LGI1 positive paraneoplastic limbic encephalitis: A case report of atypical presentation

Limbic encephalitis is a rare disorder mainly affecting the medial temporal lobe and is classically paraneoplastic. Autoimmune etiologies also exist, such as antibodies against leucine-rich glioma activated 1 (LGI1). Most cases of anti-LGI1 encephalitis are not associated with tumors. Subacute memory loss is the predominant feature, and most patients develop focal seizures, especially faciobrachial dystonic seizures (FBDSs). Immunotherapies usually show a good response, but are less effective in paraneoplastic cases. We report a case of steroid-responsive anti-LGI1 encephalitis with atypical presentations of sensory aphasia during relapse from rectal carcinoma, an atypical site.

Limbic Encephalitis with HU-Antibodies in T-cell Anaplastic Lymphoma. A Case Report

Limbic encephalitis is a rare paraneoplastic neurological syndrome usually associated withsmall cell lung cancers, testicular and breast cancers or B-cell lymphomas. We herein report thefirst patients with paraneoplastic limbic encephalitis associated with HU antibodies and anaplasticT-cell lymphoma.

Paraneoplastic Limbic Encephalitis Secondary To Mixed Non-Seminomatous Germ Cell Tumours

Casestudy: Testicular tumors account for 1–2% of all tumors in men, with 95% of these being germ cell tumors. The main risk factor for the development of testicular cancer is cryptorchidism. Paraneoplastic limbic encephalitis is a rare sequela of testicular tumor associated with anti-Ma2 and KLH11 antibodies. The most effective treatment for paraneoplastic limbic encephalitis is treatment of the primary malignancy. We present a 41-year-old male that presented to the emergency department with two weeks of episodic alteration of consciousness and memory disturbances. Negative neurologic evaluation and imaging led to concern for a paraneoplastic process from a distant malignancy. CT imaging revealed an enlarged, necrotic para-aortic lymph node and subsequent ultrasound demonstrated a right sided testicular mass. Right radical orchiectomy was performed. Microscopically, the mass consisted of mixed respiratory epithelium, gastrointestinal glands and squamous epithelium with keratinization consistent with a post-pubertal testicular teratoma with associated in-situ germ cell neoplasia. Resection of the para-aortic mass revealed large anaplastic cells with epithelioid features, nuclear pleomorphism and frequent mitoses. Immunostaining was positive for Pan-Keratin and OCT4, consistent with poorly differentiated embryonal carcinoma. Resection of the primary and metastatic disease, as well as treatment with corticosteroids resulted in resolution of the encephalitis. This presentation of severe neurological disturbances in the setting of a metastatic mixed nonseminomatous germ cell tumor represents a rare presentation of paraneoplastic limbic encephalitis.