Predictive value of clinical characteristics for 'benign' multiple sclerosis

We studied a cohort of 496 patients who had multiple sclerosis (MS) for at least 10 years. Ten years after disease onset, 151 had benign MS defined as an Extended Disability Status Scale (EDSS) ≤3. Between benign and non-benign patients we compared gender, age at clinical onset, relapsing–remitting or primary progressive, symptoms at onset, recovery from first relapse, time between first and second relapse, number of relapses in the first 5 years, use of immunomodulatory drugs, and EDSS scores at 2, 5 and 10 years. A multivariate regression analysis showed that a relapsing–remitting course, a low EDSS score at 5 years, and a low number of relapses in the first 5 years were predictive for benign MS at 10 years. Other factors had no additional value. Thirty-five of the 51 patients (69%) with benign MS at 10 years were still benign at 20 years. A low 10-year EDSS score was the only clinical variable associated with a benign course at 20 years. Our results suggest that within the first 5 years from onset it is not possible to predict a benign course. Disease course, EDSS score and relapse rate at 5 years are predictors for benign MS at 10 years.     

Prefrontal cortex dysfunction and depression in atypical parkinsonian syndromes.

Depressive symptoms are common in patients with neurodegenerative disorders. Imaging studies suggest that a disruption of frontal-subcortical pathways may underlie depression associated with basal ganglia disease. This pilot study tested the hypothesis that frontal dysfunction contributes to depression associated with multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). Depressed patients with MSA (n = 11), PSP (n = 9), and age-matched controls (n = 25) underwent measures of cerebral glucose metabolism applying positron emission tomography with (18)F-fluorodeoxyglucose. Regional metabolism in the patient groups was compared to the normal subjects using the voxel-based statistical parametric mapping. Depressive symptom severity (Hamilton Depression Rating) and degree of locomotor disability (Hoehn & Yahr) were assessed in the patient groups. The association between prefrontal metabolism and the occurrence of depressive symptoms and the degree of locomotor disability was investigated. When compared to controls, MSA patients revealed significant metabolic decreases in bilateral frontal, parietal, and cerebellar cortex and in the left putamen. In PSP patients, significant hypometabolism was demonstrated in bilateral frontal cortex, right thalamus, and midbrain. Depression severity but not the patients' functional condition was significantly associated with dorsolateral prefrontal glucose metabolism in both patient groups. The findings of this pilot study support the hypothesis that depressive symptoms in MSA and PSP are associated with prefrontal dysfunction.     

65例多发性硬化患者的临床分析

目的总结多发性硬化(MS)患者的临床特点(精神情感障碍)以及脑脊液、诱发电位、影像学改变进行分析。方法回顾分析65例MS患者的有关临床和实验室资料。结果65例中,男17例,女48例;年龄16~68岁,平均(36.5±14.1)岁,男女比为1∶2.8。MS首发及常见症状为感觉异常、肢体无力、视力减退及括约肌功能障碍,病变部位以脊髓受累最多见,部分患者存在精神情感障碍。结论出现感觉异常有助于MS早期诊断,诱发电位及MRI有助于发现亚临床病变;括约肌功能异常多见,可能与脊髓受累较多有关;MS患者的精神情感应受人们关注。     

58例多原发癌临床分析

Objective to Present 56 cases of multiple primary cancers,to make improvement of diagnosis and treatment.methods A total of 58 patients with multiple primary cancers admitted from 2003 to 2007 were analysed retrospectively in Beijing Tongren Hospital.Results Thirty-five cases were males,with sex ratio of male and female 1.5:1.The median age at the onset of the first disease was 61.5 years (ranged from 27 to 86).The onset age of the two primary cancers was mainly centered around 50～75 years,while half of the second cancers occurred within five years.Conclusion the treatment and prognosis of the two primary cancers are different from that of the recurred and metastatic malignancies and shoule be handled with care.     

伴有周围神经病变的多发性硬化

目的分析5例伴周围神经病变的多发性硬化的临床电生理、实验室检查和MRI改变,探讨多发性硬化合并周围神经损害的临床特点和发病机制。方法回顾性总结5例伴发周围神经病损的多发性硬化的脑脊液生化,免疫学检查、寡克隆带,电生理检查,MRI及其它有助于鉴别诊断的检查。结果5例均为伴有周围神经病变的多发性硬化,电生理提示广泛周围神经病损,既有脱髓鞘改变,又有轴突变性。结论由于中枢神经系统与周围神经系统髓鞘存在着共同的抗原性成分,故MS患者的中枢神经系统与周围神经系统可以同时发生脱髓鞘改变。     

The importance of self-examination in the earliest diagnosis of multiple primary cutaneous melanomas: a report of 47 cases

BACKGROUND: Development of more than one primary melanoma in a patient is a relatively uncommon but well-recognized phenomenon. Its frequency has ranged from 1.2% to 8.2% in several series. This subgroup of patients with multiple primary lesions has not been characterized sufficiently. We report the experience of the Melanoma Unit of University Hospital Spedali Civili of Brescia, Italy. METHOD: Study subjects were drawn from 1240 patients with histologically confirmed melanoma, including melanoma in situ. From this group, multiple melanomas developed in 47 patients (3.79%). Every one of our patients has been taught to perform self-examination of the skin to detect suspicious pigmented lesions. RESULTS: Of the 47 patients described in this study, 38 had two primary melanomas, 7 had three melanomas and 2 had 5 and 10 melanomas, respectively. Mean age at first diagnosis was 46.2 years. The majority of subsequent melanomas (74.5%) were removed within 5 years of the initial operation. Synchronous lesions were found in 10 patients. In male patients, the lesion appeared most frequently on the trunk; in female patients, melanoma appeared mostly on the lower extremities. The second primary melanomas developed in the same anatomic region from the first in 53.2% of our patients. The proportion of in situ to invasive melanomas was greater for the second melanomas compared with the first melanomas. Regarding invasive melanomas, the mean thickness of the first melanomas was 1.31 mm compared with 0.66 mm for the second ones. Dividing patients into two groups, of more and less than 50, it is highlighted that in older patients synchronous lesions appear more frequently (36.4% vs. 8.0%); the median time interval between sequential melanomas is longer (84 vs. 63.7 months); and the ratio between the primary and secondary melanoma mean thickness is lower (1.21 : 1.08 vs. 1.43 : 0.63 mm). CONCLUSIONS: The study confirms that second primary melanoma is usually thinner than the first lesion, and it is more common in the same region of the body as the initial melanoma. The highest risk for a second melanoma is during the first 5 years, but a much longer time interval of 28 years is possible. Continued medical follow-up with complete skin examinations seems prudent, but it is very important to promote self-skin evaluation in patients to detect not only metastases but also subsequent primary melanomas in their earliest phases.     

Successful triplet pregnancy in a patient with a unicornuate uterus with a cavitary communicating rudimentary horn

In a patient with primary infertility, ovulation was inducedby monitored stimulation with human menopausal gonadotrophins(HMG) because of polycystic ovarian disease. Infertility work-uphad shown a unicornuate uterus with a cavitary communicatingrudimentary horn. The husband showed a varicocele-related moderateoligoasthenoterato-zoospermia. A triplet pregnancy occurredin a third HMG ovulation induction cycle combined with intra-uterineinsemination of the husband's washed semen. The pregnancy wascarefully monitored, and measures to prevent premature deliverywere taken. Because of the patient's obvious discomfort in thepresence of premature labour, Caesarean section was performedat 33 weeks gestation and three healthy infants were delivered.This is the first report of a successful triplet pregnancy ina women with a unicornuate uterus. The reproductive and obstetricoutcome of this condition in general, and in the case of multiplepregnancy, is discussed.     

Bacteroides fragilis Pyomyositis in a Patient with Multiple Myeloma

Pyomyositis is a bacterial infection with abscess formation affecting large skeletal muscles. It is predominantly caused by Staphylococcus aureus. The disease is common in tropical areas, but rare in temperate climates. We report a patient with multiple myeloma who developed a giant elastic tumor on the right thigh and a hen egg-sized tumor on the right upper arm. MR imaging revealed cystic spaces in the femoral quadriceps and brachial biceps muscles. A large amount of pus with foul smell was removed by incision, drainage and aspiration of the two tumors. The lesions were successfully treated with intravenous administration of antibiotics. Repeated bacterial cultures yielded only Bacteroids fragilis. To our knowledge, this is the first report of pyomyositis due to Bacteroides fragilis.     

Cardiac MIBG scintigraphy is a sensitive tool for detecting cardiac sympathetic denervation in Parkinson's disease.

[(123)I]Metaiodobenzylguanidine ([(123)I]MIBG) cardiac scintigraphy could be helpful to differentiate Parkinson's disease (PD) from multiple system atrophy (MSA), demonstrating that, in PD with autonomic failure but not in MSA, there is a myocardial postganglionic sympathetic dysfunction. To investigate whether this method is more sensitive than standard autonomic testing to detect early involvement of sympathetic cardiac efferent, we analyse MIBG myocardial uptake in 8 PD patients with normal autonomic testing (nondysautonomia PD group, NDPD) in comparison with 10 PD patients with abnormal autonomic testing (dysautonomia PD group, DPD) and 10 MSA patients. Global MIBG uptake was assessed using the ratio of [(123)I]MIBG uptake in the heart to the upper mediastinum (H/M) on planar scintigraphic data. Regional MIBG uptake was determined on two single photon emission tomography scans in regions of the left ventricle. The mean H/M ratios were significantly different among the three groups (P < 0.0001). H/M ratios of both NDPD and DPD patients groups (H/M = 1.83 +/- 0.50 and 1.24 +/- 0.40, respectively) were significantly lower than in MSA patients (H/M = 2.52 +/- 0.60). However, in NDPD patients, H/M was significantly higher than in DPD patients. When compared to MSA patients, NDPD patients showed a regional reduction in MIBG uptake in all left ventricle regions markedly in the apex and the inferior wall. Our results suggest that MIBG myocardial scintigraphy (analysis of both H/M ratio and regional MIBG uptake) may be more sensitive than standard autonomic testing for the early detection of silent autonomic dysfunction in PD.     

Leukemia,lymphoma, and multiple myeloma following selected medical conditions

The role of selected prior medical conditions in the etiology of hematopoietic malignancies was examined in a case-control study of members of two regional branches of the Kaiser Permanente Medical Care Program (USA). Past history of chronic infectious, autoimmune, allergic, and musculoskeletal disorders was abstracted from medical records for leukemia (n = 299), non-Hodgkin's lymphoma (NHL, n = 100), and multiple myeloma (n = 175) cases and matched controls (n = 787). Little difference was found between cases and controls for most of the chronic conditions evaluated, including sinusitis, carbuncles, urinary tract infections, pelvic infections, herpes zoster, asthma, rheumatoid arthritis, psoriasis, bursitis, and gout. Only three statistically significant elevated risks were found, i.e., with combined disc disease myeloma among patients with prior eczema and disk and other musculoskeletal conditions, and NHL following tuberculosis. Only two of these associations showed consistent patterns by sex and geographic region (myeloma with eczema and with musculoskeletal conditions). While prior history of eczema and musculoskeletal conditions may slightly increase risk of myeloma, this study provided little if any support for an association of chronic infectious, autoimmune, allergic, and musculoskeletal conditions with subsequent occurrence of the leukemias or NHL. Additionally, these data did not support a role for chronic antigenic stimulation, as defined in previous epidemiologic studies, in the etiology of hematopoietic malignancies.Ms Doody and Drs Linet, Pottern, Boice, and Fraumeni are with the Epidemiology and Biostatistics Program, National Cancer Institute. Dr Glass is with the Kaiser Permanente Medical Care Program, Northwest Region, Portland, Oregon, USA. Dr Friedman is with the Kaiser Permanente Medical Care Program, Northern California Region, Oakland, California, USA. Address correspondence to Ms Doody, Radiation Epidemiology Branch, National Cancer Institute, Executive Plaza North, Room 408, Bethesda, MD 20892, USA. This research was supported in part by National Cancer Institute contracts NO1-CP-01047, NO1-CP-01054, NO1-CP-11009, NO1-CP-11037, NO1-CP-31035, and NO1-CP-61006.