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Administration of Non-Cytotoxic Medication via an Intracerebroventricular Reservoir

The purpose of this document is to provide guidance for the administration of non-cytotoxic injection or infusion via an Intracerebroventricular (ICV) reservoir at Great Ormond Street Hospital (GOSH). 

This guideline is to be used for the administration of cerliponase alfa/BMN190 and may need to be adapted for administration of other ICV medications. 

For intrathecal or ICV administration of cytotoxic drugs please see the clinical guideline; Intrathecal cytotoxic chemotherapy: administration via a lumbar puncture or Ommaya reservoir.

Ketogenic diet

The ketogenic diet (KD) is a therapeutic diet, which has been shown to improve seizure control in patients with drug resistant epilepsy, and is used in some patients with metabolic conditions for example, glucose transporter type 1 deficiency syndrome (GLUT1) and pyruvate dehydrogenase deficiency (PDH).