Autoimmune Neurology: Paraneoplastic Disorders & Beyond. Andrew McKeon, MD Mayo Clinic
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1 Autoimmune Neurology: Paraneoplastic Disorders & Beyond Andrew McKeon, MD Mayo Clinic
2 Disclosures I receive research support from Euroimmun I have consulted for Medimmune, Euroimmun & Grifols (no personal compensation) I have patent applications for MAP1B and Septin 5 Abs as markers of neurological disease and cancer.
3 Outline Risk factors for autoimmune neurological disorders? How do I evaluate further? - Basic serum/csf testing - Neural antibody (Ab) testing - Treatment trial in suspected cases The diagnostic test
4 Why Suspect an Autoimmune Neurological Disorders?
5 Phenotype Rapid onset Rapid progression Classic phenotypes Atypical phenotypes Multifocal disorders
6 Classic Disorders Limbic encephalitis Chorea Cerebellar degeneration Brainstem encephalitis Opsoclonus-myoclonus syndrome Myelopathy Stiff-person syndrome Sensory ganglionopathy/neuronopathy Lambert-Eaton myasthenic syndrome (LEMS)
7 Beyond Classical Phenotypes Disorders at presentation may be: Limited - Epilepsy - Dementia - Stiff-limb More widespread/multifocal - Encephalomyelitis - Chorea + neuropathy - Ataxia and LEMS
8 What are the risk factors? Sometimes none Coexisting autoimmune disease, e.g. thyroid disease, type 1 diabetes mellitus Cancer history Smoking history Family history of autoimmune disease or cancer
9 How do I evaluate further? Determine extent of neurological involvement: - Neurological examination - Mental status testing - Neuropsychometric testing - MRI imaging - Electrophysiology (EEG, EMG, SSEPs)
10 How do I evaluate further? Ab testing, serum: - Non-neural Abs: e.g. thyroid peroxidase Abs, connective tissue cascade - Neural Abs: main subject of this course CSF testing: protein, white cell count, IgG index and synthesis rate, oligoclonal bands, neural Abs
11 Why do autoimmune neurological diseases occur?
12
13 Neural Abs Overview IgG Antibodies targeting Neural cell surface antigens (ion channels, receptors, synapses) Neuronal nuclear, cytoplasmic antigens e.g. VGKC complex Ab, NMDA-R Ab, GlyR e.g. ANNA-1, PCA-1, CRMP-5 IgG Immunotherapy Oncological therapy
14 Neuronal nuclear or cytoplasmic Abs Antibody ANNA-1 (anti-hu) ANNA-2 (anti-ri) ANNA-3 AGNA (SOX-1) PCA-1 (anti-yo) Oncological association Small-cell carcinoma Small-cell carcinoma Breast adenocarcinoma Aerodigestive carcinomas Small-cell carcinoma Gynecological adenocarcinomas Breast adenocarcinoma PCA-2 Small-cell carcinoma CRMP-5 IgG (anti-cv2) Small-cell carcinoma Amphiphysin IgG GFAP-IgG NIF-IgGs Thymoma Small-cell carcinoma Breast adenocarcinoma Teratoma, other Neuroendocrine (small cell, Merkel cell, other)
15 Synaptic Autoantibodies Antibody Lgi1/CASPR2 Oncological association Thymoma, other NMDA receptor 50% Ovarian teratoma AMPA receptor 70% Thymoma, lung carcinoma, breast carcinoma GABA-B receptor 50% Small-cell lung carcinoma P/Q & N type calcium channel Small-cell carcinoma, breast or gynecological adenocarcinoma GlyR Thymoma DPPX Occasional B cell neoplasm IgLON5 None PCA-Tr (DNER) Hodgkin lymphoma
16 How Are Patients Evaluated in the Laboratory?
17 How are Abs detected? Indirect immunofluorescence screening Confirmation by: Western blot Cell based assay Immunoprecipitation Immunoprecipitation assay screening Cell based assay screening
18 Examples of Abs Accompanying Autoimmune CNS Disorders
19 Encephalitis Memory, mood, personality changes, seizures: limbic encephalitis Diverse autoantibody associations: - ANNA-1, 2 (anti-hu, Ri) - CRMP-5 IgG - Lgi1, CASPR2 IgGs - GAD65 Ab (High titer) - AMPA, GABA-B receptor Abs - mglur5 Ab
20 Cognitive disorders Cognitive-predominant presentations, not typical for limbic encephalitis May have coexisting neurological problems (e.g. tremor, neuropathy) Thyroid autoimmunity common VGKC complex Abs>GAD65 Ab>N or P/Q type calcium channel Abs> ANNA-1 (anti-hu) Pre- and post objective testing helpful in defining treatment response Flanagan et al, Mayo Clinic Proceedings 2010
21 Autoimmune Epilepsy May have seizure predominant presentation Scan may be normal at onset in half Dx: EEG, CSF, Ab testing VGKC complex Abs > GAD65 Ab > CRMP-5 IgG > Ma2 = NMDA receptor Ab = GABA-B-R-IgG GABA-A-R-IgG Quek et al, Arch Neurol, Localizations Mesial temporal Neocortical temporal Precentral
22 NMDA-R Encephalitis Stereotyped course: Psych F>M seizures, encephalopathy movement disorder, dysautonomia hypoventilation+coma 50% have ovarian teratoma CSF testing: more sensitive and specific Treatment: steroids/ivig or PLEX/rituximab/cyclophosphamide 80% get to mild or no disability Titulaer, Lancet Neurology 2013
23 ADEM: AQP4 and MOG Abs Assay: Cell-based format IgG1 anti-human secondary Ab for MOG Syndromes: Optic neuritis Myelitis ADEM or ADEM-like Brainstem disorders NMO Clinical course: monophasic, relapsing often ++ steroid responsive Lopez et al, in press, JAMA Neurol, 2018.
24 Inflammatory CNS disorder Meningitis Encephalitis Myelitis Inflammatory CSF Sometimes paraneoplastic Steroid responsive
25 A E B F C G H I D CC GM J WM
26 GFAP-IgG Flanagan et al, Ann Neurol, 2017
27 Opsoclonus-myoclonus Children - Neuroblastoma - ANNA-1 in a minority Adults - 15% paraneoplastic - ANNA-2 > ANNA-1 = NMDA-R Ab - Frequently idiopathic autoimmune (immunotherapy responsive) Klaas et al, Arch Neurol 2012
28 Opsoclonus OR myoclonus Opsoclonus only ANNA-2 (anti-ri) Breast adenocarcinoma Whole body tremor Small amplitude generalized polymyoclonus No opsoclonus 25% have autoimmune cause Occult cancer possible VGKC, Alpha 3 ganglionic, CRMP-5 IgG McKeon et al, Arch Neurol, 2007
29 Chorea Paraneoplastic or idiopathic autoimmune Paraneoplastic: - CRMP-5 IgG - ANNA-1 - GAD65 Idiopathic autoimmune: - Lupus, APL Ab syndrome - CASPR2 O Toole et al, Neurology 2013
30 Chorea Paraneoplastic patients more likely: - Older - Male - More frequent weight loss - More frequent coexisting peripheral neuropathy - Some improved with immunotherapy/cancer therapy Idiopathic - Often milder course - Improved/resolved with steroids O Toole et al, Neurology, 2013
31 Unknown Cerebellar ataxia Symptoms frequently overlap with brainstem disorders Rapid-onset dysarthria, incoordination, gait disturbance, vertigo Prototypic disorder: PCA-1 (anti-yo) associated cerebellar degeneration in women with mullerian or breast adenocarcinoma Other Abs: P/Q-type calcium channel Ab, GAD65 Ab, PCA- Tr, mglur1 Ab Peterson et al, Neurology 1992 McKeon et al, Arch Neurol 2010 Cerebellar atrophy Non-classic disorders also occur
32 Neurological Ataxia Limbic symptoms (rare, at onset) Dysgeusia (40%, at onset) Cancer Lymphoma (HD, non-hd), prostate adenocarcinoma Sillevis-Smitt, NEJM, 2000 Lopez et al, Neurology, mglur1 Ab
33 Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Patient 6 Control Control Control Blank Septin-5-IgG Cerebellar ataxia Oscillopsia, vertigo Improvements with immunotherapy Patient Septin-5 IgG Merged D 50 kda 37 kda 25 kda Hi GTP-binding neural protein Neurotransmitter exocytosis Honorat et al, N2, in press
34 Eye movement disorders Dysphagia, dysarthria Parkinsonism Sleep disorders e.g. ANNA-2, MaTa Pittock et al, Ann Neurol 2003 Dalmau et al, Ann Neurol 2004 Video: - Initial Dx: PSP Brainstem - Parkinsonism, narcolepsycataplexy - Ma1, Ma2 Ab positive - Tonsillar carcinoma Adams et al, Arch Neurol 2011
35 IgLON5 Kidney SMA Cerebellum Hippocampus Sleep disorders Chorea Parkinsonism Dysphagia Dysautonomia Stiff-person syndrome? Treatment responsive Honorat et al, 2017, N2
36 First case seen in 1924 Reported in 1956 with 13 other cases Moersch, Woltman. Proc Staff Meet Mayo Clin. 1956
37 GlyR-IgG in Stiff-man Syndrome 10/81 patients tested positive (12%, GlyR cell binding assay) GAD65-IgG + N=60 GAD65-IgG - N=21 Classic SMS Variant SMS PERM Classic SMS Variant SMS PERM Hyperekplexia N=43 N=16 N=1 N=4 N=14 N=2 N=1 GlyR-IgG + GlyR-IgG Improved with immunotherapy 0 5 of 6 GlyR-IgG + patients 7 of 25 GlyR IgG - patients (p=0.02) Mayo, Barcelona JAMA Neurol, 2013
38 GlyR-IgG GlyRα1-IgG +: - 21/247 patients - 8/190 healthy subjects (4%) Only SPS spectrum patients serums internalize GlyRα1 SPS spectrum phenotypes: - PERM (8) - Classic SPS (5), - Stiff-limb (5) - Stiff-trunk (1) - Isolated exaggerated startle (hyperekplexia, 1). Hinson et al, N2, 2018
39 GlyR-IgG Modulating Assay Hinson et al, N2, 2018
40 DPPX Autoimmunity, Manifestations in 20 patients Neurological Cognitive disorders 16 Brainstem/spinal cord disorders 15 Weight loss 12 Myoclonus or tremor 11 Sleep disorder 9 Gastrointestinal dysautonomia 9 Delirium 8 Cerebellar dysfunction 7 Urinary symptoms 7 Psychosis 4 Depression 4 Seizures 2 Cardiac dysrhythmia 3 Diaphoresis 2 Temperature dysregulation 1 Central hyperexcitability PERM (Rigidity + myoclonus 2 Myoclonus 8 Startle 6 Rigidity 6 Brisk reflexes 6 Stiff-man syndrome 0 Cancer B cell neoplasia 2 Tobin et al, Neurology, 2014
41 Paraneoplastic Myelopathy Subacute or insidious onset Lung, breast, kidney, thyroid, ovary/endometrium, melanoma, or other. Amphiphysin IgG, CRMP-5 IgG, ANNA-1, PCA-1, ANNA-3 Minority improve with treatment 50% Wheelchair bound Flanagan et al, Neurology, 2011.
42 Neuronal Intermediate Filaments ML PC GL Hippo MP Basal et al, AAN, Los Angeles, Friday Encephalopathy Ataxia Myelopathy Small cell carcinoma Merkel cell carcinoma 5 NIF targets - α internexin - Light chain - Medium chain - Heavy chain - Peripherin
43 Cancer screening Small cell/neuroendocrine: PET-CT, CT Other lung cancer types: PET-CT, CT Thymoma: high res CT or MRI Lymphoma: PET-CT Breast: mammogram/breast exam Lymph node disease: PET-CT Prostate: digital rectal exam, PSA Gynecological: clinical exam, US with transvaginalmri Renal: CT GI: endoscopies Thyroid: ultrasound, PET-CT Skin: dermatologist exam
44 Treatment: principles Trials of immunotherapy Measure improvement objectively Determining if short-term or long term treatment required Consider steroid-sparing agent
45 Cytotoxic T cell mediated disorders Paraneoplastic disorders - Do not generally have good responses to steroids, IVIg or plasma exchange General approach: - Oncological therapy (surgery, chemotherapy, radiation therapy) - Cyclophosphamide McKeon, Curr Treat Options Neurol, 2013
46 Antibody-mediated disorders (definite or possible) Acute (early important) - Corticosteroids - Intravenous immune globulin (IVIg) - Plasma exchange Chronic - Mycophenolate mofetil - Azathioprine - Rituximab, cyclophosphamide
47 Autoimmune Ataxia: Treatments & Outcomes 50% wheelchair dependent by 25.5 months Jones et al, JAMA Neurol 2015
48 Checkpoint inhibitors Peripheral: block PD-1 (pembrolizumab, nivolumab) Central: CTLA-4 Outcome: Autoimmunity - Retinopathy - Encephalitis - Myasthenia gravis - Necrotizing myopathy Kao et al, JAMA Neurol, 2017
49 Summary Autoimmune neurological disorders are important to consider - Potentially treatable - May be indicative of occult cancer Clues may emanate from - history - examination - serum & CSF Ab evaluations - response to treatment
50 Thank you Andrew McKeon
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