2019 ICD-10-CM Diagnosis Code G90.1

Familial dysautonomia [Riley-Day]

    2016 2017 2018 2019 Billable/Specific Code
  • G90.1 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 G90.1 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of G90.1 - other international versions of ICD-10 G90.1 may differ.
The following code(s) above G90.1 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 G90.1:
  • 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
  • G90
    ICD-10-CM Diagnosis Code G90

    Disorders of autonomic nervous system

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • dysfunction of the autonomic nervous system due to alcohol (G31.2)
    Disorders of autonomic nervous system
Approximate Synonyms
  • Disorder of autonomic nervous system
  • Dysautonomia
  • Familial dysautonomia
  • Riley day syndrome
Clinical Information
  • A congenital disorder caused by mutations in the ikbkap gene. It is characterized by damage of the sympathetic and parasympathetic and sensory nervous system.
  • An autosomal disorder of the peripheral and autonomic nervous systems limited to individuals of ashkenazic jewish descent. Clinical manifestations are present at birth and include diminished lacrimation, defective thermoregulation, orthostatic hypotension (hypotension, orthostatic), fixed pupils, excessive sweating, loss of pain and temperature sensation, and absent reflexes. Pathologic features include reduced numbers of small diameter peripheral nerve fibers and autonomic ganglion neurons. (from Adams et al., Principles of Neurology, 6th ed, p1348; nat genet 1993;4(2):160-4)
  • Autosomal disorder of the peripheral and autonomic nervous systems limited to individuals of ashkenazic jewish descent; clinical manifestations are present at birth and include diminished lacrimation, defective thermoregulation, orthostatic hypotension, fixed pupils, excessive sweating, loss of pain and temperature sensation, and absent reflexes; pathologic features include reduced numbers of small diameter peripheral nerve fibers and autonomic ganglion neurons.
ICD-10-CM G90.1 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 091 Other disorders of nervous system with mcc
  • 092 Other disorders of nervous system with cc
  • 093 Other disorders of nervous system without cc/mcc
  • 793 Full term neonate with major problems

Convert G90.1 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 G90.1:
  • Type 1 Excludes: Q85
    ICD-10-CM Diagnosis Code Q85

    Phakomatoses, not elsewhere classified

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • ataxia telangiectasia [Louis-Bar] (G11.3)
    • familial dysautonomia [Riley-Day] (G90.1)
  • Type 2 Excludes: Q07
    ICD-10-CM Diagnosis Code Q07

    Other congenital malformations of nervous system

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 2 Excludes
    • congenital central alveolar hypoventilation syndrome (G47.35)
    • familial dysautonomia [Riley-Day] (G90.1)
    • neurofibromatosis (nonmalignant) (Q85.0-)

Diagnosis Index entries containing back-references to G90.1:
  • Dysautonomia G90.1 (familial)
  • Riley-Day syndrome G90.1

ICD-10-CM Codes Adjacent To G90.1
G89.21 Chronic pain due to trauma
G89.22 Chronic post-thoracotomy pain
G89.28 Other chronic postprocedural pain
G89.29 Other chronic pain
G89.3 Neoplasm related pain (acute) (chronic)
G89.4 Chronic pain syndrome
G90 Disorders of autonomic nervous system
G90.0 Idiopathic peripheral autonomic neuropathy
G90.01 Carotid sinus syncope
G90.09 Other idiopathic peripheral autonomic neuropathy
G90.1 Familial dysautonomia [Riley-Day]
G90.2 Horner's syndrome
G90.3 Multi-system degeneration of the autonomic nervous system
G90.4 Autonomic dysreflexia
G90.5 Complex regional pain syndrome I (CRPS I)
G90.50 Complex regional pain syndrome I, unspecified
G90.51 Complex regional pain syndrome I of upper limb
G90.511 Complex regional pain syndrome I of right upper limb
G90.512 Complex regional pain syndrome I of left upper limb
G90.513 …… bilateral
G90.519 Complex regional pain syndrome I of unspecified upper limb

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