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  • Updated 11.23.2020
  • Released 08.11.2014
  • Expires For CME 11.23.2023

Nodding syndrome

Introduction

Overview

Nodding syndrome is a newly discovered tau-associated neurologic disorder with poorly understood etiology and is clinically characterized by seizures, which present as nodding of the head, global developmental delay, and neuropsychiatric dysfunctions. 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 novel tau-associated neurologic disorder with poorly understood etiology and is clinically characterized by seizures, which present as nodding of the head, global developmental delay, and neuropsychiatric dysfunctions. It usually 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 2000s.

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

• Case-control studies assessed risk factors and tested for infectious pathogens, toxin exposures, and nutritional deficiencies. Of significance are the epidemiological associations with exposure to Onchocerca volvulus and Mansonella spp, possible vitamin B6 deficiency, and a prior history of measles infection.

Historical note and terminology

In the 1990s, nodding syndrome was reported in what is now the Republic of South 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 to 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 (02; 45; 16). Regarding the Mahenge region in Tanzania, records from 1960 to 1971 have documented the presence of the disease for at least 80 years (01; 02; 40).

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