From the Guidelines
Diagnosing and managing narcolepsy requires a systematic approach, starting with a comprehensive clinical evaluation to assess excessive daytime sleepiness and other symptoms, followed by objective testing, and individualized treatment based on symptom severity, comorbidities, and patient preferences, as recommended by the American Academy of Sleep Medicine guidelines 1.
Diagnosis
Diagnosis begins with a comprehensive clinical evaluation to assess excessive daytime sleepiness and other symptoms like cataplexy, sleep paralysis, and hypnagogic hallucinations. This should be followed by objective testing including an overnight polysomnography (PSG) to rule out other sleep disorders, and a Multiple Sleep Latency Test (MSLT) performed the day after the PSG, which should show a mean sleep latency of ≤8 minutes and ≥2 sleep-onset REM periods. For narcolepsy type 1, cerebrospinal fluid hypocretin-1 levels may be measured, with levels ≤110 pg/mL supporting diagnosis 1.
Management
Management includes both non-pharmacological and pharmacological approaches. Non-pharmacological strategies involve scheduled daytime naps, good sleep hygiene, and regular sleep schedules.
- Pharmacological treatment for excessive daytime sleepiness includes:
- Modafinil (200-400 mg/day)
- Armodafinil (150-250 mg/day)
- Pitolisant (8.9-35.6 mg/day)
- Solriamfetol (75-150 mg/day)
- Traditional stimulants like methylphenidate (10-60 mg/day) or amphetamines (5-60 mg/day) 1
- For cataplexy, sodium oxybate (6-9 g/night in two divided doses), pitolisant, or antidepressants like venlafaxine (75-300 mg/day) or fluoxetine (20-60 mg/day) are recommended 1. Treatment should be individualized based on symptom severity, comorbidities, and patient preferences, with regular follow-up to assess efficacy and side effects, as clinicians should individualize treatment selections based on patients’ age, pregnancy status and reproductive planning, comorbidities, and goals of care 1.
From the Research
Diagnosis of Narcolepsy
The diagnosis of narcolepsy involves a combination of clinical evaluation, sleep studies, and laboratory tests. According to the American Academy of Sleep Medicine (AASM) guidelines, the following steps are recommended for diagnosing narcolepsy:
- Clinical history: A comprehensive clinical history is essential to diagnose narcolepsy, including excessive daytime sleepiness, cataplexy, sleep paralysis, and hypnagogic/hypnopompic hallucinations 2, 3.
- Polysomnogram (PSG): A PSG is a sleep study that measures various physiological activities during sleep, including brain waves, muscle tone, and eye movements. It is used to rule out other sleep disorders that may cause excessive daytime sleepiness 2, 3, 4.
- Multiple Sleep Latency Test (MSLT): The MSLT is a daytime sleep study that measures how quickly a person falls asleep during the day. It is used to assess excessive daytime sleepiness 2, 3, 5.
- Cerebrospinal fluid hypocretin levels: Measuring cerebrospinal fluid hypocretin levels can help confirm the diagnosis of narcolepsy, especially in patients with cataplexy 2.
Management of Narcolepsy
The management of narcolepsy involves a combination of non-pharmacological and pharmacological treatments. The following are some of the recommended treatments:
- Non-pharmacological treatments:
- Pharmacological treatments:
- Stimulants: Amphetamine-like and non-amphetamine-like stimulants, such as modafinil, can help improve alertness and reduce excessive daytime sleepiness 2, 3, 6.
- Sodium oxybate: Sodium oxybate can help improve cataplexy, sleep paralysis, and hypnagogic/hypnopompic hallucinations 2, 6.
- Antidepressants: Selective serotonin and norepinephrine reuptake inhibitors can help improve cataplexy and other symptoms of narcolepsy 2, 6.