As a result, sexual development may be halted or delayed in children, and adults may have issues with fertility or experience a low sex drive (libido).
Hypothalamic hypogonadism can be inherited or acquired and occurs as a result of known and unknown causes.
This article discusses the symptoms, causes, diagnosis, treatment, and prognosis of hypothalamic hypogonadism (HH).
Hypothalamic Hypogonadism Symptoms
HH symptoms look very different in children and adults, so it’s important to break the groups into two separate categories.
Symptoms in children include:
Lack of development at puberty (development may be very late or incomplete) Lack of breast development and menstrual periods (in females) Delayed sexual development such as little or no enlargement of the testes and penis, deepening of the voice, and facial hair (in boys) Inability to smell (called anosmia, this is especially the case in those with Kallmann syndrome) Short stature (in some cases)
Symptoms in adults vary widely but usually include:
Loss of interest in sex (libido) in men Loss of menstrual periods (amenorrhea) in women Decreased energy and interest in activities Loss of muscle mass in men Weight gain Mood changes Infertility
Causes
HH is caused by a lack of hormones that normally stimulate the ovaries or testes. These hormones, called gonadotropins, include:
Gonadotropin-releasing hormone (GnRH) Follicle-stimulating hormone (FSH) Luteinizing hormone (LH)
In normal circumstances the brain releases GnRH via the hypothalamus, stimulating the pituitary gland to release FSH and LH. These hormones signal the sex organs—female ovaries or the male testes—to release estrogen or testosterone, which are hormones that govern a variety of physiological processes in the body including:
Sexual development in pubertyMenstruationA rise in estrogen levelsFertility in adult womenNormal testosterone productionSperm production in adult men
If the hypothalamus, pituitary gland, or the areas around it are damaged in any way, this can hinder the release of hormones, causing a lack of sex hormones. This prevents normal sexual maturity in children and hinders the function of the testicles or ovaries in adults.
There are several causes of HH:
Damage to the pituitary gland or hypothalamus from surgery, injury, tumor, infection, or radiation Genetic conditions such as Kallmann syndrome, normosmic idiopathic hypogonadotropic hypogonadism (reduced levels of sex steroid), and CHARGE syndrome High doses or long-term use of opioid or steroid (glucocorticoid) medicines High prolactin level (a hormone released by the pituitary) Severe stress Nutritional problems (both rapid weight gain or weight loss) Long-term (chronic) medical diseases, including chronic inflammation or infections Drug use, such as heroin or use or abuse of prescription opiate medicines Certain medical conditions, such as iron overload
Diagnosis
The only way to confirm the presence of hypogonadism is through an assessment of levels of sex hormones and their precursors in the blood. Note, testosterone levels should be checked in the morning for accuracy.
If low levels of estrogen or testosterone are found, your healthcare provider will measure FSH and LH levels to help troubleshoot the cause of your hypogonadism.
In hypothalamic hypogonadism, FSH and LH levels are low because the hypothalamus and pituitary do not produce these hormones. As a result, they do not stimulate the ovaries and testes to produce sex hormones.
One important aspect of the HH diagnosis is that it may reflect the presence of a tumor of the hypothalamic-pituitary region or even a systemic disease. Therefore, your healthcare provider may order an MRI of the head to check for potential anatomical abnormalities of the hypothalamus and pituitary.
Additional tests include blood tests checking:
IronThyroid hormoneProlactin levelsCortisolGrowth hormone
Genetic testing may also be done.
Treatment
In men and premenopausal women, hormone replacement therapy is the standard of care.
Male
People born with testes are treated with testosterone replacement therapy (TRT), usually in the form of an injection or slow-release testosterone patches, and/or gonadotropins replacement therapy (GRT).
If a male is seeking fertility, the treatment is human chorionic gonadotropin (HCG) injections.
Female
People born with ovaries are treated with combined gonadal hormone replacement (estrogen and progesterone pills or skin patches) or pulsatile gonadotropin-releasing (GnRH) hormone.
In women seeking pregnancy, the treatment is ovarian stimulation with LH, FSH, and HCG.
Prognosis
Most people with HH will go on to live healthy and productive lives. The key is to get your diagnosis and treatment as early as possible. The right hormone treatment will likely jumpstart puberty in a child, restore fertility in an adult, and improve any related symptoms that you may have.
Summary
HH is a medical condition that arises when the hypothalamus or pituitary glands produce little or no gonadotropin hormone. As a result the ovaries and testes don’t produce sex hormones. Fortunately, hormone replacement therapy can reverse its course in most people.
A Word From Verywell
Sometimes it’s difficult to tell if you have hypogonadism and almost impossible to know if it’s hypothalamic hypogonadism even if you suspect that you have it.
Frequent checkups with a healthcare provider are one way to stay on top of your sexual health. If you are having problems with fertility, experiencing low libido, or have had a fracture later in life (and as a result of mild trauma), this may be a sign that your body is not producing enough sex hormone. Fortunately, these symptoms are often reversed with gonadotropin or sex hormone replacement therapy.
