Table 2.

SCARB2-Related Action Myoclonus – Renal Failure Syndrome: Frequency of Select Neurologic Features

FeatureFrequencyComment
Nearly allCommonInfrequent
Progressive action & reflex myoclonus●
Ataxia●Usually late in disease course
Tremor●Action & posture induced, irregular amplitude (cortical tremor)
Myoclonus at rest●
Dysarthria●Usually late in disease course
Severe epileptic photosensitivity●
Generalized tonic-clonic seizures●
Dysphagia●Not discussed in most reports, may be late finding
Cognitive decline●
Sensorineural hearing loss●

From: SCARB2-Related Action Myoclonus – Renal Failure Syndrome

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