A DESCRIPTION OF ADDISON'S DISEASE, AND ITS NEUROPSYCHIATRIC MANIFESTATIONS COMPARING THE DISEASE AS IT IS NOW TO THE
Michaela Agius1, Mark Agius2,3
1University of Malta, Msida, Malta.
Abstract:
We describe Addison's disease, which is the disease caused by Adrenal Gland Insufficiency. Addison's disease is an uncommon endocrine disorder characterised by inadequate production of hormones, predominantly aldosterone and cortisol, by the adrenal glands. This condition occurs due to damage to the adrenal cortex, the region responsible for hormone synthesis. Clinical manifestations of Addison's disease are often insidious and nonspecific, including symptoms such as myasthenia, persistent fatigue, unintentional weight loss, hypotension, and hyperpigmentation of the skin, especially in areas subjected to friction. A number of Neuropsychiatric manifestation, including Depression, and Psychosis can also occur. The aetiology is primarily autoimmune adrenalitis, where the immune system erroneously attacks and destroys adrenal cortical cells. Other aetiologies include infectious agents like Mycobacterium tuberculosis, which can infiltrate and impair the adrenal glands. Hemorrhagic damage to the adrenal cortex can occur secondary to severe stress, trauma, or coagulopathies. Additionally, metastatic neoplasms may involve the adrenal glands, leading to their destruction. Less frequently, genetic disorders such as adrenoleukodystrophy can compromise adrenal function by affecting both the cerebral white matter and adrenal cortex. Adrenal cortex damage can also result from adrenalectomy or the administration of medications that inhibit steroidogenesis. The pathophysiology of Addison's disease involves the disruption of the hypothalamic-pituitary-adrenal (HPA) axis, resulting in cortisol deficiency, which is critical for regulating metabolism, immune function, and stress responses. Aldosterone deficiency leads to dysregulation of sodium and potassium homeostasis, causing hypotension and dehydration. Management of Addison's disease requires lifelong glucocorticoid and mineralocorticoid replacement therapy, typically with hydrocortisone or fludrocortisone, along with ongoing monitoring and dosage adjustments during periods of stress, illness, or surgical interventions. While describing Addison's Disease we describe the illness as experienced by Elizabeth of the Trinity, a Catholic Nun who suffered the illness in the early part of the Twentieth Century, whose illness is described from her letters and eyewitness accounts, taken for her beatification and canonisation findings. We describe how the illness it was viewed then, when it was seen as incurable, with great deficiencies in both diagnosis and treatment, and how the aetiology has changed over the years. Hence we show how the suffering endured by this person had both mental and physical aspects.
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