New presenting symptoms of Chiari I malformation: report of two cases

Objective  

Chiari Malformation type I nowadays represents one of the most debated and treated entities of neurosurgery. Several different symptoms of cerebellar, brainstem and spinal cord pathology have been described and attributed to this malformation. In this paper, we reported a very uncommon association of Chiari I malformation with isolated hemihypertrophy in one case and clawing hands in the second case.     

Clinical features of Chiari I malformations

Background With the advent of MRI, the diagnosis of Chiari I malformation is being made in younger patients, often with minimal or no neurologic symptoms.Clinical features The symptoms and signs of a Chiari I malformation may be related to the compression of neural and/or dural structures at the cranio-cervical junction or may be related to the associated syringomyelia. Scoliosis is a particularly important and common finding in those children with syringomyelia.     

Chiari I畸形合并脑积水的手术治疗(附33例报告)

目的　研究Chiari I畸形合并脑积水的手术方式和手术方法,并评价手术效果。方法　回顾性分析33例Chiari I畸形合并脑积水患者,全部病例均采用后颅凹减压术并在显微镜、脑室镜等辅助下切除下疝的小脑扁桃体并松解去除脑室系统内外粘连,使用神经补片减张缝合硬膜。术前术后分别作MR和脑室系统径线的测量比较并随访复查。结果　全部病例MR显示颈延髓压迫解除,枕大池形态恢复,2 3例症状明显改善,2 5例脑室明显缩小,6例脑室系统变化不显著,2例脑积水仍有发展。结论　Chiari I畸形合并脑积水以梗阻因素为主,术中除作好骨性结构的充分减压外,还应松解粘连、解除梗阻并重建枕大池,争取在手术中一次性解决颈延髓压迫和脑积水两个问题。     

Spontaneous resolution of syrinx: report of two cases in adults with Chiari malformation

Idiopathic syringomyelia is a disease with variable clinical course. We report here two cases of spontaneous resolution of cervical syrinx in adults previously diagnosed of Chiari–syringomyelia complex. They are added to the nine cases previously reported, and documented the need for careful surgical indication in this disease based on the radiological images of spinal cord cavitation.     

Basilar invagination and Chiari malformation associated with cerebellar atrophy: report of two treated cases.

We report two patients with an unusual complex of anomalies wherein the basilar invagination and Chiari malformation was associated with marked cerebellar atrophy. Both patients presented with relatively severe lower cranial nerve deficits and showed clinical improvement following a posterior foramen magnum bony decompression. The pathogenesis of the anomalies is discussed and the rationale of treatment is analysed.     

History,anatomic forms,and pathogenesis of Chiari I malformations

Introduction Chiari I malformations constitute a group of entities of congenital or acquired etiology that have in common descent of the cerebellar tonsils into the cervical spinal canal. In recent years, since the advent of magnetic resonance imaging, an increasing number of asymptomatic, doubtfully symptomatic, and minimally symptomatic patients with Chiari I malformations have been diagnosed. This has resulted in controversy about the multiple therapeutic strategies indicated for these problems.Object With the intention of updating the knowledge that we currently have on the Chiari I malformation and the related syringomyelia we review the literature and discuss the historical background, pathogenesis, anatomic forms, clinical presentation, and diagnostic procedures of these abnormalities.     

Chiari畸形合并神经性关节病:10例报告

以神经性关节病为首发症状的Chiari畸形临床上较为少见,我科自1999年1月至2005年12月共收治该类患者10例,均行显微外科治疗,疗效显著,现报告如下:一、对象与方法1.一般资料:10例患者,男4例,女6例。年龄23~48岁,平均32.6岁。2.发病部位:单个肘关节8例(右侧6例,左侧2例),单个肩关     

Cerebral arteriovenous malformations in children: report on 62 cases

A series of 62 children with cerebral arteriovenous malformations admitted to our department in the course of 17 years (1975–1992) was reviewed in a retrospective study. In 54 cases hemorrhagic stroke was the first presenting symptom, followed by epilepsy in five cases. On admission 26 children presented with a neurological deficit, and 21 were admitted with a grade 3 status according to Botterell. Fifty-one malformations were supratentorial (41 hemispheric, 10 deep-seated) while 11 were infratentorial. According to Mori's criteria, 28 lesions were small, 19 medium, and 15 large. Fifty-two children were operated on, with total excision of the malformation achieved in 47 cases. In two children the malformation recurred. The evolution of neurological disorders has been studied with a mean follow-up of 8.5 years. Fifty patients had a satisfactory outcome on the Glasgow Outcome Scale. Four children died. These results were compared with those reported elsewhere in the pediatric literature.     

Endovascular treatment of brain arteriovenous malformations ruptured during pregnancy--a report of two cases

Acutely ruptured brain arteriovenous malformations (AVMs) are a known etiology of hemorrhagic stroke during pregnancy. The aim of this paper is to report two cases of patients which presented with ruptured AVMs during pregnancy and were successfully treated with endovascular techniques. Peculiar issues related to the application of this treatment strategy in this category of patients will be discussed as well. To the best of our knowledge, this therapeutic approach in cerebral AVMs ruptured during pregnancy has not been described yet.     

Melanotic craniopharyngioma: a report of two cases

We report a 74-year-old woman and a 50-year-old woman with similar histories of headache and visual disturbance who were found to have adamantinomatous craniopharyngiomas which contained melanin pigment. This finding was confirmed by the Masson Fontana method and ultrastructural studies. These are only the second and third cases reported describing melanin pigment within a craniopharyngioma. The finding of melanin in craniopharyngiomas attests to their similarities with odontogenic tumors of the jaw, which can also contain melanin pigment and also supports the hypothesis that the histogenesis of these neoplasms derives from the vestiges of Rathke’s pouch epithelium. Received: 1 September 1998 / Revised: 22 February 1999 / Accepted: 15 March 1999     

Chordoid meningioma: a report of two cases

Chordoid meningioma is an uncommon histopathological variant of meningioma. We report 2 cases of chordoid meningioma occurring in adult patients.     

舞蹈病-棘状红细胞增多症临床研究(附两例报道)

目的 探讨舞蹈病-棘状红细胞增多症(ChAc)的临床特征、辅助检查特点及治疗方法.方法 回顾性分析郑州大学第一附属医院神经内科收治的2例ChAc患者的临床资料.结果 ChAc以舞蹈样不自主运动为主要临床表现,以唇、舌自咬为典型特点,伴有外周血棘状红细胞增多,可有癫痫、周围神经损害、认知-精神障碍、肌酶谱增高及尾状核萎缩,治疗上以对症支持为主.结论 ChAc是一种罕见的进行性遗传性神经系统变性病,其诊断主要依靠临床表现、周围血棘红细胞计数增高、特征性的影像学表现及电生理改变.该病预后差,因此对疑似病例及时行外周血涂片等检查以便早期诊断、及时治疗.

Abstract：

Objective To investigate the clinical symptoms, the results of auxiliary examinations and treatment ofchorea-acanthocytosis (ChAc). Methods Retrospective analysis of the clinical data of 2 patients with ChAc were performed. Results The main clinical symptoms of ChAc were involuntary choreiform movement, with its typical characteristic of habitual tongue and lip biting; increased amount of acanthocytes in the peripheral blood was noted; epileptic seizure, peripheral nerve injury, cognitive impairment and psychiatric symptoms, elevated muscular enzyme spectrum and shrinked caudate nucleus might appear. Symptomatic and supportive treatments were needed. Conclusion ChAc is a rare progressive hereditary neurodegenerative disease. The clinical features, increased amount of acanthocyte in the peripheral blood, characteristic imaging findings and electrophysiologic changes can be help in the diagnosis of ChAc. Its prognosis is worse, thus, early diagnosis and in-time treatment are important, which can be realized by early blood smear examination for suspicious patients.     

Dyke-Davidoff-Masson syndrome: a report of two cases

Dyke-Davidoff-Masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis and mental retardation. We report the clinical and imaging features in two patients with epilepsy revealing a Dyke-Davidoff-Masson syndrome. Brain MRI showed unilateral loss of cerebral volume with hypertrophy and hyperpneumatization of the paranasal sinuses and mastoid cells. Ipsilateral fronto-parietal polymicrogyria was present in one patient.