Nodding syndrome: recent insights into etiology, pathophysiology, and treatment

Amos Deogratius Mwaka,1 Jerome Roy Semakula,2 Catherine Abbo,3 Richard Idro4 1Department of Medicine, School of Medicine, College of Health Sciences, Makerere University, Kampala, Uganda; 2Infectious Diseases Institute, Makerere University College of Health Sciences, Kampala, Uganda; 3Department of...

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Main Authors: Mwaka AD, Semakula JR, Abbo C, Idro R
Format: Article
Language:English
Published: Dove Medical Press 2018-05-01
Series:Research and Reports in Tropical Medicine
Subjects:
Online Access:https://www.dovepress.com/nodding-syndrome-recent-insights-into-etiology-pathophysiology-and-tre-peer-reviewed-article-RRTM
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author Mwaka AD
Semakula JR
Abbo C
Idro R
author_facet Mwaka AD
Semakula JR
Abbo C
Idro R
author_sort Mwaka AD
collection DOAJ
description Amos Deogratius Mwaka,1 Jerome Roy Semakula,2 Catherine Abbo,3 Richard Idro4 1Department of Medicine, School of Medicine, College of Health Sciences, Makerere University, Kampala, Uganda; 2Infectious Diseases Institute, Makerere University College of Health Sciences, Kampala, Uganda; 3Department of Psychiatry, College of Health Sciences, Makerere University, Kampala, Uganda; 4Department of Pediatrics and Child Health, College of Health Sciences, Makerere University, Kampala, Uganda Abstract: Nodding syndrome is an enigmatic neuropsychiatric and epileptiform disorder associated with psychomotor, mental, and physical growth retardation. The disorder affects otherwise previously normal children aged 3–18 years, with a slight preponderance for the male child. Nodding syndrome has been described in rural regions of some low-income countries in sub-Saharan Africa including northern Uganda, South Sudan, and a mountainous region of southern Tanzania. The cause of the disorder has hitherto eluded scientists. Neuroimaging studies show involvement of the nervous system with associated severe cortical atrophy in the affected children. The affected communities have generated a number of perceived causes including some conspiracy theories related to intentional poisoning of water sources and foods, and causes related to fumes and chemicals from ammunitions used during civil wars in the affected regions. From biomedical perspectives, the treatment of the affected children is geared towards symptoms control and rehabilitation. There is evidence that seizures and behavioral problems including wandering and episodes of aggressions are controllable with anticonvulsants, especially sodium valproate and antipsychotics. No treatments have proven effective in reversing the course of the disorder, and cure remains a distant goal. Community members have used indigenous medicines, cleansing rituals, and prayer interventions, but have not perceived any reasonable improvements. A randomized controlled clinical trial is ongoing in northern Uganda to test the efficacy and effectiveness of doxycycline in the treatment of nodding syndrome. The hypothesis underlying the doxycycline trial underscores the role of antigenic mimicry: that antibodies generated against an antigen of a microorganism that resides inside the black fly-transmitted parasite, Onchocerca volvulus becomes directed against nervous tissue in the brain. This paper reviews some of the recent advances in researches on the etiologies, pathophysiology, and treatment of nodding syndrome. Keywords: nodding syndrome, epilepsy disorders, epilepsy, conspiracy theory, Onchocerca volvulus, Northern Uganda, South Sudan 
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spelling doaj.art-d1598c7acdec4dcabf062f3e349054a12022-12-22T01:30:36ZengDove Medical PressResearch and Reports in Tropical Medicine1179-72822018-05-01Volume 9899338502Nodding syndrome: recent insights into etiology, pathophysiology, and treatmentMwaka ADSemakula JRAbbo CIdro RAmos Deogratius Mwaka,1 Jerome Roy Semakula,2 Catherine Abbo,3 Richard Idro4 1Department of Medicine, School of Medicine, College of Health Sciences, Makerere University, Kampala, Uganda; 2Infectious Diseases Institute, Makerere University College of Health Sciences, Kampala, Uganda; 3Department of Psychiatry, College of Health Sciences, Makerere University, Kampala, Uganda; 4Department of Pediatrics and Child Health, College of Health Sciences, Makerere University, Kampala, Uganda Abstract: Nodding syndrome is an enigmatic neuropsychiatric and epileptiform disorder associated with psychomotor, mental, and physical growth retardation. The disorder affects otherwise previously normal children aged 3–18 years, with a slight preponderance for the male child. Nodding syndrome has been described in rural regions of some low-income countries in sub-Saharan Africa including northern Uganda, South Sudan, and a mountainous region of southern Tanzania. The cause of the disorder has hitherto eluded scientists. Neuroimaging studies show involvement of the nervous system with associated severe cortical atrophy in the affected children. The affected communities have generated a number of perceived causes including some conspiracy theories related to intentional poisoning of water sources and foods, and causes related to fumes and chemicals from ammunitions used during civil wars in the affected regions. From biomedical perspectives, the treatment of the affected children is geared towards symptoms control and rehabilitation. There is evidence that seizures and behavioral problems including wandering and episodes of aggressions are controllable with anticonvulsants, especially sodium valproate and antipsychotics. No treatments have proven effective in reversing the course of the disorder, and cure remains a distant goal. Community members have used indigenous medicines, cleansing rituals, and prayer interventions, but have not perceived any reasonable improvements. A randomized controlled clinical trial is ongoing in northern Uganda to test the efficacy and effectiveness of doxycycline in the treatment of nodding syndrome. The hypothesis underlying the doxycycline trial underscores the role of antigenic mimicry: that antibodies generated against an antigen of a microorganism that resides inside the black fly-transmitted parasite, Onchocerca volvulus becomes directed against nervous tissue in the brain. This paper reviews some of the recent advances in researches on the etiologies, pathophysiology, and treatment of nodding syndrome. Keywords: nodding syndrome, epilepsy disorders, epilepsy, conspiracy theory, Onchocerca volvulus, Northern Uganda, South Sudan https://www.dovepress.com/nodding-syndrome-recent-insights-into-etiology-pathophysiology-and-tre-peer-reviewed-article-RRTMNodding SyndromeEpilepsy disordersEpilepsyConspiracy theoryOnchocerca volvulusNorthern UgandaSouth Sudan
spellingShingle Mwaka AD
Semakula JR
Abbo C
Idro R
Nodding syndrome: recent insights into etiology, pathophysiology, and treatment
Research and Reports in Tropical Medicine
Nodding Syndrome
Epilepsy disorders
Epilepsy
Conspiracy theory
Onchocerca volvulus
Northern Uganda
South Sudan
title Nodding syndrome: recent insights into etiology, pathophysiology, and treatment
title_full Nodding syndrome: recent insights into etiology, pathophysiology, and treatment
title_fullStr Nodding syndrome: recent insights into etiology, pathophysiology, and treatment
title_full_unstemmed Nodding syndrome: recent insights into etiology, pathophysiology, and treatment
title_short Nodding syndrome: recent insights into etiology, pathophysiology, and treatment
title_sort nodding syndrome recent insights into etiology pathophysiology and treatment
topic Nodding Syndrome
Epilepsy disorders
Epilepsy
Conspiracy theory
Onchocerca volvulus
Northern Uganda
South Sudan
url https://www.dovepress.com/nodding-syndrome-recent-insights-into-etiology-pathophysiology-and-tre-peer-reviewed-article-RRTM
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AT abboc noddingsyndromerecentinsightsintoetiologypathophysiologyandtreatment
AT idror noddingsyndromerecentinsightsintoetiologypathophysiologyandtreatment