Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Tropical Myeloneuropathies Clinical Presentation

  • Author: Friedhelm Sandbrink, MD; Chief Editor: Niranjan N Singh, MD, DM  more...
 
Updated: Jan 13, 2015
 

History

TAN

See the list below:

  • Difficulty walking
  • Burning pain in the hands and feet
  • Amblyopia (in some prisoner-of-war camps, as many as two thirds lost vision)
  • Subacute or chronic onset

HAM/TSP

See the list below:

  • Presenting neurological symptoms in 80% of cases - Gradual onset of leg weakness, back pain, paresthesias, and impairment of urinary or bowel function
  • Erectile dysfunction possible - In one case report, the presenting symptom
  • Increased urinary frequency
  • Dermatitis[5] or psoriasis[6]
Next

Physical

TAN

See the list below:

  • Impaired light touch and vibration sensation and proprioception
  • Gait ataxia
  • Romberg sign
  • Hyporeflexia or areflexia
  • Sensorineural hearing loss
  • Muscle weakness and atrophy that can involve upper extremities
  • Similar symptoms were described among prisoners of war in the tropical and subtropical regions.

HAM/TSP

See the list below:

  • Spastic paraparesis or paraplegia with hyperreflexia, clonus, and extensor plantar responses; weakness of the lower extremities, more marked proximally
  • Decreased touch and pinprick sensation in poorly defined thoracic areas
  • Vibration sensation frequently impaired, especially in the lower extremities, resulting from spinal cord or peripheral nerve involvement[7]
  • Low lumbar pain with radiation to the legs
  • Hyperreflexia of upper extremities frequently associated with Hoffmann sign
  • Less frequent neurological findings - Cerebellar signs (ie, intentional tremor, dysmetria), optic nerve atrophy, deafness, nystagmus, cranial nerve deficits, upper extremities tremor, absent or diminished ankle jerk
  • Increased urinary frequency - Due to detrusor hyperreflexia (ie, neurogenic bladder) associated with increased incidence of urinary tract infection
Previous
Next

Causes

TAN

In many cases, TAN is associated with excessive consumption of cassava, also known as the mandioca or tapioca plant, which is one of the most important sources of calories in the tropical countries. About 300 million people depend on it for subsistence, especially in the tropical regions of the Americas and in Africa. Cassava contains cyanide in the form of a cyanogenic glycoside, linamarin, which releases cyanide by the enzymatic action of linamarinase or by hydrolysis. Chronic cyanide intoxication has been confirmed as the cause of the TAN described in Nigeria and Tanzania. In these patients, treatment with high-dose vitamins was not satisfactory, suggesting that the vitamin deficiencies are not important in the etiology of the disease in these cases.

Processing of the cassava flour removes almost all the cyanide, but during a drought, these procedures tend to be shortened or ignored. Many people, especially women and children, eat the cassava raw or merely sun dried. The cyanide content of cassava increases during a drought, which may lead to a relatively higher incidence of severe cyanide intoxication.

Vitamin deficiencies and tropical malabsorption were the causes of TAN in prisoners of war. In most of the cases, the affected individuals were deficient in group B vitamins.

HAM/TSP

TSP is caused by an infection with HTLV-1.

Cases of TSP have been documented in which HTLV-1 was not detected.

Previous
 
 
Contributor Information and Disclosures
Author

Friedhelm Sandbrink, MD Assistant Professor of Neurology, Georgetown University School of Medicine; Assistant Clinical Professor of Neurology, George Washington University School of Medicine and Health Sciences; Director, EMG Laboratory and Chief, Chronic Pain Clinic, Department of Neurology, Washington Veterans Affairs Medical Center

Friedhelm Sandbrink, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Florian P Thomas, MD, PhD, Drmed, MA, MS Director, National MS Society Multiple Sclerosis Center; Professor and Director, Clinical Research Unit, Department of Neurology, Adjunct Professor of Physical Therapy, Associate Professor, Institute for Molecular Virology, St Louis University School of Medicine; Editor-in-Chief, Journal of Spinal Cord Medicine

Florian P Thomas, MD, PhD, Drmed, MA, MS is a member of the following medical societies: Academy of Spinal Cord Injury Professionals, American Academy of Neurology, American Neurological Association, Consortium of Multiple Sclerosis Centers, National Multiple Sclerosis Society, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Niranjan N Singh, MD, DM Associate Professor of Neurology, University of Missouri-Columbia School of Medicine

Niranjan N Singh, MD, DM is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Headache Society

Disclosure: Nothing to disclose.

Additional Contributors

Carmel Armon, MD, MSc, MHS Chair, Department of Neurology, Assaf Harofeh Medical Center, Tel Aviv University Sackler Faculty of Medicine, Israel

Carmel Armon, MD, MSc, MHS is a member of the following medical societies: American Academy of Neurology, Massachusetts Medical Society, American Academy of Sleep Medicine, American Stroke Association, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American College of Physicians, American Epilepsy Society, American Medical Association, American Neurological Association, Sigma Xi

Disclosure: Received research grant from: Neuronix Ltd, Yoqnea'm, Israel.

Acknowledgements

Eliad Culcea, MD Consulting Staff, Department of Neurology, Benefis Medical Group

Eliad Culcea, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

References
  1. Izumo S, Umehara F, Osame M. HTLV-I-associated myelopathy. Neuropathology. 2000 Sep. 20 Suppl:S65-8. [Medline].

  2. Gilbert DT. Human T-cell leukaemia/lymphoma virus type-1 associated myeloneuropathies--a Caribbean perspective. West Indian Med J. 2012 Jul. 61(4):408-14. [Medline].

  3. Oluwole OS, Onabolu AO, Link H, Rosling H. Persistence of tropical ataxic neuropathy in a Nigerian community. J Neurol Neurosurg Psychiatry. 2000. 69(1):96-101. [Medline].

  4. Olindo S, Lezin A, Cabre P, et al. HTLV-1 proviral load in peripheral blood mononuclear cells quantified in 100 HAM/TSP patients: a marker of disease progression. J Neurol Sci. 2005 Oct 15. 237(1-2):53-9. [Medline].

  5. de Oliveira Mde F, Bittencourt AL, Brites C, et al. HTLV-I associated myelopathy/tropical spastic paraparesis in a 7-year-old boy associated with infective dermatitis. J Neurol Sci. 2004 Jul 15. 222(1-2):35-8. [Medline].

  6. Watanabe A, Kawajiri M, Ikezoe K, et al. HTLV-1-associated myelopathy/tropical spastic paraparesis accompanied with psoriasis. J Neurol Sci. 2004 Jun 15. 221(1-2):95-7. [Medline].

  7. Kiwaki T, Umehara F, Arimura Y, et al. The clinical and pathological features of peripheral neuropathy accompanied with HTLV-I associated myelopathy. J Neurol Sci. 2003 Jan 15. 206(1):17-21. [Medline].

  8. Bagnato F, Butman JA, Mora CA, et al. Conventional magnetic resonance imaging features in patients with tropical spastic paraparesis. J Neurovirol. 2005 Dec. 11(6):525-34. [Medline].

  9. Matsuzaki T, Nakagawa M, Nagai M, et al. HTLV-I proviral load correlates with progression of motor disability in HAM/TSP: analysis of 239 HAM/TSP patients including 64 patients followed up for 10 years. J Neurovirol. 2001 Jun. 7(3):228-34. [Medline].

  10. Nakagawa M, Nakahara K, Maruyama Y, et al. Therapeutic trials in 200 patients with HTLV-I-associated myelopathy/ tropical spastic paraparesis. J Neurovirol. 1996 Oct. 2(5):345-55. [Medline].

  11. Croda MG, de Oliveira AC, Vergara MP, Bonasser F, Smid J, Duarte AJ, et al. Corticosteroid therapy in TSP/HAM patients: the results from a 10 years open cohort. J Neurol Sci. 2008 Jun 15. 269(1-2):133-7. [Medline].

  12. Izumo S, Goto I, Itoyama Y, et al. Interferon-alpha is effective in HTLV-I-associated myelopathy: a multicenter, randomized, double-blind, controlled trial. Neurology. 1996 Apr. 46(4):1016-21. [Medline].

  13. Shirabe S, Nakamura T, Tsujino A, et al. Successful application of pentoxifylline in the treatment of HTLV-1 associated myelopathy. J Neurol Sci. 1997. 151(1):97-101. [Medline].

  14. Oh U, Yamano Y, Mora CA, et al. Interferon-beta1a therapy in human T-lymphotropic virus type I-associated neurologic disease. Ann Neurol. 2005 Apr. 57(4):526-34. [Medline].

  15. Araujo AQ, Andrade-Filho AS, Castro-Costa CM, et al. HTLV-I-associated myelopathy/tropical spastic paraparesis in Brazil: a nationwide survey. HAM/TSP Brazilian Study Group. J Acquir Immune Defic Syndr Hum Retrovirol. 1998 Dec 15. 19(5):536-41. [Medline].

  16. Aye MM, Matsuoka E, Moritoyo T, 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 (Berl). 2000 Sep. 100(3):245-52. [Medline].

  17. Bangham CR. HTLV-1 infections. J Clin Pathol. 2000 Aug. 53(8):581-6. [Medline].

  18. Castillo JL, Cea JG, Verdugo RJ, Cartier L. Sensory dysfunction in HTLV-I-associated myelopathy/tropical spastic paraparesis. A comprehensive neurophysiological study. Eur Neurol. 1999 Jul. 42(1):17-22. [Medline].

  19. Cavrois M, Gessain A, Gout O, et al. Common human T cell leukemia virus type 1 (HTLV-1) integration sites in cerebrospinal fluid and blood lymphocytes of patients with HTLV-1-associated myelopathy/tropical spastic paraparesis indicate that HTLV-1 crosses the blood-brain barrier via clonal H. J Infect Dis. 2000 Oct. 182(4):1044-50. [Medline].

  20. Figueiroa FL, Andrade Filho AS, Crvalho ES. HTLV-I associated myelopathy: clinical and epidemiological profile. Braz J Infect Dis. 2000 Jun. 4(3):126-30. [Medline].

  21. Gazzola P, Cocito L, De Maria A, et al. Successful 2-year therapy with systemic interferon-alpha for HTLV-I associated myelopathy. J Neurol Sci. 1999 Jan 15. 162(2):205-7. [Medline].

  22. Giordano C, Dumas M, Hugon J, et al. [Tropical African neuromyelopathies: 61 studied cases in the Ivory Coast]. Rev Neurol (Paris). 1988. 144(10):578-85. [Medline].

  23. Guerreiro JB, Santos SB, Morgan DJ, et al. Levels of serum chemokines discriminate clinical myelopathy associated with human T lymphotropic virus type 1 (HTLV-1)/tropical spastic paraparesis (HAM/TSP) disease from HTLV-1 carrier state. Clin Exp Immunol. 2006 Aug. 145(2):296-301. [Medline].

  24. Hu CY, Lin MT, Yang YC, et al. Familial transmission of human T-lymphotropic virus type 1 (HTLV-1) in patients with adult T-cell leukemia/lymphoma or HTLV-1-associated myelopathy. J Formos Med Assoc. 1998 Feb. 97(2):101-5. [Medline].

  25. Leite AC, Mendonca GA, Serpa MJ, et al. Neurological manifestations in HTLV-I-infected blood donors. J Neurol Sci. 2003 Oct 15. 214(1-2):49-56. [Medline].

  26. Leite AC, Silva MT, Alamy AH, et al. Peripheral neuropathy in HTLV-I infected individuals without tropical spastic paraparesis/HTLV-I-associated myelopathy. J Neurol. 2004 Jul. 251(7):877-81. [Medline].

  27. Leon FE, Costa CM, Gaffga N. Discrepancy, coincidence or evidence in chronic idiopathic spastic paraparesis throughout the world. A meta-analysis on 2811 patients. Arq Neuropsiquiatr. 1997 Sep. 55(3B):530-5.

  28. Lezin A, Olindo S, Oliere S, et al. Human T lymphotropic virus type I (HTLV-I) proviral load in cerebrospinal fluid: a new criterion for the diagnosis of HTLV-I-associated myelopathy/tropical spastic paraparesis?. J Infect Dis. 2005 Jun 1. 191(11):1830-4. [Medline].

  29. Longe AC. Tropical myeloneuropathies: Clinical and electrophysiological findings in the Niger Delta area of Nigeria. East African Medical Journal. 1988. 65(9):614-620. [Medline].

  30. Manns A, Hisada M, La Grenade L. Human T-lymphotropic virus type I infection. Lancet. 1999 Jun 5. 353(9168):1951-8. [Medline].

  31. Matsuoka E, Takenouchi N, Hashimoto K, et al. Perivascular T cells are infected with HTLV-I in the spinal cord lesions with HTLV-I-associated myelopathy/tropical spastic paraparesis: double staining of immunohistochemistry and polymerase chain reaction in situ hybridization. Acta Neuropathol (Berl). 1998 Oct. 96(4):340-6. [Medline].

  32. Montanheiro PA, Montanheito PA, Oliveira AC, et al. Human T-cell lymphotropic virus type I (HTLV-I) proviral DNA viral load among asymptomatic patients and patients with HTLV-I-associated myelopathy/tropical spastic paraparesis. Braz J Med Biol Res. 2005 Nov. 38(11):1643-7. [Medline].

  33. Morgan OS, Montgomery RD, Rodgers-Johnson P. The myeloneuropathies of Jamaica: An unfolding story. Quarterly Journal of Medicine. 1988. New Series 67, No 252:273-281. [Medline].

  34. Nagai M, Usuku K, Matsumoto W, et al. Analysis of HTLV-I proviral load in 202 HAM/TSP patients and 243 asymptomatic HTLV-I carriers: high proviral load strongly predisposes to HAM/TSP. J Neurovirol. 1998 Dec. 4(6):586-93. [Medline].

  35. Nakamura T. Immunopathogenesis of HTLV-I-associated myelopathy/tropical spastic paraparesis. Ann Med. 2000 Dec. 32(9):600-7. [Medline].

  36. Olindo S, Cabre P, Lézin A, Merle H, Saint-Vil M, Signate A, et al. Natural history of human T-lymphotropic virus 1-associated myelopathy: a 14-year follow-up study. Arch Neurol. 2006 Nov. 63(11):1560-6. [Medline].

  37. Oliveira JT, Carneiro-Proietti AB, Lima-Martins MV, et al. Erectile insufficiency as first symptom of HTLV I/II associated myelopathy. Case report. Arq Neuropsiquiatr. 1998 Mar. 56(1):123-5. [Medline].

  38. Rodgers-Johnson PE, Garruto RM, Gajdusek DC. Tropical myeloneuropathies-a new aetiology. Trends Neurosci. 1988. 11, No. 12:526-532. [Medline].

  39. Roman GC. Tropical myelopathies and myeloneuropathies. Bulletin Pan Am Am Health. 1987. 21(3):293-305. [Medline].

  40. Roman GC, Spencer PS, Shoenberg BS. Tropical myeloneuropathies: the hidden endemias. Neurology. 1985. 35:1158-1170. [Medline].

  41. Saito M, Eiraku N, Usuku K, et al. ApaI polymorphism of vitamin D receptor gene is associated with susceptibility to HTLV-1-associated myelopathy/tropical spastic paraparesis in HTLV-1 infected individuals. J Neurol Sci. 2005 May 15. 232(1-2):29-35. [Medline].

  42. Shimazaki R, Ueyama H, Mori T, et al. Chronic sensory neuronopathy associated with human T-cell lymphotropic virus type I infection. J Neurol Sci. 2002 Feb 15. 194(1):55-8. [Medline].

  43. Smikle MF, Barton EN, Morgan OC, et al. The significance of immune disorder in tropical spastic paraparesis. Hum Antibodies. 1999. 9(2):133-7. [Medline].

  44. St. Clair Morgan O. The myeloneuropathies of Jamaica. Molecular Neurobiology. 1994. 8:149-153. [Medline].

  45. Taylor GP, Tosswill JH, Matutes E, et al. Prospective study of HTLV-I infection in an initially asymptomatic cohort. J Acquir Immune Defic Syndr. 1999 Sep 1. 22(1):92-100. [Medline].

  46. Touze E, Gessain A, Lyon-Caen O, Gout O. Tropical spastic paraparesis/HTLV-I-associated myelopathy in Europe and in Africa: clinical and epidemiologic aspects. J Acquir Immune Defic Syndr Hum Retrovirol. 1996. 13 Suppl 1:S38-45. [Medline].

  47. van der Ryst E, Smith MS, Visagie HM. Comparison of the polymerase chain reaction and serology for the diagnosis of HTLV-I infection. J Infect. 1996 Mar. 32(2):109-12. [Medline].

  48. Vieira Filho JPB, Oliveira ASB, Da Silva MRD, et al. Polineuropatia nutricional entre indos Xavantes. Rev Ass Med Brasil. 1997. 43(1):82-88. [Medline].

  49. Watanabe T. HTLV-1-associated diseases. Int J Hematol. 1997 Oct. 66(3):257-78. [Medline].

  50. Yata S, Ogawa T, Sugihara S, et al. HTLV-I carrier with unusual brain MR imaging findings. Neuroradiology. 2004 Sep. 46(9):755-8. [Medline].

  51. Zaninovic V. On the etiology of tropical spastic paraparesis and human T-cell lymphotropic virus-I-associated myelopathy. Int J Infect Dis. 1999. 3(3):168-76. [Medline].

Previous
Next
 
Light microscopy of thoracic spinal cord of 2 patients with HTLV-1–associated myelopathy (Klüver-Barrera staining). (Source: Aye et al, 2000, Fig. 1.)
Light microscopy of perivascular inflammatory infiltration in the spinal cord (A, C) and in the brain (B, D) (A, B H&E; C, D Elastica Van Gieson; A, C x400; B, D x200). (Source: Aye et al, 2000, Fig. 2.)
Light microscopy of the middle thoracic spinal cord (A, C, E) and subcortical white matter of the brain (B, D, F). Fibrotic changes are seen even in the capillaries (arrows) (A, B, F H&E; C-E Elastica van Gieson; A, C, D, F x400; B x300; E x100). (Source: Aye et al, 2000, Fig. 3.)
Immunostaining of the infiltrating cells in the thoracic spinal cord (A, C, E) and subcortical white matter of the brain (B, D, F) (A, B UCHL-1 [antibody to CD45RO]; C, D CD8; E, F OPD-4; A-F x150). (Source: Aye et al, 2000, Fig. 4.)
Immunostaining of the infiltrating cells in the thoracic spinal cord (A, C) and subcortical white matter of brain (B, D) (A, B UCHL-1[antibody to CD45RO]; C, D CD8; A-D x160). (Source: Aye et al, 2000, Fig. 5.)
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.