Tropical ataxic neuropathy - A century old enigma

Abstract

Tropical ataxic neuropathy, which is prevalent in the tropics causes significant disability as well as increased mortality and remains an enigmatic disease with no effective treatment or cure, even a century after its identification. The syndrome, first described in Jamaica in 1897 and christened as tropical ataxic neuropathy in 1959, is a constellation of bilateral optic atrophy, bilateral sensory neural deafness, predominant posterior column involvement and pyramidal tract myelopathy, with ataxic polyneuropathy. The exact etiopathogenesis remains unresolved, and several factors have been proposed including malnutrition, vitamin B deficiencies, malabsorption, poor protein consumption, chronic cyanide, and nitrile toxicity, with a strong geospatial endemic prevalence in areas of cassava cultivation. In this review, we summarize the history, epidemiology, clinical features, and controversies regarding the pathogenesis and differential diagnosis of the disease and identify the potential areas for further research concerning this debilitating disorder that is common in the tropics. Its multifactorial etiopathogenesis provides potential opportunities for research and international collaboration to identify novel avenues for treatment.