Publications by authors named "Elia Sechi"

47 Publications

Autoimmune encephalopathies presenting as dementia of subacute onset and rapid progression.

Ther Adv Neurol Disord 2021 19;14:1756286421998906. Epub 2021 Mar 19.

Departments of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.

The terms autoimmune dementia and autoimmune encephalopathy may be used interchangeably; autoimmune dementia is used here to emphasize its consideration in young-onset dementia, dementia with a subacute onset, and rapidly progressive dementia. Given their potential for reversibility, it is important to distinguish the rare autoimmune dementias from the much more common neurodegenerative dementias. The presence of certain clinical features [e.g. facio-brachial dystonic seizures that accompany anti-leucine-rich-glioma-inactivated-1 (LGI1) encephalitis that can mimic myoclonus] can be a major clue to the diagnosis. When possible, objective assessment of cognition with bedside testing or neuropsychological testing is useful to determine the degree of abnormality and serve as a baseline from which immunotherapy response can be judged. Magnetic resonance imaging (MRI) head and cerebrospinal fluid (CSF) analysis are useful to assess for inflammation that can support an autoimmune etiology. Assessing for neural autoantibody diagnostic biomarkers in serum and CSF in those with suggestive features can help confirm the diagnosis and guide cancer search in paraneoplastic autoimmune dementia. However, broad screening for neural antibodies in elderly patients with an insidious dementia is not recommended. Moreover, there are pitfalls to antibody testing that should be recognized and the high frequency of some antibodies in the general population limit their diagnostic utility [e.g., anti-thyroid peroxidase (TPO) antibodies]. Once the diagnosis is confirmed, both acute and maintenance immunotherapy can be utilized and treatment choice varies depending on the accompanying neural antibody present and the presence or absence of cancer. The target of the neural antibody biomarker may help predict treatment response and prognosis, with antibodies to cell-surface or synaptic antigens more responsive to immunotherapy and yielding a better overall prognosis than those with antibodies to intracellular targets. Neurologists should be aware that autoimmune dementias and encephalopathies are increasingly recognized in novel settings, including post herpes virus encephalitis and following immune-checkpoint inhibitor use.
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http://dx.doi.org/10.1177/1756286421998906DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7983436PMC
March 2021

Clinical spectrum of high-titre GAD65 antibodies.

J Neurol Neurosurg Psychiatry 2021 Feb 9. Epub 2021 Feb 9.

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA

Objective: To determine clinical manifestations, immunotherapy responsiveness and outcomes of glutamic acid decarboxylase-65 (GAD65) neurological autoimmunity.

Methods: We identified 323 Mayo Clinic patients with high-titre (>20 nmol/L in serum) GAD65 antibodies out of 380 514 submitted anti-GAD65 samples (2003-2018). Patients classified as having GAD65 neurological autoimmunity after chart review were analysed to determine disease manifestations, immunotherapy responsiveness and predictors of poor outcome (modified Rankin score >2).

Results: On review, 108 patients were classified as not having GAD65 neurological autoimmunity and 3 patients had no more likely alternative diagnoses but atypical presentations (hyperkinetic movement disorders). Of remaining 212 patients with GAD65 neurological autoimmunity, median age at symptom onset was 46 years (range: 5-83 years); 163/212 (77%) were female. Stiff-person spectrum disorders (SPSD) (N=71), cerebellar ataxia (N=55), epilepsy (N=35) and limbic encephalitis (N=7) could occur either in isolation or as part of an overlap syndrome (N=44), and were designated core manifestations. Cognitive impairment (N=38), myelopathy (N=23) and brainstem dysfunction (N=22) were only reported as co-occurring phenomena, and were designated secondary manifestations. Sustained response to immunotherapy ranged from 5/20 (25%) in epilepsy to 32/44 (73%) in SPSD (p=0.002). Complete immunotherapy response occurred in 2/142 (1%). Cerebellar ataxia and serum GAD65 antibody titre >500 nmol/L predicted poor outcome.

Interpretation: High-titre GAD65 antibodies were suggestive of, but not pathognomonic for GAD65 neurological autoimmunity, which has discrete core and secondary manifestations. SPSD was most likely to respond to immunotherapy, while epilepsy was least immunotherapy responsive. Complete immunotherapy response was rare. Serum GAD65 antibody titre >500 nmol/L and cerebellar ataxia predicted poor outcome.
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http://dx.doi.org/10.1136/jnnp-2020-325275DOI Listing
February 2021

Serum Neurofilament to Magnetic Resonance Imaging Lesion Area Ratio Differentiates Spinal Cord Infarction From Acute Myelitis.

Stroke 2021 Jan 11;52(2):645-654. Epub 2021 Jan 11.

Departments of Neurology (E.S., A.M., S.J.P., E.P.F., A.A.R., D.M.N., N.L.Z.), Mayo Clinic, Rochester.

Background And Purpose: The diagnosis of spontaneous spinal cord infarction (SCI) is limited by the lack of diagnostic biomarkers and MRI features that often overlap with those of other myelopathies, especially acute myelitis. We investigated whether the ratio between serum neurofilament light chain levels and MRI T2-lesion area (neurofilament light chain/area ratio-NAR) differentiates SCI from acute myelitis of similar severity.

Methods: We retrospectively identified Mayo Clinic patients (January 1, 2000-December 31, 2019) with (1) SCI, (2) AQP4 (aquaporin 4)-IgG or MOG (myelin oligodendrocyte glycoprotein)-IgG-associated myelitis at disease clinical presentation, or (3) idiopathic transverse myelitis from a previously identified population-based cohort of patients seronegative for AQP4-IgG and MOG-IgG. Serum neurofilament light chain levels (pg/mL) were assessed at the Verona University (SIMOA, Quanterix) in a blinded fashion on available stored samples obtained ≤3 months from myelopathy presentation. For each patient, the largest spinal cord lesion area (mm) was manually outlined by 2 independent raters on sagittal T2-weighted MRI images, and the mean value was used to determine NAR (pg/[mL·mm]).

Results: Forty-eight patients were included SCI, 20 (definite, 11; probable, 6; possible, 3); acute myelitis, 28 (AQP4-IgG-associated, 17; MOG-IgG-associated, 5; idiopathic transverse myelitis, 6). The median expanded disability status scale score (range) at myelopathy nadir were 7.75 (2-8.5) and 5.5 (2-8), respectively. Serum neurofilament light chain levels (median [range] pg/mL) in patients with SCI (188 [14.3-2793.4]) were significantly higher compared with patients with AQP4-IgG-associated myelitis (37 [0.8-6942.9]), MOG-IgG-associated myelitis (45.8 [4-283.8]), and idiopathic transverse myelitis (15.6 [0.9-217.8]); =0.01. NAR showed the highest accuracy for identification of SCI versus acute myelitis with values ≥0.35 pg/(mL·mm) yielding 86% specificity and 95% sensitivity (area under the curve=0.93). The positive and negative likelihood ratios were 6.67 and 0.06, respectively. NAR remained independently associated with SCI after adjusting for age, gender, immunotherapy before sampling, and days from myelopathy symptoms onset to sampling (=0.0007).

Conclusions: NAR is a novel and promising clinical biomarker for differentiation of SCI from acute myelitis.
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http://dx.doi.org/10.1161/STROKEAHA.120.031482DOI Listing
January 2021

Brainstem and cerebellar involvement in MOG-IgG-associated disorder versus aquaporin-4-IgG and MS.

J Neurol Neurosurg Psychiatry 2020 Dec 28. Epub 2020 Dec 28.

Neurology, Mayo Clinic, Rochester, Minnesota, USA

Objective: To determine the frequency and characteristics of brainstem or cerebellar involvement in myelin-oligodendrocyte-glycoprotein-antibody-associated-disorder (MOGAD) versus aquaporin-4-IgG-seropositive-neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and multiple sclerosis (MS).

Methods: In this observational study, we retrospectively identified 185 Mayo Clinic MOGAD patients with: (1) characteristic MOGAD phenotype, (2) MOG-IgG seropositivity by live cell-based assay and (3) MRI lesion(s) of brainstem, cerebellum or both. We compared the symptomatic attacks to AQP4-IgG-NMOSD (n=30) and MS (n=30).

Results: Brainstem or cerebellar involvement occurred in 62/185 (34%) MOGAD patients of which 39/62 (63%) were symptomatic. Ataxia (45%) and diplopia (26%) were common manifestations. The median age in years (range) in MOGAD of 24 (2-65) was younger than MS at 36 (16-65; p=0.046) and AQP4-IgG-NMOSD at 45 (6-72; p=0.006). Isolated attacks involving the brainstem, cerebellum or both were less frequent in MOGAD (9/39 (23%)) than MS (22/30 (73%); p<0.001) but not significantly different from AQP4-IgG-NMOSD (14/30 (47%); p=0.07). Diffuse middle cerebellar peduncle MRI-lesions favoured MOGAD (17/37 (46%)) over MS (3/30 (10%); p=0.001) and AQP4-IgG-NMOSD (3/30 (10%); p=0.001). Diffuse medulla, pons or midbrain MRI lesions occasionally occurred in MOGAD and AQP4-IgG-NMOSD but never in MS. Cerebrospinal fluid (CSF) oligoclonal bands were rare in MOGAD (5/30 (17%)) and AQP4-IgG-NMOSD (2/22 (9%); p=0.68) but common in MS (18/22 (82%); p<0.001). Disability at nadir or recovery did not differ between the groups.

Conclusion: Involvement of the brainstem, cerebellum or both is common in MOGAD but usually occurs as a component of a multifocal central nervous system attack rather than in isolation. We identified clinical, CSF and MRI attributes that can help discriminate MOGAD from AQP4-IgG-NMOSD and MS.
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http://dx.doi.org/10.1136/jnnp-2020-325121DOI Listing
December 2020

Variability of cerebrospinal fluid findings by attack phenotype in myelin oligodendrocyte glycoprotein-IgG-associated disorder.

Mult Scler Relat Disord 2021 Jan 23;47:102638. Epub 2020 Nov 23.

Department of Neurology and Ophthalmology, Mayo Clinic, Rochester, MN, USA. Electronic address:

Background: The variability in cerebrospinal fluid (CSF) findings of myelin oligodendrocyte glycoprotein-IgG-associated disorder (MOGAD) is not fully elucidated.

Objective And Methods: We retrospectively analyzed 203 attack-associated CSFs from Mayo Clinic patients (2000-2019) with MOGAD.

Results: White-blood-cell (>5/mm) elevation was less with clinically isolated optic neuritis (23%), compared to myelitis, brain/brainstem attacks, or combinations thereof (>70%), p<0.0001. CSF pleocytosis in optic neuritis was more common in patients with coexisting asymptomatic brain and/or spine MRI T2-lesions (53%) than in those without (16%), p=0.005. Abnormal CSF oligoclonal bands ranged from 1% (optic neuritis) to 18% (brain/brainstem attacks). CSF pleocytosis was less common after immunotherapy.

Conclusions: CSF findings in MOGAD vary by attack phenotype and preceding treatment.
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http://dx.doi.org/10.1016/j.msard.2020.102638DOI Listing
January 2021

Neural Antibody Testing in Patients with Suspected Autoimmune Encephalitis.

Clin Chem 2020 Nov 22. Epub 2020 Nov 22.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

Background: Autoimmunity is an increasingly recognized cause of encephalitis with a similar prevalence to that of infectious etiologies. Over the past decade there has been a rapidly expanding list of antibody biomarker discoveries that have aided in the identification and characterization of autoimmune encephalitis. As the number of antibody biomarkers transitioning from the research setting into clinical laboratories has accelerated, so has the demand and complexity of panel-based testing. Clinical laboratories are increasingly involved in discussions related to test utilization and providing guidance on which testing methodologies provide the best clinical performance.

Content: To ensure optimal clinical sensitivity and specificity, comprehensive panel-based reflexive testing based on the predominant neurological phenotypic presentation (e.g., encephalopathy) is ideal in the workup of cases of suspected autoimmune neurological disease. Predictive scores based on the clinical workup can aid in deciding when to order a test. Testing of both CSF and serum is recommended with few exceptions. Appropriate test ordering and interpretation requires an understanding of both testing methodologies and performance of antibody testing in different specimen types.

Summary: This review discusses important considerations in the design and selection of neural antibody testing methodologies and panels. Increased collaboration between pathologists, laboratorians, and neurologists will lead to improved utilization of complex autoimmune neurology antibody testing panels.
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http://dx.doi.org/10.1093/clinchem/hvaa254DOI Listing
November 2020

Neurologic Complications of Immune Checkpoint Inhibitors in Thoracic Malignancies.

J Thorac Oncol 2021 Mar 11;16(3):381-394. Epub 2020 Nov 11.

Department of Neurology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota. Electronic address:

Immune checkpoint inhibitors (ICIs) have transformed the prognosis of cancers previously considered lethal. The spectrum of therapeutic indications is rapidly expanding, including the vast majority of thoracic malignancies. By enhancing the immune responses against cancer, the ICI treatments lead to the development of immune-related adverse events (irAEs) that may affect any organ. Severity varies from mild to fatal clinical manifestations. Neurologic involvement is relatively rare and highly heterogeneous, including central and peripheral nervous system diseases associated with neural-specific autoantibodies or not, central nervous system vasculitis, and granulomatous and demyelinating disorders. Symptoms often manifest within the first four cycles of treatment and can develop regardless of the class of ICI used. An unfavorable outcome is found in up to one-third of patients and is generally associated with the patients' clinical characteristics (e.g., age, coexistence of systemic adverse events), cancer type (e.g., lung cancer versus other), and specific clinical setting (e.g., ICI treatment in patients with preexisting paraneoplastic neurologic autoimmunity, ICI rechallenge after a first neurologic irAE). Diagnosis should be suspected in patients with new-onset neurologic symptoms while on ICI treatment which are not explained by metastatic disease or other metabolic/infectious disorders. Recommended treatment is based on clinical severity and consists of ICI discontinuation with or without immunosuppressive/immunomodulatory therapy, although alternative approaches are reasonable depending on cancer status (e.g., aggressive immunosuppression without discontinuing ICI in patients with initial cancer response). Early recognition and appropriate treatment of these neurologic irAEs are crucial for improved patient outcomes and therapeutic planning.
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http://dx.doi.org/10.1016/j.jtho.2020.11.005DOI Listing
March 2021

Beyond Giant Cell Arteritis and Takayasu's Arteritis: Secondary Large Vessel Vasculitis and Vasculitis Mimickers.

Curr Rheumatol Rep 2020 Nov 7;22(12):88. Epub 2020 Nov 7.

Rheumatology, Mayo Clinic, Rochester, MN, USA.

Purpose Of Review: To provide an overview of mimickers of large vessel vasculitis (LVV), by the main presenting manifestation, i.e., systemic, vascular, and cranial manifestations.

Recent Findings: The main differential diagnoses in patients with giant cell arteritis (GCA) and Takayasu arteritis (TAK) presenting with systemic manifestations (i.e., fever, anorexia, weight loss, night sweats, arthralgia/myalgia, and/or increased inflammatory indexes) are neoplastic, infectious, or other inflammatory conditions. In patients with vascular manifestations (such as peripheral ischemia, vascular stenoses, or aneurysms), atherosclerosis and non-inflammatory vascular diseases should be excluded. In those presenting with predominant cranial symptoms (i.e., temporal headache, jaw claudication, scalp tenderness, transient or permanent vision loss), other causes of headache, cerebrovascular accidents, optic neuropathy, and neuromuscular syndromes need to be considered. The diagnosis of LVV maybe challenging, especially when patients present with atypical or incomplete clinical forms. In these cases, a multidisciplinary approach is strongly recommended.
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http://dx.doi.org/10.1007/s11926-020-00965-wDOI Listing
November 2020

Neuro-Ophthalmic Complications in Patients Treated With CTLA-4 and PD-1/PD-L1 Checkpoint Blockade.

J Neuroophthalmol 2020 Oct 28. Epub 2020 Oct 28.

Department of Ophthalmology (MMS, NS, LKG), Jules Stein Eye Institute, David Geffen School of Medicine at the University of California Los Angeles, Los Angeles, California; Department of Ophthalmology (JJC), Mayo Clinic, Rochester, Minnesota; Department of Neurology (JJC, AZ, ES), Mayo Clinic, Rochester, Minnesota; Department of Ophthalmology & Visual Sciences (RDW), Medical College of Wisconsin, Milwaukee, Wisconsin; Department of Ophthalmology (JDB), Park Nicollet Health Services, Minneapolis, Minnesota; Department of Ophthalmology (OS), University of Kentucky/Retina Associates of Kentucky, Lexington, Kentucky; Department of Neurology (LJM), SUNY Upstate Medical University, Syracuse, New York; Department of Ophthalmology (LJM), SUNY Upstate Medical University, Syracuse, New York; Department of Neurology (SG), University of Missouri-Kansas City, Kansas City, Missouri; Department of Ophthalmology (PMS), University of Vermont Medical Center, Burlington, Vermont; The Medical Eye Center (DAB), Manchester, New Hampshire; Department of Ophthalmology (JF), Oregon Health & Science University, Portland, Oregon; Department of Ophthalmology (CLF), University of Sydney, Sydney, Australia; Department of Neurology & Neurophysiology (CC-S), Liverpool Hospital, NSW, Australia; Department of Neurology (SRH), Virginia Commonwealth University School of Medicine, Richmond, Virginia; Department of Neurology (BH), INI Eye Center, OSF Healthcare, University of Illinois College of Medicine, Peoria, Illinois; Department of Ophthalmology (MDS), University of Utah, Salt Lake City, Utah; Department of Ophthalmology (PSS), University of Colorado, Aurora, Colorado; and Department of Ophthalmology (ZW), University of Rochester Medical Center, Rochester, New York.

Background: In recent years, CTLA-4 and PD-1/PD-L1 checkpoint inhibitors have proven to be effective and have become increasingly popular treatment options for metastatic melanoma and other cancers. These agents work by enhancing autologous antitumor immune responses. Immune-related ophthalmologic complications have been reported in association with checkpoint inhibitor use but remain incompletely characterized. This study seeks to investigate and further characterize the neuro-ophthalmic and ocular complications of immune checkpoint blockade treatment.

Methods: A survey was distributed through the secure electronic data collection tool REDCap to neuro-ophthalmology specialists in the North American Neuro-Ophthalmology Society listserv. The study received human subjects approval through the University of California at Los Angeles Institutional Review Board. The survey identified patients sent for neuro-ophthalmic consultation while receiving one or more of a PD-1 inhibitor (pembrolizumab, nivolumab, or cemiplimab); PD-L1 inhibitor (atezolizumab, avelumab, or durvalumab); or the CTLA-4 inhibitor ipilimumab. Thirty-one patients from 14 institutions were identified. Patient demographics, neuro-ophthalmic diagnosis, diagnostic testing, severity, treatment, clinical response, checkpoint inhibitor drug used, and cancer diagnosis was obtained.

Results: The checkpoint inhibitors used in these patients included pembrolizumab (12/31), nivolumab (6/31), combined ipilimumab with nivolumab (7/31, one of whom also received pembrolizumab during their course of treatment), durvalumab (3/31), ipilimumab (2/31), and cemiplimab (1/31). Malignant melanoma (16/31) or nonsmall cell lung carcinoma (6/31) were the most common malignancies. The median time between first drug administration and the time of ophthalmological symptom onset was 14.5 weeks. Eleven patients had involvement of the optic nerve, 7 patients had inflammatory orbital or extraocular muscle involvement, 6 patients had ocular involvement from neuromuscular junction dysfunction, 4 patients had cranial nerve palsy, and 4 patients had non neuro-ophthalmic complications. Use of systemic corticosteroids with or without stopping the checkpoint inhibitor resulted in improvement of most patients with optic neuropathy, and variable improvement for the other ophthalmic conditions.

Conclusion: This study describes the variable neuro-ophthalmic adverse events associated with use of immune checkpoint inhibitors and contributes a more thorough understanding of their clinical presentations and treatment outcomes. We expect this will increase awareness of these drug complications and guide specialists in the care of these patients.
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http://dx.doi.org/10.1097/WNO.0000000000001148DOI Listing
October 2020

Unfavorable outcome in highly relapsing MOGAD encephalitis.

J Neurol Sci 2020 11 10;418:117088. Epub 2020 Aug 10.

Department of Neurology, Mayo Clinic, Jacksonville, FL, USA. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2020.117088DOI Listing
November 2020

Inflammatory activity following motor progression due to critical CNS demyelinating lesions.

Mult Scler 2020 Aug 19:1352458520948745. Epub 2020 Aug 19.

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Background: New inflammatory activity is of unclear frequency and clinical significance in progressive multiple sclerosis (MS); it is uncertain in patient cohorts with motor progression due to critical demyelinating lesions.

Objectives: The aim of this study is to determine the likelihood of central nervous system (CNS) inflammatory activity, assessed by new clinical relapses or active magnetic resonance imaging (MRI) lesions, following onset of motor progression due to critical demyelinating lesions.

Methods: Patients with progressive upper motor neuron impairment for ⩾1 year attributable to critical demyelinating lesions with single CNS lesion (progressive solitary sclerosis (PSS)), 2 to 5 total CNS demyelinating lesions (progressive "pauci-sclerosis" (PPS)), or >5 CNS demyelinating lesions and progressive exclusively unilateral monoparesis or hemiparesis (PUHMS) were identified. Clinical data were reviewed for acute MS relapses, and subsequent MRI was reviewed for active T1-gadolinium-enhancing or T2-demyelinating lesions.

Results: None of the 91 patients (22 PSS, 40 PPS, 29 PUHMS) identified experienced clinical relapses over a median clinical follow-up of 93 months (range: 12-518 months). Nine patients (10%) developed active lesions over median 84 months radiologic follow-up (range: 12-518 months). Active lesions occurred in 24% PUHMS, 5% PSS, and 3% PPS cohorts.

Conclusion: New inflammatory activity, defined by active lesions and clinical relapses following motor progression in patients with critical demyelinating lesions, is low. Disease-modifying therapies that reduce demyelinating relapses and active MRI lesions are of uncertain benefit in these cohorts.
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http://dx.doi.org/10.1177/1352458520948745DOI Listing
August 2020

Neurologic autoimmunity and immune checkpoint inhibitors: Autoantibody profiles and outcomes.

Neurology 2020 10 13;95(17):e2442-e2452. Epub 2020 Aug 13.

From the Departments of Neurology (E.S., A.M., D.D., T.L., V.A.L., C.J.K., M.M., S.J.P., E.P.F., A.Z.), Oncology (S.N.M.), Laboratory Medicine and Pathology (A.M., D.D., V.A.L., S.J.P., E.P.F., A.Z.), and Immunology (V.A.L.), Mayo Clinic, Rochester, MN; and Department of Neurology (A.S.L.-C.), Mayo Clinic, Jacksonville, FL.

Objective: To describe neural autoantibody profiles and outcomes in patients with neurologic autoimmunity associated with immune checkpoint inhibitor (ICI) cancer immunotherapy.

Methods: In this retrospective descriptive study, 63 patients with ICI-related neurologic autoimmunity were included: 39 seen at the Mayo Clinic Neurology Department (clinical cohort) and 24 whose serum/CSF was referred to the Mayo Clinic Neuroimmunology Laboratory for autoantibody testing. Serum/CSF samples were tested for neural-specific autoantibodies. Predictors of unfavorable outcome (residual adverse event severity grade ≥3) were explored (logistic regression).

Results: Median age at neurologic symptom onset was 65 years (range 31-86); 40% were female. Neurologic manifestations were CNS-restricted (n = 26), neuromuscular (n = 30), combined (n = 5), or isolated retinopathy (n = 2). Neural-specific autoantibodies were common in patients with CNS involvement (7/13 [54%] in the unbiased clinical cohort) and included known or unidentified neural-restricted specificities. Only 11/31 patients with CNS manifestations had neuroendocrine malignancies typically associated with paraneoplastic autoimmunity. Small-cell lung cancer (SCLC)-predictive antibodies were seen in 3 patients with non-neuroendocrine tumors (neuronal intermediate filament immunoglobulin G [IgG] and antineuronal nuclear antibody 1 with melanoma; amphiphysin IgG with non-SCLC). A median of 10 months from onset (range, 0.5-46), 14/39 in the clinical cohort (36%) had unfavorable outcomes; their characteristics were age ≥70 years, female, CNS involvement, lung cancer, higher initial severity grade, and lack of systemic autoimmunity. By multivariate analysis, only age remained independently associated with poor outcome ( = 0.01). Four of 5 patients with preexistent neurologic autoimmunity experienced irreversible worsening after ICI.

Conclusions: Neural-specific autoantibodies are not uncommon in patients with ICI-related CNS neurologic autoimmunity. Outcomes mostly depend on the pre-ICI treatment characteristics and clinical phenotype.
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http://dx.doi.org/10.1212/WNL.0000000000010632DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7682911PMC
October 2020

Onset of progressive motor impairment in patients with critical central nervous system demyelinating lesions.

Mult Scler 2020 Jul 15:1352458520940983. Epub 2020 Jul 15.

Department of Neurology, College of Medicine, Mayo Clinic, Rochester, MN, USA.

Objective: To compare progressive motor impairment onset attributable to a "critical" central nervous system (CNS) demyelinating lesion in patients with highly restricted versus unlimited magnetic resonance imaging (MRI) lesion burden.

Methods: We identified 135 patients with progressive motor impairment for ⩾1 year attributable to a "critical" demyelinating lesion with: MRI burden of 1 lesion ("progressive solitary sclerosis"), 2-5 lesions ("progressive paucisclerosis"), or unrestricted (>5) lesions and "progressive unilateral hemiparesis." Neuroradiology review of brain and spinal cord MRI documented unequivocally demyelinating lesions.

Results: A total of 33 (24.4%) patients had progressive solitary sclerosis; 56 (41.5%) patients had progressive paucisclerosis; and 46 (34.1%) patients had progressive unilateral hemiparesis. Median age at onset of progressive motor impairment was younger in progressive solitary sclerosis (49 years; range 24-73) and progressive paucisclerosis (50 years; range 30-64) than in progressive unilateral hemiparesis (54 years; range 39-77;  = 0.02 and  = 0.003, respectively). Within progressive unilateral hemiparesis, motor-progression onset was similar between those with 4-10, 11-20, or >20 brain lesions (55, 54, 53 years of age, respectively;  = 0.44).

Conclusion: Motor-progression age is similar, but paradoxically earlier, in cohorts with highly restricted CNS lesion burden than in those with unrestricted lesion burden with progressive unilateral hemiparetic MS. The "critical" demyelinating lesion rather than total brain MRI lesion burden is the major contributor to motor-progression onset in these cohorts.
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http://dx.doi.org/10.1177/1352458520940983DOI Listing
July 2020

Serum neurofilament light chain studies in neurological disorders, hints for interpretation.

J Neurol Sci 2020 09 14;416:116986. Epub 2020 Jun 14.

Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Policlinco GB Rossi, P.le LA Scuro 10, 37134 Verona, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2020.116986DOI Listing
September 2020

FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis With Seizures (FLAMES): Is immunotherapy always needed to put out the fire?

Mult Scler Relat Disord 2020 Sep 11;44:102283. Epub 2020 Jun 11.

Department of Neurology, Mayo Clinic, 200 1st St SW, Rochester 55905, MN, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.

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http://dx.doi.org/10.1016/j.msard.2020.102283DOI Listing
September 2020

Critical spinal cord lesions associate with secondary progressive motor impairment in long-standing MS: A population-based case-control study.

Mult Scler 2021 Apr 18;27(5):667-673. Epub 2020 Jun 18.

Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA/Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA.

Background: Progressive motor impairment anatomically attributable to prominent, focally atrophic lateral column spinal cord lesions ("critical lesions") can be seen in multiple sclerosis (MS), for example, progressive hemiparetic MS.

Objective: The aim of this study was to investigate whether similar spinal cord lesions are more frequent in long-standing MS patients with secondary progressive motor impairment (secondary progressive MS (SPMS)) versus those maintaining a relapsing-remitting course (relapsing-remitting MS (RRMS)).

Methods: We retrospectively identified Olmsted County (MN, USA) residents on 31 December 2011 with (1) RRMS or SPMS for ⩾25 years, and (2) available brain and spine magnetic resonance imaging (MRI). A blinded neuroradiologist determined demyelinating lesion burden and presence of potential critical lesions (prominent focally atrophic spinal cord lateral column lesions).

Results: In total, 32 patients were included: RRMS, 18; SPMS, 14. Median (range) disease duration (34 (27-53) vs. 39 (29-47) years) and relapse number (4 (1-10) vs. 3 (1-15)) were similar. In comparison to RRMS, SPMS patients more commonly showed potential critical spinal cord lesions (8/18 (44%) vs. 14/14 (100%)), higher spinal cord (median (range) 4 (1-7) vs. 7.5 (3-12)), and brain infratentorial (median (range) 1 (0-12) vs. 2.5 (1-13)) lesion number;  < 0.05. By multivariate analysis, only the presence of potential critical lesions independently associated with motor progression ( = 0.02).

Conclusion: Critical spinal cord lesions may be important contributors to motor progression in MS.
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http://dx.doi.org/10.1177/1352458520929192DOI Listing
April 2021

Diagnostic features of initial demyelinating events associated with serum MOG-IgG.

J Neuroimmunol 2020 07 7;344:577260. Epub 2020 May 7.

Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona and Neurology Unit B, Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy.

Background: Myelin oligodendrocyte glycoprotein (MOG)-IgG associated disorders are increasingly recognized as a distinct disease entity. However, diagnostic sensitivity and specificity of serum MOG-IgG as well as recommendations for testing are still debated.

Materials And Methods: Between October 2015 and July 2017 we tested serum MOG-IgG in 91 adult patients (49 females) with a demyelinating event (DE) not fulfilling 2010 McDonald criteria for MS at sampling, negative for neuromyelitis optica (NMO)-IgG and followed-up for at least 12 months. We assessed the sensitivity and specificity of a live-cell MOG-IgG assay for each final diagnosis at last follow-up, for the 2018 international recommendations for MOG-IgG testing, and for other combinations of clinical and laboratory characteristics.

Results: Clinical presentations included acute myelitis (n = 48), optic neuritis (n = 36), multifocal encephalomyelitis (n = 4), and brainstem syndrome (n = 3). Twenty-four patients were MOG-IgG positive. Sensitivity and specificity of MOG-IgG test applied to the 2018 international recommendations were 28.4% and 86.7%, while they were 42.1% and 88.6% when applied to DE of unclear aetiology as defined above with two or more among: 1_no periventricular and juxtacortical MS-like lesions on brain MRI; 2_longitudinally extensive MRI optic nerve lesion; 3_no CSF-restricted oligoclonal bands; 4_CSF protein > 50 mg/dl.

Conclusions: Simplified requirements compared to those currently proposed for MOG-IgG testing could facilitate the applicability of the assay in the diagnosis of adults with DEs of unclear aetiology.
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http://dx.doi.org/10.1016/j.jneuroim.2020.577260DOI Listing
July 2020

Spinal arteriovenous fistula's often misdiagnosed as myelitis; can we stem the flow?

J Neurol Sci 2020 06 30;413:116868. Epub 2020 Apr 30.

Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA; Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2020.116868DOI Listing
June 2020

Frequency and characteristics of MRI-negative myelitis associated with MOG autoantibodies.

Mult Scler 2021 02 27;27(2):303-308. Epub 2020 Feb 27.

Department of Neurology, Mayo Clinic, Rochester, MN, USA/Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.

Background: Myelitis accompanied by a negative spinal cord MRI may lead to diagnostic uncertainty.

Objective And Methods: We retrospectively investigated the frequency of negative spinal cord MRI (performed <6 weeks from onset) in Mayo Clinic patients with myelin oligodendrocyte glycoprotein (MOG)-IgG-associated myelitis (2000-2019).

Results: The initial spinal cord MRI was negative in 7/73 (10%) patients, despite severe acute disability (median EDSS, 7 (range, 4.5-8)); myelitis symptoms/signs were frequent (paraparesis, neurogenic bladder, sensory level, Lhermitte's phenomenon). Myelitis lesions became overt at follow-up MRI in three patients.

Conclusions: A negative spinal cord MRI should not dissuade from MOG-IgG testing in patients with acute/subacute myelitis.
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http://dx.doi.org/10.1177/1352458520907900DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7500857PMC
February 2021

Antibody response against HERV-W in patients with MOG-IgG associated disorders, multiple sclerosis and NMOSD.

J Neuroimmunol 2020 01 6;338:577110. Epub 2019 Nov 6.

Section of Neurology, Department of Clinical, Surgery and Experimental Sciences, University of Sassari, Italy. Electronic address:

Increased expression of the retroviruses of HERV-W family has been linked to multiple sclerosis (MS) pathophysiology; nothing is known at the moment about MOG-IgG associated disorders. We compared antibody response against HERV-W peptides among patients with MOG-IgG associated disorders, multiple sclerosis (MS) and aquaporin-4 (AQP4)-IgG positive neuromyelitis optica spectrum disorder (NMOSD). A total of 102 serum samples were retrospectively analyzed. Antibody reactivity against HERV-W env peptides was similar in MOG-IgG associated disorders and MS, but different from AQP4-IgG positive NMOSD. Our findings expand the diagnostic role of HERV-W antibodies to the spectrum of demyelinating disorders associated with MOG-IgG.
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http://dx.doi.org/10.1016/j.jneuroim.2019.577110DOI Listing
January 2020

The frequency of longitudinally extensive transverse myelitis in MS: A population-based study.

Mult Scler Relat Disord 2020 Jan 31;37:101487. Epub 2019 Oct 31.

Departments of Neurology, Mayo Clinic, Rochester, MN, USA; Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA. Electronic address:

Background: Determining the frequency of longitudinally-extensive transverse myelitis (LETM: T2-lesion ≥3 vertebral segments) in multiple sclerosis (MS) is essential to assess its utility in differentiating from aquaporin-4-IgG (AQP4-IgG) positive neuromyelitis optica spectrum disorder (NMOSD) and myelin-oligodendrocyte-glycoprotein-IgG (MOG-IgG) myelitis. We sought to determine the frequency of LETM in MS during a myelitis attack.

Methods: We identified Olmsted County (MN, USA) residents on 12/31/2011 with inflammatory demyelinating disease. Inclusion criteria were: 1) Clinical myelitis episode accompanied by a new spinal magnetic resonance imaging (MRI) lesion (≤6 weeks from onset); 2) MS diagnosis by 2010 McDonald criteria; 3) Seronegative for AQP4-IgG and MOG-IgG. MRI characteristics were determined.

Results: Sixty-seven patients (median age at myelitis: 41 years [range, 16-65]; 76% females) with 92 myelitis attacks accompanied by a new MRI spinal cord lesion were identified. The frequency of LETM was 0%. The median T2-hyperintense lesion length in vertebral segments was 1.0 (range, 0.5-2.5) and 82/92 (89%) were peripheral in location on axial sequences; 58% had associated gadolinium enhancement. Two patients (2% of attacks) had multiple short lesions resembling LETM on sagittal images but axial sequences confirmed multiple non-contiguous short lesions.

Conclusion: LETM is rare in adult MS myelitis and its presence should prompt evaluation for AQP4-IgG, MOG-IgG or other etiologies. Careful scrutiny of axial images is important as coalescence of multiple short lesions may lead to the artifactual appearance of an LETM.
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http://dx.doi.org/10.1016/j.msard.2019.101487DOI Listing
January 2020

Plasma vitronectin is reduced in patients with myasthenia gravis: Diagnostic and pathophysiological potential.

J Circ Biomark 2019 Jan-Dec;8:1849454419875912. Epub 2019 Sep 11.

Department of Biomedical Sciences, University of Sassari, Viale San Pietro, Sassari, Italy.

Myasthenia gravis (MG) is an autoimmune disease leading to varying degrees of skeletal muscle weakness. It is caused by specific antibodies directed against definite components in the postsynaptic membrane at the neuromuscular junction (NMJ), such as the acetylcholine receptor (AChR) and the muscle-specific kinase (MUSK) receptor. In clinical practice, MG patients may be classified into three main subgroups based on the occurrence of serum autoantibodies directed against AChR or MUSK receptor or antibody-negative. As the MG subgroups differ in terms of clinical characteristics, disease pathogenesis, prognosis, and response to therapies, they could benefit from targeted treatment as well as the detection of other possible disease biomarkers. We performed proteomics on plasma fractions enriched in low-abundance proteins to identify potential biomarkers according to different autoimmune responses. By this approach, we evidenced a significant reduction of vitronectin in MG patients compared to healthy controls, irrespective of the autoantibodies NMJ target. The obtained results were validated by mono- and two-dimensional Western blotting analysis. Vitronectin is a multifunctional glycoprotein involved in the regulation of several pathophysiological processes, including complement-dependent immune response, coagulation, fibrinolysis, pericellular proteolysis, cell attachment, and spreading. The pathophysiological significance of the reduction of plasma vitronectin in MG patients has yet to be fully elucidated. It could be related either to a possible deposition of vitronectin at NMJ to counteract the complement-mediated muscle damage at this level or to a parallel variation of this glycoprotein in the muscle extracellular matrix with secondary induced alteration in clustering of AChRs at NMJ, as it occurs with variation in concentrations of agrin, another extracellular matrix component. The clinical value of measuring plasma vitronectin has yet to be defined. According to present findings, significantly lower plasma values of this glycoprotein might be indicative of an impaired complement-dependent immune response.
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http://dx.doi.org/10.1177/1849454419875912DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6740073PMC
September 2019

Unilateral motor progression in MS: Association with a critical corticospinal tract lesion.

Neurology 2019 08 9;93(7):e628-e634. Epub 2019 Jul 9.

From the Department of Neurology (E.S., B.M.K., O.H.K., B.G.W., E.P.F.), Department of Radiology, Division of Neuroradiology (T.J.K.), and Department of Laboratory Medicine and Pathology (E.P.F.), Mayo Clinic College of Medicine, Rochester, MN.

Objective: Progressive motor impairment anatomically attributable to a single critical demyelinating lesion on eloquent corticospinal tract locations occurs in progressive solitary sclerosis and in some patients with multiple sclerosis (MS) with highly restricted CNS lesion burden (2-5 lesions). We determined whether a similar critical lesion is found in patients with MS with unilateral motor progression and unlimited lesion burden.

Methods: In this observational study, we retrospectively identified Mayo Clinic patients (January 1, 1996-December 31, 2017) with an MS diagnosis (2017 McDonald criteria), ≥1 year of exclusively unilateral motor progression, and >5 demyelinating lesions on MRI. A blinded neuroradiologist identified a single critical lesion (last available MRI) based on prominent size, atrophy, and eloquent corticospinal tract location (spinal cord lateral columns, medullary pyramids, cerebral peduncles, internal capsules). We then determined whether the motor impairment was anatomically attributable to the identified lesion.

Results: Thirty-eight patients with MS were included: 20 (53%) with primary progressive MS and 18 (47%) with secondary progressive MS. Median age at progression onset was 54 (range 39-73) years. Median Expanded Disability Status Scale score was 5 (range 2.5-7.5) at the last follow-up (median 132.5 months from symptom onset, range 23-390 months). A single critical lesion was identified in 25 of 38 cases (66%): 19 in the cervical cord and 6 in the thoracic cord. In the remaining patients, >1 potential critical lesions were present. The overall probability to detect demyelinating lesions was higher along the corticospinal tract where the motor deficit localized (38 of 38 [100%]) than on the contralateral side (15 of 38 [39%]) ( < 0.0001).

Conclusions: In patients with MS with unilateral motor progression, the motor deficit may be attributable to a single critical corticospinal tract lesion.
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http://dx.doi.org/10.1212/WNL.0000000000007944DOI Listing
August 2019

Aquaporin-4 and MOG autoantibody discovery in idiopathic transverse myelitis epidemiology.

Neurology 2019 07 24;93(4):e414-e420. Epub 2019 Jun 24.

From the Departments of Neurology (E.S., E.S., S.J.P., B.G.W., B.M.K., N.L.Z., A.S.L.-C., E.P.F.), Health Sciences Research (J.P.W.), and Laboratory Medicine and Pathology (S.J.P., J.J., E.P.F.), Mayo Clinic College of Medicine, Rochester, MN.

Objective: Diagnostic criteria from 2002 classify transverse myelitis (TM) as idiopathic or disease associated but predate the discovery of aquaporin-4 (AQP4)-immunoglobulin G (IgG) and myelin oligodendrocyte glycoprotein (MOG)-IgG, which associate with TM. Prior incidence estimates of idiopathic TM (ITM) range from 1 to 6.2 per 1 million. We sought to determine whether the population-based incidence and prevalence of ITM were reduced by testing patients with ITM for AQP4/MOG-IgG and reclassifying seropositive cases as having disease-associated TM.

Methods: For this observational study, we retrospectively identified all cases of incident (January 1, 2003-December 31, 2016) and prevalent (December 31, 2016) ITM in Olmsted County (85% white) by using the Rochester Epidemiology Project medical records linkage system. ITM was defined by the 2002 Transverse MyelitisConsortium Working Group diagnostic criteria. Available sera were tested for AQP4-IgG and MOG-IgG.

Results: Twenty-four patients (incident 22, prevalent 17) initially met 2002 ITM criteria (longitudinally extensive TM [LETM] 6). Sera were tested for AQP4-IgG in 22 of 24 (92%) and MOG-IgG in 21 of 24 (88%). Three seropositive cases (AQP4-IgG 2, MOG-IgG 1) were identified and reclassified as having disease-associated TM, accounting for 14% of total incident and 12% of total prevalent cases. AQP4-IgG and MOG-IgG seropositive cases represented 50% (3 of 6) of idiopathic LETM. After reclassification of seropositive patients, the final ITM incidence was 8.6 per 1,000,000 and prevalence was 7.9 per 100,000. Three cases of ITM (14%) subsequently fulfilled multiple sclerosis criteria within the study period.

Conclusions: The availability of AQP4-IgG and MOG-IgG modestly reduced ITM incidence and prevalence, which remained higher than previously reported in this predominantly white population. Incorporation of these biomarkers into future revisions of TM diagnostic criteria should be considered.
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http://dx.doi.org/10.1212/WNL.0000000000007828DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7508328PMC
July 2019

Serum and CSF neurofilament light chain levels in antibody-mediated encephalitis.

J Neurol 2019 Jul 3;266(7):1643-1648. Epub 2019 Apr 3.

Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Policlinico GB Rossi, P.le LA Scuro 10, 37135, Verona, Italy.

Circulating and cerebrospinal fluid (CSF) neurofilament light chain (NfL) levels represent a reliable indicator of disease activity and axonal damage in different neuroinflammatory conditions. Recently, high CSF NfL levels have been detected in active autoimmune encephalitis, as opposed to significant lower levels after clinical improvement. The aim of the present study was to evaluate serum and CSF NfL concentration in patients with autoimmune encephalitis and to analyse the association between NfL levels and clinical, MRI, and CSF data. We retrospectively included 25 patients with neurological syndromes associated with autoantibodies to neuronal cell surface antigens and we collected clinical, MRI, CSF, and follow-up data. Using an ultrasensitive method (Simoa, Quanterix), we measured NfL levels in serum and CSF samples of all patients and in 25 sera of healthy controls. Serum NfL levels were higher in all cases, including 20 patients with inflammatory MRI/CSF features and 5 non-inflammatory cases (median 16.9 pg/ml, range 4.5-90) than in controls (median 6.9 pg/ml, range 2.7-12.8; p < 0.001). A correlation between serum and CSF NfL levels was found (r = 0.461, p = 0.023), whereas no significant association was observed between NfL levels and clinical, MRI/CSF inflammatory burden, and antibody type. In the 13 available follow-up samples, correlation between disease activity and NfL values was also observed. In conclusion, NfL levels are significantly increased in the serum of patients with antibody-mediated encephalitis, independently of the MRI/CSF inflammatory profile. These findings support the presence of ongoing axonal damage and suggest the co-occurrence of different mechanisms for neuronal/axonal involvement in antibody-associated CNS syndromes.
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http://dx.doi.org/10.1007/s00415-019-09306-zDOI Listing
July 2019

Diagnosis and Management of Autoimmune Dementia.

Curr Treat Options Neurol 2019 Feb 27;21(3):11. Epub 2019 Feb 27.

Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

Purpose Of Review: To describe the clinical, laboratory, and MRI features that characterize cognitive decline in the setting of central nervous system (CNS) autoimmunity, and provide an overview of current treatment modalities.

Recent Findings: The field of autoimmune neurology is rapidly expanding due to the increasing number of newly discovered autoantibodies directed against specific CNS targets. The clinical syndromes associated with these autoantibodies are heterogeneous but frequently share common, recognizable clinical, and MRI characteristics. While the detection of certain autoantibodies strongly suggest the presence of an underlying malignancy (onconeural autoantibodies), a large proportion of cases remain idiopathic. Cognitive decline and encephalopathy are common manifestations of CNS autoimmunity, and can mimic neurodegenerative disorders. Recent findings suggest that the frequency of autoimmune encephalitis in the population is higher than previously thought, and potentially rivals that of infectious encephalitis. Moreover, emerging clinical scenarios that may predispose to CNS autoimmunity are increasingly been recognized. These include autoimmune dementia/encephalitis post-herpes simplex virus encephalitis, post-transplant and in association with immune checkpoint inhibitor treatment of cancer. Early recognition of autoimmune cognitive impairment is important given the potential for reversibility and disability prevention with appropriate treatment. Autoimmune cognitive impairment is treatable and may arise in a number of different clinical settings, with important treatment implications. Several clinical and para-clinical clues may help to differentiate these disorders from dementia of other etiologies.
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http://dx.doi.org/10.1007/s11940-019-0550-9DOI Listing
February 2019

Hypertrophic olivary degeneration mimics relapse in neuromyelitis optica spectrum disorder.

Neurology 2019 02;92(7):343-344

From the Departments of Neurology (E.S., E.P.F.), Radiology (Division of Neuroradiology) (K.K.K.), and Laboratory Medicine and Pathology (E.P.F.), Mayo Clinic, Rochester, MN; and Department of Medicine (Division of Neurology) (N.E.P.), Dalhousie University, Halifax, Canada.

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http://dx.doi.org/10.1212/WNL.0000000000006930DOI Listing
February 2019

Area postrema syndrome in autoimmune GFAP astrocytopathy.

Mult Scler 2020 02 21;26(2):255-256. Epub 2019 Jan 21.

Department of Neurology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA/Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA.

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http://dx.doi.org/10.1177/1352458519826489DOI Listing
February 2020