG) are up-regulated in glial scars formed around the lesion site. The glial scar also contains some molecules inhibitory to axonal growth, such as myelin-associated glycoprotein, Nogo, and Semaphorin. In vitro studies revealed that CSPG largely exert a repulsive effect on axonal regeneration, and a signal from CSPG modulates the actin cytoskeleton of outgrowing neurites through the Rho/ROCK pathway. These findings suggest that CSPG are responsible for unsuccessful axonal regeneration in glial scars. Various attempts to overcome the inhibitory effect of CSPG have been pursued in vivo. Digestion of chondroitin sulfate chains by chondroitinase ABC, suppression of CSPG core protein synthesis by decorin, suppression of glycosaminoglycan chain synthesis by a DNA enzyme, and inhibition of the Rho/ROCK pathway with specific inhibitors were all successful for increasing axonal regeneration. For a clinical application, the most effective combination of these treatments needs to be examined in the future.     

急性淋巴细胞白血病并中枢神经系统白血病的诊断与治疗

目的：探讨急性淋巴细胞白血病（ALL）并中枢神经系统白血病（CNSL）的诊断与治疗及影响发病和预后的因素。方法：对1990－1999年收治117例临床资料进行回顾分析。结果：CNSL发生距确诊ALL的中位数时间为8个月,高危型组发生率（54．85）明显高于标危型组（23．7％）。31例CNSL中以脑脊液（CSF）异常作出诊断远比临床症状多。CNSL治疗效果显示,大剂量氨甲喋呤＋三联鞘注＋四组（CR＋IT）相当。结论：为避免诊断假阳性造成的过度治疗,CSF仅有幼稚细胞而白细胞计数政党者诊断CNSL应慎重,HDMTX＋IT＋FC是治疗CNSL的有效措施。     

婴儿中枢神经系统肿瘤的生存和预后影响因素多中心回顾性队列研究

背景 婴儿期起病的中枢神经系统(CNS)肿瘤临床罕见,国内少见报道.目的 探讨婴儿期起病的CNS肿瘤患儿的临床特征、病理类型、治疗结局及预后的影响因素.设计回顾性队列研究.方法 以2011年6月至2019年12月婴儿原发CNS肿瘤病例为队列起点,采用多学科诊疗模式手术联合化疗,以2020年3月31日为随访终点,采集性别...     

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??Childhood acute lymphoblastic leukemia is the first disseminated cancer shown to be curable. Central nervous system leukemia ??CNSL?? is one of reasons that cause leukemia replase and is associated with a poor prognosis. Pediatricians should master the diagnostic techniques??modalities of prophylaxis and therapy of CNSL in patients with childhood acute lymphoblastic leukemia.     

Successful treatment of central nervous system juvenile xanthogranulomatosis with cladribine

Juvenile xanthogranulomatosis (JXG) is an uncommon histiocytic disease that is usually limited to the skin. Here we describe an infant with systemic JXG including a central nervous system (CNS) lesion. He was initially treated with prednisolone and vinblastine but developed an idiosyncratic reaction to prednisolone that was discontinued. The lesion then failed to respond to vinblastine monotherapy. Treatment with cladribine (2-chlorodeoxyadenosine) was subsequently successful with radiological resolution of the CNS lesion.     

Autonomic nervous system function in childhood migraine

BACKGROUND: Although the pathogenesis of migraine is controversial, autonomic nervous system (ANS) dysfunction has been reported in patients with adult migraine in recent years. The present study was planned to investigate ANS function in childhood migraine. METHODS: The migraine and control groups consisted of 25 migraineur and 30 healthy children, respectively. Orthostatic test, sustained handgrip, Valsalva ratio, 30/15 ratio and heart rate responses to deep breathing were used as non-invasive ANS function tests in both groups. RESULTS: In the orthostatic test, systolic (SBP) and diastolic blood pressures (DBP) were higher in the upright than the supine position in the migraine group, but were higher in the supine than upright position in the control group. In the sustained handgrip test, the mean difference in SBP was higher in the migraine than the control group (P = 0.0278), but there was no significant difference in DBP between migraine and control groups (P = 0.107). The Valsalva ratio was higher in the migraine than the control group (P = 0.0002), as was the 30/15 ratio (P = 0.0108). Heart rate responses to deep breathing were not different between the migraine and control groups (P = 0.749). CONCLUSIONS: Our results demonstrate ANS dysfunction, with hyperactivity of both the sympathetic and parasympathetic nervous system, in children with migraine.     

Critical review of the management of primary central nervous nongerminomatous germ cell tumors

Multimodal strategies have significantly improved the outcomes for patients with central nervous system nongerminomatous germ cell tumors. Two large cooperative group studies have recently reported much improved outcomes compared with historical series. However, a substantial proportion of patients still attain inadequate responses to initial chemotherapy prior to irradiation, with adverse impact upon survival; optimal induction chemotherapy regimens and radiotherapy strategies are as yet unidentified. Outcomes for patients with relapsed disease remain poor. There is an obvious need to incorporate molecular studies within prospective clinical trials that will likely lead to the incorporation of targeted, more effective future treatment strategies.     

幼年型类风湿关节炎中枢神经系统损害14例

目的 探讨幼年型类风湿关节炎（ＪＲＡ）发生中枢神经系统（ＣＮＳ）受损的临床特点,指导治疗,改善预后。方法 对１４例伴ＣＮＳ损害的ＪＲＡ患者的临床资料分析、总结并进行随访。结果 有ＣＮＳ损害的ＪＲＡ患者的临床资料分析、总结并进行随访。结果 有ＣＮＳ受损的ＪＲＡ患者均为全身型,７８％ＣＮＳＸ受损出现在ＪＲＡ病程１ａ以上。临床表现有：癫痫样发作、昏迷、偏瘫、头痛、嗜睡等。糖皮质激素治疗效果较好。结论 Ｊ