TY - JOUR
T1 - MOG antibody disease manifesting as progressive cognitive deterioration and behavioral changes with primary central nervous system vasculitis
AU - Baba, Toshikazu
AU - Shinoda, Koji
AU - Watanabe, Mitsuru
AU - Sadashima, Shoko
AU - Matsuse, Dai
AU - Isobe, Noriko
AU - Yamasaki, Ryo
AU - Kaneko, Kimihiko
AU - Takahashi, Toshiyuki
AU - Iwaki, Toru
AU - Kira, Jun ichi
N1 - Funding Information:
This study was supported in part by a Grant-in-Aid for Scientific Research A ( MEXT KAKENHI grant no. 16H02657 ) and C (16K09694), a Grant-in-Aid for Young Scientists B ( 17K16124 , 17K16125 ) from the Japan Society for the Promotion of Science , and a Health and Labour Sciences Research Grant on Intractable Diseases ( H29-Nanchitou (Nan)-Ippan-043 ) from the Ministry of Health, Labour and Welfare , Japan.
Funding Information:
This study was supported in part by a Grant-in-Aid for Scientific Research A (MEXT KAKENHI grant no. 16H02657) and C (16K09694), a Grant-in-Aid for Young Scientists B (17K16124, 17K16125) from the Japan Society for the Promotion of Science, and a Health and Labour Sciences Research Grant on Intractable Diseases (H29-Nanchitou (Nan)-Ippan-043) from the Ministry of Health, Labour and Welfare, Japan.
Publisher Copyright:
© 2019
PY - 2019/5
Y1 - 2019/5
N2 - We report a 60-year-old male with anti-myelin oligodendrocyte glycoprotein (MOG) antibody who developed progressive cognitive deterioration and behavioral changes, with no other focal signs, over 9 months. MRI showed numerous T2-hyperintense lesions with partial contrast enhancement in white and grey matter of cerebrum, cerebellum and spinal cord. A brain biopsy revealed perivascular inflammatory cell infiltration, disturbed vascular continuity and no demyelination, indicative of a lymphocytic pattern of primary CNS vasculitis (PCNSV). Contrast enhancement disappeared after immunotherapy; however, cognitive impairment was not improved. Neurologists should note that MOG antibody disease can present as immunotherapy-resistant progressive cognitive impairment with PCNSV-like histopathology.
AB - We report a 60-year-old male with anti-myelin oligodendrocyte glycoprotein (MOG) antibody who developed progressive cognitive deterioration and behavioral changes, with no other focal signs, over 9 months. MRI showed numerous T2-hyperintense lesions with partial contrast enhancement in white and grey matter of cerebrum, cerebellum and spinal cord. A brain biopsy revealed perivascular inflammatory cell infiltration, disturbed vascular continuity and no demyelination, indicative of a lymphocytic pattern of primary CNS vasculitis (PCNSV). Contrast enhancement disappeared after immunotherapy; however, cognitive impairment was not improved. Neurologists should note that MOG antibody disease can present as immunotherapy-resistant progressive cognitive impairment with PCNSV-like histopathology.
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U2 - 10.1016/j.msard.2019.01.053
DO - 10.1016/j.msard.2019.01.053
M3 - Article
C2 - 30738279
AN - SCOPUS:85061051453
SN - 2211-0348
VL - 30
SP - 48
EP - 50
JO - Multiple Sclerosis and Related Disorders
JF - Multiple Sclerosis and Related Disorders
ER -