2019 ICD-10-CM Diagnosis Code G31.82

Leigh's disease

    2016 2017 2018 2019 Billable/Specific Code
  • G31.82 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2019 edition of ICD-10-CM G31.82 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of G31.82 - other international versions of ICD-10 G31.82 may differ.
Applicable To
  • Subacute necrotizing encephalopathy
The following code(s) above G31.82 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 G31.82:
  • G00-G99
    2019 ICD-10-CM Range G00-G99

    Diseases of the nervous system

    Type 2 Excludes
    • certain conditions originating in the perinatal period (P04-P96)
    • certain infectious and parasitic diseases (A00-B99)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Diseases of the nervous system
  • G31
    ICD-10-CM Diagnosis Code G31

    Other degenerative diseases of nervous system, not elsewhere classified

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 2 Excludes
    Use Additional
    • code to identify:
    • dementia with behavioral disturbance (F02.81)
    • dementia without behavioral disturbance (F02.80)
    Other degenerative diseases of nervous system, not elsewhere classified
Approximate Synonyms
  • Leighs disease
Clinical Information
  • A group of metabolic disorders primarily of infancy characterized by the subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, dysphagia, and lactic acidosis. Pathological features include spongy degeneration of the neuropile of the basal ganglia, thalamus, brain stem, and spinal cord. Patterns of inheritance include x-linked recessive, autosomal recessive, and mitochondrial. Leigh disease has been associated with mutations in genes for the pyruvate dehydrogenase complex; cytochrome-c oxidase; atp synthase subunit 6; and subunits of mitochondrial complex i. (from menkes, textbook of child neurology, 5th ed, p850).
  • An inherited disorder affecting the nervous system, caused by genetic mutations in the mitochondrial dna or deficiency of pyruvate dehydrogenase. Signs and symptoms appear in infancy and include loss of the motor abilities, poor sucking abilities, irritability, lack of muscle tone, and seizures.
ICD-10-CM G31.82 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 056 Degenerative nervous system disorders with mcc
  • 057 Degenerative nervous system disorders without mcc

Convert G31.82 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
Code annotations containing back-references to G31.82:
  • Type 1 Excludes: E74.4
    , E88.4
    , G71.3
    ICD-10-CM Diagnosis Code E74.4

    Disorders of pyruvate metabolism and gluconeogenesis

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Deficiency of phosphoenolpyruvate carboxykinase
    • Deficiency of pyruvate carboxylase
    • Deficiency of pyruvate dehydrogenase
    Type 1 Excludes
    • disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)
    • Leigh's syndrome (G31.82)
    ICD-10-CM Diagnosis Code E88.4

    Mitochondrial metabolism disorders

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • disorders of pyruvate metabolism (E74.4)
    • Kearns-Sayre syndrome (H49.81)
    • Leber's disease (H47.22)
    • Leigh's encephalopathy (G31.82)
    • Mitochondrial myopathy, NEC (G71.3)
    • Reye's syndrome (G93.7)
    ICD-10-CM Diagnosis Code G71.3

    Mitochondrial myopathy, not elsewhere classified

      2016 2017 2018 2019 Billable/Specific Code
    Type 1 Excludes
    • Kearns-Sayre syndrome (H49.81)
    • Leber's disease (H47.21)
    • Leigh's encephalopathy (G31.82)
    • mitochondrial metabolism disorders (E88.4.-)
    • Reye's syndrome (G93.7)

Diagnosis Index entries containing back-references to G31.82:
  • Encephalopathy (acute) G93.40
    ICD-10-CM Diagnosis Code G93.40

    Encephalopathy, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    • necrotizing, subacute G31.82 (Leigh)
  • Leigh's disease G31.82

ICD-10-CM Codes Adjacent To G31.82
G30.8 Other Alzheimer's disease
G30.9 Alzheimer's disease, unspecified
G31 Other degenerative diseases of nervous system, not elsewhere classified
G31.0 Frontotemporal dementia
G31.01 Pick's disease
G31.09 Other frontotemporal dementia
G31.1 Senile degeneration of brain, not elsewhere classified
G31.2 Degeneration of nervous system due to alcohol
G31.8 Other specified degenerative diseases of nervous system
G31.81 Alpers disease
G31.82 Leigh's disease
G31.83 Dementia with Lewy bodies
G31.84 Mild cognitive impairment, so stated
G31.85 Corticobasal degeneration
G31.89 Other specified degenerative diseases of nervous system
G31.9 Degenerative disease of nervous system, unspecified
G32 Other degenerative disorders of nervous system in diseases classified elsewhere
G32.0 Subacute combined degeneration of spinal cord in diseases classified elsewhere
G32.8 Other specified degenerative disorders of nervous system in diseases classified elsewhere
G32.81 Cerebellar ataxia in diseases classified elsewhere
G32.89 Other specified degenerative disorders of nervous system in diseases classified elsewhere

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