Neurosarcoidosis

Neurosarcoidosis is an uncommon disorder and requires a careful clinical evaluation to reach a diagnosis. Generally, patients with peripheral symptoms, which include paresthesias, muscle weakness, and stocking glove deficits, have a better outcome compared with those with central nervous system involvement. Patients with mass lesions or hydrocephalus tend to have more relapses and are often more resistant to routine therapy. Neurosarcoidosis often responds to glucocorticoids, usually within days or weeks of initiating therapy. Patients are usually maintained on 40 to 80 mg per day for 4 to 6 weeks, which is then tapered slowly. Alternative treatments for refractory neurosarcoidosis, or to reduce or eliminate steroids, include methotrexate, cyclophosphamide, azathioprine, cyclosporine, infliximab, chlorambucil, chloroquine, and hydroxychloroquine.     

Soft tissue sarcomas of the foot and ankle: impact of unplanned excision, limb salvage, and multimodality therapy

BACKGROUND: Foot and ankle sarcomas have historically been treated with amputation because of the difficulty in achieving local disease control and maintaining a functional foot. Potential opportunities for limb salvage may be further compromised by unplanned excisions. MATERIALS AND METHODS: We reviewed 52 consecutive patients with soft tissue sarcomas of the foot and ankle and analyzed the impact of planned versus unplanned initial excision, limb salvage, and multimodality therapy on treatment and outcomes. RESULTS: Unplanned excisions had been performed in 29 (55.8%) patients. Limb salvage was performed in 38 patients, with 14 requiring free soft tissue transfers. At an average followup of 99 (range, 24 to 216) months, the 5-year overall survival estimate was 76.3%. Although not statistically significant, we noted clinically relevant potential differences in local recurrence-free, disease-free, and oncologic survival between the planned and unplanned excision groups. Seven patients (13.5%) had a local recurrence, five of these following an unplanned excision. Functional scores averaged 83.2% for all patients which were similar between planned versus unplanned and amputation versus limb salvage groups. Significantly more patients with unplanned excisions required free flaps for limb salvage (p = 0.017) and received adjuvant radiotherapy (p = 0.0004). CONCLUSION: Unplanned surgery for soft tissue sarcomas of the foot and ankle often results in the need for more aggressive surgery and/or adjuvant radiotherapy and may impact oncologic outcomes, but does not necessarily portend worse functional outcomes. Multimodal therapy and judicious use of soft tissue flap reconstruction allows limb salvage in most patients with favorable outcomes.     

The use of computed tomography to assess acetabular morphology in Morquio-Brailsford syndrome

PURPOSE: Morquio-Brailsford syndrome (MS) is an autosomal recessive lysosomal storage disorder, a mucopolysaccharidosis, characterized by abnormal metabolism of glycosaminoglycans. Major treatable concerns in patients with MS involve C1 to C2 instability, genu valgum, and hip subluxation. Untreated hip subluxation has been shown to predispose to early onset of arthritis of the hip. Early appropriate pelvic osteotomies may restore (improve) load transmission and retard the onset of arthritis. Computed tomographic (CT) measurements can help determine the site and severity of acetabular deficiency, aiding in selection of the appropriate acetabular procedure. Acetabular morphology in MS has not been described in the literature. The purpose of this study was to evaluate morphology (shape) of the acetabulum in MS using two-dimensional (2-D) CT scans. METHODS: To assess the acetabular roof, the acetabular index was measured on anteroposterior radiographs of the pelvis. Various CT measures were used to assess the acetabular anatomy in the axial plane. RESULTS: The average acetabular index on the anteroposterior radiographs of the pelvis was 33 degrees (average age-matched difference from normal, 12 degrees). Two-dimensional CT (axial cuts) showed that the average acetabular anteversion angle was close to normal, measuring 10.9 degrees. The average anterior acetabular index was 58.8 degrees (average age-matched difference from normal, 10.6 degrees), and posterior acetabular index was 53.8 degrees (average age-matched difference from normal, 3.8 degrees). Calculated axial acetabular index ranged from 90 to 133 degrees (mean, 112.6 degrees; average difference from normal, 14.5 degrees). CONCLUSIONS: Two-dimensional CT of the hip in patients with MS demonstrated a severe dysplasia of the anterior acetabular wall and the roof of the acetabulum, although the acetabular version was normal. Treatment of hip dysplasia in MS should focus on increasing the overall depth of the acetabulum to better contain the femoral head. Two-dimensional CT is recommended before bony acetabular procedures to assess the degree of acetabular deficiencies. SIGNIFICANCE: Computed tomography of the acetabulum is helpful in preoperative decision making and planning before an acetabular procedure in patients with Morquio-Brailsford syndrome.     

分娩期连续电子胎心监护用于胎儿评估

1背景 在美国，每4例孕妇中有3例在产群中及分娩时使用电子胎儿监护（EFM）（NCHS1993）。1989年，美国妇产科学院（ACOG1989）发表了一项声明，对于低危妊娠既可使用EFM，也可使用间断性胎心听诊；但是，美国预防保健委员会（USPSTF1989）和加拿大的定期健康检查委员会（CTFPHE1994）对高危妊娠仍保留使用EFM。尽管有人对EFM的效果和安全性表示担忧（Thacker 1987：Thacker 1995），     

Recreational physical activity and risk of Parkinson's disease

The purpose of this study was to investigate associations between recreational physical activity and Parkinson's disease (PD) risk. We prospectively followed 143,325 participants in the Cancer Prevention Study II Nutrition Cohort from 1992 to 2001 (mean age at baseline = 63). Recreational physical activity was estimated at baseline from the reported number of hours per week on average spent performing light intensity activities (walking, dancing) and moderate to vigorous intensity activities (jogging/running, lap swimming, tennis/racquetball, bicycling/stationary bike, aerobics/calisthenics). Incident cases of PD (n = 413) were confirmed by treating physicians and medical record review. Relative risks (RR) were estimated using proportional hazards models, adjusting for age, gender, smoking, and other risk factors. Risk of PD declined in the highest categories of baseline recreational activity. The RR comparing the highest category of total recreational activity (men ≥ 23 metabolic equivalent task‐hours/week [MET‐h/wk], women ≥ 18.5 MET‐h/wk) to no activity was 0.8 (95% CI: 0.6, 1.2; P trend = 0.07). When light activity and moderate to vigorous activity were examined separately, only the latter was found to be associated with PD risk. The RR comparing the highest category of moderate to vigorous activity (men ≥ 16 MET‐h/wk, women ≥ 11.5 MET‐h/wk) to the lowest (0 MET‐h/wk) was 0.6 (95% CI: 0.4, 1.0; P trend = 0.02). These results did not differ significantly by gender. The results were similar when we excluded cases with symptom onset in the first 4 years of follow‐up. Our results may be explained either by a reduction in PD risk through moderate to vigorous activity, or by decreased baseline recreational activity due to preclinical PD. © 2007 Movement Disorder Society     

The partial positive allosteric GABAA receptor modulator EVT 201 is efficacious and safe in the treatment of adult primary insomnia patients

Objective: To evaluate polysomnographic (PSG) and self-reported measures of the efficacy and safety of EVT 201 in patients with primary insomnia.Patients and methods: Following clinical and PSG screening, 75 patients (mean age: 45.1 ± 11.2y; 50f, 25m) meeting DSM-IV criteria for primary insomnia entered this crossover study and were randomly assigned to double-blind treatment sequences of 1.5 mg or 2.5 mg EVT 201, or placebo using a balanced Latin square design. For each study condition study medication was administered on two consecutive nights and PSG and self-reported data were collected. Safety assessments included physical examination, clinical laboratory measures, electrocardiogram, documentation of adverse events, and the digit symbol substitution test (DSST) and self-reported sleepiness/alertness ratings to detect residual sedation. Data were collected at five US sleep laboratories. Efficacy analyses were performed for the 67 patients completing the study. Safety analyses included all 75 randomized patients.Results: On PSG measures compared to placebo, EVT 201 1.5 mg and 2.5 mg increased total sleep time (TST; 33.1, 45.0 min; both p < 0.0001), reduced wake after sleep onset (WASO; −16.7, −25.7 min; both p < 0.0001), reduced latency to persistent sleep (LPS; −17.0, −20.7 min; both p < 0.0001), and reduced the number of awakenings (−1.2, −2.6; both p < 0.0001). Significant reduction of wake time was seen with 1.5 mg during each of the first three quarters of the night (p < 0.0001–0.002), and with 2.5 mg in all four quarters (p < 0.0001–0.0005). Both doses also improved all key self-reported measures of sleep including total sleep time (rTST; 51.9, 51.1 min; both p < 0.0001), wake after sleep onset (rWASO; −29.3, −29.6 min; both p < 0.0001), sleep latency (rSL; −24.0 min, p < 0.004; −25.1 min, p < 0.0002), and number of awakenings (rNAW; −1.1, −1.2; both p < 0.0001). Sleep quality was also improved by both doses. Self-rated sleepiness in the morning did not differ from placebo for either dose; however, there was a small negative effect on the DSST for both doses. Both doses had similar effects on sleep architecture including an increase in Stage 2 sleep and REM latency and a small, but significant decrease in REM (REM −5.7, −8.3 min; p = 0.0175, p = 0.0006). No effect on other sleep architecture parameters, including SWS, was seen. EVT 201 was well tolerated. No serious or unexpected adverse events were reported.Conclusion: This first study of EVT 201 in adult patients with primary insomnia demonstrated improved measures of sleep onset and sleep maintenance, including during the third and fourth quarters of the night. Adverse events were infrequent and all were mild to moderate in severity.     

Hemostatic technique for internal mammary artery anastomotic bleeding

When internal mammary artery is used for myocardial revascularization, a not uncommon occurrence is intraoperative bleeding from the internal mammary artery to coronary artery anastomosis. The conventional method of hemostasis of placing additional sutures across the suture line may produce anastomotic stenosis or may aggravate the bleeding by producing tears, especially as these additional sutures are placed on a beating heart. We describe a simple technique by which hemostasis can be achieved without the risk of anastomotic stenosis or aggravation of the bleeding, as it avoids placing sutures over the anastomotic suture line.     

Muscle dysfunction in male hypogonadism

Twenty-eight consecutive male patients with primary and secondary hypogonadism (14 each) were evaluated clinically and electrophysiologically for muscle dysfunction. Although generalised muscle weakness was initially reported by only 9 patients, on direct questioning, it was recorded in 19. Objective weakness was found in 13 patients and it involved both the proximal and distal limb muscles. Quantitative electromyography showed evidence of myopathy in the proximal muscle in 25 patients, i.e., reduced MUP duration and amplitude with increased polyphasia in the deltoid and the gluteus maximus. There were no denervation potentials. None of the patients showed clinical neuropathy or NCV abnormalities. Thus, the profile of muscle involvement in hypogonadism closely simulates limb-girdle muscular dystrophy and other endocrine myopathies. The incidence of muscle involvement was higher in secondary hypogonadism. Diminished androgens in primary hypogonadism and diminished growth hormone in the secondary hypogonadism are probably responsible for the myopathy.