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E75.27 ICD-10-CM Code: Pelizaeus-Merzbacher disease

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

Coding at a Glance

Tabular directives
8 Excludes1 · 1 Excludes2
Risk adjustment
RxHCC V08 category 41

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; the Instructions section marks which is which.

Excludes1Never report with this code
  • adrenoleukodystrophy Addison-Schilder
  • mucolipidosis, types I-III (E77.0-E77.1)
  • Refsum's disease (G60.1)
  • androgen insensitivity syndrome (E34.5-)

+4 more in Instructions

Excludes2Not included here; may be reported together
  • Ehlers-Danlos syndromes (Q79.6-)

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 056 — DEGENERATIVE NERVOUS SYSTEM DISORDERS WITH MCC (MDC 01)
  • MS-DRG 057 — DEGENERATIVE NERVOUS SYSTEM DISORDERS WITHOUT MCC (MDC 01)

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 E75.27 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 E75.27 itself; “inherited from” names the category or block whose note applies here.

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

Coder workflow for E75.27

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

Before you code E75.27

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with E75.27. 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 E75.27’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.

    ReviewG60.1, E34.5, E25.0, D55, Q87.4, E29.1

Consider E75.27. Then 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).

Official instructions as workflow

  • Excludes1 — check before selecting E75.27(8 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with E75.27: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareG60.1, E34.5, E25.0, D55, Q87.4, E29.1

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

  • Excludes2 — not part of E75.27(1 note)

    Coding workflow: The conditions named in this note are not included in E75.27. 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

Coding decision scenarios

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

Documentation: Both the condition E75.27 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).

ReviewG60.1, E34.5, E25.0, D55, Q87.4, E29.1

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 E75.27 with…

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

The codes most likely to be picked instead of E75.27 — 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.

  • E77.0— 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 mucolipidosis, types I-III moves the case to E77.0; the two are not reported together unless documented as unrelated.

    Full comparison: E75.27 vs E77.0 →

  • E77.1— 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 mucolipidosis, types I-III moves the case to E77.1; the two are not reported together unless documented as unrelated.

    Full comparison: E75.27 vs E77.1 →

  • G60.1— 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 Refsum's disease moves the case to G60.1; the two are not reported together unless documented as unrelated.

    Full comparison: E75.27 vs G60.1 →

  • E34.5— 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 androgen insensitivity syndrome moves the case to E34.5; the two are not reported together unless documented as unrelated.

    Full comparison: E75.27 vs E34.5 →

  • E25.0— 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 congenital adrenal hyperplasia moves the case to E25.0; the two are not reported together unless documented as unrelated.

    Full comparison: E75.27 vs E25.0 →

  • D55— 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 hemolytic anemias attributable to enzyme disorders moves the case to D55; the two are not reported together unless documented as unrelated.

    Full comparison: E75.27 vs D55 →

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

Pelizaeus-Merzbacher disease is a billable ICD-10-CM diagnosis code (E75.27).

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

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 E75.27 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 3 Excludes1 notes across 3 chapters: E78 — Disorders of lipoprotein metabolism and other lipidemias (via E75.-), P59 — Neonatal jaundice from other and unspecified causes (via E75.-), R79 — Other abnormal findings of blood chemistry (via E75.-).

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

Referenced by 4 Excludes2 notes across 2 chapters: G11 — Hereditary ataxia (via E75.-), G71 — Primary disorders of muscles (via E75.-), N25.0 — Renal osteodystrophy (via E75.-), N25.81 — Secondary hyperparathyroidism of renal origin (via E75.-).

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

Referenced by 4 Code First instructions across 2 chapters: G63 — Polyneuropathy in diseases classified elsewhere (via E75.-), G73.7 — Myopathy in diseases classified elsewhere (via E75.-), H36 — Retinal disorders in diseases classified elsewhere (via E75.-), H42 — Glaucoma in diseases classified elsewhere (via E75.-).

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 24 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 2 MS-DRGs: DRG 056 (MDC 01), DRG 057 (MDC 01).

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); NVS006 — Other nervous system disorders (often hereditary or degenerative).

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.

E75.00 — GM2 gangliosidosis, unspecified, E75.01 — Sandhoff disease, E75.02 — Tay-Sachs disease, E75.09 — Other GM2 gangliosidosis, E75.10 — Unspecified gangliosidosis, E75.11 — Mucolipidosis IV, E75.19 — Other gangliosidosis, E75.23 — Krabbe disease, E75.25 — Metachromatic leukodystrophy, E75.26 — Sulfatase deficiency, E75.28 — Canavan disease, E75.29 — Other sphingolipidosis, E75.4 — Neuronal ceroid lipofuscinosis, F84.2 — Rett's syndrome, G31.2 — Degeneration of nervous system due to alcohol, G31.81 — Alpers disease, G31.82 — Leigh's disease, G93.81 — Temporal sclerosis, G93.89 — Other specified disorders of brain, G93.9 — Disorder of brain, unspecified, +3 more

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical categories (Other specified and unspecified nutritional and metabolic disorders, Other nervous system disorders (often hereditary or degenerative)).

E75.23 — Krabbe disease, E75.240 — Niemann-Pick disease type A, E75.241 — Niemann-Pick disease type B, E75.242 — Niemann-Pick disease type C, E75.243 — Niemann-Pick disease type D, E75.244 — Niemann-Pick disease type A/B, E75.248 — Other Niemann-Pick disease, E75.249 — Niemann-Pick disease, unspecified, E75.25 — Metachromatic leukodystrophy, E75.26 — Sulfatase deficiency, E75.28 — Canavan disease, E75.29 — Other sphingolipidosis, E75.3 — Sphingolipidosis, unspecified, E75.4 — Neuronal ceroid lipofuscinosis, E75.5 — Other lipid storage disorders, E75.6 — Lipid storage disorder, unspecified, E76.01 — Hurler's syndrome, E76.02 — Hurler-Scheie syndrome, E76.03 — Scheie's syndrome, E76.1 — Mucopolysaccharidosis, type II, +348 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main term “Aplasia”; these codes share that main term but sit in a different category of the Tabular List.

D60.0 — Chronic acquired pure red cell aplasia (red cell, chronic), D60.1 — Transient acquired pure red cell aplasia (red cell, transient), D60.8 — Other acquired pure red cell aplasias (red cell, specified type NEC), D60.9 — Acquired pure red cell aplasia, unspecified (red cell), D61.01 — Constitutional (pure) red blood cell aplasia (red cell, pure), D61.9 — Aplastic anemia, unspecified (bone marrow), D82.1 — Di George's syndrome (parathyroid-thymic), E03.1 — Congenital hypothyroidism without goiter (thyroid), K00.4 — Disturbances in tooth formation (cementum), Q00.0 — Anencephaly (brain), Q04.0 — Congenital malformations of corpus callosum (corpus callosum), Q04.3 — Other reduction deformities of brain (cerebellum), Q06.1 — Hypoplasia and dysplasia of spinal cord (ventral horn cell), Q11.1 — Other anophthalmos (eye), Q13.1 — Absence of iris (iris), Q14.1 — Congenital malformation of retina (fovea centralis), Q16.5 — Congenital malformation of inner ear (labyrinth, membranous), Q25.41 — Absence and aplasia of aorta (aorta), Q32.4 — Other congenital malformations of bronchus (bronchus), Q33.3 — Agenesis of lung (lung, congenital), +13 more

Lab tests where this diagnosis supports Medicare coverage (NCD)

Medicare's National Coverage Determination (NCD) program lists this diagnosis as medical justification for these lab tests.

Gamma-glutamyl Transferase (GGT) Test, Lipid Panel

Contextual Map

Every relationship of E75.27 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 E75.27 with these 11 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes

Referenced by Excludes2 notes

Referenced by Code First instructions

Clinical classification (CCSR)

MS-DRG Grouper

MDC crossing

  • MDC 01 — Diseases and Disorders of the Nervous System[MDC crossing]: “Diseases and Disorders of the Nervous System — 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. 8,919 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries

  • Aplasia, Pelizaeus-Merzbacher[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Merzbacher-Pelizaeus disease[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Pelizaeus-Merzbacher disease[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027

Nearest codes (30)

Change history

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. "E75.27 — Pelizaeus-Merzbacher disease." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/e75.27-pelizaeus-merzbacher-disease

Change history

  • FY2024 — October 1, 2023
    Added to the code set
    Pelizaeus-Merzbacher disease
    FY2024 changes

Nearest Codes in This Family

Official ICD-10-CM classifications closest to E75.27 in its code family, with their registry titles.

View all codes in the E75 family