ICD-10-CM Code G90.1

Familial dysautonomia [Riley-Day]

Version 2021 Billable Code

Valid for Submission

G90.1 is a billable code used to specify a medical diagnosis of familial dysautonomia [riley-day]. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code G90.1 might also be used to specify conditions or terms like familial dysautonomia or hereditary dysautonomia with motor neuropathy.

Short Description:Familial dysautonomia [Riley-Day]
Long Description:Familial dysautonomia [Riley-Day]

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code G90.1 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Familial dysautonomia
  • Hereditary dysautonomia with motor neuropathy

Clinical Information

  • DYSAUTONOMIA FAMILIAL-. 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;42:160 4
  • HEREDITARY SENSORY AND AUTONOMIC NEUROPATHIES-. a group of inherited disorders characterized by degeneration of dorsal root and autonomic ganglion cells and clinically by loss of sensation and autonomic dysfunction. there are five subtypes. type i features autosomal dominant inheritance and distal sensory involvement. type ii is characterized by autosomal inheritance and distal and proximal sensory loss. type iii is dysautonomia familial. type iv features insensitivity to pain heat intolerance and mental deficiency. type v is characterized by a selective loss of pain with intact light touch and vibratory sensation. from joynt clinical neurology 1995 ch51 pp142 4

Convert G90.1 to ICD-9

  • 742.8 - Nervous system anom NEC (Approximate Flag)

Code Classification

  • Diseases of the nervous system (G00–G99)
    • Other disorders of the nervous system (G89-G99)
      • Disorders of autonomic nervous system (G90)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients

Autonomic Nervous System Disorders

Also called: Dysautonomia

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

  • Blood pressure problems
  • Heart problems
  • Trouble with breathing and swallowing
  • Erectile dysfunction in men

Autonomic nervous system disorders can occur alone or as the result of another disease, such as Parkinson's disease, alcoholism and diabetes. Problems can affect either part of the system, as in complex regional pain syndromes, or all of the system. Some types are temporary, but many worsen over time. When they affect your breathing or heart function, these disorders can be life-threatening.

Some autonomic nervous system disorders get better when an underlying disease is treated. Often, however, there is no cure. In that case, the goal of treatment is to improve symptoms.

NIH: National Institute of Neurological Disorders and Stroke

  • Autonomic neuropathy (Medical Encyclopedia)
  • Multiple system atrophy (Medical Encyclopedia)

[Learn More]

Familial dysautonomia Familial dysautonomia is a genetic disorder that affects the development and survival of certain nerve cells. The disorder disturbs cells in the autonomic nervous system, which controls involuntary actions such as digestion, breathing, production of tears, and the regulation of blood pressure and body temperature. It also affects the sensory nervous system, which controls activities related to the senses, such as taste and the perception of pain, heat, and cold. Familial dysautonomia is also called hereditary sensory and autonomic neuropathy, type III.Problems related to this disorder first appear during infancy. Early signs and symptoms include poor muscle tone (hypotonia), feeding difficulties, poor growth, lack of tears, frequent lung infections, and difficulty maintaining body temperature. Older infants and young children with familial dysautonomia may hold their breath for prolonged periods of time, which may cause a bluish appearance of the skin or lips (cyanosis) or fainting. This breath-holding behavior usually stops by age 6. Developmental milestones, such as walking and speech, are usually delayed, although some affected individuals show no signs of developmental delay.Additional signs and symptoms in school-age children include bed wetting, episodes of vomiting, reduced sensitivity to temperature changes and pain, poor balance, abnormal curvature of the spine (scoliosis), poor bone quality and increased risk of bone fractures, and kidney and heart problems. Affected individuals also have poor regulation of blood pressure. They may experience a sharp drop in blood pressure upon standing (orthostatic hypotension), which can cause dizziness, blurred vision, or fainting. They can also have episodes of high blood pressure when nervous or excited, or during vomiting incidents. About one-third of children with familial dysautonomia have learning disabilities, such as a short attention span, that require special education classes. By adulthood, affected individuals often have increasing difficulties with balance and walking unaided. Other problems that may appear in adolescence or early adulthood include lung damage due to repeated infections, impaired kidney function, and worsening vision due to the shrinking size (atrophy) of optic nerves, which carry information from the eyes to the brain.
[Learn More]