G234 Acute Transverse Myelitis as the Initial Manifestation of Systemic Lupus Erythematosus in a Child. (4th June 2013)

• Record Type: Journal Article
• Authors: Shivamurthy, V; Ganesan, S; Khan, A; Sridhar, A; Hussain, N
• Is Part Of: Archives of disease in childhood. Volume 98:Supplement 1(2013)
• Page Start: A104
• Page End: A104
• Publication Date: 2013-06-04
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2013-304107.246
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Introduction: Systemic Lupus Erthematosus (SLE) is a chronic autoimmune disease characterised by multisystem involvement. It is complicated by neurological manifestations in 25–95% of the patients. Acute transverse myelitis may be a complication in 1–2% of patients but in some it may even be the initial manifestation of SLE. Case report: 13 years old previously healthy girl was admitted with a history of pain in her lower limbs for 2 weeks, fever for 2 days and sensory loss. She developed acute flaccid quadriparesis over the next 48hrs, with bilateral weakness of her lower limbs, urinary retention and sensory loss from T2 spinal segment down for light touch and pain. Her vibratory and proprioception were absent in her lower limbs. Power in her upper limbs was reduced. She had no cranial nerve involvement and her ophthalmology assessment was unremarkable. She did not have bulbar involvement or respiratory difficulties. Investigations revealed raised inflammatory markers and CSF showed pleocytosis with an elevated protein. She didn't fulfil the ACR criteria for SLE but her blood results were consistent for an active SLE (ANA positive – speckled pattern, double stranded DNA, Anti Sm and Anti RNP antibody positive with low complement C3 and C4 level). Her cultures for blood, urine, CSF were negative as well as extensive virology screen and serology for Lyme's disease. MRI Spine showed high signal within the spinal cord from C5 down to the conus suggestive of multiregional multifocal transverse myelitis. Her MRI brain was normal. She was treated with 7 cycles of cyclophosphamide and methylprednisolone and plasmapharesis. She was wheelchair bound but has responded well to the treatment and is able to walk few steps without support, manage with crutches only to walk and only uses wheelchair occasionally. She doesn't have any upper limb problems at the moment although still has neuropathic bladder. Conclusion: It is important to include autoimmune screen as part of transverse myelitis work up as SLE may not present with typical clinical features at the outset. Early diagnosis and aggressive treatment of transverse myelitis is important in SLE to prevent long-term disability.