Table 7.

Recommended Surveillance for Individuals with a Hereditary Ataxia

System/ConcernEvaluationFrequency
Neurologic
  • Neurologic assessment for progression of ataxia, UMN or LMN signs, & history of falls
  • Monitor ataxia progression w/standardized scale (SARA). 1
  • Physiatry, OT/PT assessment of mobility, & self-help skills as they relate to ataxia, spasticity, & weakness
Annually; more often for an acute exacerbation
Dysarthria Need for alternative communication method or speech therapyPer symptom progression
Dysphagia Assess aspiration risk & feeding methods.
Cognitive/
Psychiatric 		Evaluate mood, signs of psychosis, & cognitive complaints to identify need for pharmacologic & psychotherapeutic interventions.Per symptom progression & development of psychiatric symptoms
Family/
Community 		Assess family need for social work support (e.g., palliative/respite care, home nursing, other local resources), care coordination, or follow-up genetic counseling if new questions arise (e.g., family planning).At each visit

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: Hereditary Ataxia Overview

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