CAJ | 학술논문

目的：观察慢性进行性眼外肌麻痹(CPEO)和 Kearns-Sayre 综合征(KSS)患者视网膜神经纤维层(RNFL)厚度,分析其影响因素。方法经肌肉病理活检确诊的 CPEO 患者22例纳入研究。其中,男性7例,女性15例。平均年龄(29.09±13.40)岁；平均起病年龄(16.4±10.7岁)。平均病程(11.30±7.30)年。22例患者中,CPEO 患者18例(CPEO 组)；KSS 患者4例(KSS 组)。患者均行频域光相干断层扫描(SD-OCT)检查。选取左眼为受检眼,以视盘中心为圆心,直径为3.45 mm 的环形扫描。扫描分为上方颞侧(ST)、颞上方(TU)、颞下方(TL)、下方颞侧(IT)、下方鼻侧(IN)、鼻下方(NL)、鼻上方(NU)、上方鼻侧(SN)。Pearson 相关分析法分析 RNFL 厚度与患者起病时间、病程的相关性。结果22例患者视盘旁全周平均 RNFL 厚度为(101.5±14.4)μm。ST、TU、TL、IT、IN、NL、NU、SN 平均 RNFL 厚度分别为(136.3±24.1)、(85.4±25.7)、(68.2±11.7)、(128.2±28.7)、(127.3±29.5)、(66.7±16.8)、(70.1±17.6)、(122.9±20.2)μm。KSS 组视盘旁全周及各象限平均 RNFL 厚度均较 CPEO 组低,但差异无统计学意义(P >0.05)。相关性分析结果显示,RNFL 厚度下降与起病年龄无相关性(r =-0.306,P =0.11)；与病程呈负相关(r =-0.518,P =0.03),尤其是鼻侧4个象限 RNFL 厚度与病程呈负相关(IN：r =-0.555,P =0.01；NL：r=-0.630,P =0.00；NU：r =-0.559,P =0.01,SN：r =-0.557,P =0.01)。结论CPEO、KSS 患者 RNFL 厚度差异无统计学意义；RNFL 厚度特别是鼻侧 RNFL 厚度与病程呈负相关。
목적：관찰만성진행성안외기마비(CPEO)화 Kearns-Sayre 종합정(KSS)환자시망막신경섬유층(RNFL)후도,분석기영향인소。방법경기육병리활검학진적 CPEO 환자22례납입연구。기중,남성7례,녀성15례。평균년령(29.09±13.40)세；평균기병년령(16.4±10.7세)。평균병정(11.30±7.30)년。22례환자중,CPEO 환자18례(CPEO 조)；KSS 환자4례(KSS 조)。환자균행빈역광상간단층소묘(SD-OCT)검사。선취좌안위수검안,이시반중심위원심,직경위3.45 mm 적배형소묘。소묘분위상방섭측(ST)、섭상방(TU)、섭하방(TL)、하방섭측(IT)、하방비측(IN)、비하방(NL)、비상방(NU)、상방비측(SN)。Pearson 상관분석법분석 RNFL 후도여환자기병시간、병정적상관성。결과22례환자시반방전주평균 RNFL 후도위(101.5±14.4)μm。ST、TU、TL、IT、IN、NL、NU、SN 평균 RNFL 후도분별위(136.3±24.1)、(85.4±25.7)、(68.2±11.7)、(128.2±28.7)、(127.3±29.5)、(66.7±16.8)、(70.1±17.6)、(122.9±20.2)μm。KSS 조시반방전주급각상한평균 RNFL 후도균교 CPEO 조저,단차이무통계학의의(P >0.05)。상관성분석결과현시,RNFL 후도하강여기병년령무상관성(r =-0.306,P =0.11)；여병정정부상관(r =-0.518,P =0.03),우기시비측4개상한 RNFL 후도여병정정부상관(IN：r =-0.555,P =0.01；NL：r=-0.630,P =0.00；NU：r =-0.559,P =0.01,SN：r =-0.557,P =0.01)。결론CPEO、KSS 환자 RNFL 후도차이무통계학의의；RNFL 후도특별시비측 RNFL 후도여병정정부상관。
Objective To determine the retinal nerve fiber layer thickness (RNFLT)of chronic progressive external ophthalmoplegia (CPEO)and kearns-sayre syndrome (KSS)patients using spectral-domain optical coherence tomography (SD-OCT)and to analyze the potential influence factors for RNFLT of these patients.Methods 18 CPEO and 4 KSS (CPEO with retinitis pigmentosa and cardiac block)patients, all were muscle biopsy confirmed,were included in this study.There were 7 males and 1 5 females,the average age was (29.09 ± 13.40)years,the average onset age was (1 6.4 ± 10.7 )years and the average disease duration was (1 1.30 ± 7.30)years.All the patients underwent SD-OCT examination for the left eye,the peripapillary RNFL thickness was measured using the Spectralis 3.45 mm circle scan protocol.8 quadrants were scanned including superior temporal (ST),temporal upper (TU),temporal lower (TL), inferior temporal (IT),inferior nasal (IN),nasal lower (NL),nasal upper (NU)and superior nasal (SN). The relationship between RNFLT and onset time,disease duration was analyzed by Pearson correlation analysis.Results The average RNFLT of ST,TU,TL,IT,IN,NL,NU,SN in the 22 patients were (136.3±24.1),(85.4±25.7),(68.2±1 1.7),(128.2±28.7),(127.3±29.5),(66.7±1 6.8),(70.1± 1 7.6)μm,respectively.The circumferential average RNFLT was (101.5 ± 14.4 )μm.There was no significant difference between the KSS group and CPEO group (P > 0.05 ). The decrease of the circumferential RNFLT had no relationship with the onset age (r = - 0.306,P = 0.1 1 ),but a negative relationship with the disease course (r=-0.5 18,P =0.03).There were negative relationships between the disease course and RNFLT of 4 nasal quadrants (IN:r=-0.555,P =0.01,NL:r=-0.630,P =0.00 NU:r =-0.559,P =0.01,SN:r =-0.557,P =0.01).Conclusion There is no difference in RNFLT of patients with CPEO and KSS.There is a negative relation between RNFLT (especially RNFLT of 4 nasal quadrants)and disease course.