ICD-10-CM Code E88.42

MERRF syndrome

Version 2021 Billable Code

Valid for Submission

E88.42 is a billable code used to specify a medical diagnosis of merrf syndrome. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code E88.42 might also be used to specify conditions or terms like myoclonic epilepsy with ragged red fibers.

ICD-10:E88.42
Short Description:MERRF syndrome
Long Description:MERRF syndrome

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code E88.42:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Myoclonic epilepsy associated with ragged-red fibers

Code Also

Code Also
A "code also" note instructs that two codes may be required to fully describe a condition, but this note does not provide sequencing direction.
  • progressive myoclonic epilepsy G40.3

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code E88.42 are found in the index:


Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Myoclonic epilepsy with ragged red fibers

Clinical Information

  • MERRF SYNDROME-. 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

Convert E88.42 to ICD-9

  • 277.87 - Dis mitochondrial metab (Approximate Flag)

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Other and unspecified metabolic disorders (E88)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients


Metabolic Disorders

Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues, such as your liver, muscles, and body fat.

A metabolic disorder occurs when abnormal chemical reactions in your body disrupt this process. When this happens, you might have too much of some substances or too little of other ones that you need to stay healthy. There are different groups of disorders. Some affect the breakdown of amino acids, carbohydrates, or lipids. Another group, mitochondrial diseases, affects the parts of the cells that produce the energy.

You can develop a metabolic disorder when some organs, such as your liver or pancreas, become diseased or do not function normally. Diabetes is an example.

  • Acidosis (Medical Encyclopedia)
  • Alkalosis (Medical Encyclopedia)
  • Lactic acid test (Medical Encyclopedia)
  • Metabolic acidosis (Medical Encyclopedia)
  • Metabolic neuropathies (Medical Encyclopedia)
  • Pseudohypoparathyroidism (Medical Encyclopedia)

[Learn More]

Myoclonic epilepsy with ragged-red fibers Myoclonic epilepsy with ragged-red fibers (MERRF) is a disorder that affects many parts of the body, particularly the muscles and nervous system. In most cases, the signs and symptoms of this disorder appear during childhood or adolescence. The features of MERRF vary widely among affected individuals, even among members of the same family.MERRF is characterized by muscle twitches (myoclonus), weakness (myopathy), and progressive stiffness (spasticity). When the muscle cells of affected individuals are stained and viewed under a microscope, these cells usually appear abnormal. These abnormal muscle cells are called ragged-red fibers. Other features of MERRF include recurrent seizures (epilepsy), difficulty coordinating movements (ataxia), a loss of sensation in the extremities (peripheral neuropathy), and slow deterioration of intellectual function (dementia). People with this condition may also develop hearing loss or optic atrophy, which is the degeneration (atrophy) of nerve cells that carry visual information from the eyes to the brain. Affected individuals sometimes have short stature and a form of heart disease known as cardiomyopathy. Less commonly, people with MERRF develop fatty tumors, called lipomas, just under the surface of the skin.
[Learn More]