Hallervorden-Spatz disease
ICD-10 G23.0 is a billable code used to indicate a diagnosis of hallervorden-spatz disease.
Hallervorden-Spatz disease, now more commonly referred to as neurodegeneration with brain iron accumulation (NBIA), is a rare genetic disorder characterized by the accumulation of iron in the brain, leading to progressive neurological decline. Clinically, it presents with a combination of movement disorders, including dystonia, parkinsonism, and spasticity. Patients often exhibit symptoms similar to those of Parkinson's disease, such as bradykinesia, rigidity, and tremors. The onset typically occurs in childhood or early adulthood, and as the disease progresses, cognitive decline and behavioral changes may also be observed. The pathophysiology involves mutations in genes responsible for iron metabolism, particularly the PANK2 gene. Diagnosis is primarily clinical, supported by neuroimaging findings that reveal characteristic basal ganglia changes. Management focuses on symptomatic treatment, including dopaminergic medications, which may provide some relief from parkinsonian symptoms, although they do not halt disease progression. Multidisciplinary care is essential for addressing the complex needs of affected individuals.
Comprehensive neurological examination findings, including detailed descriptions of movement disorders and cognitive assessments.
Patients presenting with unexplained movement disorders, particularly in younger populations.
Documentation should clearly differentiate between Hallervorden-Spatz disease and other extrapyramidal disorders.
Results of genetic testing and family history of movement disorders.
Patients with a family history of neurodegenerative diseases presenting with movement disorders.
Genetic counseling may be necessary for affected families, and documentation should reflect this.
Used for follow-up visits in patients with Hallervorden-Spatz disease for symptom management.
Detailed history of symptoms, neurological examination findings, and treatment response.
Neurologists should ensure comprehensive documentation to support the complexity of care.
Primary symptoms include dystonia, parkinsonism, cognitive decline, and behavioral changes, often presenting in childhood or early adulthood.
