The levels of evidence (I-V) and levels of recommendation (standard, guideline, option) are defined at the end of the "Major Recommendations" field.
- An accurate diagnosis of a specific hypersomnia disorder of central origin should be established. This evaluation should include a thorough evaluation of other possible contributing causes of excessive daytime sleepiness. (Standard)
- Treatment objectives should include control of sleepiness and other sleep related symptoms when present. (Standard)
- The following are treatment options for narcolepsy.
- Modafinil is effective for treatment of daytime sleepiness due to narcolepsy (Standard).
- Sodium oxybate is effective for treatment of cataplexy, daytime sleepiness, and disrupted sleep due to narcolepsy. (Standard) Sodium oxybate may be effective for treatment of hypnagogic hallucinations and sleep paralysis. (Option)
- Amphetamine, methamphetamine, dextroamphetamine, and methylphenidate are effective for treatment of daytime sleepiness due to narcolepsy. (Guideline)
- Selegiline may be an effective treatment for cataplexy and daytime sleepiness. (Option)
- Ritanserin may be effective treatment of daytime sleepiness due to narcolepsy. (Option)
- Scheduled naps can be beneficial to combat sleepiness but seldom suffice as primary therapy for narcolepsy. (Guideline)
- Pemoline has rare but potentially lethal liver toxicity, is no longer available in the United States, and is no longer recommended for treatment of narcolepsy. (Option)
- Tricyclic antidepressants, selective serotonin reuptake inhibitors (SSRIs), venlafaxine, and reboxetine may be effective treatment for cataplexy. (Guideline)
- Tricyclic antidepressants, selective serotonin reuptake inhibitors (SSRIs), and venlafaxine may be effective treatment for treatment of sleep paralysis and hypnagogic hallucinations. (Option)
- Modafinil may be effective for treatment of daytime sleepiness due to idiopathic hypersomnia. (Option)
- The following medications may be effective treatments for specific types of hypersomnia due to a medical condition.
- Modafinil may be effective for treatment of daytime sleepiness due to Parkinson's disease. (Option)
- Modafinil may be effective for treatment of daytime sleepiness due to myotonic dystrophy. (Option)
- Methylphenidate may be effective for treatment of daytime sleepiness due to myotonic dystrophy. (Option)
- Modafinil may be effective for treatment of daytime sleepiness due to multiple sclerosis. (Guideline)
- Lithium carbonate may be effective for treatment of recurrent hypersomnia and behavioral symptoms due to Kleine-Levin syndrome. (Option)
- The following medications may be effective for treatment of daytime sleepiness in idiopathic hypersomnia (with and without long sleep time), recurrent hypersomnia, and hypersomnia due to a medical condition: amphetamine, methamphetamine, dextroamphetamine, methylphenidate, and modafinil. (Option)
- The following are treatment recommendations previously applied to narcolepsy only. Their application is now extended to the hypersomnias of central origin covered by this practice parameter paper by committee consensus.
- Combinations of long- and short-acting forms of stimulants may be indicated and effective for some patients. (Option)
- Treatment of hypersomnias of central origin with methylphenidate or modafinil in children between the ages of 6 and 15 appears to be relatively safe. (Option)
- Regular follow-up of patients with hypersomnia of central origin is necessary to monitor response to treatment, to respond to potential side effects of medications, and to enhance the patient's adaptation to the disorder. (Standard)
- A patient previously stabilized on stimulant medication should be seen regularly by a health care provider at least once per year, and preferably once every 6 months, to assess the development of medication side effects, including sleep disturbances, mood changes, and cardiovascular or metabolic abnormalities.
- Follow-up is necessary to determine adherence and response to treatment; to monitor for the safety of medications in individual patients; and to assist the patient with occupational and social problems.
- Patients with severe sleepiness should be advised to avoid potentially dangerous activities at home and at work, and should not operate a motor vehicle until sleepiness is appropriately controlled by stimulant medications.
- Of the stimulants used to treat hypersomnia of central origin, amphetamines, especially at high doses, are the most likely to result in the development of tolerance.
- Patients who fail to respond to adequate doses of stimulant medication should be carefully assessed for other sleep disorders that may contribute to excessive sleepiness such as insufficient sleep, inadequate sleep hygiene, circadian rhythm disorders, obstructive sleep apnea syndrome, or periodic limb movement disorder.
- For side effects, dosage ranges, use in pregnancy and by nursing mothers, and contraindications, see Tables 6 and 7 in the companion guideline document (see the "Companion Documents" field).
- Health care providers should assist the patient with occupational and social accommodation for disabilities due to hypersomnia of central origin.
- Polysomnographic re-evaluation of patients should be considered if symptoms of sleepiness increase significantly or if specific symptoms develop that suggest new or increased sleep abnormalities that might occur in disorders such as sleep apnea or periodic limb movement disorder.
Definitions:
Classification of Evidence
Level I: Randomized, well-designed trials with low alpha and beta error,* or meta-analyses of randomized controlled trials with homogeneity of result
Level II: Randomized trials with high alpha and beta error, methodologic problems, or high quality cohort studies*
Level III: Nonrandomized concurrently controlled studies (case-control studies)
Level IV: Case-control or cohort studies with methodological problems, or case series
Level V: Expert opinion, or studies based on physiology or bench research
*Alpha (type I error) refers to the probability that the null hypothesis is rejected when in fact it is true (generally acceptable at 5% or less, or P <0.05). Beta (Type II error) refers to the probability that the null hypothesis is mistakenly accepted when in fact it is false (generally, trials accept a beta error of 0.20). The estimation of Type II error is generally the result of a power analysis. The power analysis takes into account the variability and the effect size to determine if sample size is adequate to find a difference in means when it is present (power generally acceptable at 80% to 90%).
Levels of Recommendation
Standard: This is a generally accepted patient-care strategy that reflects a high degree of clinical certainty. The term standard generally implies the use of Level I Evidence, which directly addresses the clinical issue, or overwhelming Level II Evidence.
Guideline: This is a patient-care strategy that reflects a moderate degree of clinical certainty. The term guideline implies the use of Level II Evidence or a consensus of Level III Evidence.
Option: This is a patient-care strategy that reflects uncertain clinical use. The term option implies either inconclusive or conflicting evidence or conflicting expert opinion.