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| Disease found: | Narcolepsy Type 1 |
| Current as of: | October 8, 2024 |
| Disease Overview: | NT1 is a less common variant of narcolepsy driven by deficiency/loss of hypocretin-producing neurons in the lateral hypothalamus [more]. |
| Signs and Symptoms: | Patients with NT1 experience excessive daytime sleepiness, at times including sudden attacks of drowsiness, with cataplexy (sudden, extreme muscle weakness), sleep paralysis, and hallucinations during sleep-wake transitions [more]. |
| Diagnosis: | NT1 can be diagnosed clinically (excessive daytime sleepiness plus documented cataplexy); sleep study testing demonstrating mean sleep latency <8 min or at least two sleep-onset REM periods during sleep latency testing or polysomnography are also indicative of the disease [more]. |
| Treatment: | Lifestyle modifications are recommended as first-line therapy, including scheduled napping, regular sleeping hours, diet assessment [more]. If symptoms are uncontrolled without medication, stimulants (particularly modafinil) are a first-line approach for daytime sleepiness; sodium oxybate is approved for cataplexy [more]. |
| Clinical Management: | Patients should be counseled that lifestyle modifications generally must be continued to maintain symptom control [more]. Narcolepsy is associated with increased cardiovascular risk; regular cardiovascular assessment and low threshold to treat is warranted [more]. Mood disorders including suicidal ideation is more common in patients with poor symptom control [more]. |
| Referral: | Patients should be referred to a provider with experience in sleep disorders. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health. |
| Clinical Trials: | Several trials are currently recruiting for Narcolepsy Type 1. |