Suspected neurodevelopmental disorders in adult patients of memory clinics: Start at the beginning. GREDEV proposals for clinical practice.

Neurodevelopmental disorders are frequent but underestimated in adult populations, even though the cognitive profile of those affected remains atypical throughout adulthood and the disorders can cause significant impairment in activities of daily living. Retrospective diagnosis in this population is challenging. In this article, the GREDEV (working group for the assessment of neurodevelopmental disorders in adults) proposes a brief screening questionnaire for patients with suspected neurodevelopmental disorders, a checklist to facilitate taking the patient history, a list of self-administered questionnaires, and the different key steps of diagnosing neurodevelopmental disorders in adults.

Single-centre prospective comparison between contrast-enhanced ultrasound and computed tomography angiography after EVAR.

AIM: To evaluate contrast-enhanced ultrasound (CEUS) as an effective alternative to CT-angiography (CTA) for endoleak detection and aneurismal sac diameter measurement in the follow-up after endovascular abdominal aortic aneurysm repair (EVAR). METHODS: From January 2006 to December 2010, 395 patients underwent EVAR follow-up with both CTA and CEUS. The diameter of the aneurismal sac and the presence of endoleaks were evaluated in all the 395 paired examinations. RESULTS: Bland-Altman plots showed a good agreement in aneurismal sac diameter evaluation between the two imaging modalities. The mean diameter was 54.93 mm (standard deviation (SD) ±12.57) with CEUS and 56.01 mm (SD ± 13.23) with CTA. The mean difference in aneurismal sac diameter was -1.08 mm ± 3.3543 (95% confidence interval (CI), -0.75 to -1.41), in favour of CTA. The number of observed agreement in endoleak detection was 359/395 (90.89%). The two modalities detected the same type I and type III endoleaks. McNemar's χ(2) test confirmed that CTA and CEUS are equivalent in endoleak detection. CONCLUSIONS: CEUS demonstrated to be as accurate as CTA in endoleak detection and abdominal aortic aneurysm diameter measurements during EVAR follow-up, without carrying the risks of radiation exposure or nephrotoxicity. Even if it cannot be proposed as the sole imaging modality during follow-up, our analysis suggests that it should have a major role.

[Idiopathic vasospastic angiopathy of the internal carotid arteries: A rarely recognized cause of ischemic stroke in young individuals]. / Angiopathie vasospastique idiopathique de l'artère carotide interne : une cause peu connue d'accident vasculaire du sujet jeune.

INTRODUCTION: Idiopathic vasospastic angiopathy of the internal carotid arteries is a rare and largely unknown cause of ischemic stroke. METHODS: We report the case of a 39-year-old man with migraine treated by beta-blockers, who had been suffering from progressive right visual impairment and headache for one week. He then experienced a seizure and left hemiparesis. Ophthalmological examination revealed right retinal ischemia and partial left homonymous hemianopia. MRI revealed a long stenosis of both carotid arteries and a recent ischemic stroke in the territory of the right middle cerebral artery. The diagnosis of vasospastic angiopathy of the internal carotid arteries was made based on a second MRI and colored duplex sonography which showed a decrease in the stenosis and no intraparietal hematoma confirming the vasospasm mechanism for stenosis. The clinical course was favorable with calcium channel blockers and aspirin. Use of vasoconstrictor treatments was contraindicated. DISCUSSION/CONCLUSION: Idiopathic vasospastic angiopathy of the internal carotid arteries has been rarely documented. Association with migraine has been mentioned but remains unclear in the literature. This etiology for stroke is probably under-diagnosed due to lack of rapid and repeated examinations of the cervical arteries (angio-MR and colored duplex sonography) to confirm the vasospasm mechanism. Recurrences have been reported justifying a specific secondary preventive treatment to induce vasodilatation. Vasoconstrictor treatments should be contraindicated.

[Optic nerve sheath meningioma. Experience in Lyon with twenty patients]. / Méningiome du nerf optique. L'expérience lyonnaise.

Meningioma is an unfrequent optic nerve tumor. Clinical symptoms are not specific, and pathology exam not easily accessible. The diagnosis is usually based on imaging findings. We report our experience, based on twenty patients. Most of them were middle-aged women (mean age=47 years old). The optic nerve meningioma (ONM) was often revealed by visual dysfunction. Ophthalmological evaluation was altered in most patients (visual acuity, visual fields, fundi). The gold-standard exam was MRI of the optic nerve, with and without gadolinium, and with fat-sat sequences. Thirty percent of the lesions remain stable without treatment. Functional prognosis has been improved only by conformal fractionated radiotherapy, but indications and modalities remain to be specified.

Hyperthyroidism and cerebral venous thrombosis.

The demonstration of an underlying prothrombotic condition in cerebral venous thrombosis (CVT) may have important practical consequences in terms of prevention. Thyrotoxicosis through a hypercoagulable state may be a predisposing factor for CVT. The authors present the cases of 4 patients who developed CVT and hyperthyroidism. At the acute stage, hyperthyroidism was associated with an increase in factor VIII (FVIII). At follow-up, FVIII level remained increased in 2 patients. Hyperthyroidism may have an impact on FVIII level. Accordingly in patients with hyperthyroidism and neurological symptoms, the diagnosis of CVT should be considered and an exhaustive coagulation screening may be appropriate.

Comparison of the heme iron utilization systems of pathogenic Vibrios.

Vibrio alginolyticus, Vibrio fluvialis, and Vibrio parahaemolyticus utilized heme and hemoglobin as iron sources and contained chromosomal DNA similar to several Vibrio cholerae heme iron utilization genes. A V. parahaemolyticus gene that performed the function of V. cholerae hutA was isolated. A portion of the tonB1 locus of V. parahaemolyticus was sequenced and found to encode proteins similar in amino acid sequence to V. cholerae HutW, TonB1, and ExbB1. A recombinant plasmid containing the V. cholerae tonB1 and exbB1D1 genes complemented a V. alginolyticus heme utilization mutant. These data suggest that the heme iron utilization systems of the pathogenic vibrios tested, particularly V. parahaemolyticus and V. alginolyticus, are similar at the DNA level, the functional level, and, in the case of V. parahaemolyticus, the amino acid sequence or protein level to that of V. cholerae.

Over-the-counter availability of antimicrobial agents, self-medication and patterns of resistance in Karachi, Pakistan.

To determine whether the free availability of antimicrobial agents leads to misuse through self-medication, a house-to-house semi-structured interview was held in three different socio-economic areas of Karachi, Pakistan. Of the 2348 households visited, 1342 (57%) participated; this included 9209 individuals. Three hundred and twenty-two (3.5%) had used one or more antimicrobial in the previous 4 weeks, equivalent to 43 agents per 1000 persons per month. The most frequently used agents were amoxycillin (16.7%), co-trimoxazole (15.7%), erythromycin (10.9%), ampicillin/cloxacillin (Ampiclox, 9.1%) and metronidazole (4.5%). Of these, 91.4% were prescribed by a physician, 2.3% were advised by a chemist and 6.3% were used as self-medication. Self-medication increased with socio-economic status. High levels of resistance were found to ampicillin, co-trimoxazole, chloramphenicol and erythromycin. If these high resistance levels are related to the high frequency of antimicrobial use, over-the-counter availability cannot be held responsible. Education of the medical profession seems to be the single most important tool to control misuse of antimicrobial agents. Innovative approaches for continuous medical education are urgently needed.

Paediatric rhabdomyosarcoma: MyoD1 demonstration in routinely processed tissue sections using wet heat pretreatment (pressure cooking) for antigen retrieval.

AIMS: To investigate wet heat pretreatment (pressure cooking) as a means of antigen retrieval for demonstration of MyoD1 in paraffin wax embedded tissue. METHODS: Routinely processed tissue sections of transmission electron microscope confirmed cases of rhabdomyosarcoma were stained immunohistochemically with the MyoD1 antibody. Antigen retrieval was achieved by wet heat pretreatment of the tissue sections. RESULTS: MyoD1 was stained successfully in all seven cases. The protein was localised to nuclei and cytoplasm depending on the type of tumour cell. CONCLUSIONS: Wet heat pretreatment for antigen retrieval from routinely processed tissue sections permits excellent subsequent immunostaining for MyoD1 in rhabdomyoblasts.

Malignant ectomesenchymoma in childhood.

Five childhood malignant ectomesenchymomas are reported from three centers in three countries. The children were all younger than 3 years (four less than 12 months), four were boys, and four tumors were sited in the pelvis or external genitalia. All tumors had distinctive light microscopic features of rhabdomyosarcoma and three also demonstrated small numbers of included neuronal cells. Immunohistochemical studies and transmission electron microscopy revealed the additional presence of neurogenic components in the remaining two patients. An additional epithelial component was found by immunohistochemistry in one tumor, which suggests a pluripotential origin that still requires karyotypic investigation. Aggressive chemotherapy and adequate surgical excision have thus far been effective in providing disease-free follow-up for periods of 7 to 50 months. It is implied that because the biological behavior and morphology closely resemble those of rhabdomyosarcoma and because the neurogenic component is often inconspicuous, many malignant ectomesenchymomas may be misdiagnosed as rhabdomyosarcomas.

Nonclinical hair-pulling: affective correlates and comparison with clinical samples.

The purposes of the current study were to examine the affective states associated with hair-pulling in a nonclinical sample and to compare levels of general psychopathology in nonclinical hair-pullers and clinic patients with trichotillomania (TM) or obsessive-compulsive disorder (OCD). Subjects included 66 college undergraduates who engaged in hair-pulling unrelated to grooming, 18 patients with TM and 29 patients with OCD. Dimensional (but not categorical) ratings of affective experiences in the nonclinical sample indicated that hair-pulling was associated with decreases in tension, boredom, anger and sadness. Further, the relationship between emotional experiences before and after hair-pulling was more salient than the pre-during relationship conceptualized as central in current diagnostic criteria for TM. Comparisons of psychopathology in nonclinical and clinical samples failed to support a continuum notion of increasing symptomatology in nonclinical pullers, TM patients and individuals with OCD. Some evidence of increased pathology in nonclinical pullers relative to TM patients was obtained, as was further support for a distinction between TM and OCD. Implications of this investigation for conceptualization of TM are discussed.

Syphilitic placentitis: an immunopathy.

The placentas of eight infants with congenital syphilis were examined by both immunohistochemical and immunofluorescent techniques. Significant IgM, C3 and rheumatoid factor reactivity were observed in all the syphilitic placentas. We postulate that their presence plays an important role in the evolution of the pathological changes.

Tobacco smoking by pregnant women. Disturbances in metabolism of branched chain amino acids and fetal growth.

Pregnancy alone lowered the plasma AA concentrations of ileu, leu, and val when compared to their concentrations in nonpregnant nonsmokers. Plasma concentrations of val, ileu, and leu were significantly higher in pregnant smokers than in pregnant nonsmokers. Therefore, the utilization of BCAA was more reduced in pregnant smokers than that which could be predicted from plasma AA concentrations of nonpregnant nonsmokers.

African Burkitt's lymphoma in the Cape province of South Africa and in Namibia.

The clinical features, incidence and pattern of jaw involvement, and seasonal occurrence in relation to 22 patients with Burkitt's lymphoma in the Cape province and Southwest Africa were analyzed. The mean age, male dominance, and pattern of organ involvement paralleled typical African Burkitt's lymphoma. Development of the patients' disease was more likely during the rainy season. The frequency and pattern of jaw involvement resembled typical African Burkitt's lymphoma. The frequency of jaw involvement was similar in white and nonwhite patients. Members of all racial groups had typical African Burkitt's lymphoma.

Effect of preeclampsia on carboxyhemoglobin levels: a mechanism for a decrease in P50.

COHb levels were measured in 15 preeclamptic pregnant women and 15 normal pregnant women to investigate the cause for the decrease in P50 associated with preeclampsia. The authors also included six normal and six preeclamptic pregnant patients from the above groups in the determination of P50. Measurements of COHb levels were performed in a Radiometer OSM2 Hemoximeter. Determination of P50 was done using an IL 237 Tonometer, a Radiometer, OSM2 Hemoximeter, and a Corning 168 pH/Blood Gas Analyzer. Preeclamptic pregnant patients had a mean COHb level of 2.8%, whereas normal pregnant women had a mean COHb level of 0.7% (P less than 0.001). Preeclamptic patients also had a significantly lower (24.4 mmHg) P50 than normal pregnant women (P50 = 30.1 mmHg) (P less than 0.001). The authors conclude that a significant elevation of COHb in preeclamptic pregnant women is partly responsible for a significant decrease in P50 seen in preeclampsia.

Effect of pre-eclampsia on plasma cholinesterase activity.

Plasma cholinesterase (PCHE) activity was determined using a colorimetric method in 11 healthy non-pregnant women, 11 healthy pregnant women at term, and 11 pre-eclamptic pregnant women at term. The mean plasma cholinesterase activities for healthy non-pregnant women, healthy pregnant women and pre-eclamptic pregnant women were 438 +/- 81,257 +/- 25 and 173 +/- 18 units . ml-1, respectively. Our data suggest that there is a significant reduction in plasma cholinesterase activity in preeclamptic pregnant women when compared to healthy non-pregnant (p less than 0.001) and healthy pregnant women (p less than 0.001).

A case of the Neu-Laxova syndrome: prenatal ultrasonographic monitoring in the third trimester and the histopathological findings.

A diagnosis of the Neu-Laxova syndrome was made by ultrasonography in the third trimester of pregnancy. Initial ultrasonographic examination, at approximately 28 wk gestation, showed intrauterine growth retardation (IUGR), hypoechoic skeletal structures, kyphosis, feeble fetal activity, and restricted limb movement. Subsequent sonograms showed microcephaly with a receding forehead and prominent eyes, generalised edema and flexion deformities of limbs. Late third trimester findings included polyhydramnios, swelling and webbing of the knee and elbow joints, and severe edema of the hands and feet, giving the impression of absent digits. The clinical features of this case are consistent with group II of Curry's classification of the Neu-Laxova syndrome [Curry, 1982]. The histopathological findings, a triad of dermatological features, poor cortex formation of the long bones, and central nervous system (CNS) dysgenesis, are discussed. In view of the 25% recurrence rate, at risk pregnancies should be carefully monitored by ultrasonography: at 6-8 wk for accurate dating, at 12-16 weeks for active fetal limb movement, and at 16-24 wk for facial and skeletal anomalies, the detection of IUGR, and polyhydramnios.

Relative importance of first- and second-phase insulin secretion in glucose homeostasis in conscious dog. II. Effects on gluconeogenesis.

The normal pancreatic response to an exogenous glucagon infusion is a biphasic release of insulin. In our study the ability of each component of insulin release to counter the effects of the glucagon on gluconeogenesis and alanine metabolism was assessed by mimicking first- and/or second-phase insulin release with infusions of somatostatin and intraportal insulin. When a fourfold increase in glucagon was brought about in the presence of fixed basal insulin release, there was a large increase in overall glucose production and gluconeogenesis. The increase in the conversion of [14C]alanine into [14C]glucose (169 +/- 42%, P less than .05) was accompanied by an increase in the fractional extraction of alanine by the liver (FEA 0.32 +/- 0.06 to 0.66 +/- 0.10, P less than .05) and net hepatic alanine uptake (NHAU 2.97 +/- 0.45 to 4.61 +/- 0.48 mumol . kg-1 . min-1, P less than .05). Simulated first-phase insulin release had no effect on the ability of glucagon to increase FEA (0.32 +/- 0.03 to 0.66 +/- 0.03, P less than .05) or NHAU (3.69 +/- 0.80 to 5.10 +/- 0.69 mumol . kg-1 . min-1, P less than .05) but did limit the increase in overall gluconeogenic conversion (114 +/- 37%). Second-phase insulin release had no effect on either the glucagon-induced increase in FEA (0.35 +/- 0.08 to 0.73 +/- 0.04) or NHAU (3.35 +/- 0.92 to 5.13 +/- 0.85 mumol . kg-1 . min-1) but completely inhibited the increase in overall gluconeogenic conversion.(ABSTRACT TRUNCATED AT 250 WORDS)