Acute longitudinal myelitis as the first presentation in child with systemic lupus erythematosus
A 13-year-old Polish girl, previously fit and well, presented with history of pain in the left leg for 2 weeks progressing to bilateral weakness of legs and sensory loss. She was febrile for 2 days prior to admission. She had constipation and urinary retention. There was no history of trauma, recent vaccination, cough, skin rash, joints pain, oral ulcers, or any other clinical symptoms or signs suggestive of SLE.\nOn admission to hospital she was afebrile with normal vital observations and blood pressure. Examination of her cardiovascular and respiratory system was unremarkable. Abdominal examination revealed distended abdomen as a result of constipation and urinary retention. Neurological examination suggested normal cranial nerve examination with no bulbar palsy. The motor power in the lower limb at presentation was 3/5 MRC with areflexia. The motor power was 5/5 MRC in the upper limbs with brisk tendon reflexes. There was sensory loss from T4 below. In the next 24-48 h the motor weakness increased with complete weakness in the lower limbs and power deteriorating to 3/5 in the upper limbs. She was diagnosed with acute longitudinal myelitis and started on intravenous-pulsed methylprednisolone for 5 days followed by oral prednisolone in tapering doses.\nBlood results at presentation showed normal biochemistry but elevated CRP of 42 mg/L.(normal range 0-10 mg/L) The complete blood count was normal except for low lymphocytes of 0.77 × 109/L. MRI spine [] showed multi-focal multi-regional transverse myelitis involving spinal cord from C5 down to the conus. Extensive investigations were carried to identify the underlying cause. Cerebrospinal fluid (CSF) showed elevated white cell count (WCC) of 1570 × 106/L with predominant polymorphs and elevated protein of 0.78 g/L. All the cultures including blood, CSF, and urine were reported as no growth. The virology screen was negative including Lyme's serology. Anti nuclear antibodies (ANA) was positive with the titres being 1: 1600 and showing a speckled pattern. At this point pediatric rheumatology opinion was sought to rule out an auto-immune condition or a connective tissue disease leading to transverse myelitis. Rheumatology evaluation did not reveal any other signs suggestive of SLE or any other connective tissue disease. She did not satisfy the Americal College of Rheumatology (ACR) criteria for diagnosis of SLE.\nA further auto-antibody screen revealed significantly elevated double-stranded DNA antibody 355 iu/mL. Anti-Sm antibody, anti-U1 antibody, and anti-RNP70 antibody were positive. C3 and C4 were low. Immunoglobulin profile showed elevated IgG and IgM levels. Cardiolipin antibody and lupus anticoagulant were negative. Aquaporin IgG antibody was negative.\nThe lab markers were suggestive of SLE but with no convincing clinical features to correlate. She was transferred to local tertiary pediatric rheumatology unit. Based on the overall clinical picture and immunology markers, she was commenced on IV cyclophosphamide which was continued for seven cycles. She also underwent three cycles of plasmapheresis as treatment for SLE with ATM. After completion of IV cyclophosphamide cycles, she was commenced on azathioprine, oral prednisolone 10 mg once daily along with hydroxychloroquine. Initially, she needed subcutaneous enoxaparin therapy which was changed to Aspirin once she started mobilizing.\nShe had neuro rehabilitation in the form of intensive physiotherapy, support from the occupational therapy. She received botox injection in the lower limbs for her spasticity. Over the following few months she made significant recovery. Her repeat MRI of spine [] done 16 months later has shown complete resolution of the inflammatory changes. She was able to walk with the use of crutches to move around and uses wheel chair only for long distances. She self-catheterizes her bladder and has normal bowel movements with intermittent need for Movicol. She has normal motor power in the upper limbs and she has normal sensations both in upper and lower limbs. She has re-integrated back at her normal mainstream school. After 18 months of treatment with immunosuppressants and oral steroids, she has not developed any other clinical features of SLE.