The head nodding syndrome - Clinical classification and possible causes

Department of Neurology, University of Ulm, Ulm, Germany.
Epilepsia (Impact Factor: 4.57). 06/2008; 49(12):2008-15. 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.

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    • "In Northern Uganda, an estimated 1834 children are affected by nodding syndrome [1], a neurological syndrome of unknown pathogenesis . The disease was first described in southern Tanzania in 1962 [2] [3] [4], in Liberia in 1983 [5], and in South Sudan in 1991 [6] [7] [8]. The syndrome is described as an epidemic epileptic encephalopathy, and clinical studies have shown cerebral atrophy, gliotic lesions, and hippocampal sclerosis [2,9–11]. "
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    ABSTRACT: Nodding syndrome, an epidemic epileptic encephalopathy of unknown etiology, has affected an estimated 1834 children in Northern Uganda. Children are being treated symptomatically but inconsistently with antiepileptic drugs. Ten semistructured interviews with caregivers of affected children and five focus group discussions with 23 relatives, teachers, and religious leaders were conducted to examine the experiences of affected families and communities in Kitgum and Pader districts. The researcher also did participant observation during MoH outreach clinics. Data collection was carried out from July to September 2012, and data were analyzed through inductive thematic analysis. Nodding syndrome severely affects the children's ability to participate in daily life activities. Daily seizures and physical features such as salivating and stunting make them unable to pass as normal, and mood changes make it difficult for some to interact with others. Caregivers of children with nodding syndrome feel confined to their homes, and economic activities are reduced, which affects entire families, especially the education of healthy siblings. The familial clustering and the unknown etiology made many separate from the affected children when eating, sleeping, and having seizures because of a fear of transmission through saliva. Families struggle to provide care with minimal resources and have experienced a reduction in visitors since their children were affected by nodding syndrome. There were signs of apathy in patterns of care, and, generally, parents felt that antiepileptic medicine had brought only slight improvement in their child's condition because many had begun treatment when developmental milestones had already been lost. A consistent supply of antiepileptic medication is likely to reduce the stigma and fear of transmission, as the affected children's acceptance among others was greatly compromised whenever they had seizures. The loss and suffering involved with nodding syndrome are seen as a continuation of the confinement and trauma once caused by war, and a good regimen of medication is not the whole answer. Copyright © 2014 Elsevier Inc. All rights reserved.
    Full-text · Article · Dec 2014 · Epilepsy & Behavior
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    • "Onchocerca volvulus (OV), which is responsible for onchocerciasis (River Blindness). While OV may be a risk factor for Nodding Syndrome, there is no evidence from analysis of cerebrospinal fluid that OV microfilariae enter the central nervous system [2], [4]. Moreover, Nodding Syndrome has not been reported in epilepsy-prone populations of Central and South America where onchocerciasis is endemic. "
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    ABSTRACT: Contamination of food with fungal toxins is a plausible etiologic factor in Nodding Syndrome, a form of epilepsy affecting thousands of children in east Africa. Children developed eating-and cold-induced atonic seizures (with head nodding) and generalized seizures during periods of community displacement when food sources were unreliable and food quality was compromised. Seed of Sorghum bicolor, an important food source during displacement, was collected in a region with Nodding Syndrome. Seed extracts were subjected to a multi-mycotoxin LS-MS/MS assay for 87 analytes. Among other mycotoxins, beauvericin (BEA) was identified and confirmed by comparison with a standard using both multiple reaction monitoring (MRM) and enhanced product ion scans. Principal component analysis of all positive-mode MRM transitions revealed clustering of BEA-positive samples (328-744 µg/kg) in "old" sorghum seed samples. BEA is an apolar lipophilic cyclohexadepsipeptide that forms ionophores in membranes, disturbs intracellular calcium ion homeostasis, disrupts cellular metabolism and induces apoptosis.
    Full-text · Conference Paper · Dec 2013
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    • "Nodding syndrome is an unexplained neurologic disorder that has recently been reported among children in several sub-Saharan African countries and primarily among internally displaced persons [1–4]. The primary and characteristic feature is a paroxysmal “spell” in which the head bobs forward repeatedly over a period of minutes; in most cases the children appear unresponsive during the episode [3, 4]. "
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    ABSTRACT: We report a case of Pyomyositis in a 13-year-old boy diagnosed using WHO surveillance definition of Probable Nodding syndrome. Complete blood count showed Leukocytosis with immature granulocytes and atypical lymphocytes. Except for the liver enzymes which were high the renal functions and serum electrolytes were within normal range values. Culture of a pus-swab grew Staphylococcus aureus. Abdominal ultrasound scan showed a focal mass on the internal and external oblique muscles of the right abdominal wall. Incision and drainage was performed. Histology of the muscle showed non-specific inflammation of the external and internal oblique muscles. This finding may highlight some of the other tropical diseases that occur in children with Nodding syndrome.
    Full-text · Article · Oct 2013 · Pan African Medical Journal
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