Contributions
Abstract: EP1309
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - MS Variants
Objective: To examine cases with a clinical course, signs, and symptoms mimicking multiple sclerosis (MS), but without abnormalities on conventional magnetic resonance imaging (MRI).
Background: The widely used 2010 McDonald criteria allow diagnosis of MS in cases without abnormalities on conventional brain and spinal cord MRI. Nevertheless, such patients have generally been excluded from MS cohort studies, under the assumption that negative MRI findings could never coexist with demyelination.
Methods: Among 550 people with a tentative diagnosis of MS or neuromyelitis optica spectrum disorder (NMOSD), we selected patients, who met the 2010 McDonald diagnosis criteria for MS, but did not show abnormal findings on conventional 3 tesla-brain and spinal cord MRI. After evaluating their clinical data, we analyzed fractional anisotropy (FA) values in the brain white matter on diffusion tensor MRI, normalized brain volumes on 3D-T1 weighted MRI, and frequencies of B cell subsets with activation states in the corresponding cases as compared with healthy controls.
Results: Eleven patients met the selection criteria. They were functionally disabled, with a median expanded disability status scale of 6.0. Oligoclonal bands were negative in all cases. Intravenous methylprednisolone and plasmapheresis were found to be efficacious, whereas there was no evidence for efficacy of interferon beta. The normal-appearing white matter structural integrity was significantly reduced as shown by lower FA without any atrophy of brain parenchyma as well as anti-NMDA receptor encephalitis. The plasmablasts in the peripheral blood were significantly activated and increased in these patients, similar to NMOSD.
Conclusions: The neurological disabilities in these patients could be ascribed to brain white matter damage, as revealed by advanced MRI analysis, whereas the efficacy of plasmapheresis and B cell abnormalities in the patients suggested an autoimmune-mediated pathogenesis. In the differential diagnosis of MS, we propose that this condition be called as, “Normal-appearing Imaging-associated, Neuroimmunologically Justified, Autoimmune encephalomyelitis” (NINJA).
Disclosure: D. Takewaki reports no disclosure. Y. Lin received speaker honoraria from Asahi Kuraray Medical, Ltd. W. Sato, H. Ono, and M. Nakamura report no disclosures. M. Araki received honoraria from Chugai Pharmaceutical Co., Ltd.; Novartis Pharmaceuticals; Biogen Japan; Bayer Holding Ltd; Takeda Pharmaceuti-cal Co., Ltd. T. Okamoto received funding for a clinical trial from Takeda Pharmaceutical Co., Ltd. Y. Takahashi, Y. Kimura, M. Ota, and N. Sato report no disclosures. T. Yamamura served on the scientific advisory board for Biogen Japan, Chugai Pharmaceutical Co., Ltd. and Takeda Pharmaceutical Co., Ltd and received travel funding and/or speaker honoraria from Mitsubishi Tanabe Pharma, Novartis, Nihon, Santen, Abbott Japan/Eisai, Biogen Japan, Dainippon Sumitomo, Bayer Holding, and Astellas Pharma Inc., and Takeda Pharmaceutical Co., Ltd., and received research support from Ono, Chugai, Teva, Mitsubishi Tanabe Pharma, Asahi Kuraray Medical, and the Ministry of Health, Labor, and Welfare of Japan.
Abstract: EP1309
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - MS Variants
Objective: To examine cases with a clinical course, signs, and symptoms mimicking multiple sclerosis (MS), but without abnormalities on conventional magnetic resonance imaging (MRI).
Background: The widely used 2010 McDonald criteria allow diagnosis of MS in cases without abnormalities on conventional brain and spinal cord MRI. Nevertheless, such patients have generally been excluded from MS cohort studies, under the assumption that negative MRI findings could never coexist with demyelination.
Methods: Among 550 people with a tentative diagnosis of MS or neuromyelitis optica spectrum disorder (NMOSD), we selected patients, who met the 2010 McDonald diagnosis criteria for MS, but did not show abnormal findings on conventional 3 tesla-brain and spinal cord MRI. After evaluating their clinical data, we analyzed fractional anisotropy (FA) values in the brain white matter on diffusion tensor MRI, normalized brain volumes on 3D-T1 weighted MRI, and frequencies of B cell subsets with activation states in the corresponding cases as compared with healthy controls.
Results: Eleven patients met the selection criteria. They were functionally disabled, with a median expanded disability status scale of 6.0. Oligoclonal bands were negative in all cases. Intravenous methylprednisolone and plasmapheresis were found to be efficacious, whereas there was no evidence for efficacy of interferon beta. The normal-appearing white matter structural integrity was significantly reduced as shown by lower FA without any atrophy of brain parenchyma as well as anti-NMDA receptor encephalitis. The plasmablasts in the peripheral blood were significantly activated and increased in these patients, similar to NMOSD.
Conclusions: The neurological disabilities in these patients could be ascribed to brain white matter damage, as revealed by advanced MRI analysis, whereas the efficacy of plasmapheresis and B cell abnormalities in the patients suggested an autoimmune-mediated pathogenesis. In the differential diagnosis of MS, we propose that this condition be called as, “Normal-appearing Imaging-associated, Neuroimmunologically Justified, Autoimmune encephalomyelitis” (NINJA).
Disclosure: D. Takewaki reports no disclosure. Y. Lin received speaker honoraria from Asahi Kuraray Medical, Ltd. W. Sato, H. Ono, and M. Nakamura report no disclosures. M. Araki received honoraria from Chugai Pharmaceutical Co., Ltd.; Novartis Pharmaceuticals; Biogen Japan; Bayer Holding Ltd; Takeda Pharmaceuti-cal Co., Ltd. T. Okamoto received funding for a clinical trial from Takeda Pharmaceutical Co., Ltd. Y. Takahashi, Y. Kimura, M. Ota, and N. Sato report no disclosures. T. Yamamura served on the scientific advisory board for Biogen Japan, Chugai Pharmaceutical Co., Ltd. and Takeda Pharmaceutical Co., Ltd and received travel funding and/or speaker honoraria from Mitsubishi Tanabe Pharma, Novartis, Nihon, Santen, Abbott Japan/Eisai, Biogen Japan, Dainippon Sumitomo, Bayer Holding, and Astellas Pharma Inc., and Takeda Pharmaceutical Co., Ltd., and received research support from Ono, Chugai, Teva, Mitsubishi Tanabe Pharma, Asahi Kuraray Medical, and the Ministry of Health, Labor, and Welfare of Japan.