Infantile epileptic encephalopathy with late‐onset spasms: Report of 19 patients

Purpose: Late‐onset spasms (LOS) are epileptic spasms starting after the first year of life. Our aim was to assess the electroclinical features and the follow‐up of the patients with this particular type of epileptic seizure. Methods: We retrospectively included all patients with LOS confirmed by electroencephalography between 1989 and 2008. Clinical and electroencephalographic findings at diagnosis and during follow‐up were collected. The Vineland scale was used to evaluate the neuropsychological outcome. Results: We report 19 patients with LOS of 240 patients with recorded epileptic spasms. Eighteen patients had an epileptic encephalopathy with late‐onset spasms. The ictal electroencephalography (EEG) showed a focal or generalized discharge of triphasic slow‐waves, slow‐spikes, or slow spikes‐waves with fast activities. The interictal EEG usually showed focal or generalized slow‐waves or slow spikes‐waves without hypsarhythmia. LOS were controlled in only six patients. Three developed typical Lennox‐Gastaut syndrome and 10 had a severe epileptic encephalopathy. Neuropsychological outcome was evaluated in 15 patients with the Vineland scale. Cognitive functions were normal in only one patient, whereas severe cognitive delay was observed in 12 of 15. Conclusion: Epileptic spasms may appear after the age of one. They are more frequently observed in patients with epileptic encephalopathy. In few patients this type of seizure was observed before the patients fulfill Lennox‐Gastaut syndrome criteria. In one patient, we diagnosed a focal epilepsy with seizures occurring in cluster. When LOS are related to an epileptic encephalopathy, this epileptic syndrome seems to be linked to refractory epilepsy and severe cognitive outcome unrelated to the etiology.     

Coronary artery stenosis: direct comparison of four-section multi-detector row CT and 3D navigator MR imaging for detection--initial results

PURPOSE: To prospectively compare the diagnostic accuracy of multi-detector row computed tomography (CT) and of three-dimensional (3D) navigator magnetic resonance (MR) imaging in patients referred for conventional coronary angiography for detection of coronary artery stenosis. MATERIALS AND METHODS: All patients gave written informed consent for the study, which was approved by the local ethics committee. Twenty-seven patients underwent multi-detector row CT and 3D navigator free-breathing MR imaging a mean of 5 days before undergoing invasive coronary angiography. The acquired multi-detector row CT and MR images were graded for the presence of greater than 50% stenosis in vessels larger than 1.5 mm in diameter. The diagnostic accuracies of the two examinations were compared with that of quantitative coronary angiography (QCA) by using the McNemar test. RESULTS: Owing to claustrophobia, MR images were not acquired in one patient; thus, 26 patients were included for analysis. According to QCA findings, 21 of the 26 patients had significant coronary artery disease and 58 (20%) of a total of 294 coronary artery segments larger than 1.5 mm in diameter had significant (>50%) stenosis. Multi-detector row CT had significantly higher sensitivity (46 [79%] of 58 segments) than MR imaging (36 [62%] segments, P < .05) for detection of segments with significant stenosis. Conversely, MR imaging had significantly higher specificity (198 [84%] of 236 segments) than did CT (168 [71%] segments, P < .001) for exclusion of segmental coronary artery stenosis. Both examinations had high negative predictive value for exclusion of segmental stenosis: 93% (168 of 180 segments) for CT and 90% (198 of 220 segments) for MR imaging. The overall diagnostic accuracy of MR imaging (80% [234 of 294 segments]) was significantly higher than that of CT (73% [214 segments], P < .05). CONCLUSION: MR imaging had significantly higher diagnostic accuracy than multi-detector row CT in the evaluation of coronary artery stenosis. Both techniques have high negative predictive value, making them particularly useful for ruling out coronary artery disease in symptomatic patients.     

Usefulness of 40-slice multidetector row computed tomography to detect coronary disease in patients prior to cardiac valve surgery

Preoperative identification of significant coronary artery disease (CAD) in patients prior to valve surgery requires systematic invasive coronary angiography. The purpose of this current prospective study was to evaluate whether exclusion of CAD by multi-detector CT (MDCT) might potentially avoid systematic cardiac catheterization in these patients. Eighty-two patients (53 males, 62 ± 13 years) scheduled to undergo valve surgery underwent 40-slice MDCT before invasive quantitative coronary angiography (QCA). According to QCA, 15 patients had CAD (5 one-vessel, 6 two-vessel and 4 three-vessel disease). The remaining 67 patients had no CAD. On a per-vessel basis, MDCT correctly identified 27/29 (sensitivity 93%) vessels with and excluded 277/299 vessels (specificity 93%) without CAD. On a per-patient basis, MDCT correctly identified 14/15 patients with (sensitivity 93%) and 60/67 patients without CAD (specificity 90%). Positive and negative predictive values of MDCT were 67% and 98%. Performing invasive angiography only in patients with abnormal MDCT might have avoided QCA in 60/82 (73%). MDCT could be potentially useful in the preoperative evaluation of patients with valve disease. By selecting only those patients with coronary lesions to undergo invasive coronary angiography, it could avoid cardiac catheterization in a large number of patients without CAD. This work was supported by a grant of the Fondation Nationale de la Recherche Scientifique of the Belgian Government (FRSM 3.4557.02). Dr. Pouleur is supported by a personal grant of the Fondation Nationale de la Recherche Scientifique of the Belgian Government.     

Etiology of bacterial infections in febrile neutropenic patients: the role of the laboratory in the diagnosis

EPIDEMIOLOGICAL EVOLUTION: Until the mid-eighties, infectious complications (pneumonia, septicemia) observed in neutropenic patients were, in 70% of cases, of bacterial origin with Gram negative bacillae (Escherichia coli, Klebsiella sp, Pseudomonas aeruginosa) isolated 8 times out of 10. Among the Gram positive bacteria, Staphylococcus aureus predominated. The etiological profile of bacterial infections has since evolved with a predominance (60 to 70%) of Gram positive bacteria (coagulase-negative staphylococci, viridans streptococci) and a change in the epidemiology of the Gram positive bacteria notably with a lesser frequency of P. aeruginosa infections. THE GRAM POSITIVE BACTERIA: Coagulase-negative staphylococci are among the first germs responsible for nosocomial bacteremia (central venous catheters) and they are usually multiresistant. Viridans streptococci are a frequent cause of bacteremia; they are generally sensitive to antibiotics active on Gram positive bacteria, but the incidence of resistant strains is increasing. Enterococci are in majority responsible for colonisation in neutropenic patients and less frequently for infections; they raise the problem of resistance to antibiotics, notably to glycopeptides. Other Gram positive bacteria can be responsible for infections in neutropenic patients; it is crucial that they be identified because they require treatment with an appropriate antibiotic. GRAM NEGATIVE BACTERIA: Among the enterobacteria, Escherichia coli is predominantly isolated and raises the problem of the increasing incidence of resistance to fluoroquinolone. Pseudomonas aeruginosa, less frequently responsible today, remains associated with a far greater rate of mortality than that observed with the other microorganisms. Other Gram negative bacteria can be identified; they require an adapted antibiotherapy because they are often naturally multiresistant to antibiotics. THE ROLE OF THE LABORATORY: For the diagnosis of infections in neutropenic patients, the microbiology laboratory has a determinating role. The laboratory ensures the analysis of various biological examinations: blood cultures, methods permitting the diagnosis of an infection on a permanent catheter, copro-cultures (research for common enteropathogens, quantification in the case of digestive decontamination, screening for multiresistant bacteria), cytobacteriological examination of urine, samples of respiratory origin, cytobacteriological examination of cerebro-spinal fluid...).     

Discrimination of osteoporotic patients with quantitative ultrasound using imaging or non-imaging device

OBJECTIVES: Quantitative ultrasound (QUS) has emerged as a new tool in the assessment of fracture risk. The aim of this study was to compare the clinical utility of QUS parameters measured using imaging and non-imaging devices in the discrimination of osteoporotic patients. METHODS: QUS (Broadband Ultrasound Attenuation, BUA dB/MHz, and Speed of Sound, SOS m/s) were measured and then statistical analyses were performed. RESULTS: The 106 women included were 65 +/- 8 years aged. Using DXA, T score was < or = -2.5 at either lumbar spine or hip in 59% of patients, and 25% had osteoporotic fractures. QUS results were different among devices, and these differences were highly dependent on the measured value. There was a similar effect of age and duration of menopause for all parameters. To obtain 90% of sensitivity for the diagnosis of osteoporosis, the thresholds were 50.80 and 71.70 dB/MHz for BUA and 1544.80 and 1551.50 m/s for SOS, using imaging and non-imaging devices, respectively. Belonging to the highest tertile of QUS had a negative predictive value for osteoporosis ranging from 59% to 65%. In the lowest tertile of QUS, the proportion of osteoporotic women was between 73% and 80%. All QUS parameters, except BUA measured with the non-imaging device, were able to discriminate post-menopausal women with fractures after adjustment for age and hip BMD. CONCLUSIONS: Our data suggest that an imaging system improves the utility of BUA measurement, but not SOS, for post-menopausal osteoporosis assessment.     

A case–control study of fractures in men with idiopathic osteoporosis: Fractures are associated with older age and low cortical bone density

ObjectivesTo determine biochemical, radiological and micro-architectural bone factors related to fragility fractures in idiopathic male osteoporosis (IMO) patients. IMO is a rare disorder characterized by low areal bone mineral density (aBMD) (Z-score < ? 2) occurring in men after excluding secondary causes of low BMD.MethodsWe conducted a case–control study in 31 patients with fragility fracture (IMO F +) that had occurred after the age of 40 years and 37 without fracture (IMO F–). We first compared IMO group to 40 age-matched disease-free men. We measured aBMD and bone micro-architectural indices at distal radius and tibia sites with a HR-pQCT scan (XtremeCT) using standard and extended cortical analysis. Urine and blood samples were collected in order to determine the levels of bone-turnover markers and the potential determinant of bone fragility. Models of analysis of covariance, including age, height and weight as adjustment factors, were used to compare the groups.ResultsCompared to their controls, IMO patients showed marked disturbance of their micro‐architectural parameters at tibia and radius affecting both trabecular and cortical parameters. IMO F + subjects were significantly older than IMO F ? subjects (58 ± 8 vs. 53 ± 9 yrs, p = 0.01). BMD Z-score at the total-hip was significantly lower in IMO F + (? 1.3 ± 0.5 vs. ? 0.9 ± 0.8 g/cm², p = 0.01). After adjustment, trabecular micro‐architectural parameters, biochemical markers and hormonal parameters were not different in the 2 groups. At distal tibia, cortical v-BMD was significantly lower in IMO F + patients (799 ± 73 vs. 858 ± 60 mg/cm³, p = 0.03), while cortical thickness was not different.ConclusionOur results show that patients with IMO display a marked disturbance of trabecular and cortical bone micro-architecture, and that age and low cortical density are determinants of the fracture occurrence.     

Glial activation in white matter following ischemia in the neonatal P7 rat brain

This study examines cell death and proliferation in the white matter after neonatal stroke. In postnatal day 7 injured rat, there was a marked reduction in myelin basic protein (MBP) immunostaining mainly corresponding to numerous pyknotic immature oligodendrocytes and TUNEL-positive astrocytes in the ipsilateral external capsule. In contrast, a substantial restoration of MBP, as indicated by the MBP ratio of left-to-right, occurred in the cingulum at 48 (1.27 +/- 0.12) and 72 (1.30 +/- 0.18, P < 0.05) h of recovery as compared to age-matched controls (1.03 +/- 0.14). Ki-67 immunostaining revealed a first peak of newly generated cells in the dorsolateral hippocampal subventricular zone and cingulum at 72 h after reperfusion. Double immunofluorescence revealed that most of the Ki-67-positive cells were astrocytes at 48 h and NG2 pre-oligodendrocytes at 72 h of recovery. Microglia infiltration occurs over several days in the cingulum, and a huge quantity of macrophages reached the subcortical white matter where they engulfed immature oligodendrocytes. The overall results suggest that the persistent activation of microglia involves a chronic component of immunoinflammation, which overwhelms repair processes and contributes to cystic growth in the developing brain.     

Changes of concave and convex rib�Cvertebral angle, angle difference and angle ratio in patients with right thoracic adolescent idiopathic scoliosis

The aim of this study is to describe the radiological changes in rib–vertebral angles (RVAs), rib–vertebral angle differences (RVADs), and rib–vertebral angle ratios (RVARas) in patients with untreated right thoracic adolescent idiopathic scoliosis and to compare with the normal subjects. The concave and convex RVA from T1 to T12, the RVADs and the RVARas were measured on AP digital radiographs of 44 female patients with right convex idiopathic scoliosis and 14 normal females. Patients were divided into three groups: normal subjects (group 1), scoliotic patients with Cobb’s angle equal or <30° (group 2) and scoliotic patients with Cobb’s angle over 30° (group 3). Overall values (mean ± SD) of the RVAs on the concave side were 90.5° ± 17° in group 1, 90.3° ± 15.8° in group 2 and 88.8° ± 15.4° in group 3. On the convex side, values were 90.0° ± 17.3° in group 1, 86.3° ± 13.7° in group 2 and 80.7° ± 14.4° in group 3. Overall values (mean ± SD) of the RVADs at all levels were 0.5° ± 0.7° in group 1, 4.0° ± 4.8° in group 2 and 8.0° ± 4.0° in group 3. The RVARa values (mean ± SD) at all levels was 1.008° ± 0.012° in group 1, 1.041° ± 0.061° in group 2 and 1.102° ± 0.151° in group 3. RVAD and RVARa values in the scoliotic segment were greater in patients with untreated scoliosis over 30° than in patients with an untreated deformity of <30° or normal subjects. A significant effect between groups was observed for the RVA, RVAD and RVARa variables. Measurement of RVA, RVAD and RVARa should not only be performed at and around the apex of a thoracic spinal deformity, but also extended to the whole thoracic spine.     

Ambient temperature and ketone body plasma concentration in fasting geese

The effect on ketonemia of alternate exposure to ambient temperatures (Ta) of 25 and 5°C was investigated in fasting geese. Three experimental birds were compared to three controls continuously exposed to 25°C Ta while fasting. During the first 9 days of fasting, when both groups were exposed to 25°C, plasma concentration of -hydroxybutyrate (-OHB) increased similarly in both, from 0.10±0.02 to 6.62±0.71 mmol·L^–1. It later plateaued at 8–9 mmol·L^–1 in the control birds. When the experimental birds were exposed to 5°C Ta between the 9th and 15th day of the fast, it increased further during the first 24 h but thereafter decreased of 57%, from 8.62±1.56 to 3.73±1.24 mmol·L^–1. This decrease was reversed within the 6 days of return to 25°C Ta. In both groups, plasma acetoacetate (AcAc) concentration remained very low during the fast: 51±1 mol·L^–1. This reversible cold-induced effect on ketonemia may be used for investigating the possible role of ketone bodies in protein sparing during fasting.