Symptoms of panhypopituitarism

The clinical symptoms of the disease are very variable and consist of specific symptoms of hormonal deficiency and polymorphic neurovegetative manifestations.

Panhypopituitarism is much more common in young and middle-aged women (20-40 years), but there are individual cases of the disease in earlier and older ages. The development of Sheehan's syndrome in a 12-year-old girl after juvenile uterine bleeding has been described.

In Simmonds syndrome, the clinical picture is dominated by steadily increasing weight loss, averaging 2-6 kg per month, but reaching 25-30 kg in severe, galloping disease. Due to anorexia, the volume of food consumed is significantly reduced, but the degree and intensity of weight loss in these cases are not adequate to the changed nutritional conditions and are determined by primary disorders of the hypothalamic regulation of fat metabolism and a decrease in the level of somatotropic hormone in the body.

In rare cases, along with cachexia, there is not a decrease in appetite, but a pathological increase (bulimia). Exhaustion is usually uniform, since the subcutaneous fat layer disappears everywhere, muscles atrophy, and internal organs decrease in volume. Edema, as a rule, does not occur.

Characteristic changes in the skin include dryness, wrinkling, peeling, combined with a pale yellowish, waxy color. Sometimes, against the background of general pallor, areas of dirty, earthy pigmentation appear on the face and in the natural folds of the skin. Acrocyanosis is often observed.

Trophic disorders lead to brittleness and loss of hair, early graying, atrophic processes in bone tissue with decalcification of bones and development of osteoporosis. The lower jaw atrophies, teeth are destroyed and fall out. Signs of marasmus and senile involution rapidly increase. Extreme general weakness, apathy, adynamia up to complete immobility; hypothermia develop. Patients note dizziness and frequent fainting. Orthostatic collapses and comatose states are characteristic, leading to death without specific therapy.

One of the leading places in the clinical symptoms is occupied by sexual disorders caused by a decrease or complete loss of gonadotropic regulation of the sex glands. These disorders often precede the appearance of all other symptoms. Sexual desire is lost, potency decreases, pubic and armpit hair falls out. The external and internal genitals gradually atrophy. In women, menstruation disappears early and quickly, the mammary glands decrease in size, the areolas of the nipples become depigmented. When the disease develops after childbirth, lactation is absent, and menstruation does not resume. In rare cases of a protracted and erased course of the disease, the menstrual cycle, although disrupted, is possible. In men, secondary sexual characteristics (pubic, axillary hair, moustache, beard) disappear, the testicles, prostate gland, seminal vesicles, and penis atrophy. Oligozoospermia occurs. In rare cases, alopecia can be universal, i.e. hair loss occurs on the head, eyebrows, and eyelashes.

A decrease in the production of thyroid-stimulating hormone leads to rapid or gradual development of hypothyroidism. Drowsiness, lethargy, adynamia occur, mental and physical activity decreases. Thyroid hypofunction leads to disruption of metabolic processes in the myocardium. Contractions of the heart muscle slow down, heart sounds become muffled, arterial pressure decreases. Atony of the gastrointestinal tract and constipation develop.

Impaired water excretion, typical of hypothyroidism, manifests itself differently in patients with interstitial-pituitary insufficiency. In cases of severe exhaustion, there is usually no edema, while in patients with Sheehan's syndrome and a predominance of hypogonadism and hypothyroidism symptoms, there is usually no significant weight loss, but fluid retention is significant. The face becomes puffy, the tongue thickens, dents from teeth form on its lateral surfaces, hoarseness and a lowering of the voice (swelling of the vocal cords) may occur. Speech is slow, dysarthric.

The severity of the disease and the nature of its progression (rapid or gradual) are largely determined by the degree of decline in adrenal function. Severe hypocorticism reduces the resistance of patients to intercurrent infections and various stress situations. Hypocorticism aggravates general weakness, adynamia, hypotension and contributes to the development of hypoglycemia.

The latter occurs due to a decrease in the level of both central and counter-insular hormones (ACTH, STH) and the biosynthesis of glucocorticoids in the adrenal cortex. A decrease in gluconeogenesis processes mediated by glucocorticoids leads to a drop in blood sugar levels to 1-2 mmol/l and to the development of relative hyperinsulinism, which sharply increases the sensitivity of patients to externally administered insulin. In some cases, injections of 4-5 U of the drug led to severe hypoglycemic and even comatose states.

The pathogenesis of pituitary coma at the end of Simmonds' cachexia or severe panhypopituitarism of other etiology is determined mainly by progressive hypocorticism and hypothyroidism. The comatose state develops, as a rule, gradually, with increasing adynamia, turning into stupor, hyponatremia, hypoglycemia, convulsions and hypothermia.

Hypocorticism also causes severe dyspeptic disorders: loss of appetite, reaching complete anorexia, persistent nausea and vomiting after meals or unrelated to digestion, abdominal pain as a result of spasm of the smooth muscles of the intestine. Atrophic processes in the mucous membrane with a decrease in gastrointestinal and pancreatic secretion are also characteristic.

The hypothalamic component in clinical symptoms can manifest itself as a violation of thermoregulation, most often with hypothermia, but sometimes with subfebrile temperature and vegetative crises with hypoglycemia, chills, tetanic syndrome and polyuria.

Bone decalcification, osteoporosis, peripheral nervous system disorders with polyneuritis, polyradiculoneuritis and severe pain syndrome often develop.

Mental disorders are observed in all variants of hypothalamic-pituitary insufficiency. Characteristic are decreased emotional activity, indifference to the environment, depression and other mental deviations up to schizophrenia-like hallucinatory-paranoid psychosis. Hypothalamic-pituitary insufficiency caused by a tumor of the pituitary gland or hypothalamus is combined with a number of symptoms of increased intracranial pressure: ophthalmological, radiological and neurological (headaches, decreased visual acuity and limitation of visual fields).

The course of the disease may vary. In patients with cachexia and predominant hypocorticism symptoms, progression of all symptoms is observed, leading to a lethal outcome in a short time. In Sheehan syndrome, on the contrary, the disease develops gradually, gradually, sometimes remaining undiagnosed for many years.

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