Table 5.

Recommended Surveillance for Individuals with EPM1

System/ConcernEvaluationFrequency
Myoclonus Severity of myoclonus using UMRSAt least annually
Seizures Seizure type & frequency
Cerebellar involvement Clinical evalPer symptom progression
Dysarthria Need for alternative communication method or speech therapy
Dysphagia Assess aspiration risk & feeding methods
Weight / Nutritional status
  • Monitor BMI.
  • Consult a nutritionist
  • High-calorie supplementation
Activities of daily living Clinically to evaluate rehab planAt least annually
School performance Interview
Cognitive/Psychiatric Evaluate mood, signs of psychosis, & cognitive complaints to identify need for pharmacologic & psychotherapeutic interventions.Per symptom progression & development of psychiatric symptoms
Family support & resources InterviewAnnually

BMI = body mass index; UMRS = Unified Myoclonus Rating Scale

From: Progressive Myoclonic Epilepsy Type 1

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