[P1-122] A Case Of Acute Disseminated Encephalomyelitis Followed By Optic Neuritis
Fulfilling The Criteria For Neuromyelitis Optica
[Introduction]
Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been implicated in various demyelinating diseases such as acute disseminated encephalomyelitis (ADEM), optic neuritis (ON) and neuromyelitis optica (NMO). Recently, ADEM followed by ON with positive serum anti-MOG antibodies has been reported as a new phenotype of demyelinating diseases.
[Case]
An 8-year-old boy was admitted because of fever, headache and urinary retention. The presence of high lesions with high signal intensity in the subcortical white matter on fluid-attenuated inversion recovery magnetic resonance imaging (MRI) led to a diagnosis of ADEM. The treatment consisted of intravenous methylprednisolone (IVMP) followed by a tapered dose of oral prednisolone, resulting in the immediate improvement of his symptoms. However, his ADEM relapsed twice and spinal MRI revealed longitudinally extensive transverse myelitis extending over three vertebral segments. At the third relapse with ocular pain and visual disturbance in the right eye, the cerebrospinal fluid analysis was negative for anti-aquaporin-4 antibodies for positive anti-MOG antibodies. He was diagnosed with ON and was treated with IVMP, which allowed him to gradually recover. Although the ON relapsed in the left eye 5 months later, the clinical remission was achieved within 10 months after the last relapse by the administration of azathioprine.
[Discussion]
The patient was diagnosed with ADEM followed by ON based on his clinical course, although the clinical appearances fulfilled the criteria for NMO.
The clinicopathological significance of the anti-MOG antibodies remains unclear. Understanding their significance would be helpful for the establishment of the disease entities of demyelinating disorders.
Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been implicated in various demyelinating diseases such as acute disseminated encephalomyelitis (ADEM), optic neuritis (ON) and neuromyelitis optica (NMO). Recently, ADEM followed by ON with positive serum anti-MOG antibodies has been reported as a new phenotype of demyelinating diseases.
[Case]
An 8-year-old boy was admitted because of fever, headache and urinary retention. The presence of high lesions with high signal intensity in the subcortical white matter on fluid-attenuated inversion recovery magnetic resonance imaging (MRI) led to a diagnosis of ADEM. The treatment consisted of intravenous methylprednisolone (IVMP) followed by a tapered dose of oral prednisolone, resulting in the immediate improvement of his symptoms. However, his ADEM relapsed twice and spinal MRI revealed longitudinally extensive transverse myelitis extending over three vertebral segments. At the third relapse with ocular pain and visual disturbance in the right eye, the cerebrospinal fluid analysis was negative for anti-aquaporin-4 antibodies for positive anti-MOG antibodies. He was diagnosed with ON and was treated with IVMP, which allowed him to gradually recover. Although the ON relapsed in the left eye 5 months later, the clinical remission was achieved within 10 months after the last relapse by the administration of azathioprine.
[Discussion]
The patient was diagnosed with ADEM followed by ON based on his clinical course, although the clinical appearances fulfilled the criteria for NMO.
The clinicopathological significance of the anti-MOG antibodies remains unclear. Understanding their significance would be helpful for the establishment of the disease entities of demyelinating disorders.