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ICD Hub / Sleep–wake disorders (G47) / 7A22 - Kleine-Levin syndrome
ICD-11

ICD-11 Codes
for 7A22 - Kleine-Levin syndrome
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Understanding Kleine-Levin Syndrome (ICD-11 Code 7A22)

Kleine-Levin syndrome (KLS), identified by ICD-11 code 7A22, is a rare neurological disorder characterised by recurrent episodes of severe sleepiness. These episodes are accompanied by significant cognitive, psychiatric, and behavioural disturbances. Patients typically experience prolonged periods of hypersomnia, often sleeping 16 to 20 hours per day, waking only for essential functions like eating and voiding. Between these episodes, individuals are generally asymptomatic, returning to their normal state of sleep, cognition, mood, and eating. The condition primarily affects adolescent males, though it can occur in anyone.

Key Symptoms of Kleine-Levin Syndrome

During episodes of Kleine-Levin syndrome (7A22), individuals often present with extreme sleepiness and an inability to stay awake. Beyond hypersomnia, other common symptoms include: * Increased appetite, known as hyperphagia or megaphagia. * Elevated sex drive, termed hypersexuality. * Cognitive impairments such as confusion, slow speech, and difficulty with concentration. * Behavioural and psychological changes, including irritability, childish behaviour, anxiety, depression, and sometimes hallucinations or delusions. * A feeling of unreality or derealisation. Episodes can vary in duration, typically lasting around 10 days but potentially extending to several weeks or even months. Most affected individuals have limited recall of events during an episode.

Etiology and Risk Factors for Kleine-Levin Syndrome

The exact cause of Kleine-Levin syndrome (ICD-11 code 7A22) remains unknown. Research suggests potential links to damage in the brain's sleep-regulating regions, such as the hypothalamus. Some cases follow viral infections, leading to speculation about an autoimmune response where the body mistakenly attacks healthy brain tissue. Genetic factors are also being investigated, with potential mutations in genes like LMOD3 and TRANK1 being explored. While predominantly affecting adolescent males, KLS can occur in any demographic. Triggers for episodes can include flu-like illnesses, infections, drug or alcohol use, head trauma, physical exertion, and stress.

Diagnosing Kleine-Levin Syndrome (7A22)

Diagnosing Kleine-Levin syndrome (7A22) can be challenging due to its rarity and the absence of a specific diagnostic test. Healthcare providers typically diagnose KLS by ruling out other conditions with similar symptoms. The diagnostic process involves a thorough evaluation of the patient's medical history, symptom presentation, and the duration and frequency of episodes. Tests may include: * Sleep studies (polysomnography) to monitor brain activity during sleep. * Cognitive and memory assessments. * Blood tests to exclude other medical conditions. * Neuroimaging, such as MRI, to examine brain structure.

Management and Treatment Considerations for Kleine-Levin Syndrome

Currently, there is no definitive cure or specific treatment for Kleine-Levin syndrome (ICD-11 7A22). Management focuses on addressing symptoms and supporting the patient during episodes. During an episode, accommodations such as time off from work or school are crucial to allow for the necessary extended sleep. Some medications may be used to manage specific symptoms: * **Lithium:** May help reduce the frequency of episodes. * **Stimulants or wake-promoting agents:** Can assist in managing excessive sleepiness. * **IV steroids:** Might be used to shorten prolonged episodes (over 30 days). Supportive therapy can address behavioural changes, anxiety, and depression. It is vital for patients to avoid driving or operating heavy machinery during episodes due to the risk of falling asleep.

Alternative Names for Kleine-Levin Syndrome

Kleine-Levin syndrome (7A22) is also known by several other clinical and patient-friendly terms, reflecting its unique presentation. These include: * Periodic hypersomnia syndrome * Sleeping Beauty syndrome * Familial hibernation syndrome * Hypersomnia-bulimia syndrome * Recurrent hypersomnolence

Coding and Documentation for Kleine-Levin Syndrome (7A22)

Accurate documentation and coding are essential for billing and medical records related to Kleine-Levin syndrome. The official ICD-11 code for Kleine-Levin syndrome is 7A22. When documenting, healthcare providers should include details about the episodic nature of hypersomnia, cognitive disturbances, and behavioural changes. Common clinical synonyms and patient-friendly terms like 'periodic hypersomnia syndrome' or 'excessive sleepiness episodes' can aid in comprehensive documentation. For billing and reporting purposes, the ICD-11 code 7A22 is used to identify this specific condition. This ICD-11 code for Kleine-Levin syndrome ensures precise medical record-keeping and facilitates accurate reimbursement processes.

Frequently asked questions

What is the ICD-11 classification for Kleine-Levin syndrome?
Kleine-Levin syndrome is classified under ICD-11 code 7A22, within the chapter on Sleep-Wake Disorders.
How is Kleine-Levin syndrome characterized in ICD-11?
ICD-11 defines Kleine-Levin syndrome by recurrent episodes of severe sleepiness, accompanied by cognitive, psychiatric, and behavioural disturbances. Patients are typically normal between episodes.
What is the diagnostic status of Kleine-Levin syndrome in ICD-11?
In ICD-11, Kleine-Levin syndrome is recognized as an independent diagnostic entity.
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