2021 ICD-10-CM Code E75.25
Metachromatic leukodystrophy
Valid for Submission
E75.25 is a billable diagnosis code used to specify a medical diagnosis of metachromatic leukodystrophy. The code E75.25 is valid during the fiscal year 2021 from October 01, 2020 through September 30, 2021 for the submission of HIPAA-covered transactions.
The ICD-10-CM code E75.25 might also be used to specify conditions or terms like arylsulfatase a deficiency, metachromatic leukodystrophy, metachromatic leukodystrophy due to deficiency of cerebroside sulfatase activator, metachromatic leukodystrophy without arylsulfatase deficiency, metachromatic leukodystrophy, adult type , metachromatic leukodystrophy, congenital type, etc.
| ICD-10: | E75.25 |
| Short Description: | Metachromatic leukodystrophy |
| Long Description: | Metachromatic leukodystrophy |
Code Classification
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 E75.25 are found in the index:
- - Greenfield's disease
- - meaning
- - metachromatic leukodystrophy - E75.25
- - meaning
- - Leukoencephalopathy - See Also: Encephalopathy; - G93.49
- - metachromatic - E75.25
Approximate Synonyms
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Arylsulfatase A deficiency
- Metachromatic leukodystrophy
- Metachromatic leukodystrophy due to deficiency of cerebroside sulfatase activator
- Metachromatic leukodystrophy without arylsulfatase deficiency
- Metachromatic leukodystrophy, adult type
- Metachromatic leukodystrophy, congenital type
- Metachromatic leukodystrophy, juvenile type
- Metachromatic leukodystrophy, late infantile type
- Pelizaeus Merzbacher like disease
- Sphingolipid activator protein 1 deficiency
Clinical Information
- LEUKODYSTROPHY METACHROMATIC-. an autosomal recessive metabolic disease caused by a deficiency of cerebroside sulfatase leading to intralysosomal accumulation of cerebroside sulfate sulfoglycosphingolipids in the nervous system and other organs. pathological features include diffuse demyelination and metachromatically staining granules in many cell types such as the glial cells. there are several allelic and nonallelic forms with a variety of neurological symptoms.
Convert E75.25 to ICD-9 Code
The General Equivalency Mapping (GEM) crosswalk indicates an approximate mapping between the ICD-10 code E75.25 its ICD-9 equivalent. The approximate mapping means there is not an exact match between the ICD-10 code and the ICD-9 code and the mapped code is not a precise representation of the original code.
- 330.0 - Leukodystrophy (Approximate Flag)
Information for Patients
Leukodystrophies
The leukodystrophies are rare diseases that affect the cells of the brain. Specifically, the diseases affect the myelin sheath, the material that surrounds and protects nerve cells. Damage to this sheath slows down or blocks messages between the brain and the rest of the body. This leads to problems with
- Movement
- Speaking
- Vision
- Hearing
- Mental and physical development
Most of the leukodystrophies are genetic. They usually appear during infancy or childhood. They can be hard to detect early because children seem healthy at first. However, symptoms gradually get worse over time.
There are no cures for any of the leukodystrophies. Medicines, speech therapy and physical therapy might help with symptoms. Researchers are testing bone marrow transplantation as a treatment for some of the leukodystrophies.
NIH: National Institute of Neurological Disorders and Stroke
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Metachromatic leukodystrophy Metachromatic leukodystrophy is an inherited disorder characterized by the accumulation of fats called sulfatides in cells. This accumulation especially affects cells in the nervous system that produce myelin, the substance that insulates and protects nerves. Nerve cells covered by myelin make up a tissue called white matter. Sulfatide accumulation in myelin-producing cells causes progressive destruction of white matter (leukodystrophy) throughout the nervous system, including in the brain and spinal cord (the central nervous system) and the nerves connecting the brain and spinal cord to muscles and sensory cells that detect sensations such as touch, pain, heat, and sound (the peripheral nervous system).In people with metachromatic leukodystrophy, white matter damage causes progressive deterioration of intellectual functions and motor skills, such as the ability to walk. Affected individuals also develop loss of sensation in the extremities (peripheral neuropathy), incontinence, seizures, paralysis, an inability to speak, blindness, and hearing loss. Eventually they lose awareness of their surroundings and become unresponsive. While neurological problems are the primary feature of metachromatic leukodystrophy, effects of sulfatide accumulation on other organs and tissues have been reported, most often involving the gallbladder.The most common form of metachromatic leukodystrophy, affecting about 50 to 60 percent of all individuals with this disorder, is called the late infantile form. This form of the disorder usually appears in the second year of life. Affected children lose any speech they have developed, become weak, and develop problems with walking (gait disturbance). As the disorder worsens, muscle tone generally first decreases, and then increases to the point of rigidity. Individuals with the late infantile form of metachromatic leukodystrophy typically do not survive past childhood.In 20 to 30 percent of individuals with metachromatic leukodystrophy, onset occurs between the age of 4 and adolescence. In this juvenile form, the first signs of the disorder may be behavioral problems and increasing difficulty with schoolwork. Progression of the disorder is slower than in the late infantile form, and affected individuals may survive for about 20 years after diagnosis.The adult form of metachromatic leukodystrophy affects approximately 15 to 20 percent of individuals with the disorder. In this form, the first symptoms appear during the teenage years or later. Often behavioral problems such as alcoholism, drug abuse, or difficulties at school or work are the first symptoms to appear. The affected individual may experience psychiatric symptoms such as delusions or hallucinations. People with the adult form of metachromatic leukodystrophy may survive for 20 to 30 years after diagnosis. During this time there may be some periods of relative stability and other periods of more rapid decline.Metachromatic leukodystrophy gets its name from the way cells with an accumulation of sulfatides appear when viewed under a microscope. The sulfatides form granules that are described as metachromatic, which means they pick up color differently than surrounding cellular material when stained for examination.
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Code History
- FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
- FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
- FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
- FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
- FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
- FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)