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Background Although seizures (other than myoclonus) are frequently reported in Creutzfeldt‐Jakob disease (CJD), their frequency, clinical manifestations, and effect on the disease course is unknown. Objectives To characterize the frequency of seizures in E200K familial and sporadic CJD, to describe its semiology, EEG and MRI findings. Methods In this retrospective study, we reviewed all patients with CJD who were seen in the Sheba Medical Center between the years 2003–2012 and underwent clinical evaluation, genetic testing, EEG and MRI studies. The diagnosis of seizures was carried out based on documentation of episodes consistent with seizures or episode of unresponsiveness correlated with ictal activity in EEG. Results Sixty‐four probable patients with CJD were included in the study, 57 (89%) with E200K familial (fCJD) and 7 (11%) with sporadic (sCJD). Seizures occurred in 8 patients: 3 of 7 (43%) in patients with sCJD compared to 5/57 (9%) in patients with E200K fCJD (P = 0.04, chi‐square test). Two of E200K fCJD patients with seizures had other non‐prion etiologies for seizures (brain metastasis, known history of temporal lobe epilepsy which started 44 years before the diagnosis of CJD). Seizures occurred late in the course of the disease with an average of 12 days between the onset of seizures and death. Conclusion Seizures in E200K fCJD were infrequent and occurred late in the disease course. This difference suggests that E200K fCJD represents a separate subtype of the disease with distinct clinical characteristics. [ABSTRACT FROM AUTHOR]

A total of 600 rats was distributed equally among 15 diets formulated from mixtures of raw and toasted soy flour and casein so as to provide 5 different levels of trypsin inhibitor (TI) at 3 levels of protein (10%, 20%, and 30%). Animals were sacrificed at the end of 6 months and at 3-month intervals thereafter over a period of 22 months. Increases in the secretory activity of the pancreas, as measured by trypsin and chymotrypsin activities in the pancreas and small intestines, were associated with increasingly higher levels of TI and protein but did not change appreciably with time. Hypertrophy and hyperplasia of the pancreas, as reflected by changes in pancreas weight and RNA and DNA content, developed at an early stage (6 months) and were likewise positively correlated with the levels of TI and protein in the diet. Although the hypertrophic response remained unchanged with time, hyperplasia became more pronounced as the period of exposure to TI was prolonged. The peptide test confirmed the stimulatory effect of TI on the pancreas, but some functional impairment was noted in those animals which had been fed raw soy flour for 18 months of or longer. The appearance of nodules on the pancreas was observed by gross inspection after 15 months, and these became larger and more numerous with time. The incidence of nodules was highly correlated with the level of TI in the diet and was highest when the diet contained 20% protein. [ABSTRACT FROM AUTHOR]

1. Ducklings were given diets with vegetable protein (VP) and 0 or 600 g rice bran/kg; fish meal (60 g/kg) and a phytase (+,-) were added to the diets (VP + AP). An additional 40 g soyabean meal/kg was added to the diet with rice bran (VP ++). Amino acid digestibility and mineral retention were measured in the lower ileum of ducklings killed at 23 d of age. Acid insoluble ash was used as an inert marker. Trypsin and amylase activities were also measured and weights of the pancreas and small intestine recorded at slaughter. 2. Addition of soyabean meal (VP ++) to the diet with rice bran improved growth rate and food intake compared to the diet without (VP) and gave the same food intake and growth rate as the comparable basal diet (VP) without rice bran. Fish meal improved growth rate on the diets without rice bran and improved food intake on this diet (VP + AP). Rice bran depressed growth rate and food conversion ratio (FCR); protein source affected growth rate, food intake and FCR; phytase increased food intake only. There were several interactions. 3. Determined total amino acid composition of the diets appeared to meet the essential amino acid requirements of ducklings. Rice bran depressed the ileal digestibility of virtually all amino acids and phytase had no direct effect, although there were interactions. Fish meal addition to diets with rice bran improved the apparent digestibility of several essential amino acids as well as that of dry matter and crude protein. 4. Ileal retention of some minerals and tibia ash content were reduced by rice bran. Fish meal and phytase inclusion increased P retention and ash in tibia. 5. Higher intestinal trypsin activity and increased pancreas size were seen in ducklings on diets with rice bran compared to those without. Intestinal amylase activity was reduced in ducklings given rice bran, probably because of its low starch content. 6. The stimulating effect of fish meal on duckling performance was probably caused in part by the improvement in the digestibility of some amino acids. The addition of small amounts of minerals in fish meal may have increased mineral retention. Phytase gave benefits anticipated from our previous work, but also improved lysine and threonine digestibility in diets containing vegetable protein only. [ABSTRACT FROM AUTHOR]