Objective: We explored the utility of noninvasive electrophysiological tremor studies in patients referred  for evaluation by pediatric movement specialists  following their clinical assessment . The studies were analyzed for confirming, clarifying or amending diagnoses and whether there were resultant  changes in outcome and management.

Background: Tremor studies have been reported frequently in adults. Little literature  on the evaluation of tremor in childhood utilizing electrophysiology exists. Tremor is one of the most common movement disorders in childhood accounting for 20% of pediatric movement disorder presentations. Descriptions in pediatric literature regarding tremor have been limited to clinical aspects with major emphasis on essential tremor. Recently the utility of electrophysiology in several large adult  series has been published. Clinical evaluation alone may not differentiate various tremor subtypes.  For example, a myoclonic component may not be readily detected clinically and a functional tremor may be difficult to confirm in children even when evaluated appropriately.

Method: Patients ages 3-18 years, 10male, 8 female ,with tremor symptoms or syndromes referred to the Human Motor Control Section clinic of the NINDS, NIH participated in non-invasive tremor studies. Muscle activity was captured with surface EMG and the tremor movement with accelerometers.Simultaneous recordings of EEG and EMG were performed to ascertain cortical events from the EEG preceding a  muscle burst. Presence of cortical myoclonus   could identify cortical tremor. In suspected functional movement disorder a Bereitschaftspotential could be valuable.

Results: Al patients   completed outpatient studies without discomfort or adverse event. In 6 patients the clinical diagnosis was confirmed: essential tremor, myoclonic tremor,  and functional tremor.  For 7 patients  the diagnosis was clarified:juvenile Parkinson’s disease,essential tremor (in a 3 year old),  myoclonus dystonia, dystonic tremor, enhanced physiological tremor. In 5 patients the diagnoses were amended :neuropathic tremor, myoclonus dystonia( final diagnosis KCNN2 dystonia) , dystonia, unspecified action tremor.

Conclusion: This series demonstrates how neurophysiology performed in young patients presenting with tremor complements clinical evaluation in many patients extending or modifying the diagnosis and influencing therapy and outcome.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/application-of-neurophysiology-in-the-clinical-evaluation-of-pediatric-and-adolescent-patients-with-tremor/