Author Of 1 Presentation
P0733 - Neuromyelitis Optica Spectrum Disorder with Interstitial Pneumonia as the First Episode: A Case Report (ID 1124)
Abstract
Background
Neuromyelitis Optica Spectrum Disorder (NMOSD) describes a range of immune-mediated inflammatory conditions typically presenting as recurrent episodes of optic neuritis and myelitis. The main pathogenic factor is the presence of immunoglobulin (Ig)G antibodies against the water channel protein aquaporin4 (AQP4) in the blood and subsequently in the central nervous system (CNS). Although, initially, it was considered that AQP4-IgG particularly targets the CNS, recently few cases with concurrent manifestations from other peripheral organs have been reported.
Objectives
Herein, we describe a rare case of NMO with atypical first manifestation of pneumonia and multisystem involvement including skeletal muscles, blood system and kidneys, additionally to the CNS.
Methods
A 77-year-old female was initially admitted to the pulmonology department where she was investigated for interstitial pneumonia. The patient underwent computed tomography that revealed abnormal shadows distributed in both lungs. Bronchoscopy and lung biopsies were non-diagnostic. During hospitalization she experienced a sudden onset of hiccups and vomiting. Simultaneously, numbness and muscle weakness of lower extremities were established. Magnetic resonance imaging (MRI) showed high-intensity signals at area postrema region and intramedullary at cervical and thoracic spinal cord on T2-weighted imaging, with gadolinium enhancement on T1-weighted imaging. Cerebrospinal fluid (CSF) examination revealed mild lymphocytosis, oligoclonal bands identical in CSF and serum, compatible with systematic inflammatory disease and positive AQP4-IgG antibodies. Based on the above findings the diagnosis of NMOSD was established. In the following days myalgia with high levels of creatine phosphokinase were noticed. Furthermore, acute renal failure and severe anemia occurred. A full panel of tests for autoimmune, viral and paraneoplastic disorders was carried out to exclude other pathological conditions. Positron emission tomography (PET) scan was without pathological findings.
Results
The patient received high doses of intravenous corticosteroids followed by oral prednisolone in long term with gradual improvement of her symptoms both on the CNS and the periphery.
Conclusions
This case highlights the possible involvement of tissues other than the CNS in AQP4-IgG positive patients. Clinicians should be aware of atypical manifestations of NMOSD outside the CNS, in cases where examinations are non-diagnostic and any other autoimmune or paraneoplastic disorder is excluded.