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Título

Multiple sclerosis after anti-NMDAR encephalitis: case report and review of the literature

RESUMO

Case Report

A 19-years-old woman presented with seizures in June/2019. Brain MRI and EEG were unmarkable and she received levetiracetam. After 30 days she developed depression, aphasia, anosognosia, apathy, dyscognitive seizures, sleep disorder and recurrent vomiting. Physical examination disclosed temporal disorientation, mild ataxic gait , tremor and incoordination in left limbs. New EEG showed epileptiform discharges in right temporal lobe. CSF showed cells 11 cells/mm3, protein 23 mg/dL, glucose 68 mg/dL and positive BOC. Brain MRI showed T2/FLAIR hyperintense lesions in subcortical WM in left temporo-occiptal lobe and left precuneus associated with SWI hypossinal areas that could represent hemosiderin deposition . Anti-NMDAR antibody was detected in serum and CSF. The patient received IVIG and MPIV with improvement. After 16 months she became impulsive, irritated, with difficulties in planning complex activities. Brain MRI showed multiple demyelinating lesions in brainstem, with leptomeningeal enhancement. CSF showed 6 cells, protein 26mg/dl, glucose 56 ml/dL, BOC +. Anti-MOG, anti-APQ4 and anti-NMDAR were negative. EEG showed rare epileptiform discharges in right frontoparietal lobe. According to 2017 MacDonald criteria she was diagnosed with Multiple Sclerosis (MS). HLA-DRB1*15:01, HLA-DQA1*01:02/06:02 were negative. She was treated with MPIV, and received rituximab. In After 22 months she developed dizziness, nausea, mild upper limb ataxia, pallor of optic disc with bilateral visual acuities of 20/50. EDSS was 2,0. Brain MRI showed new infratentorial and supratentorial demyelinating lesions. Alemtuzumab was indicated.

Discussion

We report a patient that developed MS after anti-NMDAR encephalitis, a rare association. An overlap between anti-NMDAR encephalitis and neuro-inflammatory disorders, mainly with NMOSD and MOGAD was described in a large series. However, association with MS is limited to only a few case-reports. Hypothesis for such association include: medication-induced autoimmunity, exposed epitopes during disease course, or EBV trigger.

In conclusion, recurrence of neurological symptoms in anti-NMDAR encephalitis, the presence of unspecific brain MRI lesions at first attack and BOC + should trigger additional investigation and raise the possibility of MS. A question is whether the overlap between anti-NMDAR encephalitis and the demyelinating disorders occurs by chance or whether they may be mechanistically linked