Abstract
Purpose To present a systematic review of the presence and severity of neuropsychological
impairment in the six main neuropsychological domains (attention, executive function,
language, visuospatial processing, intelligence, and memory) in patients with Cushing’s
disease (CD) and/or Cushing’s Syndrome (CS) at various stages of the illness. The
work aims to identify neuropsychological leverage points for focused diagnosis and
rehabilitation in CS/CD patients.
Methods A pubmed literature search was performed and augmented by searching the reference
lists of review articles identified by this search strategy. After excluding irrelevant
hits, we systematically extracted data from 27 studies for each main neuropsychological
domain, differentiating between active disease, short- and long-term remission.
Results The literature gives evidence for neuropsychological impairment in all domains in
Cushing patients with active disease. The most consistent impairments concerned memory
and visuo-spatial processing, whereas the data are discordant for all other domains.
Significant improvement of neuropsychological function – although not returning to
normal in all domains – is shown in short-term and long-term remission of the disease.
However, the published literature is thin, suffering from repetitive subsample analyses
publishing, methodological concerns as lack of control for confounders such as depression.
Conclusions Memory is the most extensively investigated domain in CS/CD patients and impairment
is most prominent in active disease. Patients should be counseled that neuropsychological
function will improve with normalization of hypercortisolism and over time. More studies
with more stringent methodological criteria, larger patient samples and controlling
for confounders are required to enhance our understanding of neuropsychological function
in patients with CS/CD.
Keywords
Cushing’s disease - Cushing’s syndrome - neuropsychological impairment - depression
- rehabilitation
