Cushing's syndrome I

Glucocorticoid excess syndrome in which the hypersecretion of glucocorticoids is secondary to hypersecretion of adrenocorticotrophic hormone from the pituitary. It may also be caused by a basophilic adenoma of the pituitary. Clinical features are fatigue, weakness, abdominal obesity, «buffalo hump» (an excessive deposit of fat over the clavicles and back of the neck), and moon-shaped face, nervousness, irritability, depression, and amenorrhoea or menstrual irregularity. Most commonly observed in females in childbearing age. In males there is loss of potency. Hirsutism, plethora, abdominal striae, diabetes mellitus, osteoporosis, increased susceptibility to infection, cataracts, renal calculi, senile purpura, proximal myopathy, hypertension may be associated. The syndrome may be recognized soon after birth; children with it have stunted growth. It can also be caused by treatment with corticosteroids.

Some synonyms of this syndrome are used also to designate congenital adrenal hyperplasia.

The syndrome was first described by William Cooke, a British surgeon at Brentford, in 1811. But Cooke’s case was not the first description. Probably the earliest case confirmed by necropsy was that descibed by Henry Sampson, Fellow of the College of Physicians After Cooke it was described by Nikolai Mikhailovich Itsenko (1889-1954) in 1926, six years before Cushing, who in 1932 ascribed the symptom complex to a basophilic adenoma of the pituitary.

The term Cushing’s syndrome for this condition was introduced by Fuller Albright (1900-1969) in 1943.

There is some disagreement among authors as to whether Cushing’s syndrome I and the Apert-Gallais syndrome is the same entity.

We thank Dr. Malte Stoffregen for information submitted.

• Henry Sampson:
  A relation of one Hannah Taylor, a very Extraordinary Child of about Six years of Age, who in Face, &c was as large as a full grown Woman; and of what appeared on the Dissection of her Body. Philosophical Transactions of the Royal Society, London, 1695-97, 19 (217): 80-82.
• William Cooke:
  A case of Hydrocephalus internus.
  Transactions of the Royal Medical and Chirugical Society of London, 1811, 2: 17-23.
• N. Itzenko:
  Pluriglandulares Syndrom mit pathologisch-anatomischem Bild.
  Zeitschrift für die Gesamte Neurologie und Psychiatrie, Berlin, 1926, 103: 63.
• E. Apert:
  Dystrophies en relation avec des lésions des capsules surrénales. Hirsutisme et progenia.
  Bulletin de la Société de pédiatrie de Paris, 1910, 12: 501-518.
• A. Gallais:
  Le syndrome génitosurrénal, étude anatomo-clinique. Paris, 1912: 224.
• H. W. Cushing:
  The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism).
  Bulletin of the Johns Hopkins Hospital, Baltimore, 1932, 50: 137-195.
• N. M. Itsenko:
  K klinike i patogenezu cerebralnych vegetativnych syndromov v sviazi s ucheniem o mezhutocno-gipofizarnoi systeme. Voronezh, 1946.
• Malte Stoffregen:
  Zwei frühe Fallbeschreibungen des adrenogenitalen Syndroms (Bevern und Römhild (1802) - Tilesius (1803)).
  Sudhoffs Archiv 2001, 85: 138-168, see pages 144-145.