Preview

Neurology, Neuropsychiatry, Psychosomatics

Advanced search

Nonepileptic myoclonus of infancy and early childhood (an observation of 33 patients)

https://doi.org/10.14412/2074-2711-2019-2-42-45

Full Text:

Abstract

Fejerman syndrome, a benign nonepileptic myoclonus of infancy (BNMI), is a rare type of paroxysmal events, which mimics epileptic spasms. It is difficult to determine the nature of myoclonus without video electroencephalography (VEEG) monitoring.

Objective: to present the clinical and electroencephalographic characteristics of new cases of benign nonepileptic myoclonus of infancy and early childhood.

Patients and methods. The data of 33 children (19 boys and 14 girls) aged 5 months to 3 years with BNMI, who had been followed in 2011 to 2017, were analyzed.

Results and discussion. The age at onset of paroxysms ranged from 4 to 24 months and that was 5–8 months in most cases. The most common movements were extensor muscle jerks (30.3%), head titubation with rotation (27.3%), axial spasms (27.3%), and nods (24.2%). The same child may have different types of paroxysms. Motor paroxysms were sporadic in all the patients and formed into clusters in 33.3% of cases. The frequency of clusters was up to 10 times daily. There were 2 to 50 paroxysms in the cluster. There was delayed psychomotor development in 5 of the 33 children and hyperexcitability was present in 10. VEEG monitoring indicated that the brain bioelectrical activity conformed to the age in 97% of cases; none of the children showed abnormal movements accompanied by EEG pathological activity. The duration of the disease was 2 to 19 months, averaging 7 months. In all cases paroxysms were stopped without using antiepileptic drugs.

Conclusion. It is extremely important to timely recognize BNMI, since anti-epileptic therapy can be unreasonably prescribed in cases of an erroneous diagnosis. VEEG monitoring plays a crucial role in diagnosing nonepileptic and epileptic myoclonus.

About the Authors

O. K. Volkova
City Children's Clinical Emergency Hospital; Sibneiromed City Neurology Center
Russian Federation
3, Krasnyi Prospect, Novosibirsk 630007, 37, Michurin St., Novosibirsk 630091 


A. V. Kalina

Russian Federation
52, Krasnyi Prospect, Novosibirsk 630091


G. S. Karpovich
Novosibirsk State Medical University, Ministry of Health of Russia
Russian Federation


I. V. Volkov
Sibneiromed City Neurology Center
Russian Federation

Iosif Vyacheslavovich Volkov

3, Krasnyi Prospect, Novosibirsk 630007, 37, Michurin St., Novosibirsk 630091 



References

1. Shibaki H, Hallet M. Electrophysiogical studies of myoclonus. Muscle Nerve. 2005 Feb;31(2):157-74.

2. Ermakov AYu. Non-epileptic and epileptic myoclonus. In: Belousova ED, Ermakov AYu, editors. Differentsial'nyi diagnoz epilepsii [Differential diagnosis of epilepsy. Moscow: Pul's; 2007. 262 p.

3. Fejerman N. Differential diagnoses of West syndrome. Rev Neurol. 2013 Sep 6;57 Suppl 1:S125-8.

4. Mironov MB, Nogovitsyn VYu, Abramov MO, et al. Fejerman syndrome (benign non-epileptic myoclonus of infancy). Epilepsiya i paroksizmal'nye sostoyaniya. 2013;5(2):42-6. (In Russ.).

5. Maydell BV, Berenson F, Rothner AD et al. Benign myoclonus of early infancy: an imitator of West's syndrome. J Child Neurol. 2001 Feb;16(2):109-12.

6. Caraballo RH, Capovilla G, Vigevano F, et al. The spectrum of benign myoclonus of early infancy: Clinical and neurophysiologic features in 102 patients. Epilepsia. 2009 May;50(5):1176-83. doi: 10.1111/j.15281167.2008.01994.x. Epub 2009 Jan 19.

7. Fejerman N. Mioclonias benignas de la infancia temprana. Comunicacion peliminar. Actas IV Jornadas Rioplatenses de Neurologia Infantil. Neuropediatria Latinoamericana Montevideo: Delta; 1976. P. 131-4.

8. Giraud N. Les spasmes infantiles benins non epileptiques. These pour le Doctorate n Medecine. Marseille; 1982.

9. Gobbi G, Dravet C, Bureau M, et al. Les spasms benins du nourrisson (syndrome de Lombroso et Fejerman). Boll. Lega Italiana contro L`Epilepssia. 1982;39/S:17.

10. Dravet C, Giraud N, Bureau M, et al. Benign myoclonus of early infancy or benign non epileptic infantile spasms. Neuropediatrics. 1986 Feb;17(1):33-8.

11. Pachatz C, Fusco L, Vigevano F. Benign myoclonus of early infancy. Epileptic Disord. 1999 Mar;1(1):57-61.

12. Fejerman N, Caraballo R. Appendix to «Shuddering and benign myoclonus of early infancy» (Pachatz C, Fusco L, Vigevano F). In: Guerrini R, Aicardi S, Andermann F, Hallet M, editors. Epilepsy and movement disorders. Cambridge: Cambridge University Press; 2002. P. 343-51.

13. Panayiotopoulos C. A Clinical Guide to Epileptic Syndromes and their Treatment. Springer; 2010. P. 112-3.

14. Dalla Bernardina B. Benign myoclonus of early infancy or Fejerman syndrome. Epilepsia. 2009 May;50(5):1290-2. doi: 10.1111/j.15281167.2009.02154.x.

15. Kanazawa O. Shaddering attacks report of four children. Pediatr Neurol. 2000 Nov;23(5): 421-4.

16. Mukhin KYu, Mironov MB. Epileptic spasms: Nosological characteristics and approaches to therapy. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2013;5(1S): 23-8. (In Russ.). doi: 10.14412/2074-2711-2013-2484

17. Canavese C, Canafoglia L, Costa C, et al. Paroxysmal non-epileptic motor events in childhood: a clinical and video-EEG-polymyographic study. Dev Med Child Neurol. 2012 Apr; 54(4):334-8. doi: 10.1111/j.1469-8749.2011.04217.x. Epub 2012 Jan 28.


For citation:


Volkova O.K., Kalina A.V., Karpovich G.S., Volkov I.V. Nonepileptic myoclonus of infancy and early childhood (an observation of 33 patients). Neurology, Neuropsychiatry, Psychosomatics. 2019;11(2):42-45. (In Russ.) https://doi.org/10.14412/2074-2711-2019-2-42-45

Views: 48


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 2074-2711 (Print)
ISSN 2310-1342 (Online)