systemic lupus erythematosus ( sle ) is a rare connective disease affecting 6 - 19 cases per 100 000 children . the neurological manifestations are seen in 25 - 95% of patients with sle more commonly in the form of headache , psychosis , or cognitive dysfunction . in up to 1 - 2% of patients with sle it may be complicated by transverse myelitis but rarely acute transverse myelitis may be the initial manifestation of sle . we present one such case where atm was the initial and only manifestation of sle . 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 . 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 . 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 . blood 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  10/l . mri spine [ figure 1 ] showed multi - focal multi - regional transverse myelitis involving spinal cord from c5 down to the conus . cerebrospinal fluid ( csf ) showed elevated white cell count ( wcc ) of 1570  10/l with predominant polymorphs and elevated protein of 0.78 all the cultures including blood , csf , and urine were reported as no growth . 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 . t2-weighted sagittal image of the cervico - thoracic spine demonstrating enlargement of the spinal cord below c4 with high signal within it a 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 . the lab markers were suggestive of sle but with no convincing clinical features to correlate . based on the overall clinical picture and immunology markers , she was commenced on iv cyclophosphamide which was continued for seven cycles . 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 . she 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 [ figure 2 ] 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 , t2-weighted sagittal image of the cervico - thoracic cord demonstrating complete resolution of previous inflammatory changes in the spinal cord atm is seen in 1 - 2% of sle patients , but in one adult study it is reported to be a presenting feature in up to 39% of sle patients . most patients who develop complications of atm do so within 5 years of sle diagnosis . atm in sle may present with the classical picture of motor weakness , sensory disturbance , and sphincter disturbances . our patient presented with all the classical symptoms of transverse myelitis but with no clinical signs or symptoms suggestive of sle . the diagnosis of sle in our patient was made based on positive immunological markers , lymphopenia , and unusual clinical presentation on of longitudinal myelitis . the pathogenesis of atm in sle is unclear but most likely the process is immune complex mediated vasculitis or thrombosis leading to ischemic spinal lesions or anti - phospholipid antibodies cross - reacting with spinal cord phospholipids . in those cases where atm is the presenting feature of sle , many may not fulfil the criteria for the diagnosis of sle but over the course of the disease they may eventually present with other signs and symptoms of sle . in our reported case although the ana and double - stranded dna antibodies are positive , the patient has not shown any other clinical features of sle . it is likely that the clinical course has been altered with on - going immunosuppression . the auto - antibody screen may show antibodies positive suggesting sle but with no clinical features to correlate . the diagnosis of sle is made after excluding other possible causes of atm and with lab data suggesting sle . mri is very useful as it shows the extent of involvement and also helps rule out other causes of myelopathies . a majority of them are positive for anti - phospholipid syndrome , although it is not very clear whether those with apls do worse in terms of long term recovery . our patient presented with neurological signs suggestive of acute longitudinal myelitis but no clinical markers which would suggest sle . she fulfilled the acr criteria for 3 out of 11 criteria but immunological markers were highly suggestive of sle . the involvement may be limited to one particular segment or involve a long length of the spinal cord . our patient had almost complete involvement of spinal cord from c5 to conus with a small area near t10 being spared . there have been reports that in those where more than four segments are involved , a high proportion of them have varying degree of disability after treatment . they also may show greater degree of inflammation in csf and sensory involvement is more frequent . mri scan done 16 months after presentation showed complete resolution of t2 changes in the spinal cord . the general approach to treatment includes steroids in the form of iv pulse methylprednisolone and immunosuppressants such as cyclophosphamide . the role of plasmapharesis is not clear although it has been used in some patients . in general , in those patients with transverse myelitis , 1/3 recover completely , 1/3 recover partially but 1/3 are left with severe disabilities . those with hyper acute symptoms at onset , positive ana , or those caused by connective disease have poor outcome . acute longitudinal myelitis can be a devastating condition as it limits one 's quality of life . atm secondary to sle can carry a poor prognosis despite new treatment approaches and the treatment needs more than just steroids . early detection and aggressive treatment can prevent long - term permanent damage and may even have a favorable outcome . this report emphasizes the need for multi - center trials and the creation of a registry for sle patients with atm to help study optimal management options .
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 .