- Last edited on June 1, 2023
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+ | ====== Psychiatric Manifestations of Paraneoplastic Disorders ====== | ||
+ | {{INLINETOC}} | ||
+ | ===== Primer ===== | ||
+ | **Paraneoplastic disorders** can lead to severe psychiatric and behavioural symptoms. Rapid identification is required to ensure correct treatment and management to avoid misdiagnosis of a paraneoplastic neurologic disorder. Paraneoplastic neurological syndromes (PNS) are the immune-mediated effects of a remote cancer and are characterized by an autoantibody response against the antigens expressed by the tumour. <alert icon="fa fa-arrow-circle-right fa-lg fa-fw" type="success">See main article: **[[cl:0-autoimmune-encephalitis:home]]**</alert> | ||
+ | **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.]])] It is usually associated with small cell lung cancer. 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.]])] | ||
+ | |||
+ | ===== 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.]] |