Delayed Hypoxic Reversible Leukoencephalopathy Syndrome

Abstract

We report a case of postanoxic leukoencephalopathy in a patient who started to have cognitive and behavioral changes weeks after the anoxic insult along with white matter lesions on neuroimaging and demyelination on brain biopsy. His disease course followed a steady decline initially both clinically and radiologically and assumed a steady plateau. Months after his decline, the patient was seen to be completely functional with substantially improved mental status examination and resolution of white matter changes on imaging. The course of this disease entity usually assumes a plateau after clinical worsening with little improvement subsequently. However, our patient showed a dramatic recovery to his baseline after a few months. In this article, we review mechanisms, presentation and the sequelae of hypoxic injury to the brain.

The Spastic Weakness of the Lower Extremities Fol-lowing Methadone Poisoning: A Case Report

“…In the study by Sputcher et al, the evidence of demyelination with increased myelin-based protein in the cerebrospinal fluid in a case of DPHL associated with prolonged coma following opioid poisoning supported the role of myelin-based protein in this disorder [4]. There are also reports of arylsulfatase deficiency in patients with DPHL [7]. In another study, an autopsy of a patient with DPHL revealed evidence of subcortical myelin sheath loss without U-fiber involvement with prominent macrophage/microglial inflammation and extensive axonal damage [8].…”

Delayed Toxic-Hypoxic Leukoencephalopathy As Sequela of Opioid Overdose and Cerebral Hypoxia-Ischemia

“…Toxic exposure may cause myelinopathy and subsequent demyelination by impairing ATP-dependent enzymes responsible for myelin turnover in the deep white matter [1,15]. Deep white matter injury in DTHL may be a manifestation of the vulnerability of the deep white matter to hypoxia-ischemia [2]. Unlike the corpus callosum and U-fibers, the deep white matter is supplied by widely spaced arterioles with few anastomoses [1,18].…”

“…Delayed post-hypoxic leukoencephalopathy (DPHL), also known as Grinker myelinopathy, delayed anoxic encephalopathy, or post-interval syndrome, is a rare complication of hypoxic-ischemic brain injury [1]. DPHL is characterized by a biphasic clinical course, with initial complete or near-complete recovery from cerebral anoxia, followed by clinical stability over a period of weeks (lucid interval), and subsequent neurological deterioration due to diffuse white matter disease [2]. It has nearly been a century since Grinker recognized the link between delayed leukoencephalopathy and anoxic brain injury [3].…”

“…In 1962, Plum et al reported cases of delayed encephalopathy outside the context of intoxication, e.g., after cardiac arrest or surgery complicated by cerebral hypoperfusion [4]. In the vast majority of DPHL cases, the hypoxic event can be traced to a neurotoxic event, notably carbon monoxide poisoning or drug overdose [1,2].…”