CurrentUnitedHealthcarePolicy CSLA2O25DOO65L

Brineura (Cerliponase Alfa) — Medical Benefit Drug Policy (Louisiana)

Medical benefit drug policy governing coverage of Brineura (cerliponase alfa) for treatment of late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) for UnitedHealthcare Community Plan members in Louisiana.

Policy Summary

PayerUnitedHealthcare Community Plan

PolicyBrineura (Cerliponase Alfa) — Medical Benefit Drug Policy (Louisiana)

Policy CodePolicy CSLA2O25DOO65L

Change TypeRevised coverage criteria and policy language

Effective DateMar 1, 2025

Next Review Date

Key ActionObtain prior authorization documenting CLN2 diagnosis, required Clinical Scoring System motor and language scores, neurologist involvement, and plan for intraventricular administration.

SourceLink

POLICY UPDATE CHANGES

Removed criterion requiring the patient is age 3 years or older; broadened language to pediatric patients of all ages.

Revised coverage criteria and updated supporting Clinical Evidence, FDA, and References sections.

J0567HCPCS

E75.4ICD-10

≤12 moInitial auth

≤12 moReauth

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Coverage and Medical Necessity Criteria

inv-01: Initial therapy — medically necessary criteria

Covered when ALL of the following are met:

Initial therapy criteria: 1) Diagnosis of late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) by a neurologist with expertise in CLN2 or by a physician in consultation with such a neurologist; 2) Clinical Scoring System for LINCL: motor and language domain scores with a combined motor+language score of 3 to 6 (document motor and language domain scores); 3) Brineura (cerliponase alfa) is prescribed by a neurologist experienced in the treatment of CLN2 or by a physician in consultation with such a neurologist; 4) Dosing consistent with United States FDA–approved labeling; 5) Administration intraventricularly by, or under the direction of, healthcare professionals experienced in performing intraventricular infusions via an intracerebroventricular catheter; 6) Initial authorization for no more than 12 monthscombined motor+language score 3-6

Document motor and language domain scores and prescribing/administration plan.

inv-02: Continuation (reauthorization) — medically necessary criteria

Covered for continued therapy when ALL of the following are met:

Continuation criteria: 1) Ongoing diagnosis of CLN2 by a neurologist with expertise in CLN2 or by a physician in consultation with such a neurologist; 2) Patient has a score of 1 or higher in the motor domain of the Clinical Scoring System for LINCL; 3) Brineura is prescribed by a neurologist experienced in treatment of CLN2 or by a physician in consultation with such a neurologist; 4) Dosing consistent with United States FDA–approved labeling; 5) Administration intraventricularly by, or under the direction of, healthcare professionals experienced in performing intraventricular infusions via an intracerebroventricular catheter; 6) Reauthorization for no more than 12 monthsmotor domain score >=1

Provide evidence of ongoing clinical benefit or stability for reauthorization; document motor domain score and prescribing/administration plan.

Use of Brineura (cerliponase alfa) for indications other than late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) is unproven and not medically necessary.

Brineura (cerliponase alfa) is unproven and not medically necessary for other forms of neuronal ceroid lipofuscinosis (non-CLN2).

Applicable Billing and Diagnosis Codes

Applicable HCPCS CodesHCPCS

J0567

Injection, cerliponase alfa, mg

Applicable Diagnosis CodesICD-10

E75.4

Neuronal ceroid lipofuscinosis

Provider Requirements, Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required before initiation of Brineura (cerliponase alfa). Initial authorization will be for no more than 12 months; reauthorization (continuation) will also be for no more than 12 months. Authorization requests should document diagnosis, specialist involvement, baseline clinical scores, and planned dosing per FDA labeling.

Initial and reauthorization requests: limit 12 months
J-code for billing reference: HCPCS J0567
Diagnosis code reference: ICD-10 E75.4

Step Therapy

Step Therapy / Alternative Therapy

No step therapy is specified for Brineura. Use of Brineura for other forms of neuronal ceroid lipofuscinosis (non-CLN2) is considered unproven and not medically necessary; alternatives or other therapies should be considered per clinical judgment and coverage rules.

Brineura is proven and medically necessary only for CLN2 (late infantile neuronal ceroid lipofuscinosis type 2).

Clinical Background

Late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) is a pediatric-onset, autosomal recessive lysosomal storage disorder caused by deficient activity of the enzyme tripeptidyl peptidase-1 (TPP1). The enzyme deficiency leads to accumulation of storage material in neurons and progressive neurodegeneration resulting in seizures, language regression, motor deterioration, vision loss, and early mortality. Brineura (cerliponase alfa) is a recombinant form of TPP1 administered by intraventricular infusion intended to replace deficient enzyme activity and slow loss of ambulation in affected children.

Definitions and Clinical Scales

CLN2 (late infantile neuronal ceroid lipofuscinosis type 2)

DefinitionLate infantile neuronal ceroid lipofuscinosis type 2 (CLN2) is a pediatric-onset, autosomal recessive neurodegenerative lysosomal storage disorder caused by deficient tripeptidyl peptidase-1 (TPP1) activity.

Key clinical featuresCharacterized by progressive motor deterioration, seizures, language delay, movement disorders, visual loss (blindness), dementia, and early death.

PathophysiologyTPP1 deficiency leads to accumulation of lysosomal storage material in the central nervous system, causing neurodegeneration and progressive loss of ambulation.

Therapeutic implicationBrineura (cerliponase alfa) is recombinant TPP1 administered by intraventricular infusion to slow the loss of ambulation in children with CLN2.

Disease course variantsClassical CLN2: symptom onset typically between ages 3–5 years with more rapid progression; Non-classical CLN2: slower evolution with behavioral and movement disorder presentations.

Policy Summary

PayerUnitedHealthcare Community Plan

PolicyBrineura (Cerliponase Alfa) — Medical Benefit Drug Policy (Louisiana)

Policy CodePolicy CSLA2O25DOO65L

Change TypeRevised coverage criteria and policy language

Effective DateMar 1, 2025

Next Review Date

Key ActionObtain prior authorization documenting CLN2 diagnosis, required Clinical Scoring System motor and language scores, neurologist involvement, and plan for intraventricular administration.

SourceLink

On This Page

Use for other forms of neuronal ceroid lipofuscinosis is not medically necessary.

Documentation Required

Required Documentation

Documentation to support authorization must include: confirmation of CLN2 diagnosis, documentation that the prescribing clinician is a neurologist with expertise in CLN2 or that the treating physician consulted such a neurologist, baseline Clinical Scoring System for LINCL motor and language domain scores, planned intraventricular administration per FDA dosing, and evidence that administration will occur under clinicians experienced with intracerebroventricular catheter infusions.

Diagnosis of CLN2 by neurologist or physician in consultation with a neurologist
Clinical Scoring System for LINCL: motor and language domain scores
Planned dosing consistent with FDA labeling
Plan for intraventricular administration by experienced providers

Denial Risk

Denial Triggers

Denials may be issued if documentation does not demonstrate CLN2 diagnosis, specialist involvement or consultation, required baseline clinical scores, FDA-consistent dosing, or if the medication is requested for non-CLN2 forms of neuronal ceroid lipofuscinosis.

Requests for non-CLN2 indications (not medically necessary)
Missing or insufficient documentation of diagnosis or specialist consultation
Absence of required clinical scoring or evidence of planned intraventricular administration

Clinical Scoring System for LINCL/CLN2

PurposeA clinical rating scale used to quantify disease progression in late infantile neuronal ceroid lipofuscinosis (CLN2) focusing on motor and language domains.

Domains scoredMotor domain and language domain; combined motor+language score is used to assess progression and treatment effect.

Initial-therapy thresholdInitial authorization requires a combined motor+language score between 3 and 6 (document motor and language domain scores).

Reauthorization thresholdContinuation (reauthorization) requires a motor domain score of 1 or higher to demonstrate ongoing eligibility.

Use in trialsPrimary outcome in pivotal studies compared time to a 2-point decline in the motor+language score versus historical controls; response defined as absence of an unreversed 2-point decline or a score of zero at 48 weeks.