Benign myoclonic epilepsy in infancy (BMEI)

A longitudinal electroclinical study of 22 cases

Francesca Darra, Elena Fiorini, Leonardo Zoccante, Laura Mastella, Claudia Torniero, Samuele Cortese, Lisa Meneghello, Elena Fontana, Bernardo Dalla Bernardina

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

Purpose: Benign myoclonic epilepsy in infancy (BMEI) is a nosologically well-defined entity, characterized by myoclonic seizures (MS) in normal children younger than 3 years and by a good long term prognosis. In some cases the seizures are reflex. We studied 22 cases to better define the electroclinical semeiology and evolution of the disorder. Methods: Serial electroclinical and neuropsychological assessments, both during wakefulness and during sleep, were performed in 22 otherwise healthy children with spontaneous (17) or reflex (5) MS, recorded by video-EEG-polygraphy since clinical onset. Results: Seizure onset was between 3 months and 4 years 10 months (50% during first year, 86% before the third year); in reflex cases onset, was earlier than the 14th month. MS recurred during wakefulness and slow sleep in all cases and during REM sleep in reflex cases. MS and related EEG discharges were synchronous or asynchronous. Often ictal EEG discharges were limited to the rolandic and vertex regions (falsely focal paroxysms). Several seizures were subtle and could have escaped recognition. Unusually frequent sleep startles were recorded mostly in reflex cases. MS were well controlled by treatment. At follow-up, between ages 3 and 19 years, four patients had occasional seizures; two had cognitive impairment and three had learning difficulties. No other seizures or cognitive deficits were observed in reflex cases. Conclusions: Seizures associated with BMEI are rarely truly generalized and are often so subtle and related to falsely focal paroxysms that their frequency can be underestimated. The reflex form is a well-defined variant with an early onset, peculiar electroclinical features, and a good prognosis.

Original languageEnglish
Pages (from-to)31-35
Number of pages5
JournalEpilepsia
Volume47
Issue numberSUPPL. 5
DOIs
Publication statusPublished - Dec 2006

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Myoclonic Epilepsy
Longitudinal Studies
Seizures
Reflex
Electroencephalography
Sleep
Wakefulness
REM Sleep

Keywords

  • Benign epilepsy
  • Epilepsy in infancy
  • Myoclonic epilepsies
  • Reflex seizures
  • Startles

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Darra, F., Fiorini, E., Zoccante, L., Mastella, L., Torniero, C., Cortese, S., ... Bernardina, B. D. (2006). Benign myoclonic epilepsy in infancy (BMEI): A longitudinal electroclinical study of 22 cases. Epilepsia, 47(SUPPL. 5), 31-35. https://doi.org/10.1111/j.1528-1167.2006.00874.x

Benign myoclonic epilepsy in infancy (BMEI) : A longitudinal electroclinical study of 22 cases. / Darra, Francesca; Fiorini, Elena; Zoccante, Leonardo; Mastella, Laura; Torniero, Claudia; Cortese, Samuele; Meneghello, Lisa; Fontana, Elena; Bernardina, Bernardo Dalla.

In: Epilepsia, Vol. 47, No. SUPPL. 5, 12.2006, p. 31-35.

Research output: Contribution to journalArticle

Darra, F, Fiorini, E, Zoccante, L, Mastella, L, Torniero, C, Cortese, S, Meneghello, L, Fontana, E & Bernardina, BD 2006, 'Benign myoclonic epilepsy in infancy (BMEI): A longitudinal electroclinical study of 22 cases', Epilepsia, vol. 47, no. SUPPL. 5, pp. 31-35. https://doi.org/10.1111/j.1528-1167.2006.00874.x
Darra F, Fiorini E, Zoccante L, Mastella L, Torniero C, Cortese S et al. Benign myoclonic epilepsy in infancy (BMEI): A longitudinal electroclinical study of 22 cases. Epilepsia. 2006 Dec;47(SUPPL. 5):31-35. https://doi.org/10.1111/j.1528-1167.2006.00874.x
Darra, Francesca ; Fiorini, Elena ; Zoccante, Leonardo ; Mastella, Laura ; Torniero, Claudia ; Cortese, Samuele ; Meneghello, Lisa ; Fontana, Elena ; Bernardina, Bernardo Dalla. / Benign myoclonic epilepsy in infancy (BMEI) : A longitudinal electroclinical study of 22 cases. In: Epilepsia. 2006 ; Vol. 47, No. SUPPL. 5. pp. 31-35.
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abstract = "Purpose: Benign myoclonic epilepsy in infancy (BMEI) is a nosologically well-defined entity, characterized by myoclonic seizures (MS) in normal children younger than 3 years and by a good long term prognosis. In some cases the seizures are reflex. We studied 22 cases to better define the electroclinical semeiology and evolution of the disorder. Methods: Serial electroclinical and neuropsychological assessments, both during wakefulness and during sleep, were performed in 22 otherwise healthy children with spontaneous (17) or reflex (5) MS, recorded by video-EEG-polygraphy since clinical onset. Results: Seizure onset was between 3 months and 4 years 10 months (50{\%} during first year, 86{\%} before the third year); in reflex cases onset, was earlier than the 14th month. MS recurred during wakefulness and slow sleep in all cases and during REM sleep in reflex cases. MS and related EEG discharges were synchronous or asynchronous. Often ictal EEG discharges were limited to the rolandic and vertex regions (falsely focal paroxysms). Several seizures were subtle and could have escaped recognition. Unusually frequent sleep startles were recorded mostly in reflex cases. MS were well controlled by treatment. At follow-up, between ages 3 and 19 years, four patients had occasional seizures; two had cognitive impairment and three had learning difficulties. No other seizures or cognitive deficits were observed in reflex cases. Conclusions: Seizures associated with BMEI are rarely truly generalized and are often so subtle and related to falsely focal paroxysms that their frequency can be underestimated. The reflex form is a well-defined variant with an early onset, peculiar electroclinical features, and a good prognosis.",
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AU - Meneghello, Lisa

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N2 - Purpose: Benign myoclonic epilepsy in infancy (BMEI) is a nosologically well-defined entity, characterized by myoclonic seizures (MS) in normal children younger than 3 years and by a good long term prognosis. In some cases the seizures are reflex. We studied 22 cases to better define the electroclinical semeiology and evolution of the disorder. Methods: Serial electroclinical and neuropsychological assessments, both during wakefulness and during sleep, were performed in 22 otherwise healthy children with spontaneous (17) or reflex (5) MS, recorded by video-EEG-polygraphy since clinical onset. Results: Seizure onset was between 3 months and 4 years 10 months (50% during first year, 86% before the third year); in reflex cases onset, was earlier than the 14th month. MS recurred during wakefulness and slow sleep in all cases and during REM sleep in reflex cases. MS and related EEG discharges were synchronous or asynchronous. Often ictal EEG discharges were limited to the rolandic and vertex regions (falsely focal paroxysms). Several seizures were subtle and could have escaped recognition. Unusually frequent sleep startles were recorded mostly in reflex cases. MS were well controlled by treatment. At follow-up, between ages 3 and 19 years, four patients had occasional seizures; two had cognitive impairment and three had learning difficulties. No other seizures or cognitive deficits were observed in reflex cases. Conclusions: Seizures associated with BMEI are rarely truly generalized and are often so subtle and related to falsely focal paroxysms that their frequency can be underestimated. The reflex form is a well-defined variant with an early onset, peculiar electroclinical features, and a good prognosis.

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