Answer for BIR CoW 07 Feb 2021
Anti-MOG Antibody Disease
Findings
MRI showed long segment intramedullary T2 hyperintensities in the spinal cord extending from the cervico-medullary region to D1 level with relative cervical cord expansion. Subtle intramedullary T2 hyperintensities were also noted in the lower dorsal cord and conus. The hyperintensities were predominantly involving the central and posterior parts of the spinal cord with relative sparing of the anterior portion. On contrast administration, the lesion showed patchy enhancement. Discrete T2/FLAIR hyperintensities were also noted in the left anterior midbrain. Both optic nerves appeared relatively thickened (Left more than Right) with hyperintensities in the left optic nerve. Impression –NeuromyelitisOptica spectrum disorder. CSF Anti-MOG (Myelin oligodendrocyte glycoprotein) antibody was positive. DIAGNOSIS – Anti-MOG Antibody disease FOLLOW UP IMAGING - follow up scan showed significant reduction in the cervicodorsal intramedullary T2 hyperintensities and relative reduction in the size of the left midbrain FLAIR hyperintensity, reflecting good response to treatment.
Discussion
MOG encephalomyelitis (MOG-IgG disease) is an inflammatory demyelinating condition of the central nerve system characterized by a monophasic or relapsing course of neurological dysfunction, which does not meet the typical criteria for MS or other known neuroinflammatory conditions and occurs in the presence of serum MOG antibodies detected using specific cell-based assays. Features suggestive of MOG Antibody Disease as opposed to NMOSD include male gender, single or few attacks, bilateral or recurrent optic neuritis sparing the optic chiasma, LETM involving the conus medullaris and good recovery after attacks. MOG Antibody Disease shows longitudinal extensive transverse myelitis (LETM) with optic neuritis and lesions in the brain. The LETM presents as a hyperintense spinal cord lesion extending over ≥3 vertebral levels on sagittal T2-weighted spinal MR imaging. While patients with AQP4-IgG usually present cervical (with or without brainstem involvement) and thoracic lesions, patients with MOG-IgG may present lesions of the lower cord, including the conus medullaris. Bilateral, longitudinally extensive, symmetrical optic neuritis in the intraorbital segments is characteristic, with sparing of the optic chiasma. Optic nerve head swelling, retrobulbar involvement, and contrast-enhancing lesions of the optic nerves with perineural enhancement are seen more commonly in MOG antibody disease. MRI Brain in MOG Antibody Disease shows thalamic and pontine lesions. Supratentorial deep white matter lesions and small number (≤3) of poorly demarcated infratentorial lesions may also be seen. Lesions involving the deep gray matter and lesions adjacent to the fourth ventricle are more common in NMOSD than MOG Antibody Disease.
Findings
MRI showed long segment intramedullary T2 hyperintensities in the spinal cord extending from the cervico-medullary region to D1 level with relative cervical cord expansion. Subtle intramedullary T2 hyperintensities were also noted in the lower dorsal cord and conus. The hyperintensities were predominantly involving the central and posterior parts of the spinal cord with relative sparing of the anterior portion. On contrast administration, the lesion showed patchy enhancement. Discrete T2/FLAIR hyperintensities were also noted in the left anterior midbrain. Both optic nerves appeared relatively thickened (Left more than Right) with hyperintensities in the left optic nerve. Impression –NeuromyelitisOptica spectrum disorder. CSF Anti-MOG (Myelin oligodendrocyte glycoprotein) antibody was positive. DIAGNOSIS – Anti-MOG Antibody disease FOLLOW UP IMAGING - follow up scan showed significant reduction in the cervicodorsal intramedullary T2 hyperintensities and relative reduction in the size of the left midbrain FLAIR hyperintensity, reflecting good response to treatment.
Discussion
MOG encephalomyelitis (MOG-IgG disease) is an inflammatory demyelinating condition of the central nerve system characterized by a monophasic or relapsing course of neurological dysfunction, which does not meet the typical criteria for MS or other known neuroinflammatory conditions and occurs in the presence of serum MOG antibodies detected using specific cell-based assays. Features suggestive of MOG Antibody Disease as opposed to NMOSD include male gender, single or few attacks, bilateral or recurrent optic neuritis sparing the optic chiasma, LETM involving the conus medullaris and good recovery after attacks. MOG Antibody Disease shows longitudinal extensive transverse myelitis (LETM) with optic neuritis and lesions in the brain. The LETM presents as a hyperintense spinal cord lesion extending over ≥3 vertebral levels on sagittal T2-weighted spinal MR imaging. While patients with AQP4-IgG usually present cervical (with or without brainstem involvement) and thoracic lesions, patients with MOG-IgG may present lesions of the lower cord, including the conus medullaris. Bilateral, longitudinally extensive, symmetrical optic neuritis in the intraorbital segments is characteristic, with sparing of the optic chiasma. Optic nerve head swelling, retrobulbar involvement, and contrast-enhancing lesions of the optic nerves with perineural enhancement are seen more commonly in MOG antibody disease. MRI Brain in MOG Antibody Disease shows thalamic and pontine lesions. Supratentorial deep white matter lesions and small number (≤3) of poorly demarcated infratentorial lesions may also be seen. Lesions involving the deep gray matter and lesions adjacent to the fourth ventricle are more common in NMOSD than MOG Antibody Disease.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!