Kleine-Levin Syndrome
Kleine-Levin Syndrome (KLS)
Definition & Epidemiology
Kleine-Levin Syndrome is a rare neurological disorder characterized by recurrent episodes of hypersomnia accompanied by cognitive, behavioral, and psychiatric disturbances. Prevalence: 1-5 cases per million population. Male predominance (70%), typical onset during adolescence.
Pathophysiology
- Hypothalamic Dysfunction
- Altered sleep-wake regulation
- Disrupted appetite control
- Temperature regulation abnormalities
- Potential Mechanisms
- Autoimmune factors
- Genetic predisposition
- Inflammatory processes
- Neurotransmitter imbalances
Clinical Features
Core Symptoms (During Episodes)
- Hypersomnia
- Excessive sleep (15-21 hours/day)
- Difficulty staying awake
- Sleep inertia
- Vivid dreams
- Cognitive Changes
- Confusion
- Derealization
- Altered perception
- Memory problems
- Decreased concentration
- Behavioral Abnormalities
- Hyperphagia (66%)
- Hypersexuality (53%)
- Disinhibition
- Altered speech
- Childlike behavior
Episode Characteristics
- Duration
- Typically 1-3 weeks
- Range: few days to months
- Abrupt onset and resolution
- Frequency
- Variable (1-12 episodes/year)
- Median: 3.5 episodes/year
- Unpredictable occurrence
Common Triggers
- Infections
- Sleep deprivation
- Alcohol consumption
- Physical stress
- Emotional stress
- Head trauma
- Travel/jet lag
Diagnostic Approach
Diagnostic Criteria (ICSD-3)
- Essential Features
- Recurrent episodes of severe hypersomnolence
- Episodes last 2 days to 5 weeks
- Episodes recur at least once per year
- Alert and normal cognition between episodes
- Not better explained by other disorders
Clinical Assessment
- Detailed History
- Episode characteristics
- Trigger identification
- Family history
- Development history
- Physical Examination
- Neurological assessment
- Psychiatric evaluation
- General physical exam
Diagnostic Testing
- Sleep Studies
- Polysomnography
- Multiple sleep latency test
- Actigraphy
- Neuroimaging
- MRI brain
- SPECT (during episodes)
- PET studies
- Laboratory Tests
- Complete blood count
- Metabolic panel
- Thyroid function
- Inflammatory markers
- Autoimmune screening
Treatment Strategies
Acute Episode Management
- Supportive Care
- Safe environment
- Supervision
- Hydration maintenance
- Nutrition support
- Pharmacological Options
- Stimulants
- Modafinil
- Methylphenidate
- Amphetamines
- Mood Stabilizers
- Lithium
- Valproate
- Carbamazepine
- Stimulants
Preventive Strategies
- Lifestyle Modifications
- Regular sleep schedule
- Stress management
- Trigger avoidance
- Exercise regulation
- Prophylactic Medications
- Lithium (most evidence)
- Antiepileptics
- Antidepressants
Research & Future Perspectives
Natural History
- Disease Course
- Median duration: 8-14 years
- Spontaneous resolution in most cases
- Decreasing frequency with age
- Prognostic Factors
- Age of onset
- Episode frequency
- Episode duration
- Treatment response
Current Research Areas
- Pathophysiology Studies
- Genetic factors
- Autoimmune markers
- Neuroimaging patterns
- Biomarker identification
- Treatment Trials
- Novel medications
- Combination therapies
- Preventive strategies
Impact on Life
- Academic/Occupational
- School/work disruption
- Career impact
- Achievement delays
- Psychosocial
- Relationship effects
- Social isolation
- Emotional burden
