Optic neuropathy in Strumpell-Lorrain disease: presentation of a clinical case and literature review

This report describes a 36-year-old female with bilateral optic atrophy. Physical examination was normal except for subclinical signs of lower limbs spasticity. Her father was affected of Strumpell-Lorrain disease. The clinical characteristics of Hereditary Spastic Paraplegia or Strumpell-Lorrain disease are: progressive spasticity of the lower limbs and pathological reflexes. Pathologically, this disease is characterized by a degeneration or demyelinization of the cortico-spinal system and, to a lesser extent, of the posterior cord and the spino-cerebellar system. Optic neuropathy and dyschromatopsy have already been reported in a small group of patients. Visual acuity ranged from 20/20 to 20/200. Visual field alterations consisted of superior bitemporal amputation. Fundus examination showed optic atrophy. The incidence of optic neuropathy in Strumpell-Lorrain disease is quite high but its presence is often overlooked. Most patients are asymptomatic, but signs of optic nerve dysfunction are evident at clinical examination. According to some authors, there is an inverse relationship between the impairment of walking and the visual dysfunction. This was also noted in our case.     

Ultrasonographic nomograms of the fetal fourth ventricle: additional tool for detecting abnormalities of the posterior fossa.

OBJECTIVE: To characterize normal growth of the fetal fourth ventricle on ultrasonography throughout pregnancy. METHODS: Consecutive biometric measurements and fetal organ scans were obtained from 299 patients undergoing fetal anatomic surveys between 13 and 40 weeks' gestation. Using 7- and 3.5-MHz transducers for early (13- to 17-week) and late (>17-week) examinations, respectively, we scanned the fetal head in the axial plane with special focus on the posterior fossa of the brain. The fourth ventricle was identified, and its anteroposterior diameter and width were measured. A "triangle" formula was used for calculating its circumference and area. RESULTS: The fourth ventricle was shown as a hypoechoic triangle below the level of the cerebellum. A linear regression line of the fourth ventricle was observed across gestational age, and a first-degree correlation was found between gestational age and anteroposterior diameter of the fourth ventricle (r = 0.894; P < .0001; y = -0.84 + 0.23 x gestational age), its width (r = 0.657; P < .0001; y = 3.82 + 0.14 x gestational age), its circumference (r = 0.843; P < .0001; y = 5.11 + 0.58 x gestational age), and its area (r = 0.844; P < .0001; y = -10.11 + 1.17 x gestational age). Twelve enlarged fourth ventricles were found between 14 and 16 weeks, but results of follow-up scans at 20 weeks were normal. CONCLUSIONS: An isolated enlarged fourth ventricle in the early second trimester might represent a normal variant; it should be followed, but decisions about the fate of the pregnancy should not be based solely on this finding.     

Understanding youth: using qualitative methods to verify quantitative community indicators

Community- and individual-level data were collected from interviews with 1,294 boys and girls, 13 to 19 years old, in three impoverished urban communities of Beirut. Univariate analyses of variables provide quantitative indicators of adolescents' lives and communities. Researchers including the authors, interested in using these indicators to plan for community interventions with youth in the Palestinian refugee camp, discuss the pertinent results with youth from the camp in six focus groups. The authors find that many indicators misrepresent the situation of youth in the camp. For example, adolescents may have underreported cigarette and argileh (water pipe) smoking (8.3% and 22.4%, respectively) because of the lack of social desirability of these behaviors; other questions may have been misunderstood, such as perceived health and health compared to others. Also, important issues for them such as drug abuse, violence, and school problems were not asked. Implications for intervention research are discussed.     

Delays in oral cavity cancer

While the prognosis for early-stage oral cavity cancer is relatively good; the majority of patients are still diagnosed with advanced-stage disease on presentation with an associated poorer prognosis. The aims of this review are to summarize our current understanding of delays in oral cavity cancer and their impact on stage at diagnosis and survival. The delays pathway can be subdivided into three components: patient, professional, and treatment delays. Patient delay represents the longest interval in the delays pathway usually lasting between 2 and 5 months and being most influenced by cognitive and psychosocial factors. Professional and treatment delays are shorter in most studies, but highly variable depending on the respective healthcare system. Most studies indicate that advanced stage at diagnosis, primary treatment with radiotherapy, treatment at an academic center, and transitions in care are associated with an increased treatment delay. Based on our current understanding, a delay between definitive diagnosis and treatment of 4–6 weeks seems acceptable from an oncologic perspective. Further studies are needed to better define what a ‘safe’ waiting time is and to understand the psychological impact of delays for patients.     

Procalcitonin and interleukin 6 for predicting blood culture positivity in sepsis

Background

Procalcitonin and interleukin 6 (IL-6) are well-known predictors of blood culture positivity in patients with sepsis. However, the association of procalcitonin and IL-6 with blood culture positivity was assessed separately in previous studies. This study aims to examine and compare the performance of procalcitonin and IL-6, measured concomitantly, in predicting blood culture positivity in patients with sepsis.

Methods

Forty adult patients with sepsis were enrolled in the study. Blood cultures were drawn before the institution of antibiotic therapy. The area under the curve (AUC) of the receiver operating characteristic curve was estimated to assess the performance of procalcitonin and IL-6 in predicting blood culture positivity.

Results

Positive blood cultures were detected in 10 patients (25%). The AUC of procalcitonin and IL-6 was 0.85 and 0.61, respectively. The combined performance of procalcitonin and IL-6 was similar to that of procalcitonin alone, AUC of 0.85. On univariate analysis, only procalcitonin and IL-6 were associated with blood culture positivity. Multivariate logistic regression analysis showed that only procalcitonin was associated with blood culture positivity (odds ratio, 12.15 [1.29-114.0] for levels above the median compared with levels below the median). Using procalcitonin cut points of 1.35 and 2.14 (nanogram per milliliter) enabled 100% and 90% identification of positive blood cultures and reduced the need of blood cultures by 47.5% and 57.5%, respectively.

Conclusions

Compared with IL-6, procalcitonin better predicts blood culture positivity in patients with sepsis. Using a predefined procalcitonin cut points will predict most positive blood cultures and reduce the need of blood cultures in almost half of patients with sepsis.     

Association of polymorphonuclear leukocytes with sites of aortic catheter-induced injury in rabbits

The kinetics of the association of polymorphonuclear leukocytes (PMNs) with arterial balloon catheter-induced injury have been examined. An average of 6 X 10(7) PMNs were isolated from 20 ml of blood and labelled with 111In-oxine for reinfusion into the donor rabbit. The cells remained viable as demonstrated by both in vitro and in vivo tests of cell function. The abdominal aorta of rabbits was denuded of endothelium and immediately, 24 h, or 5 weeks later, exposed to autologous radiolabelled PMNs for 1 h. The presence of PMNs at sites of denudation was demonstrated by detection of the radioactive label and was confirmed by light and electron microscopy after 24 h, but not at 5 weeks. Immediately following denudation radioactivity was 2.44 +/- 0.33 times control (P = 0.006); 2.52 +/- 0.18 at 24 h (P = 0.005); and 1.88 +/- 0.32 times control at 5 weeks (P = 0.045). The presence of PMNs, or their products, 5 weeks after denudation suggests a more complex role of PMNs and possibly a direct involvement in the long term changes resulting from arterial balloon catheter injury.