2020 ICD-10-CM Diagnosis Code E88.41

MELAS syndrome

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E88.41 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM E88.41 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E88.41 - other international versions of ICD-10 E88.41 may differ.
Applicable To
  • Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes
The following code(s) above E88.41 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E88.41:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2020 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E88
    ICD-10-CM Diagnosis Code E88

    Other and unspecified metabolic disorders

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • histiocytosis X (chronic) (C96.6)
    Use Additional
    • codes for associated conditions
    Other and unspecified metabolic disorders
  • E88.4
    ICD-10-CM Diagnosis Code E88.4

    Mitochondrial metabolism disorders

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • 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)
    Mitochondrial metabolism disorders
Approximate Synonyms
  • Juvenile myopathy, encephalopathy, lactic acidosis and stroke
  • Mitochondrial encephalopathy, lactic acidosis, stroke
Clinical Information
  • A mitochondrial disorder characterized by focal or generalized seizures, episodes of transient or persistent neurologic dysfunction resembling strokes, and ragged-red fibers on muscle biopsy. Affected individuals tend to be normal at birth through early childhood, then experience growth failure, episodic vomiting, and recurrent cerebral insults resulting in visual loss and hemiparesis. The cortical lesions tend to occur in the parietal and occipital lobes and are not associated with vascular occlusion. Vascular headache is frequently associated and the disorder tends to be familial. (from Joynt, Clinical Neurology, 1992, ch56, p117)
ICD-10-CM E88.41 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E88.41 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change

Diagnosis Index entries containing back-references to E88.41:
  • Disorder (of) - see also Disease
    • metabolism NOS E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • mitochondrial E88.40
        ICD-10-CM Diagnosis Code E88.40

        Mitochondrial metabolism disorder, unspecified

          2016 2017 2018 2019 2020 Billable/Specific Code
        • MELAS syndrome E88.41
  • MELAS syndrome E88.41
  • Syndrome - see also Disease
    • MELAS E88.41

ICD-10-CM Codes Adjacent To E88.41
E88 Other and unspecified metabolic disorders
E88.0 Disorders of plasma-protein metabolism, not elsewhere classified
E88.01 Alpha-1-antitrypsin deficiency
E88.02 Plasminogen deficiency
E88.09 Other disorders of plasma-protein metabolism, not elsewhere classified
E88.1 Lipodystrophy, not elsewhere classified
E88.2 Lipomatosis, not elsewhere classified
E88.3 Tumor lysis syndrome
E88.4 Mitochondrial metabolism disorders
E88.40 Mitochondrial metabolism disorder, unspecified
E88.41 MELAS syndrome
E88.42 MERRF syndrome
E88.49 Other mitochondrial metabolism disorders
E88.8 Other specified metabolic disorders
E88.81 Metabolic syndrome
E88.89 Other specified metabolic disorders
E88.9 Metabolic disorder, unspecified
E89 Postprocedural endocrine and metabolic complications and disorders, not elsewhere classified
E89.0 Postprocedural hypothyroidism
E89.1 Postprocedural hypoinsulinemia
E89.2 Postprocedural hypoparathyroidism

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.