Background: Endogenous Cushing's syndrome, a rare endocrine disorder, characterised by chronic cortisol hypersecretion, results in neuropsychiatric disturbances and in cognitive deficits, which are only partially reversible after the biochemical remission of the disease.
Case presentation: We report a case of a woman with a profound cognitive deficit and a gradual functional decline caused by Cushing's disease of at least 10 years duration. The neurosurgical resection of her 2 mm adrenocorticotropic hormone (ACTH) secreting pituitary microadenoma resulted in a successful resolution of the patient's hypercortisolism and a significant recovery of her neurocognitive function. The patient's progress was evaluated using serial clinical observations, functional assessments, Mini-Mental Status exams and through the formal neuropsychological report. Furthermore, the patient's recovery of her neurocognitive function was reflected by a sustained improvement in the patient's specific structural brain abnormalities on radiological imaging.
Conclusions: This report illustrates the importance of early detection and treatment of Cushing's syndrome in order to prevent neurocognitive impairment and neuropsychiatric disorders which are associated with an endogenous cortisol hypersecretion. The long term adverse effects of severe hypercortisolaemia on brain function and the pathophysiological mechanisms responsible for the structural and functional changes in brain anatomy due to glucocorticoid excess are reviewed.
Keywords: Cognitive deficit; Cushing’s syndrome; Functional decline; Microvascular ischaemic changes; Pituitary microadenoma; Transsphenoidal excision.