Table 5.

Recommended Surveillance for Individuals with Spinocerebellar Ataxia Type 3

System/ConcernEvaluationFrequency
Neurologic
  • Neurologic assessment for progression of ataxia; UMN or LMN signs; dystonia & parkinsonism; autonomic dysfunction
  • Monitor ataxia progression w/standardized scale (SARA, ICARS, or BARS). 1
Annually; more often for an acute exacerbation
Physiatry, OT/PT assessment of mobility, self-help skills as they relate to ataxia, spasticity, weaknessAnnually; more often for an acute exacerbation
Dysarthria Need for alternative communication method or speech therapyPer symptom progression
Dysphagia Assess aspiration risk & feeding methods.Per symptom progression
Weight / Nutritional
status
  • Monitor BMI.
  • Consult a nutritionist.
  • High-calorie supplementation
Annually
Respiratory If symptoms, pulmonary function testsPer symptom progression
Bladder dysfunction Flow studies & eval by urologistPer symptom progression
Neuropathic pain Evaluate need for pharmacologic treatment.Per symptom progression
Cognitive/
Psychiatric
Evaluate mood, signs of psychosis, cognitive complaints to identify need for pharmacologic & psychotherapeutic interventions.Per symptom progression & development of psychiatric symptoms
Social support Assess needs of affected person & caregiver.Annually

BARS = Brief Ataxia Rating Scale; ICARS = International Co-operative Ataxia Rating Scale; LMN = lower motor neuron; OT = occupational therapy; PT = physical therapy; SARA = Scale for the Assessment and Rating of Ataxia; UMN = upper motor neuron

1.

From: Spinocerebellar Ataxia Type 3

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