Epilepsy in children with subacute sclerosing panencephalitis.
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TLDR
Atypical SSPE presentation can include mainly focal intractable seizures, and no significant influence was found of the history of epilepsy on the type of S SPE progression.Abstract:
Introduction. Subacute sclerosing panencephalitis (SSPE) is a rare,
progressive, fatal neurodegenerative disease of childhood and early
adolescence caused by defective measles virus. The initial symptoms of SSPE
usually involve regression in cognitive functioning and behavior or recurrent
myoclonic jerks. Seizures revealing SSPE and epilepsy during the clinical
course can occur. Objective. The aim of the study was to analyze clinical and
EEG characteristics of both initially occurred seizures and epilepsy which
developed in the course of the disease. Methods. Retrospective study was
carried out on 19 children (14 boys, 5 girls) with SSPE diagnosed and treated
at our Clinic from 1995 to 2010. Seizures revealed SSPE in our patients aged
from 6.5 to 11.5 years (mean 8.6 years). Results. SSPE onset ranged from 4.5
to 16.5 years (mean 10.05). Complete vaccination was performed in nine
patients. Cognitive and behavioral decline was preceeded by 6-18 months in
two children with intractable focal motor seizures with secondary
generalization, one child with complex partial seizures and one with atypical
absences. During the clinical course of the disease epilepsy developed in 10
(52.6%) cases, including four patients with seizures as the initial SSPE
sign. It occurred mainly in the first year, while in three cases seizures
appeared between 1 and 5 years of the disease evolution. Myoclonus was
present independently from seizures. No significant inter-group differences
were found relating to the type of SSPE progression and history of epilepsy.
The only child with fulminant SSPE presented with initial seizures. Favorable
seizure control was achieved in 60.0% patients. Intractable epilepsy
developed in four patients. Conclusion. Atypical SSPE presentation can
include mainly focal intractable seizures. Epilepsy developed during clinical
course in 52.6% cases. No significant influence was found of the history of
epilepsy on the type of SSPE progression.read more
Citations
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Journal ArticleDOI
Environmental, dietary and case-control study of Nodding Syndrome in Uganda: A post-measles brain disorder triggered by malnutrition?
Peter S. Spencer,Rajarshi Mazumder,Valerie S. Palmer,Michael R. Lasarev,Ryan C. Stadnik,Peter King,Margaret Kabahenda,David Lagoro Kitara,Diane D. Stadler,Breanna McArdle,James K Tumwine +10 more
TL;DR: A case-control study of medical, nutritional and other risk factors associated with NS among children of Kitgum District, northern Uganda found families with one or more NS Cases had been significantly more dependent on emergency food and, immediately prior to head nodding onset in the child, subsistence on moldy plant materials, specifically moldy maize.
Journal ArticleDOI
Subacute sclerosing panencephalitis - current perspectives.
TL;DR: Subacute sclerosing panencephalitis is a progressive neurodegenerative disease that usually occurs 7–10 years after measles infection and is preventable by ensuring that an effective vaccine program for measles is made compulsory for all children younger than 5 years in endemic countries.
Journal ArticleDOI
Subacute sclerosing panencephalitis: clinical phenotype, epidemiology, and preventive interventions
TL;DR: This report documents current data relating to the clinical phenotype, epidemiology, and understanding of SSPE, inclusive of preventive interventions, and indicates children residing in areas with poor vaccination coverage and high prevalence of human immunodeficiency virus are at increased risk of developing S SPE.
Journal ArticleDOI
Neurological Complications of Measles (Rubeola).
TL;DR: Measles and its neurological complications are preventable and must be prevented as mentioned in this paper, and the consequences of inadequate herd immunity to measles should be warned to the public and other clinicians.
Journal ArticleDOI
River blindness goes beyond the eye: autoimmune antibodies, cross-reactive with Onchocerca volvulus antigen, detected in brain of patients with Nodding syndrome.
TL;DR: Because imaging studies and analysis of cerebrospinal fluid failed to demonstrate the parasite in the central nervous system (CNS) of NS patients, it was suggested that an immunological process related to O. volvulus could play a role in the origination of NS.
References
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Journal ArticleDOI
Long term survival in subacute sclerosing panencephalitis: an enigma.
TL;DR: The observations suggest that SSPE may have a highly variable clinical course and warrants cautious approach for counseling at initial evaluation and while interpreting beneficial effect of disease modifying agent(s).
Journal ArticleDOI
Subacute sclerosing panencephalitis (SSPE): an insight into the diagnostic errors from a tertiary care university hospital.
TL;DR: Retrospective case record analysis of patients with subacute sclerosing panencephalitis, evaluated over a 10-year period, was performed to ascertain diagnostic errors and their possible causes.
Journal ArticleDOI
Characteristic clinical features in a case of fulminant subacute sclerosing panencephalitis
TL;DR: MRI study of a 12-year-old girl with fulminant subacute sclerosing panencephalitis without myoclonic jerk and periodic EEG complexes disclosed high intensity lesions in the lateral geniculate bodies, when the patient initially complained of blurred vision.
Journal ArticleDOI
Epidemiological findings and clinical and magnetic resonance presentations in subacute sclerosing panencephalitis.
TL;DR: A normal initial cranial MRI does not exclude subacute sclerosing panencephalitis, which should be kept in mind in childhood demyelinating diseases even when the presentation is unusual, according to a retrospective review of 76 cases.
Journal ArticleDOI
A study of SSPE: early clinical features
TL;DR: With progressive increase in age of presentation, in patients with features like loss of vision, seizures and behavioral changes, SSPE should be carefully considered.
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