Cognitive deficits after cryptogenic infantile spasms with benign seizure evolution

Gaily, Eija; Appelqvist, Kati; Kantola-Sorsa, Elisa; Liukkonen, Elina; Kyyrönen, Paula; Sarpola, Mia; Huttunen, Heli; Valanne, Leena; Granström, Marja‐Liisa

doi:10.1111/j.1469-8749.1999.tb00519.x

Cited by 8 publications

(3 citation statements)

References 17 publications

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“…The cause is unknown in 25% (uWS). One half of this class of WS patients shows significant long‐term cognitive impairment . Because of the absence of lesions, uWS provides a useful model for quantitative magnetic resonance imaging (MRI) study of West syndrome, without the distortions that structural lesions would introduce.…”

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confidence: 99%

Quantitative magnetic resonance imaging evidence for altered structural remodeling of the temporal lobe in West syndrome

et al. 2015

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SummaryObjectiveTo explore the structure–function relation of the temporal lobe in newly diagnosed West syndrome of unknown cause (uWS).MethodsQuantitative magnetic resonance imaging (three‐dimensional [3D] structural MRI and diffusion tensor imaging [DTI]) was analyzed using voxel‐based morphometry (VBM) and tract‐based spatial statistics (TBSS) in 22 patients and healthy age‐matched controls. The electrophysiologic responsiveness of the temporal lobe was measured using the N100 auditory event‐related potential (aERP) to a repeated 1,000 Hz tone. Neurocognitive function was assessed using the Bayley Scales of Infant Development, Second Edition (BSID‐II). Tests followed first‐line treatment with vigabatrin (17 patients) or high‐dose oral prednisolone (5 patients).ResultsTotal temporal lobe volume was similar in patients and controls. Patients had a smaller temporal stem (TS) (p < 0.0001) and planum temporale (PT) (p = 0.029) bilaterally. TS width asymmetry with a larger right‐sided width in controls was absent in patients (p = 0.033). PT asymmetry was present in both groups, being larger on the right (p = 0.048). VBM gray matter volume was increased at the left temporal lobe (superior and middle temporal gyri, the peri‐rhinal cortex, and medial temporal lobe) (p < 0.005, family wise error‐corrected). VBM gray matter volume correlated with the duration of infantile spasms (Pearson's r = −0.630, p = 0.009). DTI metrics did not differ between patients and controls on TBSS. Mean BSID‐II scores were lower (p < 0.001) and auditory N100 ERP attenuated less in patients than in controls (p = 0.002).SignificanceThe functional networking and white matter development of the temporal lobe are impaired following infantile spasms. Treatment may promote structural plasticity within the temporal lobe following infantile spasms, manifest as increased gray matter volume on VBM. It remains to be investigated further whether this predicts patients' long‐term cognitive difficulties.

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confidence: 99%

Quantitative magnetic resonance imaging evidence for altered structural remodeling of the temporal lobe in West syndrome

et al. 2015

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“…The resolution of hypsarrhythmia is considered the essential marker of primary electroclinical response, together with cessation of spasms (Lux and Osborne, 2004), while its persistence has been shown to correlate with cognitive deterioration (Gaily et al, 1999;Dulac, 2001). Prolonged hypsarrhythmia may either reflect an underlying pathology (drug unresponsive cases), or may result from a prolonged duration of IS before the initial treatment (the long treatment lag).…”

Section: Discussionmentioning

confidence: 99%

The Risk of Lower Mental Outcome in Infantile Spasms Increases after Three Weeks of Hypsarrhythmia Duration

2006

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Summary:To assess the correlation between hypsarrhythmia duration and mental outcome in infantile spasms (IS) the medical records of 48 infants with IS were reviewed retrospectively and psychological assessments undertaken at follow-up at the age of 3 to 13 years. We found 18 (38%) cryptogenic IS cases with typical hypsarrhythmia and 30 symptomatic with modified hypsarrhythmia-further classified into 15 cases as multifocal, 10 as pseudoperiodic and 5 as unilateral hypsarrhythmia. A short treatment lag (one to two weeks) occurred in 25, three to four weeks in 10 cases. Spasms ceased within one month after treatment in 23 infants. At follow-up 15 children had normal mental outcome (borderline included). A correlation between hypsarrhythmia duration longer than three weeks and lower mental outcome was found using the logistic regression model. The duration of hypsarrhythmia represents a sensitive prognostic parameter in IS; the risk of mental retardation increases after three weeks of hypsarrhythmia. Key Words: Infantile spasmsDuration of hypsarrhythmia-Mental outcome-Children.Infantile spasms (IS), i.e., West syndrome, as an epileptic encephalopathy, represents one of the major causes of acquired mental retardation in early childhood (Roger and Dulac, 1994). Prognosis largely depends on etiology, being most unfavorable in prenatal cases with evolution to resistant epilepsy and severe mental retardation. However, infants with cryptogenic/idiopathic etiology can have favorable outcome with lifelong remission of seizures and normal or nearly normal mental development (Lombroso, 1983;Dulac et al., 1993;Riikonen, 1996).Among prognostic factors, the treatment lag and duration of spasms have been recognized as being significant (Lombroso, 1983;Eisermann et al., 2003;Goh et al., 2005). In most studies a treatment lag of up to one month correlates with a better prognosis. Prolonged duration of spasms despite treatment increases the risk of mental retardation (Eisermann et al., 2003;Goh et al., 2005). In some infants hypsarrhythmia continues despite the cessation of spasms. Therefore we explored the impact of hypsarrhythmia duration on mental outcome in 48 children with IS; the epidemiologic part of the study was published in 2002 (Rener Primec et al., 2002 MATERIALS AND METHODSThe medical records of 48 infants diagnosed with IS were studied retrospectively. IS were defined as a syndromic condition (Roger and Dulac, 1994;Lux and Osborne, 2004). The methodology applied has been described previously (Rener Primec et al., 2002), when epidemiologic features of 47 children (permanent residency in Slovenia) were presented. One additional child (immigrant) was included here. The essentials for this study were standard 12-channel EEG recordings taken in wakefulness and sleep at the time of diagnosis of IS and subsequent EEG recordings obtained every 7 to 10 days during the first 2 months or until remission (whichever came first), then monthly for the first 6 months after diagnosis of IS. If the spasms reoccurred an infant was admi...

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“…In general, long-term cognitive outcome appears to depend on the underlying causes (e.g., lesion characteristics); however, about 80% of individuals with the syndrome present with mental retardation (Guzzetta 2006;Koo et al 1993;Matsumoto et al 1981). Patients with the idiopathic form of the disease whose spasms had completely resolved within the first year were evaluated immediately after spasms ceased and subsequently underwent two follow-up evaluations (4-6 years later and 6-8 years later; Gaily et al 1999). Deficits were seen in attention, learning, and memory, even in cases where intellectual functioning was normal.…”

Section: West Syndromementioning

confidence: 99%

Neuropsychological Deficits in Childhood Epilepsy Syndromes

MacAllister

Schaffer

2007

Neuropsychol Rev

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Seizure disorders are relatively common in childhood, and the International League Against Epilepsy (ILAE) provides a hierarchical classification system to define seizure types. At the final level of classification, specific epilepsy syndromes are defined that represent a complex of signs and symptoms unique to an epilepsy condition. The present review discusses the issues related to several of these epilepsy syndromes in childhood, including those classified as generalized idiopathic epilepsies (e.g., childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy), focal epilepsies (benign rolandic epilepsy, occipital epilepsy, temporal lobe epilepsy, frontal lobe epilepsy) and the "epileptic encephalopathies," including Dravet's Syndrome, West Syndrome, Lennox-Gastaut Syndrome, Myoclonic Astatic Epilepsy, and Landau-Kleffner Syndrome. For each syndrome, the epidemiology, clinical manifestations, treatments, and neuropsychological findings are discussed.

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Cognitive deficits after cryptogenic infantile spasms with benign seizure evolution

Cited by 8 publications

References 17 publications

Quantitative magnetic resonance imaging evidence for altered structural remodeling of the temporal lobe in West syndrome

Quantitative magnetic resonance imaging evidence for altered structural remodeling of the temporal lobe in West syndrome

The Risk of Lower Mental Outcome in Infantile Spasms Increases after Three Weeks of Hypsarrhythmia Duration

Neuropsychological Deficits in Childhood Epilepsy Syndromes

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