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|EXPERT COMMENTARY ON PEARLS FROM PAST - “A SPASTIC PARAPLEGIA SYNDROME OF OBSCURE ETIOLOGY”
|Year : 2020 | Volume
| Issue : 2 | Page : 268-269
Enigma of Tropical Spastic Paraplegia
Emeritus Professor of Neurology, Department of Neurology, Institute of Human Behaviour and Allied Sciences, New Delhi, Senior Consultant Neurologist, Sir Ganga Ram Hospital, New Delhi, India
|Date of Web Publication||15-May-2020|
Department of Neurology, Institute of Human Behaviour and Allied Sciences, Dilshad Garden, New Delhi - 110 095
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gourie-Devi M. Enigma of Tropical Spastic Paraplegia. Neurol India 2020;68:268-9
Identification of South Indian Paraplegia by Mani and colleagues, as an important cause of non-compressive myelopathy, with an elegant description of the clinical profile and exclusion of known causes prevalent in 1960's, including syphilis, tuberculosis, vitamin B12 deficiency and lathyrism, was a milestone in clinical neurology. South Indian Paraplegia was latter redesignated by Mani as Tropical Spastic Paraplegia (TSP). Tropical Spastic Paraplegia (TSP) denotes a group of myelopathies of unknown origin occurring mostly in tropical countries with varying causes. Jamaican neuropathy initially considered as peripheral neuropathy, on detailed review showed the presence of pyramidal tract involvement and was considered as the spastic form of Jamaican neuropathy. High incidence of serological tests for yaws and syphilis was observed and nutritional deficiency was considered but never confirmed. Tropical spastic paraplegia has also been described from geographic isolates in Tumaco, Colombia, with a high incidence of yaws but no attributable neurotoxic or nutritional factors for the causation of the disorder. A slightly different phenotype characterized by myelopathy, ataxia, symmetrical peripheral neuropathy, bilateral optic neuropathy and bilateral sensorineural deafness described by Osuntokun was termed as Nigerian tropical ataxic neuropathy. Chronic cyanide toxicity of dietary origin due to consumption of cassava had been implicated in the aetiology of this disorder., Heavy consumption of cassava has also been linked to another disorder, Konzo, which was recognized earlier in Democratic Republic of Congo (DRC) and subsequently outbreaks had occurred not only in DRC but also in many sub-Sahara African countries including Tanzania, Mozambique and quite recently in Zambia. The characteristic feature is severe spastic paraparesis of sudden onset sometimes associated with bilateral optic neuropathy and subclinical peripheral nerve involvement. The concentration of thiocyanate, the main cyanide metabolite, in urine or plasma was markedly elevated in the affected people.
An important emerging cause, a retrovirus, Human T-Lymphocyte virus 1 (HTLV-1) considered to be associated with myelopathy/tropical spastic paraparesis (HAM/TSP) was first identified in nineteenth-century in Jamaica, but definitive evidence as an aetiological factor was established in 1980s in Martinique, Colombia and Jamaica. Subsequently, HAM/TSP cases have been reported from many countries, Tumaco, Seychelles and Japan., Collaborative study between Japan and India revealed that HAM/TSP is rare in India. In the endemic regions it has been estimated that 5-10 million people may be infected with HTLV-1, but it should be recognized that the primary infection is asymptomatic. In the absence of large scale surveys the prevalence of HTLV-1 infection in India is not known. A noteworthy fact is that only 2-5% of those infected with the virus develop HAM/TSP.
Remarkable advances have been made in unravelling the molecular biology, virology and significantly the pathology of HTLV-1 virus. In HAM/TSP, in the thoracic cord infiltrates of mononuclear cells and dense infiltrate around blood vessels were notable features. The observation of inflammatory changes of perivascular cuffing with myelin loss in posterior and lateral columns in two autopsies of TSP from India is significant in the context of the current knowledge about HTLV-1 myelopathy. Recently an elegant and elaborate study of meta-analysis of high throughput data to track down genes involved in the underlying mechanisms of HAM/TSP, revealed novel hub genes targeting critical pathways in HTLV-1 infection.
In conclusion, over the last five decades, since the description of South Indian Paraplegia, remarkable developments have occurred from a change in nomenclature to Tropical Spastic Paraplegia, delineation of clinical features, identification of varying aetiological factors inclusive of nutritional deficiency, treponemal infection such as yaws, chronic cyanide toxicity due to dietary intake of cassava in different countries, to changing patterns over the years due to improved socioeconomic status and health care. Retroviral infection with HTLV-1 has brought a new dimension and HTLV-1 associated myelopathy (HAM) is now well recognized, and the increasing body of evidence has delineated the underlying pathogenic mechanism of spinal cord involvement.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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