Cerliponase Alfa (Brineura)

Section: Injections
Effective Date: August 01, 2019
Revised Date: July 29, 2019


Cerliponase alfa (Brineura) is a hydrolytic lysosomal N-terminal tripeptidyl peptidase indicated to slow the loss of ambulation in symptomatic individuals three years of age and older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency. CLN2 disease is one of a group of disorders known as neuronal ceroid lipofuscinoses, collectively called Battens disease. Deficiency in TPP1 activity results in the accumulation of lysosomal storage materials normally metabolized by this enzyme in the CNS, leading to progressive decline in motor function. Cerliponase alfa (Brineura), a proenzyme, is activated in the lysosome and cleaves tripeptides from the N-terminus of proteins. 


Coverage is subject to the specific terms of the member's benefit plan. 

Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website. 

Cerliponase alfa (Brineura) may be considered medically necessary to slow the loss of ambulation in individuals with late infantile CLN2 who meet ALL of the following: 

  • Individual is three (3) years of age or older; and
  • Individual is symptomatic (e.g. motor or language symptoms); and
  • Has a confirmed diagnosis of CLN2, defined as: 
    • Deficient TPP1 activity in leukocytes; and
    • Pathogenic variants/mutations in each allele of TPP1/CLN2 gene; and
  • Cerliponase alfa (Brineura) is prescribed by or in consultation with a neurologist with experience and expertise in the treatment and ongoing management of individuals with CLN2; and
  • Documentation of current rating on the CLN2 Clinical Rating Scale. 

Continuation of treatment with cerliponase alfa (Brineura) after initiation of therapy, may be considered medically necessary to slow the loss of ambulation in individuals late infantile CLN2 who meet the following: 

  • Documentation that individual has slowed the loss of ambulation from initial baseline CLN2 Clinical Rating Scale. 

Cerliponase alfa (Brineura) is considered experimental/investigational for any other indication and therefore non-covered. Scientific evidence does not support its use for any other indications. 

Procedure Codes


NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.

Diagnosis Codes