Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD)
Presentation
Acute right hemiparesis and aphasia. Decreased level of consciousness. History of a recent urinary tract infection.
Patient Data
There is extensive, supratentorial, multifocal, bilateral, fluffy, temporal, frontoparietal, and subcortical high signal intensity on T2 and T2 FLAIR imaging. There is low signal intensity on T1 imaging. There is an avid, irregular enhancement of these lesions on contrast administration. There is some restricted diffusion along the peripherally of the lesions with corresponding reduced average diffusivity on ADC mapping, however, the predominant appearance on ADC is of T2-shine through. The basal ganglia, thalami, brainstem and cerebellar hemispheres are spared. SWI is normal. The optic nerves are tortuous however no abnormal optic/ perioptic or retrobulbar fat enhancement is noted. There is a normal optic chiasm and retrochiasmatic pathway. The TOF MRA is normal.
Case courtesy: Dr VKS. Bhagwandas
Immunology results
Immunology testing confirms a positive titer for myelin oligodendrocyte immunoglobulin and a negative titer for aquaporin 4 antibodies.
Case Discussion
Clinically, the child presented with ADEM-like encephalitis post a recent urinary tract infection. The imaging features would also be consistent with the clinical diagnosis of ADEM. Immunology results were positive for myelin oligodendrocyte IgG at 1:320 (normal < 1:10) and this suggested MOGAD.
Screening of the spinal column revealed a normal spinal cord, conus medullaris and no longitudinally extensive spinal cord lesions (LETS) to possibly suggest neuromyelitis optica spectrum disorder (NMOSD). Aquaporin-4 antibody results were concordant and negative.
Multiple sclerosis is the usual additional differential diagnosis and was considered less likely based on clinical and imaging features especially at this young age too. In addition, the CSF oligoclonal bands were concordant and negative.
The full CSF biochemical and viral workup was negative. The blood and CSF cultures were negative. Excepting for elevated white cell count and inflammatory markers, clinical and toxicology workup was otherwise non-contributory and normal.
The patient had a dramatic clinical improvement with plasma exchange, intravenous immunoglobulin and steroid therapy.
Important negative findings in this instance include the absence of thalamic, basal ganglia, brain stem especially pontine, and cerebellar hemisphere involvement that may occur with MOGAD.
There is no history of associated seizures and no delayed onset of seizures was noted. FLAMES was considered unlikely.
Follow-up imaging was recommended on discharge.
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