Makoto Mori1, Kenji Sakai2, Yasutake Tada2, Ichiro Nozaki2, Yuta Usui2, Toshiya Ichinose3, Shingo Tanaka3, Toshiyuki Takahashi4,5, Mitsutoshi Nakada3, Masahito Yamada2. 1. Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences, 13-1 Takara-machi, Kanazawa, 920-8640, Japan. m0229maa@med.kanazawa-u.ac.jp. 2. Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences, 13-1 Takara-machi, Kanazawa, 920-8640, Japan. 3. Department of Neurosurgery, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan. 4. Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. 5. Department of Neurology, National Hospital Organization Yonezawa National Hospital, Yonezawa, Yamagata, Japan.
Abstract
BACKGROUND: Acute disseminated encephalomyelitis (ADEM) followed by optic neuritis (ADEM-ON) is characterized by the following features: early onset, monophasic or multiphasic ADEM followed by one or more episodes of ON, and the presence of serum anti-myelin oligodendrocyte glycoprotein (MOG) antibodies. CASE REPORT: We report a case of ADEM-ON without anti-MOG antibodies in a 78-year-old woman. The patient developed acute-onset neurological findings and was diagnosed with ADEM. She was treated with intravenous methylprednisolone (IVMP), and oral corticosteroids. Her clinical symptoms and MRI findings subsequently improved. Left optic neuritis emerged 6 months later, and we made a diagnosis of ADEM-ON. A brain biopsy performed during the acute phase of ADEM showed perivascular infiltration of macrophages with demyelination. CONCLUSION: The majority of the reported ADEM-ON cases are pediatric cases with serum anti-MOG antibodies, but our patient was the elderly, without anti-MOG antibodies. Moreover, the pathological features of our case were similar to those observed in patients with typical ADEM and in patients with anti-MOG antibody-positive ADEM. Although ADEM-ON is related to the presence of anti-MOG antibodies, factors other than anti-MOG antibodies could contribute to the development of ADEM-ON.
BACKGROUND:Acute disseminated encephalomyelitis (ADEM) followed by optic neuritis (ADEM-ON) is characterized by the following features: early onset, monophasic or multiphasic ADEM followed by one or more episodes of ON, and the presence of serum anti-myelin oligodendrocyte glycoprotein (MOG) antibodies. CASE REPORT: We report a case of ADEM-ON without anti-MOG antibodies in a 78-year-old woman. The patient developed acute-onset neurological findings and was diagnosed with ADEM. She was treated with intravenous methylprednisolone (IVMP), and oral corticosteroids. Her clinical symptoms and MRI findings subsequently improved. Left optic neuritis emerged 6 months later, and we made a diagnosis of ADEM-ON. A brain biopsy performed during the acute phase of ADEM showed perivascular infiltration of macrophages with demyelination. CONCLUSION: The majority of the reported ADEM-ON cases are pediatric cases with serum anti-MOG antibodies, but our patient was the elderly, without anti-MOG antibodies. Moreover, the pathological features of our case were similar to those observed in patients with typical ADEM and in patients with anti-MOG antibody-positive ADEM. Although ADEM-ON is related to the presence of anti-MOG antibodies, factors other than anti-MOG antibodies could contribute to the development of ADEM-ON.
Authors: Y Y M Wong; Y Hacohen; T Armangue; E Wassmer; H Verhelst; C Hemingway; E D van Pelt; C E Catsman-Berrevoets; R Q Hintzen; K Deiva; M J Lim; K Rostásy; R F Neuteboom Journal: Eur J Neurol Date: 2018-03-09 Impact factor: 6.089
Authors: Peter Huppke; Kevin Rostasy; Michael Karenfort; Brenda Huppke; Rainer Seidl; Steffen Leiz; Markus Reindl; Jutta Gärtner Journal: Mult Scler Date: 2012-11-05 Impact factor: 6.312