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Neurotoxicology. 2021 Jul;85:54-67. doi: 10.1016/j.neuro.2021.05.001. Epub 2021 May 05.

Konzo risk factors, determinants and etiopathogenesis: What is new? A systematic review.

Neurotoxicology

Marius Baguma, Fabrice Nzabara, Ghislain Maheshe Balemba, Espoir Bwenge Malembaka, Christiane Migabo, Germain Mudumbi, Virginie Bito, Julie Cliff, Jean-Michel Rigo, Joëlle Nsimire Chabwine

Affiliations

  1. Department of Internal Medicine, Hôpital Provincial Général de Référence de Bukavu (HPGRB), Université Catholique de Bukavu (UCB), Bukavu, Democratic Republic of the Congo; Biomedical Research Institute (BIOMED), UHasselt - Hasselt University, Agoralaan, 3590 Diepenbeek, Belgium. Electronic address: [email protected].
  2. Department of Internal Medicine, Hôpital Provincial Général de Référence de Bukavu (HPGRB), Université Catholique de Bukavu (UCB), Bukavu, Democratic Republic of the Congo; École Régionale de Santé Publique (ERSP), Université Catholique de Bukavu (UCB), Bukavu, Democratic Republic of the Congo.
  3. Department of Internal Medicine, Hôpital Provincial Général de Référence de Bukavu (HPGRB), Université Catholique de Bukavu (UCB), Bukavu, Democratic Republic of the Congo.
  4. École Régionale de Santé Publique (ERSP), Université Catholique de Bukavu (UCB), Bukavu, Democratic Republic of the Congo.
  5. Faculty of Agronomy, Université Catholique de Bukavu (UCB), Bukavu, Democratic Republic of the Congo; Department of Geography and Environmental Studies, College of Social Sciences and Humanities, Jimma University, Jimma, Ethiopia.
  6. Department of Pediatrics, Hôpital Provincial Général de Référence de Bukavu (HPGRB), Université Catholique de Bukavu (UCB), Bukavu, Democratic Republic of the Congo.
  7. Biomedical Research Institute (BIOMED), UHasselt - Hasselt University, Agoralaan, 3590 Diepenbeek, Belgium.
  8. Faculty of Medicine, Department of Community Health, Eduardo Mondlane University, Maputo, Mozambique.
  9. Department of Internal Medicine, Hôpital Provincial Général de Référence de Bukavu (HPGRB), Université Catholique de Bukavu (UCB), Bukavu, Democratic Republic of the Congo; Department of Neuroscience and Movement Science, Faculty of Science and Medicine, University of Fribourg, Fribourg, Switzerland.

PMID: 33964344 DOI: 10.1016/j.neuro.2021.05.001

Abstract

Konzo is a toxico-nutritional upper motor neuron disease causing a spastic paraparesis in schoolchildren and childbearing women in some African countries. Almost a century since the first description of konzo, its underlying etiopathogenic mechanisms and causative agent remain unknown. This paper aims at refreshing the current knowledge of konzo determinants and pathogenesis in order to enlighten potential new research and management perspectives. Literature research was performed in PubMed and Web of Science databases according to the PRISMA methodology. Available data show that cassava-derived cyanide poisoning and protein malnutrition constitute two well-documented risk factors of konzo. However, observational studies have failed to demonstrate the causal relationship between konzo and cyanide poisoning. Thiocyanate, the current marker of choice of cyanide exposure, may underestimate the actual level of cyanide poisoning in konzo patients as a larger amount of cyanide is detoxified via other unusual pathways in the context of protein malnutrition characterizing these patients. Furthermore, the appearance of konzo may be the consequence of the interplay of several factors including cyanide metabolites, nutritional deficiencies, psycho-emotional and geo-environmental factors, resulting in pathophysiologic phenomena such as excitotoxicity or oxidative stress, responsible for neuronal damage that takes place at sparse cellular and/or subcellular levels.

Copyright © 2021 Elsevier B.V. All rights reserved.

Keywords: Cassava; Cyanide intoxication; Konzo; Malnutrition; Motor neuron disease

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