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Last edited on June 1, 2023

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--- cl:paraneoplastic-disorders [on April 30, 2020]
+++ cl:paraneoplastic-disorders [on June 1, 2023]
psychdb [Primer]
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 ===== Primer =====
-**Paraneoplastic disorders** can lead to severe psychiatric and behavioural symptoms. Rapid identification is required to ensure correct treatment and management.
+**Paraneoplastic disorders** can lead to severe psychiatric and behavioural symptoms. **Paraneoplastic limbic encephalitis (PLE)** is a rare neurological syndrome associated with cancer, and selectively affects limbic system structures, including the hippocampus, hypothalamus, and amygdala.[([[https://pubmed.ncbi.nlm.nih.gov/29717222/|Shen, K., Xu, Y., Guan, H., Zhong, W., Chen, M., Zhao, J., ... & Wang, M. (2018). Paraneoplastic limbic encephalitis associated with lung cancer. Scientific reports, 8(1), 1-8.]])] The encephalitis is caused by an altered immunologic response to a malignancy that has antigens that resemble limbic antigens. The inflammatory response, however, occurs away from the original neoplasm site.[([[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6319301/|Kacem, M., Belloumi, N., Bachouche, I., Mersni, M., Chermiti Ben Abdallah, F., & Fenniche, S. (2018). Paraneoplastic limbic encephalitis revealing a small cell carcinoma of the lung. Respiratory medicine case reports, 26, 157–160.]])]
+<alert icon="fa fa-arrow-circle-right fa-lg fa-fw" type="success">See main article: **[[cl:0-autoimmune-encephalitis:home]]**</alert>
+===== Autoantibodies =====
+The autoantibody response can be psychiatric in nature, and includes:
+  * **[[cl:0-autoimmune-encephalitis:anti-nmda-receptor|NMDAR-antibody encephalitis]]**, associated with ovarian teratomas in younger women
+  * Ophelia syndrome, associated with surface-directed **mGluR5-antibodies**, seen in patients with Hodgkin’s lymphoma
+  * **Anti-neuronal nuclear antibody-1 (ANNA-1)**, also known as **Hu** paraneoplastic encephalomyelitis, associated with small cell lung cancer in 75% of cases (other tumours include lung, prostate, breast, bladder, GI tract, ovary, neuroendocrine, unknown origin), most commonly in men.
+  * **AMPA receptor antibodies**, associated with lung, breast, thymus, ovarian tumours.
+==== Diagnosis ====
+  * Individuals with a personal or family cancer history, smoking history, should be worked up for paraneoplastic involvement.
+    * The most common tumours associated with paraneoplastic limbic encephalopathy are small cell lung cancer (SCLC) (about 75% of cases), germ-cell tumours (ovarian or testicular), thymoma, Hodgkin's lymphoma, and breast cancer.
+  * The most common symptoms are a subacute cognitive (primarily amnestic) syndrome.
+  * Depression, anxiety, personality changes, and emotional lability can often precede the cognitive impairment.
+===== Investigations =====
+  * [[neurology:lumbar-puncture-lp|CSF]] evidence of inflammation
+  * Elevated tumor markers (e.g. - CEA, CA-125, PSA)
 ===== Literature =====
+  * [[https://pn.bmj.com/content/13/3/165|Ahmed, R. M., Halmagyi, G. M., & Rodriguez, M. L. (2013). Rapidly progressive dementia and ataxia in an elderly man. Practical neurology, 13(3), 165-173.]]
+  * [[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2706263/|Geschwind, M. D., Haman, A., & Miller, B. L. (2007). Rapidly progressive dementia. Neurologic clinics, 25(3), 783-807.]]
   * [[https://www.ncbi.nlm.nih.gov/pubmed/20439389|Kayser, M. S., Kohler, C. G., & Dalmau, J. (2010). Psychiatric manifestations of paraneoplastic disorders. American Journal of Psychiatry, 167(9), 1039-1050.]]
   * [[https://ajp.psychiatryonline.org/doi/full/10.1176/appi.ajp-rj.2016.111106|Reville, P. K., & Steingraeber, C. (2016). ANNA-1 Paraneoplastic Encephalomyelitis Presenting as Rapidly Progressing Dementia in a Man With Undiagnosed Small Cell Lung Cancer. American Journal of Psychiatry Residents' Journal, 11(11), 13-15.]]