What Is Kleine-Levin Syndrome (KLS)

What Is Kleine-Levin Syndrome (KLS) ?

Kleine-Levin syndrome (also known as KLS or just LSS) is a rare genetic disorder that affects the nervous system. It is characterized by muscle weakness, paralysis, and eventually death. The disease results from mutations in a gene called klebsiella pneumoniae. People with this condition have two copies of the mutated gene for one copy of the normal protein. The mutation occurs during fetal development and is passed down through families.

The most common form of the disease is KLS-I, which affects approximately 1 in every 10,000 births. Approximately 3% of cases are diagnosed as KLS-II, which affects approximately 1 in every 100,000 births. There is no cure for either type of the disease.

Symptoms of KLS-I :

Weakness on one side of your body, usually your left arm or leg. You may not be able to do any physical activity. Your muscles will often become stiff and painful. Weakness in both arms or legs is common, but sometimes only one limb becomes weak.

You may lose control over what you can do, such as walking without help or using a wheelchair.

Paralysis of your eye muscles may develop. You may become blind in one or both eyes. This happens in about 30% of people with the disease.

You may experience a loss of sensation, such as feeling no pain when you touch a hot object.

Vision changes such as blurry vision or double vision may occur.

Optic neuritis, which is decreased vision or loss of vision due to a disorder of the optic nerve, may occur.

Symptoms of KLS-II :

Depression, excessive sleep, tiredness, and lack of energy are common in both types of the disease. Depressive symptoms are more severe than those experienced in KLS-I. These symptoms may be severe enough to cause problems with friends and family. They can also lead to suicide attempts.

This is more likely if you have an underlying mental illness, which is present in a small number of people.

Confusion, personality changes, and hallucinations are also possible. You may see or hear things that aren’t there or have a hard time concentrating. Some people may experience delusions or paranoia.

Personality changes are common during the teenage years, which may be when the disease begins in KLS-II.

As with KLS-I, weakness in one limb is common, but may occur in both. This weakness may be mild or severe and may affect one limb, both limbs, or your entire body. You may or may not have problems using your muscles.

Rapid weight gain occurs in some people with KLS-II. They gain up to 20 pounds overnight. This weight gain can occur several times throughout the course of the disease.

Seizures of various types can occur, but they are rare. Most people never experience a seizure. The most common type of seizure is a generalized tonic-clonic (formerly known as grand mal) seizure. A person having this type of seizure typically loses consciousness, the body stiffens, and shaking movements may occur.

In some cases, only the arms or legs jerk instead of the entire body. Other types of seizures, such as absence (formerly known as petit mal) seizures, may occur.

Diabetes insipidus is a condition in which the kidneys are unable to conserve water properly. A person with this condition excretes large amounts of urine and becomes thirsty frequently. This condition is life-threatening unless treated. It is most often caused by damage to the pituitary gland and is more common in children.

Sources & references used in this article:

Pharmacological treatment for Kleine‐Levin syndrome by MM Oliveira, C Conti, H Saconato… – Cochrane Database of …, 2009 – cochranelibrary.com

The Kleine-Levin syndrome. Report of a case and review of the literature by SS Papacostas, V Hadjivasilis – European psychiatry, 2000 – Elsevier

Kleine-Levin syndrome following acute viral encephalitis by AE Merriam – Biological psychiatry, 1986 – biologicalpsychiatryjournal.com