CurrentBluecross IdahoPolicy N/A

Brineura (cerliponase alfa) — Coverage Criteria for CLN2

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Criteria and coverage guidance for use of Brineura (cerliponase alfa) to treat late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) for Bluecross Idaho members. Applies to providers requesting medical benefit authorization for pediatric intraventricular enzyme replacement therapy.

Policy Summary

PayerBluecross Idaho

PolicyBrineura (cerliponase alfa) — Coverage Criteria for CLN2

Policy CodePolicy N/A

Change TypeNo material change

Effective DateMay 28, 2026

Last ReviewMay 28, 2026

Next Review DateMay 28, 2026

Key ActionObtain prior authorization with documentation of diagnosis confirmation (TPP1 deficiency or two pathogenic CLN2 variants), baseline Motor+Language Hamburg scores, and supporting records.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

300 mgFDA dose q2w

6 monthsInitial approval

3-6Hamburg score

≥3 yrMinimum age

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CurrentBluecross IdahoPolicy N/A

Brineura (cerliponase alfa) — Coverage Criteria for CLN2

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Policy Summary

PayerBluecross Idaho

PolicyBrineura (cerliponase alfa) — Coverage Criteria for CLN2

Policy CodePolicy N/A

Change TypeNo material change

Effective DateMay 28, 2026

Last ReviewMay 28, 2026

Next Review DateMay 28, 2026

Key ActionObtain prior authorization with documentation of diagnosis confirmation (TPP1 deficiency or two pathogenic CLN2 variants), baseline Motor+Language Hamburg scores, and supporting records.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

300 mgFDA dose q2w

6 monthsInitial approval

3-6Hamburg score

≥3 yrMinimum age

22/42Key study counts

Coverage Criteria for Brineura (cerliponase alfa)

Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met

Initial therapy criteria: A. Diagnosis of CLN2 confirmed by TPP1 deficiency and/or molecular analysis showing two pathogenic variants in the TPP1/CLN2 gene; B. Patient is a pediatric patient ≥ 3 years of age; C. Mild to moderate disease documented by a two-domain (Motor+Language) Hamburg score of 3-6 with at least 1 in each domain; D. Motor and Language domains of the CLN2 Clinical Rating Scale have been assessed prior to starting therapy; E. Does not require ventilation support except noninvasive nocturnal support; F. Patient is ambulatory; G. No prior stem cell, gene, or enzyme replacement therapy for CLN2; H. No signs of acute or unresolved localized infection at or around the device insertion site, no suspected/confirmed central nervous system infection, no acute intraventricular access device complication, and no ventriculoperitoneal shunt; I. Administration by or under the direction of a physician knowledgeable in intraventricular administration with strict aseptic technique; J. Individuals with bradycardia, conduction disorder, or structural heart disease must have ECG monitoring during infusion.

Initial approval 6 months at FDA dose 300 mg once every other week by intraventricular infusion.

Continuation Therapy — Continued approval every 6 months when ALL of the following are met

Continued approval every 6 months when ALL of the following are met

Continuation therapy criteria: A. Continues to meet initial review criteria including a two-domain (Motor+Language) Hamburg score of 3-6 with at least 1 in each domain; B. Tolerates intraventricular infusions with no significant adverse reactions (e.g., hypersensitivity reactions, meningitis or other device-related infections, cardiovascular adverse reactions); C. Evidence of efficacy and disease stability or improvement defined as no decline in the Motor domain (no sustained 2-point decline and no unreversed motor score of 0); D. Individuals with cardiac abnormalities must have had a 12-lead ECG performed within the last 6 months.

Continued dosing 300 mg once every other week by intraventricular infusion; continuation approvals granted every 6 months.

Not Covered / Investigational

Investigational indications: Brineura (cerliponase alfa) is considered investigational for other conditions, including but not limited to other forms of neuronal ceroid lipofuscinosis, Batten Disease, and Jansky-Bielschowsky Disease.

Brineura (cerliponase alfa) is considered investigational for other indications beyond CLN2. Specifically, use for other forms of neuronal ceroid lipofuscinosis, Batten Disease, and Jansky‑Bielschowsky Disease is classified as investigational and not covered under this policy. Additionally, patients with a ventriculoperitoneal shunt are listed among conditions that preclude treatment with Brineura and should be excluded from coverage consideration per the policy criteria.

This document segment does not state any additional explicit exclusions beyond those specified in the coverage criteria and contraindications sections. Code and benefit guidance in the Codes section is provided for reference and does not itself create exclusions.

Treatment with Brineura is considered not medically necessary when the documented diagnostic or clinical eligibility criteria are not met. Examples include patients who are 3 years of age, those without confirmed TPP1 deficiency or two pathogenic CLN2 variants, individuals with a combined Motor+Language Hamburg score outside the required range 3–6 or with a score less than 1 in either domain, non‑ambulatory patients, or those with prior stem cell, gene, or enzyme replacement therapy for CLN2. Absence of required pre‑treatment assessments or failure to meet the other listed clinical eligibility requirements may also result in a determination of not medically necessary.

No explicit statements labeled as 'not medically necessary' are present in this document segment beyond the clinical criteria referenced elsewhere in the policy. Coding guidance in the Codes section reiterates that inclusion of a code does not guarantee coverage and that benefit determinations remain subject to the member‑specific plan.

Initial Therapy Dosing and Requirements

Initial Therapy Dosing

Initial approval details

Initial therapy dosing: FDA-approved dose: 300 mg administered once every other week by intraventricular infusion; initial approval duration 6 months when all eligibility criteria are met.300 mg every other week

Administration by or under the direction of a physician experienced in intraventricular administration; strict aseptic technique must be observed.

Continuation Therapy Requirements

Continuation Therapy — Criteria for ongoing approval every 6 months

Criteria for ongoing approval every 6 months

Continuation nodes: Must continue to meet initial criteria; tolerate intraventricular infusions without significant adverse reactions; demonstrate evidence of efficacy or disease stability/improvement (no sustained 2-point decline or an unreversed motor score of 0); individuals with cardiac abnormalities must have had a 12-lead ECG within the last 6 months.

Continued dosing at 300 mg every other week administered by intraventricular infusion; continuation approvals granted every 6 months.

Coding and Diagnostic Codes

Coding table referencedmixed

No codes listed

Diagnostic CPT CodesCPT

82657

Enzyme activity in blood cells, cultured cells, or tissue, not elsewhere specified; nonradioactive substrate, each specimen (can be used to test for Neuronal ceroid lipofuscinosis type 2)

Drug HCPCS CodesHCPCSCovered

J0567

Injection, cerliponase alfa, 1mg (Brineura)

Diagnosis CodesICD-10

E75.4

Neuronal ceroid lipofuscinosis

ECG timing for cardiac abnormalities

Required timing12-lead ECG performed within the last 6 months for individuals with cardiac abnormalities

ContextApplies to continuation reviews when cardiac abnormalities are present

PurposeEnsures cardiac status is recently evaluated prior to ongoing intraventricular infusions

No numeric thresholds listed

Numeric thresholds absentNo numeric laboratory or physiologic thresholds (e.g., specific ECG intervals, enzyme activity cutoffs) are listed in the coding/thresholds section of the policy

Implication for reviewers

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior authorization required — submit diagnostic confirmation and baseline Hamburg scores

Prior authorization is required for both initial and continuation therapy. Requests must document diagnostic confirmation of CLN2 (TPP1 deficiency and/or molecular analysis showing two pathogenic CLN2/TPP1 variants), baseline Motor+Language Hamburg scores, and other eligibility criteria; initial approval is for 6 months at 300 mg every other week by intraventricular infusion.

Document TPP1 deficiency and/or molecular analysis showing two pathogenic CLN2 variants
Provide baseline two-domain (Motor+Language) Hamburg score (combined 3–6 with at least 1 in each domain)
Confirm other eligibility criteria (age ≥3 years, ambulatory status, ventilation support limitations, no excluded infections/device complications)
Specify requested dosing: 300 mg IVT every other week; initial approval duration = 6 months

Prior Authorization

Prior authorization for cerliponase alfa (J0567); member benefit applies

Prior authorization applies to cerliponase alfa (HCPCS J0567) as a specialty intraventricular therapy; coverage remains subject to the member's benefit plan and policy terms.

Site of Care and Administration

Note

Infusion center requirement — physician experienced in intraventricular administration

Brineura must be administered by, or under the direction of, a physician knowledgeable in intraventricular administration, indicating infusion in a setting capable of intraventricular procedures and ECG monitoring when indicated.

Physician must be knowledgeable in intraventricular administration and strictly observe aseptic technique
Infusion setting should support intraventricular access device management and ECG monitoring if cardiac history present

Note

Outpatient site of care — therapy listed as outpatient

Therapy is listed as outpatient (place of service: outpatient); specific additional site-of-care restrictions are not detailed in this policy.

Place of service listed as Outpatient
No further site-of-care specifics provided in this document

Key Definitions and Scales

Confirmed CLN2 (TPP1 deficiency)

Confirmation criteriaDiagnosis confirmed by documented TPP1 enzyme deficiency and/or molecular analysis identifying two pathogenic variants/mutations in the TPP1/CLN2 gene on chromosome 11p15

Required documentationMedical records demonstrating enzyme assay results and/or genetic testing reports showing two pathogenic CLN2 variants

Clinical implicationConfirmation distinguishes CLN2 (TPP1 deficiency) from other forms of neuronal ceroid lipofuscinosis and supports medical necessity for therapy

Hamburg CLN2 Motor+Language score

Score requirementCombined Hamburg Motor + Language two-domain score of 3–6 with at least 1 point in each domain

When assessedMust be documented at baseline prior to initiation and reassessed for continuation approvals every 6 months

Background

CLN2 (late infantile neuronal ceroid lipofuscinosis type 2) is a rare, inherited neurodegenerative disorder caused by mutations in the TPP1/CLN2 gene leading to deficiency of the TPP1 enzyme. It typically presents in early childhood with language delay, seizures, ataxia, myoclonus and progressive motor and visual deterioration. Cerliponase alfa (Brineura) is a recombinant form of TPP1 administered by intraventricular infusion; once delivered to the cerebrospinal compartment it is taken up into lysosomes where it is activated to restore tripeptidyl peptidase activity.

Policy Summary

PayerBluecross Idaho

PolicyBrineura (cerliponase alfa) — Coverage Criteria for CLN2

Policy CodePolicy N/A

Change TypeNo material change

Effective DateMay 28, 2026

Last ReviewMay 28, 2026

Next Review DateMay 28, 2026

Key ActionObtain prior authorization with documentation of diagnosis confirmation (TPP1 deficiency or two pathogenic CLN2 variants), baseline Motor+Language Hamburg scores, and supporting records.

SourceLink

On This Page

Clinical judgment and documented test results should be used when numeric cutoffs are not specified

DocumentationProvide relevant test reports (e.g., ECG, enzyme testing) even though policy does not prescribe numeric pass/fail values

Include HCPCS J0567 on the authorization request
Benefit coverage is determined by the member-specific plan

Step Therapy

No step therapy specified

No step therapy requirements are specified in this policy.

Note

Submit comprehensive supporting medical records with authorization

Provide complete medical records that support eligibility and the prior authorization request as described below to avoid delays or denial.

Attach enzyme or genetic test results, Hamburg Motor+Language scores, documentation of ambulatory and ventilation status, infusion tolerance history, and prior therapy history

Documentation Required

Documentation required — diagnostic, Hamburg scores, functional and device status

Medical records must document diagnosis confirmation (TPP1 enzyme deficiency and/or molecular analysis showing two pathogenic CLN2 variants), Motor and Language Hamburg domain scores, ambulatory and ventilation status, absence of excluded infections or device complications, and prior therapy history.

Provide evidence of TPP1 deficiency or two pathogenic CLN2 variants
Include Motor and Language domain scores on the Hamburg Scale (combined 3–6, ≥1 in each domain)
Document ambulatory status and any ventilation needs (only nocturnal noninvasive support allowed)
Report absence of device-site infection, suspected/confirmed CNS infection, acute device complication, or ventriculoperitoneal shunt

Billing Rule

Required coding and benefit documentation (82657, J0567, E75.4) — include member benefit verification

Include relevant diagnostic and drug codes with the request: CPT 82657 for enzyme activity testing, HCPCS J0567 for cerliponase alfa, and ICD-10-CM E75.4 for neuronal ceroid lipofuscinosis; note that inclusion of codes does not guarantee coverage and benefit is subject to the member's plan.

Diagnostic CPT: 82657
Drug HCPCS: J0567
Diagnosis ICD-10-CM: E75.4
State that benefit coverage is determined by member-specific plan documentation

Denial Risk

Denial risks — missing diagnostic/clinical eligibility or excluded conditions

Denial may occur if the request fails to document TPP1 deficiency or two pathogenic CLN2 variants, the patient is under 3 years of age, Hamburg Motor+Language score is outside 3–6 or has a motor/language domain score <1, or if other exclusionary clinical conditions (e.g., non-ambulatory status, need for advanced ventilation, prior CLN2 gene/stem/enzyme therapy, device/CNS infection) are present.

Age <3 years
Hamburg Motor+Language combined score outside 3–6 or any domain <1
Lack of documented TPP1 deficiency or two pathogenic CLN2 variants
Non-ambulatory status or ventilation beyond nocturnal noninvasive support
Prior stem cell, gene, or enzyme replacement therapy for CLN2
Active device-site infection, suspected/confirmed CNS infection, acute intraventricular access device complication, or ventriculoperitoneal shunt

Denial Risk

Coding and coverage risk — include E75.4 and supportive testing to reduce denial risk

Claims or authorization requests lacking an appropriate diagnosis code (E75.4) or supportive testing (e.g., CPT 82657 enzyme activity testing) may be denied; include these codes and documentation to reduce coverage risk, but coverage remains subject to the member-specific benefit plan.

Include ICD-10-CM E75.4 on claims/authorizations
Attach CPT 82657 enzyme activity test results or molecular testing showing two pathogenic CLN2 variants
Remember that code inclusion does not guarantee coverage

Role in continuationContinuation requires the patient to continue to meet the 3–6 combined score with ≥1 in each domain

CLN2 disease

DefinitionNeuronal ceroid lipofuscinosis type 2 (CLN2) is a rare pediatric neurodegenerative genetic disorder caused by mutations in the TPP1/CLN2 gene leading to TPP1 enzyme deficiency and progressive loss of neurological function

Typical presentationSigns usually present between ages 2 and 4 and include language delay, seizures, ataxia, myoclonus and vision loss

PathophysiologyCerliponase alfa (Brineura) is a recombinant form of TPP1 delivered intraventricularly to replace deficient enzyme activity in the CNS