2022 ICD-10-CM Diagnosis Code E75.23

Krabbe disease

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
  • E75.23 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2022 edition of ICD-10-CM E75.23 became effective on October 1, 2021.
  • This is the American ICD-10-CM version of E75.23 - other international versions of ICD-10 E75.23 may differ.
The following code(s) above E75.23 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 E75.23:
  • E00-E89
    2022 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • 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
    2022 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 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Disorders of sphingolipid metabolism and other lipid storage disorders
  • E75.2
    ICD-10-CM Diagnosis Code E75.2

    Other sphingolipidosis

      2016 2017 2018 2019 2020 2021 2022 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • adrenoleukodystrophy [Addison-Schilder] (E71.528)
    Other sphingolipidosis
Approximate Synonyms
  • Galactosylceramide beta-galactosidase deficiency
  • Globoid cell leukodystrophy, late onset
  • Krabbes disease
Clinical Information
  • A degenerative disease of the central and peripheral nervous system caused by abnormal breakdown and turnover of myelin and marked by reduced galacosylceramide beta-galactosidase activity (ec 3.2.1.46). Two types based on the age of onset are recognized: infantile-onset krabbe disease is marked by the appearance of symptoms at ages 3-6 months, which include irritability, frequent crying, and increase of muscle tonus. They are followed by opisthotonos, less of tendon reflexes, visual failure, elevated cerebrospinal fluid proteins, and delayed nerve conduction velocity. Most infants die during the second year of life. Late-onset krabbe disease (lokd) has first symptoms at ages 5 to 10 years, consisting of focal neurological signs, hemiparesis, cerebellar ataxia, cortical blindness, and spastic paraplegia, followed by mental and physical deterioration. Some patients survive into adulthood.
  • A rare inherited neurodegenerative disorder that belongs to the group of leukodystrophies. It is characterized by myelin destruction, gliosis in the brain, and the presence of multinucleated globoid cells. Signs and symptoms include irritability, mental and motor developmental disturbances, muscle weakness, seizures, blindness, and deafness.
  • An autosomal recessive metabolic disorder caused by a deficiency of galactosylceramidase leading to intralysosomal accumulation of galactolipids such as galactosylceramides and psychosine. It is characterized by demyelination associated with large multinucleated globoid cells, predominantly involving the white matter of the central nervous system. The loss of myelin disrupts normal conduction of nerve impulses.
  • Inherited, demyelinating, human lipid storage disease caused by a deficiency of galactosylceramidase; manifestations include convulsions, quadriplegia, blindness, deafness, and mental retardation.
ICD-10-CM E75.23 is grouped within Diagnostic Related Group(s) (MS-DRG v39.0):
  • 056 Degenerative nervous system disorders with mcc
  • 057 Degenerative nervous system disorders without mcc

Convert E75.23 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

Diagnosis Index entries containing back-references to E75.23:
  • Krabbe's
    • disease E75.23
  • Sclerosis, sclerotic
    • brain (generalized) (lobular) G37.9
      ICD-10-CM Diagnosis Code G37.9

      Demyelinating disease of central nervous system, unspecified

        2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
      • Krabbe's E75.23

ICD-10-CM Codes Adjacent To E75.23
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
E75.248 Other Niemann-Pick disease
E75.249 …… unspecified
E75.25 Metachromatic leukodystrophy
E75.26 Sulfatase deficiency

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