ICDcodes.org
FY2027

E88.42MERRF syndrome

BillableCC

Is E88.42 billable?

Yes — E88.42 is billable for FY2027. E88.42 is a valid, billable ICD-10-CM code at the highest level of specificity in its branch. E88.42 may be submitted for encounters from October 1, 2026 through September 30, 2027.

E88.42 at a glance

CodeE88.42
DescriptionMERRF syndrome
BillableYes
Code setICD-10-CM FY2027
Valid for encountersOctober 1, 2026 – September 30, 2027

Which MS-DRGs does E88.42 group to?

E88.42 sits in MDC 10 and helps define the logic of 1 MS-DRG (version 44, FY2027). Which one a stay actually groups to also depends on procedures and secondary diagnoses.

  • 642Inborn and Other Disorders of Metabolismmedical

As a secondary diagnosis, E88.42 is a complication or comorbidity (CC), which can move a stay into a higher-paying DRG.

How is E88.42 listed in the Alphabetic Index?

These are the routes through the Alphabetic Index that lead to E88.42. They show the wording a clinician may have documented, which often differs from the Tabular description.

  • MERRF syndrome (myoclonic epilepsy associated with ragged-red fiber)
  • SyndromeMERRF
  • Disordermetabolism NOSmitochondrialMERRF syndrome (of)

Code also

Two codes may be needed to describe the condition fully. Whether E88.42 is sequenced first depends on the reason for the encounter.

  • progressive myoclonic epilepsy (G40.3-)

Inclusion terms

Alternative wording in documentation that is classified to E88.42.

  • Myoclonic epilepsy associated with ragged-red fibers

Excludes1 — never code together — inherited from Chapter 4

The conditions below can never be reported together with E88.42 on the same claim. An Excludes1 note means the two conditions cannot occur in the same patient, so reporting both is a coding error.

  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Excludes1 — never code together — inherited from E70-E88

The conditions below can never be reported together with E88.42 on the same claim. An Excludes1 note means the two conditions cannot occur in the same patient, so reporting both is a coding error.

  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)
  • 5-alpha-reductase deficiency (E29.1)

Excludes1 — never code together — inherited from E88

The conditions below can never be reported together with E88.42 on the same claim. An Excludes1 note means the two conditions cannot occur in the same patient, so reporting both is a coding error.

  • histiocytosis X (chronic) (C96.6)

Excludes1 — never code together — inherited from E88.4

The conditions below can never be reported together with E88.42 on the same claim. An Excludes1 note means the two conditions cannot occur in the same patient, so reporting both is a coding error.

  • disorders of pyruvate metabolism (E74.4)
  • Kearns-Sayre syndrome (H49.81)
  • Leber's disease (H47.22)
  • Leigh's encephalopathy (G31.82)
  • Mitochondrial myopathy, NEC (G71.3)
  • Reye's syndrome (G93.7)

Use additional code — inherited from E88

Report an additional code alongside E88.42 to fully describe the condition. E88.42 is sequenced first.

  • codes for associated conditions

Excludes2 — not included here — inherited from E70-E88

The conditions below are not part of E88.42, but a patient may have both at the same time. When documentation supports it, E88.42 and the excluded code may both be reported.

  • Ehlers-Danlos syndromes (Q79.6-)

How long has E88.42 existed?

E88.42 has been in ICD-10-CM since at least FY2016, the earliest fiscal year on record here.

Derived from the official order files for FY2016–FY2026. FY2017 and FY2020 publish no order file, so those years are not covered.

What other codes are in the E88.4 family? (4)