Version 2024

2024 ICD-10-CM Diagnosis Code E75.19

Other gangliosidosis

ICD-10-CM Code:: E75.19
ICD-10 Code for:: Other gangliosidosis
Is Billable?: Yes - Valid for Submission
Chronic Condition Indicator: ^[1]: Chronic
Code Navigator:

Code Information
Approximate Synonyms
Clinical Classification
Clinical Information
Tabular List of Diseases and Injuries
Index to Diseases and Injuries References
Convert to ICD-9 Code
Patient Education
Other Codes Used Similar Conditions
Code History

Code Classification

Endocrine, nutritional and metabolic diseases
(E00–E89)
- Metabolic disorders
  (E70-E88)
  - Disorders of sphingolipid metabolism and other lipid storage disorders
    (E75)

E75.19 is a billable diagnosis code used to specify a medical diagnosis of other gangliosidosis. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.

Approximate Synonyms

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

Adult GM1 gangliosidosis
GM1 gangliosidosis
Infantile GM1 gangliosidosis
Juvenile GM1 gangliosidosis

Clinical Classification

Clinical Category	CCSR Category Code	Inpatient Default CCSR	Outpatient Default CCSR
Other nervous system disorders (often hereditary or degenerative)	NVS006	N - Not default inpatient assignment for principal diagnosis or first-listed diagnosis.	N - Not default outpatient assignment for principal diagnosis or first-listed diagnosis.
Other specified and unspecified nutritional and metabolic disorders	END016	Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.	Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.

Clinical Information

GM1 Gangliosidosis
an autosomal recessive lysosomal storage disease characterized by deficiency of the enzyme acid beta-galactosidase, resulting in the accumulation of acid lipids in the nervous system. signs and symptoms include neurologic disturbances, muscle atrophy, dystonia, eye abnormalities, and formation of angiokeratomas.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.

Inclusion Terms

GM1 gangliosidosis
GM3 gangliosidosis

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

- Gangliosidosis - E75.10
- - GM1 - E75.19
- - GM3 - E75.19

Convert E75.19 to ICD-9-CM

ICD-9-CM Code: 330.1 - Cerebral lipidoses
Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education

Genetic Brain Disorders

A genetic brain disorder is caused by a variation or a mutation in a gene. A variation is a different form of a gene. A mutation is a change in a gene. Genetic brain disorders affect the development and function of the brain.

Some genetic brain disorders are due to random gene mutations or mutations caused by environmental exposure, such as cigarette smoke. Other disorders are inherited, which means that a mutated gene or group of genes is passed down through a family. They can also be due to a combination of both genetic changes and other outside factors.

Some examples of genetic brain disorders include:

Leukodystrophies
Phenylketonuria
Tay-Sachs disease
Wilson disease

Many people with genetic brain disorders fail to produce enough of certain proteins that influence brain development and function. These brain disorders can cause serious problems that affect the nervous system. Some have treatments to control symptoms. Some are life-threatening.

[Learn More in MedlinePlus]

GM1 gangliosidosis

GM1 gangliosidosis is an inherited disorder that destroys nerve cells (neurons) in the brain and spinal cord. This condition can be classified as one of three major types based on the age at which signs and symptoms first appear. However, the signs and symptoms of these three types can overlap, leading some researchers to believe that GM1 gangliosidosis occurs on a spectrum instead of as three distinct types.

The signs and symptoms of the most severe form of GM1 gangliosidosis, called type I or the infantile form, usually develop by the age of 6 months. Infants with this form of the disorder typically appear normal until their development slows and the muscles used for movement weaken. Affected infants eventually lose the skills they had previously acquired (developmentally regress) and may develop an exaggerated startle reaction to loud noises. Over time, children with GM1 gangliosidosis type I develop an enlarged liver and spleen (hepatosplenomegaly) and skeletal abnormalities. Affected children often have seizures and profound intellectual disability.

People with GM1 gangliosidosis type I can lose their vision due to clouding of the clear outer covering of the eye (the cornea) and the breakdown of the light-sensing tissue at the back of the eye (the retina). Affected individuals also develop a red area in the eye known as a cherry-red spot. In some cases, affected individuals have distinctive facial features that are described as "coarse," enlarged gums (gingival hypertrophy), and an enlarged and weakened heart muscle (cardiomyopathy). Individuals with type I usually do not survive past early childhood.

GM1 gangliosidosis type II occurs in one of two forms: the late infantile or the juvenile forms. Children with type II develop normally early in life, but they begin to show signs and symptoms of the condition around the age of 18 months (late infantile form) or 5 years (juvenile form). Individuals with GM1 gangliosidosis type II experience developmental regression but usually do not have cherry-red spots, coarse facial features, or enlarged organs. Type II usually progresses more slowly than type I, but it still shortens life expectancy. People with the late infantile form typically survive into mid-childhood, while those with the juvenile form may live into early adulthood.

GM1 gangliosidosis type III is the adult or chronic form of the condition, and this is the mildest form. The age at which symptoms first appear varies in people with GM1 gangliosidosis type III, although most affected individuals develop signs and symptoms in their teens. The characteristic features of this type include involuntary tensing of various muscles (dystonia) and abnormalities of the spinal bones (vertebrae). Life expectancy varies among people with GM1 gangliosidosis type III.