Nodding syndrome: origins and natural history of a longstanding epileptic disorder in sub-Saharan Africa

• Published in: African health sciences Vol. 13; no. 2; pp. 176 - 182
• Main Authors: Spencer, P S, Palmer, V S, Jilek-Aall, L
• Format: Journal Article
• Language: English
• Published: Uganda Makerere University Medical School 01-06-2013; Makerere Medical School
• Subjects: Adolescent; Adult; Africa South of the Sahara - epidemiology; amesinzia kichwa; Child; Child, Preschool; Demographics; Epilepsy; Female; Health and Medicine; History; History, 20th Century; History, 21st Century; Humans; Male; Nodding syndrome; Nodding Syndrome - epidemiology; Nodding Syndrome - history; Original; parkinsonism; primary generalized epilepsy; seizure disorder; Symptoms; Tanzania; Traditional societies; Young Adult; Africa South of the Sahara; Sub-Saharan Africa; seizure disorder; parkinsonism; amesinzia kichwa; Tanzania; primary generalized epilepsy
• ISSN: 1680-6905; 1729-0503; 1680-6905
• DOI: 10.4314/ahs.v13i2.1

Background: Repetitive involuntary head nodding was first reported in
the 1960s in the Wapogoro tribe of Tanzania. Objectives: We describe
the natural history of head nodding in the Wapogoro tribe, with special
reference to the earliest reported dates of onset. Methods: We analyzed
clinical data from 150 historical patients seen between 1960 and 1971.
Results: Head nodding with or without grand mal convulsions was present
in 33/150 (~20%) cases, was mostly familial and equally distributed by
gender. Age at onset of head nodding ranged from 2-22 years (mean: ~10
years) in the period 1934-1962. Head nodding preceded onset of grand
mal convulsions by up to 12 months, and motor and psychomotor deficits
indicative of brain damage developed with time. Fourteen of the 33
cases died at 13-39 years of age (mean: ~20 years) while nineteen aged
16-28 years (mean: ~16 years) were still alive. Conclusion: Historical
accounts of head nodding (amesinzia kichwa, Swahili) among the Wapogoro
tribe fit the August 2012 World Health Organization (WHO) case
definition of probable Nodding Syndrome. Reported to have existed in
this population for at least 80 years, Nodding Syndrome is a
progressive seizure disorder that leads to generalized convulsions
(kifafa), brain damage and death.