造血干细胞移植术后合并韦尼克脑病4例
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摘要
目的:提高对造血干细胞移植(HSCT)术后不常见的神经系统并发症-韦尼克脑病(WE)的临床表现、诊断和治疗的认识及掌握。方法:回顾分析自2016年1月至2017年1月在航天中心医院血液科接受HSCT治疗,术后诊断WE的4例病例的临床特点,诊疗经过以及预后随访。结果:4例确诊WE病例约占同期移植患者的3%,其中男性3例,女性1例,年龄为8-20岁,2例AML,2例ALL。4例中仅1例具有典型的眼颤,共济失调和全脑功能障碍的三联征,所有病例均早期表现为神志模糊和语言障碍。头颅CT平扫均阴性,无提示意义。头颅MRI检查显示,3例具有典型部位(丘脑、第四脑室底、第三脑室和中脑导水管)结构异常病变,另1例仅在乳头体发现病变。全部病例确诊后均接受每日100 mg的维生素B_1肌内注射治疗,分别于治疗后2 d、不详、3 d、4 d后症状改善,2周内全部病例神经系统症状获得完全缓解,随访7-12个月后均未见WE复发。结论:造血干细胞移植后发生神经系统症状,鉴别诊断需考虑WE的可能性,尽早给予维生素B1补充治疗以避免漏诊误诊,从而降低死亡率。
Objective: To make through introduction of Wernicke' s encephalopathy( WE) following hematopoietic stem cell transplantation( HSCT) in terms of clinical characteristics,diagnostic process and treatment.Methods: The clinical charactaristics,diagnostic and therapeutic process and prognostic follow-up in 4 patients diagnosed of WE following HSCT between January 2016 to January 2017 at Department of Hematology,Chinese Aerospace Center Hospital were retrospectively analyzed.Results: Four patients included 2 ALL and 2 AML,and 3 males and 1 female,their age ranged from 8 to 20 years old.4 patients accouted for about 3% of all petients who received HSCT at that time.Typical triad syndrome consisting of ocular motility disorders,ataxia,global confusion was seen in only 1 patient.However,confusion and heterophthongia as onset of this complication were seen in all patients.Cerebral computed tomograph scan was universally unremarkable and useless.Cerebral MRI scan disclosed that typical involvement including thalamus,fourth ventricle,third ventricle,middle cerebral aqueduct was seen in 3,while untypical site including mamillary body was in the remaining 1 patient.All received vitamin B_1 supplement therapy by intramuscular injection at a dose of 100 mg each day.Initial response was observed at 2,unknown,3,4 days after treatment and all obtained complete remission within 2 weeks without any event of relapse after median follow-up period of 8(7-12) months.Conclusion: Any recipient of HSCT with clinical signs or symptoms of central nervous system should receive vitamin B1 supplementary therapy immediately to decrease risk of mortality of WE even if the diagnosis of WE is uncertain.
Objective: To make through introduction of Wernicke' s encephalopathy( WE) following hematopoietic stem cell transplantation( HSCT) in terms of clinical characteristics,diagnostic process and treatment.Methods: The clinical charactaristics,diagnostic and therapeutic process and prognostic follow-up in 4 patients diagnosed of WE following HSCT between January 2016 to January 2017 at Department of Hematology,Chinese Aerospace Center Hospital were retrospectively analyzed.Results: Four patients included 2 ALL and 2 AML,and 3 males and 1 female,their age ranged from 8 to 20 years old.4 patients accouted for about 3% of all petients who received HSCT at that time.Typical triad syndrome consisting of ocular motility disorders,ataxia,global confusion was seen in only 1 patient.However,confusion and heterophthongia as onset of this complication were seen in all patients.Cerebral computed tomograph scan was universally unremarkable and useless.Cerebral MRI scan disclosed that typical involvement including thalamus,fourth ventricle,third ventricle,middle cerebral aqueduct was seen in 3,while untypical site including mamillary body was in the remaining 1 patient.All received vitamin B_1 supplement therapy by intramuscular injection at a dose of 100 mg each day.Initial response was observed at 2,unknown,3,4 days after treatment and all obtained complete remission within 2 weeks without any event of relapse after median follow-up period of 8(7-12) months.Conclusion: Any recipient of HSCT with clinical signs or symptoms of central nervous system should receive vitamin B1 supplementary therapy immediately to decrease risk of mortality of WE even if the diagnosis of WE is uncertain.
引文
1 冯书贤,程坚.造血干细胞移植后的神经系统并发症及诊治进展.东南大学学报(医学版),2015;34(2):285-290.
2 杨益莲,丁家华.118例造血干细胞移植患者中枢神经系统并发症的临床研究.现代医学,2016;44(5):716-720.
3 尹周,张平,张莹,等.造血干细胞移植后神经系统并发症的临床分析.中国临床医学,2016;23(6):776-781.
4 王恒湘,纪树荃,朱玲,等.单倍相合骨髓移植术后中枢神经系统并发症.中国实验血液学杂志,2005;13(6):1128-1130.
5 Azik F,Yazal Erdem A,Tavil B,et al.Neurological complications after allogeneic hematopoietic stem cell transplantation in children,a single center experience.Pediatr Transplant,2014;18(4):405-411.
6 Teive HA,Funke V,Bitencourt MA,et al.Neurological complications of hematopoietic stem cell transplantation(HSCT):a retrospective study in a HSCT center in Brazil.Arq Neuropsiquiatr,2008;66(3B):685-690.
7 Rubin J,Wide K,Remberger M,et al.Acute neurological complications after hematopoietic stem cell transplantation in children.Pediatr Transplant,2005;9(1):62-67.
8 张晓辉.造血干细胞移植后中枢神经系统合并症临床处理.中国实用内科杂志,2014;34(2):142-145.
9 Solmaz S,Gereklioglu,Tan M,et al.A rare complication developing after hematopoietic stem cell transplantation:Wernicke's encephalopathy.Turk J Haematol,2015;32(4):367-370.
10 Han JW,Lim S,Shin HS,et al.Two cases of Wernicke's encephalopathy in young age patients receiving allogeneic hematopoietic stem cell transplantation.Yonsei Med J,2012;53(5):1049-1053.
11 Choi YJ,Park SJ,Kim JS,et al.Wernicke's encephalopathy following allogeneic hematopoietic stem cell transplantation.Korean J Hematol,2010;45(4):279-281.
12 Doss A,Mahad D,Romanowski CA.Wernicke encephalopathy:unusual findings in nonalcoholic patients.J Comput Assist Tomogr,2003;27(2):235-240.
13 Ogershok PR,Rahman A,Nestor S,et al.Wernicke encephalopathy in nonalcoholic patients.Am J Med Sci,2002;323(2):107-111.
14 费国强,钟春玖,金莉蓉,等.非酒精中毒性韦尼克脑病的临床与MRI特征.中国医学计算机成像杂志,2008;14(1):11-16.
15 Harper CG,Giles M,Finlay-Jones R.Clinical signs in the Wernicke-Korsakoff complex:a retrospective analysis of 131 cases diagnosed at necropsy.J Neurol Neurosurg Psychiatry,1986;49(4):341-345.
16 Liu YT,Fuh JL,Lirng JF,et al.Correlation of magnetic resonance images with neuropathology in acute Wernicke's encephalopathy.Clin Neurol Neurosurg,2006;108(7):682-687.
17 Carvajal T,Franco DL,Martínez A,et al.Wernicke's encephalopathy and polyneuropathy associated with vitamin B complex deficiency after a bariatric surgery.Biomedica,2012;32(4):474-484.
18 钟春玖,金莉蓉.颅脑MRI检查在非乙醇中毒性韦尼克脑病诊断中的价值.临床神经病学杂志,2005;18(2):100-102.
19 陈宏,吴学勇,杨茹怡,等.胃肠道肿瘤术后并发韦尼克脑病的诊断与治疗.重庆医科大学学报,2016;41(3):261-265.
20 Renthal W,Marin-Valencia I,Evans PA.Thiamine deficiency secondary to anorexia nervosa:an uncommon cause of peripheral neuropathy and Wernicke encephalopathy in adolescence.Pediatr Neurol,2014;51(1):100-103.
21 牛铭锋,高波,吕翠,等.韦尼克脑病的MRI表现及DWI对临床预后的评价.医学影像学杂志,2010;20(7):941-944.
22 任永富,李保华.脑梗死并发韦尼克脑病8例报告.中国乡村医药,2012;19(1):10.
2 杨益莲,丁家华.118例造血干细胞移植患者中枢神经系统并发症的临床研究.现代医学,2016;44(5):716-720.
3 尹周,张平,张莹,等.造血干细胞移植后神经系统并发症的临床分析.中国临床医学,2016;23(6):776-781.
4 王恒湘,纪树荃,朱玲,等.单倍相合骨髓移植术后中枢神经系统并发症.中国实验血液学杂志,2005;13(6):1128-1130.
5 Azik F,Yazal Erdem A,Tavil B,et al.Neurological complications after allogeneic hematopoietic stem cell transplantation in children,a single center experience.Pediatr Transplant,2014;18(4):405-411.
6 Teive HA,Funke V,Bitencourt MA,et al.Neurological complications of hematopoietic stem cell transplantation(HSCT):a retrospective study in a HSCT center in Brazil.Arq Neuropsiquiatr,2008;66(3B):685-690.
7 Rubin J,Wide K,Remberger M,et al.Acute neurological complications after hematopoietic stem cell transplantation in children.Pediatr Transplant,2005;9(1):62-67.
8 张晓辉.造血干细胞移植后中枢神经系统合并症临床处理.中国实用内科杂志,2014;34(2):142-145.
9 Solmaz S,Gereklioglu,Tan M,et al.A rare complication developing after hematopoietic stem cell transplantation:Wernicke's encephalopathy.Turk J Haematol,2015;32(4):367-370.
10 Han JW,Lim S,Shin HS,et al.Two cases of Wernicke's encephalopathy in young age patients receiving allogeneic hematopoietic stem cell transplantation.Yonsei Med J,2012;53(5):1049-1053.
11 Choi YJ,Park SJ,Kim JS,et al.Wernicke's encephalopathy following allogeneic hematopoietic stem cell transplantation.Korean J Hematol,2010;45(4):279-281.
12 Doss A,Mahad D,Romanowski CA.Wernicke encephalopathy:unusual findings in nonalcoholic patients.J Comput Assist Tomogr,2003;27(2):235-240.
13 Ogershok PR,Rahman A,Nestor S,et al.Wernicke encephalopathy in nonalcoholic patients.Am J Med Sci,2002;323(2):107-111.
14 费国强,钟春玖,金莉蓉,等.非酒精中毒性韦尼克脑病的临床与MRI特征.中国医学计算机成像杂志,2008;14(1):11-16.
15 Harper CG,Giles M,Finlay-Jones R.Clinical signs in the Wernicke-Korsakoff complex:a retrospective analysis of 131 cases diagnosed at necropsy.J Neurol Neurosurg Psychiatry,1986;49(4):341-345.
16 Liu YT,Fuh JL,Lirng JF,et al.Correlation of magnetic resonance images with neuropathology in acute Wernicke's encephalopathy.Clin Neurol Neurosurg,2006;108(7):682-687.
17 Carvajal T,Franco DL,Martínez A,et al.Wernicke's encephalopathy and polyneuropathy associated with vitamin B complex deficiency after a bariatric surgery.Biomedica,2012;32(4):474-484.
18 钟春玖,金莉蓉.颅脑MRI检查在非乙醇中毒性韦尼克脑病诊断中的价值.临床神经病学杂志,2005;18(2):100-102.
19 陈宏,吴学勇,杨茹怡,等.胃肠道肿瘤术后并发韦尼克脑病的诊断与治疗.重庆医科大学学报,2016;41(3):261-265.
20 Renthal W,Marin-Valencia I,Evans PA.Thiamine deficiency secondary to anorexia nervosa:an uncommon cause of peripheral neuropathy and Wernicke encephalopathy in adolescence.Pediatr Neurol,2014;51(1):100-103.
21 牛铭锋,高波,吕翠,等.韦尼克脑病的MRI表现及DWI对临床预后的评价.医学影像学杂志,2010;20(7):941-944.
22 任永富,李保华.脑梗死并发韦尼克脑病8例报告.中国乡村医药,2012;19(1):10.