Moran, Joseph, DO

Prousi, George, MD

Smith, Stacey, MD, FACP

English

Lehigh Valley Health Network: LVHN Scholarly Works

Lehigh Valley Health NetworkLVHN Scholarly WorksDepartment of MedicineAMPA-R Antibody Positive AutoimmuneEncephalitis: An Under Recognized Cause ofAcute Mental Status Changes.Joseph Moran DOLehigh Valley Health Network, Joseph.Moran@lvhn.orgGeorge Prousi MDLehigh Valley Health Network, George.Prousi@lvhn.orgStacey Smith MD, FACPLehigh Valley Health Network, Stacey_J.Smith@lvhn.orgFollow this and additional works at: https://scholarlyworks.lvhn.org/medicinePart of the Internal Medicine Commons, and the Medical Sciences CommonsThis Poster is brought to you for free and open access by LVHN Scholarly Works. It has been accepted for inclusion in LVHN Scholarly Works by anauthorized administrator. For more information, please contact LibraryServices@lvhn.org.Published In/Presented AtMoran, J. Prousi, G. Smith, S. (2017, October 28). AMPA-R Antibody Positive Autoimmune Encephalitis: An Under Recognized Cause ofAcute Mental Status Changes. Poster Presented at: American College of Physicians (ACP), Harrisburg, PA.Joseph Moran DO; George Prousi MD and Stacey Smith MDAMPA-R Antibody Positive Autoimmune Encephalitis: An Under Recognized Cause of Acute Mental Status Changes© 2017 Lehigh Valley Health NetworkINTRODUCTIONDepartment of Medicine, Lehigh Valley Health Network, Allentown, Pennsylvania•  Considered to be a rare culprit of acute mental status changes, autoimmune encephalitis accounts for only 4% of encephalitis cases1•  The presentation of Anti-AMPA-R encephalitis is acute in nature and symptomatology consists of psychiatric symptoms, behavioral abnormalities and movement disorders•  Women over the age of 50 with known autoimmune disorders have been most commonly affected •  At present, 58% of women and 23% of men who develop this pathologic process are found to have an underlying malignancy2References:1.  Graus F, Boronat A, Xifro X, et al. The Expanding Clinical Profile Of Anti-Ampa Receptor Encephalitis. Neurology. 2010;74(10):857-859.2.  Hoftberger R, Sonderen AV, Leypoldt F, et al. Encephalitis and AMPA receptor antibodies: Novel findings in a case series of 22 patients. Neurology. 2015;84(24):2403-2412.3.  Lai M, Hughes EG, Peng X, et al. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Annals of Neurology. 2009;65(4):424-434.4.  Asztely F, Kumlien E. The diagnosis and treatment of limbic encephalitis. Acta Neurol Scand. 2012 Dec; 126(6):365-3755.  Griswold W, Giometto B, Vitaliani R, Obernforfer S. Current approaches to treatment of paraneoplastic encephalitis. Ther Adv Neurol Disord. 2011 Jul;4(4) 237-248CASE PRESENTATION•  A 60 year old female with a history of rheumatoid arthritis and depression presented with a one-month history of altered mental status, abnormal behavior and unsteady gait•  Presenting to an outside hospital, she underwent metabolic, infectious, and neuroimaging studies that were unremarkable. It was suspected that she had either a rapidly progressive dementia or a brief psychotic disorder triggered by severe depression•  Her mental status did not improve over a 15-day hospital course and she was transferred to our facility. On presentation the patient was not oriented to person place or time, was writhing, moaning and completely non verbal.•  Differentials included CJD, Vasculitis, and paraneoplastic / autoimmune encephalitis. Diagnostic studies returned showing positivity for Anti-AMPA-R antibodies, indicating the patient had a rare form of autoimmune encephalitis. Extensive work up for underlying malignancy was negative•  The patient made significant improvement with plasma exchange therapy, cyclophosphamide and steroids. Her mental status and behavior returned to baseline and she was discharged in stable conditionTREATMENT•  Treatment is largely based on the evaluation of case reports and case series with the majority of approaches revolving around combined therapies. •  Immunotherapy with either corticosteroids alone or in conjunction with IV immunoglobulin or plasma exchange is the mainstay of therapy4,5•  Second line therapies with rituximab, cyclophosphamide, or combined approach are possibilities for those who fail to improve within 4 weeks of immunotherapy4,5•  It should also be noted that given a significant association with primary tumor diagnoses, tumor removal is suggested and has lead to a reduction of antibody levels4,5DISCUSSION•  This case in combination with review of several case series in literature suggests that the detection of AMPA-R antibodies be considered in patients, particularly women, older than 50 years who present not only with limbic encephalitis but also with rapidly progressive abnormal behaviors resembling acute psychosis1•  This patient’s history of a previously diagnosed autoimmune disease should also have prompted consideration. Literature suggests that up to 50% of patients diagnosed with AMPA-R Ab positive autoimmune encephalitis had history of another systemic autoimmune disease2•  Early detection is crucial due to the high association AMPA-R Ab positive autoimmune encephalitis has with underlying malignancy.  An underlying tumor was discovered in 64% of patients in a case series of 22 patients2   •  Patients without malignancy tend to respond very well to immunotherapy; however, close follow up is indicated due to a relapse rate of up to 50% and diagnosis of a primary tumor has occurred during relapses2,3•  Overall, the prognosis for this patient is very favorable with literature showing a 100% 5 year survival in patients without underlying malignancy compared with a 50% 5 year survival in those with underlying malignancy2

AMPA-R Antibody Positive Autoimmune Encephalitis: An Under Recognized Cause of Acute Mental Status Changes.

https://scholarlyworks.lvhn.org/cgi/viewcontent.cgi?article=2479&context=medicine

AMPA-R Antibody Positive Autoimmune Encephalitis: An Under Recognized Cause of Acute Mental Status Changes.

Abstract

Similar works

Full text

Available Versions

Lehigh Valley Health Network: LVHN Scholarly Works