Nodding Syndrome in Ugandan Children—Clinical Features, Brain Imaging and Complications: A Case Series
Date
2013-04-08Author
Idro, Richard
Opoka, Opika Robert
Aanyu, T Hellen
Kakooza-Mwesige, Angelina
Piloya-Were, Theresa
Namusoke, Hanifa
Musoke, Bonita Sarah
Nalugya, Joyce
Bangirana, Paul
Mwaka, Amos Deogratius
White, Steven
Chong, King
Atai-Omoruto, D Anne
Mworozi, Edison
Nankunda, Jolly
Kiguli, Sarah
Aceng, Ruth Jane
Tumwine, K James
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Show full item recordAbstract
Objectives Nodding syndrome is a devastating neurological disorder of uncertain aetiology affecting children in Africa. There is no diagnostic test, and risk factors and symptoms that would allow early diagnosis are poorly documented. This study aimed to describe the clinical, electrophysiological and brain imaging (MRI) features and complications of nodding syndrome in Ugandan children.
Design Case series.
Participants 22 children with nodding syndrome brought to Mulago National Referral Hospital for assessment.
Outcome measures Clinical features, physical and functional disabilities, EEG and brain MRI findings and a staging system with a progressive development of symptoms and complications.
Results The median age of symptom onset was 6 (range 4–10) years and median duration of symptoms was 8.5 (range 2–11) years. 16 of 22 families reported multiple affected children. Physical manifestations and complications included stunting, wasting, lip changes and gross physical deformities. The bone age was delayed by 2 (range 1–6) years. There was peripheral muscle wasting and progressive generalised wasting. Four children had nodding as the only seizure type; 18 in addition had myoclonic, absence and/or generalised tonic–clonic seizures developing 1–3 years after the onset of illness. Psychiatric manifestations included wandering, aggression, depression and disordered perception. Cognitive assessment in three children demonstrated profound impairment. The EEG was abnormal in all, suggesting symptomatic generalised epilepsy in the majority. There were different degrees of cortical and cerebellar atrophy on brain MRI, but no hippocampal changes. Five stages with worsening physical, EEG and brain imaging features were identified: a prodrome, the development of head nodding and cognitive decline, other seizure types, multiple complications and severe disability.
Conclusions Nodding syndrome is a neurological disorder that may be characterised as probably symptomatic generalised epilepsy. Clinical manifestations and complications develop in stages which might be useful in defining treatment and rehabilitation. Studies of risk factors, pathogenesis, management and outcome are urgently needed.