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IMMUNOLOGICAL STUDIES IN SUBACUTE SCLEROSING PANENCEPHALITIS

Subacute sclerosing panencephalitis ( SSPE) has been diagnosed in three patients, aged 12-14 years, since January 1969. The disease followed a uniform course in all three, ending fatally within 9 to 12 months after onset. Immunosuppressive therapy in two cases had no definite effect. The diagnosis was confirmed by brain biopsy and/or autopsy in two cases. Serum measles antibody titres were increased in all three patients, all of whom had had clinical measles infection 10-1 2 years previously. Examination of cerebrospinal fluid JCSF) showed an increase in gamma-globulin. Immuno-electrophoresis and agarose-gel-electrophoresis revealed abnormalities of CSF IgG, the most marked of these being the presence of 2-5 abnormal subfractions appearing as sharp bands on agarose-gel-electrophoresis. In two patients, two or three of these subfractions could also be demonstrated in the serum. Immunization procedures in vivo and lymphocyte cultures in vitro revealed no definite abnormalities in two patients, whereas some evidence of impaired cell-mediated immune response was found in one patient. In vitro response to measles virus antigen could not be demonstrated in any of the three cases. Immunofluorescent studies of brain tissue in two patients revealed evidence of IgG production in perivascular infiltrates. Most striking, however, was the presence of IgG deposits on nerve and glia cells, suggesting antibody bound to antigen(s) in the tissue. Extraction of water-soluble proteins from one SSPE brain showed a marked increase in brain IgG content (81 mg% ) as compared with normal brain (5.2 mg% ) . Evidence suggesting increased amounts of complement (beta-lc) in the SSPE brain was also revealed. SSPE brain IgG, isolated from brain extract by gel filtration, showed the same abnormal pattern as CSF IgG, and preliminary studies suggest that this IgG contains a very high specific measles antibody activity.