Hereditary ataxia
Chapter 6:Diseases of the nervous system
ICD-10 G11 is a billable code used to indicate a diagnosis of hereditary ataxia.
Hereditary ataxia refers to a group of genetic disorders characterized by progressive loss of coordination and balance due to degeneration of the cerebellum and its connections. These conditions can be inherited in various patterns, including autosomal dominant, autosomal recessive, and X-linked inheritance. The clinical presentation often includes gait disturbances, dysarthria, and oculomotor dysfunction. Patients may also experience associated symptoms such as spasticity, sensory loss, and cognitive decline, depending on the specific type of ataxia. Common forms of hereditary ataxia include Friedreich's ataxia, spinocerebellar ataxias (SCAs), and ataxia-telangiectasia. Diagnosis typically involves a combination of clinical evaluation, family history assessment, genetic testing, and neuroimaging studies. Management focuses on symptomatic relief and supportive care, as there is currently no cure for these degenerative conditions. Understanding the genetic basis of hereditary ataxia is crucial for accurate diagnosis and potential future therapies.
Comprehensive neurological examination findings, family history, and results of genetic testing.
Patients presenting with progressive ataxia, gait disturbances, and associated neurological symptoms.
Ensure clarity in documenting the specific type of hereditary ataxia and any associated conditions.
Detailed family pedigree, genetic test results, and interpretation of findings.
Patients referred for genetic counseling due to family history of ataxia.
Accurate coding requires understanding of the genetic basis and inheritance patterns.
Used for assessing cognitive function in patients with hereditary ataxia.
Document the rationale for testing and results.
Neurologists should ensure comprehensive cognitive assessments are included.
Hereditary ataxia is caused by genetic mutations passed through families, while acquired ataxia results from external factors such as stroke, alcohol abuse, or vitamin deficiencies.
