CurrentCignaPolicy IP0175

Neurology - Brineura

Defines Cigna coverage and prior authorization requirements for cerliponase alfa (Brineura) administered via intracerebroventricular infusion for treatment of neuronal ceroid lipofuscinosis type 2 (CLN2). Specifies diagnostic, prescriber, dosing, and exclusion criteria and billing code.

Policy Summary

PayerCigna

PolicyNeurology - Brineura

Policy CodePolicy IP0175

Change TypeAdded dosing; revised terminology; removed age requirement

Effective DateJul 1, 2025

Next Review Date

Key ActionPrior Authorization is required; document biallelic pathogenic or likely pathogenic TPP1 variants and reduced TPP1 enzyme activity, and ensure specialist prescribing/consultation for approval.

SourceLink

POLICY UPDATE CHANGES

Added dosing.

Condition name changed from 'Late Infantile Neuronal Ceroid Lipofuscinosis Type 2 (CLN2)' to 'Neuronal Ceroid Lipofuscinosis Type 2 (CLN2)'.

Updated terminology from 'mutation' to 'pathogenic variant' in criteria.

Annual Revision noted with 'No criteria changes' on 7/1/2025.

1Covered Indication (CLN2 only)

≥1Required Diagnostic Tests

1 yrApproval Duration

Scope: This policy defines Cigna coverage and prior authorization requirements for cerliponase alfa (Brineura) administered via intracerebroventricular (ICV) infusion for treatment of Neuronal Ceroid Lipofuscinosis Type 2 (CLN2). Coverage stance: covered_with_criteria. Status: CURRENT.

Approval duration and threshold: when medically necessary criteria are met, initial approvals are issued for 1 year and require documentation of diagnostic testing, specialist prescribing/consultation, and adherence to dosing/administration requirements.

Key Policy Facts

Policy NumberIP0175

Policy Title/SubjectBrineura (cerliponase alfa) coverage for CLN2 disease

PayerCigna

Coverage Stancecovered_with_criteria

Approval Duration1 year

Effective Date2025-07-01

Last Review2025-07-01

Medically Necessary Criteria

Medically Necessary Criteria - Initial Approval (Approve for 1 year)

Cerliponase alfa (Brineura) is considered medically necessary when ALL of the following are met:

Medically necessary criteria

Diagnostic criteria
- Diagnosis of Neuronal Ceroid Lipofuscinosis Type 2 (CLN2).
- Genetic confirmation: Genetic test confirming the diagnosis of CLN2 disease (biallelic pathogenic or likely pathogenic variants in the TPP1 gene).
- Enzyme activity: Test confirming reduced activity of tripeptidyl peptidase 1 (TPP1).
Prescriber requirement: Brineura is prescribed by or in consultation with a metabolic specialist, geneticist, pediatric neurologist, or a physician specializing in the treatment of neuronal ceroid lipofuscinoses (NCLs).
Dosing and administration
- Dose frequency: 300 mg via intracerebroventricular (ICV) infusion administered once every other week (QOW).
- Electrolytes: Each dose is followed by an infusion of intraventricular electrolytes (supplied in the Brineura package).
- Administration route and provider: Administer via surgically implanted reservoir and catheter and only by or under the direction of a physician knowledgeable in ICV administration.

Not Medically Necessary / Exclusions

Cerliponase alfa (Brineura) is considered not medically necessary when used for any indication not listed above, including but not limited to:

Not medically necessary / exclusions

Other NCL subtypes: Neuronal Ceroid Lipofuscinoses (NCLs) other than CLN2 (examples: CLN1, CLN3, CLN10, CLN13, and others).
Brineura has not been studied for NCLs involving genes other than CLN2.
Other uses: Any other use of cerliponase alfa not meeting the specified diagnostic, prescriber, or dosing criteria.

Denial Risk

Exclusion Risk for Non-CLN2 NCLs

Claims for Brineura for NCL subtypes other than CLN2 are considered not medically necessary and at risk for denial. Document that the treated condition is CLN2; claims for other NCL subtypes may be denied.

Examples of non-CLN2 subtypes at risk for denial: CLN1
CLN3
CLN10
CLN13

Provider Actions

Prior Authorization

Prior Authorization Required

Prior Authorization is required for coverage of Brineura; approvals are provided for the duration noted (1 year) and require documentation of diagnostic criteria and specialist prescribing/consultation.

Affected code: J0567
Approval duration: 1 year

Documentation Required

Diagnostic Documentation Required

Provider must document genetic testing showing biallelic pathogenic or likely pathogenic variants in the TPP1 gene and testing demonstrating reduced TPP1 enzyme activity.

Genetic testing: biallelic pathogenic or likely pathogenic TPP1 variants
TPP1 enzyme activity: reduced activity in leukocytes, fibroblasts, or dried blood spots

Documentation Required

Specialist Prescriber or Consultation

Brineura must be prescribed by or in consultation with a specialist; document specialist involvement.

Metabolic specialist
Geneticist
Pediatric neurologist
Physician specializing in neuronal ceroid lipofuscinoses (NCL specialist)

Billing Rule

Dosing and Administration Requirements

Administer 300 mg ICV once every other week; each dose must be followed by intraventricular electrolytes as supplied. Brineura is administered via a surgically implanted reservoir/catheter into the cerebrospinal fluid and should be given by or under the direction of a clinician experienced in ICV administration.

Dosing: 300 mg intracerebroventricular (ICV) every other week (QOW)
Each dose followed by intraventricular electrolytes (supplied in the Brineura package)
Route/site: ICV via surgically implanted reservoir and catheter
Clinician requirement: administered by or under direction of a physician experienced in ICV administration
Affected code: J0567

Background and Evidence

Background: Brineura (cerliponase alfa) is recombinant human TPP1 indicated to slow loss of ambulation in symptomatic pediatric patients with CLN2 disease.

Administration and provider expertise: Brineura is given via intracerebroventricular (ICV) infusion into the cerebrospinal fluid through a surgically implanted reservoir and catheter and should be administered by or under the direction of a physician experienced in ICV administration; prior authorization and specialist prescribing or consultation are required.

Boxed warning: Brineura carries a Boxed Warning for hypersensitivity reactions including anaphylaxis; initiation should occur in a healthcare setting with appropriate monitoring.

Evidence References

Definitions

Revision History

8/1/2024material_change

Added dosing: specified recommended dose of 300 mg administered once every other week (QOW) via intracerebroventricular (ICV) infusion and requirement that each dose be followed by an infusion of intraventricular electrolytes.

11/1/2024material_change

Removed age requirement (previously patient ≥ 3 years) and changed condition title from 'Late Infantile Neuronal Ceroid Lipofuscinosis Type 2 (CLN2)' to 'Neuronal Ceroid Lipofuscinosis Type 2 (CLN2)'; updated wording from 'mutation' to 'pathogenic variant' in criteria.

7/1/2025administrative_reviewLatest

Annual revision noted; no clinical criteria changes on this annual review.

7/2024background_update

Policy effective date and prescribing information referenced (Brineura indicated for CLN2 to slow loss of ambulation in symptomatic pediatric patients); boxed warning for hypersensitivity/anaphylaxis emphasized and recommendation for administration by clinicians experienced in ICV infusion.

Policy Summary

PayerCigna

PolicyNeurology - Brineura

Policy CodePolicy IP0175

Change TypeAdded dosing; revised terminology; removed age requirement

Effective DateJul 1, 2025

Next Review Date

SourceLink

On This Page

OpenPayer

Neurology - Brineura

Coverage Summary

Medically Necessary Criteria

Not Medically Necessary / Exclusions

Coding

Provider Actions

Background and Evidence

Revision History