DOI: https://doi.org/10.21203/rs.3.rs-460503/v1
Background: Isolated adrenocorticotropic hormone (ACTH) deficiency (IAD) is an adrenal insufficiency caused by a decrease in ACTH alone among the anterior pituitary hormones. IAD is often overlooked because it causes nonspecific symptoms. We report a case of IAD diagnosed after the appearance of mild cognitive impairment (MCI).
Case presentation: Our department was consulted on a case of a 46-year-old man for whom dementia was suspected because he was speaking incoherently, wiped his nose with his hands and showed mild brain atrophy on MRI. He was previously diagnosed with some psychiatric diseases, such as narcolepsy and adjustment disorder. He could not continue working due to somnolence, general fatigue and appetite loss. At the examination, he had difficulty concentrating and sustaining attention, exhibited restlessness, and scored 25 points on the Mini Mental State Examination-Japanese (MMSE-J); MCI was observed. Blood tests showed decreased ACTH, cortisol and free thyroxine and increased thyroid-stimulating hormone levels. He received further evaluation, and he was diagnosed with IAD and primary hypothyroidism. Hydrocortisone (15 mg) was started, after which his physical problems and his difficulty concentrating disappeared. His MMSE-J score also increased to 30. His cognitive functions completely recovered. He returned to work and continued to work stably.
Conclusion: This patient was diagnosed with IAD and treated with adrenocortical hormone replacement therapy. His cognitive functions recovered, his physical and mental disorders were alleviated, and he returned to society. It is important not to miss neurological and psychiatric symptoms that can be cured by physical treatment.
Isolated adrenocorticotropic hormone (ACTH) deficiency (IAD) is a cause of adrenal insufficiency in which secretion of only ACTH is impaired, while secretion of other anterior pituitary hormones is maintained. Diagnosis is often delayed due to nonspecific symptoms such as general fatigue, appetite loss, weight loss, hypoglycemia, and hypotension. We herein report a case of a Japanese man with IAD complicated by primary hypothyroidism presenting with several neuropsychiatric symptoms, especially cognitive dysfunction, before diagnosis who showed complete improvement of cognitive function after administration of hydrocortisone.
IAD is a pituitary disorder characterized by decreased secretion of ACTH, which causes secondary adrenal insufficiency (AI). The main symptoms of IAD are caused by glucocorticoid deficiency. Steinberg reported the first case of IAD in 1954. Patients with IAD present with general fatigue, weight loss and hypoglycemia, which improve after ACTH administration [1]. The prevalence of IAD was found to be 1.91–7.3 per 100,000 persons in a Japanese cohort [2–3]. Individuals with IAD primarily present with symptoms of AI, such as general fatigue, loss of appetite, nausea, and vomiting. IAD is often misdiagnosed as a psychological disorder when patients have psychological symptoms, including apathy, depression, delusion and abnormal behavior [4]. In this case, the patient was diagnosed with some neuropsychiatric disorders, including narcolepsy, adjustment disorder, and mild cognitive impairment. Nagai reported that a 69-year-old man was admitted because of abdominal pain with IAD accompanying dementia [5]. Matsuo reported cognitive impairment and a depressive state caused by IAD in a 45-year-old Japanese man༻6༽. Goto reported an IAD case presenting with gait disturbance necessitating differential diagnosis from idiopathic normal pressure hydrocephalus༻7༽. Cognitive dysfunction is considered to be one of the symptoms of IAD. This case showed complete recovery of cognitive function after the administration of hydrocortisone. The neuropsychiatric symptoms were also improved, such as difficulty in sustaining attention, impulsiveness and restlessness. The MMSE-J and WAIS-Ⅲ scores increased. Since cognitive dysfunction resulting from IAD is treatable, clinicians should keep IAD in mind when assessing patients with impaired cognitive functions.
Adrenocortical hormone replacement therapy is dramatically effective for the symptoms of IAD. This patient recovered from his cognitive dysfunction and physical and mental disorders and returned to society. The patient had IAD accompanied by impaired cognitive functions and dramatically improved after treatment with adrenocortical hormone replacement therapy. Clinicians must consider the possibility of IAD when assessing patients with impaired cognitive functions.
Acknowledgments
We thank the members of the Medical Center for Dementia, the university hospital for discussing this patient's care.
Funding: Not applicable.
Conflicts of interest: Not applicable.
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Consent for publication: Written informed consent was obtained from the patient for publication of this case report.
Availability of data and materials: All data generated or analyzed during this study are included in this published article.
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Authors’ contributions: AI, IS, NO, and YK were involved in the clinical investigations. AI wrote the first draft of the manuscript. RY and AI were involved in the literature review and corrections. All authors read and approved the final manuscript.