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ICD-10-CM Codes
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G00-G99
Diseases of the nervous system
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G89-G99
Other disorders of the nervous system
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G90-
Disorders of autonomic nervous system
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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:
- 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:
- 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
G89.3 Neoplasm related pain (acute) (chronic)
G89.4 Chronic pain syndrome
G90 Disorders of autonomic nervous system
G90.0 Idiopathic peripheral autonomic neuropathy
G90.09 Other idiopathic peripheral autonomic neuropathy
G90.1
Familial dysautonomia [Riley-Day]
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
G90.519 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.
