Abstract
Usually, demyelinating diseases of human central nervous system manifest as chronic illnesses with episodic exacerbations followed by remissions, which may be spontaneous or follow treatment with corticosteroids or other immunosuppressive agents. Yet, occasionally inflammatory demyelinating diseases manifest acutely with a rapidly progressive course that may even cause life-threatening complications, thus demanding drastic treatment decisions. Such potentially disabling and lethal complications stem from extensive brain, brainstem, and spinal cord involvement in a number of pathologic processes, such as edema with mass effect, herniation syndrome, progressive weakness with its adverse impact on respiratory function, and other medical complications. The present chapter reviews a wide range of acute inflammatory demyelinating diseases, including acute disseminated encephalomyelitis, neuromyelitis optica, optic neuritis, and transverse myelitis. Imitators of these acute demyelinating events are also discussed. Diagnostic procedures and optimal treatment options are presented.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Lucchinetti CF, Mandler RN, McGavern D, Bruck W, Gleich G, Ransohoff RM, Trebst C, Weinshenker B, Wingerchuk D, Parisi JE, Lassmann H. A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica. Brain. 2002;125:1450–61.
Karussis D. The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. J Autoimmun. 2014;48–49:134–42.
Bunyan RF, Tang J, Weinshenker B. Acute demyelinating disorders: emergencies and management. Neurol Clin. 2012;30:285–307.. ix-x
Sonneville R, Klein I, de Broucker T, Wolff M. Post-infectious encephalitis in adults: diagnosis and management. J Infect. 2009;58:321–8.
Noorbakhsh F, Johnson RT, Emery D, Power C. Acute disseminated encephalomyelitis: clinical and pathogenesis features. Neurol Clin. 2008;26:759–80.. ix
San Pedro EC, Mountz JM, Liu HG, Deutsch G. Postinfectious cerebellitis: clinical significance of Tc-99m HMPAO brain SPECT compared with MRI. Clin Nucl Med. 1998;23:212–6.
Brass SD, Duquette P, Proulx-Therrien J, Auerbach S. Sleep disorders in patients with multiple sclerosis. Sleep Med Rev. 2010;14:121–9.
Leary SM, Porter B, Thompson AJ. Multiple sclerosis: diagnosis and the management of acute relapses. Postgrad Med J. 2005;81:302–8.
Capello E, Mancardi GL. Marburg type and Balo’s concentric sclerosis: rare and acute variants of multiple sclerosis. Neurol Sci. 2004;25(Suppl 4):S361–3.
Darke M, Bahador FM, Miller DC, Litofsky NS, Ahsan H. Balo’s concentric sclerosis: imaging findings and pathological correlation. J Radiol Case Rep. 2013;7:1–8.
Lucchinetti CF, Gavrilova RH, Metz I, Parisi JE, Scheithauer BW, Weigand S, Thomsen K, Mandrekar J, Altintas A, Erickson BJ, Konig F, Giannini C, Lassmann H, Linbo L, Pittock SJ, Bruck W. Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Brain. 2008;131:1759–75.
Marignier R, Confavreux C. Devic’s neuromyelitis optica and related neurological disorders. Presse Med. 2010;39:371–80.
Clark D, Kebede W, Eggenberger E. Optic neuritis. Neurol Clin. 2010;28:573–80.
Atkins EJ, Biousse V, Newman NJ. The natural history of optic neuritis. Rev Neurol Dis. 2006;3:45–56.
Wilhelm H, Schabet M. The diagnosis and treatment of optic neuritis. Dtsch Arztebl Int. 2015;112:616–25.. quiz 626
Costello F, Hodge W, Pan YI, Metz L, Kardon RH. Retinal nerve fiber layer and future risk of multiple sclerosis. Can J Neurol Sci. 2008;35:482–7.
Beh SC, Greenberg BM, Frohman T, Frohman EM. Transverse myelitis. Neurol Clin. 2013;31:79–138.
Scott TF, Frohman EM, De Seze J, Gronseth GS, Weinshenker BG. Therapeutics and Technology Assessment Subcommittee of American Academy of N. Evidence-based guideline: clinical evaluation and treatment of transverse myelitis: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2011;77:2128–34.
Campi A, Pontesilli S, Gerevini S, Scotti G. Comparison of MRI pulse sequences for investigation of lesions of the cervical spinal cord. Neuroradiology. 2000;42:669–75.
Weinshenker BG. Therapeutic plasma exchange for acute inflammatory demyelinating syndromes of the central nervous system. J Clin Apher. 1999;14:144–8.
Rae-Grant A, Day GS, Marrie RA, Rabinstein A, Cree BAC, Gronseth GS, Haboubi M, Halper J, Hosey JP, Jones DE, Lisak R, Pelletier D, Potrebic S, Sitcov C, Sommers R, Stachowiak J, Getchius TSD, Merillat SA, Pringsheim T. Practice guideline recommendations summary: disease-modifying therapies for adults with multiple sclerosis: report of the guideline development, dissemination, and implementation Subcommittee of the American Academy of Neurology. Neurology. 2018;90(17):777–88.
Wu Y, Zhong L, Geng J. Neuromyelitis optica spectrum disorder: pathogenesis, treatment, and experimental models. Mult Scler Relat Disord. 2019;27:412–8.
Bizzi A, Ulug AM, Crawford TO, Pase T, Bugiani M, Bryan RN, Barke PB. Quantitative proton MR spectroscopic imaging in acute disseminated encephalomyelitis. Am J Neuroradiol. 2001;22(6):1125–30.
Saindane AM. Proton MR spectroscopy of tumefactive demyelinating lesions. Am J Neuroradiol. 2002;23(8):1378–86.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Bir, S.C., Gonzalez-Toledo, E., Minagar, A. (2020). Emergencies of Demyelinating Diseases. In: Rabinstein, A. (eds) Neurological Emergencies. Springer, Cham. https://doi.org/10.1007/978-3-030-28072-7_17
Download citation
DOI: https://doi.org/10.1007/978-3-030-28072-7_17
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-28071-0
Online ISBN: 978-3-030-28072-7
eBook Packages: MedicineMedicine (R0)