Table of Contents

• Burden of Disease Overview
• Diagnosis, Coding, and Assessment
• DSM-5 Narcolepsy Diagnostic Criteria
  DSM-5 Narcolepsy Severity Criteria
  DSM-5 Narcolepsy Subtypes – Diagnostic Criteria and Coding
  ICSD-3 Hypersomnia Disorders – Diagnostic Criteria and Coding
  ICSD-3 Narcolepsy Diagnostic Criteria
  • ICSD-3 Narcolepsy Diagnostic Criteria: Narcolepsy Type 1
    ICSD-3 Narcolepsy Diagnostic Criteria: Narcolepsy Type 2
• Suggested Clinical Interview Topics for Decision Support in the Diagnosis and Assessment of Narcolepsy
• Clinical Management
  • Sample Health Plan Diagnosis and Treatment Algorithm for Decision Support in Narcolepsy
    Sample Health Plan Care Coordination and Medical Management Hierarchy
    Behavioral Approaches for the Management of Narcolepsy
    
• Pharmacy Coverage and Benefit Design
  • Pharmacotherapies Available for the Management of Narcolepsy
    Sample Monograph Template for P&T Review and Benefit Design Consideration
    Medication Therapy Management: Medical Contraindications and Potential Drug Interactions Associated with Pharmacotherapies Available for the Management of Narcolepsy
    
• Abbreviations and References

Burden of Disease Overview

Effective Treatment in Managed Care Is Hindered by Under-Recognition and Misdiagnosis^1,2,3

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Diagnosis, Coding, and Assessment

DSM-5* NARCOLEPSY DIAGNOSTIC CRITERIA⁴

1. Recurrent periods of an irrepressible need to sleep, lapsing into sleep, or napping occurring within the same day. These must have been occurring at least 3 times per week over the past 3 months.
2. The presence of at least one of the following:
   1. Episodes of cataplexy, defined as either (a) or (b), occurring at least a few times per month:
      1. In individuals with long-standing disease, brief (seconds to minutes) episodes of sudden bilateral loss of muscle tone with maintained consciousness that are precipitated by laughter or joking.
      2. In children or in individuals within 6 months of onset, spontaneous grimaces or jaw-opening episodes with tongue thrusting or a global hypotonia, without any obvious emotional triggers.
   2. Hypocretin deficiency, as measured using CSF (cerebrospinal fluid) hypocretin-1 immunoreactivity values (≤⅓ of values obtained in healthy subjects tested using the same assay, or ≤110 pg/mL).
      1. Low CSF levels of hypocretin-1 must not be observed in the context of acute brain injury, inflammation, or infection.
   3. Nocturnal sleep polysomnography showing REM (rapid eye movement) sleep latency ≤15 minutes, or an MSLT (multiple sleep latency test) showing a mean sleep latency ≤8 minutes and ≥2 SOREMPs (sleep onset rapid eye movement periods).

*Diagnostic and Statistical Manual of Mental Disorders, 5th Edition

DSM-5 NARCOLEPSY SEVERITY CRITERIA⁴

MILD

Infrequent cataplexy (less than once per week), need for naps only once or twice per day, and less disturbed nocturnal sleep.

MODERATE

Cataplexy once daily or every few days, disturbed nocturnal sleep, and need for multiple naps daily.

SEVERE

Drug-resistant cataplexy with multiple attacks daily, nearly constant sleepiness, and disturbed nocturnal sleep (ie, movements, insomnia, and vivid dreaming).

DSM-5 NARCOLEPSY SUBTYPES – DIAGNOSTIC CRITERIA AND CODING⁴

Including ICD-9 code (ICD-10 code in parentheses)
ICD: International Classification of Diseases

347.00 (G47.419)

Narcolepsy without cataplexy but with hypocretin deficiency: Criterion B requirements of low CSF hypocretin-1 levels and positive PSG (polysomnography)/MSLT are met, but no cataplexy is present (Criterion B1 not met).

347.01 (G47.411)

Narcolepsy with cataplexy but without hypocretin deficiency: In this rare subtype (less than 5% of narcolepsy cases), Criterion B requirements of cataplexy and positive PSG/MSLT are met, but CSF hypocretin-1 levels are normal (Criterion B2 not met).

347.00 (G47.419)

Autosomal dominant cerebellar ataxia, deafness, and narcolepsy: This subtype is caused by exon 21 DNA (cytosine-5)-methyltransferase-1 mutations and is characterized by late-onset (age 30-40 years) narcolepsy (with low or intermediate CSF hypocretin-1 levels), deafness, cerebellar ataxia, and eventually dementia.

347.00 (G47.419)

Autosomal dominant narcolepsy, obesity, and type 2 diabetes: Narcolepsy, obesity, and type 2 diabetes and low CSF hypocretin-1 levels have been described in rare cases and are associated with a mutation in the myelin oligodendrocyte glycoprotein gene.

347.10 (G47.429)

Narcolepsy secondary to another medical condition: This subtype is for narcolepsy that develops secondary to medical conditions that cause infectious (eg, Whipple's disease, sarcoidosis), traumatic, or tumoral destruction of hypocretin neurons.

ICSD-3 HYPERSOMNIA DISORDERS – DIAGNOSTIC CRITERIA AND CODING¹

ICSD: International Classification of Sleep Disorders
Including ICD-9 code (ICD-10 code in parentheses)

• 347.01 (G47.411) Narcolepsy Type 1 (Narcolepsy with Cataplexy)
• 347.00 (G47.419) Narcolepsy Type 2 (Narcolepsy without Cataplexy)
• Idiopathic Hypersomnia
• Kleine-Levin Syndrome
• Hypersomnia due to a medical condition, psychiatric disorder, or medications
• Behaviorally Induced Insufficient Sleep Syndrome

ICSD-3 NARCOLEPSY DIAGNOSTIC CRITERIA¹

347.01 (G47.411) Narcolepsy Type 1 (Narcolepsy with Cataplexy): Excessive sleepiness for 3 months

At least 1 of the following:

1. Cataplexy, and on MSLT, MSL <8 mins, ≥2 SOREMPs (one SOREMP may be on the preceding night’s PSG)

OR

2. CSF hypocretin-1 levels <110 pg/mL or <⅓ the baseline normal levels, and on MSLT MSL <8 mins, ≥2 SOREMPs (one SOREMP may be on the preceding night’s PSG)

In children, actigraphy is required before the MSLT

347.00 (G47.419) Narcolepsy Type 2 (Narcolepsy without Cataplexy): Positive polysomnography/multiple sleep latency test are met, but no cataplexy is present

ICSD-3 Narcolepsy Diagnostic Criteria: Narcolepsy Type 1¹

A. The patient has daily periods of irrepressible need to sleep or daytime lapses into sleep occurring for ≥3 months

Note: In young children, narcolepsy may sometimes present as excessively long night sleep or by resumption of previously discontinued daytime napping

Sleep log and/or actigraphy is recommended before laboratory sleep testing, and in children, actigraphy is required before the MSLT

B. The presence of ≥2 of the following:

1. Cataplexy

2. Mean sleep latency of <8 minutes and ≥2 SOREMPs on an MSLT performed according to standard techniques. A SOREMP (within 15 minutes of sleep onset) on the preceding nocturnal PSG may replace one of the SOREMPs on the MSLT

Note: If narcolepsy Type 1 is strongly suspected clinically but criteria B2 are not met, a possible strategy is to repeat the MSLT

3. CSF hypocretin-1 concentrations measured by immunoreactivity either <110 pg/mL or <⅓ of mean values obtained in normal subjects with the same assay

ICSD-3 Narcolepsy Diagnostic Criteria: Narcolepsy Type 2¹

A. The patient has daily periods of irrepressible need to sleep or daytime lapses into sleep occurring for at least 3 months

Note: In young children, narcolepsy may sometimes present as excessively long night sleep or by resumption of previously discontinued daytime napping

B. Mean sleep latency of <8 minutes and ≥2 SOREMPs on an MSLT performed according to standard techniques. A SOREMP (within 15 minutes of sleep onset) on the preceding nocturnal PSG may replace one of the SOREMPs on the MSLT

In children, actigraphy is required before the MSLT

C. Hypersomnia not better explained by another sleep disorder, medical or neurologic disorder, mental disorder, medication use, or substance use disorder

SUGGESTED CLINICAL INTERVIEW TOPICS FOR DECISION SUPPORT IN THE DIAGNOSIS AND ASSESSMENT OF NARCOLEPSY⁵

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Clinical Management

SAMPLE HEALTH PLAN DIAGNOSIS AND TREATMENT ALGORITHM FOR DECISION SUPPORT IN NARCOLEPSY⁶

SAMPLE HEALTH PLAN CARE COORDINATION AND MEDICAL MANAGEMENT HIERARCHY

BEHAVIORAL APPROACHES FOR THE MANAGEMENT OF NARCOLEPSY⁷

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Pharmacy Coverage and Benefit Design

PHARMACOTHERAPIES AVAILABLE FOR THE MANAGEMENT OF NARCOLEPSY⁵

SAMPLE MONOGRAPH TEMPLATE FOR P&T REVIEW AND BENEFIT DESIGN CONSIDERATION⁸

NARCOLEPSY: Formulary Monograph Template

• Individual Drug Review
• Generic Name: [Name]
• Brand Name: [Name]
• Manufacturer: [Text]
• Date of Review: Month Year
• Reason for Review: [Text]

TABLE OF CONTENTS: Executive Summary Recommendations Key Questions/Issues: Issue 1: Efficacy Issue 2: Comparative Effectiveness Issue 3: Safety Issue 4: Value Proposition Issue 5: Cost-effective Patient Subgroups Clinical Evidence Tables Cost-effectiveness Evidence Tables Background Disease Background Pharmacotherapy Product Background Methodology Authorship References

Abbreviations used in this monograph:

MEDICATION THERAPY MANAGEMENT: MEDICAL CONTRAINDICATIONS AND POTENTIAL DRUG INTERACTIONS ASSOCIATED WITH PHARMACOTHERAPIES AVAILABLE FOR THE MANAGEMENT OF NARCOLEPSY⁵

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Abbreviations

DSM-5 = Diagnostic and Statistical Manual of Mental Disorders, 5th Edition ICSD-3 = International Classification of Sleep Disorders, 3rd Edition ICD-9 = International Classification of Diseases, 9th Edition ICD-10 = International Classification of Diseases, 10th Edition CSF = Cerebrospinal fluid REM = Rapid eye movement SOREMP = Sleep onset rapid eye movement period MSLT = Multiple sleep latency test PSG = Polysomnography EDS = Excessive daytime sleepiness SDB = Sleep-disordered breathing RLS = Restless leg syndrome BMI = Body mass index RBD = REM sleep behavior disorder

REFERENCES

1. American Academy of Sleep Medicine. Central disorders of hypersomnolence. In: The International Classification of Sleep Disorders, 3rd Edition (ICSD-3). Darien, IL: American Academy of Sleep Medicine; 2014.
2. Carter LP, Acebo C, Kim A. Patients’ journeys to a narcolepsy diagnosis: a physician survey and retrospective chart review. Postgrad Med. 2014;126:216-224.
3. Ahmed I, Thorpy M. Clinical features, diagnosis and treatment of narcolepsy. Clin Chest Med. 2010;31:371-381.
4. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, 5th Edition. Arlington, VA: American Psychiatric Publishing; 2013.
5. Thorpy MJ, Dauvilliers Y. Clinical and practical considerations in the pharmacologic management of narcolepsy. Sleep Med. 2015;16:9-18.
6. Morgenthaler TI, Kapur VK, Brown TM, et al., for the Standards of Practice Committee of the AASM. Practice parameters for the treatment of narcolepsy and other hypersomnias of central origin. SLEEP. 2007;30:1705-1711.
7. Agudelo HAM, Correa UJ, Sierra JC, Pandi-Perumal SR, Schenck CH. Cognitive behavioral therapy for narcolepsy: can it complement pharmacotherapy? Sleep Sci. 2014;7:30-42.
8. Academy of Managed Care Pharmacy. The AMCP Format for Formulary Submissions, Version 4.0. http://www.amcp.org/FormatV4/ Accessed November 2, 2016.

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