Resumen
Introducción: la leucoencefalitis hemorrágica aguda (AHLE, por sus siglas en inglés) es una enfermedad inflamatoria del cerebro que conduce a una desmielinización hemorrágica del sistema nervioso central (SNC), de mal pronóstico y alta mortalidad. Muchas veces se asocia a diferentes patógenos que provocan un mimetismo molecular.
Caso clínico: presentamos el caso de una paciente mexicana, que presentó una clínica de una evolución aguda de tipo multifocal. Inicialmente asociado a un cuadro de origen infeccioso, aparentemente viral. Posterior a ese cuadro la paciente presenta una evolución tórpida, con retraso del diagnóstico. Acude con las manifestaciones clínicas, radiológicas y en líquido cefalorraquídeo compatibles con la enfermedad, aunque se da tratamiento inmunosupresor de manera energética la paciente presenta poca respuesta al tratamiento, con muchas secuelas por la enfermedad.
Conclusión: existe poca evidencia sobre la evolución clínica y el manejo médico de la enfermedad y se necesitan más estudios para caracterizarla mejor y brindar más información sobre su pronóstico y manejo. En este artículo se provee una revisión sistemática de la bibliografía.
Abstract
Background: Acute hemorrhagic leukoencephalitis (AHLE) is an inflammatory disease of the brain, with a fulminant course that leads to a hemorrhagic demyelination of the central nervous system, having a poor prognosis and high mortality. Most of the times associated to crossed reactivity and molecular mimicry.
Clinical case: We present a case report of a previously healthy young woman with an acute and multifocal clinical course, preceded by a viral respiratory tract infection, followed by a rapid disease progression and a delay in the diagnosis. The clinical, neuroimaging and cerebrospinal fluid featured suggested the diagnosis of AHLE, despite efforts and management with immunosuppression and intensive care, the response to treatment was poor leaving the patient with a severe neurological impairment.
Conclusion: There is little evidence regarding the clinical course and treatment of this disease, and more studies are needed to better characterize it and to provide further information about its prognosis and management. This paper gives a systematic review of the literature.
Grzonka P, Scholz MC, De Marchis GM, Tisljar K, Rüegg S, Marsch S, Fladt J, Sutter R. Acute Hemorrhagic Leukoencephalitis: A Case and Systematic Review of the Literature. Front Neurol. 2020 Aug 20;11:899. doi: 10.3389/ fneur.2020.00899.
Waak M, Malone S, Sinclair K, Phillips G, Bandodkar S, Wienholt L, Robertson T, Whitehead B, Trnka P, Kothur K, Dale RC. Acute Hemorrhagic Leukoencephalopathy: Pathological Features and Cerebrospinal Fluid Cytokine Profiles. Pediatr Neurol. 2019;100:92-96. doi: 10.1016/j.pediatrneurol.2019.06.013.
Francisci D, Sensini A, Fratini D, Moretti MV, Luchetta ML, Di Caro A, Stagni G, Baldelli F. Acute fatal necrotizing hemorrhagic encephalitis caused by Epstein-Barr virus in a young adult immunocompetent man. J Neurovirol. 2004;10(6):414-7. doi: 10.1080/13550280490521050.
Martins HM, Teixeira AL Jr, Lana-Peixoto MA; Brazilian Committee for Treatment and Research in Multiple Sclerosis. Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report. Arq Neuropsiquiatr. 2004;62(1):139- 43. doi: 10.1590/s0004-282x2004000100024.
Broderick L, Gandhi C, Mueller JL, Putnam CD, Shayan K, Giclas PC, Peterson KS, Aceves SS, Sheets RM, Peterson BM, Newbury RO, Hoffman HM, Bastian JF. Mutations of complement factor I and potential mechanisms of neuroinflammation in acute hemorrhagic leukoencephalitis. J Clin Immunol. 2013;33(1):162-71. doi: 10.1007/s10875-012-9767-z.
Robinson CA, Adiele RC, Tham M, Lucchinetti CF, Popescu BF. Early and widespread injury of astrocytes in the absence of demyelination in acute haemorrhagic leukoencephalitis. Acta Neuropathol Commun. (2014) 2:52. doi: 10.1186/2051-5960-2-52.
Karussis D. The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. J Autoimmun. 2014;48-49:134-42. doi: 10.1016/j.jaut.2014.01.022.
Pfausler B, Engelhardt K, Kampfl A, Spiss H, Taferner E, Schmutzhard E. Post-infectious central and peripheral nervous system diseases complicating Mycoplasma pneumoniae infection. Report of three cases and review of the literature. Eur J Neurol. 2002;9(1):93-6. doi: 10.1046/j.1468-1331.2002.00350.x.
Meilof JF, Hijdra A, Vermeulen M. Successful recovery after high-dose intravenous methylprednisolone in acute hemorrhagic leukoencephalitis. J Neurol. 2001;248(10):898-9. doi: 10.1007/s004150170076.
Tanser SJ, Walker MB, Hilton DA. Acute haemorrhagic leucoencephalitis complicating sepsis. Anaesth Intensive Care. 2001;29(1):54-7.
Gibbs WN, Kreidie MA, Kim RC, Hasso AN. Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. J Comput Assist Tomogr. 2005;29(5):689-93.
Scolding N. Inflammatory demyelinating disease (II): syndromes and disorders related to multiple sclerosis. In: Scolding N, ed. Immunological and Inflammatory Disorders of the Central Nervous System. Oxford: Butterworth-Heinemann; 1999:74-92.
Bernarding J, Braun J, Koennecke H. Diffusion- and perfusion-weighted MR imaging in a patient with acute demyelinating encephalomyelitis (ADEM). J Magn Reson Imaging. 2002;15:96-100.
Dale RC. Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis. 2003;14(2):90-5. doi: 10.1053/ spid.2003.127225.
Granerod J, Ambrose HE, Davies NW, Clewley JP, Walsh AL, Morgan D, et al. Causes of encephalitis and differences in their clinical presentations in England: a multicentre, population-based prospective study. Lancet Infect Dis. 2010.
Lee HY, Chang KH, Kim JH, Na DG, Kwon BJ, Lee KW, et al. Serial MR imaging findings of acute hemorrhagic leukoencephalitis: a case report. AJNR Am J Neuroradiol. 2005;26: 1996-9.
Sureshbabu S, Babu R, Garg A, Peter S, Sobhana C, Mittal GK. Acute hemorrhagic leukoencephalitis unresponsive to aggressive immunosuppression. Clin Exp Neuroimmunol. 2017;2017:63-6.
Payne ET, Rutka JT, Ho TK, Halliday WC, Banwell BL. Treatment leading to dramatic recovery in acute hemorrhagic leukoencephalitis. J Child Neurol. 2007;22(1):109-13.
Ryan LJ, Bowman R, Zantek ND, Sherr G, Maxwell R, Clark HB, Mair DC. Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature. Transfusion. 2007;47(6):981-6. doi: 10.1111/j.1537-2995.2007.01227.
Nabi S, Badshah M, Ahmed S, Nomani AZ. Weston-Hurst syndrome: a rare fulminant form of acute disseminated encephalomyelitis (ADEM). BMJ Case Rep. 2016.