低血糖脑病的临床和神经影像学特点

目的 探讨低血糖脑病(HE)的临床和神经影像学特点.方法 回顾性分析49例HE患者的临床和神经影像学资料.结果 本组HE患者临床表现为意识障碍26例,精神行为异常19例,癫癎发作6例,言语不清、反应迟钝10例,偏瘫和单肢瘫5例,去脑强直发作3例.23例行头颅CT检查,除10例见有脑梗死和脑萎缩外余无异常改变.13例行MRI检查,7例异常,其中显示弥漫性皮质和海马受累者3例(2例同时波及基底节区,1例仅累及右侧大脑皮质,MRS示病变区乳酸峰无明显异常),胼胝体压部受损2例(1例患者10 d后病变恢复,1例伴内囊后肢受累),壳核和放射冠区白质受累各1例,表现为等或稍长T1、长T2异常信号,DWI呈高信号伴表观扩散系数(ADC)降低.结论 HE的临床表现多不典型;MRI有高度特异性,DWI序列对病变更敏感.     

可逆性后部脑病综合征的临床和影像学特点

目的探讨可逆性后部脑病综合征的临床表现和影像学特点。方法回顾性分析3例PRES的临床和影像学资料。结果本组病例继发于系统性红斑狼疮、显微镜下多发性血管炎、病毒性脑膜炎各1例,其临床表现主要有头痛、意识障碍、痫性发作、视力受损和血压升高。3例均行头颅MRI检查,显示以双侧顶、枕叶为主的皮层和/或皮层下T1低或等信号,T2及FLAIR高信号。治疗后3例于2周内临床症状均明显好转,1例于起病后14d复查MRI基本恢复正常。结论PRES具有特征性的临床表现和影像学特点,提高对该病认识,早期诊断和适当治疗有助于恢复,避免疾病进展和发生不可逆的脑损伤。     

老年人低血糖脑病的临床及神经影像学特征

目的 探讨老年人低血糖脑病的临床及神经影像学特点.方法 对36例低血糖伴神经系统表现的患者进行病史回顾、临床分析及颅脑CT和MRI检查.结果 36例低血糖患者11例发现颅脑MRI典型的双侧尾状核和豆状核对称性异常信号,而脑CT相应部位未见明显异常,病前27例有感染、发热等诱因,临床表现多种多样.低血糖脑病的发生以老年糖尿病患者口服或注射降糖药者居多.随访6个月,神经影像学无明显好转.结论 低血糖脑病临床表现多不典型,对意识障碍的患者,要注意低血糖反应.颅脑CT对低血糖脑病的诊断价值不大,颅脑MRI对重症患者的诊断具有重要价值.特征性的磁共振表现预示着病情危重,且预后不良.     

Hypertensive brainstem encephalopathy: clinical and radiographic features

Reversible posterior leukoencephalopathy syndrome associated with hypertension rarely presents with predominant involvement of the brainstem and relative sparing of the supratentorial regions. We describe the clinical and neuroimaging features of three patients with reversible hypertensive brainstem encephalopathy. Headache and confusion in the setting of severe hypertension, with a relative paucity of brainstem signs and symptoms, despite extensive neuroimaging abnormalities in the brainstem support the diagnosis. The clinical findings and neuroimaging abnormalities resolve with treatment of the hypertension, further distinguishing this syndrome from brainstem infarction.     

Leptomeningeal carcinomatosis: Prognostic value of clinical,cerebrospinal fluid,and neuroimaging features

Introduction

Leptomeningeal carcinomatosis (LC) is a devastating complication occurring in 5% of all patients with cancer. To date there are no well-established prognostic markers in patients with LC, except for the presence of cerebrospinal fluid (CSF) blocks and the Karnofsky performance status scale (KPS). We aimed to identify clinical, neuroradiologic and CSF prognostic factors related to LC survival and to develop an easy-to-use Prognostic Scoring Scale (PSS) to identify patients who are more likely to benefit from receiving treatment.

Methods

Single-center retrospective study evaluating patients who had a diagnosis of LC during a 10-year period. Diagnosis was made by malignant cytology or imaging; suspicious cases treated as LC were also included.

Results

Fifty patients with LC were analyzed (58% women). Median age was 54.4 years, and KPS was 60%. The most common types of tumor were breast (35%), lung (24%), and hematologic malignancies (16%). Thirty-two percent of patients were diagnosed by imaging, 22% by cytology, and 40% by both. Median overall survival (OS) was 10 weeks (95% confidence interval 5.1–14.9). Median OS for patients who received specific treatment was 21.2 weeks vs. 6.38 weeks for patients receiving supportive care only (p < 0.001). In multivariate analysis, initial KPS, initial CSF protein level (<112 mg/dL) and time from diagnosis of primary tumor to diagnosis of LC (>67 weeks) were significant and independent predictors of increased survival.

Conclusions

Prognosis remains poor in LC. The predictive factors for patients with LC here identified could help to improve the selection of patients who are more likely to benefit from receiving treatment.     

Vogt-小柳-原田综合征五例临床及神经影像学特点

目的 探讨Vogt-小柳-原田综合征的临床及神经影像学特点。方法 回顾我院收治的5例Vogt-小柳-原田综合征患者,总结其临床、脑脊液细胞学、神经影像学特点及药物治疗的效果。结果 5例患者均在发病早期确诊葡萄膜炎,并出现头痛症状,3例出现脑膜刺激征,所有患者颅脑MRI强化扫描均显示软脑膜较均匀强化。脑脊液白细胞计数均增高[(4 - 196)×106/L]。5例患者经大剂量激素联合环磷酰胺治疗均得到有效控制。结论 假性脑膜炎是Vogt -小柳-原田综合征最常见的中枢神经系统表现,脑脊液细胞学检查主要表现为白细胞计数增高,颅脑MRI平扫及强化对该病的诊断具有一定价值。大剂量激素联合环磷酰胺治疗疗效确切。     

Novel form of intermediate salla disease: clinical and neuroimaging features

The objective of this article is to describe the clinical, radiographic, and molecular genetic features of a new intermediate form of free sialic storage disease. Free sialic storage disease is a rare autosomal recessive lysosomal disorder that results from mutations in SLC17A5, a gene that codes for sialin, a lysosomal membrane sialic acid transporting protein. Infantile sialic acid storage disease has a severe phenotype, and Salla disease (Finnish variant) is generally milder in phenotype; intermediate forms have also been described. There have been few reports of magnetic resonance imaging (MRI) in the sialic acid storage disorders; leukodystrophy has been the characteristic finding, along with hypoplasia of the corpus callosum. An 8-month-old non-Finnish child presented with hypotonia and global developmental delay. Serial MRIs with magnetic resonance spectroscopy at 9 and 16 months revealed severe hypomyelination and hypogenesis of the corpus callosum. There was mild elevation of urinary sialic acid (4.5 times above normal). Electron microscopy of a skin biopsy showed lysosomal enlargement with oligosaccharide storage, and confirmatory molecular genetic testing revealed compound heterozygosity for two new SLC17A5 mutations. Free sialic storage disease of the intermediate type is an important part of the differential diagnosis of a hypotonic, delayed child with abnormal white matter on MRI. Intermediate types of free sialic acid overlap in phenotype with infantile sialic acid storage disease and the milder Salla disease and thus might be more difficult to identify clinically; the lack of Finnish ethnicity should not preclude testing for this probably under-recognized disorder. White-matter abnormalities appear to be characteristic of the entire phenotypic spectrum.     

Registration of neuroimaging data: implementation and clinical applications.

Image registration brings images into a form in which each voxel corresponds to a predetermined anatomic entity and is necessary for comparisons of data across scans. Intrasubject registration is a matter of translating and rotating one image volume into correspondence with another. Intersubject registration is more difficult because it requires the removal of individual anatomy dependence from the data. This article describes, with the help of clinical examples, automated methods for intrasubject registration of scans within and between modalities, and intersubject registration used for registering a three-dimensional brain atlas with a patient's brain scan.     

Long-term neurocognitive and neuroimaging outcomes in posterior reversible encephalopathy syndrome: two case reports and implications

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic and neurometabolic state associated with a distinct appearance on computed tomography and magnetic resonance imaging, as well as particular clinical features. While PRES is defined as a reversible condition, instances of irreversible or persisting cytotoxic edema have been reported and appear to be associated with poor outcomes. Studies addressing the post-acute neurocognitive findings in persisting/irreversible PRES are lacking. We report two cases of persisting PRES with associated neurocognitive deficits and functional limitations following acute presentation. Neuroimaging revealed abnormalities within the posterior parietal lobes, along with bilateral involvement of the occipital and frontal lobes. The results from the neuropsychological examination revealed a pattern of impairment generally affecting spatial-perceptual and related functions and aspects of executive function. These cases document instances of irreversible/persisting PRES with associated neurocognitive deficits and functional limitations generally corresponding with the areas implicated on neuroimaging. Studies addressing the post-acute neurocognitive presentation and functional outcomes of PRES are needed.     

Subacute Hashimoto's encephalopathy, treated with plasmapheresis

We report a patient with subacute diffuse encephalopathy characterised by rapidly progressive dementia with visual hallucinations, myoclonus and generalised seizures. She was euthyroid but showed high serum levels of thyreoglobulin and thyreoperoxidase antibodies. Hashimoto's encephalopathy was diagnosed. MRI of the brain in the acute phase demonstrated no structural abnormalities. However in the mesotemporal regions and the anterior parts of the brain, a decrease of the N-acetylaspartate and an increase of the Choline-containing compounds was found on MRI-spectroscopy. Reversal of these abnormalities was demonstrated a few months later after starting therapy. Plasmapheresis resulted in normalisation of serum levels of the antibodies and rapid clinical improvement. This observation supports the idea that a correlation exists between the serum levels of the thyroid auto-antibodies and the course of the clinical illness.     

神经元腊样质脂褐素沉积病的临床和影像学特点

目的探讨神经元腊样质脂褐素沉积病（NCL）的临床表现规律和影像学特点。方法回顾分析我院经病理检查确诊的11例NCL患者的临床和影像学资料，对比分析国内报道的15例同类型NCL的临床和影像学资料。结果26例患者中青少年型NCL12例，占46％，3～15岁发病，首发症状多为智力减退或癫痫发作。晚期婴儿型NCL8例，占31％，1～7岁发病，首发症状表现为癫痫发作。婴儿型NCL4例，占15％，出生后4～9个月发病，首发症状表现为智能和运动发育停滞。成年型NCL2例，占8％，26及32岁发病，以痴呆和精神异常为首发症状。磁共振成像（MRI）特点主要表现为弥漫性脑萎缩，部分患者伴随白质损害，婴儿型和晚期婴儿型NCL出现丘脑改变。结论NCL患者的发病类型以青少年型为主，不同类型的NCL临床症状及出现顺序各异，影像学改变类似，婴儿型及晚发婴儿型伴丘脑损害。     

INCloud: integrated neuroimaging cloud for data collection,management, analysis and clinical translations

BackgroundNeuroimaging techniques provide rich and accurate measures of brain structure and function, and have become one of the most popular methods in mental health and neuroscience research. Rapidly growing neuroimaging research generates massive amounts of data, bringing new challenges in data collection, large-scale data management, efficient computing requirements and data mining and analyses.AimsTo tackle the challenges and promote the application of neuroimaging technology in clinical practice, we developed an integrated neuroimaging cloud (INCloud). INCloud provides a full-stack solution for the entire process of large-scale neuroimaging data collection, management, analysis and clinical applications.MethodsINCloud consists of data acquisition systems, a data warehouse, automatic multimodal image quality check and processing systems, a brain feature library, a high-performance computing cluster and computer-aided diagnosis systems (CADS) for mental disorders. A unique design of INCloud is the brain feature library that converts the unit of data management from image to image features such as hippocampal volume. Connecting the CADS to the scientific database, INCloud allows the accumulation of scientific data to continuously improve the accuracy of objective diagnosis of mental disorders.ResultsUsers can manage and analyze neuroimaging data on INCloud, without the need to download them to the local device. INCloud users can query, manage, analyze and share image features based on customized criteria. Several examples of ''mega-analyses'' based on the brain feature library are shown.ConclusionsCompared with traditional neuroimaging acquisition and analysis workflow, INCloud features safe and convenient data management and sharing, reduced technical requirements for researchers, high-efficiency computing and data mining, and straightforward translations to clinical service. The design and implementation of the system are also applicable to imaging research platforms in other fields.     

Dengue encephalopathy: very unusual neuroimaging findings

Neuroimaging, in many patients with dengue encephalopathy, may reveal periventricular signal changes. We report a 25-year-old man, who presented with altered sensorium. Dengue-IgM test in serum was positive. Cerebrospinal fluid examination was normal. MRI brain revealed presence of bilateral parieto-occipital intraparenchymal bleed with mass effect. Neuroimaging was consistent with posterior reversible encephalopathy syndrome. We report posterior reversible encephalopathy syndrome in a normotensive patient with dengue encephalopathy and systemic metabolic alterations.     

Subacute spongiform encephalopathy: Electron microscopic studies

Electron microscopic changes in brain biopsies from two cases of subacute spongiform encephalopathy are described. Large vacuoles seen in astrocytic and neuronal processes were surrounded by one or more concentric membranes. Dense-core vesicles were found in axonal endings and in nerve cell perikarya; these particles could represent catecholamine-containing vesicles.     

Cefepime-induced encephalopathy: clinical and electroencephalographic features in seven patients

Cefepime, a fourth-generation cephalosporin, with large antibacterial spectrum, is a commonly used antibiotic for the treatment of serious hospital infections. Its security report is considered favourable. Recently, many cases of a severe and reversible cefepime-induced encephalopathy were described. In this paper, we report seven patients with reversible cefepime-induced encephalopathy, with a peculiar EEG pattern, characterized by semiperiodic diffuse triphasic waves. We discuss the EEG abnormalities found and their association with nonconvulsive status epilepticus.     

Benign acute childhood myositis: laboratory and clinical features

BACKGROUND: Benign acute myositis of childhood is a disorder of midchildhood, typically affecting boys. Symptoms include calf pain and difficulty walking after a viral illness. There is an epidemiologic association with influenza. OBJECTIVES: To describe the clinical and laboratory features of benign acute myositis. RESULTS: Thirty-eight children (32 boys, 6 girls) were seen with 41 episodes of myositis between 1978 and 1997. Two were siblings and three had recurrent episodes. Mean age at onset of symptoms was 8.1 years. Children remained ambulant during 33 of 41 episodes. Two characteristic gaits were noted: toe-walking in 13, with a wide-based stiff-legged gait in another 7. Muscle tenderness was isolated to the gastrocnemius-soleus muscles in 82% of episodes. Recovery occurred within 1 week. Creatine kinase levels were elevated during all episodes. Viral studies were positive in 10 of 24 episodes, 5 because of influenza B. CONCLUSION: Benign acute myositis is a syndrome of midchildhood that can be differentiated from more serious causes of walking difficulty by the presence of calf tenderness, normal power, intact tendon reflexes, and elevated creatine kinase. The gait patterns noted may minimize power generation of the calf muscles by splinting the ankles. Onset in childhood may reflect an age-related response to viral infection, and occurrence primarily in boys may reflect a genetic predisposition or an as-yet unknown metabolic defect.