Fatal Enterovirus-related Myocarditis in a Patient with Devic’s Syndrome Treated with Rituximab

Enteroviruses are a frequent source of infection and among the most common central nervous system viral pathogens. Enteroviruses – in particular, the Coxsackie B viruses – are a known cause of myocarditis. Rituximab is a genetically engineered chimeric anti-CD20 monoclonal antibody. Many reports in the literature suggest a higher risk of infection following repeated rituximab therapy, including viral infection. However, observations of enterovirus-related myocarditis in the context of rituximab treatment are scarce. The authors describe the case of a patient with neuromyelitis optica spectrum disorder who developed severe and fatal enterovirus-related myocarditis after rituximab therapy with a difficult differential diagnosis of autoimmune or giant-cell myocarditis. This case highlights the importance of complete diagnostic workup in difficult cases of myocarditis, including endomyocardial biopsies.

Rehabilitation in Devica’s Syndrome. Case Report

Introduction: Devic’s syndrome also known as neuromyelitis optica is an autoimmune disease of central nervous system (CNS).It has an inflammatory, demyelizatory, chronic and relapsing nature, where ones own immune system attacks the spinal cord and optic nerves. Material and Methods: The authors presented a case report of a 54-year-old woman diagnosed with Devic’s syndrome 2 years ago. The physiotherapeutic examination revealed: visual and balance disturbances, decrease in muscle strength of upper and lower limbs, unsteady and shaky gait with a need to use a zimmer frame. At the start and at the end of a therapy, to objectively monitor the progress of rehabilitation treatment, a number of functional tests were used that included: Barthel Index, Brunnstrom’s test, Rankin Scale, Ashworth’s scale, Lovett’s test and a timed 20m walk. In physiotherapeutic treatment process patterns and techniques of PNF (prioprioceptive neuromuscular facilitation) ware used as well as exercise to correct muscles tone and strengthening exercise of trunk and lower limbs. Results: As a result of applying a comprehensive rehabilitation approach an increase of muscles strength of upper and lower limbs was obtained (Lovett’s scale R/L: shoulder 5/5, elbow 5/5, hand 5/5, hip 4+/4+, knee 4+/4+, foot 4+/4+). In addition all postural muscles gained in strength and an improvement in hands dexterity, body coordination and balance was noticed. Conclusion: Multidisciplinary approach and an individually selected rehabilitation program proves to show beneficial effects in a treatment process of patients with Devic’s syndrome.

Systemic lupus erythematosus with the development of neuromyelitis optica (Devic's syndrome) is a rare combination of autoimmune diseases

Neuromyelitis optica ((NMO), Devic's syndrome) is an immune-mediated inflammatory demyelinating disease characterized by transverse myelitis and optic neuritis. Determination of the level of antibodies to aquaporin 4 (NMO-IgG) is presently one of the key methods for the diagnosis and assessment of the activity of ONM, which allows this disease to be differentiated from multiple sclerosis and other demyelinating CNS lesions. ONM can occur not only as an independent disease, but also as a syndrome in different systemic diseases, such as: systemic lupus erythematosus (SLE), antineutrophilic cytoplasmic antibody-associated vasculitides, Sjögren's disease, etc. (up to 50–70%). In such situations, the clinician is always confronted with a question as whether the patient can have two rare autoimmune diseases or develop ONM as a systemic manifestation of rheumatic disease.The paper describes a clinical case of a young female patient with SLE concurrent with a CNS lesion, the manifestations of which corresponded to ONM. The patient had focal changes in the substance of the brain and spinal cord, as evidenced by magnetic resonance imaging, as well as high NMO-IgG titers. The development of ONM worsens SLE prognosis and requires active immunosuppressive therapy. The patient received three plasmapheresis sessions, ultrahigh-dose glucocorticoid and cyclophosphamide therapy, followed by replacement with azathioprine, causing a stable clinical and laboratory disease remission to be achieved.