Marchiafava-Bignami Disease

Marchiafava-Bignami disease is a rare demyelinating disease involving the corpus callosum and other central white matter tracts. In the patient described here, the disease produced extensive demyelination of the corpus callosum and deep cerebral white matter. This widespread demyelination, confirmed pathologically, was associated with a fulminant fatal course. The magnetic resonance imaging appearance is quite suggestive of Marchiafava-Bignami disease and plays an important role in the premortem diagnosis.     

Pulmonary mucinous cystic tumors of borderline malignancy

Mucus-filled cystic tumors of low or borderline malignant potential, well recognized in ovary and appendix, have received little attention in the lung. We present data on 11 patients, all of whom had solitary pulmonary nodules resected in which mucus was the major histologic component. Prognosis appears good; no patient had developed local recurrence or metastatic spread of tumor (follow-up, 1 to 9.5 years; mean, 4.7 years). Columnar mucus-producing cells lined the cysts in all cases, with cytologic and architectural atypia varying from minimal to microscopic foci of carcinoma; paucicellular mucus dissection into surrounding lung analogous to pseudomyxoma peritonei was seen in seven cases. The histologic and clinical findings are consistent with a mucinous cystic tumor of low or borderline malignant potential.     

Mutations Affecting the Rossman Fold of Isoleucyl-tRNA Synthetase Are Correlated with Low-Level Mupirocin Resistance in Staphylococcus aureus

The isoleucyl-tRNA synthetase (ileS) gene was sequenced in toto from 9 and in part from 31 Staphylococcus aureus strains with various degrees of susceptibility to mupirocin. All strains for which the mupirocin MIC was greater than 8 microg/ml contained point mutations affecting the Rossman fold via Val-to-Phe changes at either residue 588 (V588F) or residue 631 (V631F). The importance of the V588F mutation was confirmed by an allele-specific PCR survey of 32 additional strains. Additional mutations of uncertain significance were found in residues clustered on the surface of the IleS protein.     

The value of ventilation scintigraphy after single lung transplantation.

BACKGROUND: A decrease in forced expiratory volume in 1 second (FEV(1)) as a diagnostic criterion for bronchiolitis obliterans syndrome (BOS) after single lung transplantation may be influenced significantly by the presence of the native lung. To quantify and to discriminate between the relative contribution of graft and native lung to the FEV(1), we retrospectively investigated the diagnostic value of combined FEV(1) measurements and ventilation scintigraphy in pulmonary dysfunction after single lung transplantation in 11 recipients with pulmonary vascular disease, 3 with obstructive lung disease, and 3 with restrictive lung disease. METHODS: We assessed function of the native lung and the graft, and subsequently calculated an adjusted grading of BOS by correcting routine FEV(1) measurements using linear interpolation of bi-annual lung ventilation scans. RESULTS: The contribution of the native lung to the total FEV(1) was slight (median, 9%) in recipients with obstructive disease compared with recipients with vascular (38%) or restrictive lung diseases (27%). Adjusted BOS grading was not useful in patients with obstructive disease. In the other patient groups, the onset of adjusted BOS Grade 1 and standard BOS Grade 1 was at a median of 220 days (range, 127-1146 days) and 836 days (184-3065 days), respectively. CONCLUSION: Ventilation scintigraphy is a useful adjunct in the (early) diagnosis of BOS in recipients of single lung transplants who have vascular and restrictive lung diseases.     

Opioid binding in DYT1 primary torsion dystonia: an 11C-diprenorphine PET study.

The opioid transmitters enkephalin and dynorphin are known to regulate pallidal output and consequently cortical excitability. Indeed, abnormal basal ganglia opioid transmission has been reported in several involuntary movement disorders, including levodopa-induced dyskinesias in Parkinson's disease (PD), tardive dyskinesias/dystonia, Huntington's disease, and Tourette's syndrome. Moreover, a previous 11C-diprenorphine PET study investigating levodopa-induced dyskinesias found reduced opioid receptor availability in PD with but not without dyskinesias. We wished to investigate if a similar alteration in basal ganglia opioid binding was present in DYT1 primary torsion dystonia (PTD). Regional cerebral 11C-diprenorphine binding was investigated in 7 manifesting carriers of the DYT1 gene and 15 age-matched normal controls using a region-of-interest (ROI) approach and statistical parametric mapping (SPM). No difference in regional mean 11C-diprenorphine binding was found between DYT1-PTD and controls, and no correlation between the severity of dystonia and opioid binding was seen. We conclude that aberrant opioid transmission is unlikely to be present in DYT1-PTD and altered opioid transmission is not a common mechanism underlying all disorders of involuntary movement.     

Non-Cartesian data reconstruction using GRAPPA operator gridding (GROG).

A novel approach that uses the concepts of parallel imaging to grid data sampled along a non-Cartesian trajectory using GRAPPA operator gridding (GROG) is described. GROG shifts any acquired data point to its nearest Cartesian location, thereby converting non-Cartesian to Cartesian data. Unlike other parallel imaging methods, GROG synthesizes the net weight for a shift in any direction from a single basis set of weights along the logical k-space directions. Given the vastly reduced size of the basis set, GROG calibration and reconstruction requires fewer operations and less calibration data than other parallel imaging methods for gridding. Instead of calculating and applying a density compensation function (DCF), GROG requires only local averaging, as the reconstructed points fall upon the Cartesian grid. Simulations are performed to demonstrate that the root mean square error (RMSE) values of images gridded with GROG are similar to those for images gridded using the gold-standard convolution gridding. Finally, GROG is compared to the convolution gridding technique using data sampled along radial, spiral, rosette, and BLADE (a.k.a. periodically rotated overlapping parallel lines with enhanced reconstruction [PROPELLER]) trajectories.     

Predictive power of various models for longitudinal attachment level change

Abstract Several statistical models that have been suggested in the periodontal literature for describing longitudinal attachment level changes, such as the gradual loss, single-burst, multiple-burst, and random walk models as well as other models introduced in this paper are compared by their power to predict future attachment loss. The data used in this analysis is from 1061 sites of 8 subjects, with moderate to severe periodontal disease, monitored monthly for about a year. This study found that none of the suggested models could significantly outperform the naive mean predictor, which predicts the future attachment level from the past mean. It was also found that no single model, such as the burst, gradual, or random walk, together with measurement error can fully explain the variation in the data. These results indicate that in the course of one year, the attachment level change may not follow the same model. Consequently, a model that fits well to past data cannot be accurately extended to the future.     

Ainhum (dactylolysis spontanea): A Radiological survey of 6000 patients

Dactylolysis spontanea is an idiopathic condition affecting the fifth toe, and sometimes other toes, that is frequently bilateral, with lesions in different stages. Between 1977 and 1999, a total 6000 radiographic studies of the feet were reviewed in a mainly African American population in Chicago, Illinois. After an initial screening based on the Cole criteria, 581 patients were selected and re-examined, and amplification techniques were performed. After reviewing the complementary exams, 102 patients were diagnosed with dactylolysis spontanea or ainhum. Soft-tissue constriction was the most frequently presented radiological sign on the initial screening. Kurtosis at the digit plantar fold and marked rotation of the fifth toe were normal findings in asymptomatic patients. Demographics, comorbidities, and radiological findings were analyzed in the selected population. Associated diseases occurring in these patients appeared to have no specific etiologic correlation with ainhum. African Americans and the dark-skinned population are affected exclusively by this condition, presumably due to the fibrogenic tendency of these individuals. Early diagnosis and accurate staging of ainhum are facilitated by radiological examination of the feet. The findings suggest that this condition is underdiagnosed and overlooked because its low prevalence and variable clinical presentations that might mimic more common etiologies, including localized trauma.     

Dentists evaluate their patients: An empirical investigation of preferences

Dental treatment is a dyadic encounter. Yet research on the dentist-patient dyad has tended to focus on patient perceptions of dentist, to the neglect of dentist perceptions of patient. Previous theoretic and qualitative work on caregiver perceptions of patients suggested three dimensions of evaluation. Dentists (N=618) rated their patients on items taken from two prior studies. Dentists' responses were subjected to confirmatory factor analysis. The analysis substantiated the existence of three evaluative dimensions: compliance, tractability, and likability. Further analysis showed that the three dimensions formed a Guttman simplex, revealing a second-order factor of selectivity and allowing a classification of dentists on the basis of patient selectivity.This study was funded by a grant from the University of Connecticut Research Foundation.     

Choreic syndrome and coeliac disease: a hitherto unrecognised association.

Coeliac disease has been associated with a variety of neurological conditions, most frequently cerebellar ataxia and peripheral neuropathy. To date, chorea has not been associated with coeliac disease. We present the case histories of 4 individuals with coeliac disease and chorea (4 women, average age of onset of chorea 61 years). Unexpectedly, most of these patients showed a notable improvement in their motor symptoms after the introduction of a gluten-free diet.