Apheresis in Autoimmune Encephalitis and Autoimmune Dementia
Abstract
:1. Introduction
1.1. Antibody-Mediated AE
1.2. Paraneoplastic AE
1.3. Therapy for AE
2. Search Strategy
2.1. Inclusion Criteria
2.2. Search Strategy
3. Results
3.1. Therapeutic Apheresis in Autoimmune Encephalitides
3.2. Therapeutic Procedure for Apheresis
3.3. Initiation of Therapy with Apheresis and Prior Treatment
3.4. Effects of Treatment with Apheresis in Patients with AE
3.5. Future Treatment Options for Apheresis
3.6. Apheresis in Children with AE
3.7. Autoimmune Dementia and Treatment with Apheresis
3.8. Closing Remarks and Outlook
Author Contributions
Funding
Conflicts of Interest
Ethics Approval
Consent for Publication
References
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Antigen | Clinical Presentation | Age/Gender | Tumor Type |
---|---|---|---|
Antibodies against neurotransmitter receptors [6] | |||
NMDAR [7] | Schizophreniform psychosis, perioral dyskinesia, epileptic seizures, coma, dystonia, hypoventilation | All ages, peak in childhood and youth, 75% women | Ovarian teratoma |
GABAaR | Epileptic seizures, schizophreniform syndrome, refractory status epilepticus and epilepsia partialis continua | Younger adults; m > f (1.5:1) | Hodgkin lymphoma |
GABAbR | LE with frequent epileptic seizures | Older adults f = m | 50% lung cancer (SCLC) |
AMPAR | LE, Epileptic seizures, memory deficits, psychosis | Older Adults f > m (2.3:1) | In 70% lung/breast cancer |
mGluR5 | LE, Ophelia syndrome (depression, agitation, hallucination, memory deficits, personality changes) | Young adults, m > f (1.5:1) | Hodgkin lymphoma |
GlycinR | PERM (progressive encephalomyelitis with rigidity and myoclonus), SPS, cognitive deficits | Older adults f = m | Thymoma (<10%) |
DPPX | LE with tremor, myoclonus, hallucinations, therapy refractory diarrhea | Older adults f < m (1:2.3) | Not known |
Antibodies against ion channel subunits or cell adhesion molecules [8,9] | |||
LGI1 | Facio-brachial dystonic seizures (FBDS), amnesia, psychosis, LE, hyponatremia | Adults > 40 years, m > f (2:1) | Rare |
Caspr2 | LE, neuro-myotonia, Morvan syndrome, can slowly progress over up to 1 year;similar to LGI1, but no hyponatremia | Elderly m > f (9:1) | Thymoma possible |
IgLON5 | REM- and non-REM sleep disorders, sleep apnea, stridor, dysarthria, dysphagia, dysautonomia, movement disorders, dementia | Older adults, f = m | Not known |
Antibodies against intracellular (onconeural) antigens [10,11] | |||
Hu (ANNA-1) | Encephalomyelitis, brainstem encephalitis, LE, Denny-Brown syndrome | Large variability, depending on tumor type | >90%, SCLC |
Ri (ANNA-2) | OMS, CS, encephalomyelitis | >90%, Ovary, breast cancer | |
Yo (PCA-1) | CS | >90%, Ovary cancer | |
Ma2 | LE, CS, diencephalic/hypothalamic involvement | >90%, Testicular, lung cancer | |
CV2 (CRMP5) | Encephalomyelitis, LE, CS | >90%, SCLC, thymoma | |
Amphiphysin | SPS | >90%, Breast, SCLC | |
GAD | SPS, LE, ataxia | Middle aged, f > m (4:1) | Tumor association rare |
Author | Year | Journal | Study Type | AE Type | Sample Size | Procedure | Outcome Measurement | Results | Ref. |
---|---|---|---|---|---|---|---|---|---|
DeSena AD | 2015 | J Clin Aph | Retrospective | NMDAR | 10 | PE | Modified Rankin scale (mRS) | Steroids alone not as effective as steroids followed by PE | [17] |
Ehrlich S | 2012 | Nervenarzt | Retrospective | Antibody-mediated, paraneoplastic | 30 | PE, IA | mRS | Improvement of mRS after PE or IA | [18] |
Heine J | 2016 | J Neurol | Prospective | NMDAR, LGI1, Caspr2, GAD, mGluR5, Hu | 21 | PE, IA | mRS | Improvement of mRS in 60% of patients | [19] |
Hempel P | 2016 | Ther Apher Dial | Prospective | agAAB | 8 | IA | Neuropsychological test | Stabilized cognitive performance after 4-day treatment | [20] |
Köhler W | 2014 | Eur J Neurol | Retrospective | NMDAR, GABA, LGI1, GAD | 13 | IA | mRS | Improvement of mRS in 11/13 patients | [21] |
Onugoren MD | 2016 | Neurol Neuroimmunol Neuroinflamm | Retrospective | LGI1, Caspr2, NMDAR, GAD | 19 | IA | mRS | Improvement of mRS in patients with LGI1, Caspr2, NMDAR, no improvement in patients with GAD | [22] |
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Rössling, R.; Prüss, H. Apheresis in Autoimmune Encephalitis and Autoimmune Dementia. J. Clin. Med. 2020, 9, 2683. https://doi.org/10.3390/jcm9092683
Rössling R, Prüss H. Apheresis in Autoimmune Encephalitis and Autoimmune Dementia. Journal of Clinical Medicine. 2020; 9(9):2683. https://doi.org/10.3390/jcm9092683
Chicago/Turabian StyleRössling, Rosa, and Harald Prüss. 2020. "Apheresis in Autoimmune Encephalitis and Autoimmune Dementia" Journal of Clinical Medicine 9, no. 9: 2683. https://doi.org/10.3390/jcm9092683