We have studied the brains of 10 patients with clinically and pathologically defined Huntington's disease and graded the degree of striatal pathology according to the Vonsattel grading system. Sections from nine cerebral cortical areas (Brodmann areas 8, 10, 24, 33, 28, 38, 7, 39, 18), the cerebellum, hypothalamus, medulla and caudate nucleus were stained with antibodies to ubiquitin and ubiquitin C-terminal hydrolase (PGP 9.5). Dystrophic neurites, immunoreactive with ubiquitin and PGP 9.5 were detected in all cortical areas, in layers 3, 5 and 6, of all brains studied. No dystrophic neurites were found in subcortical areas or cerebellum. Sections from cortical areas 8 and 24 from the two brains with the most and least ubiquitin-immunoreactive neurites were stained with antibodies to beta-amyloid precursor protein, tau, glial fibrillary acidic protein, neurofilament protein, alpha B crystallin, GABA, cholecystokinin and somatostatin. The dystrophic neurites were found to also react with beta-amyloid precursor protein. Electron microscopy showed the abnormal neurites to contain granulofilamentous material. Granular deposits with a diameter of 40-100 nm were interspersed between randomly orientated 'fuzzy' or coated, straight or slightly curved filaments measuring 10-15 nm in diameter. These structures have not been seen in control brain and differ from age-related neuritic degeneration and neurites associated with amyloid. Immunohistochemically these structures most resemble CA 2/3 neurites seen in Lewy body disease, and, ultrastructurally, the intraneuronal filamentous inclusions in motor neuron disease.(ABSTRACT TRUNCATED AT 250 WORDS)
PurposeThe purpose of this research is to report the results of research that was carried out to evaluate the effectiveness of a number of web sites selling chocolate.Design/methodology/approachThe research was founded on a series of criteria for evaluating such web sites, based upon a review of the literature. A series of criteria were developed and a scoring system associated with them was generated. Following a pilot test, a panel of eight volunteers evaluated ten chocolate‐related web sites which were carefully chosen for their range of characteristics.FindingsAspects evaluated included presentation, content, accessibility, language, navigation and structure, transaction page, security, privacy and authority, and marketing factors. The resulting scores were then analysed. Using two different methods of analysis, it was found that the best web site was www.danschocolates.com, but that there was significant inter‐scorer discrepancy in some of the scores assigned.Research limitations/implicationsOnly a small number of sites were evaluated, using a relatively small number of evaluators.Practical implicationsConclusions are drawn regarding the most important factors that web designers should take into account when developing a site for selling chocolate or similar products. Comments are also made on the need to involve real users when developing criteria for the evaluation of web sites.Originality/valueThe paper provides a possible general methodology for evaluating web sites.
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