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Segmental myoclonus in a child with spinal cord tumour
F Renault1, R Flores-Guevara, A M D'Allest
1Laboratoire de Neurophysiologie Clinique de l'Enfant, Hôpital Armand-Trousseau, Paris, France.
Insights
Segmental spinal myoclonus (SSM) in a child
Area of Science:
- Pediatric Neurology
- Spinal Cord Disorders
- Clinical Electrophysiology
Background:
- Segmental spinal myoclonus (SSM) is a rare neurological condition characterized by involuntary muscle jerks.
- Pediatric cases of SSM are infrequently documented, particularly those associated with spinal cord pathologies.
- Understanding the developmental trajectory of SSM is crucial for managing associated complications.
Observation:
- A case study of an infant presenting with lower limb SSM, later diagnosed with a T8-T12 spinal cord tumor.
- Clinical observation revealed that early-onset SSM did not impede walking development but led to orthopedic issues.
- Longitudinal electromyographic (EMG) recordings tracked changes in SSM characteristics over several years.
Findings:
- Electromyographic analysis demonstrated the topographical spread of SSM and its evolution towards rhythmicity and variable muscle activation patterns.
- The study documented changes in SSM frequency and muscle activation sequences between 23 months and 7 years 10 months of age.
- These electrophysiological findings provide insights into the dynamic nature of spinal cord generator activity in pediatric SSM.
Implications:
- The findings suggest a link between spinal cord tumors and the development of pediatric segmental spinal myoclonus.
- This case highlights the importance of early diagnosis and monitoring of SSM in children to prevent orthopedic complications.
- The study contributes to understanding the pathophysiology of SSM, referencing neonatal motor automatisms and spinal generators.
Abstract:
Segmental spinal myoclonus (SSM) involving a child's lower limb revealed the presence of a spinal cord tumour from T8 to T12. The clinical and electrophysiological features of SSM-during the child's development were studied. The presence of this disorder at two months of age did not disturb the development of walking, but did lead to orthopaedic complications. Four electromyographic recordings performed between 23 months and 7 years 10 months of age revealed the topographical extension of the SSM, its passage to rhythmicity, variable frequency and change in the sequence of activation of the muscles. These electroclinical features enable discussion of the pathophysiology of SSM by reference to neonatal motor automatisms and spinal generators.