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Benign myoclonus of early infancy
C Pachatz1, L Fusco, F Vigevano
1Neurophysiology Unit, Bambino Gesù Children's Hospital Rome, Italy.
Insights
Benign myoclonus of early infancy is a common, non-epileptic event in healthy infants. Neurophysiological studies confirm it involves brief tonic limb contractions without EEG changes.
Area of Science:
- Pediatric Neurology
- Neurophysiology
Background:
- Benign myoclonus of early infancy is a paroxysmal motor phenomenon occurring in healthy infants.
- It is typically triggered by excitement or frustration and observed during wakefulness.
Purpose of the Study:
- To investigate the neurophysiological characteristics of benign myoclonus of early infancy.
- To differentiate this benign condition from epileptic seizures in infants.
Main Methods:
- Video-electroencephalogram (EEG) monitoring was performed on five infants (7-11 months old).
- Polygraphic recordings were utilized in three of the five cases to assess motor activity.
- Clinical manifestations were correlated with neurophysiological data.
Main Results:
- The phenomenon presented as shudder-type motor events, primarily affecting the trunk and head, with associated tonic limb contractions.
- No modifications were observed in the EEG during these events.
- Polygraphic recordings confirmed brief tonic limb contractions, supporting a non-epileptic origin.
Conclusions:
- Benign myoclonus of early infancy is characterized by specific polygraphic findings of brief tonic limb contractions.
- These findings aid in distinguishing benign myoclonus from infantile spasms and other early infancy seizures.
- The study contributes to understanding the pathophysiology of this benign paroxysmal manifestation.
Abstract:
Benign myoclonus of early infancy, first described by Fejerman and Lombroso, is a paroxysmal phenomenon of the first 2 years of life which occurs in neurologically healthy infants during wakefulness, and is usually triggered by excitement or frustration. We studied the neurophysiological features of the phenomenon in five children, aged 7 to 11 months, who were monitored by video-EEG recording, and by polygraphic recording in 3 of the 5 cases. The phenomenon is characterized by a shudder-type, paroxysmal motor manifestation involving mainly the trunk and sometimes the head, associated with tonic limb contractions of variable intensity, from hardly noticeable to more sustained. The EEG counterpart never showed modifications, the polygraphic study demonstrated a brief tonic limb contraction. The clinical manifestation should not be confused with the spasms of epileptic infantile spasms syndrome, or with tonic reflex seizures of early infancy. Although the phenomenon is already widely known, its polygraphic recording is rarely reported in literature. The polygraphic data of our patients contributes to the diagnosis and understanding of the pathophysiology of this paroxysmal manifestation.
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