G90.1

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.

Details

• G90.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM G90.1 became effective on October 1, 2022.

• This is the American ICD-10-CM version of G90.1 – other international versions of ICD-10 G90.1 may differ.