title: Nodding syndrome in Ugandan children--clinical features, brain imaging and complications: a case series.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Case series.
  
  
  METHODS
  22 children with nodding syndrome brought to Mulago National Referral Hospital for assessment.
  
  
  METHODS
  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.
authors: Department of Paediatrics, Mulago Hospital/Makerere University College of Health Sciences, Kampala, Uganda.
authors: Richard
authors: R
authors: Idro
authors: Robert Opika
authors: RO
authors: Opoka
authors: Hellen T
authors: HT
authors: Aanyu
authors: Angelina
authors: A
authors: Kakooza-Mwesige
authors: Theresa
authors: T
authors: Piloya-Were
authors: Hanifa
authors: H
authors: Namusoke
authors: Sarah Bonita
authors: SB
authors: Musoke
authors: Joyce
authors: J
authors: Nalugya
authors: Paul
authors: P
authors: Bangirana
authors: Amos Deogratius
authors: AD
authors: Mwaka
authors: Steven
authors: S
authors: White
authors: Kling
authors: K
authors: Chong
authors: Anne D
authors: AD
authors: Atai-Omoruto
authors: Edison
authors: E
authors: Mworozi
authors: Jolly
authors: J
authors: Nankunda
authors: Sarah
authors: S
authors: Kiguli
authors: Jane Ruth
authors: JR
authors: Aceng
authors: James K
authors: JK
authors: Tumwine
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