E88.40 — Mitochondrial metabolism disorder, unspecified
ICD-10-CM
Prior Authorization
No Prior Auth Required
Code is covered without prior authorization (high confidence)
Documentation Required
- Documentation of treatment-resistant epilepsy: prior failure of anti-epileptic drugs (Neal et al. trial required failure of at least 2 AEDs) and seizure frequency records (e.g., seizure calendar) as used in multiple cited studies (Weber et al., 2009; Neal et al., 2008).
- Seizure outcome documentation: seizure frequency logs/calendars and follow-up seizure outcome assessments (eg, ≥50% seizure reduction or seizure freedom at defined follow-ups) as used in trials cited.
- Monitoring during KD and initiation: urine ketone monitoring and serum ketone levels (Cervenka et al., 2017: 'significant urine and serum ketone body production as a biomarker of feasibility'); daily urine ketones during initiation (studies cited); serum bicarbonate monitoring (association with topiramate use noted); blood glucose and metabolic monitoring (eg, HbA1c, insulin, lipid profile) as reported in multiple studies.
Key Coverage Criteria
- Covered ICD-10 diagnosis codes if selection criteria are met: E74.4 (disorders of pyruvate metabolism and gluconeogenesis).
- Covered ICD-10 diagnosis codes if selection criteria are met: E88.09 (glucose transporter protein type 1 deficiency).
- Covered ICD-10 diagnosis codes if selection criteria are met: Epilepsy and recurrent seizures, without status epilepticus (e.g., G40.009, G40.019, G40.109, G40.119, G40.209, G40.219, G40.309, G40.319, G40.A09, G40.A19, G40.B09, G40.B19, G40.409, G40.419, G40.509, G40.802, G40.804, G40.822, G40.824, G40.909, G40.919).
- Covered ICD-10 diagnosis codes if selection criteria are met: Lennox-Gastaut syndrome (G40.811 - G40.814).
- The ketogenic diet... has been used for the treatment of uncontrolled seizures.
- The ketogenic diet may be considered as an adjunctive treatment in children with drug-resistant epilepsy. (NICE, 2004)
3 Active Policies
AMBETTER-CG-Metab-Endo-Mito-DO-2025.1 — Concert Genetic Testing: Metabolic, Endocrine, and Mitochondrial Disorders
AETNA-CPB-0202 — Magnetic Resonance Spectroscopy (MRS)
AETNA-CPB-0226 — Hospitalization for the Initiation of Ketogenic Diet for the Treatment of Intractable Seizures
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