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CD8 and CD4 T cell-mediated polymyositis complicating the HTLV-1 associated myelopathy. Quantitative evaluation of
A J Waclawik1, R Fadic, B P Lotz
1Neuromuscular Unit, University of Wisconsin Medical School, Madison 53792, USA.
Introduction:
Inflammatory myopathy is a treatable cause of worsening in the spectrum of neurological conditions that may develop during the course of HTLV-1 infection.
Material And Methods:
To investigate the cause of subacute worsening in the strength of a 46-y-old black male with HTLV-1 associated myelopathy we performed electrodiagnostic examination and a muscle biopsy which was studied with histochemistry, immunocytochemistry and electron microscopy. Serial measurements of isometric muscle strength were performed during the course of corticosteroid treatment.
Results:
The muscle biopsy showed evidence of denervation atrophy and prominent inflammatory changes with autoaggressive features. Lymphocyte typing showed a predominance of CD8+ T cells. The patient had sustained, marked improvement in strength, especially of the upper extremities, with oral, high single-dose, alternate-day prednisone therapy.
Conclusion:
A muscle biopsy should be considered in all patients with HTLV-1 associated weakness, especially when electromyography indicates possible coexisting primary muscle involvement and/or serum creatine kinase levels are elevated. HTLV-1-associated polymyositis can be successfully treated with corticosteroids.
Insights
Human T-lymphotropic virus type 1 (HTLV-1) associated polymyositis, a treatable neurological condition, can cause significant muscle weakness. Corticosteroid treatment led to marked strength improvement in a patient with HTLV-1 associated myelopathy.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Human T-lymphotropic virus type 1 (HTLV-1) infection can lead to a spectrum of neurological complications, including inflammatory myopathy.
- Inflammatory myopathy presents as a treatable cause of worsening neurological function in HTLV-1 patients.
Observation:
- A 46-year-old male with HTLV-1 associated myelopathy experienced subacute worsening of muscle strength.
- Diagnostic evaluations included electrodiagnostic examination and muscle biopsy with advanced microscopic analysis.
- Serial isometric muscle strength measurements were conducted during corticosteroid treatment.
Findings:
- Muscle biopsy revealed denervation atrophy and significant inflammatory changes with autoaggressive features, characterized by a predominance of CD8+ T cells.
- The patient demonstrated sustained and marked improvement in muscle strength, particularly in the upper extremities, following treatment with oral, high single-dose, alternate-day prednisone.
Implications:
- Muscle biopsy is recommended for HTLV-1 patients with weakness, especially if electromyography suggests primary muscle involvement or creatine kinase levels are elevated.
- Corticosteroid therapy is an effective treatment for HTLV-1-associated polymyositis, leading to significant functional recovery.