A distinctive childhood epilepsy syndrome

AbstractRasmussen's encephalitis (RE) is a childhood epilepsy syndrome characterized by drug-refractory focal seizures especially epilepsia partialis continua, progressive hemiparesis, and neurocognitive decline. Diagnosis is mainly clinical and can be ambiguous during the early stages of the disease. Its underlying etiology is still elusive, but is suspected to be immune mediated. Treatment of seizures and disease progression is challenging, and although immunotherapy may delay its course, to date, surgical disconnection of the diseased hemisphere remains to be the only effective treatment providing seizure freedom in 60 to 85% of cases. The following review summarizes the current knowledge of the pathophysiology, clinical presentation, management, and treatment of RE.

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Syndromic Classification in the Management of Childhood Epilepsy

Journal of Child Neurology ◽

10.1177/0883073894009002031 ◽

1994 ◽

Vol 9 (2_suppl) ◽

pp. 2S14-2S18 ◽

Cited By ~ 7

Author(s):

Jean Aicardi

Keyword(s):

Juvenile Myoclonic Epilepsy ◽

Partial Epilepsy ◽

Myoclonic Epilepsy ◽

Epilepsy Syndrome ◽

Childhood Epilepsy ◽

Grand Mal ◽

Selection Of

Epilepsy syndromes, defmed as clusters of symptoms or signs occurring consistently together, form the basis of the currently accepted classification of the epilepsies. The concept of epilepsy syndrome is practical for the diagnosis, prognosis, orientation of treatment, and selection of appropriate investigations, but it is of variable specificity and usually does not give information on causes and mechanisms of an epilepsy. Some syndromes, such as childhood absences, partial epilepsy with centrotemporal spikes, or juvenile myoclonic epilepsy, are precisely characterized while other syndromes such as grand mal on awakening or the multiple syndromes with myoclonic seizures are poorly delineated. The usefulness of the concept is limited to well-defined and generally accepted syndromes and many cases of epilepsy do not fit easily in recognizable syndromes. (J Child Neurol 1994; 9(Suppl):2S14-2S18).

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Age, sex and ethnic differentials in the prevalence and control of epilepsy among Sri Lankan children: a population-based study

BMJ Paediatrics Open ◽

10.1136/bmjpo-2018-000430 ◽

2019 ◽

Vol 3 (1) ◽

pp. e000430 ◽

Cited By ~ 1

Author(s):

Jithangi Wanigasinghe ◽

Carukshi Arambepola ◽

Roshini Murugupillai ◽

Thashi Chang

Keyword(s):

Sri Lanka ◽

Focal Epilepsy ◽

Age Groups ◽

Population Based ◽

Cross Sectional Study ◽

Male Dominance ◽

Epilepsy Syndrome ◽

Childhood Epilepsy ◽

Age Group ◽

Cross Sectional

ObjectiveTo estimate the prevalence of childhood epilepsy in Sri Lanka by different age groups (0–5, 6–10 and 11–16 years), sex and ethnicity, and to describe the types and outcomes of epilepsy.Design and patientsA population-based, cross-sectional study was conducted in the district considered to be ethnically most balanced in Sri Lanka. A door-to-door survey was performed in the 0–5 year age group (60 geographically defined areas as clusters; 19 children per cluster), and a school-based survey in the 6–16 year age group (150 classes as clusters; 25 children per cluster). The screened children with epilepsy were reviewed individually for confirmation of the diagnosis of epilepsy, typing of the underlying epilepsy syndrome and assessment of control. The same group of children were re-evaluated 1 year later to reconfirm the syndromic diagnosis and to assess the stability of control of epilepsy.ResultsThe overall prevalence of childhood epilepsy was 5.7 per 10 000 children aged 0–16 years (95% CI: 38 to 87). It was higher with younger ages (73.4 per 10 000 children aged 0–5 years; 55.1 per 10 000 children aged 6–10 years and 50.4 per 10 000 children aged 11–16 years). A male dominance was noted in both age groups. In each age group, the prevalence was highest in children of Sinhalese ethnicity. Symptomatic focal epilepsy was the single most common group of epilepsy in both age groups. Majority of children remained well controlled on medications.ConclusionThe findings indicate a relatively high burden of epilepsy among children in Sri Lanka, however, these were comparable to the burden of disease reported from other countries in the region.

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Early Onset Benign Occipital Epilepsy (Panayiotopoulos syndrome): a case Report & Literature Review

Journal of National Institute of Neurosciences Bangladesh ◽

10.3329/jninb.v1i1.22945 ◽

2015 ◽

Vol 1 (1) ◽

pp. 27-30

Author(s):

Md Enayet Hussain ◽

Rajib Nayan Chowdhury ◽

Md Nahidul Islam ◽

AFM Al Masum Khan ◽

Md Ferdous Miah

Keyword(s):

Case Report ◽

Status Epilepticus ◽

Literature Review ◽

Febrile Seizures ◽

Great Variability ◽

Epilepsy Syndrome ◽

Childhood Epilepsy ◽

High Prevalence ◽

Occipital Spikes ◽

Panayiotopoulos Syndrome

Panayiotopoulos syndrome is a type of benign childhood epilepsy syndrome characterized by episodic autonomic and mainly emetic symptoms. It predominantly affects children of 3-6 years of age (13% of those with one or more non-febrile seizures). EEG shows great variability, with occipital, extra-occipital spikes or brief generalised discharges alone or in combination; it may also be consistently normal. Despite the high prevalence of autonomic status epilepticus, the prognosis of Panayiotopoulos syndrome is usually excellent and most do not require anti epileptic medications. Remission usually occurs within 1-2 years from onset. One third have a single seizure but 5-10% may have more than 10 seizures or a more prolonged course. We present a 5 year old boy with this condition, who presented with episodes of severe vomiting, deviation of eyes, focal seizure, altered sensorium, characteristic EEG in the form of multiple occipital spikes and normal neuroimaging studies.J. Natl Inst. Neurosci Bangladesh 2015;1(1):27-30

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