The authors report a case of 4 year old boy with subacute sclerosing panencephalitis (SSPE), withunusual immunologic findings.
The patient was well until 3 years and 4 months of age, when he developed a generalized convulsion and ataxic gait. About 3 months prior to the onset, he had had a febrile episode with exanthema, presumably of measles. Within several weeks, progressive mental deterioration was noted, and hedeveloped myoclonic movements, muscle rigidity, and pyramidal tract signs. Ophthalmologic examinationsrevealed optic and retinal atrophy. The EEG showed a paroxysmal slow wave burst with spikedischarge predominantly in frontal area. The PEG revealed slight dilatation of lateral ventricles.
The measles antibody titers in serum were abnormal but curious, CF titers to one measles antigenbeing high (2048X), while that to. another antigen being not so elevated (32-64X). In addition, thereware noted several abnormal autoantibody reactions, including ANF, RA, cold hemoagglutination, and Paul-Bunnell reaction. The delayed dermal reactions to measles, mumps, rubella, mycoplasma, and SK-SD antigens were all negative, but the reaction to PPD was positive.
The patient has been currently in coma with neurovegetative manifestations, including obesity, hyperhidrosis, and unexplained fever.
This case has several atypical features for SSPE, such as earlier onset, acutely progressive course, suggestive but not characteristic EEG, variability of antibody titers to different measles antigens, andseveral abnormal autoantibodies.
