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E88.41 ICD-10-CM Code: MELAS syndrome

Compare with another code for E88.41Check this code on a claim

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Official instructions
1 inclusion term · 12 Excludes1 · 1 Excludes2 · 1 use-additional instruction
Risk adjustment
CMS-HCC V22 category 23 · RxHCC V08 category 43

What these terms mean →

Billable · FY2027A valid, specific ICD-10-CM code, reportable for dates of service in FY2027.

What you need to know

Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too, and is marked with where it comes from.

Use additional codefrom E88Report with this code when documented
  • codes for associated conditions
Excludes1Not reported with this code unless documented as unrelated
  • disorders of pyruvate metabolism (E74.4) (from E88.4)
  • Kearns-Sayre syndrome (H49.81) (from E88.4)
  • Leber's disease (H47.22) (from E88.4)
  • Leigh's encephalopathy (G31.82) (from E88.4)

+8 more in Instructions

Excludes2from E70-E88Not included here; may be reported together
  • Ehlers-Danlos syndromes (Q79.6-)
IncludesWhat this code covers
  • Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes

Where it sits Source: CMS/NCHS

Under:Chapter 4: Endocrine, Nutritional and Metabolic DiseasesE70-E88 Metabolic disordersE88 Other and unspecified metabolic disordersE88.4 Mitochondrial metabolism disorders

Most relevant related codes MedCoder-derived

Read off the official notes above and this code’s own position in the tabular list. Which to report is a documentation question; Compare shows the two side by side.

CompareCheck ClaimView Related Codes

Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v44, Appendix B.

  • MS-DRG 642 — INBORN AND OTHER DISORDERS OF METABOLISM (MDC 10)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for E88.41 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2027Effective: October 1, 2026

Trace:FY2027 changesChange historyRelease, file and checksum

Notes without a marker are published on E88.41 itself; “inherited from” names the category or block whose note applies here.

Inclusion Terms

Alternative terms the tabular list files under this code.

  • Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes

Excludes1 — Not Coded Here

Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Excludes2 — Not Included Here

Conditions not covered by this code, but which may be reported alongside it when both are present.

Source: inherited from E70-E88

Use Additional Code

Supplementary codes the tabular list directs you to add.

  • codes for associated conditions

Source: inherited from E88

Coder workflow for E88.41

MedCoder structured workflow — derived from this code’s own official record

Before you code E88.41

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with E88.41. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

    See the official tabular notes

Choose the right path

  1. Does the documentation support a condition named in E88.41’s Excludes1 note?
    Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
    No → Continue.

    ReviewE74.4, H49.81, H47.22, G31.82, G71.3, G93.7

Consider E88.41. Then work the Use Additional Code note, and confirm the code is valid for the date of service in the Verify section.

Documentation check

The provider’s diagnostic statement
Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).
The conditions the Use Additional Code note names
Reported with this code when documented; a conditional instruction (“if applicable”, “if known”) applies only when the record supports it.

Official instructions as workflow

  • Excludes1 — check before selecting E88.41(12 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with E88.41: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareE74.4, H49.81, H47.22, G31.82, G71.3, G93.7

    See the official tabular notes · Guidelines I.A.12.a

  • Excludes2 — not part of E88.41(1 note)

    Coding workflow: The conditions named in this note are not included in E88.41. When the record documents both, both may be reported; the note is a boundary, not a prohibition.

    CompareQ79.6

    See the official tabular notes · Guidelines I.A.12.b

  • Use Additional Code — after identifying E88.41(1 note)

    Coding workflow: Check whether the documentation supports the additional code(s) the note names, and report them with E88.41 when it does. Where the instruction is conditional (“if applicable”, “if known”), it applies only when the record documents the condition.

    See the official tabular notes · Guidelines I.A.13

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Documentation: Both the condition E88.41 describes and a condition named in its Excludes1 note are documented for the same encounter.

Coding question: Can both codes be reported?

Path: Review the Excludes1 note and the excluded code, and look for a provider statement on whether the two conditions are related.

Reason: Excludes1 means the two are not coded together; the exception is when the record shows the conditions are unrelated to each other (Guidelines I.A.12.a).

ReviewE74.4, H49.81, H47.22, G31.82, G71.3, G93.7

Documentation: A condition the Use Additional Code note names is documented.

Coding question: Is a second code reported with E88.41?

Path: Review the Use Additional Code note and the code it names.

Reason: The additional code is reported when the record documents the condition; a conditional instruction applies only when its condition is met (Guidelines I.A.13).

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Don’t confuse E88.41 with…

MedCoder analysis — pairs from this code’s official notes, distinctions written by MedCoder

The codes most likely to be picked instead of E88.41 — named in its own official notes, or sharing its laterality or encounter family — with what separates them and which documentation moves the case. Each links to the full comparison.

  • E74.4— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of disorders of pyruvate metabolism moves the case to E74.4; the two are not reported together unless documented as unrelated.

    Full comparison: E88.41 vs E74.4 →

  • H49.81— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of Kearns-Sayre syndrome moves the case to H49.81; the two are not reported together unless documented as unrelated.

    Full comparison: E88.41 vs H49.81 →

  • H47.22— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of Leber's disease moves the case to H47.22; the two are not reported together unless documented as unrelated.

    Full comparison: E88.41 vs H47.22 →

  • G31.82— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of Leigh's encephalopathy moves the case to G31.82; the two are not reported together unless documented as unrelated.

    Full comparison: E88.41 vs G31.82 →

  • G71.3— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of Mitochondrial myopathy, NEC moves the case to G71.3; the two are not reported together unless documented as unrelated.

    Full comparison: E88.41 vs G71.3 →

  • G93.7— Excludes1 conflict (Excludes1 — generally not reported together)

    Not coded here — the tabular list excludes this pair for the same encounter.

    Not reported together with this code, except where the two conditions are documented as unrelated.

    What moves you there: Documentation of Reye's syndrome moves the case to G93.7; the two are not reported together unless documented as unrelated.

    Full comparison: E88.41 vs G93.7 →

Relationships are read from this code's own official notes, its laterality siblings and its encounter family; the wording of each distinction is MedCoder editorial, and the documentation named in each “what moves you there” line is the official note phrase or sibling title itself. Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a.

Coding context

Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

Code Overview

MELAS syndrome is a billable ICD-10-CM diagnosis code (E88.41).

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Decision Points

The directives on this code's own record, as a pre-claim checklist.

  1. 1 Use Additional Code instruction — report the named additional code(s) when the documentation supports them. See the Use Additional Code notes
  2. 12 Excludes1 entries — codes named there are generally not reported together with this code (Guidelines I.A.12.a). See the Excludes1 notes
  3. 1 Excludes2 entry — those conditions are not part of this code and may be reported additionally when documented. See the Excludes2 notes

Checklist rows are derived from this code's own official directives; the wording of each check is MedCoder editorial. The official notes themselves are in the sections each row links to.

Verify Before Coding

  • CC as a secondary diagnosis (FY2027). Can raise the stay's MS-DRG severity tier.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder-derived relationships — computed from published CMS and AHRQ datasets

Other codes that name E88.41 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 1 Excludes1 note: P59 — Neonatal jaundice from other and unspecified causes (via E88.-).

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

Referenced by 14 Excludes2 notes across 3 chapters: G11 — Hereditary ataxia (via E88.-), G71 — Primary disorders of muscles (via E88.-), N25.0 — Renal osteodystrophy (via E88.-), N25.81 — Secondary hyperparathyroidism of renal origin (via E88.-), Q90 — Down syndrome (via E88.4.-), Q90-Q99 — Chromosomal abnormalities, not elsewhere classified (Q90-Q99) (via E88.4.-), Q91 — Trisomy 18 and Trisomy 13 (via E88.4.-), Q92 — Other trisomies and partial trisomies of the autosomes, not elsewhere classified (via E88.4.-), Q93 — Monosomies and deletions from the autosomes, not elsewhere classified (via E88.4.-), Q95 — Balanced rearrangements and structural markers, not elsewhere classified (via E88.4.-), Q96 — Turner's syndrome (via E88.4.-), Q97 — Other sex chromosome abnormalities, female phenotype, not elsewhere classified (via E88.4.-), Q98 — Other sex chromosome abnormalities, male phenotype, not elsewhere classified (via E88.4.-), Q99 — Other chromosome abnormalities, not elsewhere classified (via E88.4.-).

These codes’ tabular lists name this diagnosis as distinct — both may be reported when both are documented.

Referenced by 2 Code First instructions across 2 chapters: G63 — Polyneuropathy in diseases classified elsewhere (via E88.-), H42 — Glaucoma in diseases classified elsewhere (via E88.-).

Each of these codes carries a Code First note naming this condition — when that code is reported, THIS code is sequenced first, ahead of it.

MS-DRG Grouper Relationships (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 MS-DRG: CC — Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 41 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 1 MS-DRG: DRG 642 (MDC 10).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):END016 — Other specified and unspecified nutritional and metabolic disorders (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same clinical process (MS-DRG)

Acts as CC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.

E71.520 — Childhood cerebral X-linked adrenoleukodystrophy, E71.521 — Adolescent X-linked adrenoleukodystrophy, E71.522 — Adrenomyeloneuropathy, E71.528 — Other X-linked adrenoleukodystrophy, E71.529 — X-linked adrenoleukodystrophy, unspecified type, E71.53 — Other group 2 peroxisomal disorders, E71.540 — Rhizomelic chondrodysplasia punctata, E71.541 — Zellweger-like syndrome, E71.542 — Other group 3 peroxisomal disorders, E71.548 — Other peroxisomal disorders, E80.3 — Defects of catalase and peroxidase, E80.4 — Gilbert syndrome, E80.5 — Crigler-Najjar syndrome, E80.6 — Other disorders of bilirubin metabolism, E80.7 — Disorder of bilirubin metabolism, unspecified, E88.811 — Insulin resistance syndrome, Type A, E88.818 — Other insulin resistance, E88.819 — Insulin resistance, unspecified, E88.82 — Obesity due to disruption of MC4R pathway, E88.89 — Other specified metabolic disorders, +20 more

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Other specified and unspecified nutritional and metabolic disorders).

E88.1 — Lipodystrophy, not elsewhere classified, E88.10 — Lipodystrophy, unspecified, E88.11 — Partial lipodystrophy, E88.12 — Generalized lipodystrophy, E88.13 — Localized lipodystrophy, E88.14 — HIV-associated lipodystrophy, E88.19 — Other lipodystrophy, not elsewhere classified, E88.2 — Lipomatosis, not elsewhere classified, E88.3 — Tumor lysis syndrome, E88.40 — Mitochondrial metabolism disorder, unspecified, E88.42 — MERRF syndrome, E88.43 — Disorders of mitochondrial tRNA synthetases, E88.49 — Other mitochondrial metabolism disorders, E88.81 — Metabolic syndrome and other insulin resistance, E88.810 — Metabolic syndrome, E88.811 — Insulin resistance syndrome, Type A, E88.818 — Other insulin resistance, E88.819 — Insulin resistance, unspecified, E88.82 — Obesity due to disruption of MC4R pathway, E88.89 — Other specified metabolic disorders, +258 more

Contextual Map

Every relationship of E88.41 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run E88.41 with these 11 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes

Referenced by Excludes2 notes (14)

Referenced by Code First instructions

Clinical classification (CCSR)

MS-DRG Grouper

  • CC — Complication or Comorbidity [MS-DRG severity]: “As a secondary diagnosis this code can raise the stay's MS-DRG severity tier (CC).”— CMS MS-DRG Definitions Manual (Appendix C) · FY2027
  • DRG 642 — INBORN AND OTHER DISORDERS OF METABOLISM[MS-DRG]: “INBORN AND OTHER DISORDERS OF METABOLISM (MDC 10)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2027

MDC crossing

  • MDC 10 — Endocrine, Nutritional and Metabolic Diseases and Disorders[MDC crossing]: “Endocrine, Nutritional and Metabolic Diseases and Disorders — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 5,013 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries

  • Disorder (of), metabolism NOS, mitochondrial, MELAS syndrome[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • MELAS syndrome[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Syndrome, MELAS[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027

Nearest codes (29)

Change history

  • FY2016 — In the code set at ICD-10-CM adoption [Change history]— CMS release files (code change ledger) · icd10cm-fy2016

Reference

Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.

Indexed Clinical Terms (3)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v44.0 (October 2026) Release, file and checksum · Publisher’s page
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v44 Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder-derived relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder editorial explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 28, 2026 · All releases and sources

Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "E88.41 — MELAS syndrome." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/e88.41-melas-syndrome

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    MELAS syndrome

No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027, and none are recorded for this code. Note changes are tracked from FY2027 only.

Nearest Codes in This Family

Official ICD-10-CM classifications closest to E88.41 in its code family, with their registry titles.

View all codes in the E88 family