Two Tanzanian patients with konzo were severely disabled by a non-progressive spastic paraparesis, since the sudden onset during an epidemic six years earlier. At the time of onset they had a high dietary intake of cyanide from exclusive consumption of insufficiently processed bitter cassava roots. MRI of brain and spinal cord were normal but motor evoked potentials on magnetic brain stimulation were absent, even in the only slightly affected upper limbs. Other neurophysiological investigations were largely normal but the more affected patient had central visual field defects. Konzo is a distinct disease entity with selective type upper motor neuron damage.
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The gut microbiome in konzo.
Nat Commun
September 2021
Center for Genetic Medicine Research, Children's Research Institute, Children's National Hospital, Washington, DC, USA.
Konzo, a distinct upper motor neuron disease associated with a cyanogenic diet and chronic malnutrition, predominately affects children and women of childbearing age in sub-Saharan Africa. While the exact biological mechanisms that cause this disease have largely remained elusive, host-genetics and environmental components such as the gut microbiome have been implicated. Using a large study population of 180 individuals from the Democratic Republic of the Congo, where konzo is most frequent, we investigate how the structure of the gut microbiome varied across geographical contexts, as well as provide the first insight into the gut flora of children affected with this debilitating disease using shotgun metagenomic sequencing.
View Article and Find Full Text PDFKonzo: a distinct neurological disease associated with food (cassava) cyanogenic poisoning.
Brain Res Bull
February 2019
Department of Neurology, University of Kinshasa, Democratic Republic of Congo; Department of Tropical Medicine, University of Kinshasa & National Institute of Biomedical Research (INRB), Democratic Republic of Congo; Department of Neurology & School of Public Health, Oregon Health & Science University, Portland OR, USA.
Epidemics of neurodegenerative diseases putatively caused by food toxins have been reported in the tropics with no clear understanding of their pathogenetic mechanisms. These diseases include the disease named Konzo that has been well documented in sub-Sahara Africa, mostly among children and women of childbearing age. Outbreaks of Konzo have occurred in the Democratic Republic of Congo, Mozambique, Tanzania, Central African Republic, Angola, Cameroun, and most recently in Zambia.
View Article and Find Full Text PDFCyanide and the human brain: perspectives from a model of food (cassava) poisoning.
Ann N Y Acad Sci
August 2016
National Nutrition Program, Ministry of Health, and Kinshasa School of Public Health, Kinshasa, Congo.
Threats by fundamentalist leaders to use chemical weapons have resulted in renewed interest in cyanide toxicity. Relevant insights may be gained from studies on cyanide mass intoxication in populations relying on cyanogenic cassava as the main source of food. In these populations, sublethal concentrations (up to 80 μmol/l) of cyanide in the blood are commonplace and lead to signs of acute toxicity.
View Article and Find Full Text PDFLower sulfurtransferase detoxification rates of cyanide in konzo-A tropical spastic paralysis linked to cassava cyanogenic poisoning.
Neurotoxicology
March 2017
Department of Neurology, University of Kinshasa, Congo-Kinshasa, Democratic Republic of the Congo; Institut National de Recherches Biomédicales (INRB), Congo-Kinshasa, Democratic Republic of the Congo; Department of Neurology, Oregon Health & Science University, Portland, OR, USA. Electronic address:
Using a matched case-control design, we sought to determine whether the odds of konzo, a distinct spastic paraparesis associated with food (cassava) cyanogenic exposure in the tropics, were associated with lower cyanide detoxification rates (CDR) and malnutrition. Children with konzo (N=122, 5-17 years of age) were age- and sex-matched with presumably healthy controls (N=87) and assessed for motor and cognition performances, cyanogenic exposure, nutritional status, and cyanide detoxification rates (CDR). Cyanogenic exposure was ascertained by thiocyanate (SCN) concentrations in plasma (P-SCN) and urine (U-SCN).
View Article and Find Full Text PDFResearch on motor neuron diseases konzo and neurolathyrism: trends from 1990 to 2010.
PLoS Negl Trop Dis
November 2012
Programme National de Nutrition, Kinshasa, Democratic Republic of the Congo.
Konzo (caused by consumption of improperly processed cassava, Manihot esculenta) and neurolathyrism (caused by prolonged overconsumption of grass pea, Lathyrus sativus) are two distinct non-infectious upper motor neurone diseases with identical clinical symptoms of spastic paraparesis of the legs. They affect many thousands of people among the poor in the remote rural areas in the central and southern parts of Africa afflicting them with konzo in Ethiopia and in the Indian sub-continent with neurolathyrism. Both diseases are toxico-nutritional problems due to monotonous consumption of starchy cassava roots or protein-rich grass pea seeds as a staple, especially during drought and famine periods.
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