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EXPERT COMMENTARY ON PEARLS FROM PAST - “A SPASTIC PARAPLEGIA SYNDROME OF OBSCURE ETIOLOGY”
Year : 2020  |  Volume : 68  |  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 Publication15-May-2020

Correspondence Address:
M Gourie-Devi
Department of Neurology, Institute of Human Behaviour and Allied Sciences, Dilshad Garden, New Delhi - 110 095
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.284367

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How to cite this article:
Gourie-Devi M. Enigma of Tropical Spastic Paraplegia. Neurol India 2020;68:268-9

How to cite this URL:
Gourie-Devi M. Enigma of Tropical Spastic Paraplegia. Neurol India [serial online] 2020 [cited 2020 May 26];68:268-9. Available from: http://www.neurologyindia.com/text.asp?2020/68/2/268/284367






Identification of South Indian Paraplegia by Mani and colleagues,[1] 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).[2] 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.[3] 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.[4] A slightly different phenotype characterized by myelopathy, ataxia, symmetrical peripheral neuropathy, bilateral optic neuropathy and bilateral sensorineural deafness described by Osuntokun[5] 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.[5],[6] 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.[7] 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.[7]

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.[8] Subsequently, HAM/TSP cases have been reported from many countries, Tumaco, Seychelles and Japan.[9],[10] Collaborative study between Japan and India revealed that HAM/TSP is rare in India.[11] 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.[12] 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.[13] In HAM/TSP, in the thoracic cord infiltrates of mononuclear cells and dense infiltrate around blood vessels were notable features.[14] 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.[2] 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.[15]

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.



 
  References Top

1.
Mani KS, Punekar BD, Rao KT, Nair D. South Indian paraplegia- A spastic paraplegic syndrome of obscure etiology. Neurol India 1966;14:125-30.  Back to cited text no. 1
    
2.
Mani KS. Neurological disease in South India. In: Spillane JD. (Editor). Tropical Neurology. Oxford University Press: London; 1973. p. 78-85.  Back to cited text no. 2
    
3.
Montgomery RD, Cruickshank EK, Robertson WB, McMenemey WH. Clinical and pathological observations on Jamaican neuropathy; A report on 206 cases. Brain 1964;87:425-62.  Back to cited text no. 3
    
4.
Román GC, Román LN, Spencer PS, Schoenberg BS. Tropical spastic paraparesis: A neuroepidemiological study in Colombia. Ann Neurol 1985;174:361-5.  Back to cited text no. 4
    
5.
Osuntokun BO. An ataxic neuropathy in Nigeria. A clinical, biochemical and electrophysiological study. Brain 1968;91:215-48.  Back to cited text no. 5
    
6.
Oke OL. Cassava as food in Nigeria. World Rev Nutr Diet 1968;9:227-50.  Back to cited text no. 6
    
7.
Kashala-Abotnes E, Okitundu D, Mumba D, Boivin MJ, Tylleskär T, Tshala-Katumbay D. Konzo: A distinct neurological disease associated with food (cassava) cyanogenic poisoning. Brain Res Bull 2019;145:87-91.  Back to cited text no. 7
    
8.
Rodgers-Johnson P, Gajdusek DC, Morgan OS, Zaninovic V, Sarin PS, Graham DS. HTLV-I and HTLV-III antibodies and tropical spastic paraparesis. Lancet 1985;2:1247-8.  Back to cited text no. 8
    
9.
Román GC. The neuroepidemiology of tropical spastic paraparesis. Ann Neurol 1988;23(Suppl):S113-20.  Back to cited text no. 9
    
10.
Coler-Reilly AL, Yagishita N, Suzuki H, Sato T, Araya N, Inoue E, et al. Nation-wide epidemiological study of Japanese patients with rare viral myelopathy using novel registration system (HAM-net). Orphanet J Rare Dis 2016;11:69.  Back to cited text no. 10
    
11.
Hashimoto K, Lalkaka J, Fujisawa J, Singhal BS, Machigashira K, Kubota R, et al. Limited sequence divergence of HTLV-I of Indian HAM/TSP patients from a prototype Japanese isolate. AIDS Res Hum Retroviruses 1993;9:495-8.  Back to cited text no. 11
    
12.
Gessain A, Cassar O. Epidemiological aspects and world distribution of HTLV-1 infection. Front Microbiol 2012;3:388.  Back to cited text no. 12
    
13.
Bangham CR, Araujo A, Yamano Y, Taylor GP. HTLV-1-associated myelopathy/tropical spastic paraparesis. Nat Rev Dis Primers 2015;1:15012.  Back to cited text no. 13
    
14.
Aye MM, Matsuoka E, Moritoyo T, Umehara F, Suehara M, Hokezu Y, et al. Histopathological analysis of four autopsy cases of HTLV-I-associated myelopathy/tropical spastic paraparesis: Inflammatory changes occur simultaneously in the entire central nervous system. Acta Neuropathol 2000;100:245-52.  Back to cited text no. 14
    
15.
Mozhgani SH, Piran M, Zarei-Ghobadi M, Jafari M, Jazayeri SM, Mokhtari-Azad T, et al. An insight to HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) pathogenesis; Evidence from high-throughput data integration and meta-analysis. Retrovirology 2019;16:46.  Back to cited text no. 15
    




 

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