Nodding syndrome

Thomas Wagner MD (Dr. Wagner of the University of Heidelberg has no relevant financial relationships to disclose.)
Jim J Sejvar MD (Dr. Sejvar of the Centers for Disease Control and Prevention has no relevant financial relationships to disclose.)
Erich Schmutzhard MD (Dr. Schmutzhard of Medical University Innsbruck has no relevant financial relationships to disclose.)
Andrea S Winkler MD PhD (Dr. Winkler of Technische Universit)
Jerome Engel Jr MD PhD, editor. (Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.)
Originally released August 11, 2014; last updated June 5, 2015; expires June 5, 2018

This article includes discussion of nodding syndrome, amesinzia kichwa, head nodding disease, lucluc, nodding disease, yengo wic, HN only, HN plus, mixed nodding syndrome, neurologic nodding syndrome, and psychiatric nodding syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Nodding syndrome is a recently reported disorder of yet unknown etiology characterized by nodding of the head and, in some children, progressive cognitive dysfunction and stunted growth. It affects previously healthy children between 3 and 18 years of age. It was first documented in the United Republic of Tanzania in the 1960s, but mainly came to public attention when epidemic outbreaks were reported in what is now the Republic of South Sudan in the 1990s and in northern Uganda in the late 2000s. In this article, available data from different case series and case-control studies are reviewed.

Key points

 

• Nodding syndrome is a disorder of yet unknown etiology characterized by nodding of the head and, in some children, progressive cognitive dysfunction and stunted growth affecting previously healthy children between 3 and 18 years of age.

 

• It was first documented in the United Republic of Tanzania in the 1960s, but mainly came to public attention when epidemic outbreaks were reported in what is now the Republic of South Sudan in the 1990s and in northern Uganda in the 2000s.

 

• Case series described clinical features and performed investigations of cerebrospinal fluid, electroencephalography, and neuroimaging (magnetic resonance imaging).

 

• Case-control studies assessed risk factors and tested for infectious pathogens, toxin exposures, and nutritional deficiencies. Of significance are the persistent epidemiological associations with Onchocerca volvulus and Mansonella spp, as well as a possible vitamin B6 deficiency.

Historical note and terminology

In the 1990s, nodding syndrome was reported in southern Sudan and northern Uganda as a distinct novel entity characterized by repetitive head nodding, neurologic and cognitive impairment, delayed puberty, and growth retardation variably associated with other seizures. During 2001–2002, the disease subsequently reached the attention of local authorities, the World Health Organization (WHO), and media. However, in retrospect, similar cases of children with head nodding, cognitive impairment, and abnormal growth and development were previously described in Tanzania, Liberia, and western Uganda (Aall-Jilek 1965; Van der Waals et al 1983; Kaiser et al 2000). Regarding the Mahenge region in Tanzania, records from 1960 to 1971 have documented the presence of the disease for at least 80 years (Aall-Jilek 1962; Aall-Jilek 1965; Spencer et al 2013a).

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