ICD10 code of Holmes-Adie Syndrome and ICD9 code

Holmes-Adie Syndrome is currently classified under ICD-10 code H57.8 (other specified disorders of eye and adnexa) and historically under ICD-9 code 379.8. As a benign neurological condition characterized by a tonic pupil and diminished deep tendon reflexes, it does not have a unique, disease-specific diagnostic code in either system.

What are the primary clinical features of Holmes-Adie Syndrome?

The hallmark of Holmes-Adie Syndrome is a tonic, dilated pupil that reacts slowly to light but shows a stronger response to near-vision accommodation. Patients often experience blurred vision or light sensitivity. Additionally, Holmes-Adie Syndrome is clinically defined by the presence of diminished or absent deep tendon reflexes (areflexia or hyporeflexia), typically in the lower extremities, which can sometimes lead to an incorrect suspicion of peripheral neuropathy.

How is Holmes-Adie Syndrome diagnosed?

Diagnosis of Holmes-Adie Syndrome is primarily clinical, often involving a pharmacological test using diluted pilocarpine. In a healthy eye, this concentration has no effect, but in a patient with Holmes-Adie Syndrome, the pupil will constrict due to denervation supersensitivity. Key diagnostic steps include:

• Detailed ophthalmologic evaluation to confirm tonic pupil response.


• Neurological examination to assess deep tendon reflexes.


• Blood tests to rule out other causes of autonomic dysfunction, such as syphilis or diabetes.


• Imaging (MRI) if central nervous system involvement is suspected.

Is Holmes-Adie Syndrome a progressive condition?

Holmes-Adie Syndrome is generally considered a non-progressive, benign condition. While the pupil abnormalities and reflex loss persist, they do not typically lead to systemic organ failure or life-threatening complications. Within our community at DiseaseMaps.org, 53 people with Holmes-Adie Syndrome have shared their experiences, noting that while the diagnosis can be alarming, the long-term prognosis for daily functioning remains excellent for most individuals.

Next steps

• Consult a neuro-ophthalmologist to confirm the diagnosis and rule out underlying mimics.


• Request a baseline eye examination to manage light sensitivity or near-vision difficulties.


• Connect with the 53 members at DiseaseMaps.org to share coping strategies for living with Holmes-Adie Syndrome.

Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Holmes-Adie Syndrome.


• Orphanet: Tonic pupil - Adie syndrome (ORPHA:98808).


• OMIM (Online Mendelian Inheritance in Man): Adie Syndrome (#103100).


• American Academy of Ophthalmology: Tonic Pupil Overview.