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Answer to Case of the Week: Apr 8-Apr 15, 2010

9 year old male with AML with renal failure and hypertension


 




CT demonstrates low attenuation consistent with edema in the posterior temporal lobes, parietal lobes and occipital lobes bilaterally.

Diagnosis: Acute Hypertensive Encephalopathy-PRES

Posterior reversible leukoencephalopathy syndrome (PRES) is a condition in which patients present with headache, altered mental status, confusion, drowsiness, visual disturbances and/or seizures.  

They also have hypertension, metabolic derangements and commonly renal failure.

The etiology is most frequently renal failure with hypertension, but also occurs in eclampsia lower<1%

Pre-eclampsia -5%Immunosupression:Cyclosporine or FK-506

Tacrolimus

Cisplatin

Interferon-alpha

Erythropoietin   

    There are case reports in patients with lupus, polyarteritis nodosa, acute porphyria, and post-organ transplantation, thrombotic thrombocytopenic purpura.  

     Acute hypertension or drug toxicity damages vascular endothelium and breakthrough of autoregulation causes blood brain barrier disruption. 

   Vasogenic edema with arteriolar dilatation with cerebral hyperperfusion. Hydrostatic leakage( extravasation, transduation of fluid and macromacules through arteriolar walls)      

    Interstitial fluid accumulates in cortex,subcortical white matter.   

   Posterior circulation sparsely innervated by sympathetic nerves ( predilection for parietal,occipital lobes) Progression to frank infarction with cytotoxic edema rare in PRES.        

   Patient show edema in the occipital and parietal lobe white and grey matter primarily, but can also have similar findings in the brain stem, basal ganglia and frontal lobes. Rarely enhancement or hemorrhage occurs.

  Differntial Diagnosis:  

Acute Cerebral Ischemia

Acute Cerebral Hyperemia

Inborn Error of Metabolism

Progressive Multifocal Leukoencephalopathy( PML)

Acute Demyelinating Disease 

     Findings are completely reversible, but this may take weeks to months to occur. 

Take Home Message; Favorable outcome with prompt recognition, treatment of hypertension.


Residents Submitting Correct Diagnosis - Case of the Week
Radiology
Pediatrics
VCU Resident
  • Aaron Nordgren
  • Charlotte Cockrell
  • John Fahrner
  • Shep Morano
    Others
  • Brian TrottaUnited States of America
  • Flavia GaspariniBrazil
  • Pushpinder Singh kheraIndia
  • Nicole KelleherUnited States of America
  • Nishard AbdeenCanada
  • Wael NemattallaEgypt
  • Gitanjali BajajIndia
  • Matthew ChaneyUnited States of America
  • BOB WONGCanada
  • Monika BagadeIndia
  • Chad St. GermainUnited States of America
  • Umapathi MaheshIndia
  • Khalid KhashoggiSaudi Arabia
  • Naganathan ManiUnited States of America
  • Maddalena DuarteUnited States of America
  • Robert PalmerUnited States of America
  • John KirkhamUnited States of America
  • Simon DupreAustralia
  • Jason AllenUnited States of America
  • Shohreh RezaiAustralia
  • Hani AlSalamSaudi Arabia
  • Oussama IsmaeelSyrian Arab Republic
  • PRAGATI KUMARUnited States of America
  • Seth AndersonUnited States of America
  • Rajesh SIndia
  • Anil HondappanavarIndia
  • Yogeshwari DeshmukhIndia
  • Sarah AbdullahSaudi Arabia
  • Fernando TorresUnited States of America

    Disclaimer: This information is intended solely for resident review of presented cases which may or may not be pathologically proven. Information is derived from a number of published sources of varying reliability and does not represent original research from the institution. It is not intended to be comprehensive and should therefore not substitute for careful review of the literature.