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Spectrum of Movement Disorders Among Children With Subacute Sclerosing Panencephalitis: A Cross-Sectional Study
Divyani Garg1, Vanshika Kakkar2, Ashna Kumar2
1Department of Neurology, 28856Lady Hardinge Medical College, New Delhi, India.
Insights
Movement disorders are common in subacute sclerosing panencephalitis (SSPE), a measles virus-related neurological condition in children. Hyperkinetic movement disorders, including dystonia and chorea-athetosis, frequently occur and can be early signs of SSPE.
Area of Science:
- Neurology
- Pediatrics
- Infectious Diseases
Background:
- Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disease caused by persistent measles virus infection in children and adolescents.
- It is characterized by myoclonus, seizures, and neuropsychiatric symptoms, with other movement disorders considered uncommon.
- This study investigates the frequency of diverse movement disorders in SSPE, challenging previous assumptions.
Purpose of the Study:
- To determine the frequency and proportion of various movement disorders in children diagnosed with subacute sclerosing panencephalitis (SSPE).
- To identify the types of movement disorders present in SSPE patients.
- To explore if movement disorders can be an early or presenting feature of SSPE.
Main Methods:
- A cross-sectional study involving 50 children diagnosed with SSPE based on modified Dyken criteria.
- Assessment for movement disorders, clinical profile, and disease severity using Jabbour staging and modified Rankin Scale.
- Comparison of demographic, clinical, and laboratory features between children with and without movement disorders.
Main Results:
- 56% of enrolled children (n=50) exhibited movement disorders.
- Myoclonus was the most frequent (92%), followed by ataxia (18%), chorea-athetosis (14%), and dystonia (12%).
- Movement disorders presented as the initial symptom in 7 children; no significant clinical or laboratory differences were found between groups.
Conclusions:
- Movement disorders are frequent in subacute sclerosing panencephalitis (SSPE), contrary to prior reports.
- Hyperkinetic movement disorders, particularly dystonia and chorea-athetosis, are common non-myoclonus manifestations.
- Recognizing these movement disorders is crucial for early diagnosis, management, and reducing morbidity in SSPE patients.
Abstract:
Background: Subacute sclerosing panencephalitis is a progressive devastating condition due to persistence of mutant measles virus, affecting children and adolescents, characterised by myoclonus, seizures, and neuropsychiatric issues. Movement disorders apart from myoclonus are reportedly uncommon. We aimed to describe frequency and proportion of movement disorders among children with subacute sclerosing panencephalitis, hypothesizing that these occur more frequently than previously reported. Methods: In this cross-sectional study, we enrolled children with subacute sclerosing panencephalitis between 1 month and 18 years of age who fulfilled the diagnosis of subacute sclerosing panencephalitis as per modified Dyken criteria, and examined them for movement disorders. We also assessed their clinical profile and disease severity via Jabbour staging and modified Rankin Scale score. We compared demographic, clinical, and laboratory features of children with and without movement disorders. Results: We enrolled 50 children (36 males; 72%) (age range 1.5-14 years). Of these, 28 (56%) had movement disorders. Among movement disorders, the most frequent was myoclonus (92%), followed by ataxia (9; 18%), chorea-athetosis (7; 14%), dystonia (6; 12%), tremor (4; 8%), repetitive behavior (4; 8%), and parkinsonism (3; 6%). Movement disorders were the presenting feature of subacute sclerosing panencephalitis among 7 children. There were no significant differences in clinical or laboratory features among children with and without movement disorders. Conclusions: Movement disorders were frequent in subacute sclerosing panencephalitis. Hyperkinetic disorders were dominant. Dystonia and chorea-athetosis occurred more commonly among nonmyoclonus movement disorders. Movement disorders may manifest even in earlier stages of subacute sclerosing panencephalitis and may be the heralding feature. Recognition of these features is important to plan management and reduce morbidity.
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