Longitudinally Extensive Transverse Myelitis in a Lupus-Neuromyelitis Optica Overlap.

Yonit Tavor,Galia Ronen,Alexandra Balbir-Gurman,Moshe Herskovitz

doi:10.5041/rmmj.10429

Abstract

Transverse myelitis is an inflammatory lesion of the spinal cord, occurring in different autoimmune, infectious, and traumatic diseases but is the hallmark of neuromyelitis optica (NMO), a rare neurologic autoimmune disease. Patients with systemic lupus erythematosus (SLE) may develop transverse myelitis as a neuropsychiatric complication of active disease; however, at times, NMO co-exists as an additional primary autoimmune condition in a SLE patient. Correct diagnosis of a SLE–NMO overlap is important not only for the different disease course and prognosis compared with SLE-related LETM, but especially for the emerging and highly specific NMO treatment options, not established for SLE-related LETM—such as anti-aquaporin 4 antibodies, anti-VEGF antibodies, complement modulation, or IVIg.

Highlights

Transverse myelitis is an inflammatory lesion of the spinal cord, causing significant morbidity and disability
We present a case of relapsing neuromyelitis optica (NMO) in a patient with systemic lupus erythematosus (SLE) and review the literature
Patients with longitudinally extensive transverse myelitis (LETM) represent a particular subgroup of transverse myelitis, different from those with shorter lesions; they have a low risk of evolution towards multiple sclerosis, but more severe clinical symptoms.[1]

Summary

INTRODUCTION

Transverse myelitis is an inflammatory lesion of the spinal cord, causing significant morbidity and disability. Spinal MRI revealed a longitudinal white matter lesion extending from D7 to D11 with a high signal on T2 images compatible with myelitis (Figure 1A) She was treated with i.v. pulses of methylprednisolone, and plasma exchange; induction treatment with monthly 1 g i.v. cyclophosphamide (CYC) infusions was introduced. Every attempt to space between IVIg pulses resulted in another exacerbation Another episode of severe para-paresis, urinary retention, and sensory level at D8 developed soon after discontinuation of treatment by the patient (due to incompliance) but responded well to an additional course of methylprednisolone, plasma exchange, and reconstitution of azathioprine and IVIg. Neurological examination demonstrated a D7 sensory level. The patient’s condition stabilized, and she remained with minimal left-hand paresis and mild spinal ataxia and sensory loss, with improvement visible on repeat MRI (C)

Findings

DISCUSSION

SUMMARY