Introduction: Acute transverse myelitis (ATM) is a rare disorder (1-8 new cases per million of population per year), with 20% of all cases occurring in patients younger than 18years of age. Diagnosis requires clinical symptoms and evidence of inflammation within the spinal cord (cerebrospinal fluid and/or magnetic resonance imaging). ATM due to neuroborreliosis typically presents with impressive clinical manifestations. Case presentation: Here we present a case of Lyme neuroborreliosis-associated ATM with severe MRI and CSF findings, but surprisingly few clinical manifestations and late conversion of the immunoglobulin G CSF/blood index of Borrelia burgdorferi sensu lato. Conclusion: Clinical symptoms and signs of neuroborrelial ATM may be minimal, even in cases with severe involvement of the spine, as shown by imaging studies. The CSF/blood index can be negative in the early stages and does not exclude Lyme neuroborreliosis; if there is strong clinical suspicion of Lyme neuroborreliosis, appropriate treatment should be started and the CSF/blood index repeated to confirm the diagnosi

Aebi, C.

Bigi, S.

Bigler, S.

Nauer, C.

Steinlin, M.

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CASE REPORTAcute transverse myelitis in Lyme neuroborreliosisS. Bigi • C. Aebi • C. Nauer • S. Bigler •M. SteinlinReceived: 14 January 2010 / Accepted: 3 May 2010 / Published online: 27 May 2010 Urban & Vogel 2010AbstractIntroduction Acute transverse myelitis (ATM) is a raredisorder (1–8 new cases per million of population peryear), with 20% of all cases occurring in patients youngerthan 18 years of age. Diagnosis requires clinical symptomsand evidence of inflammation within the spinal cord(cerebrospinal fluid and/or magnetic resonance imaging).ATM due to neuroborreliosis typically presents withimpressive clinical manifestations.Case presentation Here we present a case of Lymeneuroborreliosis-associated ATM with severe MRI andCSF findings, but surprisingly few clinical manifestationsand late conversion of the immunoglobulin G CSF/bloodindex of Borrelia burgdorferi sensu lato.Conclusion Clinical symptoms and signs of neuroborre-lial ATM may be minimal, even in cases with severeinvolvement of the spine, as shown by imaging studies.The CSF/blood index can be negative in the early stagesand does not exclude Lyme neuroborreliosis; if there isstrong clinical suspicion of Lyme neuroborreliosis, appro-priate treatment should be started and the CSF/blood indexrepeated to confirm the diagnosis.Keywords Lyme  Neuroborreliosis  Transverse myelitisIntroductionAcute transverse myelitis (ATM) is an inflammatorymyelopathy with an incidence of 1–8 cases per million ofpopulation per year. Of all affected patients, 20% areyounger than 18 years of age [1]. Diagnosis is based onclinical symptoms, cerebral spinal fluid (CSF) findings andspinal neuroimaging results. In addition to the inflamma-tory myelopathies, demyelination, infection (e.g. poly-myelitic enteroviral infection) and other inflammatorydisorders (i.e. systemic lupus erythematodes, neurosar-coidosis) must be considered in the differential diagnosis[2, 3]. In adults, multiple sclerosis and neuromyelitis opticatogether with infectious/parainfectious aetiologies are themost frequent causes of ATM [4], while in children theaetiology is mainly postinfectious [5]. Clinical featuresconsist of sensory disturbances in most of the patients,followed by weakness and—especially in childhood—uri-nary tract dysfunction. Children suffer from more severeclinical impairment then adults. In the series of Pidcocket al., 89% of the paediatric patients were bed- or wheel-chair bound or required assisted ventilation, whereas themajority of adults showed only mild impairment [1, 6].Outcome is generally favourable. Chronic bladder dys-function has been shown to be the main sequelae [1].ATM due to Lyme neuroborreliosis has rarely beenreported. However, those cases that have been documented,the majority of patients presented with impressive clinicalS. Bigi (&)  M. SteinlinNeuropaediatrics, Department of Paediatrics,University Children’s Hospital, Inselspital,Bern, Switzerlande-mail: Sandra.bigi@insel.chC. AebiPaediatric Infectious Diseases, Department of Paediatrics,University Children’s Hospital, Inselspital, Bern, SwitzerlandC. NauerNeuroradiology, Department of Radiology, University Hospital,Inselspital, Bern, SwitzerlandS. BiglerInstitute of Infectious Diseases, University Hospital,Inselspital, Bern, SwitzerlandInfection (2010) 38:413–416DOI 10.1007/s15010-010-0028-xsymptoms [7–9]. Here, we report a case of ATM due toLyme neuroborreliosis, which—despite severe neuroim-aging (magnetic resonance imagining, MRI) and CSFfindings—was characterized by surprisingly few clinicalsigns.Case reportA 12.5-year-old boy presented to our emergency depart-ment in November with a [4-week history of increasingfrontal headache that got worse in the evening. He notedintermittent subfebrile temperatures. Additional complaintswere fatigue, diffuse non-radicular back/neck pain andnausea. There was no vomiting. In this period, a weightloss of 2–3 kg was documented. Three weeks beforeadmission he had been immunized (dTap = diphtheria,tetanus, acellular pertussis) according to the recommendedSwiss vaccination schedule. His personal history is unre-markable. There was no tick bite reported, and no an ery-thema migrans.On admission, the general physical condition of thepatient was slightly lower than would normally be expec-ted, he was afrebrile, and the results of a general clinicalexamination were normal. His neurological examinationwas remarkable only for nuchal rigidity. In particular, therewas no bladder dysfunction, and there was no sensorydisturbance or weakness.Laboratory investigations on admission included a nor-mal complete blood cell count, inflammatory markers(C-reactive protein), liver enzymes and kidney functionparameters.Because of the clinical constellation (neck stiffness in anafebrile patient), MRI of the brain was performed toexclude a haemorrhagic or space-occupying lesion. Thebrain scan was normal, but there were signal alterations(T2w hyperintensities) within the medulla down to cervicalspine. A consecutive MRI of the spinal cord was added tothe tests, which revealed a longitudinal signal alteration(T2w hyperintensities) from the pons to the conus, accen-tuated centromedullarily with a patchy enhancement(Fig. 1). The myelon was swollen, especially in the cer-vical region. There was no tumor or cerebral or meningealenhancement on the MRI scan.There was marked CSF pleocytosis (787cells/ll; 734mononuclear cells), elevated protein (6.24 g/l) anddecreased glucose (1.79 mmol/l), with a glucose CSF/plasma ratio of 0.28. Microbiological investigationrevealed increased serum immunoglobulin G (IgG) andIgM for Epstein–Barr virus (EBV) (EBV viral capsidantigen-IgM and -IgG, anti-early-antigen IgG p54) andBorrelia burgdorferi sensu lato. Serological testing forFig. 1 Left T2-weightedsagittal scan of thecervicothoracic spine. There isan extensive centromedullaryhyperintensity (arrow) andswelling as an expression of thespinal cord edema. RightT2-weighted axial scan at thelevel of the cervical spine.The edema involving the grayand white matter is discernible.The hyperintensity of the graymatter (arrow) is particularlyprominent414 S. Bigi et al.B. burgdorferi was performed using a recomWell enzyme-linked immunosorbent assay (ELISA; IgM and IgG;Mikrogen, Neuried, Germany) and confirmed by recomBlot(Mikrogen; IgM and IgG). The Western blot revealed thatthe patient had borrelia-specific positive IgM and IgGbands that were distributed as follows: anti-OspC IgM(B. burgdorferi sensu lato); anti-p41 flagellin IgM; anti-p41intern IgM (B. afzelii); anti-VisE IgG (B. afzelii); anti-p41flagellin IgG; anti-p41-intern IgG (B. garinii); anti-p41-intern IgG (B. afzelii). The EBV DNA PCR of the CSF andthe CSF/blood index for B. burgdorferi sensu lato IgM andIgG were negative. The CSF/blood index was performedusing the IDEIA test (Oxoid, Cambridge, UK) for Lymeneuroborreliosis. The results of the CSF PCR for herpessimplex virus (HSV) and enteroviruses were negative.With the suspicion of Lyme neuroborreliosis, ceftriax-one therapy for 14 days was started. Antiviral therapy withaciclovir was added at the beginning of the therapeuticregimen and stopped when the negative HSV PCR resultbecame available. Steroids had not been given. The patientrecovered almost completely within the first 24 h of ther-apy, with only residual mild neck stiffness that persisted for1 week. Eight days after the initiation of therapy, a follow-up MRI of the spinal cord showed improvement withreduced swelling and less signal alteration (Fig. 2).Three weeks after admission, an outpatient controlshowed a completely normal clinical examination. In thefollow-up lumbar puncture performed at this time, 27 cells/llwere detected (all mononuclear). Protein and glucose(including CSF/plasma ratio) were normal. Examination ofthe oligoclonal IgG bands in the CSF/serum revealed anintrathecal IgG production. The IgG CSF/blood index forB. burgdorferi sensu lato was 41.73 (\0.3), and the EBVDNA PCR of the CSF remained negative.DiscussionLyme neuroborreliosis as a cause of ATM is known, butreports are limited to single cases, both in children andadults. The usual manifestations are severe and ofteninclude dramatic signs of motor weakness and bladderdysfunction [7]. In discussing our case, we wish toemphasize two aspects which—to the best of our knowl-edge—have not been reported to date: the striking dis-crepancy between the severe imaging manifestations andthe minimal clinical signs and symptoms, and the promi-nent MRI findings involving most of the medulla spinalisin a patient with transverse myelitis of borrelial origin. Inpediatric Lyme neuroborreliosis, CNS symptoms arerare—in contrast to symptoms of the peripheral nervoussystem. Children with CNS symptoms may present withacute myelitis. In all such cases reported so far, the childrensuffered from acute symptoms due to their myelitis, such asFig. 2 Left T2-weightedsagittal scan of thecervicothoracic spine. Thecentromedullary edema isregressive but still visible. RightT2-weighted axial scan at thelevel of the cervical spine.Central hyperintensity withaccentuation in the central greymatter was present, but was lessmarked than in the initial scanAcute transverse myelitis in Lyme neuroborreliosis 415pareses, urinary tract dysfunction and sensory disturbances.All of these symptoms and signs were lacking in ourpatient, possibly because of the early diagnosis and treat-ment [10]. The description of an extension to spineinvolvement in a transverse myelitis of borrelial origin inchildren is limited to single case reports. In these casereports, the involvement of the spine is limited and mostlycervically [7, 11]. At the moment of first lumbar puncture,our patient had a symptom duration of \6 weeks, whichexplains the first negative CSF/blood index, as delayedintrathecal antibody synthesis in Lyme neuroborreliosis(positive CSF/blood index) is well known in patients with asymptom duration of \6 weeks. Therefore, the positiveCSF/blood index in the follow-up lumbar puncture con-firms the assumed diagnosis of Lyme neuroborreliosis, andearly detection and treatment may have prevented furtherprogression of the disease. [12–14] In the literature, EBV—in addition to many other occasionally incriminated viru-ses—is a rare but recognized agent causing ATM [5, 15].Serologically, our patient had a co-infection with EBV atthe time of his CNS manifestation, even though there wasno classic mononucleosis (tonsillitis, lymphadenopathy,hepatosplenomegaly) and the EBV DNA PCR of the CFSremained negative over time. We conclude that the ATMwas related to Lyme neuroborreliosis and not to the EBVinfection. However, this patient most likely has had arecent infection with EBV, which may or may not havepaved the way for progression of early, local borreliosis toLyme neuroborreliosis.Single cases of ATM after vaccination have beenreported, but in these, in contrast to our case, there were noother aetiological findings. Pidcock et al. reported that 28%of the reported children with ATM received immunizationor ‘‘allergy shots’’ within 30 days prior to onset, but theycould not demonstrate a potential causal link betweenvaccination and ATM. In our case, the onset of symptoms1 week prior to vaccination renders a vaccine-relatedadverse effect unlikely, but the possibility of vaccinationsupporting the development of Lyme neuroborreliosisbecause of an already present infection cannot be entirelyruled out or resolved.In conclusion, we report the case of a patient with ATMcaused by Lyme neuroborreliosis with surprisingly fewclinical signs—in contrast to the severe MRI and CSFfindings. If there is strong clinical suspicion of Lymeneuroborreliosis—irrespective of a history of a recent tickbite or erythema migrans—appropriate treatment should bestarted, and the CSF/blood index should be repeated toconfirm the diagnosis if the initial results are negative.Conflict of interest statement None.References1. Pidcock FS, Krishnan C, Crawfor TO, Salorio CF, Trovato. M,Kerr DA. Acute transverse myelitis in childhood: center-basedanalysis of 47 cases. Neurology. 2007;68:1474–80.2. Defresne P, Hollenberg H, Husson B, et al. Acute transversemyelitis in children: clinical course and prognostic factors. JChild Neurol. 2003;18:401–6.3. Jacob A, Weinshenker BG. An approach to the diagnosis of acutetransverse myelitis. Semin Neurol. 2008;28:105–20.4. de Seze J, Lanctin C, Lebrun C, et al. Idiopathic acute transversemyelitis: application of the recent diagnostic criteria. Neurology.2005;65:1950–3.5. Knebusch M, Strassburg HM, Reiners K. Acute transversemyelitis in childhood: nine cases and review of the literature. DevMed Child Neurol. 1998;40:631–9.6. Sellner J, Lu¨thi N, Schu¨pbach WMM, et al. Diagnostic workup ofpatients with acute transverse myelitis: spectrum of clinical pre-sentation, neuroimaging and laboratory findings. Spinal Cord.2009;47:312–7.7. Huisman TA, Wohlrab G, Nadal D, Boltshauser E, Martin E.Unusual presentations of neuroborreliosis (Lyme disease) inchildhood. J Comput Assist Tomogr. 1999;23:39–42.8. Olivares JP, Pallas F, Cecaldi M, Viton JM, et al. Lyme diseasepresenting as isolated acute urinary retention caused by transversemyelitis: an electrophysiological and urodynamical study. ArchPhys Med Rehabil. 1995;76:1171–2.9. Rousseau JJ, Lust C, Zangerlie PF, Bigaigon G. Acute transversemyelitis as presenting neurological feature of Lyme disease.Lancet. 1986;2:1222–3.10. Mygland A, Ljøstad U, Fingerle V, Rupprecht T, Schmutzhard E,et al. EFNS guidelines on the diagnosis and management ofEuropean Lyme neuroborreliosis. Eur J Neurol. 2010; 17:8–1611. Meurs L, Labeye, D., Declercq, I., Pieret, F., and Gille M. Acutetransverse myelitis as a main manifestation of early stage IIneuroborreliosis in two patients. Eur Neurol. 2004;52:186–8.12. Christen HJ, Hanefeld, F, Eiffert, H, Thomssen, R. Epidemiologyand clinical manifestations of Lyme borreliosis in childhood. Aprospective multicentre study with special regard to neuroborre-liosis. Acta Paediatr Suppl. 1993;386:1–75.13. Blanc F, Jualhac B, Fleury M, et al. Relevance of the antibodyindex to diagnose Lyme neuroborreliosis among seropositivepatients. Neurology. 2007;69:953–8.14. Ljostad U, Skarpaas T, Mygland A. Clinical usefulness ofintrathecal antibody testing in acute Lyme neuroborreliosis. Eur JNeurol. 2007;14:873–6.15. Doja A, Bitnun A., Jones EL, et al. Pediatric Epstein–Barr virus-associated encephalitis: 10-year review. J Child Neurol.2006;21:385–91.416 S. 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Acute transverse myelitis in Lyme neuroborreliosis

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