NODDING DISEASE

An unexplained neurologic condition characterized by episodes of repetitive dropping forward of the head,
often accompanied by other seizure-like activity, such as convulsions or staring spells.

The condition predominantly affects children aged 5–15 years and has been reported in South Sudan from the states
of Western and Central Equatoria, Northern Uganda and southern Tanzania.

Cause

  • Not yet certain but consistent association with onchocerciasis has been found
  • Other associated factors: malnutrition, pyridoxine deficiency

Clinical features

  • Starts at age 5-7 years in previously normal child
  • Early symptoms: problems in concentration and thinking
  • Then “nodding” starts, which is a type of seizure (atonic seizures) often triggered by eating or cold temperatures
  • Cognitive impairment appears
  • Neurological deterioration, delayed puberty and growth retardation progresses until the child becomes mentally and physically disabled

Investigations

  • No diagnostic investigations have been identified

Management

  • Supportive
  • Antiepileptic drugs as above (valproate and phenobarbital)