Nodding syndrome phenotypes

P. S. Spencer, R. Mazumder, V. S. Palmer, M. S. Pollanen

Research output: Contribution to journalReview article

Abstract

Nodding syndrome (NS) is a progressive encephalopathy of children and adolescents characterized by seizures, including periodic vertical head nodding. Epidemic NS, which has affected parts of East Africa, appears to have clinical overlap with sub-Saharan Nakalanga syndrome (NLS), a brain disorder associated with pituitary dwarfism that appears to have a patchy distribution across sub-Sahara. Clinical stages of NS include inattention and blank stares, vertical head nodding, convulsive seizures, multiple impairments, and severe cognitive and motorsystem disability, including features suggesting parkinsonism. Head nodding episodes occur in clusters with an electrographic correlate of diffuse high-amplitude slow waves followed by an electrodecremental pattern with superimposed diffuse fast activity. Brain imaging reveals differing degrees of cerebral cortical and cerebellar atrophy. Brains of NS-affected children with mild frontotemporal cortical atrophy display neurofibrillary pathology and dystrophic neurites immunopositive for tau, consistent with a progressive neurodegenerative disorder. The etiology of NS and NLS appears to be dominated by environmental factors, including malnutrition, displacement, and nematode infection, but the specific cause is unknown.

Original languageEnglish (US)
Pages (from-to)679-685
Number of pages7
JournalRevue Neurologique
Volume175
Issue number10
DOIs
StatePublished - Dec 2019

Fingerprint

Nodding Syndrome
Phenotype
Head
Brain Diseases
Atrophy
Seizures
Pituitary Dwarfism
Nematode Infections
Northern Africa
Eastern Africa
Parkinsonian Disorders
Neurites
Neuroimaging
Malnutrition
Neurodegenerative Diseases
Pathology
Brain

Keywords

  • Electrocencephalography
  • Endocrine disorder
  • Environmental etiology
  • Nakalanga syndrome
  • Tauopathy

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Spencer, P. S., Mazumder, R., Palmer, V. S., & Pollanen, M. S. (2019). Nodding syndrome phenotypes. Revue Neurologique, 175(10), 679-685. https://doi.org/10.1016/j.neurol.2019.09.005

Nodding syndrome phenotypes. / Spencer, P. S.; Mazumder, R.; Palmer, V. S.; Pollanen, M. S.

In: Revue Neurologique, Vol. 175, No. 10, 12.2019, p. 679-685.

Research output: Contribution to journalReview article

Spencer, PS, Mazumder, R, Palmer, VS & Pollanen, MS 2019, 'Nodding syndrome phenotypes', Revue Neurologique, vol. 175, no. 10, pp. 679-685. https://doi.org/10.1016/j.neurol.2019.09.005
Spencer PS, Mazumder R, Palmer VS, Pollanen MS. Nodding syndrome phenotypes. Revue Neurologique. 2019 Dec;175(10):679-685. https://doi.org/10.1016/j.neurol.2019.09.005
Spencer, P. S. ; Mazumder, R. ; Palmer, V. S. ; Pollanen, M. S. / Nodding syndrome phenotypes. In: Revue Neurologique. 2019 ; Vol. 175, No. 10. pp. 679-685.
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