Article

AIM: To examine the cognitive functioning of young people with
pantothenate-kinase-associated neurodegeneration (PKAN) after pallidal deep brain
stimulation (DBS). PKAN is characterized by progressive generalized dystonia and
has historically been associated with cognitive decline. With growing evidence
that DBS can improve motor function in adults and children with PKAN, there is
now the opportunity to study the cognitive profiles of these patients over time.
METHOD: We present a case series of seven children (mean age 11 y 7 mo, SD 3 y 2
mo) undergoing bilateral pallidal DBS for the management of severe
PKAN-associated dystonia. We administered standardized measures of intellectual
ability and memory where possible, before DBS and 1 to 4 years after DBS.
RESULTS: No cognitive decline was observed and scores improved in all but one
child (whose dystonia could not be adequately controlled owing to multiple
medical problems). In line with a stabilization or reduction in their dystonia,
all but one child was able to tolerate longer assessment sessions and complete
either the same or a greater number of subtests. INTERPRETATION: These findings
suggest that apparent cognitive impairments may reflect difficulties in accessing
cognition owing to severity of dystonia. Intellectual decline previously
associated with PKAN may have been overestimated.
CI - (c) The Authors. Journal compilation (c) Mac Keith Press 2010.

Langue : ANGLAIS