[Downloaded free from http://www.pediatricneurosciences.com on Friday, April 6, 2018, IP: 81.2.6.230] Case Report Acute longitudinal myelitis as the first presentation in child with systemic lupus erythematosus Vinay M. Shivamurthy, Subramanian Ganesan 1 , Arif Khan 1 , Nahin Hussain 1 , Arani V. Sridhar Departments of Paediatric Rheumatology, 1 Paediatric Neurology, Children’s Hospital, Leicester Royal Infjrmary, University Hospitals of Leicester, Leicester, UK Address for correspondence: Dr. Arani V. Sridhar, Children’s Hospital, Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Infirmary Square, Leicester - LE1 5WW, UK. E-mail: arani.sridhar@uhl-tr.nhs.uk ABSTRACT Systemic lupus erythematosus (SLE) is a multi‑system auto‑immune disorder that is characterized by widespread immune dysregulation, formation of auto–antibodies, and immune complexes, resulting in inflammation and potential damage to variety of organs. It is complicated by neurological manifestations in 25‑95% of the patients. Acute transverse myelitis (ATM) may be a complication in 1‑2% of patients with SLE but in some patients it may be the initial manifestation of SLE. This sub‑group of patients where ATM is the presenting feature may not fulfil the ACR criteria for the diagnosis of SLE which may delay the diagnosis and may affect the outcome. In those patients where the involvement is more than four segments of the spine are believed to have poor prognosis, but early diagnosis and treatment may alter the course and lead to a better outcome. We describe a young Polish girl where ATM was the initial manifestation of SLE involving almost the whole length of spine but she had a reasonably good outcome following early diagnosis and aggressive treatment. Key words: Acute transverse myelitis, paediatric systemic lupus erythematosus, Acute longitudinal myelitis Introduction Case report Systemic lupus erythematosus (SLE) is a rare connective A 13-year-old Polish girl, previously fit and well, presented disease affecting 6-19 cases per 100 000 children. The with history of pain in the left leg for 2 weeks progressing to neurological manifestations are seen in 25-95% of patients bilateral weakness of legs and sensory loss. She was febrile for with SLE more commonly in the form of headache, psychosis, 2 days prior to admission. She had constipation and urinary or cognitive dysfunction. [1,2] In up to 1-2% of patients with retention. There was no history of trauma, recent vaccination, SLE it may be complicated by transverse myelitis but rarely cough, skin rash, joints pain, oral ulcers, or any other clinical acute transverse myelitis may be the initial manifestation of symptoms or signs suggestive of SLE. SLE. We present one such case where ATM was the initial and only manifestation of SLE. On admission to hospital she was afebrile with normal vital observations and blood pressure. Examination of her cardiovascular and respiratory system was unremarkable. Access this article online Abdominal examination revealed distended abdomen as a Quick Response Code: result of constipation and urinary retention. Neurological Website: examination suggested normal cranial nerve examination www.pediatricneurosciences.com 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. DOI: 10.4103/1817-1745.117854 There was sensory loss from T4 below. In the next 24-48 h the motor weakness increased with complete weakness in 150 / Journal of Pediatric Neurosciences / Volume 8 / May-Aug / 2013
[Downloaded free from http://www.pediatricneurosciences.com on Friday, April 6, 2018, IP: 81.2.6.230] Shivamurthy, et al. : Acute longitudinal myelitis in paediatric SLE the lower limbs and power deteriorating to 3/5 in the upper as treatment for SLE with ATM. After completion of limbs. She was diagnosed with acute longitudinal myelitis and IV cyclophosphamide cycles, she was commenced on started on intravenous-pulsed methylprednisolone for 5 days azathioprine, oral prednisolone 10 mg once daily along with followed by oral prednisolone in tapering doses. hydroxychloroquine. Initially, she needed subcutaneous enoxaparin therapy which was changed to Aspirin once she Blood results at presentation showed normal biochemistry started mobilizing. but elevated CRP of 42 mg/L.(normal range 0-10 mg/L) The complete blood count was normal except for low lymphocytes She had neuro rehabilitation in the form of intensive of 0.77 × 10 9 /L. MRI spine [Figure 1] showed multi-focal physiotherapy, support from the occupational therapy. multi-regional transverse myelitis involving spinal cord from She received botox injection in the lower limbs for her C5 down to the conus. Extensive investigations were carried spasticity. Over the following few months she made to identify the underlying cause. Cerebrospinal fluid (CSF) significant recovery. Her repeat MRI of spine [Figure 2] showed elevated white cell count (WCC) of 1570 × 10 6 /L done 16 months later has shown complete resolution of the with predominant polymorphs and elevated protein of 0.78 inflammatory changes. She was able to walk with the use of g/L. All the cultures including blood, CSF, and urine were crutches to move around and uses wheel chair only for long reported as no growth. The virology screen was negative distances. She self-catheterizes her bladder and has normal including Lyme’s serology. Anti nuclear antibodies (ANA) bowel movements with intermittent need for Movicol. She was positive with the titres being 1: 1600 and showing a has normal motor power in the upper limbs and she has speckled pattern. At this point pediatric rheumatology normal sensations both in upper and lower limbs. She has opinion was sought to rule out an auto-immune condition re-integrated back at her normal mainstream school. After or a connective tissue disease leading to transverse myelitis. 18 months of treatment with immunosuppressants and oral Rheumatology evaluation did not reveal any other signs steroids, she has not developed any other clinical features suggestive of SLE or any other connective tissue disease. of SLE. She did not satisfy the Americal College of Rheumatology (ACR) criteria for diagnosis of SLE. Discussion A further auto-antibody screen revealed significantly elevated double-stranded DNA antibody 355 iu/mL. Anti-Sm SLE is an auto-immune disease affecting various organ antibody, anti-U1 antibody, and anti-RNP70 antibody were systems. It is complicated by neurological symptoms in positive. C3 and C4 were low. Immunoglobulin profile showed 25-95% of the cases. [1,2] The common symptoms are elevated IgG and IgM levels. Cardiolipin antibody and lupus headache, seizures, or psychosis. ATM is seen in 1-2% of anticoagulant were negative. Aquaporin IgG antibody was SLE patients, but in one adult study it is reported to be a negative. presenting feature in up to 39% of SLE patients. [1,3,4] Most patients who develop complications of ATM do so within 5 The lab markers were suggestive of SLE but with no convincing years of SLE diagnosis. ATM in SLE may present with the clinical features to correlate. She was transferred to local classical picture of motor weakness, sensory disturbance, and tertiary pediatric rheumatology unit. Based on the overall sphincter disturbances. Our patient presented with all the clinical picture and immunology markers, she was commenced classical symptoms of transverse myelitis but with no clinical on IV cyclophosphamide which was continued for seven signs or symptoms suggestive of SLE. The diagnosis of SLE cycles. She also underwent three cycles of plasmapheresis in our patient was made based on positive immunological Figure 1: T2-weighted sagittal image of the cervico-thoracic spine Figure 2: T2-weighted sagittal image of the cervico-thoracic cord demonstrating demonstrating enlargement of the spinal cord below C4 with high signal within it complete resolution of previous infmammatory changes in the spinal cord 2013 / May-Aug / Volume 8 / Journal of Pediatric Neurosciences / 151
Recommend
More recommend
