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Case Reports
. 2015 Feb 28:15:18.
doi: 10.1186/s12883-015-0275-7.

Clinical presentation of axial myopathy in two siblings with HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP)

Eiji Matsuura  1 , Akiko Yoshimura  2 , Satoshi Nozuma  3 , Itsuro Higuchi  4 , Ryuji Kubota  5 , Hiroshi Takashima  6
Affiliations
Case Reports

Clinical presentation of axial myopathy in two siblings with HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP)

Eiji Matsuura et al. BMC Neurol. .

Abstract

Background: The clinical features of myositis related with Human T-cell leukemia virus type 1 (HTLV-1) remains unclear despite epidemiological studies suggesting inflammatory myopathy associated with the virus.

Case presentation: Here, we described the clinical presentations, muscle biopsy studies and laboratory results of two siblings with HTLV-1-associated myelopathy / tropical spastic paraparesis (HAM/TSP) who were affected with lumbar lordosis. Computed tomography (CT) scans demonstrated marked paraspinal muscle atrophy in both patients. Immunohistochemical studies of biopsy tissue obtained from one of the patients revealed inflammatory change of the muscle. Upon oral prednisolone therapy, the patient showed improvement in muscle strength and serum creatine kinase (CK) level.

Conclusion: Myopathy or specifically axial myopathy should be considered as clinical symptom when treating the patients with HTLV-1 infection.

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Figures

Figure 1
Figure 1
Atrophy in paraspinal muscles of the siblings. (A-E) Computed tomography studies of 5 patients with HAM/TSP at several truncal levels (upper thoracic, lower thoracic, lumbar and sacral levels). (A, B, E) The degree of muscle atrophy of the control HAM/TSP patients was in accordance with the duration of HAM/TSP. (C, D) The two siblings showed extreme atrophy of the paraspinal muscles (arrow) in comparison to the iliopsoas muscles (IP). The difference in atrophy between iliopsoas and paraspinal muscle of the two siblings is conspicuous in comparison to case B with similar disease duration. (D) The gluteus medius and minimus (arrow head) were also selectively atrophied in the elder brother. PS, paraspinal muscles; IP, iliopsoas; MAX, gluteus maximus; MED, gluteus medius; MIN, gluteus minimus.
Figure 2
Figure 2
Muscle biopsy. Biopsy findings of the left deltoid muscle in Patient 1. (A) Hematoxylin and eosin staining. Mononuclear inflammatory cells were observed in the endomysium, perimysium and around the vessels. (B) Infiltrations of CD3-positive lymphocytes were occasionally observed in the endomysium. (C) An expression of HLA-ABC was markedly increased in the membrane of muscle fibers. (D-F) Sequential section of muscle tissue (D) A non-necrotic muscle fiber is surrounded by CD3+ lymphocytes. (E, F) The lymphocytic infiltrates were composed of CD4+ and CD8+ lymphocytes. Bar = 100 μm.
See this image and copyright information in PMC

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