Simona Bertoli

The ketogenic diet in children, adolescents and young adults with refractory epilepsy: An Italian multicentric experience

PURPOSE This collaborative study by three Italian groups of child neuropsychiatrists was carried on to evaluate the efficacy and safety of the classic 4:1 ketogenic diet as add-on treatment in refractory partial or generalized epilepsy in children, adolescents and young adults. METHODS We performed a prospective add-on study in 56 refractory epilepsy young patients (age 1-23 years, mean 10.4 years), all with both symptomatic and cryptogenic, generalized or partial epilepsies. Child neuropsychiatrists worked with nutritional team for sample selection and patients management. The ketogenic diet was added to the baseline antiepileptic drugs and the efficacy was rated according to seizure type and frequency. During treatment, seizure frequency, side effects, urine and blood ketone levels and other parameters were systematically evaluated. RESULTS Patients have been treated for 1-18 months (mean 5 months). A >50% reduction in seizure frequency was gained in 37.5 and 26.8% of patients after 3 and 6 months, respectively, at 12 months, this number fell by 8.9%. No significant relationship between diet efficacy and seizure or epilepsy type, age at diet onset, sex and etiology of epilepsy was noted. Nevertheless, it seems noteworthy that 64% of our patients with neuronal migration disorders improved on this diet. Adverse effects occurred, mainly in the first weeks of treatment, in 32 patients (57.1%), but were generally mild and transient. In seven patients (12.5%) it was possible to withdraw one to two AED after 3-4 months on ketogenic diet. CONCLUSION This initial experience with the ketogenic diet was effective in difficult-to-treat patients with partial and generalized epilepsies, though its efficacy dropped significantly by 9-12 months.

Body composition assessment in spinal cord injury subjects.

Abstract.Total and segmental body composition (fat mass, FM; fat-free mass, FFM; bone mineral density, BMD) were evaluated in 13 sedentary spinal cord injury (SCI) subjects and in 13 able-bodied healthy males (control, C) using dual X-ray absorptiometry (DXA) and skinfold methods. In the SCI group, total FM was significantly higher (31.1±8.2 vs. 20.8±6.9%) and total FFM was significantly lower (62.2±8.9 vs. 73.5±6.4%) than in C subjects. Total BMD did not differ between the SCI and C groups (1.20±0.11 vs. 1.30±0.11 g/cm2). In the SCI group, segmental FM was higher in the legs and trunk, whereas BMD was lower in legs only. The skinfold method significantly underestimated FM in the SCI group. Body composition is severely modified in paralyzed segments. The predictive equations developed for healthy populations appear to be inapplicable to SCI subjects.

Cultivation, Genetic, Ethnopharmacology, Phytochemistry and Pharmacology of Moringa oleifera Leaves: An Overview.

Moringa oleifera is an interesting plant for its use in bioactive compounds. In this manuscript, we review studies concerning the cultivation and production of moringa along with genetic diversity among different accessions and populations. Different methods of propagation, establishment and cultivation are discussed. Moringa oleifera shows diversity in many characters and extensive morphological variability, which may provide a resource for its improvement. Great genetic variability is present in the natural and cultivated accessions, but no collection of cultivated and wild accessions currently exists. A germplasm bank encompassing the genetic variability present in Moringa is needed to perform breeding programmes and develop elite varieties adapted to local conditions. Alimentary and medicinal uses of moringa are reviewed, alongside the production of biodiesel. Finally, being that the leaves are the most used part of the plant, their contents in terms of bioactive compounds and their pharmacological properties are discussed. Many studies conducted on cell lines and animals seem concordant in their support for these properties. However, there are still too few studies on humans to recommend Moringa leaves as medication in the prevention or treatment of diseases. Therefore, further studies on humans are recommended.

Evaluation of nutritional status in children with refractory epilepsy

Backgroundchildren affected by refractory epilepsy could be at risk of malnutrition because of feeding difficulties (anorexia, chewing, swallowing difficulties or vomiting) and chronic use of anticonvulsants, which may affect food intake and energy metabolism. Moreover, their energy requirement may be changed as their disabilities would impede normal daily activities. The aim of the present study was to evaluate nutritional status, energy metabolism and food intake in children with refractory epilepsy.Methods17 children with refractory epilepsy (13 boys and 4 girls; mean age 9 ± 3,2 years; Body Mass Index 15,7 ± 3,6) underwent an anthropometric assessment, body composition evaluation by dual-energy X-ray absorptiometry, detailed dietetic survey and measurement of resting energy expenditure by indirect calorimetry. Weight-for-age, height-for-age (stunting) and weight-for-height (wasting) were estimated compared to those of a reference population of the same age.Results40% of children were malnourished and 24% were wasted. The nutritional status was worse in the more disabled children. Dietary intake resulted unbalanced (18%, 39%, 43% of total daily energy intake derived respectively from protein, lipid and carbohydrate). Adequacy index [nutrient daily intake/recommended allowance (RDA) × 100] was < 60% for calcium iron and zinc.Conclusionmany children with refractory epilepsy would benefit from individual nutritional assessment and management as part of their overall care.

Effects of liver transplantation on the nutritional status of patients with cystic fibrosis.

The long‐term effects of liver transplantation on nutritional status, body composition and pulmonary function in patients with liver disease associated with cystic fibrosis (CF) are poorly defined. We studied 15 patients with CF‐associated biliary cirrhosis and severe portal hypertension. Seven underwent liver transplantation (age: 14.8 ± 6.2 years), and eight were treated conservatively (age: 15.9 ± 6.7 years). All patients were evaluated at baseline and thereafter yearly for a median duration of 5 years. During follow‐up, transplanted patients gained weight and showed a significant increment in body mass index (P < 0.004), whereas patients without transplantation remained stable (P = 0.063). Baseline bone mineral content (dual energy X‐ray absorptiometry scan) was lower than normal in all patients (more in transplanted patients) and increased in transplanted patients (P < 0.05), but not in patients without transplantation. In both groups percent body fat did not change, whereas fat free mass increased only in the transplant group (P = 0.06) (P < 0.03 versus nontransplanted patients). Only in transplanted patients’ plasma concentrations of vitamin E and A increased (P < 0.05 versus nontransplanted patients). Forced espiratory volume in 1 s and forced vital capacity showed similar deterioration in transplanted and in nontransplanted patients. Liver transplantation is associated with long‐term beneficial effects on the nutritional status of CF patients and seems to favor bone mineralization.

A pilot study of a ketogenic diet in patients with Lafora body disease

PURPOSE Lafora body disease (LBD) is severe and rapidly worsening progressive myoclonus epilepsy (PME), not treatable with specific therapy. In LBD patients, typical polyglucosan accumulations result from alterations of proteins involved in the regulation of glycogen metabolism. Thus, a ketogenic regimen might reasonably be expected to counteract the disease progression. We set out to assess the feasibility and tolerability of a long-term ketogenic diet (KD) in LBD patients and to make a preliminary evaluation of its effect on the disease course. METHODS We treated five LBD patients with KD and evaluated the changes in the clinical, neuropsychological and neurophysiological findings over 10-30 months. RESULTS The KD was well tolerated in all the patients for the first 16 months. Nutritional measures and laboratory findings remained substantially stable. The disease progressed in all the patients, reaching an advanced stage in one. Electrophysiological findings indicated the presence of increased cortical excitability in four patients, paralleling the worsening of the myoclonus. CONCLUSION KD was unable to stop the disease progression. However, given the considerable heterogeneity of the natural history of LBD, we cannot exclude the possibility that KD has the potential to slow down the disease progression. The application of this nutritional approach should be further evaluated in larger case series.

Effects of the ketogenic diet on nutritional status, resting energy expenditure, and substrate oxidation in patients with medically refractory epilepsy: A 6-month prospective observational study

BACKGROUND & AIMS This 6-month prospective, single-arm observational study was designed to assess the effects of the KD on the nutritional status, resting energy expenditure (REE), and substrate oxidation in patients with drug-resistant epilepsy. METHODS Eighteen patients with medically refractory epilepsy underwent assessment of body composition, REE, and substrate oxidation rates before and after 6 months of KD. RESULTS Compared with baseline, there were no statistically significant differences at 6 months in terms of height, weight, BMI z-scores, and REE. However, the respiratory quotient decreased significantly (from 0.80 ± 0.06 to 0.72 ± 0.05, p < 0.001) whereas fat oxidation was significantly increased (from 50.9 ± 25.2 mg/min to 97.5 ± 25.7 mg/min, p < 0.001). Interestingly, we found that the increase in fat oxidation was the main independent predictor of the reduction in seizure frequency (beta = -0.97, t = -6.3, p < 0.05). CONCLUSIONS Administering a KD for 6 months in patients with medically refractory epilepsy increases fat oxidation and decreases the respiratory quotient, without appreciable changes in REE.

Poor agreement between a portable armband and indirect calorimetry in the assessment of resting energy expenditure

BACKGROUND & AIMS To evaluate the agreement between resting energy expenditure (REE) estimated by a portable armband and measured by indirect calorimetry. METHODS One-hundred and twenty-seven women and 42 men with a mean (SD) age of 44 (12) years and a body mass index of 30.2 (5.4) kg/m(2) were studied. REE was estimated using the Sense Wear Pro 2 Armband (SWA), measured using the Sensor Medics 29 metabolic cart (V(max)), and estimated using Schofields equation. The limits of agreement (LOA) and the concordance correlation coefficient (CCC) were used to evaluate the interchangeability of the methods. RESULTS The LOA between REE(SWA) and REE(Vmax) were wide in both women (-269 to 378 kcal/day) and men (-330 to 545 kcal/day) and CCC was low (0.579 in females and 0.583 in males, p<0.0001 for both). REE(Schofield) agreed with REE(Vmax) to a similar degree (CCC=0.563 in females and 0.500 in males, p<0.0001 for both). CONCLUSIONS SWA and indirect calorimetry are not interchangeable methods for the assessment of REE in normal-weight and obese subjects.

Feasibility of air plethysmography (BOD POD) in morbid obesity: a pilot study.

Abstract.The assessment of body composition (BC) in morbidly obese patients is a difficult procedure. Air-displacement plethysmography (ADP), which measures body density, is a very promising technique for BC assessment in health and disease. However, there are very few data about the feasibility of applying ADP on morbidly obese patients, which theoretically could be affected by large body size and difficulty in lung volume measurements. The main aim of this pilot study was to evaluate the feasibility of using ADP for BC assessment in morbidly obese patients. We studied nine subjects (6 males and 3 females) who had a mean age (±SD) of 47.0±13.5 years and body mass index (BMI) of 46.6±7.7 kg/m2 (range 36.4–58.8). All patients could fit into the instrument chamber and perform the manoeuvre for pulmonary plethysmography. Mean lung volume was 3.9±1.2 l and mean percent body fat was 53.1±6.6 (range 46.0–67.5). These results indicate that ADP appears to be suitable for patients with BMI over 40 kg/m2 and produces realistic BC data.

Nutritional Characterization and Phenolic Profiling of Moringa oleifera Leaves Grown in Chad, Sahrawi Refugee Camps, and Haiti

Moringa oleifera is a plant that grows in tropical and subtropical areas of the world. Its leaves are rich of nutrients and bioactive compounds. However, several differences are reported in the literature. In this article we performed a nutritional characterization and a phenolic profiling of M. oleifera leaves grown in Chad, Sahrawi refugee camps, and Haiti. In addition, we investigated the presence of salicylic and ferulic acids, two phenolic acids with pharmacological activity, whose presence in M. oleifera leaves has been scarcely investigated so far. Several differences were observed among the samples. Nevertheless, the leaves were rich in protein, minerals, and β-carotene. Quercetin and kaempferol glycosides were the main phenolic compounds identified in the methanolic extracts. Finally, salicylic and ferulic acids were found in a concentration range of 0.14–0.33 and 6.61–9.69 mg/100 g, respectively. In conclusion, we observed some differences in terms of nutrients and phenolic compounds in M. oleifera leaves grown in different countries. Nevertheless, these leaves are a good and economical source of nutrients for tropical and sub-tropical countries. Furthermore, M. oleifera leaves are a source of flavonoids and phenolic acids, among which salicylic and ferulic acids, and therefore they could be used as nutraceutical and functional ingredients.