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Neurogenic Precocious Puberty: Causes, Symptoms, and Treatment Options

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Attaining puberty at an early age is called precocious puberty. Hypothalamic hamartoma is considered a common cause of the same. Read the article to know more.

Medically reviewed by

Dr. Sugreev Singh

Published At December 21, 2023
Reviewed AtDecember 21, 2023

Introduction:

The fast growth of bones and muscles, changes in body shape and size, and early development of the reproductive system are called precocious puberty. The exact cause is not known. Some conditions like infections, tumors, brain defects, and hormone disorders rarely cause precocious puberty. Neurogenic precocious puberty is a neuroendocrine disease that involves hormonal abnormalities.

What Is Precocious Puberty?

Puberty is a stage where a child steps into adulthood. Based on gonadotropins, precocious puberty can be classified into central or true precocious puberty and peripheral or pseudo-precocious puberty. Puberty is attained at the early age of eight years in girls and nine years among boys in the case of precocious puberty.

  • Central Precocious Puberty (CPP): It occurs due to premature activation of the hypothalmopituitary-gonadal axis and is isosexual (early appearance of secondary sexual characteristics). CPP is the most commonly occurring and generally seen among 50 % of children and is more frequently observed in girls. Noticed at the age of four to eight years. CPP starts due to the secretion of gonadotropin hormones. These hormones include luteinizing hormone (LH) and follicle-stimulating hormones (FSH). CPP is also called neurogenic central isosexual precocious puberty (CIPP). According to research, the most common cause of CIPP is hypothalamic hamartoma (noncancerous tumor) which is most common among boys than girls.

  • Pseudo-Precocious Puberty (PPP): It occurs as a result of the production of sex steroids and is not associated with the hypo thalamo pituitary-gonadal axis. It may be isosexual or heterosexual. PPP is not dependent on gonadotropins. It is dependent on male androgens and female estrogens.

What Are the Symptoms of Precocious Puberty?

Symptoms of precautious puberty are as follows :

  • Breast enlargement and the start of the menstrual cycle in girls.

  • Enlarged testicles and penis, growth of hair on the face, and voice change in boys.

  • Growth of pubic or underarm hair.

  • Fast growth.

  • Acne.

  • Change of body odor.

What Are the Causes of Precocious Puberty?

To know about the causes of precocious puberty, we need to know about the gonadotropin-releasing hormone (GnRH). The brain releases GnRH. When GnRH reaches the pituitary gland (the endocrine gland that helps in growth metabolism and reproduction), it secretes more hormones in the ovaries for females (estrogen) and testicles for males (testosterone). Estrogen and testosterone are responsible for the growth and development of the reproductive system of females and males, respectively. Based on GnRH dependency, precocious puberty is divided into two types;

CPP: In CPP cause may not be pointed out. The majority of children with this condition show normal steps and patterns of the process, except that puberty starts earlier than usual time. These children did not show any underlying medical conditions or other reasons for early puberty. Some causes of CPP in rare conditions include;

  • Tumors in the brain or spinal cord.

  • Defects in the brain present at birth, like excess fluid buildup (hydrocephalus) or a noncancerous tumor (hamartoma).

  • Radiation to the brain or spinal cord.

  • Injury to the brain or spinal cord.

  • McCune-Albright syndrome (a rare disorder that affects the bones, skin, and many tissues that produce hormones).

  • Congenital adrenal hyperplasia (a group of genetic disorders affecting adrenal glands present above the kidney).

  • Hypothyroidism (the thyroid gland does not produce enough hormones or underactive thyroid gland).

  • Other causes of neurogenic CIPP are neuro tuberculosis, pituitary adenoma, hydrocephalus, post-radiotherapy, central nervous system (CNS) tumors, and malformations.

PPP: The cause of PPP is due to estrogen and testosterone hormones produced in the body. PPP is less common in nature and occurs without involving the hormone GnRH. Increased estrogen and testosterone are due to problems related to ovaries, testicles, adrenal glands, or pituitary glands.

Some of the causes of PPP include;

  • A tumor of the adrenal glands or pituitary gland.

  • McCune-Albright syndrome.

  • Exposure to creams and ointments, which are sources of estrogen or testosterone.

  • Ovarian tumors and ovarian cysts may cause PPP among girls.

  • Tumors of germ cells (which make sperm cells) or Leydig cells (which make testosterone) among boys.

  • Gonadotropin-independent familial sexual precocity (a rare disease caused due to a defect in the gene among boys of age one-four years).

What Are the Risk Factors of Precocious Puberty?

The risk factors of precocious puberty are as follows-

  • Females are affected more.

  • African-Americans are affected more.

  • Obese children are more prone to this condition.

  • Getting exposure to sources containing sex hormones.

  • Complications of McCune-Albright syndrome or congenital adrenal hyperplasia.

  • Hypothyroidism.

  • Radiation therapy for tumors, leukemia, and other conditions may increase the risk.

What Are the Complications of Precocious Puberty?

Complications of precocious puberty are as follows:

  • Short Height: Children having precocious puberty grow fast, but as their bones mature early, they stop growing and become short.

  • Social and Emotional Problems: These children will be more conscious as their body changes will differ from their peers. This may lead to depression affecting their self-esteem.

How to Prevent Precocious Puberty?

  • Gender and race cannot be prevented, but factors like sources of estrogen and testosterone like ointments, creams, medications, and dietary supplements should be avoided.

  • Maintaining a healthy diet, regular exercise and good sleep in children may prevent precocious puberty.

How to Diagnose Precocious Puberty?

  • Family history and symptoms of precocious puberty are checked.

  • Physical examination of the child by a physician.

  • Blood tests are advised to measure hormones like luteinizing hormone (LH), follicle-stimulating hormone (FSH), estrogen, testosterone, GnRH, and thyroid hormones.

Other tests include:

  • X-rays of the left hand and wrist using less amount of radiation.

  • Ultrasound (Sonography): Sound waves are used to make images on computers of blood vessels, tissues, and organs. Done to see adrenal glands and ovaries, or testes.

  • Magnetic Resonance Imaging (MRI): This is an imaging procedure using large magnets and computers to obtain images of tissues.

How to Treat Precocious Puberty?

Treatment depends on the cause of precocious puberty. In some cases, the cause is not known. In such instances, the child’s doctor may observe the child for several months. The child may not need the treatment. Treatment of precocious puberty can be done based on the type and symptoms to stop the onset of early puberty. Synthetic GnRH agonists can be used in CPP (gonadotropin dependent). These stop the maturity of the reproductive system by stopping the pituitary gland, which secretes gonadotropin hormones. If the cause of precocious puberty is due to an underlying medical cause, then it should be treated.

Conclusion:

Stepping into puberty is a precious and important stage of one’s life and parents should be cautious about their children at this juncture. If they find any abnormal changes occurring in their child they should seek help from their doctor. Early identification always helps in treating better. Hence it is essential to have a thorough knowledge of precocious puberty in detail as it can help children boost their self-esteem and seek help from a child counselor.

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Dr. Sugreev Singh
Dr. Sugreev Singh

Internal Medicine

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