N-methyl-D-aspartate receptor autoimmune encephalitis presenting with opsoclonus-myoclonus: treatment response to plasmapheresis.

摘要: Objectives To report the clinical, laboratory, and radiographic features response to plasmapheresis in a patient with encephalopathy, opsoclonus, myoclonus whose cerebrospinal fluid was positive for N-methyl-D-aspartate receptor–IgG. Design Case report. Setting St Marys Hospital, Rochester, Minnesota. Patient A 27-year-old woman history of episodic migraine developed subacute progressive myoclonus, encephalopathy. Results Magnetic resonance imaging demonstrated nodular leptomeningeal enhancement superior cerebellar folia subsequent T2 hyperintensities periventricular regions amygdala. positron emission tomographic scan head predominantly frontotemporoparietal cortical hypometabolism sparing primary sensory motor cortices. Cerebrospinal examination revealed lymphocytic pleocytosis, mildly elevated protein level, IgG index, oligoclonal banding. Autoimmune screening neural-specific that bound synapse-rich mouse hippocampus granular layer; confirmed be receptor specific. No neoplasm detected by physical or whole-body computed tomography tomography. 5-day course high-dose intravenous methylprednisolone sodium succinate yielded limited improvement, subsequently required intensive care unit admission following pulseless electrical activity arrest associated pulmonary embolism. The encephalopathy improved dramatically after plasmapheresis. Conclusions This case highlights opsoclonus as manifestations autoimmune encephalitis setting novel appearance on tomography, it shows remarkable clinical