The early clinical features, age at onset, and relation to immunization of 32 patients (24 males, 8 females) with SSPE are reported from Grant Medical College, Mumbia, India. Diagnosis was confirmed by measles antibody in CSF and by electroencephalography. The mean age of patients was 13.4 years (range, 4-21 years) (mean of 15.7 years in 9 who had received measles vaccination and 12.4 years in the unvaccinated group). Loss of vision (cortical) in 7 cases, seizures in 6, and behavioral change in 7 were the presenting symptoms in 40% of patients. More classic symptoms of myoclonus and cognitive decline, present at first examination in 27 and 26 patients, respectively, were delayed for a mean of 8 months after onset of symptoms. MRI or CT in 21 was normal in one-third and showed abnormalities in 13, with predominantly white matter lesions in the parietal-occipital areas. Compared to a similar study of 39 patients at the same institution in 1974, the present series shows a later mean age of onset (13.4 cf 11.2 years), a similar male preponderance (24:8 cf 36:3), myoclonus (84% cf 95%). cognitive decline (81% cf 97%), less seizures (19% cf 33%), hemiparesis (16% cf 5%), chorioretinitis (13% cf 2%), and more prevalent visual loss (22% cf 0). [1]

COMMENT. The changing character and resurgence of SSPE in the US was referred to by Dyken PR in 1989 and is readdressed in an editorial [2]. The above reports emphasize that measles and SSPE are not disappearing entities, even in “developed” countries, and that complacency among pediatric neurologists should be avoided. In addition to the typical subacute progressive form (SPF) that represents about 75% of reported cases, atypical cases are not stereotyped and consist of an acute progressive form (APF), a chronic progressive form (CPF), and two remitting forms, one chronic and one subacute. In the current report from India, 3 of 9 patients (9% of the total) who were vaccinated may have had an APF, and 6 with early onset loss of vision could be classified as chronic atypical (19% of total). Visual loss associated with posterior cerebral demyelination demonstrated in this series was not reported in earlier publications. The role of immunization in the changing character and age at onset of SSPE requires further study.