Differentiation of myoclonic seizures in epileptic syndromes: A video‐polygraphic study of 26 patients

Hirano, Yoshiko; Oguni, Hirokazu; Funatsuka, Makoto; Imai, Kaoru S.; Ōsawa, Makiko

doi:10.1111/j.1528-1167.2008.01859.x

Cited by 26 publications

(16 citation statements)

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“…These discharges can be similar to those observed in benign myoclonic epilepsy of infancy (BMEI) (Fig. 6), even if, like documented by Hirano et al (2009), the jerks are characterized predominantly by extensor postural changes of the trunk and upper limbs. According to Guerrini et al (2005) the generalized jerks appear to originate from the spreading of central myoclonic activity.…”

Section: Myoclonic Seizuressupporting

confidence: 77%

Electroencephalographic characteristics of Dravet syndrome

Bureau

Bernardina

2011

Epilepsia

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SUMMARYIn Dravet syndrome, interictal and ictal electroencephalography (EEG) recording may remain misleading, and are not specifically altered. Moreover, there is a great polymorphism of clinical and EEG seizure types. Some can be observed in other epileptic syndromes, but others are more specific-particularly the peculiar unilateral seizures, the falsely generalized seizures, probably with a focal onset, and the unstable seizures. In some cases, the ictal manifestations are characterized by the persistent predominant recurrence of convulsive seizures, often induced by body temperature increase, eventually associated with partial complex seizures. The myoclonic events, absences with myoclonic component, obtundation status, and photosensitivity and/or pattern sensitivity are absent or appear relatively late and recur transiently for short periods. In these cases interictal EEG is characterized by the persistent paucity of paroxysmal discharges. In other cases, on a background of convulsive seizures and body temperature sensitivity, one may find a variable association of (1) myoclonic seizures of different types, (2) a strong sensitivity to light and pattern stimulations, with early onset and persistent in time. In these cases, interictal paroxysms and spontaneous and induced (intermittent photic stimulation, patterns, and eye closure) stimulation tend to appear early and to be frequent and persistent during the evolution. According to these electroclinical patterns it is possible to divide the population into two subsets, both sharing common genetic mechanisms but with a different clinical outcome.

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Section: Myoclonic Seizuressupporting

confidence: 77%

Electroencephalographic characteristics of Dravet syndrome

Bureau

Bernardina

2011

Epilepsia

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“…Our series of patients is similar to the cases published previously (Dravet & Bureau, ; Auvin et al., ; Darra et al., ; Hirano et al., ) not only from the electroclinical, but also from the therapeutic and prognostic point of view. However, data obtained in an important electroclinical study showed that the myoclonic seizures may have electroclinical features of focal manifestations (Darra et al., ), as has been documented in patients with juvenile myoclonic epilepsy (Thomas et al., ).…”

Section: Discussionsupporting

confidence: 90%

Myoclonic epilepsy in infancy: An electroclinical study and long‐term follow‐up of 38 patients

et al. 2013

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SUMMARYPurpose: Myoclonic epilepsy in infancy (MEI) is characterized by brief generalized myoclonic seizures associated with generalized spike-wave paroxysms without other seizure types occurring in the first 3 years of life in developmentally normal children. In this study we analyze the electroclinical features, treatment, and outcome of 38 patients with MEI. Methods: A retrospective chart review was conducted in 38 patients followed at the Neurology Department of the Pediatric Hospital Juan P. Garrahan in Buenos Aires, Argentina, between 1990 and 2012. Key Findings: A total of 24 boys and 14 girls were identified. The mean and median ages at seizure onset were 16 and 18 months, respectively (range 3-40 months). Ten patients (28.9%) had a family history of epilepsy, and six (15.8%) had a family history of febrile seizures. All patients had several daily brief and isolated myoclonic seizures during wakefulness and predominantly in the first two stages of sleep. Twelve children (31.5%) had reflex myoclonus, triggered by a tactile stimulus in 10 and additionally by noise and light in 2. The remaining two had photosensitive myoclonic jerks. The interictal electroencephalography (EEG) recordings evidenced generalized spike waves, polyspikes, and polyspike-wave paroxysms. The interictal EEG was normal in 12 patients. The abnormalities on the ictal EEG were similar to those on the interictal EEG. Most of the patients responded well to valproic acid. After a mean follow-up of 13.5 years, 24 patients (63%) were without treatment. At the last examination, 32 patients had normal neurologic and neuropsychological evaluations. Two patients (5.2%) had significant cognitive impairment (an IQ of 60 and 63, respectively) despite good seizure control. Four patients (10.4%) had significant learning impairment, two of whom also had attention deficit hyperactivity disorder. Significance: MEI is a well-defined epileptic syndrome of unknown etiology, but likely of a genetic cause. It is selflimited and pharmacosensitive mainly to valproic acid.

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“…Myoclonic and astatic seizures were also reported. As absence, myoclonic, and astatic seizures are generally produced by generalized spike and wave complexes, the frequent occurrence of interictal generalized 2.5-4 Hz spike-wave EEG discharges in our patients was consistent with these seizure phenotypes [27].…”

Section: Discussionmentioning

confidence: 67%