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تعداد آیتم قابل مشاهده باقیمانده : -6 مورد

Antibodies associated with autoimmune neurologic syndromes including paraneoplastic neurologic syndromes*

Antibodies associated with autoimmune neurologic syndromes including paraneoplastic neurologic syndromes*
Antibody (alternative name) Likely pathogenic mechanism Neurologic phenotypes Frequency of cancer (%) Usual tumors Sex, age-related, and other specificities
High-risk antibodies (>70% associated with cancer)
Hu (ANNA-1) T cell-mediated Sensory neuronopathy, chronic gastrointestinal pseudo-obstruction, encephalomyelitis, limbic encephalitis 85 SCLC >> NSCLC, other neuroendocrine tumors, neuroblastoma Limbic encephalitis is usually nonparaneoplastic in patients <18 years of age.
CV2/CRMP5 T cell-mediated Encephalomyelitis, sensory neuronopathy >80 SCLC, thymoma Patients with an associated thymoma are younger and present less commonly with neuropathy.
SOX1 Uncertain LEMS with and without rapidly progressive cerebellar syndrome >90 SCLC Stronger correlation with SCLC than with a particular neurologic presentation.
PCA-2 (MAP1B) T cell-mediated Sensorimotor neuropathy, rapidly progressive cerebellar syndrome, encephalomyelitis 80 SCLC, NSCLC, breast cancer  
Amphiphysin Uncertain; possibly antibody-mediated Polyradiculopathy, sensory neuronopathy, encephalomyelitis, stiff-person syndrome 80 SCLC, breast cancer Associated antibodies commonly coexist. Patients with isolated antiamphiphysin are more likely to be females with breast cancer and stiff-person syndrome.
Ri (ANNA-2) T cell-mediated Brainstem/cerebellar syndrome, opsoclonus-myoclonus-ataxia syndrome >70 Breast > lung (SCLC and NSCLC) Breast cancer in females; lung cancer in males.
Yo (PCA-1) T cell-mediated Paraneoplastic cerebellar degeneration >90 Ovarian cancer, breast cancer Almost all female; in males, antigen expression by tumor should be proven.
Ma2 and/or Ma T cell-mediated Limbic encephalitis, diencephalitis, brainstem encephalitis >75 Testicular cancer, NSCLC Young males: testicular tumors and isolated Ma2 positivity; older patients: SCLC and both Ma1/2 positivity.
Tr (DNER) Uncertain Paraneoplastic cerebellar degeneration 90 Hodgkin lymphoma  
KLHL11 T cell-mediated Brainstem/cerebellar syndrome 80 Testicular cancer Young males.
Intermediate-risk antibodies (30 to 70% associated with cancer)
AMPAR Antibody-mediated Limbic encephalitis >50 SCLC, malignant thymoma Paraneoplastic origin is more likely when other onconeuronal antibodies co-occur.
GABABR Antibody-mediated Limbic encephalitis >50 SCLC Paraneoplastic cases are more commonly observed in older males, in smokers, and in association with anti-KCTD16 antibodies. Most cases in young patients are not paraneoplastic.
mGluR5 Antibody-mediated Encephalitis ~50 Hodgkin lymphoma  
P/Q VGCC Antibody-mediated for LEMS; uncertain for cerebellar syndrome LEMS, rapidly progressive cerebellar syndrome 50 (LEMS; nearly 90 for rapidly progressive cerebellar syndrome) SCLC Co-occurrence with N-type VGCC antibodies might be slightly more common in paraneoplastic LEMS.
NMDAR Antibody-mediated Anti-NMDAR encephalitis 38 Ovarian or extraovarian teratomas Tumor (mostly ovarian teratomas) predominates in females 18 to 45 years of age (50%). Older patients less frequently have tumors (<25%), usually carcinomas. Paraneoplastic cases in children are very rare (<10%).
Caspr2 Antibody-mediated Morvan syndrome, limbic encephalitis, acquired neuromyotonia (Isaac syndrome)

50 (for Morvan syndrome)

<30 (for all other syndromes)
Malignant thymoma Caspr2 should be considered as an intermediate-risk antibody only in the setting of Morvan syndrome. When associated with other neurologic syndromes, the risk of cancer is very low.
Lower-risk antibodies (<30% associated with cancer)
mGluR1 Antibody-mediated Isolated cerebellar ataxia 11 Mostly hematologic  
GABAAR Antibody-mediated Encephalitis <30 Malignant thymoma Paraneoplastic origin is less frequent in children (10%) than in adults (60%).
GFAP Uncertain Meningoencephalitis ~20 Ovarian teratomas, adenocarcinomas May occur as an immunologic accompaniment in anti-NMDAR encephalitis with ovarian teratomas.
GAD65 Uncertain Limbic encephalitis, stiff-person syndrome, cerebellar ataxia <15 SCLC, other neuroendocrine tumors, malignant thymoma Paraneoplastic origin more likely in older patients, males, and in association with neuronal antibodies or atypical clinical presentations.
LGI1 Antibody-mediated Limbic encephalitis <10 Malignant thymoma, neuroendocrine tumors Paraneoplastic cases are mainly observed in patients with Morvan syndrome and both serum LGI1 and Caspr2 antibodies.
DPPX Antibody-mediated Encephalitis with CNS hyperexcitability, PERM <10 B cell neoplasms  
GlyR Antibody-mediated Limbic encephalitis, PERM <10 Malignant thymoma, Hodgkin lymphoma  
AQP4 Antibody-mediated Neuromyelitis optica spectrum disorder <5 Adenocarcinomas Paraneoplastic origin associated with older age, male sex, and severe nausea/vomiting at onset.
MOG Uncertain MOG antibody-associated disease 5 cases reported Mostly ovarian teratomas  
AK5 T cell-mediated Limbic encephalitis 0   No known cancer association
GluK2 Antibody-mediated Encephalitis with prominent cerebellar involvement <1   1 of 8 patients had a remote history of Hodgkin lymphoma
SEZ6L2 Uncertain Subacute cerebellar syndrome with extrapyramidal symptoms 0   Only 6 cases reported

AK5: adenylate kinase 5; AMPAR: α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; ANNA: antineuronal nuclear antibody; AQP4: aquaporin 4; Caspr2: contactin-associated protein-like 2; CNS: central nervous system; CRMP5: collapsin-responsive mediator protein 5; DNER: delta/notch-like epidermal growth factor-related receptor; DPPX: dipeptidyl-peptidase-like protein; GABAAR: gamma-aminobutyric acid-A receptor; GABABR: gamma-aminobutyric acid-B receptor; GFAP: glial fibrillary acidic protein; GAD: glutamic acid decarboxylase; GluK2: glutamate kainate receptor subunit 2; GlyR: glycine receptor; KCTD16: potassium channel tetramerization domain containing; KLHL11: Kelch-like protein 11; LGI1: leucine-rich glioma inactivated protein 1; LEMS: Lambert-Eaton myasthenic syndrome; MAP1B: microtubule-associated protein 1B; MG: myasthenia gravis; mGluR1: metabotropic glutamate receptor 1; mGluR5: metabotropic glutamate receptor 5; MOG: myelin oligodendrocyte glycoprotein; NMDAR: N-methyl-D-aspartate receptor; NSCLC: non-small cell lung cancer; PCA: Purkinje cell antibody; PERM: progressive encephalomyelitis with rigidity and myoclonus; P/Q VGCC: P/Q type voltage-gated calcium channel; SCLC: small cell lung cancer; SEZ6L2: seizure-related 6 homolog like 2; SOX1: SRY-box transcription factor 1.

* Antibodies to mGluR2 are not listed as there is an insufficient number of cases to determine risk of cancer association.
Adapted from: Graus F, Vogrig A, Muñiz-Castrillo S, et al. Updated diagnostic criteria for paraneoplastic neurologic syndromes. Neurol Neuroimmunol Neuroinflamm 2021; 8:e1014. Copyright © The Authors. Available at: https://nn.neurology.org/content/8/4/e1014.long (Accessed on March 28, 2022). Adapted under the terms of the Creative Commons Attribution License 4.0.
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