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Subacute sclerosing panencephalitis (SSPE) is a slowly progressive and highly lethal disease of the central nervous system. Although the primary cause of SSPE is believed to be persistent infection of neuron and glial cells by a measles virus, the precise mechanism of the progression of this disease has not yet been elucidated. CD9, a member of the tetraspanin family, is expressed in myelin and other nervous tissues. This study detected significant amounts of anti-CD9 antibodies in the cerebrospinal fluid (CSF) of all patients with SSPE included in the study. Anti-CD9 antibodies were also detected in the CSF of some patients with other neurologic disorders, but those patients had lower levels of anti-CD9 antibodies than did the patients with SSPE. The level of anti-CD9 antibodies was elevated and reached a peak that coincided with the appearance of brain atrophy. These findings shed light on a new aspect of the causes and progression of SSPE. [ABSTRACT FROM AUTHOR]

Myoclonus is a characteristic neurological sign of subacute sclerosing panencephalitis (SSPE). Attempts were made to induce myoclonus in a large proportion of hamsters with a cell-associated strain of SSPE virus (the Biken strain) and thereby to establish an experimental model for study of the mechanism of development of this condition. When injected intracerebrally, Biken virus induced myoclonus within two to 14 days in 84% of the three- to nine-week-old hamsters tested. Electroencephalographic traces showed a periodic and synchronous discharge consisting of high-voltage slow waves and spikes that appeared coincidentally with myoclonus. Neurons in the cortex and thalamus of the affected animals had severely degenerated cytoplasm. Inflammatory changes, such as perivascular cuffing or infiltration of mononuclear cells, were not detected. Staining with immunoperoxidase revealed measles viral antigens in the cytoplasm and dendrites of the affected neurons. SSPE virus with the same properties as the parent virus was recovered from brain cells of sick animals by cocultivation with Vero cells. [ABSTRACT FROM PUBLISHER]

Okuno Y (Research Institute for microbial Diseases, Osaka University, yamada-oka, Suita, Osaka 565, Japan), Nakao T, Ishida N, Konno T, Mizutani H, Fukuyama Y, Sato T, Isomura S, Ueda S, Kitamura I and Kaji M. Incidence of subacute sclerosing panencephalitis following measles and measles vaccination in Japan. 1989, : 684–689. The Japanese Committee for the National Registry of Subacute Sclerosing Panencephalitis (SSPE) confirmed that 215 cases of SSPE occurred in the 20 years from 1966 to 1985, as discovered in the 10-year surveillance from April 1976 through March 1986. The annual incidence in recent years has been between 10 and 23 cases. Among cases with a certain history of measles illness or measles vaccination, 184 (90.2%) had a history of measles illness without receiving measles vaccine. There were 11 probable measles vaccine-associated cases (5.4%) three (1.5%) being vaccinated with a combined use of killed and live vaccine and eight (3.9%) with further attenuated live vaccine. There were nine cases (4.4%) Without a history of either measles illness or measles vaccination. Intervals between measles illness and the onset of SSPE varied from 1 to 16 years (mean, 7.0 years). The periods following measles vaccination with further attenuated live vaccine were 2 to 11 years (mean, 4.6 years). Annual incidence rates of SSPE per million cases of measles ranged between 6.1 and 40.9 (mean, 16.1) in the 10 measles epidemic years 1968–1977, and those following vaccination with further attenuated live vaccine were zero in most years and at the highest 3.08 (mean, 0.9) per million doses of distributed vaccine. [ABSTRACT FROM PUBLISHER]