Seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitis

Abstract Background The clinical features and outcomes of subacute sclerosing panencephalitis (SSPE) in younger children are different from those of adults, leading easily to misdiagnosis during the early stage. So far, there are limited data related to SSPE in preschool children. Methods In order t...

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Main Authors: Shuang Liao, Min Zhong, Nan Zou, Tingsong Li, Li Jiang
Format: Article
Language:English
Published: Wiley 2021-04-01
Series:Brain and Behavior
Subjects:
Online Access:https://doi.org/10.1002/brb3.2051
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author Shuang Liao
Min Zhong
Nan Zou
Tingsong Li
Li Jiang
author_facet Shuang Liao
Min Zhong
Nan Zou
Tingsong Li
Li Jiang
author_sort Shuang Liao
collection DOAJ
description Abstract Background The clinical features and outcomes of subacute sclerosing panencephalitis (SSPE) in younger children are different from those of adults, leading easily to misdiagnosis during the early stage. So far, there are limited data related to SSPE in preschool children. Methods In order to summarize the clinical data and evolution of SSPE in preschool children and to expand the phenotypes of SSPE, the medical charts of preschool patients diagnosed with SSPE were retrospectively reviewed and analyzed; the clinical outcomes of the enrolled cases were evaluated and followed up. Results Overall, we included three cases in the study. Their onset age was 5 years and 2 months, 4 years and 3 months, and 4 years and 2 months, respectively. All patients presented drop attacks or jerks as the onset symptom, and one patient had concurrent gait disturbance. Atypical periodic complexes on electroencephalography (EEG) were recorded in all patients. The brain magnetic resonance imaging (MRI) findings of two cases showed demyelinating lesions predominantly on the white matter. The neurological conditions of all cases deteriorated rapidly. Two children died at 21 months and 6 months after onset, respectively. The other case progressively developed vegetative status and akinetic mutism within 4 months. Conclusions In younger children, the characteristic features of SSPE may be seizures and gait instability as onset manifestations, atypical periodic complexes on EEG, and rapid worsening of neurological conditions.
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spelling doaj.art-61aa13d2b156457bae7d19337ac2eeab2022-12-21T21:25:14ZengWileyBrain and Behavior2162-32792021-04-01114n/an/a10.1002/brb3.2051Seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitisShuang Liao0Min Zhong1Nan Zou2Tingsong Li3Li Jiang4Department of Neurology Children’s Hospital of Chongqing Medical University Ministry of Education Key Laboratory of Child Development and Disorders National Clinical Research Center for Child Health and Disorders (Chongqing) International Science and Technology Cooperation Base of Child Development and Critical Disorders Chongqing Key Laboratory of Pediatrics Chongqing ChinaDepartment of Neurology Children’s Hospital of Chongqing Medical University Ministry of Education Key Laboratory of Child Development and Disorders National Clinical Research Center for Child Health and Disorders (Chongqing) International Science and Technology Cooperation Base of Child Development and Critical Disorders Chongqing Key Laboratory of Pediatrics Chongqing ChinaDepartment of Neurology Children’s Hospital of Chongqing Medical University Ministry of Education Key Laboratory of Child Development and Disorders National Clinical Research Center for Child Health and Disorders (Chongqing) International Science and Technology Cooperation Base of Child Development and Critical Disorders Chongqing Key Laboratory of Pediatrics Chongqing ChinaDepartment of Neurology Children’s Hospital of Chongqing Medical University Ministry of Education Key Laboratory of Child Development and Disorders National Clinical Research Center for Child Health and Disorders (Chongqing) International Science and Technology Cooperation Base of Child Development and Critical Disorders Chongqing Key Laboratory of Pediatrics Chongqing ChinaDepartment of Neurology Children’s Hospital of Chongqing Medical University Ministry of Education Key Laboratory of Child Development and Disorders National Clinical Research Center for Child Health and Disorders (Chongqing) International Science and Technology Cooperation Base of Child Development and Critical Disorders Chongqing Key Laboratory of Pediatrics Chongqing ChinaAbstract Background The clinical features and outcomes of subacute sclerosing panencephalitis (SSPE) in younger children are different from those of adults, leading easily to misdiagnosis during the early stage. So far, there are limited data related to SSPE in preschool children. Methods In order to summarize the clinical data and evolution of SSPE in preschool children and to expand the phenotypes of SSPE, the medical charts of preschool patients diagnosed with SSPE were retrospectively reviewed and analyzed; the clinical outcomes of the enrolled cases were evaluated and followed up. Results Overall, we included three cases in the study. Their onset age was 5 years and 2 months, 4 years and 3 months, and 4 years and 2 months, respectively. All patients presented drop attacks or jerks as the onset symptom, and one patient had concurrent gait disturbance. Atypical periodic complexes on electroencephalography (EEG) were recorded in all patients. The brain magnetic resonance imaging (MRI) findings of two cases showed demyelinating lesions predominantly on the white matter. The neurological conditions of all cases deteriorated rapidly. Two children died at 21 months and 6 months after onset, respectively. The other case progressively developed vegetative status and akinetic mutism within 4 months. Conclusions In younger children, the characteristic features of SSPE may be seizures and gait instability as onset manifestations, atypical periodic complexes on EEG, and rapid worsening of neurological conditions.https://doi.org/10.1002/brb3.2051childrenclinical manifestationprognosissubacute sclerosing panencephalitis
spellingShingle Shuang Liao
Min Zhong
Nan Zou
Tingsong Li
Li Jiang
Seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitis
Brain and Behavior
children
clinical manifestation
prognosis
subacute sclerosing panencephalitis
title Seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitis
title_full Seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitis
title_fullStr Seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitis
title_full_unstemmed Seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitis
title_short Seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitis
title_sort seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitis
topic children
clinical manifestation
prognosis
subacute sclerosing panencephalitis
url https://doi.org/10.1002/brb3.2051
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AT nanzou seizuresasonsetsymptomsandrapidcourseinpreschoolchildrenwithsubacutesclerosingpanencephalitis
AT tingsongli seizuresasonsetsymptomsandrapidcourseinpreschoolchildrenwithsubacutesclerosingpanencephalitis
AT lijiang seizuresasonsetsymptomsandrapidcourseinpreschoolchildrenwithsubacutesclerosingpanencephalitis