Mario Habek1, Ivan Adamec, Vesna V Brinar. 1. University Department of Neurology, Zagreb School of Medicine and University Hospital Center, Kišpaticeva 12, Zagreb, Croatia. mhabek@mef.hr
Abstract
BACKGROUND CONTEXT: Longitudinally extensive transverse myelitis (LETM) is one of the defining features of neuromyelitis optica (NMO). Despite the well-established criteria, clinical and paraclinical features, the disease is often misdiagnosed and erroneously treated. PURPOSE: We report on a case of LETM in a patient with spatially limited NMO spectrum disorder that was misdiagnosed as spinal cord tumor and underwent spinal cord biopsy. STUDY DESIGN: A 43-year-old female patient is described. METHODS: The patient developed spastic tetraparesis over 1 week. Spinal cord magnetic resonance imaging (MRI) revealed LETM, and she was treated with steroids and recovered. Nine months later, her condition worsened and repeat spinal cord MRI was interpreted as a large intramedullary tumor in the cervical region with irregular postcontrast enhancement. Biopsy revealed demyelination. Cerebrospinal fluid (CSF) analysis revealed positive oligoclonal IgG bands, and serum was positive for NMO-IgG antibody. RESULTS: The patient was diagnosed with spatially limited NMO spectrum disorder, treated with plasma exchange, high-dose corticosteroids, and cyclophosphamide, and with good recovery. CONCLUSIONS: The factors favoring inflammatory LETM are acute or subacute onset of clinical symptoms, positive oligoclonal bands in the CSF, positive NMO-IgG or other antibodies, and brain MRI showing demyelinating lesions. Postcontrast axial MRI sequences of the spinal cord can also be helpful. In doubtful situations, a trial of therapy and follow-up MRI a month later might be a more prudent approach if the patient is not rapidly deteriorating.
BACKGROUND CONTEXT: Longitudinally extensive transverse myelitis (LETM) is one of the defining features of neuromyelitis optica (NMO). Despite the well-established criteria, clinical and paraclinical features, the disease is often misdiagnosed and erroneously treated. PURPOSE: We report on a case of LETM in a patient with spatially limited NMO spectrum disorder that was misdiagnosed as spinal cord tumor and underwent spinal cord biopsy. STUDY DESIGN: A 43-year-old female patient is described. METHODS: The patient developed spastic tetraparesis over 1 week. Spinal cord magnetic resonance imaging (MRI) revealed LETM, and she was treated with steroids and recovered. Nine months later, her condition worsened and repeat spinal cord MRI was interpreted as a large intramedullary tumor in the cervical region with irregular postcontrast enhancement. Biopsy revealed demyelination. Cerebrospinal fluid (CSF) analysis revealed positive oligoclonal IgG bands, and serum was positive for NMO-IgG antibody. RESULTS: The patient was diagnosed with spatially limited NMO spectrum disorder, treated with plasma exchange, high-dose corticosteroids, and cyclophosphamide, and with good recovery. CONCLUSIONS: The factors favoring inflammatory LETM are acute or subacute onset of clinical symptoms, positive oligoclonal bands in the CSF, positive NMO-IgG or other antibodies, and brain MRI showing demyelinating lesions. Postcontrast axial MRI sequences of the spinal cord can also be helpful. In doubtful situations, a trial of therapy and follow-up MRI a month later might be a more prudent approach if the patient is not rapidly deteriorating.
Authors: M Ringelstein; O Aktas; J Harmel; D Prayer; S Jarius; B Wildemann; H-P Hartung; S Salhofer-Polanyi; F Leutmezer; P S Rommer Journal: Nervenarzt Date: 2014-10 Impact factor: 1.214
Authors: Sven Jarius; Klemens Ruprecht; Ingo Kleiter; Nadja Borisow; Nasrin Asgari; Kalliopi Pitarokoili; Florence Pache; Oliver Stich; Lena-Alexandra Beume; Martin W Hümmert; Marius Ringelstein; Corinna Trebst; Alexander Winkelmann; Alexander Schwarz; Mathias Buttmann; Hanna Zimmermann; Joseph Kuchling; Diego Franciotta; Marco Capobianco; Eberhard Siebert; Carsten Lukas; Mirjam Korporal-Kuhnke; Jürgen Haas; Kai Fechner; Alexander U Brandt; Kathrin Schanda; Orhan Aktas; Friedemann Paul; Markus Reindl; Brigitte Wildemann Journal: J Neuroinflammation Date: 2016-09-27 Impact factor: 8.322