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Abnormal neurons in teratomas in NMDAR encephalitis.
JAMA Neurol. 2014 Jun; 71(6):717-24.JN

Abstract

IMPORTANCE

Ovarian teratomas are frequently described in patients with N-methyl-d-aspartate receptor (NMDAR) encephalitis, yet NMDAR encephalitis is rarely described in patients with ovarian teratomas. Understanding why a minority of patients with teratomas are seen with autoimmune encephalitis may improve the management of NMDAR encephalitis and other teratoma-associated autoimmune diseases.

OBJECTIVE

To characterize the unique organization of neuroglial elements within ovarian teratomas resected from patients with NMDAR encephalitis.

DESIGN

Case-control study comparing the pathological features of ovarian teratomas resected from consecutively accrued cases with NMDAR encephalitis between January 1, 2009, and December 15, 2013, and ovarian teratomas resected from controls between June 1, 2012, and June 30, 2013.

SETTING

Pathology tissue database at a tertiary academic care center.

PARTICIPANTS

Five cases with teratoma-associated NMDAR encephalitis and serum or cerebrospinal fluid autoantibodies against central nervous system (CNS) NMDAR and 38 controls (39 ovarian teratomas) without neurological symptoms or signs.

EXPOSURES

Formalin-fixed, paraffin-embedded ovarian teratomas were examined for the presence of CNS tissue and inflammatory infiltrates using direct microscopy, enhanced with standard histopathological and immunological stains.

MAIN OUTCOMES AND MEASURES

Frequency of detection of atypical (dysplastic) CNS neuronal elements in ovarian teratomas resected from cases vs controls, as well as characterization of the relationship between atypical neurons and immune infiltrates.

RESULTS

Central nervous system neuronal elements were detected in 4 of 5 teratomas resected from cases with NMDAR encephalitis and in 20 of 39 controls (P = .36). Atypical neurons were seen within teratomas resected from 4 of 5 cases but not in 39 controls, reliably distinguishing teratomas associated with NMDAR encephalitis (P < .001). If found within the CNS, these histological abnormalities would have received the diagnosis of gangliogliomas (n = 3) and ganglioneuroblastoma (n = 1). Reactive changes were present in teratomas from controls, including ferruginated neurons and Rosenthal fibers. Abnormal neuroglial elements were closely related to immune infiltrates in teratomas resected from 4 of 4 cases. Inflammatory infiltrates were not associated with neuroglial tissue in 20 controls, further differentiating these populations (P < .001).

CONCLUSIONS AND RELEVANCE

Abnormal neurons within teratomas distinguish cases with NMDAR encephalitis from controls and may promote the development of autoimmunity.

Authors+Show Affiliations

Division of Neurology, University of Toronto, Toronto, Ontario, Canada2University Health Network Memory Clinic, Toronto Western Hospital, Toronto, Ontario, Canada.Division of Pathology, Department of Laboratory Medicine, Saint Michael's Hospital, Toronto, Ontario, Canada4Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.Division of Neurology, University of Toronto, Toronto, Ontario, Canada2University Health Network Memory Clinic, Toronto Western Hospital, Toronto, Ontario, Canada.Division of Pathology, Department of Laboratory Medicine, Saint Michael's Hospital, Toronto, Ontario, Canada4Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

24781184

Citation

Day, Gregory S., et al. "Abnormal Neurons in Teratomas in NMDAR Encephalitis." JAMA Neurology, vol. 71, no. 6, 2014, pp. 717-24.
Day GS, Laiq S, Tang-Wai DF, et al. Abnormal neurons in teratomas in NMDAR encephalitis. JAMA Neurol. 2014;71(6):717-24.
Day, G. S., Laiq, S., Tang-Wai, D. F., & Munoz, D. G. (2014). Abnormal neurons in teratomas in NMDAR encephalitis. JAMA Neurology, 71(6), 717-24. https://doi.org/10.1001/jamaneurol.2014.488
Day GS, et al. Abnormal Neurons in Teratomas in NMDAR Encephalitis. JAMA Neurol. 2014;71(6):717-24. PubMed PMID: 24781184.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Abnormal neurons in teratomas in NMDAR encephalitis. AU - Day,Gregory S, AU - Laiq,Simin, AU - Tang-Wai,David F, AU - Munoz,David G, PY - 2014/5/1/entrez PY - 2014/5/2/pubmed PY - 2014/8/16/medline SP - 717 EP - 24 JF - JAMA neurology JO - JAMA Neurol VL - 71 IS - 6 N2 - IMPORTANCE: Ovarian teratomas are frequently described in patients with N-methyl-d-aspartate receptor (NMDAR) encephalitis, yet NMDAR encephalitis is rarely described in patients with ovarian teratomas. Understanding why a minority of patients with teratomas are seen with autoimmune encephalitis may improve the management of NMDAR encephalitis and other teratoma-associated autoimmune diseases. OBJECTIVE: To characterize the unique organization of neuroglial elements within ovarian teratomas resected from patients with NMDAR encephalitis. DESIGN: Case-control study comparing the pathological features of ovarian teratomas resected from consecutively accrued cases with NMDAR encephalitis between January 1, 2009, and December 15, 2013, and ovarian teratomas resected from controls between June 1, 2012, and June 30, 2013. SETTING: Pathology tissue database at a tertiary academic care center. PARTICIPANTS: Five cases with teratoma-associated NMDAR encephalitis and serum or cerebrospinal fluid autoantibodies against central nervous system (CNS) NMDAR and 38 controls (39 ovarian teratomas) without neurological symptoms or signs. EXPOSURES: Formalin-fixed, paraffin-embedded ovarian teratomas were examined for the presence of CNS tissue and inflammatory infiltrates using direct microscopy, enhanced with standard histopathological and immunological stains. MAIN OUTCOMES AND MEASURES: Frequency of detection of atypical (dysplastic) CNS neuronal elements in ovarian teratomas resected from cases vs controls, as well as characterization of the relationship between atypical neurons and immune infiltrates. RESULTS: Central nervous system neuronal elements were detected in 4 of 5 teratomas resected from cases with NMDAR encephalitis and in 20 of 39 controls (P = .36). Atypical neurons were seen within teratomas resected from 4 of 5 cases but not in 39 controls, reliably distinguishing teratomas associated with NMDAR encephalitis (P < .001). If found within the CNS, these histological abnormalities would have received the diagnosis of gangliogliomas (n = 3) and ganglioneuroblastoma (n = 1). Reactive changes were present in teratomas from controls, including ferruginated neurons and Rosenthal fibers. Abnormal neuroglial elements were closely related to immune infiltrates in teratomas resected from 4 of 4 cases. Inflammatory infiltrates were not associated with neuroglial tissue in 20 controls, further differentiating these populations (P < .001). CONCLUSIONS AND RELEVANCE: Abnormal neurons within teratomas distinguish cases with NMDAR encephalitis from controls and may promote the development of autoimmunity. SN - 2168-6157 UR - https://www.unboundmedicine.com/medline/citation/24781184/Abnormal_neurons_in_teratomas_in_NMDAR_encephalitis_ L2 - https://jamanetwork.com/journals/jamaneurology/fullarticle/10.1001/jamaneurol.2014.488 DB - PRIME DP - Unbound Medicine ER -