Encephalitis and Myelitis in a Young Woman: Overlap Syndrome, Thyroiditis, and Occult Tumor From the National Multiple Sclerosis Society Case Conference Proceedings

Published on Jun 23, 2021in Neuroimmunology and Neuroinflammation7.724
· DOI :10.1212/NXI.0000000000001026
Neda Zarghami Esfanhani (KP: Kaiser Permanente), Annette Wundes (KP: Kaiser Permanente)+ 7 AuthorsScott D. Newsome (KP: Kaiser Permanente)
A 31-year-old woman presented with fever, ataxia, double vision, confusion, and auditory hallucinations, preceded by 2 weeks of cough, increased oral secretions, and dermatomal rash. Her medical history was unremarkable. Her family history was notable for first-degree relatives with autoimmune
#2Mar GuaspH-Index: 5
Last. Josep DalmauH-Index: 121
view all 13 authors...
Objective To determine the frequency and significance of concurrent glial (glial-Ab) or neuronal-surface (NS-Ab) antibodies in patients with anti–NMDA receptor (NMDAR) encephalitis. Methods Patients were identified during initial routine screening of a cohort (C1) of 646 patients consecutively diagnosed with anti-NMDAR encephalitis and another cohort (C2) of 200 patients systematically rescreened. Antibodies were determined with rat brain immunostaining and cell-based assays. Results Concurrent ...
36 CitationsSource
#1Michael J. Bradshaw (Harvard University)H-Index: 8
#2Robert P. Lisak (WSU: Wayne State University)H-Index: 66
Last. Elliot M. FrohmanH-Index: 81
view all 13 authors...
A 17-year-old man was brought to the emergency department with several weeks of irritability, insomnia, and depression, followed by 1 week of nonsensical speech and visual, nonthreatening hallucinations. His medical history included depression and Sydenham chorea diagnosed when he was aged 12 years with positive ASO titer and anti-DNAse B antibodies. The National Multiple Sclerosis Society Case Conference Proceedings are both a live educational Webinar, in conjunction with a peer review publishi...
1 CitationsSource
#1Francesc GrausH-Index: 116
#2Maarten J. Titulaer (EUR: Erasmus University Rotterdam)H-Index: 51
Last. J. Dalmau (UPenn: University of Pennsylvania)H-Index: 8
view all 28 authors...
Summary Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. However, existing criteria for autoimmune encephalitis are too reliant on antibody testing and response to immunotherapy, which might delay the diagnosis. We reviewed the li...
1,444 CitationsSource
#1Manan Shah (University of Texas Health Science Center at San Antonio)H-Index: 4
#2R Bhavaraju-Sanka (University of Texas Health Science Center at San Antonio)
1 CitationsSource
#1Sarah Zubkov (NYU: New York University)H-Index: 2
#2Puja Aggarwal Joshi (NYU: New York University)H-Index: 1
Last. Sanjeev V. Kothare (NYU: New York University)H-Index: 14
view all 4 authors...
A 17-year-old girl presented with a month of anxiety followed by acute psychosis, catatonia, choreoathetosis, seizures, autonomic instability, lower extremity spasticity, and hyperreflexia. CSF was positive for the NMDA antibody; additional antibodies and infectious etiologies tested negative. MRI brain was normal. MRI spine revealed symmetric T2 hyperintensities in ventral gray matter (figure). EEG showed extreme delta brush. She received the following sequentially: IV gammaglobulin, methylpred...
2 CitationsSource
#1Maarten J. Titulaer (University of Barcelona)H-Index: 51
#2Romana Höftberger (University of Barcelona)H-Index: 53
Last. Josep Dalmau (Catalan Institution for Research and Advanced Studies)H-Index: 121
view all 21 authors...
Objective To report the clinical, radiological, and immunological association of demyelinating disorders with anti–N-methyl-D-aspartate receptor (NMDAR) encephalitis. Methods Clinical and radiological analysis was done of a cohort of 691 patients with anti-NMDAR encephalitis. Determination of antibodies to NMDAR, aquaporin-4 (AQP4), and myelin oligodendrocyte glycoprotein (MOG) was performed using brain immunohistochemistry and cell-based assays. Results Twenty-three of 691 patients with anti-NM...
297 CitationsSource
#1Marina FrasquetH-Index: 6
#2Luis BatallerH-Index: 21
Last. Bonaventura CasanovaH-Index: 26
view all 10 authors...
Abstract Paraneoplastic myelitis is a rare inflammatory disorder most frequently associated with solid tumors or lymphoproliferative disorders. Patients often harbor onconeuronal antibodies and their prognosis is usually poor. Here we report a 42-year old woman with longitudinally extensive transverse myelitis and aquaporin-4 (AQP4) antibodies that led to the diagnosis of ovarian teratoma. After tumor removal and immune therapy (including corticosteroids, plasma exchange, intravenous immunoglobu...
31 CitationsSource
#1Maarten J. Titulaer (University of Barcelona)H-Index: 51
#2Lindsey McCracken (HUP: Hospital of the University of Pennsylvania)H-Index: 8
Last. Josep Dalmau (University of Barcelona)H-Index: 121
view all 19 authors...
Summary Background Anti-NMDA receptor (NMDAR) encephalitis is an autoimmune disorder in which the use of immunotherapy and the long-term outcome have not been defined. We aimed to assess the presentation of the disease, the spectrum of symptoms, immunotherapies used, timing of improvement, and long-term outcome. Methods In this multi-institutional observational study, we tested for the presence of NMDAR antibodies in serum or CSF samples of patients with encephalitis between Jan 1, 2007, and Jan...
1,606 CitationsSource
#1Sarah E. Schmitt (HUP: Hospital of the University of Pennsylvania)H-Index: 10
#2Kimberly PargeonH-Index: 3
Last. Daniel FriedmanH-Index: 70
view all 6 authors...
Objectives: To determine continuous EEG (cEEG) patterns that may be unique to anti-NMDA receptor (NMDAR) encephalitis in a series of adult patients with this disorder. Methods: We evaluated the clinical and EEG data of 23 hospitalized adult patients with anti-NMDAR encephalitis who underwent cEEG monitoring between January 2005 and February 2011 at 2 large academic medical centers. Results: Twenty-three patients with anti-NMDAR encephalitis underwent a median of 7 (range 1−123) days of cEEG moni...
443 CitationsSource
#1Josep Dalmau (UPenn: University of Pennsylvania)H-Index: 121
#2Amy J. Gleichman (UPenn: University of Pennsylvania)H-Index: 13
Last. David A. Lynch (UPenn: University of Pennsylvania)H-Index: 124
view all 10 authors...
Summary Background A severe form of encephalitis associated with antibodies against NR1–NR2 heteromers of the NMDA receptor was recently identified. We aimed to analyse the clinical and immunological features of patients with the disorder and examine the effects of antibodies against NMDA receptors in neuronal cultures. Methods We describe the clinical characteristics of 100 patients with encephalitis and NR1–NR2 antibodies. HEK293 cells ectopically expressing single or assembled NR1–NR2 subunit...
2,096 CitationsSource
Cited By1