The head nodding syndrome - Clinical classification and possible causes

ArticleinEpilepsia 49(12):2008-15 · June 2008with45 Reads
DOI: 10.1111/j.1528-1167.2008.01671.x · Source: PubMed
In the 1960s in Tanzania, L. Jilek-Aall observed a seizure disorder characterized by head nodding (HN). Decades later, "nodding disease," reminiscent of what was seen in Tanzania, was reported from Sudan. To date this seizure disorder has not been classified and possible causes still remain obscure. In a prospective study in southern Tanzania, we evaluated 62 patients with HN. Selected patients underwent blood (n = 51) and cerebrospinal fluid (CSF) (n = 48) analyses. Others were chosen for MRI (n = 12) and EEG (n = 10). Seizure type was classified as "head nodding only" and "head nodding plus," the latter being combined with other types of seizure (n =34). During HN, consciousness was impaired in 11 patients (17.7%) and supportive signs of epileptic seizures were described by 15 (24.2%) patients. Precipitating factors were confirmed by 11 (17.7%) patients. Fifty-six (90.3%) patients had at least one relative with epilepsy. EEG confirmed interictal epileptic activity in two patients and unspecific changes in four patients. MRI showed hippocampus pathologies (n = 5) and gliotic changes (n = 5). Skin polymerase chain reaction (PCR) positivity for Onchocerca volvulus was significantly associated with lesions on MRI. However, PCR of the CSF was negative in all cases. We present a comprehensive clinical description of the "HN syndrome," possibly a new epilepsy disorder in sub-Saharan Africa. MRI lesions and their association with positive skin PCR for O. volvulus despite negative PCR of the CSF is intriguing and deserves attention. Furthermore, the high prevalence of hippocampus sclerosis and familial clustering of epilepsy may point toward other potential pathogenetic mechanisms.
    • "In World War 1, Mahenge was held as a military garrison by Germany, which had zero tolerance for cattle infected by rinder- pest [99]. Head nodding in this population was reported as early as 1934 [4], with the first clinical descriptions in the 1960s [2,3]. At this time, the Wapogoro were subsistence farmers who cultivated maize and millet, lacked food storage systems, and did not keep livestock [99]. "
    [Show abstract] [Hide abstract] ABSTRACT: Nodding Syndrome (NS) is an epileptic encephalopathy characterized by involuntary vertical head nodding, other types of seizures, and progressive neurological deficits. The etiology of the east African NS epidemic is unknown. In March 2014, we conducted a case-control study of medical, nutritional and other risk factors associated with NS among children (aged 5–18 years) of Kitgum District, northern Uganda (Acholiland). Available data on food availability, rainfall, and prevalent disease temporally related to the NS epidemic were also analyzed. In NS cases, the mean age of reported head nodding onset was 7.6 years (range 1–17 years). The epidemiologic curve of NS incidence spanned 2000–2013, with peaks in 2003 and 2008. Month of onset of head nodding was non-uniform, with all-year-aggregated peaks in April and June when food availability was low. 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. Medical history revealed a single significant association with NS, namely prior measles infection. NS is compared with the post-measles disorder subacute sclerosing panencephalitis, with clinical expression perhaps triggered by factors associated with poor nutrition.
    Full-text · Article · Aug 2016 · International Journal of Infectious Diseases
    • "Specific suspected causal exposures which were evaluated in previous studies include Onchocerca volvulus, bombs dropped during wartime, nutritional deficiencies, measles infection, consumption of monkey meat, and consumption of possibly contaminated relief seeds and relief food [11]. However, the exact cause and the pathophysiology of nodding syndrome remain unknown [1,4]. Low level of vitamin B6 was reported among cases and control in one study [10] and testing has failed to demonstrate associations between nodding syndrome and vitamin B6 [10,12]. "
    [Show abstract] [Hide abstract] ABSTRACT: Introduction: Nodding syndrome was first reported in Uganda in 2003 among internally displaced populations. Risk factors for the syndrome remain unknown. We therefore explored vitamin B6 deficiency and resulting high 3-hydroxykynurenine (3-HK) levels as risk factor for nodding syndrome in Northern Uganda. Methods: Case-control study conducted in Gulu and Amuru districts. Cases were children/young adults with nodding syndrome. Healthy children/young adults were recruited as controls from same community as cases. Data on socio-demographic and other risk factors was collected using questionnaires. Whole blood was collected in EDTA tubes for assay of 3-HK and vitamin B6 using sandwich ELISA. Conditional logistic regression model was used to assess associations. Results: 66 cases and 73 controls were studied. Factors associated with nodding syndrome were being positive for 3-HK (AOR=4.50, p=0.013), vitamin B6 concentration below mean (AOR=7.22, P=0.001), child being taken care of by mother only (AOR=5.43, p=0.011), child being taken care of by guardian (AOR=5.90, p=0.019) and child consuming relief food at weaning (AOR=4.05, p=0.021). Conclusion: Having low vitamin B6 concentration which leads to a build up of 3-hydroxykynurenine concentration in cases as a main risk factor. Therefore, cases should be treated with vitamin B6 and community members should be sensitise to ensure adequate dietary intake of vitamin B6 so that the risk of nodding syndrome among children is averted. We encourage future prospective intervention study to be conducted to assess the effect of low vitamin B6 on the development of nodding syndrome via raised 3-HK concentration.
    Full-text · Article · Jun 2016
    • "(NS) has been described [5][6][7][8]. The cause of NS remains unclear [5,9], but case control studies have regularly found an association between onchocerciasis and NS [7,[10][11][12]. "
    [Show abstract] [Hide abstract] ABSTRACT: Background: The reason for the high prevalence of epilepsy in onchocerciasis endemic areas remains unknown. The aim of this study was to detect risk factors associated with epilepsy in a region endemic for onchocerciasis. Methods: In June 2014, a case–control study was performed in Titule, Bas-Uélé Province in the Democratic Republic of the Congo. Individuals with unprovoked convulsive epilepsy of unknown aetiology were enrolled as cases (n = 59). Healthy members of families without cases of epilepsy in the same village were recruited as controls (n = 61). A multivariate binomial logistic regression analysis was performed to identify potential risk factors associated with epilepsy. To evaluate the potential protective effect of ivermectin treatment on the development of epilepsy, a nested age-matched case–control study was performed including only those who were eligible for ivermectin treatment in the year before they developed epilepsy. Results: Suspected onchocerciasis skin lesions were more often present in cases than in controls: 12/41 (29%) vs. 1/56 (2%), respectively (odds ratio (OR) 20.26, 95% confidence interval (CI) 2.42–170; p
    Full-text · Article · May 2016
Show more