Psychological Follow-Up in Childhood Dementia: A Longitudinal Study of Subacute Sclerosing Panencephalitis

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Abstract
The article describes a methodology for longitudinal psychological follow-up in diseases which involve intellectual deterioration and presents data collected over a 7-year period on subacute sclerosing panencephalitis (SSPE), a rare, neurodegenerative disease of viral origin which affects predominantly children and adolescents. A total of 66 evaluations were carried out with 19 SSPE patients, all of whom were receiving experimental treatment with an immunomodulator, inosiplex. Of the 19 study patients, 5 have died and 14 survive. Psychological tests were selcected for each evaluation according to the subject's level of ability which ranged from normal functioning to profound dementia. Using results of psychological testing, subjects were rated on a seven-point Psychological Disability Rating Scale indicating extent of disability in the following categories: gross motor skills, self-care skills, social skills, language skills and verbal IQ, visual motor skills and performance IQ, and academic skills. The scale was found to have high inter-rater reliability with correlations of 0.99. The scale also showed strong correspondence to a measure of neurological deficit with a Pearson's r of .86. The study confirmed and extended previous observations of a high verbal, low performance pattern on intellectual testing in high functioning SSPE patients. Early age at onset (4-13 years) was associated with relatively more severe disability at latest follow-up than older onset (14-18 years).

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