Abstract
Acute disseminated encephalomyelitis (ADEM), which is a disease that causes multifocal inflammatory demyelination of the central nervous system, occurs predominantly in children and young adults. We report an autopsy case of an elderly man with brainstem ADEM that progressed over a period of about 3 months. An 82-year-old man developed disturbance of consciousness, dysphagia, and ataxic gait over a period of about 3 months. He was admitted to another hospital for aspiration pneumonia and recovered but was transferred to our hospital due to prolonged disturbance of consciousness. The patient was able to follow simple commands but had a tendency to somnolence. In addition to meningeal stimulation signs, the patient had left-dominant upper and lower limb ataxia and right-dominant limb spasticity. Brain FLAIR/T2-weighted imaging showed high-intensity lesions from the brainstem to the middle cerebellar peduncle bilaterally, medulla oblongata and upper cervical spinal cord, and T1-weighted imaging revealed contrast-enhanced lesions in the left middle cerebellar peduncle and cervical spinal cord. Although spinal fluid examination revealed elevated proteins, other laboratory tests indicated no evidence of infection, vasculitis, collagen diseases or tumors, and anti-ganglioside, anti-AQP4 and anti-MOG antibodies were negative. After admission, the patient again developed aspiration pneumonia, which progressed to acute respiratory distress syndrome, and he died on the 15th day of hospitalization. Autopsy findings indicated acute and subacute demyelination mainly in the brainstem and cerebellum, and perivascular lymphocyte and macrophage infiltration in the areas of demyelination. A postmortem diagnosis of ADEM was made based on the generally monophasic course of the disease and the absence of regenerating myelinated sheaths. There are very few reports of elderly patients with brainstem ADEM. ADEM should be considered as a differential diagnosis in patients with brainstem encephalitis.
© 2023 The Author(s). Published by S. Karger AG, Basel
References
Koelman DLH, Mateen FJ. Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome. J Neurol. 2015;262(9):2013–24.
Jarius S, Paul F, Aktas O, Asgari N, Dale RC, de Seze J, et al. MOG encephalomyelitis: international recommendations on diagnosis and antibody testing. J Neuroinflammation. 2018;15(1):134.
Tan IL, Mowry EM, Steele SU, Pardo CA, McArthur JC, Nath A, et al. Brainstem encephalitis: etiologies, treatment, and predictors of outcome. J Neurol. 2013;260:2312–9.
Wender M. Acute disseminated encephalomyelitis (ADEM). J Neuroimmunol. 2011;231(1–2):92–9.
Young NP, Weinshenker BG, Parisi JE, Scheithauer B, Giannini C, Roemer SF, et al. Perivenous demyelination: association with clinically defined acute disseminated encephalomyelitis and comparison with pathologically confirmed multiple sclerosis. Brain. 2010;133(Pt 2):333–48.
Miller DH, Scaravilli F, Thomas DC, Harvey P, Hirsch NP. Acute disseminated encephalomyelitis presenting as a solitary brainstem mass. J Neurol Neurosurg Psychiatry. 1993;56(8):920–2.
Alper G, Sreedher G, Zuccoli G. Isolated brain stem lesion in children: is it acute disseminated encephalomyelitis or not?AJNR Am J Neuroradiol. 2013;34(1):217–20.
Ketelslegers IA, Visser IER, Neuteboom RF, Boon M, Catsman-Berrevoets CE, Hintzen RQ. Disease course and outcome of acute disseminated encephalomyelitis is more severe in adults than in children. Mult Scler. 2011;17(4):441–8.
Matejuk A, Hopke C, Vandenbark AA, Hurn PD, Offner H. Middle-age male mice have increased severity of experimental autoimmune encephalomyelitis and are unresponsive to testosterone therapy. J Immunol. 2005;174(4):2387–95.
Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain. 2000;123(Pt 12):2407–22.
Moreira I, Cruto C, Correia C, Alves JE, Taipa R, Pires MM. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): postmortem findings. J Neuropathol Exp Neurol. 2015;74(2):186–90.
Krupp LB, Tardieu M, Amato MP, Banwell B, Chitnis T, Dale RC, et al. International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions. Mult Scler. 2013;19(10):1261–7.
Cohen O, Steiner-Birmanns B, Biran I, Abramsky O, Honigman S, Steiner I. Recurrence of acute disseminated encephalomyelitis at the previously affected brain site. Arch Neurol. 2001;58(5):797–801.
Author Contacts
Yasuyuki Takai, tkysyk3@gmail.com
Article / Publication Details
Received: October 28, 2022
Accepted: January 09, 2023
Published online: February 14, 2023
Issue release date: January – December
Number of Print Pages: 10
Number of Figures: 3
Number of Tables: 3
eISSN: 1662-680X (Online)
For additional information: https://www.karger.com/CRN
Open Access License / Drug Dosage / Disclaimer
This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
留言 (0)