A case of frontotemporal dementia with amyotrophic lateral sclerosis presenting with pathological gambling.

BACKGROUND: Pathological gambling (PG), which is characterized by consistent, repetitive gambling and unsuccessful quitting attempts, is classified as an impulse control disorder. PG has also been reported in patients with Parkinson's disease, frontotemporal dementia, and amyotrophic lateral sclerosis. CASE REPORT: A 53-year-old male visited the outpatient clinic due to excessive gambling and personality changes. Based on electrophysiological findings and neuropsychiatric assessment, he was diagnosed as frontotemporal dementia-amyotrophic lateral sclerosis. CONCLUSIONS: This case report underlines that PG can also be seen in patients with neurological disorders involving the orbitofrontal cortex.

Head and neck cancer patients' perspective of carer burden.

There is a growing appreciation of the important role that carers have in supporting patients following treatment for head and neck cancer. We asked patients about the role fulfilled by their carer(s) and the support they give, and for their thoughts on the burden this placed on the carers. We did a cross-sectional survey of 751 patients with head and neck cancer who were alive and disease-free using two questionnaires: one combined study-specific questions about carers with questions from Khafif et al., and the other was the University of Washington Quality of Life questionnaire version 4 (UW-QoL). There were 386 replies. Nearly half (46%, 162/354) had carers who were mainly family members. Patients identified their main roles as providing emotional support (75%), taking them to healthcare appointments (67%), cleaning the home (62%), and shopping for food (59%). Around a third felt that their care was a considerable burden, and a similar proportion felt that it was very hard for their carers. Patients over 65 years of age were the most likely to need a considerable amount of care and support, and those with low socio-emotional UW-QoL composite scores were most likely to need a considerable amount of care and support, to regard the burden on their carers as considerable, and to think that it was very hard for their carers to take care of them. The study emphasises the necessity to take account of the needs of carers. More research is required on the patient-carer relationship and how best to support it.

Development of the International Classification of Functioning, Disability and Health as a brief head and neck cancer patient questionnaire.

WHO has adopted the International Classification of Functioning, Disability and Health (ICF) to assess functioning and disability. A Brief ICF Core Set for head and neck cancer comprises 19 items. This study developed a patient self-completed questionnaire from the items of the brief core set (BCSQ-H&N), compared the BCSQ-H&N questionnaire with the University of Washington v.4 (UW-QOLv4) and compared the BCSQ-H&N results with a clinician-rated evaluation. UW-QOL v4 and BCSQ-H&N were sent to 751 disease-free head and neck cancer patients in April 2008. 376 patients responded to the questionnaire and 25 were interviewed. The percentage reporting significant problems in BCSQ-H&N items ranged between 11% and 43%. The type of problem varied with tumour site. Patients with smaller tumours and patients without radiotherapy reported better outcomes. The BCSQ-H&N correlated well with appropriate items in the UW-QOLv4 especially for functional outcome. There were systematic differences between observer-rated scores and patient self-completed questionnaire responses. Patients suggested additional items for inclusion, namely taste, jaw opening, articulation function, structure of shoulder region, loss of function at the free flap donor site, and intimate relationships. Further validation is required but BCSQ-H&N shows promise as an outcome measure for global use.

Antifungal activity of a novel endochitinase gene (chit36) from Trichoderma harzianum Rifai TM.

A novel 36-kDa endochitinase named chit36 has been isolated and characterized from Trichoderma harzianum Rifai TM. Partial amino acid sequences from the purified protein were used to clone the fungal cDNA, based on polymerase chain reaction with degenerate primers. The complete open reading frame encodes a 344-amino acid protein which shows 84% similarity to a putative chitinase from Streptomyces coelicolor. Chit36 was overexpressed under the pki1 constitutive promoter from Trichoderma reesei via biolistic transformation of T. harzianum TM. Stable transformants showed expression and endochitinase activity of chit36 in glucose-rich medium. Culture filtrates containing secreted CHIT36 as the sole chitinolytic enzyme completely inhibited the germination of Botrytis cinerea conidia. Growth of Fusarium oxysporum f. sp. melonis and Sclerotium rolfsii were significantly inhibited on agar plates on which the Trichoderma transformants had previously been grown.

Characterization of Arabidopsis acid phosphatase promoter and regulation of acid phosphatase expression.

The expression and secretion of acid phosphatase (APase) was investigated in Indian mustard (Brassica juncea L. Czern.) plants using sensitive in vitro and activity gel assays. Phosphorus (P) starvation induced two APases in Indian mustard roots, only one of which was secreted. Northern-blot analysis indicated transcriptional regulation of APase expression. Polymerase chain reaction and Southern-blot analyses revealed two APase homologs in Indian mustard, whereas in Arabidopsis, only one APase homolog was detected. The Arabidopsis APase promoter region was cloned and fused to the beta-glucuronidase (GUS) and green fluorescent protein (GFP) reporter genes. GUS expression was first evident in leaves of the P-starved Arabidopsis plants. In P-starved roots, the expression of GUS initiated in lateral root meristems followed by generalized expression throughout the root. GUS expression diminished with the addition of P to the medium. Expression of GFP in P-starved roots also initiated in the lateral root meristems and the recombinant GFP with the APase signal peptide was secreted by the roots into the medium. The APase promoter was specifically activated by low P levels. The removal of other essential elements or the addition of salicylic or jasmonic acids, known inducers of gene expression, did not activate the APase promoter. This novel APase promoter may be used as a plant-inducible gene expression system for the production of recombinant proteins and as a tool to study P metabolism in plants.

Role of the Trichoderma harzianum endochitinase gene, ech42, in mycoparasitism.

The role of the Trichoderma harzianum endochitinase (Ech42) in mycoparasitism was studied by genetically manipulating the gene that encodes Ech42, ech42. We constructed several transgenic T. harzianum strains carrying multiple copies of ech42 and the corresponding gene disruptants. The level of extracellular endochitinase activity when T. harzianum was grown under inducing conditions increased up to 42-fold in multicopy strains as compared with the wild type, whereas gene disruptants exhibited practically no activity. The densities of chitin labeling of Rhizoctonia solani cell walls, after interactions with gene disruptants were not statistically significantly different than the density of chitin labeling after interactions with the wild type. Finally, no major differences in the efficacies of the strains generated as biocontrol agents against R. solani or Sclerotium rolfsii were observed in greenhouse experiments.

Chitinolytic activity in Chromobacterium violaceum: substrate analysis and regulation by quorum sensing.

Quorum sensing control mediated by N-acyl homoserine lactone (AHL) signaling molecules has been established as a key feature of the regulation of exoenzyme production in many gram-negative bacteria. In Chromobacterium violaceum ATCC 31532 a number of phenotypic characteristics, including production of the purple pigment violacein, hydrogen cyanide, antibiotics, and exoproteases are known to be regulated by the endogenous AHL N-hexanoyl-L-homoserine lactone (HHL). In this study we show that C. violaceum produces a set of chitinolytic enzymes whose production is regulated by HHL. The chitinolytic activity was induced in strains grown in the presence of chitin as the sole carbon source and quantitated in the secreted proteins by using p-nitrophenol analogs of disaccharide, trisaccharide, and tetrasaccharide oligomers of N-acetylglucosamine. By using 4-methylumbelliferyl analogs of the same oligomers of N-acetylglucosamine as substrates for proteins separated and renatured by sodium dodecyl sulfate-polyacrylamide gel electrophoresis, at least six enzymes were detected: a chitobiase with high specificity to a dimeric substrate of 87 kDa, two N-acetylglucosaminidases with apparent molecular masses of 162 and 133 kDa, two endochitinases of 108 and 67 kDa, and a chitobiosidase of 56 kDa. In addition, two unidentified bands of >205 kDa were found where a tetrameric chitin derivative was used as a substrate. A pleiotropic mini-Tn5 mutant of C. violaceum (CV026) that is defective in HHL production and other quorum-sensing-regulated factors was also found to be completely deficient in chitinolytic activity. Growth of this mutant on minimal medium with chitin supplemented with culture supernatant from the C. violaceum wild-type strain or 10 microM synthetic HHL restored chitinase production to the level shown by the parental strain. These results constitute the most complete evidence so far for regulation of chitinolytic activity by AHL signaling in a gram-negative bacterium.

Molecular cloning, structural analysis, and expression in Escherichia coli of a chitinase gene from Enterobacter agglomerans.

The gene chiA, which codes for endochitinase, was cloned from a soilborne Enterobacter agglomerans. Its complete sequence was determined, and the deduced amino acid sequence of the enzyme designated Chia_Entag yielded an open reading frame coding for 562 amino acids of a 61-kDa precursor protein with a putative leader peptide at its N terminus. The nucleotide and polypeptide sequences of Chia_Entag showed 86.8 and 87.7% identity with the corresponding gene and enzyme, Chia_Serma, of Serratia marcescens, respectively. Homology modeling of Chia_Entag's three-dimensional structure demonstrated that most amino acid substitutions are at solvent-accessible sites. Escherichia coli JM109 carrying the E. agglomerans chiA gene produced and secreted Chia_Entag. The antifungal activity of the secreted endochitinase was demonstrated in vitro by inhibition of Fusarium oxysporum spore germination. The transformed strain inhibited Rhizoctonia solani growth on plates and the root rot disease caused by this fungus in cotton seedlings under greenhouse conditions.

Chitinolytic Enterobacter agglomerans Antagonistic to Fungal Plant Pathogens.

Three Enterobacter agglomerans strains which produce and excrete proteins with chitinolytic activity were found while screening soil-borne bacteria antagonistic to fungal plant pathogens. The chitinolytic activity was induced when the strains were grown in the presence of colloidal chitin as the sole carbon source. It was quantitated by using assays with chromogenic p-nitrophenyl analogs of disaccharide, trisaccharide, and tetrasaccharide derivatives of N-acetylglucosamine. A set of three fluorescent substrates with a 4-methylumbelliferyl group linked by (beta)-1,4 linkage to N-acetylglucosamine mono- or oligosaccharides were used to identify the chitinolytic activities of proteins which had been renatured following their separation by electrophoresis. This study provides the most complete evidence for the presence of a complex of chitinolytic enzymes in Enterobacter strains. Four enzymes were detected: two N-acetyl-(beta)-d-glucosaminidases of 89 and 67 kDa, an endochitinase with an apparent molecular mass of 59 kDa, and a chitobiosidase of 50 kDa. The biocontrol ability of the chitinolytic strains was demonstrated under greenhouse conditions. The bacteria decreased the incidence of disease caused by Rhizoctonia solani in cotton by 64 to 86%. Two Tn5 mutants of one of the isolates, which were deficient in chitinolytic activity, were unable to protect plants against the disease.

PERSONAL STYLES OF REACTION TO THE FRUSTRATION OF OTHERS AS PORTRAYED BY MULTIDIMENSIONAL SCALOGRAM ANALYSIS.

A questionnaire for the assessment of reactions to the frustration of others was constructed and administered to 56 Ss. This questionnaire concentrated on the reported intensity of eight reactions, namely: Pity, Attempt to Help, Reproach, Withdrawal, Disgust, Mockery, Anger, and Malicious Joy. Each one of the 56 Ss had a total profile of the eight reactions, and these profiles were analyzed by Multidimensional Scalogram Analysis (MSA). MSA led to the conclusion that the personal styles of reaction could be characterized as a Cartesian product of two facets: a ) Activity-Passivity of the reaction, b) High vs. Low Intensity of the reaction. A third facet was hinted, which relates to the time-sequence of the reactions.