Citation Publication
Title Study location Study date Study design Cases Controls Key finding(s) Journal
43 2008 The head nodding syndrome - clinical classification and possible causes of a new epilepsy disorder Tanzania N/A Cohort 62 - 90.3% had at at least one relative with epilepsy. Association with OV: 31/51 SS and 12/20 PCRs were OV+. CSF PCR negative (0/48). Neurology
26 2012 Nodding syndrome - South Sudan South Sudan 2010 Case-control 38 38 NS associated with OV+ SS. Total study figues included Maridi and Witto communities. Among the 25 Maridi matched pairs, 88% of cases vs 44% of controls were OV+. No significant OV association between the 13 Witto pairs. Morbidity and Mortality Weekly Report
7 2012 Clinical and epidemiologic characteristics of nodding syndrome in Mundri County, southern Sudan South Sudan 2001-02 Case-control 82 81 No association with MV: 2/13 NS patients had a history of MV vs 11/19 controls. Association with OV: 76/82 NS patients had OV+ SS vs 39/81 controls. HN triggered by food (73% of 39 cases). Association with serena (sorghum): OR=6.22; P=0.049. Association with MP: OR=3.22; P= 0.05. African Health Sciences
6 2013 An epidemiologic investigation of potential risk factors for nodding syndrome in Kitgum District, Uganda Uganda 2009 Case-control 49 49 No association with MV or sorghum. Serological association with OV, exposure to munitions (AOR=13.9 [1.4-135.5]), consumption of crushed cassava roots (AOR=5.4 [1.3-22.1]), visual hallucinations (AOR=13.6 [1.5-121.7]) and vitamin B6 deficiency (84% cases, 75% controls). Matched seological AORs for Ov16 and OvFAR/MSA were 3.16 (0.84-11.89) and 20.80 (1.37-316.24) respectively. PLoS One
29 2013 Nodding syndrome in Ugandan children-clinical features, brain imaging and complications: a case series Uganda 2012 Case series 22 - MRI and EEG findings described. 12/22 NS patients had psychiatric morbidities. A 57% reduction in total seizure burden was achieved with sodium valproate. BMJ Open
33 2013 Nodding syndrome (NS) in Northern Uganda: a probable metabolic disorder Uganda 2012 Case-control 101 101 NS associated with malnutrition, OV and a high anion gap. British Journal of Medicine and Medical Research
38 2013 Clinical, neurological, and electrophysiological features of nodding syndrome in Kitgum, Uganda: an observational case series Uganda 2009 Case series 23 - MRI, CSF and EEG findings described. No evidence of inflammation found. Lancet Neurology
2 2013 Nodding syndrome: origins and natural history of a longstanding epileptic disorder in sub-Saharan Africa Tanzania 1960-71 Retrospective 150 - Historical evidence for probable NS cases with onset between 1934 and 1962. African Health Sciences
40 2013 Nodding syndrome in Mundri county, South Sudan: environmental, nutritional and infectious factors South Sudan 2001/2012 Case-control Analysis of data from Tumwine and colleagues 2012. Adds no association with cassava consumption. African Health Sciences
44 2013 MRI findings in people with epilepsy and nodding syndrome in an area endemic for onchocerciasis: an observational study Tanzania N/A Cohort 12 20 MRI findings described. Cerebral pathology associated with OV (SS/PCR). Control group was OCE patients. African Health Sciences
27 2014 Nodding syndrome in Tanzania may not be associated with circulating anti-NMDA- and anti-VGKC receptor antibodies or decreased pyridoxal phosphate serum levels-a pilot study Tanzania N/A Case-control 22 8 Controls=7 healthy, 1 OCE. No evidence for NMDA or VKGC autoantibodies. No association with pyridoxal-phosphate serum levels. African Health Sciences
31 2014 Detection of auto-antibodies to leiomodin-1 in patients with nodding syndrome N/A N/A Case-control 19 19 Anti-LM-1 Ig was detected in the serum of 11/19 NS patients and 5/19 controls. Anti-LM-1 identified in the CSF of an unreported number of NS patients, shown to be neurotoxic in vitro and cross-react with OV tropomyosin. LM-1 expression shown in CNS regions comparable with reported MRI findings. Journal of Neuroimmunology
34 2014 High anion gap metabolic acidosis among children with nodding syndrome (NS) in Northern Uganda: case series Uganda 2012 Case series 10 - Of the 10 probable NS cases all had hypocalcaemia, raised bicarbonate and a high anion gap suggestive of metabolic acidosis. British Journal of Medicine and Medical Research
35 2014 Nodding syndrome in Kitgum District, Uganda: association with conflict and internal displacement N/A N/A Retrospective N/A N/A Retrospective analyses suggested that conflict and movement of people into IDP camps were associated with rises in NS 5-6 and 7 years later respectively. BMJ Open
37 2014 Physical growth, puberty and hormones in adolescents with nodding syndrome; a pilot study Uganda N/A Cross-sectional 10 - Endocrinological analysis suggestive of pituitary growth and gonadal hormone axis dysfunction. 5/8 NS patients had stunting, 8/8 had a normal serum GH level, but 3/8 had low IGF1/IGFBP3. Thyroid, adrenal, parathyroid, prolactin levels were normal. BMC Research Notes
32 2015 Nodding episodes and high anion gap in a 13-year-old child with nodding syndrome: a case report Uganda N/A Case report 1 - Report of a 13-year-old male with probable NS and pyomystitis, as reported in Kitara 2013. Adds temporal association between an elevated anion gap and the presence of seizures. British Journal of Medicine and Medical Research
39 2015 Nodding syndrome: an epileptic disorder restricted to Africa? N/A 2014 Case-control 50 50 Much data available in Spencer and colleagues 2016. This adds that mycotoxin analysis of plasma and urine found no difference between cases and controls. Journal of the Neurological Sciences
30 2016 Is nodding syndrome an Onchocerca volvulus-induced neuroinflammatory disorder? Uganda's story of research in understanding the disease N/A 2013 Case-control 31 11 Review, included as it contains data not published elsewhere. 15/31 NS patients vs 1/11 controls were anti-VGKC Ig positive. International Journal of Infectious Diseases
36 2016 Reduced plasma concentrations of vitamin B6 and increased plasma concentrations of the neurotoxin 3-hydroxykynurenine are associated with nodding syndrome: a case control study in Gulu and Amuru districts, Northern Uganda Uganda 2013 Case-control 66 73 Low plasma vitamin B6 (AOR=7.22, P=0.001), raised 3-HK (AOR=4.50, P=0.0130), child being cared for by mother only (AOR=5.43, P=0.011), child being cared for by guardian (AOR=5.90, P=0.019) and consumption of relief food at weaning (AOR= 4.05, P=0.021) were associated with NS. Pan African Medical Journal
42 2016 Nodding syndrome: 2015 international conference report and Gulu Accord N/A N/A Mixed N/A N/A Report from the 2015 international conference on NS. Results presented, not found elsewhere, include a case-control study (n=39 and 41 respectively) that associated low houshold income and not purifying water with NS. A cross-sectional study investigating the psychiatric and psychological features of 225 Ugandan NS patients, the majority of cases (51.6% male, 48.4% female) started HN in IDP camps between 2005-10. Psychiatric disorders included major episodic depression (25.3%), PTSD (16.4%), GAD (30.7%) and PDD (4%). eNeurologicalSci
41 2016 Environmental, dietary and case-control study of nodding syndrome in Uganda: a post-measles brain disorder triggered by malnutrition? Uganda 2014 Case-control 50 50 Onset of NS peaked in 2003 and 2008, primarily in April and June. Eating emergency food (OR=4.0 [1.3-17.6], p=0.016), maize (OR=4.00 [1.0-26.5]), mouldy maize (OR=4.33 [1.4-18.9], p=0.009) and a history of MV (OR=6.00 [1.03-113], p=0.047) were associated with NS. Journal of the Neurological Sciences
25 2017 Metabolic analysis of children affected with nodding syndrome in north Uganda: A pilot study Uganda 2014 Cohort 48 - Much data available in Denis and colleagues 2017. This study adds clinical biochemistry findings. 37/47 had low biotinidase. Mean BMI was 16.9. Mean acetyl carnitine level was low. Proprionyl carnitine and butyryl carnitine levels were elevated. Developmental Medicine and Child Neurology
17 2017 Is there a line between internal displacement; environmental and dietary factors in the onset of nodding syndrome in northern Uganda? A clinical observational study design Uganda 2014 Cohort 48 - Duration of stay in IDP camps and being in a family in which the firstborn had NS were strongly associated with NS. World Journal of Pharmaceutical and Medical Research
28 2017 Nodding syndrome: multimycotoxin case-control study in Northern Uganda Uganda 2014 Case-control 50 50 Analysis of plasma and urine samples from Spencer and colleagues 2016. Mycotoxins were detected in 14% and 12% of NS and control cases respectively; concluded the mycotoxin burden was similar. Annals of Neurology
8 2017 Nodding syndrome may be an autoimmune reaction to the parasitic worm Onchocerca volvulus N/A N/A Case-control 55 55 Added data for a further 36 cases and controls to the data from Johnson 2014, giving 55 matched pairs. Of the NS cases, 80% were OV+ and 52.7% were anti-LM-1 Ig+. Of the controls, 45.5% were OV+ and 30.9% were anti-LM-1 Ig+. The finding that anti-LM-1 Ig to be neurotoxic in vitro and cross-react with OV tropomyosin was confirmed. LM-1 expression shown in CNS regions comparable with reported MRI findings. 8/16 CSF samples from NS patients were anti-LM-1 Ig+, compared to 0/8 North American controls. Science Translational Medicine
16 2018 Could nodding syndrome in Northern Uganda be a form of autism Uganda 2014 Cohort ? ? Much data available in Denis and colleagues 2017. This study conjectures that NS may be akin to ASD. Pan African Medical Journal