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Hypothalamic dysfunction (hypothalamic syndromes) 

Hypothalamic dysfunction (hypothalamic syndromes)
Chapter:
Hypothalamic dysfunction (hypothalamic syndromes)
Author(s):

M. Guftar Shaikh

DOI:
10.1093/med/9780199235292.003.2181
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date: 18 September 2018

The hypothalamus is a complex area of the brain and is important in co-coordinating signals between the nervous system and the endocrine system, primarily via the pituitary gland. Various processes throughout life, such as birth, puberty, and pregnancy, as well as neurological and psychiatric disorders are regulated by the hypothalamus (1). It influences many hormonal and behavioural circadian rhythms, as well as being involved in the control of body temperature, hunger, and thirst. Damage to the hypothalamus whether it is congenital or acquired will lead to significant clinical morbidity (Box 2.4.1.1). Recent advances in molecular techniques and improved neuroimaging, particularly MRI and positron emission tomography (PET) have given us a better understanding of hypothalamic syndromes and their clinical manifestations.

It may be very difficult to differentiate between hypothalamic and pituitary disease as the endocrine abnormalities are often similar. As the hypothalamus regulates both endocrine and autonomic function, there is usually a combination of endocrine and neurological disturbance in hypothalamic damage. This includes abnormal behaviour, eating disorders, and thermoregulation.

The hypothalamus consists of a number of different nuclei which have very specific functions and also secretion of hypothalamic hormones and neuropeptides (1). The clinical syndrome will depend on the location and extent of the underlying lesion. The lesion may be very small and only affect specific hypothalamic nuclei which will result in discrete symptoms; however larger lesions, which are more likely, will present with a variety of problems (Fig. 2.4.1.1). The endocrine abnormalities seen in hypothalamic syndromes usually result in pituitary hyposecretion; however due to loss of inhibitory factors hypersecretion can also occur.

Children and adolescents usually present with growth failure and disorders of puberty, which can be both delayed and precious. Adults with hypothalamic dysfunction can present with dementia, disturbances in appetite and sleep, as well as hormonal deficiencies. Causes of hypothalamic damage, particularly the anterior hypothalamus, include tumours such as craniopharyngiomas, optic nerve gliomas, and inflammatory conditions such as histiocytosis and sarcoidosis.

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