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Early-onset spontaneously relieved spasms of infancy in sleep: Electroclinical characteristics and differential diagnoses

  • Qiao Hu
    Affiliations
    Department of Neurology, Children’s Hospital of Chongqing Medical University, China

    National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, China
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  • Yuanyuan Luo
    Affiliations
    Department of Neurology, Children’s Hospital of Chongqing Medical University, China

    National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, China
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  • Tingsong Li
    Affiliations
    Department of Rehabilitation, Children’s Hospital of Chongqing Medical University, China
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  • Jiannan Ma
    Affiliations
    Department of Neurology, Children’s Hospital of Chongqing Medical University, China

    National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, China
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  • Jin Chen
    Affiliations
    Department of Neurology, Children’s Hospital of Chongqing Medical University, China

    National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, China
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  • Siqi Hong
    Affiliations
    Department of Neurology, Children’s Hospital of Chongqing Medical University, China

    National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, China
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  • Shuang Liao
    Correspondence
    Corresponding author at: Department of Neurology, Building 6, No. 136, Zhongshan Er Road, Yuzhong District, Chongqing 400014, China.
    Affiliations
    Department of Neurology, Children’s Hospital of Chongqing Medical University, China

    National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, China
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  • Li Jiang
    Affiliations
    Department of Neurology, Children’s Hospital of Chongqing Medical University, China

    National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, China
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      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

        • Maydell B.V.
        • Berenson F.
        • Rothner A.D.
        • Wyllie E.
        • Kotagal P.
        Benign myoclonus of early infancy: an imitator of West's syndrome.
        J Child Neurol. 2001; 16: 109-112
        • Fernández-Alvarez E.
        Transient benign paroxysmal movement disorders in infancy.
        Eur J Paediatr Neurol. 2018; 22: 230-237
        • Pavone P.
        • Striano P.
        • Falsaperla R.
        • Pavone L.
        • Ruggieri M.
        Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013.
        Brain Dev. 2014; 36: 739-751
      1. Fejerman N. Myoclonies et épilepsies chez l'enfant [Myoclonus and epilepsies in children]. Rev Neurol (Paris). 1991;147:782-97. French.

        • Dravet C.
        • Giraud N.
        • Bureau M.
        • Roger J.
        • Gobbi G.
        • Dalla Bernardina B.
        Benign myoclonus of early infancy or benign non-epileptic infantile spasms.
        Neuropediatrics. 1986; 17: 33-38
        • Ghossein J.
        • Pohl D.
        Benign spasms of infancy: a mimicker of infantile epileptic disorders.
        Epileptic Disord. 2019; 21: 585-589
        • Álvarez N.Á.
        • de la Vega R.P.
        • Trueba M.A.G.
        • González L.S.
        • Acero A.G.
        • Blanco-Lago R.
        • et al.
        Benign spasms of infancy or benign myoclonus of early infancy: polygraph-EEG recordings.
        Epileptic Disord. 2021; 23: 801-802
        • Pavone P.
        • Polizzi A.
        • Marino S.D.
        • Corsello G.
        • Falsaperla R.
        • Marino S.
        • et al.
        West syndrome: a comprehensive review.
        Neurol Sci. 2020; 41: 3547-3562
        • Messer R.
        • Knupp K.G.
        Infantile Spasms: Opportunities to Improve Care.
        Semin Neurol. 2020; 40: 236-245
        • Fusco L.
        • Vigevano F.
        Ictal clinical electroencephalographic findings of spasms in West syndrome.
        Epilepsia. 1993; 34: 671-678
        • Caraballo R.H.
        • Capovilla G.
        • Vigevano F.
        • Beccaria F.
        • Specchio N.
        • Fejerman N.
        The spectrum of benign myoclonus of early infancy: Clinical and neurophysiologic features in 102 patients.
        Epilepsia. 2009; 50: 1176-1183
        • Pavone P.
        • Corsello G.
        • Ruggieri M.
        • Marino S.
        • Marino S.
        • Falsaperla R.
        Benign and severe early-life seizures: a round in the first year of life.
        Ital J Pediatr. 2018; 44: 54
        • Maurer V.O.
        • Rizzi M.
        • Bianchetti M.G.
        • Ramelli G.P.
        Benign neonatal sleep myoclonus: a review of the literature.
        Pediatrics. 2010; 125: e919-e924
        • Tibussek D.
        • Karenfort M.
        • Mayatepek E.
        • Assmann B.
        Clinical reasoning: shuddering attacks in infancy.
        Neurology. 2008; 70: e38-e41