2026 ICD-10-CM Diagnosis Code G90.3Multi-system degeneration of the autonomic nervous system
ICD-10-CM Codes›G00–G99›G89-G99›G90
- Billable — Valid for Submission
- Chronic Condition
G90.3 is a billable ICD-10-CM diagnosis code for multi-system degeneration of the autonomic nervous system. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other nervous system disorders (often hereditary or degenerative).
Code Identity
Code Classification
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Amyotrophic lateral sclerosis plus syndrome
- Amyotrophic lateral sclerosis with multiple system atrophy
- Ataxia due to multiple system atrophy, cerebellar variant
- Chronic hypotension
- Chronic orthostatic hypotension
- Dementia due to multiple system atrophy
- Multiple system atrophy
- Multiple system atrophy, cerebellar variant
- Multiple system atrophy, Parkinson's variant
- Multisystem degeneration of autonomic nervous system
- Orthostatic hypotension
- Orthostatic hypotension co-occurrent and due to Parkinson's disease
- Parkinsonian syndrome with idiopathic orthostatic hypotension
- Parkinson's disease
- Pure autonomic failure
- Sympathotonic orthostatic hypotension
Tabular List NotesGuidance
Coding notes and annotation back-references applicable to this code.
Inclusion Terms
- Neurogenic orthostatic hypotension Shy-Drager
Type 1 Excludes
- orthostatic hypotension NOS I95.1
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
- multi-system - G90.3
- Hypotension (arterial) (constitutional) - I95.9
- neurogenic, orthostatic - G90.3
- orthostatic (chronic) - I95.1
- neurogenic - G90.3
- Shy-Drager syndrome - G90.3
External Cause of Injuries IndexGuidance
References for this code in the External Cause of Injuries Index.
- Degeneration, degenerative
- multi-system
- Hypotension(arterial) (constitutional)
- neurogenic, orthostatic
- Hypotension(arterial) (constitutional)
- orthostatic (chronic)
- neurogenic
- Parkinsonism(idiopathic) (primary)
- with neurogenic orthostatic hypotension (symptomatic)
- Shy-Drager syndrome
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Multiple System Atrophy
a syndrome complex composed of three conditions which represent clinical variants of the same disease process: striatonigral degeneration; shy-drager syndrome; and the sporadic form of olivopontocerebellar atrophies. clinical features include autonomic, cerebellar, and basal ganglia dysfunction. pathologic examination reveals atrophy of the basal ganglia, cerebellum, pons, and medulla, with prominent loss of autonomic neurons in the brain stem and spinal cord. (from adams et al., principles of neurology, 6th ed, p1076; baillieres clin neurol 1997 apr;6(1):187-204; med clin north am 1999 mar;83(2):381-92)Pure Autonomic Failure
a degenerative disease of the autonomic nervous system that is characterized by idiopathic orthostatic hypotension and a greatly reduced level of catecholamines. no other neurological deficits are present.
Patient EducationClinical
Autonomic Nervous System Disorders
Your autonomic nervous system is the part of your nervous system that controls involuntary actions, such as the beating of your heart and the widening or narrowing of your blood vessels. When something goes wrong in this system, it can cause serious problems, including:
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert G90.3 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About G90.3Overview
Is G90.3 (Disorders of autonomic nervous system) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report multi-system degeneration of the autonomic nervous system on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
What is the ICD-9 equivalent of G90.3?
Under the General Equivalence Mappings, multi-system degeneration of the autonomic nervous system converts to ICD-9-CM 333.0 (degen basal ganglia NEC). The mapping is approximate, so confirm the match fits the documentation.
Footnotes
[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:
- The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
- The condition places limitations on self-care, independent living, and social interactions.
