CurrentCignaPolicy IP0175

Brineura (cerliponase alfa) coverage for CLN2

Defines prior authorization, medical necessity criteria, dosing, and coding for Brineura (cerliponase alfa) for treatment of neuronal ceroid lipofuscinosis type 2 (CLN2) for Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyBrineura (cerliponase alfa) coverage for CLN2

Policy CodePolicy IP0175

Change TypeDosing addedage requirement removedterminology updated

Effective DateJul 1, 2025

Next Review Date

Key ActionObtain prior authorization and document genetic confirmation of biallelic pathogenic variants in TPP1 and reduced TPP1 enzyme activity; approval is for 1 year when criteria are met.

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)'.

Removed requirement that patient is ≥ 3 years of age.

Terminology updated from 'mutation' to 'pathogenic variant'.

300 mg QOWrecommended dose and frequency

J0567HCPCS code

1 yearinitial authorization duration

Boxed Warningsafety warning

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Coverage Criteria for Brineura (cerliponase alfa)

Initial Therapy — Cerliponase alfa (Brineura)

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

Initial approval for CLN2: 1) Diagnosis: Neuronal Ceroid Lipofuscinosis Type 2 (CLN2). 2) Genetic confirmation: patient has had a genetic test confirming biallelic pathogenic or likely pathogenic variants in the TPP1 gene. 3) Biochemical confirmation: patient has had testing that confirms reduced activity of tripeptidyl peptidase 1 (TPP1). 4) 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).Approve for 1 year

Approval is for 1 year when all criteria are met.

Dosing — Dosing requirements

Dosing requirements (must be met with approvals):

Dosing: 300 mg via intracerebroventricular (ICV) infusion administered once every other week (QOW). Each dose must be followed by an infusion of intraventricular electrolytes (supplied in the Brineura package).

Use of cerliponase alfa (Brineura) for any other use is considered not medically necessary. This includes treatment of neuronal ceroid lipofuscinoses (NCLs) other than CLN2 (for example, CLN1, CLN3, CLN10, CLN13) and other non‑CLN2 indications; the list is not exhaustive and will be updated as new published data become available.

Brineura is not medically necessary for neuronal ceroid lipofuscinoses other than CLN2 or for other non‑CLN2 indications. This policy note is not all‑inclusive; Brineura has not been studied for NCLs caused by variants in genes other than TPP1 (CLN2), and coverage will follow available published data.

Billing and Coding

Considered Medically Necessary when criteria metHCPCSCovered

J0567

Injection, cerliponase alfa, 1 mg

inv-06: Dose per administration — 300 mg via ICV every other week.

Dose per administration300 mg via intracerebroventricular (ICV) infusion administered once every other week (QOW)

Administration adjunctEach dose is followed by an infusion of intraventricular electrolytes (supplied in the Brineura package)

Route and deviceAdministered into the cerebrospinal fluid via a surgically implanted reservoir and catheter (ICV)

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required; approval is for 1 year when all medical necessity criteria are met. Brineura must be prescribed by or in consultation with a metabolic specialist, geneticist, pediatric neurologist, or a physician specializing in neuronal ceroid lipofuscinoses (NCLs).

Prior authorization required for prescription benefit coverage of Brineura
Approvals provided for 1 year when medical necessity criteria are met

Denial Risk

Claims and Billing — Denial Risk

Claims submitted without covered codes under the applicable Coverage Policy will be denied as not covered. Prior authorization must be obtained before services are rendered.

Claims lacking covered codes under this Coverage Policy will be denied as not covered
Prior authorization is required

Documentation Required

Documentation Requirements

Documentation must include genetic and biochemical confirmation of CLN2 prior to approval. Maintain records showing both genetic testing and enzyme activity testing, and documentation that Brineura was prescribed or consulted on by an appropriate specialist.

Document genetic test confirming diagnosis of CLN2: biallelic pathogenic or likely pathogenic variants in the TPP1 gene
Document test confirming reduced TPP1 (tripeptidyl peptidase 1) enzyme activity
Document that Brineura is prescribed by or in consultation with a metabolic specialist, geneticist, pediatric neurologist, or other physician specializing in NCLs

Billing Rule

Provider Actions — Administration and Coding

Include dosing and administration details on the claim and in the medical record to support medical necessity and coverage.

Dose: 300 mg via intracerebroventricular (ICV) infusion once every other week (QOW)
Each Brineura dose must be followed by an infusion of intraventricular electrolytes (supplied with the product)
Use J0567 (Injection, cerliponase alfa, 1 mg) when submitting drug administration coding as appropriate

Definitions

inv-12: Definition — Neuronal Ceroid Lipofuscinosis Type 2 (CLN2): form of NCL caused by TPP1 deficiency due to biallelic pathogenic variants.

Condition nameNeuronal Ceroid Lipofuscinosis Type 2 (CLN2)

CauseDeficiency of tripeptidyl peptidase 1 (TPP1) due to biallelic pathogenic or likely pathogenic variants in the TPP1 gene

Clinical impactUltra‑rare progressive neurodegenerative lysosomal storage disorder with early vision impairment, loss of cognitive and motor function, seizures, and premature death

inv-13: Definition — Intracerebroventricular (ICV) infusion: administration into cerebrospinal fluid via a surgically implanted reservoir and catheter.

DefinitionIntracerebroventricular (ICV) infusion: administration of Brineura into the cerebrospinal fluid via a surgically implanted reservoir and catheter

Setting/providerShould be administered by or under the direction of a physician knowledgeable in ICV administration

Post‑dose requirementEach ICV administration of Brineura must be followed by an infusion of intraventricular electrolytes (provided in the package)

Background

Neuronal Ceroid Lipofuscinosis Type 2 (CLN2) is an ultra‑rare, progressive neurodegenerative lysosomal storage disorder caused by biallelic pathogenic or likely pathogenic variants in the TPP1 gene, which result in deficient tripeptidyl peptidase 1 (TPP1) enzyme activity. Symptom onset is typically in early childhood with progressive loss of vision, cognition, and motor function. Brineura (cerliponase alfa) is recombinant human TPP1 formulated for administration by intracerebroventricular (ICV) infusion via a surgically implanted reservoir and catheter to slow loss of ambulation in symptomatic pediatric patients with CLN2.