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[Pedaling automatism in atypical West syndrome: a case report]
M Fukumizu1, T Kurokawa, H Yoshikawa
1Division of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Discorders, Tokyo.
Insights
A rare case of infantile spasms with pedaling automatism in a seven-month-old girl is presented. This finding offers new insights into early infantile epileptic encephalopathy and its varied clinical presentations.
Area of Science:
- Neurology
- Pediatrics
- Epileptology
Background:
- West syndrome, a severe form of epilepsy, typically presents in infancy.
- Early infantile epileptic encephalopathy with suppression-burst (EIEE) is a severe neonatal epilepsy syndrome.
Observation:
- A seven-month-old girl exhibited atypical West syndrome with pedaling automatism.
- Spasms included pedaling movements, upper extremity tonic spasms, and trunk writhing.
- EEG revealed suppression-burst patterns and ictal abnormalities.
Findings:
- Brain imaging showed cerebral atrophy, microcephaly, immature myelination, and reduced right-sided blood flow.
- Pedaling automatism is an uncommon infantile spasm presentation.
- The case highlights the diverse clinical manifestations of EIEE and West syndrome.
Implications:
- This case expands the understanding of infantile spasms and their potential presentations.
- It underscores the importance of recognizing rare automatisms in diagnosing and managing epilepsy syndromes.
- Further research into the neurobiological underpinnings of such movements is warranted.
Abstract:
A seven-month-old girl with atypical West syndrome with pedaling automatism was reported. She started to have early infantile epileptic encephalopathy with suppression-burst (EIEE) at age 14 days followed by infantile spasms at 3 months of age. She began to have spasms with automatism at 4 month, mainly consisting of pedaling movements of both feet associated with tonic spasms of upper extremities and writhing of trunk. Ictal EEG showed irregular slow waves with occasional spikes predominantly over the right hemisphere mixed with artifacts. Interictal EEG during sleep showed suppression-burst patterns. Cerebral atrophy and microcephaly were revealed on brain CT, immature myelination on MRI and decreased blood flow in the frontal, temporal, and parietal lobes on the right on PET scan. Pedaling automatism is rare in infancy and its clinical significance was discussed in relation to West syndrome and complex partial seizures.