Diagnosis of Delayed Post-Hypoxic Leukoencephalopathy (Grinker's Myelinopathy) with MRI Using Divided Subtracted Inversion Recovery (dSIR) Sequences: Time for Reappraisal of the Syndrome?

Gil Newburn; Paul Condron; Eryn E Kwon; Joshua P McGeown; Tracy R Melzer; Mark Bydder; Mark Griffin; Miriam Scadeng; Leigh Potter; Samantha J Holdsworth; Daniel M Cornfeld; Graeme M Bydder

doi:10.3390/diagnostics14040418

Diagnosis of Delayed Post-Hypoxic Leukoencephalopathy (Grinker's Myelinopathy) with MRI Using Divided Subtracted Inversion Recovery (dSIR) Sequences: Time for Reappraisal of the Syndrome?

Diagnostics (Basel). 2024 Feb 14;14(4):418. doi: 10.3390/diagnostics14040418.

Authors

Gil Newburn¹, Paul Condron^{1

2}, Eryn E Kwon^{1

2}, Joshua P McGeown¹, Tracy R Melzer^{3

4}, Mark Bydder¹, Mark Griffin^{1

5}, Miriam Scadeng^{1

2}, Leigh Potter¹, Samantha J Holdsworth^{1

2}, Daniel M Cornfeld^{1

2}, Graeme M Bydder^{1

6}

Affiliations

¹ Mātai Medical Research Institute, Tairāwhiti Gisborne 4010, New Zealand.
² Department of Anatomy and Medical Imaging, Faculty of Medical and Health Sciences & Centre for Brain Research, University of Auckland, Auckland 1010, New Zealand.
³ Department of Medicine, University of Otago, Christchurch 8011, New Zealand.
⁴ New Zealand Brain Research Institute, Christchurch 8011, New Zealand.
⁵ Insight Research Services Associated, Gold Coast 4215, Australia.
⁶ Department of Radiology, University of California San Diego, San Diego, CA 92093, USA.

Abstract

Background: 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. T₂-weighted spin echo (T₂-wSE) and/or T₂-Fluid Attenuated Inversion Recovery (T₂-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. Methods and Results: Two cases of DPHL imaged with MRI nine months and two years after probable hypoxic episodes are described. No abnormalities were seen on the T₂-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 T₁. The clinical findings in both cases were of cognitive impairment without Parkinsonism or akinetic mutism. Conclusion: 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.

Keywords: Grinker’s myelinopathy; T1-bipolar filter; delayed post-hypoxic leukoencephalopathy; divided subtracted inversion recovery sequences; targeted MRI of the brain; white matter disease of the brain.

Grants and funding

This research was funded by the Fred Lewis Enterprise Foundation, the Hugh Green Foundation, Manaakia Moves Trust, the JN & HB Williams Foundation, the Mangatawa Beale Williams Memorial Trust and Kānoa New Zealand. We are also grateful for support from GE Healthcare and Mātai Ngā Māngai Māori.