About babinski-froelich syndrome

What is babinski-froelich syndrome?

Froehlich syndrome is a constellation of endocrine abnormalities believed to result from damage to the hypothalamus, a part of the brain where certain functions such as sleep cycles and body temperature are regulated. Froehlich syndrome appears to be acquired while certain other disorders that resemble it, such as Prader-Willi syndrome, are genetic.

This syndrome appears to affect males mostly. The more obvious and frequently encountered characteristics are delayed puberty, small testes, and obesity. Teenage boys with this disorder must be distinguished from those who have inherited growth delay disorders or Prader Willi syndrome.

What are the symptoms for babinski-froelich syndrome?

Irregulated body temperature symptom was found in the babinski-froelich syndrome condition

Froehlich syndrome is characterized by increased or excessive eating that leads to obesity, small testes, and a delay in the onset of puberty. It is also common for children with Froehlich syndrome to experience the delay in physical growth and the development of secondary sexual characteristics. In addition to delayed growth and puberty, children with this syndrome tend to be short in stature. As a result of tumor growth, some children with Froehlich syndrome may also develop intellectual difficulties, poor vision – due optic nerve damage-, somnolence, and diabetes insipidus – known as Infundibulo-tuberal syndrome (Froehlich+somnolence+diabetes insipidus), lower than normal body temperature (hypothermia) and very delicate skin.

What are the causes for babinski-froelich syndrome?

Froehlich syndrome (hypogonadism with obesity) results from an injury of a part of the hypothalamus (arcuatus nucleus and ventromedial nueclei. The hypothalamic gland is the endocrine gland that produces substances that stimulate the pituitary and regulate appetite. In Froehlich syndrome, the front portion (anterior) of the pituitary gland fails to secrete the hormones that are necessary for the onset of normal puberty.

The most frequent cause of Froehlich syndrome is a specific tumor of the pituitary-hypothalamus area, an expanding hollow (cystic) lesion (craniopharyngioma) or its surgical treatment (injury of this area during surgery).

Lesions resulting from inflammation from an infection such as tuberculosis or an acute inflammation of the brain (encephalitis) can also be responsible for causing Froehlich syndrome.

What are the treatments for babinski-froelich syndrome?

Pituitary extracts may be administered through hormone replacement therapy (HRT) to replace the missing hormones in patients with Froehlich syndrome. For males, this usually includes administration of human chorionic gonadotropin to reach puberty followed up by testosterone. For females, HRT typically is the administration of estrogen during teenage years followed by complex estrogen-progestin therapy with age. If possible, tumors of the hypothalamus should be surgically removed if. Though appetite may be very difficult to manage, weight control depends on successful management. Some modern treatments can help to control obesity.

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