| NMDAR |
21 (2 months- 85 year)/1:4 |
Clinical features on children’s presentation are usually with neurological symptoms: seizures and dyskinesias; in adults: behavioral changes. |
Varies with age and sex Teratoma in almost 50% of young women (aged between 12-45 year) Rare in children and males (6%) titulaer 2013, Graus 2021 Elderly patients (23%), but usually tumors are carcinomas (titulaer late onset) |
| LGI1 |
64 years (31-84 years)/2:1 |
Limbic encephalitis. subtle focal seizures (66%, autonomic or dyscognitive) and faciobrachial dystonic seizures (FBDS, 47%) mostly occurred before onset of cognitive deficits, hyponatremia (60%). MRI normal in 26% od patients, unilateral hippocampal T2/FLAIR hypersignal in 60%, bilateral in 14% CSF cell count and protein unremarkable in 75% |
Malignant thymoma and neuroendocrine (<10% of cases) |
| CASPR2 |
66 years(25-77)/9:1 |
Morvan Syndrome and Limbic Encephalitis Seventy-seven percent of the patients had 3 or more of the following symptoms: encephalopathy (cognitive deficits/seizures), cerebellar dysfunction, peripheral nervous system hyperexcitability, dysautonomia, insomnia, neuropathic pain, or weight loss. May have a more protracted clinical course. Median time to nadir of disease was 4 months, and in 30% of patients in 1 year. Increased T2/FLAIR signal in medial temporal lobes in 45% of patients |
Tumor association varies with the syndrome. When clinical is Morvan Syndrome, tumor (usually thymoma) if found in 50% of patients When associated with other syndromes, association is low |
| AMPAR |
56 years (23-81)/1:2.3 |
Limbic encephalitis, Limbic encephalitis with multifocal or diffuse encephalopathy, in rare cases with prominent psychiatric features Increased T2/FLAIR signal in medial temporal loes in 67% of patients |
SCLC and malignant thymoma Presence of tumor is higher when other onconeural antibodies occur simultaneously |
| GABAbR |
61 year (16-77)/ 1.5:1 |
Limbic encephalitis, prominent seizures Increased T2/FLAIR signal in medial temporal lobes in 45% of patients |
SCLC Tumor association is higher in elderly men, smokers and co-occurrence of anti-KCTD16 antibodies |
| GABAaR |
40 years (2 mo-88 years); 1:1 |
Seizures, confusion, behavioral changes. Encephalitis with frequent status epilepticus Cortical and subcortical multifocal abnormalities |
Thymoma, paraneoplastic origin is more frequent in adults (60%) than in children (10%) |
| mGLUR1 |
55 year (43-64)/1.3 |
Subacute cerebellar syndrome |
30% most hematologic |
| mGLUR5 |
29 year (6-75)/ 1.5:1 |
Encephalitis, main clinical features are psychiatric (Ophelia Syndrome), cognitive , movement disorders, sleep dysfunction, and seizures MRI abnormalities usually involving extra limbic regions |
Hodgkin lymphoma in approximately 50% of patients |
| DPPX |
52 year (13-76)/ 2.3:1 |
Prodrome with diarrhea and weight loss. Encephalitis with hyperekplexia, myoclonus and tremors |
B cell neoplasms(<10%) |
| Neurexin 3-alfa |
44 year (23-57)/2:4 |
Encephalitis, patients may have history or laboratory findings suggestive of systemic autoimmunity, such as increased antinuclear antibodies (ANA), Raynaud < arthralgias |
No associated cancer |
| GluK2 |
28 years (14-75 )/1.6:1 |
Encephalitis with prominent clinicoradiological cerebellar involvement, cases of patients with obstructive hydrocephalus) |
Few published cases, 2 patients with tumor (Hodgkin’s lymphoma, ovarian teratoma) In this paper there 5 additional patients that had other antibodies concurrent with Gluk2 antibodies, in those 4 had tumors (3 thymoma, 1 small cell lung cancer) |
| GlyR |
50 year(1-75) /1:1 |
PERM (progressive encephalopathy with rigidity and myoclonus), Limbic encephalitis |
(<10%) Malignant thymoma and Hodgkin lymphoma |
| MOG |
37 year (1-74) 1:1 |
Msost important biomarker of autoimmune encephalitis in children, other than ADEM spectrum, Imaging may resemble that of GABAaR e anti-dopamine 2 receptor ). Phenotype associated with bilateral cortical involvement and leukodystrophy-like has a poor prognosis In adults, beyond the well known presentation (ADEM, optic neuritis, transverse myelitis, demyelinating brain or brainstem syndromes) patients may have overlap with anti-NMDAR encephalitis or present with a benign, unilateral, cerebral cortical encephalitis with epilepsy |
Low risk, only 5 cases reported, mostly teratomas |
| IgLON5 |
62 year (42-91) 1,25:1 |
distinctive sleep disorder in association with one or more of the following symptoms: bulbar dysfunction, gait difficulties, oculomotor abnormalities, chorea, or cognitive deterioration. |
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