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Ictal pattern of EEG and muscular activation in symptomatic infantile spasms: a videopolygraphic and computer
Francesca Bisulli1, Lilia Volpi, Stefano Meletti
1Department of Neurological Sciences, University of Bologna, Bellaria Hospital, Bologna, Italy. francesca.bisulli@neuro.unibo.it
Insights
This study reveals that infantile spasms (ISs) display complex and varied muscular activation patterns, not explained by simple cortical or reticular generators, highlighting their heterogeneity.
Area of Science:
- Neurology
- Pediatric Epilepsy
- Clinical Neurophysiology
Background:
- Symptomatic infantile spasms (ISs) are a severe epilepsy syndrome in infants.
- Understanding the ictal muscular phenomena and their electroencephalogram (EEG) correlation is crucial for diagnosis and treatment.
Purpose of the Study:
- To investigate the ictal muscular phenomena of symptomatic infantile spasms (ISs).
- To explore the relationship between these muscular events and ictal EEG patterns.
Main Methods:
- Videopolygraphic recordings with surface electromyogram (EMG) from cranial and limb muscles were used.
- Four children with severe encephalopathy and refractory ISs underwent detailed off-line analysis.
- Evaluation focused on muscular recruitment, duration, and asymmetry of spasms.
Main Results:
- Infantile spasms (ISs) exhibited complex, heterogeneous muscular activation patterns without consistent rostrocaudal propagation.
- Long intervals (100-200 ms) were observed between EMG onset in different muscles.
- Asymmetric spasms in two patients showed contralateral muscle activity preceding ipsilateral, linked to EEG findings on the dysplastic side.
Conclusions:
- Motor phenomena in infantile spasms (ISs) are complex and heterogeneous, despite clinical similarity.
- The findings challenge simple explanations involving cortical or reticular generators.
- Further research is needed to elucidate the underlying mechanisms of these spasms.
Purpose:
To investigate ictal muscular phenomena characterizing symptomatic infantile spasms (ISs) and their relation to ictal EEG.
Methods:
Four children with severe encephalopathy, neurologic impairment, and refractory ISs related to different dysplastic lesions, underwent videopolygraphic recordings collecting surface electromyogram (EMG) activity from several cranial and limb muscles to evaluate the pattern of muscular recruitment, duration, and side-to-side asymmetry of ISs. Acquired data were stored for off-line analysis by a computerized polygraphic system.
Results:
Spasms were characterized by a complex pattern of muscular activation. A constant or rostrocaudal propagation pattern was lacking in all patients. Intervals between the onset of EMG activity in different muscles in each spasm were very long: =100-200 ms. Two patients, with hemispheric cortical dysplasia and agenesis of the corpus callosum, had asymmetric and asynchronous spasms in which the EMG onset of the muscles contralateral to the affected hemisphere constantly anticipated that of the ipsilateral ones. Backaveraging of EEG activity disclosed a high-amplitude EEG complex on the same side as the dysplastic lesion, preceding or succeeding the contralateral muscle activity onset. In the other two patients with diffuse cortical dysplasia, no EEG transient was related to EMG activity onset.
Conclusions:
Despite clinical similarity between spasms in the same patient, our data demonstrate the complexity and heterogeneity of these motor phenomena. Our findings cannot be explained simply in terms of cortical or reticular generators like other motor phenomena such as cortical myoclonus or startle reflex.