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Seizures as onset symptoms and rapid course in preschool children with subacute sclerosing panencephalitis
Shuang Liao1, Min Zhong1, Nan Zou1
1Department 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, China.
Insights
Subacute sclerosing panencephalitis (SSPE) in preschool children presents with seizures and gait issues, rapid neurological decline, and poor outcomes. Early diagnosis is crucial for this rare condition.
Area of Science:
- Pediatric Neurology
- Neurodegenerative Diseases
- Viral Encephalitis
Background:
- Subacute sclerosing panencephalitis (SSPE) presents differently in children versus adults, often leading to early misdiagnosis.
- Limited data exists on SSPE specifically in preschool-aged children.
- Understanding preschool SSPE is vital for accurate diagnosis and management.
Purpose of the Study:
- To summarize clinical data and disease progression of SSPE in preschool children.
- To expand the known clinical phenotypes of SSPE in this age group.
- To evaluate the outcomes and follow-up of preschool SSPE cases.
Main Methods:
- Retrospective review and analysis of medical charts from preschool patients diagnosed with SSPE.
- Evaluation and follow-up of clinical outcomes for enrolled cases.
- Case series analysis of three preschool children with SSPE.
Main Results:
- Three preschool children diagnosed with SSPE were included, with onset ages between 4 years 2 months and 5 years 2 months.
- All patients exhibited drop attacks or jerks as initial symptoms; one also had gait disturbance.
- Electroencephalography (EEG) showed atypical periodic complexes, and MRI revealed white matter demyelination in two cases. Neurological deterioration was rapid, with two deaths and one case progressing to vegetative state within months.
Conclusions:
- SSPE in young children often manifests with seizures and gait instability.
- Characteristic EEG findings include atypical periodic complexes.
- Rapid neurological decline is a hallmark, underscoring the need for timely diagnosis and intervention.
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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