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Benign Myoclonic Epilepsy in Infancy (BMEI): a longitudinal electroclinical study of 22 Cases

Benign Myoclonic Epilepsy in Infancy (BMEI): a longitudinal electroclinical study of 22 Cases
Benign Myoclonic Epilepsy in Infancy (BMEI): a longitudinal electroclinical study of 22 Cases
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.
0013-9580
31-35
Darra, Francesca
5bf69219-e295-4b64-bf45-04286a7ad53d
Fiorini, Elena
2a3abda2-d3b6-4eef-a89c-2ab3f05531ca
Zoccante, Leonardo
61549ebd-a3ea-4dec-bc5e-ddefecd8dafe
Mastella, Laura
dcbc9c2a-30fb-46f5-99d1-0028b54ba8c5
Torniero, Claudia
f42e5b57-eb63-4878-975e-cfb3fcd9771d
Cortese, Samuele
53d4bf2c-4e0e-4c77-9385-218350560fdb
Meneghello, Lisa
35b09938-554d-4b9f-8213-4827c5abc9cb
Fontana, Elena
e257f9e5-20f7-4a50-9210-edd7e5fe6bcd
Bernardina, Bernardo Dalla
5325874e-719c-4763-8a4a-f9ffe2288e12
Darra, Francesca
5bf69219-e295-4b64-bf45-04286a7ad53d
Fiorini, Elena
2a3abda2-d3b6-4eef-a89c-2ab3f05531ca
Zoccante, Leonardo
61549ebd-a3ea-4dec-bc5e-ddefecd8dafe
Mastella, Laura
dcbc9c2a-30fb-46f5-99d1-0028b54ba8c5
Torniero, Claudia
f42e5b57-eb63-4878-975e-cfb3fcd9771d
Cortese, Samuele
53d4bf2c-4e0e-4c77-9385-218350560fdb
Meneghello, Lisa
35b09938-554d-4b9f-8213-4827c5abc9cb
Fontana, Elena
e257f9e5-20f7-4a50-9210-edd7e5fe6bcd
Bernardina, Bernardo Dalla
5325874e-719c-4763-8a4a-f9ffe2288e12

Darra, Francesca, Fiorini, Elena and Zoccante, Leonardo et al. (2006) Benign Myoclonic Epilepsy in Infancy (BMEI): a longitudinal electroclinical study of 22 Cases. Epilepsia, 47 (S5), 31-35. (doi:10.1111/j.1528-1167.2006.00874.x). (PMID:17239103)

Record type: Article

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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Published date: 2006
Organisations: Clinical Neuroscience

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Local EPrints ID: 380472
URI: https://eprints.soton.ac.uk/id/eprint/380472
ISSN: 0013-9580
PURE UUID: a000ed32-fe6b-4ab9-8547-cf21c752f880
ORCID for Samuele Cortese: ORCID iD orcid.org/0000-0001-5877-8075

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Date deposited: 15 Sep 2015 12:50
Last modified: 06 Jun 2018 12:21

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Contributors

Author: Francesca Darra
Author: Elena Fiorini
Author: Leonardo Zoccante
Author: Laura Mastella
Author: Claudia Torniero
Author: Samuele Cortese ORCID iD
Author: Lisa Meneghello
Author: Elena Fontana
Author: Bernardo Dalla Bernardina

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