Abstract
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect any organ, including the nervous system. Estimates of the incidence and prevalence of neurologic and psychiatric symptoms in SLE patients vary widely, largely due to heterogeneity in definition and methodology. In total, studies report that approximately one-third to one-half of SLE patients have neurologic or neuropsychiatric symptomatology. For many of the phenotypic manifestations of neuropsychiatric SLE, no biomarker or diagnostic test is specific enough to link the neurological diagnosis to SLE. Myelitis in SLE is a rare but morbid condition that occurs in approximately 1 to 2 percent of SLE patients in some cohorts. In this case report, we aimed to present the diagnosis and treatment of transverse myelitis in a patient who presented with acute flaccid paralysis in the lower extremity.
INTRODUCTION
Systemic lupus erythematosus (SLE) is a chronic autoimmune connective tissue disease of unknown etiology that is common in women of childbearing age and can affect almost every organ of the body. Acute transverse myelitis (TM) is a rare but serious acquired neuro-immune spinal cord disorder that may present with sudden loss of muscle strength, sensory changes and bowel or bladder dysfunction. It is observed in 1-2% of SLE patients (1-5). Currently, only a very limited number of cases of TM-associated SLE have been reported. A 2010 epidemiologic study showed that the incidence of TM at 3.6 per 100.000 (6). The pathogenesis is largely unknown and the clinical presentation is variable. There are still no established guidelines for diagnosis, management or monitoring and the role of autoantibodies is controversial. Lesions often involve the thoracic medulla and the primary clinical manifestations include paralysis of the limbs below the lesion site, conduction bundle sensory disturbance and dysphoria, and voiding dysfunction (7) In this case report, we aimed to present the diagnosis and treatment of TM in a patient who presented with acute flaccid paralysis in the lower extremity.
CASE REPORT
A 27-year-old woman with SLE since the age of 20 years, married, with one child, presented to the emergency department with the complaint of loss of strength in the legs. Two days ago, antibiotics were started by the dentist due to toothache and the patient discontinued the medication. Sudden loss of strength in the lower extremities develops after a stress. On examination, finger flexion muscle was 3/5 in both hands. Upper extremity deep tondon reflexes were normal and no pathological reflex was found. Deep tendon reflexes of the patient with flaccid paralysis in the lower extremity could not be obtained, but no pathological reflex was detected. The patient was taken over by neurology. No abnormality was detected on brain imaging and increased intensity at the C5-T2 level on spinal magnetic resonance imaging (MRI) was interpreted in favour of myelopathy (Figure 1). No abnormality was found in cerebrospinal fluid (CSF) analysis. Laboratory tests revealed white blood count: 7.6/mm3 (NE# 11.5/mm3), hemoglobin 11.4 g/dL, normal platelet count, erythrocyte sedimentation rate 40 mm/hour, C-reactive protein level of 53 mg/dL, anti nuclear antibody homogenous 3+ and anti-ribosomal P and anti-histone antibody positivity in the extractable nuclear antigen profile. Anti-neutrophil cytoplasmic anti-body immune florescence, antiPR3, anti-myeloperoxidase Enzyme-Linked Immunosorbent Assay test were negative, anti-dsDNA was positive and C3-C4 was low. In addition, neuromyelitis optica IgG was negative. Liver and renal function tests were within normal limits. The patient was treated with 1 g/day methylprednisolone (MP) and 120 g intravenous immunoglobulin (IVIG) followed by 1000 mg cyclophosphamide (CTX). Plasma Exchange (PLEX) was performed in the patient in whom no significant clinical change was observed. The patient is in the 3rd month of treatment and started to walk with person-assisted walking with rehabilitation.
CONCLUSION
TM is one of the neuropsychiatric involvements associated with SLE (NPSLE). Patients present with acute to subacute paraparesis or quadriparesis, usually bilateral but not always symmetrical; sensory impairment, which may be localised at the level of spinal sensation; and/or impaired bowel or bladder function. In SLE-associated myelitis, MRI often shows T2 hyperintensity in the affected area of the spinal cord. CSF examination may show a pleocytosis, usually lymphocytic. The outcome of CSF in SLE patients with TM is currently controversial because some studies have shown that CSF is normal in SLE patients with TM. Treatment is started with pulse glucocorticoids. CTX and Rituximab (RTX) are first-line agents in TM. Plasmapheresis can be applied simultaneously. CTX treatment is usually continued for three to six months and then switched to a less toxic agent for maintenance therapy such as mycophenolate, azathioprine or rituximab to control SLE disease activity and reduce the risk of relapse (8-10). Early aggressive treatment, especially for initially severe myelitis, may be crucial to achieve a favorable outcome. Recently, MP pulse therapy in combination with CTX has been recommended for NPSLE. RTX, a promising modality, has also been reported to be effective in the treatment of new-onset SLE-associated TM (11) and the positive benefits of PLEX and IVIG in patients with refractory NPSLE have also been recognized (12). In recent clinical trials, anti-interleukin 6 agents (tocilizumab and satralizumab) have also shown efficacy in preventing TM attacks (13). Only 40-50% of NPSLE occurs in the presence of active disease in other organ systems of SLE (14). SLE-associated TM can also develop without active disease in other organ systems (15). Therefore, disease activity factors may not predict a worse prognosis. A literature review has shown that recurrence of myelitis will occur several months after the first event, and recurrence occurs at least once in 21-55% of patients (16). Due to its poor prognosis, SLE-TM brings emotional stress and economic burden to the patient’s family and society. In the future, there is a need for controlled clinical treatment studies, which can especially inspire the management of this disease.
