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category: "Brain"
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documentVersionId: "cc8c3891-88b7-44bb-9e2a-3a321d092f4c"
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imageCount: 22
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isBookmarked: false
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isComparable: true
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isInCompareCart: false
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lastUpdated: "08/05/20"
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pageDescription: "Autoimmune Encephalitis"
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pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Autoimmune Encephalitis"
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pageTitle: "Autoimmune Encephalitis | STATdx"
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enhancedTitle: "Autoimmune Encephalitis"
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type: "DX"
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references: true
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breadcrumbs:
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- "Brain"
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- "Diagnosis"
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@@ -354,39 +352,123 @@ breadcrumbs:
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89d11c00-03ef-4d5a-aea9-2d9c2669fa58
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## References
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# Selected References
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1. [Iorio R et al: Clinical characteristics and outcome of patients with autoimmune encephalitis: clues for paraneoplastic etiology. Eur J Neurol. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32412135%5Bpmid%5D)
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1. [Mongay-Ochoa N et al: Anti-Hu-associated paraneoplastic syndromes triggered by immune-checkpoint inhibitor treatment. J Neurol. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32451614%5Bpmid%5D)
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1. [Vogrig A et al: Central nervous system complications associated with immune checkpoint inhibitors. J Neurol Neurosurg Psychiatry. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32312871%5Bpmid%5D)
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1. [Bradshaw MJ et al: An overview of autoimmune and paraneoplastic encephalitides. Semin Neurol. 38(3):330-43, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30011413%5Bpmid%5D)
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1. [Long JM et al: Autoimmune dementia. Semin Neurol. 38(3):303-15, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30011411%5Bpmid%5D)
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1. [Kelley BP et al: Autoimmune encephalitis: pathophysiology and imaging review of an overlooked diagnosis. AJNR Am J Neuroradiol. 38(6):1070-8, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28183838%5Bpmid%5D)
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1. [da Rocha AJ et al: Recognizing Autoimmune-Mediated Encephalitis in the Differential Diagnosis of Limbic Disorders. AJNR Am J Neuroradiol. 36(12):2196-205, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26381566%5Bpmid%5D)
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1. [Höftberger R et al: Encephalitis and AMPA receptor antibodies: novel findings in a case series of 22 patients. Neurology. 84(24):2403-12, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25979696%5Bpmid%5D)
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1. [Thomas AC et al: Autoimmune limbic encephalitis detected on FDG brain scan performed for the evaluation of dementia. Clin Nucl Med. 40(4):358-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25608163%5Bpmid%5D)
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1. [Zekeridou A et al: Treatment and outcome of children and adolescents with N-methyl-D-aspartate receptor encephalitis. J Neurol. 262(8):1859-66, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25987208%5Bpmid%5D)
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1. [Kotsenas AL et al: MRI findings in autoimmune voltage-gated potassium channel complex encephalitis with seizures: one potential etiology for mesial temporal sclerosis. AJNR Am J Neuroradiol. 35(1):84-9, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=23868165%5Bpmid%5D)
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1. [Masangkay N et al: Brain 18F-FDG-PET characteristics in patients with paraneoplastic neurological syndrome and its correlation with clinical and MRI findings. Nucl Med Commun. 35(10):1038-46, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25023997%5Bpmid%5D)
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1. [Sarria-Estrada S et al: Neuroimaging in status epilepticus secondary to paraneoplastic autoimmune encephalitis. Clin Radiol. 69(8):795-803, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24824979%5Bpmid%5D)
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1. [Baumgartner A et al: Cerebral FDG-PET and MRI findings in autoimmune limbic encephalitis: correlation with autoantibody types. J Neurol. 260(11):2744-53, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23900756%5Bpmid%5D)
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1. [Aye MM et al: CD8 positive T-cell infiltration in the dentate nucleus of paraneoplastic cerebellar degeneration. J Neuroimmunol. 208(1-2):136-40, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19217169%5Bpmid%5D)
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1. [Berger JR et al: A brainstem paraneoplastic syndrome associated with prostate cancer. J Neurol Neurosurg Psychiatry. 80(8):934-5, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19608787%5Bpmid%5D)
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1. [Damek DM: Cerebral edema, altered mental status, seizures, acute stroke, leptomeningeal metastases, and paraneoplastic syndrome. Emerg Med Clin North Am. 27(2):209-29, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19447307%5Bpmid%5D)
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1. [Honnorat J et al: Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies. J Neurol Neurosurg Psychiatry. 80(4):412-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18931014%5Bpmid%5D)
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1. [Jaster JH: Reversible autoimmune encephalopathy spectrum. Arch Neurol. 66(7):916, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19597101%5Bpmid%5D)
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1. [Khan NL et al: Histopathology of VGKC antibody-associated limbic encephalitis. Neurology. 72(19):1703-5, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19433746%5Bpmid%5D)
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1. [Kröll-Seger J et al: Non-paraneoplastic limbic encephalitis associated with antibodies to potassium channels leading to bilateral hippocampal sclerosis in a pre-pubertal girl. Epileptic Disord. 11(1):54-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19251579%5Bpmid%5D)
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1. [McKeon A et al: Reversible extralimbic paraneoplastic encephalopathies with large abnormalities on magnetic resonance images. Arch Neurol. 66(2):268-71, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19204167%5Bpmid%5D)
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1. [Modoni A et al: Successful treatment of acute autoimmune limbic encephalitis with negative VGKC and NMDAR antibodies. Cogn Behav Neurol. 22(1):63-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19372772%5Bpmid%5D)
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1. [Saiz A et al: Anti-Hu-associated brainstem encephalitis. J Neurol Neurosurg Psychiatry. 80(4):404-7, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19015226%5Bpmid%5D)
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1. [Uribe-Uribe NO et al: Paraneoplastic sensory neuropathy associated with small cell carcinoma of the gallbladder. Ann Diagn Pathol. 13(2):124-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19302962%5Bpmid%5D)
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1. [Anderson NE et al: Limbic encephalitis - a review. J Clin Neurosci. 15(9):961-71, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18411052%5Bpmid%5D)
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1. [Basu S et al: Role of FDG-PET in the clinical management of paraneoplastic neurological syndrome: detection of the underlying malignancy and the brain PET-MRI correlates. Mol Imaging Biol. 10(3):131-7, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18297363%5Bpmid%5D)
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1. [Blaes F et al: Autoantibodies in childhood opsoclonus-myoclonus syndrome. J Neuroimmunol. 201-202:221-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18687475%5Bpmid%5D)
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1. [Corapcioglu F et al: Response to rituximab and prednisolone for opsoclonus-myoclonus-ataxia syndrome in a child with ganglioneuroblastoma. Pediatr Hematol Oncol. 25(8):756-61, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19065442%5Bpmid%5D)
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1. [Dalakas MC: Invited article: inhibition of B cell functions: implications for neurology. Neurology. 70(23):2252-60, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18519875%5Bpmid%5D)
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1. [Eker A et al: Testicular teratoma and anti-N-methyl-D-aspartate receptor-associated encephalitis. J Neurol Neurosurg Psychiatry. 79(9):1082-3, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18708569%5Bpmid%5D)
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1. [Fitzpatrick AS et al: Opsoclonus-myoclonus syndrome associated with benign ovarian teratoma. Neurology. 70(15):1292-3, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18391162%5Bpmid%5D)
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1. [Geschwind MD et al: Voltage-gated potassium channel autoimmunity mimicking creutzfeldt-jakob disease. Arch Neurol. 65(10):1341-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18852349%5Bpmid%5D)
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1. [Hassan KA et al: Long-term survival in paraneoplastic opsoclonus-myoclonus syndrome associated with small cell lung cancer. J Neuroophthalmol. 28(1):27-30, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18347455%5Bpmid%5D)
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1. [Jarius S et al: Relative frequency of VGKC and 'classical' paraneoplastic antibodies in patients with limbic encephalitis. J Neurol. 255(7):1100-1, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18574619%5Bpmid%5D)
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1. [Johnson V et al: Immune mediated neurologic dysfunction as a paraneoplastic syndrome in renal cell carcinoma. J Neurooncol. 90(3):279-81, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18726185%5Bpmid%5D)
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1. [Ko MW et al: Neuro-ophthalmologic manifestations of paraneoplastic syndromes. J Neuroophthalmol. 28(1):58-68, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18347462%5Bpmid%5D)
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1. [Musunuru K et al: Paraneoplastic opsoclonus-myoclonus ataxia associated with non-small-cell lung carcinoma. J Neurooncol. 90(2):213-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18618225%5Bpmid%5D)
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1. [Novillo-López ME et al: Treatment-responsive subacute limbic encephalitis and NMDA receptor antibodies in a man. Neurology. 70(9):728-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18299525%5Bpmid%5D)
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1. [Pellkofer HL et al: Lambert-eaton myasthenic syndrome differential reactivity of tumor versus non-tumor patients to subunits of the voltage-gated calcium channel. J Neuroimmunol. 204(1-2):136-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18809213%5Bpmid%5D)
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1. [Sabater L et al: SOX1 antibodies are markers of paraneoplastic Lambert-Eaton myasthenic syndrome. Neurology. 70(12):924-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18032743%5Bpmid%5D)
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1. [Sekiguchi Y et al: Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction. J Neurol Sci. 269(1-2):176-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18378260%5Bpmid%5D)
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1. [Tan KM et al: Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology. 70(20):1883-90, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18474843%5Bpmid%5D)
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1. [Darnell RB et al: Paraneoplastic syndromes affecting the nervous system. Semin Oncol. 33(3):270-98, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16769417%5Bpmid%5D)
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1. [Dropcho EJ: Update on paraneoplastic syndromes. Curr Opin Neurol. 18(3):331-6, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15891421%5Bpmid%5D)
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1. [Dadparvar S et al: Paraneoplastic encephalitis associated with cystic teratoma is detected by fluorodeoxyglucose positron emission tomography with negative magnetic resonance image findings. Clin Nucl Med. 28(11):893-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14578703%5Bpmid%5D)
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1. [Messori A et al: Resolution of limbic encephalitis with detection and treatment of lung cancer: clinical-radiological correlation. Eur J Radiol. 45(1): 78-80, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12499067%5Bpmid%5D)
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1. [Barnett M et al: Paraneoplastic brain stem encephalitis in a woman with anti-Ma2 antibody. J Neurol Neurosurg Psychiatry. 70(2):222-5, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11160472%5Bpmid%5D)
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1. [Gultekin SH et al: Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 123 ( Pt 7):1481-94, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10869059%5Bpmid%5D)
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1. [Dalmau J et al: Paraneoplastic neurologic syndromes: pathogenesis and physiopathology. Brain Pathol. 9(2):275-84, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10219745%5Bpmid%5D)
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1. [Scaravilli F et al: The neuropathology of paraneoplastic syndromes. Brain Pathol. 9(2):251-60, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10219743%5Bpmid%5D)
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1. [Voltz R et al: A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med. 340(23): 1788-95, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10362822%5Bpmid%5D)
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## Images
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### Selected Images
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*Axial FLAIR MR shows abnormal hyperintensity in the bilateral medial temporal lobes <img src='/img/arrows/CS.png'/>, characteristic of limbic encephalitis (LE), the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.*
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*Axial FLAIR MR shows abnormal hyperintensity in the bilateral medial temporal lobes <img src='/img/arrows/CS.png'/>, characteristic of limbic encephalitis (LE), the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.*
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*Axial FLAIR MR shows abnormal hyperintensity in the bilateral medial temporal lobes <img src='/img/arrows/CS.png'/>, characteristic of limbic encephalitis (LE), the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.*
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*Axial T1 C+ MR in the same patient shows no significant enhancement in the medial temporal lobes. Enhancement is often present in limbic encephalitis. The patient's symptoms often improve after treatment of the primary tumor.*
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*Axial T1 C+ MR in the same patient shows no significant enhancement in the medial temporal lobes. Enhancement is often present in limbic encephalitis. The patient's symptoms often improve after treatment of the primary tumor.*
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*Axial FLAIR MR in a 61 year old with multiple myeloma who presented with seizures shows abnormal hyperintensity <img src='/img/arrows/CS.png'/> in the cortex and subcortical white matter of the temporal lobes.*
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*Axial FLAIR MR in a 61 year old with multiple myeloma who presented with seizures shows abnormal hyperintensity <img src='/img/arrows/CS.png'/> in the cortex and subcortical white matter of the temporal lobes.*
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*Axial T1 C+ FS MR in the same patient shows no enhancement. Differential considerations include autoimmune encephalitis (AE), acute demyelinating encephalomyelitis (ADEM), viral encephalitis, and vasculitis in this case. AE related to GABAr was diagnosed by CSF and serum markers.*
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*Axial T1 C+ FS MR in the same patient shows no enhancement. Differential considerations include autoimmune encephalitis (AE), acute demyelinating encephalomyelitis (ADEM), viral encephalitis, and vasculitis in this case. AE related to GABAr was diagnosed by CSF and serum markers.*
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*Axial FLAIR MR in a 71 year old with altered mental status shows abnormal hyperintensity in the left temporal lobe <img src='/img/arrows/CS.png'/>. Differential considerations include infectious, inflammatory, and neoplastic etiologies. GAD65 AE was diagnosed at brain biopsy.*
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*Axial FLAIR MR in a 71 year old with altered mental status shows abnormal hyperintensity in the left temporal lobe <img src='/img/arrows/CS.png'/>. Differential considerations include infectious, inflammatory, and neoplastic etiologies. GAD65 AE was diagnosed at brain biopsy.*
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*Axial FLAIR MR shows hyperintensity in the medial temporal lobes <img src='/img/arrows/CS.png'/> in this patient with subacute dementia and voltage-gated potassium channel (VGKC) autoimmunity. VGKC may occur with or without a primary neoplasm and may have an LE pattern.*
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*Axial FLAIR MR shows hyperintensity in the medial temporal lobes <img src='/img/arrows/CS.png'/> in this patient with subacute dementia and voltage-gated potassium channel (VGKC) autoimmunity. VGKC may occur with or without a primary neoplasm and may have an LE pattern.*
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*Axial T2 MR shows midbrain hyperintensity <img src='/img/arrows/CS.png'/> related to brainstem encephalitis, which is characterized by hyperintensity in the midbrain, pons, cerebellar peduncle, and basal ganglia.*
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*Axial T2 MR shows midbrain hyperintensity <img src='/img/arrows/CS.png'/> related to brainstem encephalitis, which is characterized by hyperintensity in the midbrain, pons, cerebellar peduncle, and basal ganglia.*
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*Axial T1 C+ MR in the same patient shows patchy enhancement of the midbrain lesions <img src='/img/arrows/CS.png'/> and the medial temporal lobe <img src='/img/arrows/CO.png'/>. This patient was diagnosed with LE with new brainstem symptoms. Multiple paraneoplastic syndromes may occur in the same patient.*
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*Axial T1 C+ MR in the same patient shows patchy enhancement of the midbrain lesions <img src='/img/arrows/CS.png'/> and the medial temporal lobe <img src='/img/arrows/CO.png'/>. This patient was diagnosed with LE with new brainstem symptoms. Multiple paraneoplastic syndromes may occur in the same patient.*
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*Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, related to LE. As imaging mimics herpes encephalitis, most patients are initially treated with antiviral therapy until HSV titers are found to be negative. < 1% of cancer patients develop a paraneoplastic syndrome.*
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*Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, related to LE. As imaging mimics herpes encephalitis, most patients are initially treated with antiviral therapy until HSV titers are found to be negative. < 1% of cancer patients develop a paraneoplastic syndrome.*
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