Newburn Gil, Condron Paul, Kwon Eryn E, McGeown Joshua P, Melzer Tracy R, Bydder Mark, Griffin Mark, Scadeng Miriam, Potter Leigh, Holdsworth Samantha J, Cornfeld Daniel M, Bydder Graeme M
Mātai Medical Research Institute, Tairāwhiti Gisborne 4010, New Zealand.
Department of Anatomy and Medical Imaging, Faculty of Medical and Health Sciences & Centre for Brain Research, University of Auckland, Auckland 1010, New Zealand.
Diagnostics (Basel). 2024 Feb 14;14(4):418. doi: 10.3390/diagnostics14040418.
Delayed Post-Hypoxic Leukoencephalopathy (DPHL), or Grinker's myelinopathy, is a syndrome in which extensive changes are seen in the white matter of the cerebral hemispheres with MRI weeks or months after a hypoxic episode. T-weighted spin echo (T-wSE) and/or T-Fluid Attenuated Inversion Recovery (T-FLAIR) images classically show diffuse hyperintensities in white matter which are thought to be near pathognomonic of the condition. The clinical features include Parkinsonism and akinetic mutism. DPHL is generally regarded as a rare condition. Two cases of DPHL imaged with MRI nine months and two years after probable hypoxic episodes are described. No abnormalities were seen on the T-FLAIR images with MRI, but very extensive changes were seen in the white matter of the cerebral and cerebellar hemisphere on divided Subtraction Inversion Recovery (dSIR) images. dSIR sequences may produce ten times the contrast of conventional inversion recovery (IR) sequences from small changes in T. The clinical findings in both cases were of cognitive impairment without Parkinsonism or akinetic mutism. The classic features of DPHL may only represent the severe end of a spectrum of diseases in white matter following global hypoxic injury to the brain. The condition may be much more common than is generally thought but may not be recognized using conventional clinical and MRI criteria for diagnosis. Reappraisal of the syndrome of DPHL to include clinically less severe cases and to encompass recent advances in MRI is advocated.
迟发性缺氧后白质脑病(DPHL),即格林克髓鞘病,是一种综合征,在缺氧发作数周或数月后,通过磁共振成像(MRI)可观察到大脑半球白质出现广泛变化。T加权自旋回波(T-wSE)和/或T液体衰减反转恢复(T-FLAIR)图像通常显示白质弥漫性高信号,这被认为是该病症的特征性表现。临床特征包括帕金森综合征和运动不能性缄默症。DPHL通常被认为是一种罕见病症。本文描述了两例可能的缺氧发作后9个月和2年进行MRI检查的DPHL病例。MRI的T-FLAIR图像未见异常,但在分阶段减法反转恢复(dSIR)图像上,大脑和小脑半球白质出现了非常广泛的变化。dSIR序列可能会因T值的微小变化而产生比传统反转恢复(IR)序列高10倍的对比度。两例患者的临床症状均为认知障碍,无帕金森综合征或运动不能性缄默症。DPHL的典型特征可能仅代表脑整体缺氧性损伤后白质疾病谱中的严重端。该病症可能比一般认为的更为常见,但使用传统的临床和MRI诊断标准可能无法识别。提倡重新评估DPHL综合征,将临床症状较轻的病例纳入其中,并涵盖MRI的最新进展。