Myoclonus generators in sialidosis

Myoclonus generators in sialidosis

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Objective: Sialidosis ds durga hand soap is an inborn error of metabolism.There is evidence that the myoclonic movements observed in this disorder have a cortical origin, but this mechanism does not fully explain the bilaterally synchronous myoclonus activity frequently observed in many patients.We present evidence of a subcortical basis for synchronous myoclonic phenomena.

Methods: Electromyographic investigations were undertaken in two molecularly and biochemically confirmed patients with sialidosis type-1.Results: The EMG recordings showed clear episodes click here of bilaterally synchronous myoclonic activity in contralateral homologous muscles.We also observed a high muscular-muscular coherence with near-zero time-lag between these muscles.

Conclusion: The absence of coherence phase lag between the right-and-left homologous muscles during synchronous events indicates that a unilateral cortical source cannot fully explain the myoclonic activity.There must exist a subcortical mechanism for bilateral synchronization accounting for this phenomenon.Significance: Understanding this mechanism may illuminate cortical-subcortical relationships in myoclonus.