Original article
Developmental Outcomes of Cryptogenic West Syndrome

https://doi.org/10.1016/j.jpeds.2006.12.004Get rights and content

Objective

To elucidate factors affecting the developmental outcome of cryptogenic West syndrome.

Study design

Medical records of 32 patients, who were followed-up regularly for more than 1 year, were reviewed for clinical features: treatment lag, electroencephalography findings, and seizure evolution. Those features were compared between the normal outcome group (12 patients) and the delayed outcome group (20 patients). The outcomes were determined at the average age of 8.6 ± 4.7 years.

Results

The duration from onset to any treatment of the delayed group was longer than that of the normal group (P < .05). Evolution of electroencephalographic findings showed that paroxysmal discharges reappeared in frontal regions more frequently in the delayed group than in the normal group (P < .05). In the delayed group, other types of seizure except for spasms occurred more commonly than in the normal group (P < .05). More patients of the delayed group evolved to focal epilepsy than those of the normal group (P < .05).

Conclusions

Shorter treatment lag might be associated with a favorable outcome in cryptogenic West syndrome. Reappearance of paroxysmal discharges in the frontal regions and evolution to other types of seizure may be associated with undetectable lesions in the frontal regions.

Section snippets

Methods

We investigated 32 patients (16 boys and 16 girls) in whom cryptogenic West syndrome was diagnosed, who were referred to Saitama Children’s Medical Center, Saitama, Japan, and who were examined regularly for more than 1 year by pediatric neurologists. Cryptogenic West syndrome was defined according to the following criteria: (1) clusters of epileptic spasms with onset <3 years, (2) hypsarrhythmia on electroencephalography (EEG), (3) normal pregnancy, normal development and no eventful history

Results

Thirty-two patients were divided into two groups: 12 patients in the normal outcome group and 20 in the delayed outcome group (Table I). The delayed outcome group consisted of 5 patients with mild mental retardation (IQ, DQ ≥50 and <75), 8 with moderate (IQ, DQ ≥25 and <50), and 7 with severe (IQ, DQ <25). There was no statistical difference in the onset age of spasms and the follow-up duration. The duration from onset of spasms to any treatment of the delayed outcome group was longer than that

Discussion

Our study revealed two significant points regarding the outcome of cryptogenic West syndrome. First, the duration from onset of spasms to any treatment of the delayed outcome group was longer than that of the normal outcome group. Thus, a shorter treatment lag may be associated with a favorable outcome in cryptogenic West syndrome. This result implies the importance of early treatment, which coincides with numerous previous reports.3, 6, 9, 10, 11, 12, 13 Kivity et al9 mentioned that once major

References (19)

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Cited by (25)

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    Similar results were reported by Mohamed et al. (2011) and Matsuo et al. (2001), who indicated that children who responded to first-line treatments had a more favorable long-term developmental outcome than the other groups. Several authors (Hamano et al., 2007; Karvelas et al., 2009; Kivity et al., 2004) have also addressed the early treatment of infantile spasms and shorter treatment lag after the diagnosis was associated with more favorable long-term cognitive outcomes.Since rapid standard treatment was started for most of our patients, our study was not conclusive enough regarding the effects of delay in treatment on the neurodevelopmental outcome of these patients. In conclusion, our study is the first report on neurodevelopmental outcomes of Iranian children with WS.

  • Efficacy and safety of pyridoxal in West syndrome: A retrospective study

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    Ohtsuka et al. reported that all eight of the cryptogenic patients who had complete cessation of spasms had normal to borderline intelligence [5]. Cryptogenic patients who showed complete cessation of spasms with effective therapy in early phases had favorable developmental outcomes [13,14]. Complete cessation in cryptogenic patients and in early phases may be related to favorable developmental outcomes after pyridoxal therapy.

  • Relationship between initial electroencephalographic characteristics and seizure outcomes in children with non-lesional West syndrome

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    The diversities of underlying causes are probably associated with multifarious outcomes of cryptogenic WS. In a study of the variables affecting the developmental outcome of cryptogenic WS (Hamano et al., 2007), it was suggested that a shorter treatment lag may be associated with a favorable outcome, and that paroxysmal discharges in the frontal area and symptom evolution to other seizure types may be related to unidentified lesions there. Another study revealed that the hypometabolism of positron emission tomography (PET) after initial pharmacologic treatment was correlated with the poor seizures and developmental outcomes (Natsume et al., 2014).

  • Infantile spasms syndrome, West syndrome and related phenotypes: What we know in 2013

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    Overall, factors that have been recognized as predictive for a better prognosis are: (a) cryptogenic etiology; (b) age at onset of the spasms older than 4 months; (c) absence of seizures before spasms; (d) no asymmetry at EEG recording; (e) early and rapid response to treatment [35,103]. In a long term (>20 years) study, performed on 259 ISs patients in Japan 2/259 had died: among the 257/259 patients who survived, 40% had daily or weekly seizures while 25.2% had been seizure-free for at least 3 years [104–105]. According to Arce-Portillo [106] the statistically significant poor prognostic factors were linked to the age at onset of spasms (<4 months) and the presence of epileptic seizures and delayed psychomotor development before the onset of spasms.

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    Previous studies have reported on both patient and treatment variables that seem to be implicated in the neurodevelopmental and seizure prognosis. Although these studies yielded conflicting results,3–11 there is general agreement that outcomes are most dependent on underlying disorders and may be more favorable in cryptogenic etiology. Early recognition and prompt treatment may improve outcomes in some patients, particularly in those with cryptogenic West syndrome.2,6

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