Neurological disorders associated with cassava diet: a review of putative etiological mechanisms

Metab Brain Dis. 2011 Mar;26(1):79-85. doi: 10.1007/s11011-011-9237-y. Epub 2011 Feb 16.

Abstract

Tropical ataxic neuropathy (TAN) and epidemic spastic paraparesis (konzo) are two neurological disorders associated with the consumption of cassava (Manihot esculenta) in several African countries. TAN is characterized by sensory polyneuropathy, sensory ataxia, bilateral optic atrophy and bilateral sensori-neural deafness. It occurs in elderly individuals subsisting on a monotonous cassava diet with minimal protein supplementation. Konzo is a syndrome of symmetrical spastic paraparesis with a predilection for children and young women and invariably associated with consumption of inadequately processed bitter cassava roots with minimal protein supplementation. Despite numerous epidemiological, clinical and biochemical studies aimed at elucidating the etiological mechanisms of these disorders, their etiologies remain unknown, and there is no known treatment. The diseases continue to be prevalent in endemic areas, causing significant disability and increased mortality. A fresh appraisal of the putative etiologic mechanisms proposed for these intriguing and enigmatic syndromes is presented in this paper. Evidences against a causal role for cyanide intoxication are discussed, and evidences implicating thiamine deficiency as a unifying etiological mechanism for these neurological syndromes are presented. It is concluded that urgent research is needed to evaluate thiamine status and implement a therapeutic trial of thiamine in these debilitating neurological disorders.

Publication types

  • Review

MeSH terms

  • Africa
  • Ataxia / etiology
  • Ataxia / pathology
  • Child
  • Child, Preschool
  • Cyanides / chemistry
  • Cyanides / metabolism
  • Diet / adverse effects*
  • Female
  • Glucosides / chemistry
  • Glucosides / metabolism
  • Hearing Loss, Sensorineural / etiology
  • Hearing Loss, Sensorineural / pathology
  • Humans
  • Male
  • Manihot / chemistry*
  • Neurotoxicity Syndromes / etiology*
  • Nitriles / chemistry*
  • Optic Atrophy / etiology
  • Optic Atrophy / pathology
  • Paraparesis, Spastic / etiology
  • Paraparesis, Spastic / pathology
  • Plant Roots / chemistry
  • Polyneuropathies / etiology
  • Polyneuropathies / pathology
  • Thiamine Deficiency / etiology*
  • Thiamine Deficiency / pathology
  • Thiocyanates / metabolism
  • Thiocyanates / urine
  • Young Adult

Substances

  • Cyanides
  • Glucosides
  • Nitriles
  • Thiocyanates
  • linamarin
  • thiocyanate