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Pituitary tumours and acromegaly (Pierre Marie’s disease)
  1. J M S Pearce
  1. 304 Beverley Road, Anlaby, Hull HU10 7BG, UK; jmspearce{at}freenet.co.uk

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    Because physicians of the early nineteenth century were unaware of the endocrine system, acromegaly was regarded as an intrinsic bone disease. Andrea Verga1 in 1864 and Vincenzo Brigidi in 1877 reported early macroscopic and microscopic descriptions of pituitary tumours in acromegalic patients.2

    However, Saucerotte3 reported in 1772 an atypical case in a 39 year old man published in 1801.4 Lombroso had in 1869 described the enlarged organs and limbs as macrosomia. Major also relates a case with an enlarged pituitary gland at autopsy reported before Marie’s paper by Klebs, and Fritzsche in 1884.

    Marie published his pathological studies in two patients in 1886,5 and gave his name to the disorder; he mentions Saucerotte’s patient. This was followed by longer communications in the Nouvelle Iconographie de la Salpêtriére, and in Brain in 1890. By this time, 38 examples had been reported.

    In September 1885 Pierre Marie6 proposed the term acro-megalie.

    “The two cases were observed in Professor Charcot’s clinic. Case I. Fusch, a woman, aged 37 single . . .At the age of twenty-four, following great fatigue and exposure in washing a house, her menstruation suddenly ceased. . . .She remained in bed three weeks; there was no pain in the joints, but she noticed, on raising herself, a weakness of the left hand, with tingling sensations in it . . .. From this time she went for three months without menstruating.

    “…she was troubled with violent pains, sometimes over the forehead, the parietal eminences, or the temples. . . .It was at the age of twenty four, at the time the menstruation suddenly ceased, that she noticed the sudden increase in her hands. Her face at this time also underwent changes, …so that when the patient returned home none of her relatives could recognize her.

    “Present condition. The whole feet are large, including the toes. Though the latter are increased in size, they have preserved their form, there is no true deformity, their appearance is simply that of a very big person.

    “The hands are very large, but of regular form; their thickness and width are relatively greater than their length, and attention is at once attracted to them on seeing the patient. . . .There is apparent atrophy of the interosseous muscles, which, according to the account of the patient, took place early in the disease. Movements of the fingers are well performed. . . .The thyroid cartilage seems increased in size, but the thyroid gland is found with difficulty.

    “The tongue is enlarged. The patient is a little deaf, and the sight is also slightly defective . . .The cranial vertex is of nearly the same size as the end of the chin. The lower jaw is well developed. . . .The patient’s thirst is intense, obliging her to beg tea of her friends in order to satisfy it. The quantity of urine is excessive; . . .there being no sugar.”

    His second patient, also a female, was aged 54 years. Menstruation had ceased abruptly when she was 29. At 30 she completely lost her eyesight. After eight months she recovered some vision but after two years, she became permanently blind. From the age of 32, though previously referred to as “la petite,” her waist, hands, and feet were getting bigger. Excessive thirst was another symptom. Marie described her “acro-megalie” in detail.

    How to identify this strange symptom puzzled Pierre Marie and his chief, Charcot. He found five similar case reports (Saucerotte (1772); Alibert (1822); Friedreich, two cases (1868); and Henrot (1877, 1882)). In Henrot’s case, at postmortem an ovoid tumour the size of a hen’s egg was found near the pituitary body. This finding anticipated Minkowski’s demonstration of pituitary enlargement in acromegaly. In 18877 Marie recognised the important association with pituitary tumours, and described recognisable features of kyphosis, thirst polyuria, and carpal tunnel syndrome.

    A sad postscript is the moving autobiography by Leonard Portal Mark, MD, himself a sufferer.8 His friends had noticed signs in his early twenties, but it was not until he was 49 when

    “during [a] French medical visit to London I was spotted in a crowd by Dr Pierre Marie as a typical acromegalic… Post tenebras lux!”

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