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Endocrine Abstracts (2014) 35 P219 | DOI: 10.1530/endoabs.35.P219

1Department of Nephrology and Endocrinology, John Paul II Regional Hospital, Zamosc, Poland; 2Chair of Physiotherapy, Zamosc University of Administration and Management, Zamosc, Poland.

Introduction: In the testicular adrenal rest tumor (TART) syndrome, doctors think before all to preserve fertility and oncological risk. Meanwhile, for the patient are relevant primarily those disorders that hinder him daily functioning and affect the quality of life. Because of it, we would like to introduce our patient with cognitive impairment.

Case report: A 20-year-old male, working in the factory, came to an endocrinologist at the request of his mother’s. In the family is known a history of 21-hydroxylase deficiency in the classic form with the loss of salt. The patients have a substitution of hydrocortisone from the neonatal period. In medical examination we find tumorous changed testes. Our attention drew a very high ACTH (1933 pg/ml) and 17-OH-progesterone (>19 ng/ml) with normal testosterone (4.3 ng/ml). The examination of cognitive function (TYM test 20/50) indicated a mild degree of dementia.

The patient admitted that he often forgets about taking drugs and generally has a growing feeling of bad memory. In pediatric medical history we find information testicular tumorous changes disappearing when ACTH levels normalized during the regular substitution.

Cognitive and executive dysfunctions have been described in 21-hydroxylase deficiency, more common in women. It is difficult to decide whether an important pathogenic factor is, an excess of glucocorticoids, their insufficient substitution and/or associated increase of ACTH or whether cognitive impairment a independent of disorders resulting from 21-hydroxylase deficiency. But we cannot rule out the mechanism vicious circle, where irregular intake of drugs affects memory and memory disorders impede regular medication.

Conclusion: According to our knowledge this is the first report of cognitive impairment in TART syndrome.

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