• Miscellaneous neurodegenerative disorders

Brineura Generic Name & Formulations

General Description

Cerliponase alfa 30mg/mL; soln for intraventricular infusion.

Pharmacological Class

Hydrolytic lysosomal N-terminal tripeptidyl peptidase.

How Supplied

Single-dose vials (5mL)—2 (w. Intraventricular Electrolytes 5mL vial) + Administration Kit—1 (infusion supplies)

Generic Availability


Brineura Indications


To slow the loss of ambulation in late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency.

Brineura Dosage and Administration


Not applicable.


<3yrs: not established. Give by intraventricular infusion via implanted access device; administer first dose at least 5–7 days post-implantation. Pre-treat with antihistamines ± antipyretics or corticosteroids 30–60mins prior to infusion. Infuse Brineura first, followed by Intraventricular Electrolytes each at a rate of 2.5mL/hr. ≥3yrs: 300mg once every other week. See full labeling.

Brineura Contraindications


Any sign or symptom of acute, unresolved localized infection on or around the device insertion site (eg, cellulitis, abscess). Suspected or confirmed CNS infection (eg, meningitis, cloudy CSF, positive CSF gram stain). Patients with acute intraventricular access device-related complications (eg, leakage, fluid extravasation, device failure) or ventriculoperitoneal shunts.

Brineura Boxed Warnings

Not Applicable

Brineura Warnings/Precautions


Should be administered by trained healthcare providers. Inspect the scalp to ensure access device is not compromised prior to each infusion. Discontinue if access device-related complications develop. Monitor for meningitis and other device-related infections; obtain CSF sample for cell count and culture prior to each infusion. Have appropriate medical support readily available. Discontinue immediately if anaphylaxis or severe hypersensitivity reactions occur; consider risks/benefits prior to readministration. Monitor BP and HR before starting, during, and post-infusion. History of bradycardia, conduction disorder, structural heart disease: perform ECG during infusion; without cardiac abnormalities: perform ECG every 6 months. Pregnancy. Nursing mothers.

Brineura Pharmacokinetics

See Literature

Brineura Interactions


Do not mix with other drugs.

Brineura Adverse Reactions

Adverse Reactions

Pyrexia, ECG abnormalities, CSF protein increase/decrease, vomiting, seizures, hypersensitivity, hematoma, headache, irritability, pleocytosis, device-related infection, bradycardia, feeling jittery, hypotension; meningitis, cardiovascular events.

Brineura Clinical Trials

See Literature

Brineura Note

Not Applicable

Brineura Patient Counseling

See Literature