Asymmetric and Asynchronous Infantile Spasms

Ek, Gaily; Da, Shewmon; Ht, Chugani; Jg, Curran

doi:10.1111/j.1528-1157.1995.tb01630.x

Cited by 66 publications

(50 citation statements)

References 16 publications

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“…In addition, one patient of the non-PS-ES group had a cluster of spasms followed by a PS. The same seizure sequence was noted by other investigators as well (8,(10)(11)(12)(13). These observations suggest that an electrophysiologic mechanism at work in the evolution from a PS to spasms may not be the same as that underlying a secondarily generalized tonic-clonic convulsion evolving from a partial or rnyoclonic seizure.…”

Section: Discussionsupporting

confidence: 84%

“…Other investigators also reported a relatively high incidence of partial seizures followed by ESs. Gaily et al (13) reported that 210 (17%) of 60 children with West syndrome had partial seizures with lateralizing motor behavior that occurred in close association with clusters of spasms. Ohtsuka et al (1 2) reported that 15 (1 6%) of 92 patients with West syndrome had partial seizures preceded by clustering spasms.…”

Section: Discussionmentioning

confidence: 99%

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Epileptic Spasms Preceded by Partial Seizures with a Close Temporal Association

et al. 1999

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Summary: Purpose: To investigate the distinctive features of patients with West syndrome who had partial seizures followed by epileptic spasms (PS-ES).Methods: We examined 45 patients with West syndrome whose epileptic spasms were recorded with simultaneous video-electroencephalography (EEG) monitoring between 1982 and 1996. We investigated the patients who had PS-ES and compared the PS-ES patients with the 37 patients without Results: Of the 45 patients who had epileptic spasms in clusters (ES) and hypsarrhythmia on the interictal EEG, eight (1 7%) had ES preceded by partial seizures (PS) with a close temporal association. Seven of these eight were female patients. The underlying disorders were tuberous sclerosis (one patient), Aicardi syndrome (one), nonketotic hyperglycinemia PS-ES.(one), and focal cortical dysplasia (one). The etiology was unknown in the remaining four patients, but was suspected to be of prenatal origin in three. Three types of seizure sequence were identified: PS followed several seconds later by ES (two patients), alternating PS and ES starting with PS (three), and PS gradually replaced by ES with overlapping of the two (three). PS-ES disappeared or was replaced by other types of seizures in 1-34 months. Six patients could not walk, and all patients could not speak any sentences at age 3 years.Conclusions: Compared with patients without PS-ES, those with PS-ES more often had organic brain lesions of prenatal origin, other types of seizures before the onset of ES, asymmetric hypsarrhythmia on the EEG, and poor psychomotor outcome. Key Words: West syndrome-Partial seizureEpileptic spasms-Development-Infant.West syndrome is characterized by certain types of seizures [i.e., epileptic spasms (ESs)], which often occur in clusters. However, some patients with this epileptic syndrome have other types of seizures, especially partial seizures (PSs), either dependent on or independent of ES (1-13). An increasing number of reports on the temporal association of ESs and PSs have been published (4-6,8-10,12,13). Hrachovy et al. (1 1) stated that the coupling of PSs with ESs occurs rarely and in some instances is explained by chance coincidence. Other investigators, however, reported much higher incidences of ES, which were closely associated with PS in West syndrome patients (1 0,12,13). To elucidate this seizure sequence and the relation between ESs and PSs in West syndrome, we retrospectively reviewed patients who had hypsarrhythmia on interictal EEG and ESs evidenced by simultaneous video-EEG monitoring (SVEM).Accepted April 12, 1999. Address correspondence and reprint requests to Dr. T. Kubota at Department of Pediatrics, Nagoya University School of Medicine, 65 Tsurumai, Showa-ku, Nagoya, Japan, 466-8550. METHODS AND SUBJECTSBetween 1982 and 1996, ES was recorded at least once in 45 patients with SVEM at Nagoya University Hospital. They all had hypsarrhythmia on the interictal EEG and were admitted for the treatment of the epileptic seizures. After admission, all of the patients underwent a full ...

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Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Epileptic Spasms Preceded by Partial Seizures with a Close Temporal Association

et al. 1999

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“…On the other hand, Gaily et al (9) reported focal spikes, focal sharp waves, and unilateral or asynchronous fast activities as ictal EEGs of tonic spasms, especially asymmetric or asynchronous tonic spasms. Moreover, Gobbi et al (10) reported "periodic spasms," which were similar to tonic spasms in WS but were observed in patients with localization-related epilepsy.…”

Section: Discussionmentioning

confidence: 99%

Analysis of Ictal EEGs of Epilepsy Associated with Tuberous Sclerosis

Ohmori¹,

Ohtsuka²,

Ohno³

et al. 1998

Epilepsia

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Summary: Purpose:To investigate the significance of cortical pathology of tonic spasms in patients with tuberous sclerosis.Methods: The subjects were 38 patients with epilepsy associated with tuberous sclerosis. We analyzed ictal EEGs of tonic spasms and partial seizures by means of video-EEG monitoring for a total of 763 tonic spasms in 20 patients and 107 partial seizures in 15 patients. We also investigated the relation between partial seizures and magnetic resonance imaging (MRI) findings of these patients.Resulrs: Ictal EEG patterns of tonic spasms were divided into generalized and focal patterns. Thirteen patients had only generalized patterns, whereas seven had both patterns. In five patients who had focal ictal patterns of tonic spasms and partial Tuberous sclerosis is one of the main causes of intractable generalized epilepsies such as West syndrome (WS) and Lennox-Gastaut syndrome (LGS) (1-3). Moreover, frontal lobe epilepsies with tuberous sclerosis frequently exhibit the Lennox-like EEG patterns (4). In a preceding article (3, we described how the nature of the epilepsy changed between generalized and localizationrelated epilepsies during the clinical course in a significant number of patients with tuberous sclerosis. Seizure patterns also changed between generalized and partial seizures in an individual patient. In many patients with WS whose epilepsy is associated with tuberous sclerosis, tonic spasms were preceded by partial seizures, or partial seizures appeared after tonic spasms had been suppressed. These findings suggest that cortical pathology plays a significant role in generalized epilepsies associated with tuberous sclerosis.Further to investigate this problem, we carried out a detailed analysis of ictal EEGs of patients with epilepsy with tuberous sclerosis. We also investigated the relation

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“…Spasms were determined to be independent if interictal epileptiform activity resumed between individual spasms (Dulac et al 1993, Fusco and, and clustering if the interspasm interval showed significant decrease of epileptiform activity, background slowing, or attenuation as compared with the interictal state. Significant asymmetry in spasm behaviour and ictal EEG was recognized if more than half of the spasms were asymmetrical (at least 50% amplitude difference between right and left) or asynchronous (Gaily et al 1995).…”

Section: Methodsmentioning

confidence: 99%

“…Asymmetric as well as subtle spasms have also been described (Kellaway et al 1979, Donat et al 1991, Gaily et al 1995, Haga et al 1995. Dulac and coworkers (1993) and Fusco and Vigevano (1993) separated 'independent spasms', where hypsarrhythmia reappears between spasms, and 'clustering spasms' where no hypsarrhythmia is seen between spasms.…”

mentioning

confidence: 99%

Infantile spasms: diagnosis and assessment of treatment response by video‐EEG

Gaily

Liukkonen

Paetau

et al. 2001

Develop Med Child Neuro

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The purpose of this study was to investigate early electroclinical manifestations and evaluate treatment responses by video-EEG in infants with newly diagnosed spasms. Spasms were recorded in 44 infants (27 males, 17 females) before adequate treatment. Mean ages at onset of spasms and at first video-EEG were 5.3 months (range 0.9 to 9 months) and 5.9 months (range 2.4 to 11.5 months) respectively. Thirteen infants had cryptogenic and 31 had symptomatic aetiology. First treatment was vigabatrin in 36 infants. All infants were followed until 12 months of age or death. Treatment response in the first months of therapy was assessed by repeated video-EEG studies in 23 infants. On the first video-EEG, 34 infants had typical symmetric motor spasms, three infants showed asymmetric or asynchronous behaviour, and seven infants had only subtle spasms. Interictal EEG showed hypsarrhythmia in 27 infants and multifocal spikes with normal or nearly normal background in 17 infants. Subtle spasms, asymmetric or asynchronous spasms, and asymmetric ictal or interictal EEG abnormalities were associated with symptomatic aetiology and poor cognitive and seizure outcome at 12 months. Serial video-EEG recordings showed a transition from motor to subtle spasms during the first 2 weeks of vigabatrin therapy in four infants and only subtle spasms in two therapy-resistant infants at 12 months. Cessation of spasms usually preceded disappearance of hypsarrhythmia or multifocal spikes, but persistence of multifocal spikes over several weeks was always associated with existing spasms. Transition of hypsarrhythmia into multifocal spikes was observed during vigabatrin therapy even in infants with intractable spasms. Initial diagnosis of infantile spasms requires video-EEG studies especially in infants with symptomatic aetiology who may show only subtle spasms. Video-EEG is the only reliable method for assessing treatment response as spasms and interictal EEG abnormalities are modified by treatment and may become subtle.

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Asymmetric and Asynchronous Infantile Spasms

Cited by 66 publications

References 16 publications

Epileptic Spasms Preceded by Partial Seizures with a Close Temporal Association

Epileptic Spasms Preceded by Partial Seizures with a Close Temporal Association

Analysis of Ictal EEGs of Epilepsy Associated with Tuberous Sclerosis

Infantile spasms: diagnosis and assessment of treatment response by video‐EEG

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