Syringohydromyelia or HTLV-I-associated myelopathy/tropical spastic paraparesis: a diagnostic challenge (case report)

Araújo, Abelardo de Queiroz Campos; Leite, Ana Cláudia Celestino Bezerra; Oliveira, André Luiz dos Anjos de; Afonso, Carmelinda Monteiro Costa; Pies, Juliana

doi:10.1590/s0004-282x1999000200019

Cited by 2 publications

(2 citation statements)

References 8 publications

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“…This pattern of lesion distribution supports the hypothesis of a multifocal degenerative process in the pathogenesis of the disease [18,40,41]. Moreover, MRI of the spinal cord is extremely valuable to rule out other conditions that resemble HAM/ TSP, particularly in HTLV-1 endemic areas [42].…”

Section: Discussionsupporting

confidence: 74%

Human T Cell Lymphotropic Virus Type 1-Associated Myelopathy in São Paulo, Brazil

et al. 2002

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We report epidemiologic and clinical features of human T cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis in a Brazilian cohort of 86 patients from a university hospital. Cerebrospinal fluid (CSF) abnormalities and magnetic resonance imaging (MRI) findings were correlated with neurologic signs and symptoms. The patients’ mean age at disease onset was 43.2 years and the female to male ratio 1.5:1. Risk factors for retroviral exposure included blood transfusions, sexual transmission and intravenous drug use. Neurologic manifestations consisted mostly of spastic paraparesis, of gradual and asymmetric onset, with increased deep-tendon reflexes. Disability was associated with disease duration. CSF findings included mild mononuclear pleocytosis and elevated protein levels. MRI showed hemispheric areas of high signal intensity in 32.7% and spinal cord atrophy in 5.6% of cases, particularly in patients with longer duration of disease. A high frequency of past blood transfusion (32.6%) suggests that blood-borne infection was an important means of HTLV infection before implementation of screening of blood donors in Brazil.

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Section: Discussionsupporting

confidence: 74%

Human T Cell Lymphotropic Virus Type 1-Associated Myelopathy in São Paulo, Brazil

et al. 2002

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“…All over the world, many patients must endure a long struggle with mysterious symptoms before they are finally given a correct diagnosis, causing psychological strain and perhaps lowering their chances of managing the disease effectively [ 35 ]. Experts recommend that all spastic paraparesis patients in or near endemic areas be screened for HTLV-1 to ameliorate this problem [ 36 , 37 ].…”

Section: Discussionmentioning

confidence: 99%

Nation-wide epidemiological study of Japanese patients with rare viral myelopathy using novel registration system (HAM-net)

Coler‐Reilly

Yagishita

Suzuki

et al. 2016

Orphanet J Rare Dis

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BackgroundAt least one million people are infected with human T-lymphotropic virus type 1 (HTLV-1) in Japan, a small percentage of whom develop HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) or adult T-cell leukemia/lymphoma (ATLL). Patients with HAM/TSP suffer from progressively worsening myelopathic symptoms, such as motor disability and bladder dysfunction, and may become wheelchair-bound or even bedridden.MethodsTo learn more about this rare, debilitating disease, we established the national registration system “HAM-net” in March 2012. We continuously obtain detailed data from enrolled patients using the registration forms and an annual telephone interview. In this retrospective study, we describe the demographics and clinical histories of 383 registered patients from all over Japan.ResultsPatients were diagnosed at a median of 53 years old, long after disease onset at 45. Most (55.3 %) were originally from the southernmost regions, Kyushu and Okinawa. The main initial symptoms were difficulty walking (81.9 %), urinary dysfunction (38.5 %), and lower limb sensory disturbances (13.9 %). Many patients reported frequent leg numbness and leg pain, and the vast majority required medical intervention for urinary symptoms and constipation. A median of 8 years elapsed from the onset of motor symptoms to Osame Motor Disability Score (OMDS) 5 (requiring unilateral support), 12.5 years to OMDS 6 (requiring bilateral support), and 18 years to OMDS 9 (unable to walk). Health Assessment Questionnaire - Disability Index (HAQ-DI) tasks related to mobility, as opposed to hand motions, were very difficult for HAM/TSP patients and well-correlated with OMDS. Scores on the MOS 36-Item Short-Form Health Survey (SF-36) indicated that physical functioning was severely impaired in HAM/TSP patients. Patients with a history of blood transfusion (19.1 %) were older and suffered from more severe disability as indicated by their high HAQ-DI scores. Patients with a family history of HAM/TSP (8.4 %) were younger and had relatively mild symptoms given their long disease durations; many (15.6 %) also had a relative with ATLL.ConclusionsThe HAM-net national registration system has been an effective tool for gathering personal and clinical data from HAM/TSP patients scattered throughout Japan. We expect to conduct many retrospective and prospective epidemiological studies using HAM-net in the future.

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Syringohydromyelia or HTLV-I-associated myelopathy/tropical spastic paraparesis: a diagnostic challenge (case report)

Cited by 2 publications

References 8 publications

Human T Cell Lymphotropic Virus Type 1-Associated Myelopathy in São Paulo, Brazil

Human T Cell Lymphotropic Virus Type 1-Associated Myelopathy in São Paulo, Brazil

Nation-wide epidemiological study of Japanese patients with rare viral myelopathy using novel registration system (HAM-net)

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