Wu Guolu, Shi Yanhui, Sun Jialan, Wu Feifei, Jiang Mei, Li Qiang
Department of Neurology, Shanghai Gongli Hospital of Pudong New Area, Shanghai, China.
Department of Neurology, Xuhui District Central Hospital, Shanghai, China.
Medicine (Baltimore). 2025 Feb 14;104(7):e41534. doi: 10.1097/MD.0000000000041534.
Anti-dipeptidyl-peptidase-like-protein 6 (DPPX) encephalitis is a rare form of autoimmune encephalitis, with no more than 80 cases reported to date. Cases of anti-DPPX encephalitis comorbid malignancy are exceedingly rare. Limited cases and diverse clinical presentations bring difficulties in the understanding of this disease, including the etiology, diagnosis, treatment, and prognosis. Herein, we report a case of insidious-onset anti-DPPX encephalitis in a patient with a prior history of Epstein-Barr Virus (EBV) meningitis diagnosed with gastric cancer after 2 years of follow-up.
A 72-year-old emaciated male presented with recurrent seizures over the last 20 years, personality changes over 10 years, and memory loss lasting 2 years.
The patient was diagnosed with epilepsy, psychiatric symptoms (agitation and irritability), and mild cognitive impairment. Anti-DPPX encephalitis and prior EBV infection were ultimately diagnosed based on the combination of his symptoms and positive DPPX antibody in serum (titer 1:10) and positive EBV-IgG antibody in cerebrospinal fluid.
The patient received a course of intravenous methylprednisolone and oral sodium valproate to treat the seizures.
After 10 days of treatment, no seizures reoccurred, although the psychiatric symptoms persisted, and his serum antibody against DPPX was still positive (titer 1:10). Unfortunately, the family members asked for the patient to be discharged automatically and refused oral steroids after discharge. Through regular telephone follow-ups for 2 years after discharge, we learned that the patient did not experience any similar convulsions but still showed irritability when administered perphenazine. Unfortunately, the patient had gastric cancer with multiple metastases and was receiving palliative care.
This report illustrates a rare case of EBV meningitis in childhood, resulting in a long-standing, stable course of anti-DPPX encephalitis, and subsequent gastric cancer. This case broadens the atypical presentation spectrum of anti-DPPX encephalitis and emphasizes the need for screening for malignant tumors, including lymphoma, nasopharyngeal carcinoma, and gastric cancer, particularly in patients with latent EBV infection.
抗二肽基肽酶样蛋白6（DPPX）脑炎是自身免疫性脑炎的一种罕见形式，迄今为止报告的病例不超过80例。抗DPPX脑炎合并恶性肿瘤的病例极为罕见。病例有限且临床表现多样给理解这种疾病带来困难，包括病因、诊断、治疗和预后。在此，我们报告一例隐匿性起病的抗DPPX脑炎病例，该患者既往有EB病毒（EBV）脑膜炎病史，随访2年后诊断为胃癌。
一名72岁消瘦男性，过去20年反复出现癫痫发作，10年来有性格改变，记忆力减退持续2年。
该患者被诊断为癫痫、精神症状（烦躁和易怒）及轻度认知障碍。最终根据其症状以及血清中DPPX抗体阳性（滴度1:10）和脑脊液中EBV-IgG抗体阳性，诊断为抗DPPX脑炎和既往EBV感染。
患者接受了一个疗程的静脉注射甲泼尼龙和口服丙戊酸钠以治疗癫痫发作。
治疗10天后，未再出现癫痫发作，尽管精神症状仍持续存在，且其抗DPPX血清抗体仍为阳性（滴度1:10）。不幸的是，家属要求自动出院并拒绝出院后口服类固醇药物。出院后通过2年的定期电话随访，我们了解到患者未再出现任何类似抽搐，但在服用奋乃静时仍表现出易怒。不幸的是，患者患有胃癌伴多发转移，正在接受姑息治疗。
本报告阐述了一例儿童期EBV脑膜炎的罕见病例，导致了抗DPPX脑炎的长期稳定病程以及随后的胃癌。该病例拓宽了抗DPPX脑炎的非典型表现谱，并强调了对恶性肿瘤进行筛查的必要性，包括淋巴瘤、鼻咽癌和胃癌，尤其是在潜伏性EBV感染患者中。
