Introduction: Nodding Syndrome (NS) remains a poorly understood disorder. For a long time, it has been thought to be restricted to East Africa, however cases in Central Africa have been increasing over time. The objective of this systematic review (SR) was to provide a summary of the state of knowledge on NS to date.Methods: All original articles published on NS up to November 2021 were searched in four major databases and in the grey literature. Commentaries, editorials, book chapters, books, conference paper, qualitative studies that mentioned NS cases were also included. Data retrieved included study location, year of study and of publication, population characteristics, definition and diagnosis of NS, associated factors, and treatment if applicable. A meta-analysis of associated factors was performed where possible and results were presented as Odds Ratios and visualized as Forrest Plots. Geographic information systems were used for cartographic representations. The quality of the articles included was assessed.Results: Of the 876 articles initially identified, 67 (corresponding to 59 studies) were included in the SR. NS is only present in Central and East Africa. Interestingly, there were reports of NS in Central Africa prior to 2010, earlier than previously thought. The way NS diagnosis was established varies according to studies, and the 2012 WHO classification was used in only 60% of the studies. Approximately 11% of the articles did not meet the quality requirements set for this review. In our meta-analysis, the main factor associated with NS was onchocerciasis (OR = 8.8 [4.8, 15.9]). However, the pathophysiology of the disease remains poorly understood. The lack of common antiepileptic drugs is a significant barrier for the management of head nodding and associated epileptic seizures. Discussion/conclusion: The lack of an operational definition of NS is an obstacle to its diagnosis and thus to its appropriate treatment. Indeed, diagnostic difficulties might have led to false positives and false negatives which could have altered the picture of NS presented in this article. Treatment should take into account nutritional and psychological factors, as well as associated infections. Some risk factors deserve further investigation; therefore, we suggest a multicentric study with an etiological focus using a more operational definition of NS.

Introduction: Nodding Syndrome (NS) remains a poorly understood disorder. For a long time, it has been thought to be restricted to East Africa, however cases in Central Africa have been increasing over time. The objective of this systematic review (SR) was to provide a summary of the state of knowledge on NS to date.Methods: All original articles published on NS up to November 2021 were searched in four major databases and in the grey literature. Commentaries, editorials, book chapters, books, conference paper, qualitative studies that mentioned NS cases were also included. Data retrieved included study location, year of study and of publication, population characteristics, definition and diagnosis of NS, associated factors, and treatment if applicable. A meta-analysis of associated factors was performed where possible and results were presented as Odds Ratios and visualized as Forrest Plots. Geographic information systems were used for cartographic representations. The quality of the articles included was assessed.Results: Of the 876 articles initially identified, 67 (corresponding to 59 studies) were included in the SR. NS is only present in Central and East Africa. Interestingly, there were reports of NS in Central Africa prior to 2010, earlier than previously thought. The way NS diagnosis was established varies according to studies, and the 2012 WHO classification was used in only 60% of the studies. Approximately 11% of the articles did not meet the quality requirements set for this review. In our meta-analysis, the main factor associated with NS was onchocerciasis (OR = 8.8 [4.8, 15.9]). However, the pathophysiology of the disease remains poorly understood. The lack of common antiepileptic drugs is a significant barrier for the management of head nodding and associated epileptic seizures. Discussion/conclusion: The lack of an operational definition of NS is an obstacle to its diagnosis and thus to its appropriate treatment. Indeed, diagnostic difficulties might have led to false positives and false negatives which could have altered the picture of NS presented in this article. Treatment should take into account nutritional and psychological factors, as well as associated infections. Some risk factors deserve further investigation; therefore, we suggest a multicentric study with an etiological focus using a more operational definition of NS.

Introduction: Nodding Syndrome (NS) remains a poorly understood disorder. For a long time, it has been thought to be restricted to East Africa, however cases in Central Africa have been increasing over time. The objective of this systematic review (SR) was to provide a summary of the state of knowledge on NS to date.Methods: All original articles published on NS up to November 2021 were searched in four major databases and in the grey literature. Commentaries, editorials, book chapters, books, conference paper, qualitative studies that mentioned NS cases were also included. Data retrieved included study location, year of study and of publication, population characteristics, definition and diagnosis of NS, associated factors, and treatment if applicable. A meta-analysis of associated factors was performed where possible and results were presented as Odds Ratios and visualized as Forrest Plots. Geographic information systems were used for cartographic representations. The quality of the articles included was assessed.Results: Of the 876 articles initially identified, 67 (corresponding to 59 studies) were included in the SR. NS is only present in Central and East Africa. Interestingly, there were reports of NS in Central Africa prior to 2010, earlier than previously thought. The way NS diagnosis was established varies according to studies, and the 2012 WHO classification was used in only 60% of the studies. Approximately 11% of the articles did not meet the quality requirements set for this review. In our meta-analysis, the main factor associated with NS was onchocerciasis (OR = 8.8 [4.8, 15.9]). However, the pathophysiology of the disease remains poorly understood. The lack of common antiepileptic drugs is a significant barrier for the management of head nodding and associated epileptic seizures. Discussion/conclusion: The lack of an operational definition of NS is an obstacle to its diagnosis and thus to its appropriate treatment. Indeed, diagnostic difficulties might have led to false positives and false negatives which could have altered the picture of NS presented in this article. Treatment should take into account nutritional and psychological factors, as well as associated infections. Some risk factors deserve further investigation; therefore, we suggest a multicentric study with an etiological focus using a more operational definition of NS.

Introduction: Nodding Syndrome (NS) remains a poorly understood disorder. For a long time, it has been thought to be restricted to East Africa, however cases in Central Africa have been increasing over time. The objective of this systematic review (SR) was to provide a summary of the state of knowledge on NS to date.Methods: All original articles published on NS up to November 2021 were searched in four major databases and in the grey literature. Commentaries, editorials, book chapters, books, conference paper, qualitative studies that mentioned NS cases were also included. Data retrieved included study location, year of study and of publication, population characteristics, definition and diagnosis of NS, associated factors, and treatment if applicable. A meta-analysis of associated factors was performed where possible and results were presented as Odds Ratios and visualized as Forrest Plots. Geographic information systems were used for cartographic representations. The quality of the articles included was assessed.Results: Of the 876 articles initially identified, 67 (corresponding to 59 studies) were included in the SR. NS is only present in Central and East Africa. Interestingly, there were reports of NS in Central Africa prior to 2010, earlier than previously thought. The way NS diagnosis was established varies according to studies, and the 2012 WHO classification was used in only 60% of the studies. Approximately 11% of the articles did not meet the quality requirements set for this review. In our meta-analysis, the main factor associated with NS was onchocerciasis (OR = 8.8 [4.8, 15.9]). However, the pathophysiology of the disease remains poorly understood. The lack of common antiepileptic drugs is a significant barrier for the management of head nodding and associated epileptic seizures. Discussion/conclusion: The lack of an operational definition of NS is an obstacle to its diagnosis and thus to its appropriate treatment. Indeed, diagnostic difficulties might have led to false positives and false negatives which could have altered the picture of NS presented in this article. Treatment should take into account nutritional and psychological factors, as well as associated infections. Some risk factors deserve further investigation; therefore, we suggest a multicentric study with an etiological focus using a more operational definition of NS.