Abstract
We aimed to evaluate the long-term (>2 years) outcome of acute and subacute myelopathies (ASM). We systematically followed-up consecutive patients presenting with a first episode of ASM, defined by spinal cord symptoms with an onset <3 weeks and duration ≥48 h. Patients with compressive or traumatic spinal cord lesions are excluded from this report. Our cohort consisted of 170 patients (median age 39.0 years, median duration of follow-up 73.2 months). The death rate was 8.8%, Lipton and Teasdall’s functional score was bad or fair in 38.2%, and 37.1% of the survivors who worked when the ASM occurred were unable to carry on with the same profession. Unfavorable functional outcome was more frequent when (1) symptoms were initially severe, (2) the lesion was located centrally on spinal cord MRI and (3) the etiology was neuromyelitis optica (NMO) or systemic disease (SD). In one-third of patients the etiology at the end of follow-up differed from the etiology suspected after the initial diagnostic workup. Over half of patients initially diagnosed with myelopathy of undetermined cause subsequently developed multiple sclerosis, NMO or SD. ASM is a severe condition with a bad or fair functional outcome and a major impact on professional activity in one-third of the patients. Central lesions on spinal cord MRI and etiologies such as NMO or SD are associated with a worse functional outcome. Finally, a long-term follow-up is important given the large number of causes that are identified at a distance from the initial event.
Similar content being viewed by others
References
Barkhof F, Filippi M, Miller DH, Scheltens P, Campi A, Polman CH, Comi G, Ader HJ, Losseff N, Valk J (1997) Comparison of MRI criteria at first presentation to predict conversion to clinically definite multiple sclerosis. Brain 120(Pt 11):2059–2069
Baughman RP, Lower EE, du Bois RM (2003) Sarcoidosis. Lancet 361:1111–1118
Berman M, Feldman S, Alter M, Zilber N, Kahana E (1981) Acute transverse myelitis: incidence and etiologic considerations. Neurology 31:966–971
Bot JC, Barkhof F, Polman CH, Lycklama a Nijeholt GJ, de Groot V, Bergers E, Ader HJ, Castelijns JA (2004) Spinal cord abnormalities in recently diagnosed MS patients: added value of spinal MRI examination. Neurology 62:226–233
Breteau G, Stojkovic T, De Seze J, Gauvrit J, Pruvo J, Vermersch P (2000) Adenovirus et Epstein-Barr virus: deux étiologies rares au profil identique. Rev Neurol Paris 156:786–789
Catz A, Goldin D, Fishel B, Ronen J, Bluvshtein V, Gelernter I (2004) Recovery of neurologic function following nontraumatic spinal cord lesions in Israel. Spine 29:2278–2282 discussion 2283
Cheshire WP, Santos CC, Massey EW, Howard JF Jr (1996) Spinal cord infarction: etiology and outcome. Neurology 47:321–330
Christensen PB, Wermuth L, Hinge HH, Bomers K (1990) Clinical course and long-term prognosis of acute transverse myelopathy. Acta Neurol Scand 81:431–435
Citterio A, Franceschini M, Spizzichino L, Reggio A, Rossi B, Stampacchia G (2004) Nontraumatic spinal cord injury: an Italian survey. Arch Phys Med Rehabil 85:1483–1487
Cordonnier C, de Seze J, Breteau G, Ferriby D, Michelin E, Stojkovic T, Pruvo JP, Vermersch P (2003) Prospective study of patients presenting with acute partial transverse myelopathy. J Neurol 250:1447–1452
Dalecky A, Pelletier J, Cherif AA, Levrier O, Khalil R (1997) Acute myelopathies in young patients and multiple sclerosis. Prospective study of 20 cases. Rev Neurol (Paris) 153:569–578
de Seze J, Lanctin C, Lebrun C, Malikova I, Papeix C, Wiertlewski S, Pelletier J, Gout O, Clerc C, Moreau C, Defer G, Edan G, Dubas F, Vermersch P (2005) Idiopathic acute transverse myelitis: application of the recent diagnostic criteria. Neurology 65:1950–1953
de Seze J, Stojkovic T, Breteau G, Lucas C, Michon-Pasturel U, Gauvrit JY, Hachulla E, Mounier-Vehier F, Pruvo JP, Leys D, Destee A, Hatron PY, Vermersch P (2001) Acute myelopathies: clinical, laboratory and outcome profiles in 79 cases. Brain 124:1509–1521
de Seze J, Stojkovic T, Ferriby D, Gauvrit JY, Montagne C, Mounier-Vehier F, Verier A, Pruvo JP, Hache JC, Vermersch P (2002) Devic’s neuromyelitis optica: clinical, laboratory, MRI and outcome profile. J Neurol Sci 197:57–61
de Seze J, Stojkovic T, Hachulla E, Breteau G, Michon-Pasturel U, Mounier-Vehier F, Hatron PY, Vermersch P (2001) Myélopathies et syndrome de Gougerot-Sjögren: étude clinique, radiologique et profil évolutif. Rev Neurol (Paris) 157:669–678
Debette S, Gauvrit JY, Desseaux G, Nibbio A, Henon H, Pruvo JP, Leys D (2003) Paraplegia after ligation of esophageal varices. Neurology 60:879–880
Farkkila M, Tiainen T, Koskiniemi M (1997) Epidemiology and prognosis of acute myelitis in Southern Finland. J Neurol Sci 152:140–146
Ford B, Tampieri D, Francis G (1992) Long-term follow-up of acute partial transverse myelopathy. Neurology 42:250–252
Henon H, Vermersch P, Dutoit E, Dubois F, Camus D, Petit H (1995) Myelite a anguillule. Rev Neurol (Paris) 151:139–141
Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725
Iseli E, Cavigelli A, Dietz V, Curt A (1999) Prognosis and recovery in ischaemic and traumatic spinal cord injury: clinical and electrophysiological evaluation. J Neurol Neurosurg Psychiatry 67:567–571
Jeffery DR, Mandler RN, Davis LE (1993) Transverse myelitis. Retrospective analysis of 33 cases, with differentiation of cases associated with multiple sclerosis and parainfectious events. Arch Neurol 50:532–535
Kidd D, Thorpe JW, Thompson AJ, Kendall BE, Moseley IF, MacManus DG, McDonald WI, Miller DH (1993) Spinal cord MRI using multi-array coils and fast spin echo. II. Findings in multiple sclerosis. Neurology 43:2632–2637
Kovacs B, Lafferty TL, Brent LH, DeHoratius RJ (2000) Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature. Ann Rheum Dis 59:120–124
Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101–1107
Lennon VA, Wingerchuk DM, Kryzer TJ, Pittock SJ, Lucchinetti CF, Fujihara K, Nakashima I, Weinshenker BG (2004) A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 364:2106–2112
Lipton HL, Teasdall RD (1973) Acute transverse myelopathy in adults. A follow-up study. Arch Neurol 28:252–257
Lu X, Gu Y, Wang Y, Chen S, Ye S (2008) Prognostic factors of lupus myelopathy. Lupus 17:323–328
Lucchinetti CF, Mandler RN, McGavern D, Bruck W, Gleich G, Ransohoff RM, Trebst C, Weinshenker B, Wingerchuk D, Parisi JE, Lassmann H (2002) A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica. Brain 125:1450–1461
Mandler RN (2006) Neuromyelitis optica—Devic’s syndrome, update. Autoimmun Rev 5:537–543
Masson C, Pruvo JP, Meder JF, Cordonnier C, Touze E, De La Sayette V, Giroud M, Mas JL, Leys D (2004) Spinal cord infarction: clinical and magnetic resonance imaging findings and short term outcome. J Neurol Neurosurg Psychiatry 75:1431–1435
Maynard FM Jr, Bracken MB, Creasey G, Ditunno JF Jr, Donovan WH, Ducker TB, Garber SL, Marino RJ, Stover SL, Tator CH, Waters RL, Wilberger JE, Young W (1997) International standards for neurological and functional classification of spinal cord injury. American Spinal Injury Association. Spinal Cord 35:266–274
McDonald WI, Compston A, Edan G, Goodkin D, Hartung HP, Lublin FD, McFarland HF, Paty DW, Polman CH, Reingold SC, Sandberg-Wollheim M, Sibley W, Thompson A, van den Noort S, Weinshenker BY, Wolinsky JS (2001) Recommended diagnostic criteria for multiple sclerosis: guidelines from the international panel on the diagnosis of multiple sclerosis. Ann Neurol 50:121–127
McKinley WO, Seel RT, Hardman JT (1999) Nontraumatic spinal cord injury: incidence, epidemiology, and functional outcome. Arch Phys Med Rehabil 80:619–623
Miller DH, Ormerod IE, Rudge P, Kendall BE, Moseley IF, McDonald WI (1989) The early risk of multiple sclerosis following isolated acute syndromes of the brainstem and spinal cord. Ann Neurol 26:635–639
Mok CC, Lau CS, Chan EY, Wong RW (1998) Acute transverse myelopathy in systemic lupus erythematosus: clinical presentation, treatment, and outcome. J Rheumatol 25:467–473
Nedeltchev K, Loher TJ, Stepper F, Arnold M, Schroth G, Mattle HP, Sturzenegger M (2004) Long-term outcome of acute spinal cord ischemia syndrome. Stroke 35:560–565
O’Riordan JI, Gallagher HL, Thompson AJ, Howard RS, Kingsley DP, Thompson EJ, McDonald WI, Miller DH (1996) Clinical, CSF, and MRI findings in Devic’s neuromyelitis optica. J Neurol Neurosurg Psychiatry 60:382–387
Polman CH, Reingold SC, Edan G, Filippi M, Hartung HP, Kappos L, Lublin FD, Metz LM, McFarland HF, O’Connor PW, Sandberg-Wollheim M, Thompson AJ, Weinshenker BG, Wolinsky JS (2005) Diagnostic criteria for multiple sclerosis: 2005 revisions to the “McDonald criteria”. Ann Neurol 58:840–846
Pradhan S, Gupta RK, Ghosh D (1997) Parainfectious myelitis: three distinct clinico-imagiological patterns with prognostic implications. Acta Neurol Scand 95:241–247
Provenzale JM, Barboriak DP, Gaensler EH, Robertson RL, Mercer B (1994) Lupus-related myelitis: serial MR findings. AJNR Am J Neuroradiol 15:1911–1917
Ramirez C, de Seze J, Delalande S, Michelin E, Ferriby D, Al Khedr A, Stojkovic T, Destee A, Vermersch P (2004) Myelopathies infectieuses: profils cliniques, paracliniques et evolutifs. Rev Neurol (Paris) 160:1048–1058
Ronen J, Goldin D, Bluvshtein V, Fishel B, Gelernter I, Catz A (2004) Survival after nontraumatic spinal cord lesions in Israel. Arch Phys Med Rehabil 85:1499–1502
Salvador de la Barrera S, Barca-Buyo A, Montoto-Marques A, Ferreiro-Velasco ME, Cidoncha-Dans M, Rodriguez-Sotillo A (2001) Spinal cord infarction: prognosis and recovery in a series of 36 patients. Spinal Cord 39:520–525
Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ, Tan EM, Tan EM (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus special antibodies for the study of systemic lupus erythematosus: an analysis autoantibodies to nuclear antigens (ANA): their immunobiology and medicine. Arthritis Rheum 25:1271–1277
Tartaglino LM, Friedman DP, Flanders AE, Lublin FD, Knobler RL, Liem M (1995) Multiple sclerosis in the spinal cord: MR appearance and correlation with clinical parameters. Radiology 195:725–732
Transverse Myelitis Consortium Working Group (2002) Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology 59:499–505
Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH (2002) Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European consensus group. Ann Rheum Dis 61:554–558
Wingerchuk DM, Weinshenker BG (2003) Neuromyelitis optica: clinical predictors of a relapsing course and survival. Neurology 60:848–853
Zaffaroni M (2004) Cerebrospinal fluid findings in Devic’s neuromyelitis optica. Neurol Sci 25(Suppl 4):S368–S370
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Debette, S., de Sèze, J., Pruvo, JP. et al. Long-term outcome of acute and subacute myelopathies. J Neurol 256, 980–988 (2009). https://doi.org/10.1007/s00415-009-5058-x
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00415-009-5058-x