DSS ITSEC 2013 Conference 07.11.2013 - Cadence - ExtremeNetworks
DSS 2013-Case 2
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Transcript of DSS 2013-Case 2
DSS 2013-Case 2
Declan McGuone, Jeremy Schmahmann,
E. Tessa Hedley-Whyte, Matthew P. Frosch
MASSACHUSETTSGENERAL HOSPITAL
PATHOLOGY
HARVARDMEDICAL SCHOOL
Disclosures
NONE
MASSACHUSETTSGENERAL HOSPITAL
PATHOLOGY
HARVARDMEDICAL SCHOOL
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
MASSACHUSETTSGENERAL HOSPITAL
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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
MASSACHUSETTSGENERAL HOSPITAL
PATHOLOGY
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T2/FLAIR
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
MASSACHUSETTSGENERAL HOSPITAL
PATHOLOGY
HARVARDMEDICAL SCHOOL
MASSACHUSETTSGENERAL HOSPITAL
PATHOLOGY
HARVARDMEDICAL SCHOOL
1300g
MASSACHUSETTSGENERAL HOSPITAL
PATHOLOGY
HARVARDMEDICAL SCHOOL
MASSACHUSETTSGENERAL HOSPITAL
PATHOLOGY
HARVARDMEDICAL SCHOOL
Discussion
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PATHOLOGY
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MASSACHUSETTSGENERAL HOSPITAL
PATHOLOGY
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Neurofilament GFAP
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PATHOLOGY
HARVARDMEDICAL SCHOOL
CD68 CD45
CD45
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PATHOLOGY
HARVARDMEDICAL SCHOOL
Diagnosis
Delayed post-hypoxic leukoencephalopathy
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)
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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
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PATHOLOGY
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Neuropathology
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PATHOLOGY
HARVARDMEDICAL SCHOOL
• 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
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)
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PATHOLOGY
HARVARDMEDICAL SCHOOL
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.
MASSACHUSETTSGENERAL HOSPITAL
PATHOLOGY
HARVARDMEDICAL SCHOOL