●症例 7  女児(幼児)

●診断:Posterior reversible encephalopathy

●Diagnostic Keys
 ・Clinical
   During chemotherapy
   Increased blood pressure
 ・Imaging
   Bilateral occipital
   Subcortical white matter
   Symmetrical
   Mild mass effect

Posterior reversible encephalopathy (PRES)
●Anonymous

 ・Reversible posterior leukoencephalopathy
 ・Reversible posterior cerebral edema syndrome
●Etiologies (numerous!)
 ・Hypertensive encephalopathy
  ・HT, eclampsia, cyclosporine toxicity
 ・Uremic encephalopathy
  ・Uremia, GN, HUS,TTP

Mechanism of PRES (1)
Overaction of cerebral autoregulation system

Reversible vasospasm

Potentially reversible ischemia

Breakthrough of cerebral autoregulation system

Arterial dilatation

Brain hyperperfusion

Extravasation of fluid & macromolecules

Mechanism of PRES (2)
Toxins associated with uremia

Endothelial injury

Breakdown of the BBB

Concentration of intracranial ‘uremic toxins’

Intravascular microthrombi

Occlude cerebral arterioles

Hypoxia & associated edema

Infarction with or w/o hemorrhage

Imaging findings of PRES
●Location
 ・White matter > gray matter
 ・Posterior circulation > anterior circulation
   ・Poorer sympathetic innervation (?)
 ・Basal ggl, int. et ext. capsule
   ・Fed by small fragile end-arteries
●Generally symmetric
●Decreased SI in T1, increased SI in T2
●Mild mass effect
●Contrast enhancement can be seen
●Completely resolves without any sequelae

第18病日

この時はほぼ ‘reversible’ …

同一患者の後日談(付録)
左副腎原発神経芽細胞腫 stage II
化学療法第2クール。第9日に熱発と一過性血圧上昇あり。第13日、数日前より骨髄抑制強く感染徴候あり。朝再び意識レベル低下に続き間代性痙攣出現。
今回は血圧上昇なし

発症当日CT, 第2病日MRI

第38病日 MRI

必ずしも ‘reversible’ ではない!

References
1. Port JD, Beauchamp NJ, Jr. Reversible intracerebral pathologic entities mediated by vascular autoregulatory dysfunction. Radiographics 1998;18(2):353-67.
2. Casey SO, Sampaio RC, Michel E, Truwit Cl. Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. Am J Neuroradiol 2000; 21(7): 1199-206.
3. Ito Y, Arahata Y, Goto Y, Hirayama M, Nagamutsu M, Yasuda T, et al. Cisplatin neurotoxicity presenting as reversible posterior leukoencephalopathy syndrome. Am J Neuroradiol 1998;19(3):415-7.

 
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