FOCAL EPILEPTIC MYOCLONUS: DIFFERENTIAL DIAGNOSIS AND PROGNOSIS

The paper describes the clinical, electroencephalographic (EEG), and neuroimaging features of focal epileptic myoclonus (FEM) and the results of therapy in patients with this type of seizure. The latter was identified in 2.5 % of the cases of epilepsy with onset beyond the age of 18 years (n = 1261)...

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Main Authors: N. E. Kvaskova, K. Yu. Mukhin, M. B. Mironov
Format: Article
Language:Russian
Published: ABV-press 2015-04-01
Series:Русский журнал детской неврологии
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Online Access:https://rjdn.abvpress.ru/jour/article/view/25
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author N. E. Kvaskova
K. Yu. Mukhin
M. B. Mironov
author_facet N. E. Kvaskova
K. Yu. Mukhin
M. B. Mironov
author_sort N. E. Kvaskova
collection DOAJ
description The paper describes the clinical, electroencephalographic (EEG), and neuroimaging features of focal epileptic myoclonus (FEM) and the results of therapy in patients with this type of seizure. The latter was identified in 2.5 % of the cases of epilepsy with onset beyond the age of 18 years (n = 1261). The age of onset of FЕМ was from 5 months to 23 years (mean 8.1 ± 1.6 years). The addition of secondary generalized seizures was stated in 90.3% of cases; that of focal motor seizures, epileptic aura, and negative FЕМ was seen in 64.5, 25.8, and 12.8 %, respectively. FЕМ was most commonly located in the flexor muscles of the upper extremities (22.6 %) and facial and upper limb muscles (25.6 %). The regional epileptoformactivity maximally located in the frontocentroparietal leads is a typical EEG pattern of FЕМ in all the patients. Magnetic resonance imaging most frequently revealed diffuse cortical and subcortical atrophy (29 %) and total cortical hemiatrophy (32 %). There was no case of complete remission of FЕМ. Monotherapy with antiepileptic drugs (AEDs) resulted in reduction of FЕМ in only 6.4 % of cases. During duo therapy, there was more than a 50 % reduction in FЕМ in 67.7 %. Our investigation has indicated that active FЕМ is a seizure type resistant to AEDs. This fact necessitates the detection of FЕМ at its early stages in order to predict the course of the disease and search for new therapies for epileptic syndromes associated with this type of seizures.
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spelling doaj-art-aa3bc74f1bc144e897bffe9199cee0052025-08-20T02:56:09ZrusABV-pressРусский журнал детской неврологии2073-88032412-91782015-04-019361210.17650/2073-8803-2014-9-3-6-1219FOCAL EPILEPTIC MYOCLONUS: DIFFERENTIAL DIAGNOSIS AND PROGNOSISN. E. Kvaskova0K. Yu. Mukhin1M. B. Mironov2Svt. Luka’s Institute of Child Neurology and Epilepsy, MoscowSvt. Luka’s Institute of Child Neurology and Epilepsy, MoscowSvt. Luka’s Institute of Child Neurology and Epilepsy, MoscowThe paper describes the clinical, electroencephalographic (EEG), and neuroimaging features of focal epileptic myoclonus (FEM) and the results of therapy in patients with this type of seizure. The latter was identified in 2.5 % of the cases of epilepsy with onset beyond the age of 18 years (n = 1261). The age of onset of FЕМ was from 5 months to 23 years (mean 8.1 ± 1.6 years). The addition of secondary generalized seizures was stated in 90.3% of cases; that of focal motor seizures, epileptic aura, and negative FЕМ was seen in 64.5, 25.8, and 12.8 %, respectively. FЕМ was most commonly located in the flexor muscles of the upper extremities (22.6 %) and facial and upper limb muscles (25.6 %). The regional epileptoformactivity maximally located in the frontocentroparietal leads is a typical EEG pattern of FЕМ in all the patients. Magnetic resonance imaging most frequently revealed diffuse cortical and subcortical atrophy (29 %) and total cortical hemiatrophy (32 %). There was no case of complete remission of FЕМ. Monotherapy with antiepileptic drugs (AEDs) resulted in reduction of FЕМ in only 6.4 % of cases. During duo therapy, there was more than a 50 % reduction in FЕМ in 67.7 %. Our investigation has indicated that active FЕМ is a seizure type resistant to AEDs. This fact necessitates the detection of FЕМ at its early stages in order to predict the course of the disease and search for new therapies for epileptic syndromes associated with this type of seizures.https://rjdn.abvpress.ru/jour/article/view/25epilepsyfocal epileptic myoclonuskozhenvikov’s epilepsykozhevnikov–rasmussen’s encephalitisvideoelectroencephalographic monitoring
spellingShingle N. E. Kvaskova
K. Yu. Mukhin
M. B. Mironov
FOCAL EPILEPTIC MYOCLONUS: DIFFERENTIAL DIAGNOSIS AND PROGNOSIS
Русский журнал детской неврологии
epilepsy
focal epileptic myoclonus
kozhenvikov’s epilepsy
kozhevnikov–rasmussen’s encephalitis
videoelectroencephalographic monitoring
title FOCAL EPILEPTIC MYOCLONUS: DIFFERENTIAL DIAGNOSIS AND PROGNOSIS
title_full FOCAL EPILEPTIC MYOCLONUS: DIFFERENTIAL DIAGNOSIS AND PROGNOSIS
title_fullStr FOCAL EPILEPTIC MYOCLONUS: DIFFERENTIAL DIAGNOSIS AND PROGNOSIS
title_full_unstemmed FOCAL EPILEPTIC MYOCLONUS: DIFFERENTIAL DIAGNOSIS AND PROGNOSIS
title_short FOCAL EPILEPTIC MYOCLONUS: DIFFERENTIAL DIAGNOSIS AND PROGNOSIS
title_sort focal epileptic myoclonus differential diagnosis and prognosis
topic epilepsy
focal epileptic myoclonus
kozhenvikov’s epilepsy
kozhevnikov–rasmussen’s encephalitis
videoelectroencephalographic monitoring
url https://rjdn.abvpress.ru/jour/article/view/25
work_keys_str_mv AT nekvaskova focalepilepticmyoclonusdifferentialdiagnosisandprognosis
AT kyumukhin focalepilepticmyoclonusdifferentialdiagnosisandprognosis
AT mbmironov focalepilepticmyoclonusdifferentialdiagnosisandprognosis