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Dentatorubral-pallidoluysian atrophy (DRPLA) is one of the CAG-repeat diseases, and is classified into juvenile and early adult types showing progressive myoclonus epilepsy (PME) in addition to late adult type. We immunohistochemically examined accumulation of oxidative products and expression of superoxide dismutase (SOD) in autopsy cases of DRPLA. Oxidative products to nucleosides, 8-hydroxy-2'-deoxyguanosine and 8-hydroxyguanosine, were accumulated in the lenticulate nucleus predominantly in DRPLA cases having PME. Neuronal accumulation of 4-hydroxy nonenal, a reactive lipid aldehyde, was found in the hippocampus, globus pallidus and cerebellar dentate nucleus in adult DRPLA cases and controls. Cytoplasmic immunoreactivity for Cu/ZnSOD was reduced in the external segment of globus pallidus, dentate nucleus and cerebellar cortex in DRPLA cases. Mitochondrial immunoreactivity for MnSOD was reduced in the lenticulate nucleus and cerebellum in DRPLA cases having PME. Some DRPLA cases showed reduced immunoreactivity for MnSOD in the cerebral cortex. Coexistence of reduced SOD expression and polyglutamine was observed in a few cases. It has been discussed in Huntington's disease that expanded polyglutamine can lead to oxidative neurodegeneration. It is likely that oxidative stress can be involved in DRPLA, although relationship with expanded polyglutamine remains to be elusive.

Experimental autoimmune myositis (EAM) was induced in Lewis rats by immunization with partially purified and purified skeletal myosin. Although clinical signs such as muscle weakness were very mild, multiple inflammatory lesions in the skeletal muscle, but not in the heart, were found by histological examination. Immunohistochemical staining revealed that muscle fiber-infiltrating cells were CD8+ and CD11b+ cells and that CD4+, TCR alphabeta+, B and NK cells were mainly located in the endomysium and interfiber connective tissue. These findings were in contrast to those obtained in experimental autoimmune encephalomyelitis lesions in which CD4+ cells predominate over CD8+ cells. T cells and sera isolated from myosin-immunized animals responded vigorously to myosin. However, neither sensitized lymphoid cells mainly comprising CD4+ cells nor purified anti-myosin immunoglobulin G mediated the disease into naive rats, suggesting that T cells other than CD4+ cells such as CD8+ cells may be the final effector. Taken together, EAM induced in Lewis rats is similar to human polymyositis (PM). EAM can serve as a good model for human PM and give insight into the pathogenesis of the disease.

We describe a 12-year-old girl, who had been medicated with theophylline for bronchial asthma and developed acute encephalopathy with refractory status epilepticus, showing bilateral mesial temporal and claustral lesions, which were evident on fluid-attenuated inversion recovery images, obtained with 1.5 T magnetic resonance imaging. To date, oxidative stress has been implicated in aging or various disorders, including inflammatory or degenerative neurological disorders. One of the oxidative stress markers, 8-hydroxydeoxyguanosine, was increased in our patient's cerebro-spinal fluid, plasma and urine. We speculate that augmented oxidative stress was associated with refractory status epilepticus in our patient, accompanying bilateral mesial temporal, claustral lesions and severe neuronal damage. Serial measurements of oxidative stress markers in acute encephalitis, encephalopathy, or status epilepticus could clarify the relationships between acute brain damage and free radicals.

a Department of Pediatrics, Tokyo Kita Shakai Hoken Hospital, 4-17-56 Akabanedai, Kita-ku, Tokyo 115-0053, Japan b Department of Pediatrics, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8519, Japan c Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, 2-6 Musashidai, Fuchu-shi, Tokyo 183-8526, Japan d Department of Pediatrics, Tokyo Metropolitan Fuchu Medical Center for the Disabled, 2-9-2 Musashidai, Fuchu-shi, Tokyo 183-8553, Japan e Department of Neuropathology, Saitama Medical College, 38 Morohongo, Moroyamamachi, Iruma-gun, Saitama 350-0495, Japan