KEARNS-SAYRE SYNDROME - 2 CLINICOPATHOLOGICAL CASES

被引：15

作者：

BORDARIER, C

DUYCKAERTS, C

ROBAIN, O

PONSOT, G

LAPLANE, D

机构：

[1] LA SALPETRIERE HOSP,NEUROPATHOL LAB,PARIS,FRANCE

[2] HOP ST VINCENT DE PAUL,SERV NEUROPEDIAT,F-75014 PARIS,FRANCE

[3] LA SALPETRIERE HOSP,MALAD SYST NERVEUX CLIN,PARIS,FRANCE

来源：

NEUROPEDIATRICS | 1990年 / 21卷 / 02期

关键词：

capillary proliferation; demyelination; Kearns-Sayre syndrome; Leigh's disease; mitochondrial DNA deletion; spongiform encephalopathy;

D O I：

10.1055/s-2008-1071473

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Two clinico-pathological cases of Kearns-Sayre syndrome are reported. In both cases the typical triad (progressive external ophthalmoplegia, heart block, retinitis pigmentosa) was present and spongiosis was the main pathological finding. In one case there was also a marked capillary proliferation, significance of which is discussed. A deletion of the mitochondrial DNA was found in the muscle, spinal cord and the brain of this last case.

引用

页码：106 / 109

页数：4

共 34 条

[1] BASTIAENSEN LAK, 1978, ACTA NEUROL SCAND, V58, P9
[2] LUMPING OR SPLITTING - OPHTHALMOPLEGIA-PLUS OR KEARNS-SAYRE SYNDROME
BERENBERG, RA
PELLOCK, JM
DIMAURO, S
SCHOTLAND, DL
BONILLA, E
EASTWOOD, A
HAYS, A
VICALE, CT
BEHRENS, M
CHUTORIAN, A
ROWLAND, LP
[J]. ANNALS OF NEUROLOGY, 1977, 1 (01) : 37 - 54
[3] LEUKOENCEPHALOPATHY IN OCULOCRANIOSOMATIC NEUROMUSCULAR DISEASE WITH RAGGED-RED FIBERS - MITOCHONDRIAL ABNORMALITIES DEMONSTRATED BY COMPUTERIZED TOMOGRAPHY
BERTORINI, T
ENGEL, WK
DICHIRO, G
DALAKAS, M
[J]. ARCHIVES OF NEUROLOGY, 1978, 35 (10) : 643 - 647
[4] CASTAIGNE P, 1977, REV NEUROL, V133, P369
[5] CASTAIGNE P, 1971, REV NEUROL, V126, P454
[6] Cerri C, 1983, MITOCHONDRIAL PATHOL, P141
[7] CHOU SM, 1974, NEUROLOGY, V24, P599, DOI 10.1212/WNL.24.6.599
[8] ABRUPT NEUROLOGICAL DETERIORATION IN CHILDREN WITH KEARNS-SAYRE SYNDROME
COULTER, DL
ALLEN, RJ
[J]. ARCHIVES OF NEUROLOGY, 1981, 38 (04) : 247 - 250
[9] DIAGNOSIS OF LEIGHS DISEASE QUESTIONED, DEFENDED - REPLY
CROSBY, TW
[J]. NEUROLOGY, 1974, 24 (06) : 600 - 600
[10] RAGGED-RED FIBERS IN LEIGHS DISEASE
CROSBY, TW
CHOU, SM
[J]. NEUROLOGY, 1974, 24 (01) : 49 - 54

← 1 2 3 4 →