Autoimmune Encephalitis With Ganglionic a3-AChR Antibodies

Objective To describe a case of autoimmune encephalitis presented as new onset refractory nonconvulsive status epilepticus and autonomic failure caused by alpha-3 ganglionic acetylcholine receptor autoantibodies (a3-AChR Ab). Background Autoimmune ganglionopathy (AAG) is rare acquired dysautonomia in adults due to a3-AChR Ab. A patient typically presents with subacute dysfunction, has not been reported yet. Design/Methods NA. Results 60 years old female progressive worsening mental for two weeks. Vital signs were normal. The was somnolent, nonverbal, tonic non-reactive pupils on the exam. CT head showed multiple subcortical hypodensities. MRI brain demonstrated T2-FLAIR hyperintense lesions bifrontal lobes sparing U-fibers, abnormal leptomeningeal/pial perivenular type enhancement. C/T spine, chest/Abd/pelvis negative. Although serum CSF testing inflammatory markers, meningoencephalitis panel negative, except Paraneoplastic positive high titer Abs. EEG epilepticus, which required management three antiseizure medications. Initially, had little response empirical pulse methylprednisolone therapy. Later good clinical plasmapheresis. she nonobstructive small bowel obstruction, resolved another steroid therapy, discharged dexamethasone taper. On three-month follow-up, near remission, supported repeat MRI. She never relapsed after follow-up. Conclusions Studies have shown that Abs can result diverse neurological manifestations, mainly dysautonomia. Our encephalitis, dysautonomia, epilepticus. Scans suggested diffuse leukoencephalopathy, found 3-ACHR antibody seropositivity. steroid-resistant but improvement We described an interesting likely associated Ab previously described. More studies are confirm this association.