2023 ICD-10-CM Diagnosis Code E75.19

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Other gangliosidosis

    2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
  • E75.19 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 E75.19 became effective on October 1, 2022.
  • This is the American ICD-10-CM version of E75.19 - other international versions of ICD-10 E75.19 may differ.
Applicable To
  • GM1 gangliosidosis
  • GM3 gangliosidosis
The following code(s) above E75.19 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 E75.19:
  • E00-E89
    2023 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    • 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
    2023 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 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-)
    Metabolic disorders
  • E75
    ICD-10-CM Diagnosis Code E75

    Disorders of sphingolipid metabolism and other lipid storage disorders

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Disorders of sphingolipid metabolism and other lipid storage disorders
Clinical Information
  • A ganglioside biosynthesis disorder caused by (n-acetylneuraminyl)-galactosylglucosylceramide n-acetyl transferase (e.c. 2.4.192) deficiency with excessive accumulation of ganglioside gm3 in the liver and brain tissue and absence of higher ganglioside homologs. Clinical features include limpness, retarded psychomotor development, coarsening of facial features, macroglossia, gingival hypertrophy, hepatosplenomegaly, inguinal hernia, and stubby hands and feet. Most patients die in infancy.
  • A ganglioside storage disorder due to beta-galactosidase (ec deficiency and abnormal accumulation of gm1 ganglioside in neurons and in hepatic, splenic and other histiocytes and in renal glomerular epithelium due. The symptoms appear shortly after birth; they include retarded psychomotor development, failure to thrive, startle reaction to sounds, feeding difficulty, hepatosplenomegaly, hurler (gargoyle-like) facies (coarse facial features, macrocephaly, broad nose, frontal bossing, long philtrum, prominent maxilla, and macroglossia), bone defects similar to those seen in hurler syndrome (mainly dysostosis multiplex and long bone and vertebral anomalies), and other abnormalities. Severe cerebral degeneration follows with death in the first two years of life usually due to bronchopneumonia. The affected infants are often blind, deaf, and quadriplegic.
  • 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.
  • An autosomal recessive neurodegenerative disorder caused by the absence or deficiency of beta-galactosidase. It is characterized by intralysosomal accumulation of g(m1) ganglioside and oligosaccharides, primarily in neurons of the central nervous system. The infantile form is characterized by muscle hypotonia, poor psychomotor development, hirsutism, hepatosplenomegaly, and facial abnormalities. The juvenile form features hyperacusis; seizures; and psychomotor retardation. The adult form features progressive dementia; ataxia; and muscle spasticity. (from menkes, textbook of child neurology, 5th ed, pp96-7)
  • Form of gangliosidosis characterized by accumulation of g(m1) ganglioside and oligosaccharides in lysosomes caused by an absence or severe deficiency of the enzyme beta-galactosidase (type a1); three phenotypes of this disorder are infantile (generalized), juvenile, and adult; the infantile form is characterized by skeletal abnormalities, hypotonia, poor psychomotor development, hirsutism, hepatosplenomegaly, and facial abnormalities; the juvenile form features hyperacusis, seizures, and psychomotor retardation; the adult form features progressive intellectual deterioration, involuntary movements, ataxia, and spasticity.
ICD-10-CM E75.19 is grouped within Diagnostic Related Group(s) (MS-DRG v40.0):
  • 056 Degenerative nervous system disorders with mcc
  • 057 Degenerative nervous system disorders without mcc

Convert E75.19 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

Diagnosis Index entries containing back-references to E75.19:
  • Gangliosidosis E75.10
    ICD-10-CM Diagnosis Code E75.10

    Unspecified gangliosidosis

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
    Applicable To
    • Gangliosidosis NOS
    • GM1 E75.19
    • GM3 E75.19

ICD-10-CM Codes Adjacent To E75.19
E74.9 Disorder of carbohydrate metabolism, unspecified
E75 Disorders of sphingolipid metabolism and other lipid storage disorders
E75.0 GM2 gangliosidosis
E75.00 …… unspecified
E75.01 Sandhoff disease
E75.02 Tay-Sachs disease
E75.09 Other GM2 gangliosidosis
E75.1 Other and unspecified gangliosidosis
E75.10 Unspecified gangliosidosis
E75.11 Mucolipidosis IV
E75.19 Other gangliosidosis
E75.2 Other sphingolipidosis
E75.21 Fabry (-Anderson) disease
E75.22 Gaucher disease
E75.23 Krabbe disease
E75.24 Niemann-Pick disease
E75.240 …… type A
E75.241 …… type B
E75.242 …… type C
E75.243 …… type D
E75.244 …… type A/B

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