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
Subacute sclerosing panencephalitis. Clinical aspects and prognosis. The Brazilian registry.
Magda Lahorgue Nunes,Jaderson Costa da Costa,Vanessa M. Stancher,Aron J. Diament,F. N. Arita,Sérgio Rosemberg,Paul Dyken +6 more
TL;DR: Epidemiological aspects of subacute sclerosing panencephalitis in Brazil shows endemic regions in the country, a high incidence of post-immunization SSPE and a delay between first symptom and diagnosis.
Subacute sclerosing panencephalitis with atypical early features
TL;DR: Later age of onset and atypical SSPE presentation could be an increasing differential problem leading to erroneous diagnosis of other neurological condition and subsequently to an inappropriate treatment.
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