[Psychomotor development rate of children with infantile neuronal ceroid lipofuscinosis].

Background: Late infantile neuronal ceroid lipofuscinosis (LINCL) is the most common childhood progressive encephalopathy. Severe psychodegradation with diminish of cognitive functions together with ataxia, myoclonus, resistant epilepsy, paresis and blindness in aged 3-6 yrs are observed.

The Aim Of Study: Cognition of psychomotor development in children with LINCL.

Material And Methods: In group of 65 children with LINCL diagnosed by ultrastructural conjunctive examinations and/or low activity of TPP1 enzyme in leukocytes, the psychological assessment during 3 years of disease was performed. 25 children had done psychological test with use Psyche Cattell Scale for Small Children twice in 1-3 years intervals. In next 40 children clinical, neurological observations during some years was made.

Results: In 25 children Psyche Cattell tests revealed severe mental retardation (IQ 20-35) in 16% and profound mental retardation in 84%. In the first year of disease 56% of children was degraded to the profound mental retardation (44% in moderate and 12% in severe mental retardation). In all 65 affected children already in the first neurological visit the delay and regress in global of mental development was observed (any of children hadn't have IQ rates on average level). Analysis of psychodegradation rate revealed in 60% of children after 1-3 years from onset of disease very rapid mental retardation and decreased IQ above 2 SD and in 24% above 3SD.

Conclusions: Common progressive encephalopathy LINCL caused very rapid severe mental degradation, already at the first stage of the development of disease and that is very important diagnostic sign.