Clinical Guidance

The clinical findings of spinocerebellar ataxia type 10 (SCA10) are relatively homogeneous. Ataxia causes progressive disability, and seizures may become life threatening if status epilepticus emerges. Reported age of onset ranges from 12 to 83 years [, , , , ]. To date, more than 300 individuals have been identified with SCA10. The following description of the phenotypic features associated with this condition is based on these reports. Ataxia. The central feature of the phenotype is slowly progressive cerebellar ataxia that usually starts as poor balance and unsteady gait. The gait ataxia gradually worsens, leading to an increasing number of falls and necessitating use of a cane, walker, and eventually wheelchair.

Diagnostic Criteria

Suggestive Findings

Spinocerebellar ataxia type 10 (SCA10) should be suspected in individuals with the following findings:

• Slowly progressive cerebellar ataxia starting as poor balance and unsteady gait

• Scanning dysarthria, dysphagia, and upper-limb ataxia following the gait ataxia

• Family history consistent with autosomal dominant inheritance and Native American or East Asian ancestry

Management Guidelines

Evaluations Following Initial Diagnosis To establish the extent of disease and needs in an individual diagnosed with spinocerebellar ataxia type 10 (SCA10), the evaluations summarized (if not performed as part of the evaluation that led to the diagnosis) are recommended. Table 3. Recommended Evaluations Following Initial Diagnosis in Individuals with SCA10

Surveillance Guidelines

Table 5.

Recommended Surveillance for Individuals with SCA10

System/Concern | Evaluation | Frequency

Neurologic

| Clinical evaluation w/neurologist | Every 4-6 mos to identify early signs of potential complications to adjust anticonvulsant treatments

Video esophagrams can identify consistency of food least likely to trigger aspiration. | As needed in individuals w/dysphagia