Neuropediatrics 2015; 46 - PS02-07
DOI: 10.1055/s-0035-1550720

Longitudinally Extensive Transverse Myelitis in Four Children

C. Reihle 1, T. von Kalle 2, G. Schmiedel 3, C. Severin 4, T. Sauter 5, K. Marquard 1, M. Blankenburg 1
  • 1Klinikum Stuttgart Olgahospital und Frauenklinik, Pädiatrie 1, Stuttgart, Germany
  • 2Klinikum Stuttgart Olgahospital und Frauenklinik, Institut für Kinderradiologie, Stuttgart, Germany
  • 3Klinikum Esslingen, Klinik für Kinder und Jugendliche, Esslingen, Germany
  • 4Klinikum Sindelfingen-Böblingen, Kliniken Böblingen, Böblingen, Germany
  • 5Gemeinschaftspraxis für Kinder- und Jugendmedizin, Leinfelden-Echterdingen, Germany

Introduction: Longitudinally extensive transverse myelitis (LETM) is a rare form of myelitis in children. MRI shows extensive T2-weighted high-signal intensity extending across three or more vertebral segments. Etiology is not uniform, outcome may be severe. We report four cases who were seen by us within the past 2 years.

Case Reports: Case 1 represents a 13-year-old girl Represented with subacute weakness of legs and arms within days, opticus neuritis. A few months ago she was suffering from brain stem encephalitis of unknown origin. Gait pattern normalized after steroid pulse therapy and plasmapheresis, and vision was only partially improved. MRI showed LETM mainly cervicothoracic. Aquaporin-4-antibodies were positive. The diagnosis followed was neuromyelitis optica (NMO).

Case 2 represents a 13-year-old girl suffering from arthritis during the last months. The symptoms found in the girl were febrile disease with diarrhea, vomiting, and headache, followed by acute paraparesis within hours. Few neurological improvements despite of steroids, plasmapheresis, and immunosuppression were observed. Aquaporin-4-antibodies were slightly elevated. MRI showed LETM of the complete myelon. The diagnosis was NMO spectrum disorder.

Case 3 represents a 9-year-old boy suffering from fever, followed by back pain, subacute weakness of legs within a few days. The MRI showed LETM, mainly thoracic. The patient was clinically normalized within a few days after steroid therapy. The diagnosis followed was parainfectious myelitis possibly by reactivation of Epstein-Barr virus.

Case 4 represents a 13-year-old girl suffering from backpain since 3 weeks, then meninges without fever along with tetraplegic within days. The diagnosis of acute neuroborreliosis was followed. The therapy given to the patient was with antibiotics plus steroids, no plasmapheresis because of the acute infection. Temporary need of mechanical ventilation after tracheostomy was given. Neurological rehabilitation with significant improvement so far is observed.

Discussion and Conclusion: LETM is seen as rather typical for NMO, but other, mainly neuroimmunological or infectious diseases have to be considered as well. In severe cases with no response to steroids, plasmapheresis should be considered. MRI plays an essential role for diagnosis and during follow-up.

Keywords: myelitis, neuromyelitis optica, LETM, plasmapheresis.