Alexander disease
ICD-10 G31.86 is a billable code used to indicate a diagnosis of alexander disease.
Alexander disease is a rare, progressive neurological disorder characterized by the accumulation of abnormal protein (GFAP) in the brain, leading to the degeneration of white matter. It primarily affects infants and young children, although adult-onset forms exist. Symptoms may include developmental delays, seizures, cognitive decline, and motor dysfunction. The disease is classified as a leukodystrophy, which means it affects the myelin sheath that insulates nerve fibers. Diagnosis is typically made through clinical evaluation, MRI findings, and genetic testing for mutations in the GFAP gene. The condition is progressive, and management focuses on symptomatic relief and supportive care, as there is currently no cure. Cognitive decline in patients with Alexander disease can resemble that seen in other degenerative diseases, such as Alzheimer's disease, making differential diagnosis crucial. Understanding the nuances of Alexander disease is essential for accurate coding and appropriate patient management.
Comprehensive neurological assessments, MRI results, and genetic testing documentation.
Diagnosis of Alexander disease in pediatric patients presenting with developmental delays and seizures.
Ensure all neurological evaluations are documented to support the diagnosis.
Genetic testing results and family history documentation.
Counseling families with a history of Alexander disease and discussing genetic testing options.
Document the rationale for genetic testing and results to support coding.
Used to assess cognitive function in patients with suspected Alexander disease.
Detailed report of cognitive assessments and findings.
Neurologists should ensure comprehensive documentation of cognitive decline.
Common symptoms include developmental delays, seizures, cognitive decline, and motor dysfunction, which can vary based on the age of onset.
