Abstract
Purpose
Infantile spasm-like paroxysms are often difficult to classify as epileptic or non-epileptic.
We aimed to study spontaneously relieved (non-epileptic) spasms of infancy in sleep.
Elucidation of the electroclinical characteristics and differential diagnoses of such
spasms could facilitate accurate diagnosis in the future.
Methods
We retrospectively analyzed the clinical data, video-EEG recordings, and other laboratory
test results of patients with spontaneously relieved spasms of infancy during sleep.
All the enrolled patients were followed up for at least 5 months.
Results
Seven infants were included in this study. The median age at spasm onset was 0.5 months
(range: 0.1–2 months). The episodes were characterized by clusters of non-epileptic
spasms of the head, trunk, or extremities lasting approximately 0.5–2 s, and were
validated by ictal electromyography (EMG)/video EEG. Episodes occurred several times
daily in clusters, particularly during sleep; two patients also experienced episodes
while awake. Additionally, non-epileptic jerks were recorded in 3 patients. All non-epileptic
spasms were completely resolved 2 weeks to 3 months after onset. Moreover, neuropsychomotor
development in all patients was normal at the last follow-up (5 to 12 months).
Conclusions
Spontaneously relieved spasms of infancy in sleep is a self-limiting movement disorder
characterized by onset between 0.1 and 2 months of age, and by clusters of spasms
occurring in sleep. Correct differential diagnosis relies on familiar clinical and
electrophysiological features.
Keywords
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References
- Benign myoclonus of early infancy: an imitator of West's syndrome.J Child Neurol. 2001; 16: 109-112
- Transient benign paroxysmal movement disorders in infancy.Eur J Paediatr Neurol. 2018; 22: 230-237
- Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013.Brain Dev. 2014; 36: 739-751
Fejerman N. Myoclonies et épilepsies chez l'enfant [Myoclonus and epilepsies in children]. Rev Neurol (Paris). 1991;147:782-97. French.
- Benign myoclonus of early infancy or benign non-epileptic infantile spasms.Neuropediatrics. 1986; 17: 33-38
- Benign spasms of infancy: a mimicker of infantile epileptic disorders.Epileptic Disord. 2019; 21: 585-589
- Benign spasms of infancy or benign myoclonus of early infancy: polygraph-EEG recordings.Epileptic Disord. 2021; 23: 801-802
- West syndrome: a comprehensive review.Neurol Sci. 2020; 41: 3547-3562
- Infantile Spasms: Opportunities to Improve Care.Semin Neurol. 2020; 40: 236-245
- Ictal clinical electroencephalographic findings of spasms in West syndrome.Epilepsia. 1993; 34: 671-678
- The spectrum of benign myoclonus of early infancy: Clinical and neurophysiologic features in 102 patients.Epilepsia. 2009; 50: 1176-1183
- Benign and severe early-life seizures: a round in the first year of life.Ital J Pediatr. 2018; 44: 54
- Benign neonatal sleep myoclonus: a review of the literature.Pediatrics. 2010; 125: e919-e924
- Clinical reasoning: shuddering attacks in infancy.Neurology. 2008; 70: e38-e41
Article info
Publication history
Published online: June 16, 2022
Accepted:
June 3,
2022
Received in revised form:
May 12,
2022
Received:
December 9,
2021
Identification
Copyright
© 2022 The Japanese Society of Child Neurology Published by Elsevier B.V. All rights reserved.
