A hereditary ceruloplasmin deficiency associated with severe iron deposition in visceral organ and brain tissues found on histopathological examination at autopsy is discussed. Three siblings of consanguineous Japanese parents were studied. Their clinical symptoms were progressive dementia, extrapyramidal disorders, cerebellar ataxia, and diabetes mellitus, all of which appeared when they were between 30 and 50 years old. All had serum ceruloplasmin deficiencies and increased serum ferritin concentrations. The dentate nucleus, thalamus, putamen, caudate nucleus, and liver of each one showed low signal intensities on T1- and T2-weighted magnetic resonance images. Examination of the central nervous system revealed severe destruction of the basal ganglia and dentate nucleus, with considerable iron deposition in neuronal and glial cells, whereas the cerebral cortex showed mild iron deposition in glial cells without neuronal involvement. An electron microscopic study with energy-dispersive x-ray analysis showed iron depositions in the hepatocytes, of both the neural and glial cells of the brain. We consider this a new disease entity because of the primary ceruloplasmin deficiency.

The conformation of a single poly(methyl methacrylate) (PMMA) chain was studied by scanning near-field optical microscopy (SNOM). Here we discuss the conformation of PMMA in the confined geometry of the thin film with a thickness less than the unperturbed chain dimension in the bulk state. The direct observation by SNOM was performed for individual fluorescence-labeled PMMA chains dispersed in the thin films of the unlabeled PMMA prepared by the spin coating and Langmuir-Blodgett techniques. We examined the effect of the constraint in the height direction on the radius of gyration in the direction parallel to the film surface, R xy , i.e., the chain dimension normal to the confinement. In the thickness range of 1-100 nm, R xy in an ultra-thin film was not significantly altered from that in the bulk state, indicating that the PMMA chain has lowered interchain entanglement in the ultra-thin film. Polymer thin films have been widely used in various fields (e.g., coatings and adhesives) and have been extensively explored from both the fundamental and applied points of view. The physical properties of the polymeric thin film differ from those in the three-dimensional bulk state when the thickness is less than the unperturbed dimension of the polymer chain.1-8 The effect of the interaction between the polymer chain and the interface is a crucial factor to determine the characteristic properties of the thin film. As well as the interface effect, the effect of the reduced degree of freedom of a polymer chain due to the spatial confinement is also important. The properties of the individual chain and the interaction among the surrounding chains would be altered from the bulk state. The conformation of the single polymer chain is one of the most fundamental issues to understand the physics of the polymer thin film; therefore, it has attracted much attention and has been extensively studied by many researchers. The scaling theory predicted that the radius of gyration, R g , of the polymer chain with the degree of polymerization of N would be expressed as R g $ N 0:5 for the two-dimensional chain, indicating that the two-dimensional chain would be segregated. 9 Computer simulation studies have shown that for the polymer chain restricted between two walls separated by less than the bulk R g the chain dimension parallel to the surface is not dependent on the gap between the walls and that the value of R g is scaled by a factor of $ 0:5 in the two-dimensional limit. [10][11][12][13][14][15] Although the experimental method to study the conformation of the polymer chain in the ultra-thin film is limited because of the weak signal from the low sample volume, the small angle neutron scattering (SANS) is a powerful technique to probe the chain conformation. Kraus et al. studied the chain morphology of polystyrene (PS) in the thin film of the regular and deuterated PS by SANS experiments. 16 The radius of gyration, R g , in the parallel direction to the film surface increased with the decrease of the film thickness for the thicknes...

Predominance of 018 (smz) and 369 (trf) resistance against gatifloxacin, moxifloxacin, erythromycin and clindamycin were observed in the PCR-ribotype 018 isolates. Interestingly, all trf isolates were toxin A-negative, toxin B-positive, but they did not correspond to PCR-ribotype 017, thus being assigned a new ribotype (PCRribotype 369). In conclusion, PCR-ribotypes 018 (smz) and 369 (trf) were identified as major circulating strains in both outbreak and non-outbreak settings in Japan. Given their epidemiological relevance, molecular investigations are warranted to clarify potential evolutionary links with related strains found elsewhere, such as PCR-ribotypes 018 and 017 from Europe and North America.

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