Is nodding syndrome an onchocerca volvulus associated epilepsy?

Globally, epilepsy is the most common chronic neurological disorder. The incidence in sub-Saharan Africa is 2-3 times higher than that in high income countries. Infection by Onchocerca volvulus may be an underlying risk factor for the high burden and based upon epidemiological associations, has been proposed to cause a group of disorders—Onchocerca associated epilepsies (OAE) like nodding syndrome (NS). To improve our understanding of the disease spectrum, we described the clinical, electroencephalographic (EEG) and magnetic resonance imaging (MRI) features of children with epilepsy and sero-positive for Onchocerca volvulus (possible OAEs other than nodding syndrome). Twenty-nine children and adolescents with non-nodding syndrome OAE in northern Uganda were enrolled. A diagnosis of OAE was made in patients with epilepsy and seizure onset after age 3 years, no reported exposure to perinatal severe febrile illness or traumatic brain injury, no syndromic epilepsy diagnosis and a positive Ov-16 ELISA test. Detailed clinical evaluation including psychiatric, diagnostic EEG, a diagnostic brain MRI (in 10 patients) and laboratory testing were performed. Twenty participants (69%) were male. The mean age was 15.9 (standard deviation [SD] 1.9) years while the mean age at seizure onset was 9.8 (SD 2.9) years. All reported normal early childhood development. The most common clinical presentation was a tonic-clonic seizure. The median number of seizures was 2 (IQR 1–4) in the previous month. No specific musculoskeletal changes, or cranial nerve palsies were reported, neither were any vision, hearing and speech difficulties observed. The interictal EEG was abnormal in the majority with slow wave background activity in 52% (15/29) while 41% (12/29) had focal epileptiform activity. The brain MRI showed mild to moderate cerebellar atrophy and varying degrees of atrophy of the frontal, parietal and occipital lobes. The clinical spectrum of epilepsies associated with Onchocerca may be broader than previously described. In addition, focal onset tonic-clonic seizures, cortical and cerebellar atrophy may be important brain imaging and clinical features.

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Nodding syndrome mystery deepens

Nature ◽

10.1038/nature.2013.12520 ◽

2013 ◽

Author(s):

Hannah Hoag

Keyword(s):

Nodding Syndrome

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The putatively protective Onchocerca volvulus neuronal protein E1 is a member of the death domain protein family

FEBS Letters ◽

10.1016/0014-5793(96)00620-5 ◽

1996 ◽

Vol 390 (1) ◽

pp. 21-24 ◽

Cited By ~ 2

Author(s):

K.D. Erttmann ◽

A. Domeyer ◽

M. Gallin

Keyword(s):

Protein Family ◽

Onchocerca Volvulus ◽

Death Domain ◽

Neuronal Protein ◽

Domain Protein

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Nodding syndrome: bridging the gap—a scoping review protocol

BMJ Open ◽

10.1136/bmjopen-2019-035269 ◽

2020 ◽

Vol 10 (10) ◽

pp. e035269

Author(s):

Ana Cristina De Castro ◽

Ivan Ortega-Deballon

Keyword(s):

Social Science ◽

Scoping Review ◽

Science Citation Index ◽

Citation Index ◽

Grey Literature ◽

Social Science Citation Index ◽

Science Citation ◽

Cochrane Library ◽

Future Research ◽

Nodding Syndrome

IntroductionNodding syndrome (NS) is an encephalopathy of unknown origin that affects children aged between 3 and 15 years old. Cases have been reported since the 1950 in Tanzania and South Sudan, the most heavily affected population is the Acholi community in Uganda. In response to the high incidence of the disease, the Ugandan Government has developed a management algorithm, but access to such measures in affected communities is limited. There is little funding for research on the disease, consequently, few studies have been conducted to date. Nevertheless, the number of scientific publications on NS has increased since 2013, reporting several aetiological hypotheses, management algorithms and cases of stigmatisation; however, none has obtained conclusive results.This document describes a protocol for a scoping review of NS to date aimed at obtaining a broad overview of the disease. The results will identify gaps in knowledge in order to better guide future research, intervention strategies, health policies in areas at risk and cooperation and development programmes.Methods and analysisTo identify the relevant data, we will conduct a literature search using the electronic databases PubMed/Medline, Embase, Social Science Citation Index Scopus, Scientific Electronic Library Online (SciELO), Literatura Latinoamericana y del Caribe en Ciencias de la Salud (LILACS), Social Science Citation Index Expanded and The Cochrane Library. We will also include grey literature. The search strategy will be designed by a librarian.Two members of the team will work independently to identify studies for inclusion and perform data extraction. The search results will be assessed by two independent reviewers and data from the included studies will be charted and summarised in duplicate. The data will be summarised in tables and figures to present the research landscape and describe and map gaps.Ethics and disseminationEthical approval is not required. The scoping review will adhere to the Preferred Reporting Items for Systematic Reviews andMeta-Analyses-ScR guidelines. The results will be disseminated at scientific congresses and meetings.

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