The ketogenic diet improves recently worsened focal epilepsy

Aim  We observed a dramatic response to the ketogenic diet in several patients with highly refractory epilepsy whose seizure frequency had recently worsened. This study aimed to identify whether this characteristic was a useful indication for the ketogenic diet.
Method  From the 70 patients who received the ketogenic diet during a 3-year period at our institution, we retrospectively selected patients with focal epilepsy. There were 22 children, 13 females and nine males, aged from 5 months to 18 years 6 months (mean 6y 9mo, SD 5y 11mo). Fifteen had symptomatic and seven had cryptogenic focal epilepsy. Seizure frequency 1 week before initiating the ketogenic diet was compared with that at 1 month and at the last visit on the diet.
Results  Eleven patients were responders (defined as reduction of seizures by more than 50%) at 1 month. Responders were higher ( p =0.046) in the group with a recent worsening of seizures than in those with stable seizure frequency. Seven patients were still seizure-free at 6 months on the diet. Tolerability was excellent in 10 patients. Five patients stopped the diet because of early side effects.
Interpretation  The ketogenic diet may be a valuable therapeutic option for children with pharmacoresistant focal epilepsy, particularly those with a recent deterioration of seizure control and neurological status. Because of its rapid effect, the ketogenic diet may be a useful support to intravenous emergency drugs in such a situation.     

Access to health and social services for IDU: The impact of a medically supervised injection facility

Introduction and Aims. Injection drug users (IDU) often experience barriers to conventional health‐care services, and consequently might rely on acute and emergency services. This study sought to investigate IDU perspectives regarding the impact of supervised injection facility (SIF) use on access to health‐care services. Design and Methods. Semi‐structured qualitative interviews were conducted with 50 Vancouver‐based IDU participating in the Scientific Evaluation of Supervised Injecting cohort. Audio‐recorded interviews elicited IDU perspectives regarding the impact of SIF use on access to health and social services. Interviews were transcribed verbatim and a thematic analysis was conducted. Results. Fifty IDU, including 21 women, participated in this study. IDU narratives indicate that the SIF serves to facilitate access to health care by providing much‐needed care on‐site and connects IDU to external services through referrals. Participants’ perspectives suggest that the SIF has facilitated increased uptake of health and social services among IDU. Discussion and Conclusions. Although challenges related to access to care remain in many settings, SIF have potential to promote health by facilitating enhanced access to health‐care and social services through a model of care that is accessible to high‐risk IDU.[Small W, Van Borek N, Fairbairn N, Wood E, Kerr T. Access to health and social services for IDU: The impact of a medically supervised injection facility. Drug Alcohol Rev 2009;28:341–346]     

MICROSATELLITE INSTABILITY IN INTESTINAL- AND DIFFUSE-TYPE GASTRIC CARCINOMA

To investigate the role of genetic instability in the development of intestinal- and diffuse-type gastric cancers, six microsatellite loci were analysed in 98 carcinomas of the two main histotypes, at both early and advanced stages of progression, and in five preneoplastic lesions. RER+ phenotype frequency proved to be significantly higher ( P =0·013) in intestinal (23 per cent) than in diffuse cancers (5 per cent) and slightly higher in advanced (19 per cent) than in early (12 per cent) tumours. When comparing early and advanced tumours of the same histotype, a similar frequency was found for diffuse tumours (4 per cent vs. 6 per cent), and an increase from 19 to 30 per cent for intestinal cancers. Instability at more than one locus was limited to intestinal tumours and replication errors were also detected in an intestinal dysplasia. On the whole, these data suggest that genetic instability has an important and early role in gastric carcinogenesis of the intestinal type and a less important role in gastric carcinogenesis of the diffuse type. Most tumours of this panel had previously been characterized for p53 gene mutations. p53 screening was extended to all samples, to investigate the possible association between gene mutations and microsatellite instability. Analysis showed a trend ( P =0·07, Fisher's exact test) towards a negative association between these two genetic lesions in tumours of the intestinal type. © 1997 John Wiley & Sons, Ltd.     

Muscle MRI findings in a three-generation family affected by Bethlem myopathy

We report clinical and muscle magnetic resonance imaging (MRI) findings in three individuals (aged 6, 26 and 73 years) from a three-generation family with Bethlem myopathy, confirmed by molecular genetic analysis which showed an exon skipping mutation in the COL6A1 gene. The clinical severity ranged from mild proximal weakness and distal laxity in the younger patients, to inability to stand or walk and severe contractures in the 76-year-old grandmother. The pattern of muscle involvement showed variable severity in parallel with the severity of motor function impairment. Although there was a marked variability in the severity of the MRI findings, it was possible to recognize a specific pattern of muscle involvement in all three patients. This consisted of involvement of the peripheral region of the vastus lateralis and hamstrings muscles with relative sparing of their central part. This was best appreciated in the third decade of life, but could also be identified both in the younger patient with minimal MRI changes and in the oldest patient, despite her more severe and diffuse muscle involvement. This report suggests that muscle MRI could be used as an additional tool to establish the pattern and the degree of muscle involvement in patients with Bethlem myopathy. Further studies in a larger cohort are needed to evaluate the specificity of these findings.     

Screening for deep venous thrombosis after idiopathic scoliosis surgery in children: a pilot study

Background: Venous thrombosis remains an uncommon disorder in childhood. However, the incidence appears to be increasing for a multitude of reasons. The aim of the study was to detect asymptomatic deep venous thrombosis and prothrombotic diseases in nonsyndromic children undergoing scoliosis surgery. Methods: A prospective study including forty successive teenagers scheduled for posterior spinal fusion. Patients with scoliosis with a history of hemoglobinopathies, cardiac defects, blood clots, early onset osteoporosis, as well as patients with skeletal dysplasias and nonskeletal dysplastic syndromic entities have been excluded. The protocol was designed for active screening of deep venous thrombosis using color Doppler ultrasonography on a day before surgery and repeated on the 3rd, 7th and 15th day postoperatively. Evaluation of prothrombotic disorders included antithrombin and protein C activities, and total protein S antigen level. Results: No patient has manifested clinical symptoms of venous thrombosis in our study. Preoperative Doppler and ultrasound examinations were normal in all patients. Although repeated Doppler ultrasonography demonstrated a transient small clot in two patients. Congenital antithrombin deficiency of 5% has been observed in one child only, without the development of deep venous thrombosis. Conclusion: Thromboembolic event seems to be rare after scoliosis surgery. Prophylaxis for venous thrombosis should not be recommended in such patient. But, larger series are required to confirm such results.     

Perceived social support and dysfunctioning in “clinical” and “normal” adolescents

The purpose of this study was to examine the extent to which inpatient and non-patient samples of youngsters differ from each other with regard to prevalence and co-occurrence of emotional and behavioural problems and negative perceptions of family, school and peers. The sample comprised 202 12–21-year-old adolescents: 101 psychiatric inpatients and 101 matched “community-based” youngsters. Possible differences between the subgroups were tested by means of multi-variate analysis of variance and relative risk ratios.All specific problems and combination patterns were reported significantly more often by the “inpatient” than by the “non-patient” youngsters. However, after correcting for differences in base rates, only the combination of emotional and/or behavioural problems and family problems remained significant. The combination of emotional and family problems was reported about 12 times more often and the combination of behavioural and family problems even about 21 times more often by adolescents in the “clinical” sample than by those in the “normal” sample.Two important conclusions were drawn: (1) co-occurrence in itself did not appear to be the distinguishing factor between the “clinical” and the “normal” sample; and (2) a dominant role of negatively perceived family support in adolescent functioning was suggested. The implications of these findings are discussed.     

EFFECT OF KETANSERIN ON SODIUM NITROPRUSSIDE REQUIREMENTS, ARTERIAL PRESSURE CONTROL AND HEART RATE FOLLOWING CORONARY ARTERY BYPASS SURGERY

In a double-blind, placebo controlled study ketanserin, a serotoninS₂ antagonist, was administered to hypertensive patients whohad undergone coronary artery bypass surgery. Patients wereallocated randomly to receive placebo or ketanserin at an infusionrate of 0.05, 1 or 2 mg kg^–1 h^–1. Sodium nitroprussidewas used as escape medication. Ketanserin reduced the nitroprussiderequirements and improved the quality of arterial pressure controlin all groups, and this was significant in the low- and high-dosegroups. There was a significant decrease in heart rate in thelow- and high-dose groups compared with placebo, and no effectin patients who received the medium dose of ketanserin. Ketanserinmay be a useful treatment for hypertension following coronaryartery surgery as it reduced arterial pressure without reflextachycardia.     

MEMORY FUNCTIONING IN SOCIAL DRINKERS: A STUDY OF EVENT-RELATED POTENTIALS

Verbal memory functioning was assessed in light and heavy socialdrinkers by recording event-related potentials in subjects instructedto memorize lists of words using either repetition or elaborativelearning strategies. Although overall performance did not differsignificantly between light and heavy drinkers, heavy drinkersshowed reduced within-list organization compared with lightdrinkers. There were statistically significant differences inthe event-related potential indices of memory functioning elicitedduring complex elaborative processing which were consistentwith decreased within-list organization in heavy drinkers.