Autoimmune limbic encephalitis: Difference between revisions
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Created page with "== Diagnosis == * Based on presence of all 4 of the following criteriaCiteRef::graus2016a ** Subacute onset (rapid progression of less than 3 mo) of working memory deficits, seizures, or psychiatric symptoms suggesting involvement of the limbic system. ** Bilateral brain abnormalities on ''T''2-weighted fluid-attenuated inversion recovery MRI highly restricted to the medial temporal lobes ** At least one of the following: *** CSF pleocytosis (white blood cell count..." |
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== Clinical Manifestations == |
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* Subacute onset of short-term memory deficits, behaviour changes, anxiety and depression, psychosis, and seizures |
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* From symptom onset to clinical assessment is typically several weeks |
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== Diagnosis == |
== Diagnosis == |
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** Reasonable exclusion of alternative causes |
** Reasonable exclusion of alternative causes |
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== Investigations == |
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=== Antibiotics === |
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{| class="wikitable" |
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|+From [[CiteRef::budhram2019di]] |
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! colspan="1" rowspan="1" |Antibodies |
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! colspan="1" rowspan="1" |Main tumour association |
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! colspan="1" rowspan="1" |Approximate tumour frequency, % |
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|- |
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! colspan="3" |Antibodies to extracellular cell surface or synaptic proteins |
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|- |
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| colspan="1" rowspan="1" |LGI1 |
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| colspan="1" rowspan="1" |Various (thymoma, breast, thyroid, colon, pancreatic and other cancers) |
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| colspan="1" rowspan="1" |10 |
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|- |
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| colspan="1" rowspan="1" |CASPR2 |
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| colspan="1" rowspan="1" |Thymoma |
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| colspan="1" rowspan="1" |20 |
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|- |
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| colspan="1" rowspan="1" |GABABR |
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| colspan="1" rowspan="1" |SCLC |
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| colspan="1" rowspan="1" |50 |
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|- |
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| colspan="1" rowspan="1" |AMPAR |
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| colspan="1" rowspan="1" |SCLC, thymoma |
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| colspan="1" rowspan="1" |60 |
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|- |
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| colspan="1" rowspan="1" |[[Anti-NMDAR encephalitis|NMDAR]] |
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| colspan="1" rowspan="1" |Ovarian teratoma |
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| colspan="1" rowspan="1" |40 |
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|- |
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| colspan="1" rowspan="1" |mGluR5 |
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| colspan="1" rowspan="1" |Hodgkin’s lymphoma |
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| colspan="1" rowspan="1" |50 |
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|- |
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| colspan="1" rowspan="1" |Neurexin-3-α |
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| colspan="1" rowspan="1" |None identified |
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| colspan="1" rowspan="1" |NA |
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|- |
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! colspan="3" |Antibodies to intracellular proteins |
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|- |
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| colspan="1" rowspan="1" |Hu |
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| colspan="1" rowspan="1" |SCLC |
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| colspan="1" rowspan="1" |> 90 |
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|- |
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| colspan="1" rowspan="1" |Ma2 |
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| colspan="1" rowspan="1" |Testicular tumour |
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| colspan="1" rowspan="1" |> 90 |
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|- |
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| colspan="1" rowspan="1" |GAD |
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| colspan="1" rowspan="1" |SCLC, thymoma |
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| colspan="1" rowspan="1" |25 |
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|- |
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| colspan="1" rowspan="1" |Amphiphysin |
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| colspan="1" rowspan="1" |SCLC, breast cancer |
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| colspan="1" rowspan="1" |> 90 |
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|- |
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| colspan="1" rowspan="1" |CV2/CRMP5 |
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| colspan="1" rowspan="1" |SCLC, thymoma |
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| colspan="1" rowspan="1" |> 90 |
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|- |
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| colspan="1" rowspan="1" |AK5 |
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| colspan="1" rowspan="1" |None identified |
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| colspan="1" rowspan="1" |NA |
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|} |
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[[Category:Neurology]] |
[[Category:Neurology]] |
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[[Category:Rheumatology]] |
[[Category:Rheumatology]] |
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Latest revision as of 18:14, 10 April 2026
Clinical Manifestations
- Subacute onset of short-term memory deficits, behaviour changes, anxiety and depression, psychosis, and seizures
- From symptom onset to clinical assessment is typically several weeks
Diagnosis
- Based on presence of all 4 of the following criteria1
- Subacute onset (rapid progression of less than 3 mo) of working memory deficits, seizures, or psychiatric symptoms suggesting involvement of the limbic system.
- Bilateral brain abnormalities on T2-weighted fluid-attenuated inversion recovery MRI highly restricted to the medial temporal lobes
- At least one of the following:
- CSF pleocytosis (white blood cell count of more than 5 cells per mm3)
- EEG with epileptic or slow-wave activity involving the temporal lobes
- Reasonable exclusion of alternative causes
Investigations
Antibiotics
| Antibodies | Main tumour association | Approximate tumour frequency, % |
|---|---|---|
| Antibodies to extracellular cell surface or synaptic proteins | ||
| LGI1 | Various (thymoma, breast, thyroid, colon, pancreatic and other cancers) | 10 |
| CASPR2 | Thymoma | 20 |
| GABABR | SCLC | 50 |
| AMPAR | SCLC, thymoma | 60 |
| NMDAR | Ovarian teratoma | 40 |
| mGluR5 | Hodgkin’s lymphoma | 50 |
| Neurexin-3-α | None identified | NA |
| Antibodies to intracellular proteins | ||
| Hu | SCLC | > 90 |
| Ma2 | Testicular tumour | > 90 |
| GAD | SCLC, thymoma | 25 |
| Amphiphysin | SCLC, breast cancer | > 90 |
| CV2/CRMP5 | SCLC, thymoma | > 90 |
| AK5 | None identified | NA |
References
- ^ Francesc Graus, Maarten J Titulaer, Ramani Balu, Susanne Benseler, Christian G Bien, Tania Cellucci, Irene Cortese, Russell C Dale, Jeffrey M Gelfand, Michael Geschwind, Carol A Glaser, Jerome Honnorat, Romana Höftberger, Takahiro Iizuka, Sarosh R Irani, Eric Lancaster, Frank Leypoldt, Harald Prüss, Alexander Rae-Grant, Markus Reindl, Myrna R Rosenfeld, Kevin Rostásy, Albert Saiz, Arun Venkatesan, Angela Vincent, Klaus-Peter Wandinger, Patrick Waters, Josep Dalmau. A clinical approach to diagnosis of autoimmune encephalitis. The Lancet Neurology. 2016;15(4):391-404. doi:10.1016/s1474-4422(15)00401-9.
- ^ Adrian Budhram, Andrew Leung, Michael W. Nicolle, Jorge G. Burneo. Diagnosing autoimmune limbic encephalitis. Canadian Medical Association Journal. 2019;191(19):E529-E534. doi:10.1503/cmaj.181548.
