Cutaneous ultrastructural diagnosis of ceroid-lipofuscinosis

A skin biopsy from a 6-year-old girl was examined by electron microscopy and the diagnosis of ceroid-lipofuscinosis (CL) was confirmed. This has not been reported in the dermatology literature, because of the absence of specific skin manifestations. However, as we have shown, various cells of skin, including fibroblasts, Schwann cells, eccrine serous cells, endothelial cells and smooth muscle cells, accumulated characteristic substances to produce the finger print pattern (FPP) or the curvilinear profile (CLP) which are thought to be diagnostic for CL. Cutaneous ultrastructural research in such cases is important as asymptornatic skin disorders may exist in other congenital storage diseases.     

Hypersensitivity pneumonitis with antibodies to Cryptococcus neoformans

Clinical and immunological studies were made in forty-two patients diagnosed as suffering from hypersensitivity pneumonitis at Osaka Prefectural Habikino Hospital between 1973 and 1977. All the sera from forty-one patients tested had high litres of antibody against Cryptococcus neoformans in indirect fluorescent antibody tests, and twelve also had precipitins against Cryptococcus neoformans polysaccharide. Only about 10% of control sera from patients with otherlung diseases had low titresof antibody against Cryptococcus neoformans. Antibody against Cryptococcus neoformans was also found frequently in the sera of asymptomatic members of the families of the patients. A possible relationship of Cryptococcus neoformans to hypersensitivity pneumonitis is suggested.     

Lateralized Appearance of Fourteen and Six per Second Positive Spikes

In this study of the 15 cases who showed persistent lateralized appearance of 14 and 6 per second positive spikes, it was found that positive spikes in 14 cases appeared on the side opposite to the hemisphere, where organic focal EEG signs were found; in the 4 cases with hemiplegia out of these, it was found that positive spikes appeared at the hemiplegic side. Thus, it has been emphasized that when a persistent lateralized appearance of positive spikes is confirmed in the successive EEGs, the absence of positive spikes in one hemisphere is as diagnostically significant as a focal sign as other EEG abnormalities are.