Cushing's syndrome historical perspective
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Furqan M M. M.B.B.S[2] Mohamad Alkateb, MBBCh [3]
Overview
Cushing's syndrome was first identified by Dr. Harvey Cushing (an American physician, surgeon, and endocrinologist) in 1932 as a polyglandular disorder. It was later named as Cushing's syndrome.
Cushing's syndrome historical perspective
- In 1924, the Soviet neurologist Nikolai Mikhailovich Itsenko reported two patients with pituitary adenoma. The resulting excessive adrenocorticotropic hormone secretion led to the production of large amounts of cortisol by the adrenal glands.
- The disease associated with this increased secretion of cortisol was described by Harvey Cushing in 1932, after Cushing was presented with a unique case of the disease.
- In 1910, the American neurosurgeon Harvey Cushing (1869-1939) was presented with a case of a 23-year-old woman called Minnie G whose symptoms included painful obesity, amenorrhea, hypertrichosis (abnormal hair growth), underdevelopment of secondary sexual characteristics, hydrocephalus and cerebral tension.[1][2]
- This combination of symptoms was not yet described as any medical disorder at that time.[1] However, Cushing was confident that Minnie’s symptoms were due to dysfunction of the pituitary gland, and resembled those associated with an adrenal tumor.
- He introduced the term “pluriglandular syndrome” to describe this disorder. This was later identified as Cushing's syndrome.[3] Given this conviction, and his knowledge of the three anterior pituitary cell types, Cushing hypothesized that if acidophil hyperpituitarism (excess secretion from the acidophil cells) caused acromegaly, then an excess of basophil cells must be involved in another pituitary disorder that involves sexual dysfunction (amenorrhea in females and erectile dysfunction in males) and could explain Minnie's symptoms.[1] Experimental evidence and case reports by Cushing led to his publication in 1932 on pituitary basophilism as the cause of Cushing's disease. In this publication, the clinical symptoms of the disease, named after Harvey Cushing, were described.[4][5]
- Out of the 12 cases with hypercortisolism described in Cushing’s monograph on the pituitary body, 67% died within a few years after symptom presentation, whereas Minnie G. survived for more than 40 years after symptom presentation, despite the fact that she did not receive any treatments for a pituitary tumor.[1] The prolonged survival led to the uniqueness of Minnie's case. The reason behind this survival remains a mystery since an autopsy of Minnie was refused after her death.[1] However, the most likely explanation, proposed by J. Aidan Carney and based on statistical evidence, was that the basophil adenoma Minnie might have harbored underwent partial infarction, leading to symptom regression.[1] The other hypothesis was that Minnie might have suffered from Primary Pigmented Nodular Adrenocortical Disease (PPNAD), which when associated with Cushing's syndrome (Carney complex) can infrequently cause spontaneous symptom regression of the latter.[1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Lanzino, Giuseppe; Maartens, Niki F.; Laws, Edward R. (2002). "Cushing's case XLV: Minnie G.". Journal of Neurosurgery. 97 (1): 231–234. doi:10.3171/jns.2002.97.1.0231. PMID 12134925.
|access-date=requires|url=(help) - ↑ Laws Jr., E.R., Ezzat, S., Asa, S.L., Rio, L.M., Michel, L. & Knutzen, R. (2013). Pituitary Disorders: Diagnosis and Management. United Kingdom: Wiley-blackwell. p. xiv. ISBN 978-0-470-67201-3.
- ↑ Loriaux DL (2017). "Diagnosis and Differential Diagnosis of Cushing's Syndrome". N. Engl. J. Med. 376 (15): 1451–1459. doi:10.1056/NEJMra1505550. PMID 28402781.
- ↑ Cushing, Harvey (1932). "The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism)". Bulletin of the Johns Hopkins Hospital. 50 (4): 137–95. PMC 2387613. PMID 19310569.
- ↑ "Dr. Cushing Dead; Brain Surgeon, 70. A Pioneer Who Won Fame as Founder of New School of Neuro-Surgery. Discovered Malady Affecting Pituitary dre. Was Noted Teacher and Author". New York Times. 8 October 1939. Retrieved 2010-03-21.