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

Jonathan H. Smith, Radhika Dhamija, Brian D. Moseley, Paola Sandroni, Claudia F Lucchinetti, Vanda A Lennon, Orhun H Kantarci

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

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

Original languageEnglish (US)
Pages (from-to)1069-1072
Number of pages4
JournalArchives of Neurology
Volume68
Issue number8
DOIs
StatePublished - Aug 2011

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Opsoclonus-Myoclonus Syndrome
Plasmapheresis
N-Methyl-D-Aspartate Receptors
Brain Diseases
Immunoglobulin G
Cerebrospinal Fluid
Methylprednisolone Hemisuccinate
Leukocytosis
Motor Cortex
Encephalitis
Therapeutics
Amygdala
Migraine Disorders
Pulmonary Embolism
Positron-Emission Tomography
Synapses
Physical Examination
Intensive Care Units
Hippocampus
Head

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

N-methyl-D-aspartate receptor autoimmune encephalitis presenting with opsoclonus-myoclonus : Treatment response to plasmapheresis. / Smith, Jonathan H.; Dhamija, Radhika; Moseley, Brian D.; Sandroni, Paola; Lucchinetti, Claudia F; Lennon, Vanda A; Kantarci, Orhun H.

In: Archives of Neurology, Vol. 68, No. 8, 08.2011, p. 1069-1072.

Research output: Contribution to journalArticle

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