Abstract Details

Title Delayed Hypoxic Reversible Leukencephalopathy Syndrome( DHRLS):

Topic MS and Related Diseases

Presentation(s) P02 - (-)

Poster/Presentation
Number 122

Objective

Background BACKGROUND: Delayed hypoxic leukoencephalopathy is a rare complication of hypoxic-ischemic encephalopathy.Prolonged hypoxia leading to impaired oxygen delivery is thought to disrupt ATP-dependent enzymatic pathways involved in myelin turnover, resulting in delayed demyelination.Majority recover spontaneously.

Design/Methods DESIGN/METHODS: Case report and review of literature.

Results RESULTS: A 36 year old male admitted after being found unresponsive for unknown period. He has a significant history of hypertension, obstructive sleep apnea, depression and substance abuse. He developed multiorgan failure but made complete recovery within a week and was discharged home. Three weeks later,he was readmitted with acute onset neuropsychiatric menaifestations, incontenence and gait imbalalance. His examiantiuon showed gross impairement of memory, language function( MMSE 14), disinhibited behavior,primitive reflexes without any weakness, normal DTrs and flexor plantar. The Brain MRI showed non enhancing diffuse white matter lesions.He has normal CSF with normal MBP, IgG index along with normal level of Arylsulphatase.The SPECT scan showed normal cortical perfusion. EEG showed diffuse slowing with disruption of sleep architecture. Brain biospy showed leukoencephalopathy with marked gliosis and microglial activation.Cerebral angiogram was normal.A trial of steroids did not bring upon any change. His declining cognition reached a nadir in 3 weeks at which time he was discharged to a nursing home. A follow up at three months showed significant recovery with normal cognitive fucntions( MMSE30) with normal gait and no incontinenence.His MRI brain also showed significant reduction in white matter lesions.

Conclusions CONCLUSIONS: Delayed hypoxic encephalopathy is a rare condition mostly reported with CO poisoning.There are also case reports with pseudo arylsulphatase deficiency.Most common pathology is demylination and most patients recover.Our patient has normal arylsuphatase, demyelination on biopsy, complete clinical recovery with significant radiological resolution of white matter lesion.The pathophysiological picture has resemblalne to acute disseminated encephalopmeylitis but exact pathogenesis still eludes us.

Authors/Disclosures
Pradeep C. Bollu, MD PRESENTER	Dr. Bollu has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Abbvie.
Shi Y. Lim, MD (Kaiser Fremont Medical Center)	No disclosure on file
Anantha Vellipuram, MD	Dr. Vellipuram has nothing to disclose.
Gulshan Uppal, MD, FAAN (Freeman Neurology and Headache Clinic)	Dr. Uppal has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Abbvie. Dr. Uppal has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Pfizer.
Niranjan N. Singh, MD, FAAN	Dr. Singh has nothing to disclose.
Kate Bushby, MD	Dr. Bushby has nothing to disclose.

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