Extradural cavernous haemangioma simulating a disc protrusion

Cavernous haemangiomas confined to the epidural space are rare and are therefore infrequently considered in the differential diagnosis of spinal epidural masses. In order to draw attention to this diagnosis, a case in which an epidural cavernous haemangioma simulates a lateral/foraminal disc protrusion is presented.     

Clinical phenotype and neuroimaging findings in a French family with hereditary ferritinopathy (FTL498‐499InsTC)

To describe a family with a hereditary ferritinopathy (HF) due to a mutation in the ferritin light chain gene (FTL498‐499InsTC mutation). Case reports of the clinical features, MRI, ¹⁸FDG PET, and pathological findings observed in this family with two patients described in more details. Postural tremor (phenotype‐1) or cerebellar signs (phenotype‐2) were the first neurological symptoms detected. Parkinsonian, cerebellar and pyramidal syndromes, abnormal involuntary movements, dementia were observed in both phenotypes at more advanced stages. Beside characteristics T2* hypointense signals suggestive of iron accumulation in the striatum, mesencephalon, and cerebellum, we detected more diffuse changes including cerebellar, cortical and subcortical atrophy, cortical iron deposition, and severe leukoencephalopathy. ¹⁸FDG PET showed frontal and cerebellum hypometabolism with more severe frontal defect in patients with cognitive decline. Pathological examination showed ferritin and iron deposition in the liver, kidney, muscle, skin, and in the central nervous system. Members of this family affected by HF due to the FTL498‐499InsTC mutation have a specific clinical presentation with initial postural tremor or cerebellar ataxia, followed by pyramidal and extrapyramidal motor syndromes and late severe subcortical dementia. © 2009 Movement Disorder Society     

A French retrospective study on clinical outcome in 102 choroid plexus tumors in children

The aim of this study was to review and describe therapeutic approaches in children with choroid plexus tumor (CPT) based on a nationwide series. The World Health Organization classification subdivides these rare tumors into three histological subtypes corresponding to three grades of malignancy: low grade (grade I) choroid plexus papilloma (CPP), intermediate grade (grade II) atypical choroid plexus papilloma (aCPP) and high grade (grade III) choroid plexus carcinoma (CPC). This retrospective study included 102 French children younger than 18 years, treated from 2000 to 2012: 54 CPP, 26 aCPP and 22 CPC. The 5 year overall survival was 100% in CPP, 96.2% in aCPP and 64.7% in CPC. In patients with localized disease, complete surgical resection was achieved in 48/52 CPP, 20/26 aCPP and 7/14 CPC. In this group, patients with complete surgical resection had better event free survival than patients with partial resection (88.9 vs. 41.6%). 28 patients (1 CPP, 6 aCPP and 22 CPC) had adjuvant chemotherapy. 2 aCPP and 9 CPC had radiotherapy. We underlined the need for a central histological review to accurately analyze clinical data; we reported a much higher overall survival for CPC than in most previous CPT series probably including atypical teratoid rhabdoid tumors. In our series, the 5 years overall survival in CPC (64.7%) was higher than event free survival (25.2%) and could be interpreted as a clue for the efficiency of adjuvant/salvage therapy even if the heterogeneity of applied treatments in this retrospective series does not allow for meaningful statistical comparisons.     

Harmful or Helpful: Perceived Solicitous and Facilitative Partner Responses Are Differentially Associated with Pain and Sexual Satisfaction in Women with Provoked Vestibulodynia

IntroductionProvoked vestibulodynia (PVD) is a highly prevalent vulvovaginal pain condition that negatively affects women's emotional, sexual, and relationship well‐being. Recent studies have investigated the role of interpersonal variables, including partner responses.AimWe examined whether solicitous and facilitative partner responses were differentially associated with vulvovaginal pain and sexual satisfaction in women with PVD by examining each predictor while controlling for the other.MethodsOne hundred twenty‐one women (M age = 30.60, SD = 10.53) with PVD or self‐reported symptoms of PVD completed the solicitous subscale of the spouse response scale of the Multidimensional Pain Inventory, and the facilitative subscale of the Spouse Response Inventory. Participants also completed measures of pain, sexual function, sexual satisfaction, trait anxiety, and avoidance of pain and sexual behaviors (referred to as “avoidance”).Main Outcome MeasuresDependent measures were the (i) Pain Rating Index of the McGill Pain Questionnaire with reference to pain during vaginal intercourse and (ii) Global Measure of Sexual Satisfaction Scale.ResultsControlling for trait anxiety and avoidance, higher solicitous partner responses were associated with higher vulvovaginal pain intensity (β = 0.20, P = 0.03), and higher facilitative partner responses were associated with lower pain intensity (β = ?0.20, P = 0.04). Controlling for sexual function, trait anxiety, and avoidance, higher facilitative partner responses were associated with higher sexual satisfaction (β = 0.15, P = 0.05).ConclusionsFindings suggest that facilitative partner responses may aid in alleviating vulvovaginal pain and improving sexual satisfaction, whereas solicitous partner responses may contribute to greater pain. Rosen NO, Bergeron S, Glowacka M, Delisle I, and Baxter ML. Harmful or helpful: Perceived solicitous and facilitative partner responses are differentially associated with pain and sexual satisfaction in women with provoked vestibulodynia. J Sex Med 2012;9:2351–2360.     

Usefulness of in vitro 1H magnetic resonance spectroscopy for the characterization of primary brain tumors: report of two cases

Pathological diagnosis of brain tumors provides an index of brain disease severity and guides clinical practice in their treatment. Diagnoses are often made from biopsy material obtained using stereotactic techniques with the difficulty of making a histological diagnosis in small samples. In our experience, the estimation of the degree of malignancy on the basis of in vitro 1H magnetic resonance spectroscopy (1H MRS) appears to well correlate with histology and clinical evolution. We report two cases with a discordance between the diagnoses on the basis of histology examination and in vitro 1H MRS whose evolution seems to correlate better with the data of 1H MRS.     

Prevalence of anemia among schoolchildren in the province of Kenitra in Morocco

Anemia is a widespread public health problem in developed and developing nations, and its prevalence is highest among pregnant women. In developing countries, schoolchildren constitute the population with the next-highest prevalence. Because there are few studies of anemia in schoolchildren in Morocco, this study aimed to determine its prevalence and its risk factors among preadolescents. We recruited 306 pupils from seven primary schools; blood samples were taken with their parents' or guardians' consent. We also collected anthropometric data, information about social and demographic characteristics (parent questionnaire) and school attendance and performance. RESULTS: More than 30% of these children had anemia: prevalence did not differ by sex, but was higher among those living in urban environments. Factors related to food behavior, especially diet diversity, appeared to be important. Our results found no relation between anemia and school performance. In the future more detailed cognitive tests should be used for this type of study. CONCLUSION: The prevalence of anemia among schoolchildren is high in the province of Kénitra, and the school health system is weak. Decision-makers have recently become aware of the need for an integrated approach to this age group: schools offer an opportunity for prevention and cure.     

Comparative single-dose pharmacokinetics of clonazepam following intravenous,intramuscular and oral administration to healthy volunteers

The objective was to assess the single-dose pharmacokinetics of clonazepam following i.m., p.o. and i.v. administration. In an open-label, three-way crossover study, 12 healthy volunteers were randomized to receive a single dose of 2 mg clonazepam either by the i.m., p.o. or i.v. route. Serial blood samples were collected up to 120 h after drug administration. Plasma concentrations of clonazepam were determined by electron-capture gas-liquid chromatography. The absorption rates of clonazepam after i.m. and p.o. administration of clonazepam were significantly different from each other, as reflected by the respective mean values of maximum plasma concentration (C(max) 11.0 vs. 14.9 ng.ml(-1)) and time to reach maximum concentration (t(max) 3.1 vs. 1.7 h). Secondary plasma peaks of clonazepam were observed in 9 volunteers after i.m. injection (C(max) 9.9 ng.ml(-1); t(max) 10.4 h). A comparison of the area under the plasma concentration-time curves (AUC) shows that the i.m. route is equivalent to the oral route (AUC(0- infinity ) 620 vs. 561 ng.h.ml(-1)). Clonazepam was almost completely absorbed after i.m. and p.o. administration, as shown by the mean absolute bioavailability of 93 and 90%, respectively. No significant differences existed between the elimination half-lives (i.v. 38.0 h; i.m. 43.6 h; p.o. 39.0 h). The average clearance and volume of distribution (V(Z)) were 55 ml.min(-1) and 180 liters, respectively. In conclusion, the observed differences in C(max) and t(max) after i.m. and p.o. administration were consistent with a slower absorption rate of clonazepam after i.m. injection. The systemic exposure to clonazepam was not affected by the route of extravascular administration. Statistical evaluation of these kinetic data showed differences in the absorption rate, so that clonazepam given by the i.m. route is not bioequivalent to the oral route. On the basis of the results of this study, we would recommend the same i.m. and p.o. dose in epileptic patients, but therapeutic response would be expected to be less predictable and to occur later in the case of i.m. administration.     

Rhino-cerebral fungal infection successfully treated with supplementary hyperbaric oxygen therapy

Rhino-cerebral fungal infections are rare and difficult disorders to cure. We report the case of a woman presenting a left trigeminal neuralgia complicated by ophthalmoplegia and blindness. MRI demonstrated a lesion of the left orbital apex with extension into the cavernous sinus. Fungal infiltration (aspergillosis or mucormycosis), was seen on biopsy. High-dose liposomal Amphotericin B (5mg/kg/day) for six weeks was unsuccessful. Adjunctant hyperbaric oxygen therapy led to clinical and radiological improvement. Hyperbaric oxygen therapy is discussed in the medical management of rhino-cerebral yeast abscesses.     

Expression of CCK2 receptors in the murine pancreas: proliferation, transdifferentiation of acinar cells, and neoplasia

BACKGROUND & AIMS: To explore the pancreatic function of CCK2/gastrin receptor, we created ElasCCK2 transgenic mice expressing the human receptor in pancreatic exocrine cells. In previous studies, the transgenic CCK2/gastrin receptor was demonstrated to mediate enzyme release and protein synthesis. We now report results of phenotypic and long-term studies. METHODS: Pancreas was characterized using morphometry and immunohistochemistry. ElasCCK2 mice were crossed with INS-GAS mice expressing gastrin in pancreatic beta cells to achieve continuous stimulation of the CCK2/gastrin receptor. RESULTS: The pancreatic weight of ElasCCK2 mice was increased by 40% and correlated with an increase in the area of exocrine tissue. Alterations in pancreatic histology were apparent from postnatal day 50. Crossing the ElasCCK2 mice with INS-GAS mice resulted in development of morphologic changes in younger animals. Malignant transformation occurred in 3 of 20 homozygous ElasCCK2 mice. Although tumors had different phenotypes, they all developed through an acinar-ductal carcinoma sequence. CONCLUSIONS: Our data show that transgenic expression of a G protein-coupled receptor can lead to cancer. This study also supports a key role of the CCK2/gastrin receptor in the development of pre- and neoplastic lesions of the pancreas. ElasCCK2 mice provide a model for carcinogenesis by transformation and dedifferentiation of acinar cells.