●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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