Declan Brennan1, Olwen C Murphy1,2, Conor Fearon1, Francesca Brett3, Brian Murray1, Tim Lynch1. 1. Centre for Brain Health Dublin Neurological Institute at the Mater Misericordiae University Hospital Dublin Ireland. 2. Division of Neuroimmunology Johns Hopkins Hospital Baltimore Maryland USA. 3. Department of Neuropathology Beaumount Hospital Dublin Ireland.
Abstract
BACKGROUND: We describe the clinical and neuropathological features of a patient with T-cell-mediated paraneoplastic limbic encephalitis, parkinsonism, hypothermia, and narcolepsy-like presentation associated with endometrial carcinoma. OBJECTIVES: This patient with prominent parkinsonism and narcolepsy broadens the phenotype of known paraneoplastic syndromes and demonstrates the importance of investigation for occult malignancy even in the absence of paraneoplastic antibodies. METHODS: This is a case report with diagnosis confirmed at postmortem. RESULTS: Paraneoplastic antibodies were not detected. The initial improvement with immunosuppression was short lived, and postmortem neuropathological examination demonstrated encephalitis with predominant T-cell infiltration affecting the hypothalamus and extending to the brainstem, suggestive of a paraneoplastic syndrome. CONCLUSIONS: Although the possibility of a novel antibody cannot be ruled out, consideration must also be given to recent demonstration of purely T-cell-mediated neuronal destruction in the context of paraneoplastic syndromes.
BACKGROUND: We describe the clinical and neuropathological features of a patient with T-cell-mediated paraneoplastic limbic encephalitis, parkinsonism, hypothermia, and narcolepsy-like presentation associated with endometrial carcinoma. OBJECTIVES: This patient with prominent parkinsonism and narcolepsy broadens the phenotype of known paraneoplastic syndromes and demonstrates the importance of investigation for occult malignancy even in the absence of paraneoplastic antibodies. METHODS: This is a case report with diagnosis confirmed at postmortem. RESULTS: Paraneoplastic antibodies were not detected. The initial improvement with immunosuppression was short lived, and postmortem neuropathological examination demonstrated encephalitis with predominant T-cell infiltration affecting the hypothalamus and extending to the brainstem, suggestive of a paraneoplastic syndrome. CONCLUSIONS: Although the possibility of a novel antibody cannot be ruled out, consideration must also be given to recent demonstration of purely T-cell-mediated neuronal destruction in the context of paraneoplastic syndromes.
Authors: B Ripley; S Overeem; N Fujiki; S Nevsimalova; M Uchino; J Yesavage; D Di Monte; K Dohi; A Melberg; G J Lammers; Y Nishida; F W Roelandse; M Hungs; E Mignot; S Nishino Journal: Neurology Date: 2001-12-26 Impact factor: 9.910
Authors: Caleigh Mandel-Brehm; Divyanshu Dubey; Thomas J Kryzer; Brian D O'Donovan; Baouyen Tran; Sara E Vazquez; Hannah A Sample; Kelsey C Zorn; Lillian M Khan; Ian O Bledsoe; Andrew McKeon; Samuel J Pleasure; Vanda A Lennon; Joseph L DeRisi; Michael R Wilson; Sean J Pittock Journal: N Engl J Med Date: 2019-07-04 Impact factor: 91.245
Authors: F Graus; J Y Delattre; J C Antoine; J Dalmau; B Giometto; W Grisold; J Honnorat; P Sillevis Smitt; Ch Vedeler; J J G M Verschuuren; A Vincent; R Voltz Journal: J Neurol Neurosurg Psychiatry Date: 2004-08 Impact factor: 10.154