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ICD-10-CM Codes
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E00-E89
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E70-E88
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E88-
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2023 ICD-10-CM Diagnosis Code E88.42
2023 ICD-10-CM Diagnosis Code E88.42
MERRF syndrome
2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
- E88.42 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
- The 2023 edition of ICD-10-CM E88.42 became effective on October 1, 2022.
- This is the American ICD-10-CM version of E88.42 - other international versions of ICD-10 E88.42 may differ.
Applicable To- Myoclonic epilepsy associated with ragged-red fibers
Code AlsoCode Also Help
A code also note instructs that 2 codes may be required to fully describe a condition but the sequencing of the two codes is discretionary, depending on the severity of the conditions and the reason for the encounter.
The following code(s) above
E88.42 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
E88.42:
- E00-E89
2023 ICD-10-CM Range E00-E89
Endocrine, nutritional and metabolic diseasesNote- 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)
- E70-E88
2023 ICD-10-CM Range E70-E88
Metabolic disordersType 1 Excludes- androgen insensitivity syndrome (E34.5-)
- congenital adrenal hyperplasia (E25.0)
- hemolytic anemias attributable to enzyme disorders (D55.-)
- Marfan's syndrome (Q87.4)
- 5-alpha-reductase deficiency (E29.1)
Type 2 Excludes- Ehlers-Danlos syndromes (Q79.6-)
- E88
ICD-10-CM Diagnosis Code E88
Other and unspecified metabolic disorders
2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
Type 1 Excludes- histiocytosis X (chronic) (C96.6)
Use Additional- codes for associated conditions
- E88.4
ICD-10-CM Diagnosis Code E88.4
Mitochondrial metabolism disorders
2016 2017 2018 2019 2020 2021 2022 2023 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)
- Myoclonus epilepsy and ragged red fibers
- Myoclonus epilepsy and ragged red fibers syndrome
Clinical Information
- A mitochondrial encephalomyopathy characterized clinically by a mixed seizure disorder, myoclonus, progressive ataxia, spasticity, and a mild myopathy. Dysarthria, optic atrophy, growth retardation, deafness, and dementia may also occur. This condition tends to present in childhood and to be transmitted via maternal lineage. Muscle biopsies reveal ragged-red fibers and respiratory chain enzymatic defects. (from Adams et al., Principles of Neurology, 6th ed, p986)
- A very rare mitochondrial abnormality characterized by myoclonic epilepsy and the microscopic finding of ragged-red fibers in muscle tissues.
ICD-10-CM E88.42 is grouped within Diagnostic Related Group(s) (MS-DRG v40.0):
- 642 Inborn and other disorders of metabolism
Convert E88.42 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
- 2021 (effective 10/1/2020): No change
- 2022 (effective 10/1/2021): No change
- 2023 (effective 10/1/2022): No change
Code annotations containing back-references to E88.42:
- Disorder (of) - see also Disease
- metabolism NOS E88.9
ICD-10-CM Diagnosis Code E88.9
Metabolic disorder, unspecified
2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
- mitochondrial E88.40
ICD-10-CM Diagnosis Code E88.40
Mitochondrial metabolism disorder, unspecified
2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
- MERRF syndrome E88.42 (myoclonic epilepsy associated with ragged-red fibers)
- MERRF syndrome E88.42 (myoclonic epilepsy associated with ragged-red fiber)
- Syndrome - see also Disease
- MERRF E88.42 (myoclonic epilepsy associated with ragged-red fibers)
ICD-10-CM Codes Adjacent To E88.42
E88.0 Disorders of plasma-protein metabolism, not elsewhere classified
E88.01 Alpha-1-antitrypsin 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
E88.42
MERRF syndrome
E88.49 Other mitochondrial metabolism disorders
E88.8 Other specified metabolic disorders
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
E89.3 Postprocedural hypopituitarism
Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.
