Life-threatening respiratory failure due to cranial dystonia after dental procedure in a patient with multiple system atrophy.

We report on a woman with a an 8-year history of multiple system atrophy with predominance of parkinsonism who developed jaw-locking oromandibular dystonia within hours after insertion of ill-fitting dentures. Dystonia spread rapidly to involve other facial muscles and the larynx causing stridor with respiratory failure necessitating crush intubation.     

Prevalence of autoimmune thyroid disorders in a Spanish multiple sclerosis cohort

The aim of the study was to determine the prevalence of thyroid autoimmune disorders in a cohort of untreated multiple sclerosis (MS) patients and compare it with a stratified sample of an adult population. We prospectively studied 93 untreated MS patients. The control group included 401 healthy subjects selected by stratified sampling in a non-iodine-deficient area. Antithyroid antibodies (ATA) (antibodies against peroxidase and thyroglobulin) were considered positive at titres ≥149 IU/ml. Antibodies were positive in 11 MS patients (11.8%; 95% CI 5.3–18.4%). This prevalence was five times higher ( P  = 0.0001) when compared with that in the control population. We found six cases with subclinical hypothyroidism (6.45%; 95% CI 11.4–1.5) in contrast to 2.24% in the control group. Comparing MS with positive and negative ATA, there was a non-significant, slightly higher frequency of low Expanded Disability Status Scale (EDSS) score in the ATA-positive group (81% vs. 73.2%). One year after start of interferon (IFN) treatment, only one patient developed subclinical thyroid dysfunction. MS patients have a higher prevalence of ATA compared with the general population. An initial ATA and thyroid-stimulating hormone (TSH) determination is recommended in all MS patients. A periodic assessment of thyroid function during IFN treatment only seems to be justified in those cases where positive ATA or dysfunction is present before treatment.     

反应停治疗难治性多发性骨髓瘤6例

目的：探讨反应停治疗难治性多发性骨髓瘤(MM)的临床疗效。方法：对6例难治疗性多发性骨髓瘤应用反应停治疗后的临床资料进行分析，并结合文献复习，为该病的治疗提供一条思路。结果：1例患者达完全缓解；1例患者达部分缓解；1例进步，血清M蛋白减少35％、骨痛明显减轻、血沉降低40％、血红蛋白上升20g／L；2例无效；1例死亡。结论：反应停对多发性骨髓瘤有较好的疗效，不良反应可以耐受且不产生骨髓抑制：抗血管新生是其主要疗效机制。     

Phase II Trial of Fludarabine Phosphate in Multiple Myeloma Using a Loading Dose and Continuous Infusion Schedule

A phase II trial of fludarabine phosphate using a bolus and continuous infusion regimen in previously treated multiple myeloma was performed. No responses were observed in eleven patients. There was no significant non-hematologic toxicity noted. Fludarabine phosphate is inactive in multiple myeloma using this schedule.     

基于64层CT对心脏体模冠脉狭窄成像的客观评估方法

本文设计了一种心脏冠脉狭窄程度的客观评估方法，并自行开发了工具软件对图像数据进行自动处理和计算。此后，本文采用该方法对四组心脏动态体模冠脉狭窄成像的数据进行了分析，对该客观评估方法各方面的特性进行了检验和评定。     

Nucleolus-associated J chains in myeloma cells: clinical significance

Bone marrow aspirates from 20 patients with multiple myeloma (MM), 4 with smoldering multiple myeloma (S-MM), 1 with idiopathic Bence Jones proteinuria (I-BJP), and 6 with primary macroglobulinemia (PMG) were examined for nucleolus-associated J chain. The incidence of nucleolar J chain-positive (J+) cells among nucleolated cells producing M-component was measured. This incidence (94.0-100%) in terminal MM was significantly higher than that (0-58.0%) in non-terminal MM. Judging from a low incidence in the remission phase, chemotherapy might cause a selective elimination of less differentiated myeloma cells with J+ nucleoli and might have some effect on J chain synthesis. The incidence of nucleolar J+ cells was very low in S-MM. The IgM cells in PMG, where J chain is present in a disulfide-linked form, had no or few J+ nucleoli. No correlation between the incidence of nucleolar J+ cells among nucleolated plasma cells and the percentage of nucleolated cells or that of J+ cells was found. Large J+ nucleoli seemed to be another morphological feature indicating anaplastic myeloma cells. A high incidence of nucleolar J+ cells may be one of the indicators for progressive disease.     

Free amino acid pattern of cerebrospinal fluid in meningeal pathology

Free amino acid concentrations of CSF were measured in bacterial meningitis, aseptic meningitis, meningoradiculitis Garin-Bujadoux-Bannwarth, multiple sclerosis, carcinomatous meningitis, and controls. Almost all CSF amino acids were highly elevated in bacterial but not in aseptic meningitis, meningoradiculitis Garin-Bujadoux-Bannwarth or carcinomatous meningitis thus providing a laboratory tool for their differential diagnosis. In carcinomatous meningitis the amino acid pattern indicates metabolic activity of tumor cells. Minimal alterations were found in multiple sclerosis which have no diagnostic value.     

Delineating the sites and progression of in vivo atrophy in multiple system atrophy using fluid-registered MRI.

We describe the pattern and progression of atrophy delineated using fluid registration of serial magnetic resonance imaging scans in a case of multiple system atrophy (MSA). The in vivo findings were consistent with those found at postmortem, including significant supratentorial atrophy concurrent with an unusual degree of cognitive impairment for MSA.