Rarer disorders

Empty Sella Syndrome

This condition occurs when pituitary tissue is destroyed without undergoing pituitary surgery or Radiotherapy, but there is no evidence of a Pituitary tumour. The initial cause of empty sella syndrome may not be clear and may have occurred much earlier.

Familial Multiple Endocrine Neoplasia Type 1 (MEN1)

Familial multiple endocrine neoplasia type 1 is an inherited disorder affecting the endocrine glands. The disorder affects both males and females equally. MEN1 is also sometimes called Wermer Syndrome.

People with MEN1 carry a gene which makes them prone to the development of pituitary tumours, parathyroid disease, tumours in the pancreas and occasionally tumours in the other endocrine glands. The parathyroids are the glands most often affected by MEN1 and the pituitary becomes overactive in about 1 in 6 persons. The cause of pitutary overactivity is usually a Benign tumour called a prolactinoma.

A support group exists specifically for those with Multiple Endocrine Neoplasia.  For more information, contact The Pituitary Foundation on helpline@pituitary.org.uk or visit the AMEND website at:  www.amend.org.uk.

Kallmann's Syndrome

Kallmann's syndrome is a form of hypogonadotrophic Hypogonadism characterised by an absence of GnRH, a hormone naturally released by the Hypothalamus. Sufferers fail to go through puberty unless they are given sex hormone replacement therapy and in addition usually have no sense of smell (anosmia).

A support group exists specifically for those with hypogonadotrophic hypogonadism. For more details contact The Pituitary Foundation on helpline@pituitary.org.uk or visit the HYPOHH website.

Pituitary Infarction

If the blood supply to the pituitary is restricted, the gland tissue can die resulting in hypopituitarism. Infarctions can occur as a result of impaired blood flow to the Pituitary gland or head trauma.

Rathke's Cleft Cysts

Rathke's cleft cysts are not tumours, but instead are classified as developmental abnormalities. Like craniopharyngiomas, these cysts form during early development of the foetus from a structure known as Rathke's pouch. Small Rathke's cleft cysts are not uncommon and do not usually cause any symptoms. Problems can occur if these cysts enlarge and interfere with pituitary production or exert pressure on the optic chiasm.

Septo-Optic Dysplasia (SOD)

Septo-optic dysplasia is a rare condition affecting both children and adults which consists of three main abnormalities. These are as follows:

Abnormal eye development

Occasionally one or both eyes may be abnormal or the nerves connecting the eyes to the brain may be abnormal.

Abnormal development of the front part of the brain (forebrain)

Structures called the septum pellucidum and the corpus callosum which divide the forebrain into two may be absent.

Abnormal pituitary gland development

The pituitary gland produces a number of chemicals called hormones controlling growth and development in children. Additionally, the posterior part of the pituitary produces a hormone called Vasopressin which makes sure that fluid is retained in the body and patients do not become dehydrated. The pituitary gland is very much a master gland which controls other glands in the body such as the adrenal and Thyroid Gland.

Adults and children with SOD may have any of these features. Most have two out of the three cardinal features. Approximately one third will have all three problems.

Sheehan's Syndrome

The pituitary gland doubles in size during normal pregnancy. Under these circumstances a sudden drop in blood pressure can result in pituitary infarction leading to pan-hypopituitarism.

Wolfram Syndrome

Consists of diabetes insipidus, diabetes mellitus, optic atrophy and deafness. Also known by the acronym DIDMOAD. It is usually a familial disorder.

Resources available from The Pituitary Foundation


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Last Updated ( Monday, 28 July 2008 )