Reversible brain atrophy and cognitive impairment in an adolescent Japanese patient with primary adrenal Cushing’s syndrome

Abstract

Endogenous Cushing’s syndrome is an endocrine disease resulting from chronic exposure to excessive glucocorticoids produced in the adrenal cortex. Although the ultimate outcome remains uncertain, functional and morphological brain changes are not uncommon in patients with this syndrome, and generally persist even after resolution of hypercortisolemia. We present an adolescent patient with Cushing’s syndrome who exhibited cognitive impairment with brain atrophy. A 19-year-old Japanese male visited a local hospital following 5 days of behavioral abnormalities, such as money wasting or nighttime wandering. He had hypertension and a 1-year history of a rounded face. Magnetic resonance imaging (MRI) revealed apparently diffuse brain atrophy. Because of high random plasma cortisol levels (28.7 μg/dL) at 10 AM, he was referred to our hospital in August 2011. Endocrinological testing showed adrenocorticotropic hormone-independent hypercortisolemia, and abdominal computed tomography demonstrated a 2.7 cm tumor in the left adrenal gland. The patient underwent left adrenalectomy in September 2011, and the diagnosis of cortisol-secreting adenoma was confirmed histologically. His hypertension and Cushingoid features regressed. Behavioral abnormalities were no longer observed, and he was classified as cured of his cognitive disturbance caused by Cushing’s syndrome in February 2012. MRI performed 8 months after surgery revealed reversal of brain atrophy, and his subsequent course has been uneventful. In summary, the young age at onset and the short duration of Cushing’s syndrome probably contributed to the rapid recovery of both cognitive dysfunction and brain atrophy in our patient. Cushing’s syndrome should be considered as a possible etiological factor in patients with cognitive impairment and brain atrophy that is atypical for their age.

Region‐specific disturbed iron redistribution in Cushing's disease measured by magnetic resonance imaging‐based quantitative susceptibility mapping

“…In the past three decades, decreased grey matter volumes and increased CSF volumes were continuously reported in cross‐sectional MRI studies. On the other hand, some other longitude studies also demonstrated that these alterations were only partially recoverable after the resolution of hypercortisolism 20–39 …”

“…On the other hand, some other longitude studies also demonstrated that these alterations were only partially recoverable after the resolution of hypercortisolism. [20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39] On the other hand, in studies of normal ageing in the human brain, despite the decline in processing speed, working memory, inhibitory function and long-term memory, as well as decreases in brain structure size and WM integrity, surprisingly, reliable increases in prefrontal activation have been found. 40 The scaffolding theory of ageing and cognition (STAC) was proposed to account for these common phenomena.…”

Reversible Non-parkinsonian Bradykinesia with Impaired Frontal Lobe Function as the Predominant Manifestation of Adrenal Insufficiency

“…Unlike hypercortisolemia-induced brain impairments, such as the brain atrophy associated with cognitive impairment (30,31), few studies have focused on the imaging features of the brain affected by hypocortisolemia. A previously reported patient with Addison's disease who showed psychiatric symptoms as the only manifestation of AI, showed no morphological abnormalities on brain MRI (4).…”

The role of MR volumetry in brain atrophy assessment in multiple sclerosis: A review of the literature

“…All this may affect the accuracy of BV measurements. 25,26 Duning et al 27 showed that hydration status can significantly change BV: a lack of fluid intake for 16 h decreased BV by 0.55% (standard deviation (SD) = 0.69) and after rehydration total BV increased by 0.72% (SD = 0.21). Changes as high as 30-40 mL have also been observed after dialysis in patients with renal failure.…”

“…Enlargement of the cortical sulci and cerebrospinal fluid spaces as well as pituitary gland atrophy in patients with AN have been reported. 25 Heinz et al postulated that the mechanism responsible for the atrophic changes may be related to protein loss and fluid retention caused by hypercortisolism and loss of serum protein. This suggests that there is no simple correlation between cerebral atrophy and malnutrition.…”