Article

Cognitive impairments have been established as part of the non-motor
phenomenology of adult dystonia. In childhood dystonia, the extent of cognitive
impairments is less clear. This systematic review aims to present an overview of
the existing literature to elucidate the cognitive profile of primary and
secondary childhood dystonia. METHOD: Studies focusing on cognition in childhood
dystonia were searched in MEDLINE and PsychInfo up to October 2017. We included
studies on idiopathic and genetic forms of dystonia as well as dystonia secondary
to cerebral palsy and inborn errors of metabolism. RESULTS: Thirty-four studies
of the initial 527 were included. Studies for primary dystonia showed intact
cognition and IQ, but mild working memory and processing speed deficits. Studies
on secondary dystonia showed more pronounced cognitive deficits and lower IQ
scores with frequent intellectual disability. Data are missing for attention,
language, and executive functioning. INTERPRETATION: This systematic review shows
possible cognitive impairments in childhood dystonia. The severity of cognitive
impairment seems to intensify with increasing neurological abnormalities.
However, the available data on cognition in childhood dystonia are very limited
and not all domains have been investigated yet. This underlines the need for
future research using standardized neuropsychological procedures in this group.
WHAT THIS PAPER ADDS: There is limited data on cognition in childhood dystonia.
Primary dystonia showed intact cognition and IQ, but mild working memory and
processing speed deficits. Secondary dystonia showed more pronounced deficits and
lower IQ, with frequent intellectual disability. There is a strong need for
case-control studies assessing cognition using standardized neuropsychological
tests.
CI - (c) 2017 The Authors. Developmental Medicine & Child Neurology published by John
Wiley & Sons Ltd on behalf of Mac Keith Press.

Langue : ANGLAIS