Overview

Frohlich syndrome, also known as adiposogenital dystrophy, is a rare acquired endocrine disorder caused by lesions, tumours or damage to a region of the brain called the hypothalamus.¹ The hypothalamus is important for regulating many of the body’s natural processes, such as body temperature, appetite and sleeping patterns. As well as this, it interacts with and regulates another important gland in your brain called the pituitary gland. The pituitary gland releases many hormones that are vital for bodily functions, so damage to the hypothalamus will also affect the functioning of this gland.²

Due to the complex nature of this Frohlich syndrome, it manifests in many ways with many symptoms. Commonly affecting males, symptoms of Frohlich syndrome include overeating, obesity, small testes, stunted growth and delayed puberty. Patients can also experience intellectual and sensory disabilities as well as suffer from metabolic conditions as a result of the condition.² Frohlich’s syndrome, although similar to Prader-Willi syndrome, is an acquired condition that cannot be passed on to offspring unlike Prader-Willi syndrome, which is inherited.³ Whilst Frohlich syndrome is a significantly life-altering disease there are therapies available to both treat the cause of the disease and to manage the symptoms.

Treatments targeting the cause of Frohlich Syndrome

Frohlich syndrome can be caused by tumours in the hypothalamus of the brain; these tumours alter the function of the hypothalamus (and the pituitary gland by association), and thus best interventions in this instance involve the treatment and removal of the tumour itself. This can be done by surgical resection of the tumour or by radiation treatment.² Whilst surgery is preferable if possible,² hypothalamic surgery carries a number of risks with it, as do all surgeries. These risks include bleeding, infection and reaction to anaesthesia.² Radiation therapy is another method that may be used to remove the tumour that may be causing the symptoms of Frohlich syndrome, and if successful, will alleviate the patient’s symptoms.⁴ As with surgery, radiation therapy does not come without risks. Side effects of radiotherapy include tiredness, hair loss and nausea.⁵

Hormone replacement therapy as a treatment for Frohlich Syndrome

Sex hormone replacement

Dysfunction of the hypothalamus and pituitary gland in patients suffering from Frohlich syndrome leads to low levels of hormones called Gonadotropins.⁶ Gonadotropins are hormones that regulate reproductive processes and stimulate the release of other sexual hormones like testosterone, oestrogen and progesterone.^7,8 Lower levels of these gonadotropin hormones lead to a delayed onset of puberty and underdeveloped sexual organs such as testes and ovaries.⁹ For males with Frohlich syndrome, the treatment to correct this involves giving human chorionic gonadotropin (hCG) to allow puberty to be reached and then giving the patient testosterone. For females, hormone replacement therapy with oestrogen is given first whilst a teenager and then a combination of oestrogen and progesterone is given as the patient matures.²

Managing obesity and metabolic conditions caused by Frohlich syndrome

Lifestyle and dietary approaches

For the average person, managing weight is a careful balance between diet and exercise, and this is even more so the case with those suffering from Frohlich syndrome. The hypothalamus is what regulates appetite in a healthy human, it acts as the control centre for hunger and satiety.¹⁰ In those people with Frohlich syndrome, damage to the hypothalamus by lesions, infections, or tumours means that the signalling involved in feeling either hungry or full is distorted. This causes overeating and, by extension, obesity. ²

The regulation of appetite can be difficult to manage, however, problems with weight can be managed. Diet plans and exercise plans created by dieticians together with the patient should be crafted, allowing a personalised and tailored approach to weight loss and weight management. With a sustainable diet and exercise plan to keep the patient at a healthy weight, this should also reduce the risk of acquiring other conditions as a result of obesity.⁹

Pharmacological approaches

As well as weight gain, type 2 diabetes, and insulin resistance are other complications that can arise with Frohlich syndrome. Modern treatment interventions such as GLP1 analogues can be used to manage blood glucose levels, as well as weight loss and suppress appetite.² GLP1 analogues are a new class of drugs that mimic a natural hormone in the body; this hormone is called glucagon-like peptide 1 (GLP1). GLP1 analogues lower blood glucose and treat type 2 diabetes, but they have also been shown to cause weight loss and appetite suppression in patients they would be a good treatment option for those with Frohlich syndrome.¹¹

Other diabetes treatments that may be used include Metformin. Metformin is another drug that treats type 2 diabetes. Its mechanism of action is complicated but involves lowering blood glucose levels by reducing glucose production in the liver and counteracting the effects of glucagon (a hormone that functions to increase blood glucose levels).¹² Metformin is another pharmacological intervention that can be used to treat the complications of Frohlich syndrome.

Bariatric surgery for weight management

In severe cases of obesity from Frohlich syndrome, bariatric surgery may be considered. Gastric banding or gastric bypass surgery can be used to maintain a healthy weight.⁸ Gastric band surgery involves the surgeon placing a band around your stomach, which restricts its capacity and slows the digestion of food. ¹³ Gastric bypass surgery involves the surgeon ‘rerouting’ the way the food flows through the stomach to the small intestine, this will reduce food intake and will lead to weight loss.¹⁴

Psychological support and follow-up care

Counselling and support groups

Whilst medications, treatments and surgeries may be used to address the physical symptoms of Frohlich syndrome, it is important to consider the mental implications of this condition. For patients with Frohlich syndrome, their physical appearance may vastly differ from that of their peers, which may cause problems with body image and low self-esteem. In these instances, counselling may provide a substantial benefit for the patient’s mental health and self-worth. After diagnosis, medical professionals may be able to signpost to various charities and support groups that can provide support for both the patients and their families. 

Follow-up care

In the long term, a Frohlich syndrome patient’s care plan must be regularly monitored and assessed. Hormone replacement is a common treatment avenue for these patients, but levels of hormones in the blood need to be monitored and dosages updated regularly.² Weight is another aspect that requires constant monitoring to ensure the patient doesn’t experience any further complications, as well as regular blood tests to check for diabetes, such as the HbA1c test.¹⁵

Summary

Whilst Frohlich syndrome is both a rare and complex condition, there are still many treatment options available that involve both targeting the root cause, managing the symptoms and treating the complications as they arise. All of these strategies together mean that the patient’s quality of life can be vastly improved. As well as this, there is also psychological and emotional support available in the form of counselling and support groups. As always, please speak to a medical professional who can give the best medical advice and can signpost you to local and regional support groups that may be able to help you.