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DSS 2013-Case 2

This case study examines a rare complication of prolonged cerebral hypoxia, known as delayed post-hypoxic leukoencephalopathy (DPHL). The patient initially recovers within 24 hours but later develops a sudden onset of neuropsychiatric symptoms. The exact incidence and etiology of DPHL are not known, but it is believed to involve mitochondrial dysfunction and reduced cerebral blood flow. The neuropathological findings include severe diffuse hemispheric demyelination. This case highlights the importance of recognizing and managing this rare condition.

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DSS 2013-Case 2

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  1. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY DSS 2013-Case 2 Declan McGuone, Jeremy Schmahmann, E. Tessa Hedley-Whyte, Matthew P. Frosch

  2. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Disclosures NONE

  3. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Clinical History • 69 yo pain & stiffness 2 days after neck lipectomy • No sx of infection or neurologic symptoms • Hydromorphone (1.5mg + 1mg) & diphenhydramine (25+25 mg) • Admitted. Next AM unresponsive, hypotensive & hypoxic. Resuscitated and receives Narcan • Discharged home at baseline 3 days later

  4. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Clinical History, cont. • 3 WKS : • Behavioral, memory & attention deficits appeared. • Over next 72 hrs rapid decline in mental status with progressive akinesia, mutism & rigidity • Lab investigations • Unremarkable T2/FLAIR

  5. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Clinical History • 3 MOS : she was alert, responsive, followed simple commands. • 6-9 MOS: fluent speech, paranoid & delusional with impaired visuo-spatial, memory encoding, exec. & behavioral functions. • Death 2 years following presentation

  6. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY 1300g

  7. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY

  8. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Discussion

  9. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY

  10. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Neurofilament GFAP

  11. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY CD45 CD68 CD45

  12. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Diagnosis Delayed post-hypoxic leukoencephalopathy

  13. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Delayed post-hypoxic leukoencephalopathy • A rare complication of prolonged cerebral hypoxia • The classic clinical presentation is biphasic – patients recover within 24hours of a prolonged hypoxic injury and return to baseline before developing a sudden onset neuropsychiatric syndrome within 1-3 weeks • Exact incidence is not known • Etiology of insults reported to cause DPHL is heterogeneous (e.g. anoxic anoxia, anemic anoxia and ischemic anoxia)

  14. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Delayed post-hypoxic leukoencephalopathy • Two clinical phenotypes: • Parkinsonism • Rigidity, tremor, masked facies. dystonic posturing, agitation, apathy, hallucinations, odd behavior, impaired cognition, emotional lability • Akinetic mutism • Apathy, functional incontinence, pathologic laughter or crying, • Examination: • Frontal release signs • CST signs (hyperreflexia, Babinski) • Frontal-executive dysfunction

  15. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Neuropathology • Severe diffuse hemispheric demyelination (sparing of U-fibers) • Morphologically normal oligodendroglia • No vacuolar change (as sometimes seen with inhaled heroin) • Preserved neocortical and hippocampal architecture • Elevated CSF myelin basic protein (marker of acute widespread demyelination) Plum and Posner, Archives internal medicine, 1962

  16. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY Mechanism • Unknown but likely multifactorial • ? Mitochondrial dysfunction • DPHL can be reproduced in animals using potassium cyanide to impair cytochrome c (Shprecher D, Mehta L, et al. Neurorehabilitation, 2010) • ? Reduced cerebral blood flow (DPHL more prevalent and more severe in older patients with cerebrovascular disease) • ? Pseudodeficiency of Arylsulfatase A (Gottfried JA, Mayer SA et al. Neurology 1997) • ? Delayed oligodendroglial apoptosis (Chu K, Jung KH et al. Eur Neurol 2004)

  17. HARVARDMEDICAL SCHOOL MASSACHUSETTS GENERAL HOSPITAL PATHOLOGY References [1] Plum F, Posner JB, et al. Delayed neurological deterioration after anoxia. Arch Intern Med, 1962 [2] Shprecher D, Mehta L. The syndrome of delayed post-hypoxic leukoencephalopathy. Neurorehabilitation, 2010. [3] Shprecher D, Flanigan K et al. Clinical and diagnostic features of delayed hypoxic leukeoncephalopathy. J Neuropsychitary Clin Neurosci, 2008 [4] Choi IS. Delayed neurologic sequelae in carbon monoxide intoxication. Arch Neurol, 1983 [5] Gottfried JA, et al. Delayed posthypoxic demyelination. Association with arylsulfatase A deficiency and lactic acidosis on proton MR spectroscopy. Neurology 1997.

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