Diffuse cerebral white matter T2-weighted hyperintensity: a new finding of general paresis.

General paresis (parenchymatous neurosyphilis) is a rare disease, and in recent years the number of papers published on the magnetic resonance imaging findings has been limited. The findings are as follows: cerebral atrophy; mesiotemporal T2 hyperintensity; ventriculomegaly; pathological T2 hypointensity of the globus pallidus, putamen, the head of the caudate nucleus and thalamus. We present a new finding, diffuse cerebral white matter T2 hyperintensity, observed in a patient with general paresis with a 5-year history of progressive dementia.

Polymorphisms of two fucosyltransferase genes (Lewis and Secretor genes) involving type I Lewis antigens are associated with the presence of anti-Helicobacter pylori IgG antibody.

Helicobacter pylori attach to the gastric mucosa with adhesin, which binds to Lewis b (Le(b)) or H type I carbohydrate structures. The Secretor (Se) gene and Lewis (Le) gene are involved in type I Le antigen synthesis. The present study was performed to investigate the possibility that Se and Le gene polymorphisms alter the risk of H. pylori infection. Two hundred thirty-nine participants were genotyped for Se and Le and tested for the presence of anti-H. pylori IgG antibodies. Using the normal gastric mucosa from 60 gastric cancer patients, we assessed immunohistochemically whether type I Le antigen expression depended on the Se and Le genotypes. The H. pylori infection rate was positively associated with the number of Se alleles (se/se group, 45.1%; Se/se group, 64.6%; and Se/Se group, 73.3%) and negatively associated with the number of Le alleles (le/le group, 76.4%; Le/le group, 68.3%; and Le/Le group, 55.6%). When the subjects were classified into three groups [low risk, (se/se, Le/Le) genotype; high risk, (Se/Se, le/le), (Se/Se, Le/le), and (Se/se, le/le) genotypes; moderate risk, other than low- or high-risk group], the odds ratio relative to the low-risk group was 3.30 (95% confidence interval, 1.40-7.78) for the moderate-risk group and 10.33 (95% confidence interval, 3.16-33.8) for the high-risk group. Immunohistochemical analysis supported the finding that Se and Le genotypes affected the expression of H. pylori adhesin ligands. We conclude that Se and Le genotypes affect susceptibility to H. pylori infection.

Giant neuroendocrine (Merkel cell) carcinoma of the skin.

An 82-year-old woman had a dark red to purple tumor on the left buttock that had gradually enlarged during the last 5 years. Although routine histologic examination was not sufficient for diagnosis, neuroendocrine carcinoma was diagnosed by immunohistochemical and ultrastructural studies. Immunohistochemical-positive reactions to neurofilament, cytokeratin, neuron-specific enolase, and epithelial membrane antigen were noted. Electron microscopically, membrane-bound, dense core granules that yielded a positive uranaffin reaction and intermediate filaments in the perinuclear area were observed in the cytoplasm of most tumor cells. Desmosome-like structure between them was also found. Approximately 6 months after local excision, metastatic lesions developed in the regional lymph nodes and liver.

Linear epidermal nevus with acantholytic dyskeratosis in an infant.

A 7-month-old boy came to use with flat papules and small erosions on the extensor aspect of his left forearm and a linear arrangement of verrucous papules on the dorsum of his left hand. Histological examination revealed hyperkeratosis, parakeratosis, irregular acanthosis, and marked acantholysis from suprabasal through upper epidermis. This is the first reported case of linear epidermal nevus with acantholytic dyskeratosis in Japan.

Characterization of fibroblast-derived prolidase. The presence of two forms of prolidase.

Crude enzyme solutions of prolidase were extracted from cultured human skin fibroblasts derived from control and prolidase-deficient sisters. Two forms of prolidases (prolidase-I and II) were partially purified by high performance liquid chromatography equipped with an ion exchange column. On gel filtration, the relative molecular weights of prolidase-I and II were estimated to be MW = 105,000 and 151,000, respectively. The substrate specificity of partially purified prolidase-I and II in control fibroblasts was estimated against Gly-Pro, Ala-Pro, Met-Pro. Each form of prolidase differed in its substrate specificity. In prolidase-deficient sisters, the elder with typical clinical manifestations and the younger with only slight clinical manifestations, the activity of prolidase-I was absent. However, the activity of prolidase-II was sufficiently present in both sisters. The substrate specificity of prolidase-II in the patients was similar to that of control. No difference in substrate specificity was found between these two patients.