De novo epileptic confusional status in a patient with cobalamin deficiency

A 80-year-old man with cobalamin deficiency and no history of epilepsy developed a partial complex epileptic confusional status (ECS) unresponsive to acute i.v. diazepam. Brain CT scan and MRI investigation ruled out a focal cerebral lesion. Therapy with high doses (10,000 micrograms i.m. daily) of cobalamin alone was started, and the patient fully recovered in the following 72-hour. Control EEGs repeatedly performed days and weeks later showed progressive disappearance of the frontal interictal spiking, while the patient was on monotherapy with cobalamin (5,000 micrograms i.m. weekly). A month later the patient unfortunately discontinued replacement therapy and 13 weeks later he developed a fatal convulsive epileptic status. To our knowledge the association of ECS and cobalamin deficiency has not been previously reported.     

On the congruity between internal states and action.

Addresses the problem of incongruity between beliefs and behaviors. The theories of attribution and cognitive dissonance are briefly discussed, and their implications for behavior are noted. Issues that arise from obtaining behavioral effects in the absence of effects on beliefs and evaluations are delineated, and previous theories to account for these results are reviewed. Studies cited by R. E. Nisbett and T. D. Wilson (see record 1978-00295-001), by Wilson (1983), and by D. J. Bem (1972) are scrutinized with respect to the present author's critical and theoretical objectives, and a similar analysis is applied to more recent experiments on overjustification. It is concluded that (a) self-report effects are significant and congruent with behavior more frequently than previous reviewers have indicated, (b) behavior may interfere with inferential processes and thereby erase or prevent from occurring internal state changes that would otherwise be found, and (c) incongruity and other problems are symptomatic of there being no sophisticated theoretical account of how internal states mediate behavior. Basic facts about studies reviewed by Nisbett and Wilson are appended. (109 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Thymosin β4 is differentially expressed in the cerebrospinal fluid of Creutzfeldt‐Jakob disease patients: a MALDI‐TOF MS protein profiling study

MALDI‐TOF protein profiling analysis permits the detection of peptides and small proteins in complex protein mixtures with great accuracy. We applied this analysis to cerebrospinal fluid (CSF) from 15 patients affected by Creutzfeldt‐Jakob disease (CJD). We compared the levels of the normalized ion signals of 11 sporadic and 4 genetic CJD forms with those from ten healthy control subjects and eight non‐CJD relapsing‐remitting multiple sclerosis patients. In so doing, we detected 61 differentially expressed ion signals in CJD samples compared to controls. Among the 61 signals, 3 signals had significantly increased levels with high statistical significance (p <0.0001) and were located at 3238.3 m/z, 4963.7 m/z, and 8565.3 m/z. We characterized the 5.0 and 8.6 kDa proteins as thymosin β4 N‐acetylated and free ubiquitin, respectively, while the 3.2‐kDa peptide remained uncharacterized. Although elevated ubiquitin levels have previously been described in CJD, we have demonstrated for the first time the involvement of thymosin β4 in a neurodegenerative disease. To support the validity of thymosin β4 levels obtained by MALDI‐TOF analysis, an independent enzyme immunoassay analysis was performed. Moreover, a validation cohort consisting of CSF from three CJD patients, five healthy subjects, and six non‐CJD relapsing‐remitting multiple sclerosis patients was analyzed in a similar way, yielding superimposable results. We propose that thymosin β4 is a potential new candidate marker for the ante mortem diagnosis of CJD disease.     

A query expansion and user profile enrichment approach to improve the performance of recommender systems operating on a folksonomy

In this paper we propose a query expansion and user profile enrichment approach to improve the performance of recommender systems operating on a folksonomy, storing and classifying the tags used to label a set of available resources. Our approach builds and maintains a profile for each user. When he submits a query (consisting of a set of tags) on this folksonomy to retrieve a set of resources of his interest, it automatically finds further “authoritative” tags to enrich his query and proposes them to him. All “authoritative” tags considered interesting by the user are exploited to refine his query and, along with those tags directly specified by him, are stored in his profile in such a way to enrich it. The expansion of user queries and the enrichment of user profiles allow any content-based recommender system operating on the folksonomy to retrieve and suggest a high number of resources matching with user needs and desires. Moreover, enriched user profiles can guide any collaborative filtering recommender system to proactively discover and suggest to a user many resources relevant to him, even if he has not explicitly searched for them.     

Integration of XML Schemas at various “severity” levels

This paper presents a novel approach for the integration of a set of eXtensible Markup Language (XML) Schemas. The proposed approach is specialized for XML, almost automatic, semantic and “light”. Further, an original peculiarity is that it is parametric w.r.t. a “severity” level against which the integration task is performed. The paper describes the approach in all details, illustrates various theoretical results, presents the experiments we have performed for testing it and, finally, compares it with various related approaches already proposed in the literature.     

Genetic characterization of HHV-8/KSHV-positive primary effusion lymphoma reveals frequent mutations of BCL6: implications for disease pathogenesis and histogenesis

A new type of antimicrobial peptide, snakin-1 (SN1), has been isolated from potato tubers and found to be active, at concentrations < 10 microM, against bacterial and fungal pathogens from potato and other plant species. The action of SN1 and potato defensin PTH1 was synergistic against the bacterium Clavibacter michiganensis subsp. sepedonicus and additive against the fungus Botrytis cinerea. Snakin-1 causes aggregation of both gram-positive and gram-negative bacteria. The peptide has 63 amino acid residues (M(r) 6,922), 12 of which are cysteines, and is unrelated to any previously isolated protein, although it is homologous to amino acid sequences deduced from cloned cDNAs that encode gibberellin-inducible mRNAs and has some sequence motifs in common with kistrin and other hemotoxic snake venoms. A degenerate oligonucleotide probe based on the internal sequence CCEECKC has been used to clone an SN1 cDNA. With the cDNA used as probe, one copy of the StSN1 gene per haploid genome has been estimated and expression of the gene has been detected in tubers, stems, axillary buds, and young floral buds. Expression levels in petals and carpels from fully developed flowers were much higher than in sepals and stamens. The expression pattern of gene StSN1 suggests that protein SN1 may be a component of constitutive defense barriers, especially those of storage and reproductive plant organs.     

Negative myoclonic status due to antiepileptic drug tapering: report of three cases

PURPOSE: Epileptic negative myoclonus (ENM) has been increasingly recognized in different epilepsies, but the reasons for its appearance and prognosis remain uncertain. We report 3 patients who developed de novo, almost continuous ENM, triggered by antiepileptic drug (AED) tapering, that resolved with treatment. METHODS: Three patients aged 16, 19, and 65 years with a 13- to 36-year history of partial epilepsy were receiving a therapeutic dosage of carbamazepine or phenobarbital plus either clobazam (CLB) or valproate (VPA). None had previously had ENM. Forty-eight to 72 h after CLB or VPA withdrawal, the habitual seizures recurred. The patient also began to report repetitive postural lapses of one or more limbs that interfered with eating or writing. At this time, each patient underwent polygraphy with simultaneous surface electromyography (EMG) of deltoid, biceps, and triceps muscles and of the wrist extensor and flexor bilaterally. RESULTS: In all patients, EEGs demonstrated almost continuous epileptiform discharges whose spatial distribution was similar to that observed before ENM appearance. Polygraphic recordings showed repetitive loss of postural EMG activity in one or more limbs, 100-400 ms in duration, which occurred in conjunction with the spike-waves. One milligram of clonazepam intravenously always terminated ENM status, which has not recurred in the ensuing 9-36 months. CONCLUSIONS: ENM may emerge as a new type of seizure due to tapering of AED therapy. This effect is possibly related to the great activation of epileptiform activity with consequent interference with cortical activity.     

MRI of skeletal muscles in autosomal recessive hereditary motor and sensory neuropathy with focally folded myelin sheaths

A 33-day-old male infant who developed central diabetes insipitus as a complication of congenital toxoplasmosis is presented. He had polyuria and hypernatremia on admission and responded to Intranasal desmopressin acetate with the normalization of above mentioned findings. Computed tomographic (CT) scan of the brain showed obstructive hydrocephaly with periventricular and right basal ganglion calcification. CT scan of the pituitary gland, thyroid function tests, and serum cortisol levels were all normal. This is the first report of isolated diabetes insipitus with congenital toxoplasmosis in literature and central diabetes insipitus should be remembered if polyuria and hypernatremia develops in a patient with congenital toxoplasmosis.     

Causal versus diagnostic contingencies: On self-deception and on the voter's illusion.

Two experiments tested the notion that people select actions that are diagnostic of favorable outcomes even though the actions do not cause those outcomes. In Exp I, 38 undergraduates immersed their forearm into a chest of circulating cold water before and after physical exercise. Depending on condition, Ss learned that a long life expectancy was associated with either increases or decreases in tolerance to cold water after the exercise. As predicted, Ss showed changes in tolerance on the 2nd trial in the direction correlated with a long, healthy life. In Exp II, 315 undergraduates encountered 1 of 2 theories about the sort of voters who determine the margin of victory in an election. Only one of the theories would enable voting Ss to imagine that they could induce other like-minded persons to vote. As predicted, more Ss indicated that they would vote given that theory than given a theory in which the S's vote would not be diagnostic of the electoral outcome, although the causal impact of the S's vote was the same under both theories. (14 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)