Aanchal Arora¹*, Manasa Mudalagiri², Bhavna Kaul³ and YC Porwal⁴

¹Senior Resident, Department of Medicine, VMMC and Safdarjung Hospital, New Delhi, India
²Junior Resident, Department of Medicine, VMMC and Safdarjung Hospital, New Delhi, India
³Consultant and Head, Department of Neurology, VMMC and Safdarjung Hospital, New Delhi, India
⁴Consultant and Professor, Department of Medicine, VMMC and Safdarjung Hospital, New Delhi, India

*Corresponding Author: Aanchal Arora, Senior Resident, Department of Medicine, VMMC and Safdarjung Hospital, New Delhi, India.

Received: March 14, 2018; Published: April 25, 2018

Citation: Aanchal Arora., et al. “Sjogren’s Syndrome Presenting as Longitudinally Extensive Transverse Myelitis”. Acta Scientific Medical Sciences 2.2 (2018).

Abstract

  Longitudinal extensive transverse myelitis (LETM) is defined as a spinal cord lesion that spans three or more contiguous vertebral segments in length. We present the case of a 30 year old woman who presented with weakness and dysaesthesias of both lower limbs along with urinary retention and constipation. She had history of joint pains, dry eyes and mouth for the past 3 years. Paraplegia with hyperreflexia and an extensor plantar reflex was evident on neurological examination. Cerebrospinal fluid was acellular, with mildly raised protein (60 mg/dl) and sugar of 40 mg/dl. MRI of the spinal cord revealed longitudinally extensive diffuse hyperintensities extending from C4 to conus medullaris. MRI of the brain was normal. Visual evoked potential (VEP) was abnormal. Antibodies for neuromyelitis optica (anti-aquaporin-4 antibody) were negative. Patient turned out to be positive for antinuclear antibodies and anti SS-A antibodies. Schirmer’s test was positive and patient was diagnosed as a case of Sjogren’s syndrome with LETM. The patient was given corticosteroids and intravenous immunoglobulin (IVIg) following which she showed partial recovery. LETM is a characteristic feature of neuromyelitis optica (NMO) but such spinal lesions can also occur in systemic autoimmune diseases like Sjogren’s syndrome.

Keywords: Longitudinal Extensive Transverse Myelitis; Sjogren’s Syndrome; Neuromyelitis Optica; NMO Spectrum Disorders

Copyright: © 2018 Aanchal Arora., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.