An unusual association of diffuse adenomyomatosis with dysplastic adenoma in chronic calculous cholecystitis: case presentation

<p>Abstract</p> <p>Background</p> <p>Gallbladder adenomyomatosis is an epithelial proliferation and hypertrophy of the muscularis mucosae of the gallbladder. Rokitansky-Aschoff sinuses are a characteristic of this condition. The segmental adenomyomatosis has a higher risk of developing into gallbladder carcinoma, especially in the fundal region of elderly patients.</p> <p>We report the case of a patient affected by chronic calculous cholecystitis with diffuse adenomyomatosis associated with dysplastic adenoma.</p> <p>Case presentation</p> <p>An 81-year-old woman presented at our hospital with a 1-year history of intermittent pain localized at the right upper abdominal quadrant, without diffusion to any other body part. On physical examination the abdomen was soft, not distended, and tender to palpation in the right upper quadrant. Murphy sign was negative. Laboratory tests were normal. The patient was scheduled for a laparoscopic cholecystectomy, and neither endoscopic ultrasonographic scan nor magnetic resonance imaging was performed. The operation, performed after obtaining informed consent, was uncomplicated and the intra-operative pathological examination showed no malignancy. The definitive pathological examination of the gallbladder showed: multiple stones of cholesterol origin; diffuse mucosal adenomyomatosis; and a 1.1 cm pedunculated mass localized at the fundus, whose surface was lumpy. This mass was diagnosed as an adenoma with multiple areas of severe dysplasia.</p> <p>Conclusions</p> <p>The adenoma of the gallbladder, together with the dysplasia, represents a biological carcinogenetic model. Carcinoma has rarely been reported in adenomyomatosis. Degenerative risk suggests surgery should be mandatory when there is a concomitant presence of large adenoma and adenomyomatosis.</p

The cerebral correlates of different types of perseveration in the Wisconsin Card Sorting Test

Objectives: To explore the neural substrates corresponding to the perseverative errors in the Wisconsin Card Sorting Test (WCST). Methods: The study examined the correlations between the WCST performances and the SPECT measurements of regional cerebral blood flow (rCBF) in subjects with neurodegenerative dementia. Negative non-linear correlations between the rCBF and the two different types of the perseverative errors ("stuck-in-set" and "recurrent" perseverative errors) were calculated on a voxel basis and volume-of-interest basis in the mixed groups of 72 elderly and dementia patients. Results: The stuck-in-set perseverative error was associated with the reduced rCBF in the rostrodorsal prefrontal cortex, whereas the recurrent perseverative error was related to the left parietal activity but not to the prefrontal activity. Conclusions: These findings augment evidence that the rostrodorsal prefrontal cortex crucially mediates attentional set shifting, and suggest that the stuck-in-set perseverative errors would be a true pathognomonic sign of frontal dysfunction. Moreover, this study shows that the recurrent perseverative errors may not be associated closely with the prefrontal function, suggesting that this error and the stuck-in-set error should be differentially estimated in the WCST

Neuroradiologic Features of CASK Mutations

SUMMARY: Mutations of the CASK gene are associated with X-linked mental retardation with microcephaly and disproportionate brain stem and cerebellar hypoplasia in females. The areas of the cerebrum, corpus callosum, pons, midbrain, and cerebellar vermis and hemisphere and a ratio of cerebrum/ corpus callosum areas were measured in 5 female patients with CASK mutations, 67 female controls, and 5 patients with pontine hypoplasia. MR imaging in patients with CASK mutations revealed a normal size of the corpus callosum and a low ratio of the cerebrum/corpus callosum with a reduced area of the cerebrum, pons, midbrain, and cerebellar vermis and hemispheres. The 5 patients with pontine hypoplasia showed thinning of the corpus callosum and a high ratio of the cerebrum/corpus callosum, irrespective of the size of the cerebrum. The normal size of the corpus callosum, which gives an impression of callosal thickening at first glance, may be an imaging clue to detect patients with CASK mutations. ABBREVIATIONS: CASK ϭ calcium/calmodulin-dependent serine protein kinase; CINAP ϭ CASK interacting nucleosome assembly protein; PEHO ϭ progressive encephalopathy, edema, hypsarrhythmia, and optic atrophy; TBR1 ϭT-brain-1; RELN ϭ reelin A dvances in MR imaging and molecular biology have revolutionized the analysis and understanding of malformations of the brain. A classification of malformations of the midbrain and hindbrain has been proposed on the basis of embryology and genetics, 1 dividing these disorders into 4 groups: 1) malformations secondary to early anteroposterior and dorsoventral patterning defects or to misspecification of midhindbrain germinal zones, 2) malformations associated with later generalized developmental disorders that significantly affect the brain stem and cerebellum, 3) localized brain malformations that significantly affect the brain stem and cerebellum, and 4) combined hypoplasia and atrophy in putative prenatal-onset degenerative disorders