Fine-scale mapping of a novel dementia gene, PLOSL, by linkage disequilibrium.

Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL; MIM 221770) is a rare hereditary cause of presenile dementia with autosomal recessive inheritance. Its unique feature is the cystic bone lesions that accompany the dementia. About 160 cases have been reported to date, mostly in Finland and Japan.

Assignment of the locus for PLO-SL, a frontal-lobe dementia with bone cysts, to 19q13.

PLO-SL (polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy) is a recessively inherited disorder characterized by systemic bone cysts and progressive presenile frontal-lobe dementia, resulting in death at <50 years of age. Since the 1960s, approximately 160 cases have been reported, mainly in Japan and Finland. The pathogenesis of the disease is unknown.

Vascular changes and blood-brain barrier damage in the pathogenesis of polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (membranous lipodystrophy).

The histopathological, immunohistochemical and electron microscopic findings in eight patients with polycystic lipomembranous osteodysplasia and sclerosing leukoencephalopathy (PLO-SL) are described. This autosomally recessively inherited disease is first manifested by multiple bone cysts, which are later followed around the age of 30 by severe neuropsychiatric syndrome. The pathogenesis of PLO-SL has not been established, and the search for the most suspected error in lipid metabolism has been unsuccessful.

Adenoma residua in colorectal cancer. A study of 34 colorectal cancers detected by an immunological occult blood test.

A retrospective histological study was made searching for adenoma remnants in 34 colorectal cancer specimens detected by the quantitative and human Hb-specific immunological FECA-EIA test for occult blood in the faeces. As a control group, 34 colorectal cancer preparations from routine hospital material were studied in a similar manner. A difference was found between these materials, 12 out of 34 study cancers showing adenoma residua compared with only two in the control group.

Decreased incidences of intestinal and diffuse types of gastric carcinoma in Finland during a 20-year period.

The incidence of gastric carcinoma (GCA) has decreased throughout the world. This decrease is attributed to a decline in incidence of the intestinal type of GCA (IGCA), whereas the diffuse (DGCA) type of GCA is considered to be endemic in nature and more stable in incidence. In the present study we have estimated how much the incidences of IGCA and DGCA have decreased in percentage in Finland from 1952-61 to 1972-81.

Further studies of intestinal heterotopia in urethral caruncle.

Colonic mucosa-like intestinal heterotopia in a urethral caruncle is reported in a 2 yr 7 mth old girl and three women; 55, 71 and 78 yrs old. The occurrence of the anomaly in a child supports the first two author's earlier suggestion that the condition is congenital in nature. In princip this could also be the case in post-menopausal women, although the possibility of metaplasia must also be considered.

Gastric dysplasia. Significance and pathologic criteria.

In view of uncertainty regarding the criteria and significance of gastric dysplasia as a precancerous lesion, members of the Pathology Panel of the International Study Group on Gastric Cancer (ISGGC) reviewed microslides of 93 gastric lesions showing varying degrees of mucosal abnormality, and reached the following consensus: (1) immature and proliferating gastric epithelium can be divided into two categories: hyperplastic and dysplastic; (2) the term dysplasia, especially of high-grade type, should be restricted to precancerous lesions, and hyperplasia is applied to regenerative changes; (3) regenerative hyperplasia may be simple or atypical, but dysplasia includes both moderate and severe abnormalities, since they often coexist and can not be sharply separated; and (4) occasionally the possibility of malignancy can not be excluded in a severely dysplastic epithelium; in such a case rebiopsy and diligent follow-up are necessary to establish the diagnosis. Criteria for diagnosing dysplasia and hyperplasia are presented and discussed. The opinions are offered as guidelines for establishing the diagnosis of gastric dysplasia and for prospective studies.

Canine gastric glycoprotein antigens in early carcinogenesis.

The effect of one single dose of N-methyl-N'-nitro-N-nitrosoguanidine (MNNG) on the antigenic structures of gastric juice glycoproteins, was studied in dogs. Antisera to glycoproteins of the fetal alimentary canal were raised. Histologic mucosal specimens and glycoprotein fractions of gastric juice which were taken from four dogs during a 15.

Radiologic bone changes of polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy.

More than 50 cases of polycystic lipomembranous osteodysplasia (PLO) with sclerosing leukoencephalopathy (SL) have been described in Finland, Sweden, Japan, and in the USA. Radiographic bone changes, including symmetrical cystic lesions in the small bones of the extremities and trabecular loss in the distal ends of the long tubular bones, represent primary abnormalities in the diagnosis of the disease. Neuropsychiatric symptoms, frontal syndrome, and pyramidal signs make the patients dangerous to themselves.

Clinicopathological study of gastric cancers and precancerous states detected by fetal sulfoglycoprotein antigen screening.

A clinicopathological analysis is presented of gastric cancer cases detected in a mass screening trial in Finland, using the oncofetal antigen, fetal sulfoglycoprotein antigen, as a marker. The survey covered a population of 53,020 between the ages of 40 and 70, the percentage of participation being 74.8%.

Ultrastructure of intestinal and diffuse type gastric carcinoma.

Samples from 56 resected stomachs, including 28 cases of intestinal and 14 of diffuse type gastric carcinoma, were studied by electron microscopy. The main ultrastructural features of intestinal and diffuse type gastric carcinomas were described and compared to the ultrastructure of normal gastric mucosal cells and cells occurring in intestinal metaplasia of the gastric mucosa. Many similarities were demonstrated between intestinal metaplasia and intestinal type carcinomas.

Intracellular cysts in gastric carcinoma.

Intracellular cysts were often found in the tumour cells in gastric carcinomas of intestinal as well as diffuse type in specimens from stomachs resected for gastric cancer. In the light microscope, the cysts appeared usually as solitary cytoplasmic vacuoles. In the electron microscope, the intracellular cysts were seen as round cavities, often containing homogenous or granular mucous material which stained with the periodic acid-silver methenamine (PASM)-method.

Subclinical elastofibromas in the scapular region in an autopsy series.

In a series of 235 autopsies, changes in the subcapscular thoracic fascia similar to elastofibroma dorsi (Järvi & Saxen 1959, -et al. 1969) were found in 39 cases, all at least 58 years old. In people over 55 years, the frequency was 24.