语义性痴呆患者的临床及影像学特点分析

Analysis of clinical and imaging characteristics of patients with semantic dementia

目的:语义性痴呆(Semantic dementia,SD)为额颞叶变性的一个亚型,目前国内相关报道较少。本文通过分析4例语义性痴呆患者的临床及影像学资料,以提高对该病认识。方法:收集4例语义性痴呆患者人口学信息,病史及体征,神经心理学量表,语言学量表、核磁共振(MRI)及正电子发射计算机断层显像(PET-CT)资料,进行回顾性分析。结果:4例患者中男性1例,女性3例,均以言语功能障碍起病,主要表现为词汇理解障碍,例1同时出现命名障碍。就诊时4例患者均存在命名障碍,尤以对证命名受损严重,其中例3、例4患者语法正确,自发性谈话语言表达流利,但名词使用减少,常伴有赘语及错语。例1及例2患者随病程进展出现言语欠流利,自发性谈话有明显停顿,找词困难。4例患者执行口头命令,复述及计算力、注意力及日常生活能力均相对保留,均早期出现记忆力下降,3例患者出现视空间能力受损,其中例4受累明显。精神行为方面,例2患者出现睡眠障碍及食欲减退,睡眠障碍主要表现为入睡困难、早醒,例4患者出现明显精神行为异常,表现出激越攻击行为,易激惹及睡眠障碍。头结构MRI示例1-例3以左侧颞叶萎缩为主,例4以双侧颞叶及海马为著,例2-例4行PET/CT检查,18F-氟脱氧葡萄糖成像,例2示双额内外侧、双岛叶、左颞内外侧及左尾状核代谢减低,例3示双额内外侧、双颞外侧、双海马区、双岛叶及双尾状核代谢减低,例4示双额内外侧、双颞内外侧、右岛叶及右尾状核代谢减低。结论:本文4例语义性痴呆患者临床特征及影像学检查表现出各自特点,存在个体间差异。

Objective:Semantic dementia(SD)is a subtype of frontotemporal lobe degeneration and rarely reported in China.In order to improve the understanding of semantic dementia,the clinical and imaging data of 4 cases were analyzed.Methods:The demographic information,medical history,neuropsychological scale,linguistic scale,magnetic resonance imaging(MRI)and positron emission computed tomography(PET-CT)data of 4 patients with semantic dementia were retrospectively analyzed.Results:Among the 4 patients,1 was male and 3 was female.All of them onset with speech dysfunction,mainly manifested as vocabulary comprehension disorder.Case 1 had naming disorder at the same time.Among them,cases 3 and 4 had correct grammar and fluent spontaneous speech,but the use of nouns was reduced,often accompanied by redundant words and wrong words.Patients in case 1 and case 2 had poor fluency,obvious pause in spontaneous conversation and difficulty in finding words.Retelling and calculation ability,attention and daily life ability were relatively reserved.All patients had early memory decline.Three patients had visual spatial ability impairment,of which case 4 were significantly affected.In the aspect of mental behavior,case 2 had sleep disorder and anorexia.Case 4 had obvious mental behavior abnormality,showing aggressive behavior,irritability and sleep disorder.On MRI of head structure,cases 1-3 showed atrophy of left temporal lobe,case 4 showed atrophy of bilateral temporal lobe and hippocampus,case 2-4 underwent PET/CT and 18F fluorodeoxyglucose(FDG)imaging,case 2 showed decreased metabolism of bilateral medial and lateral frontal lobe,bilateral insular lobe,bilateral temporal lobe and caudate nucleus,case 3 showed decreased metabolism of bilateral medial and lateral frontal lobe,bilateral temporal lobe,bilateral hippocampus,bilateral insular lobe and bilateral caudate nucleus,Case 4 showed decreased metabolism in bilateral medial and lateral frontal,bilateral medial and lateral temporal,right insular lobe and right caudate nucleus.Conclusion:The clinical and imaging features of 4 patients with semantic dementia were different from each other.