Rapid intracranial pressure drop as a cause for posterior reversible encephalopathy syndrome: Two case reports
- Department of Neurosurgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan
Department of Neurosurgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan
DOI:10.4103/sni.sni_55_17Copyright: © 2017 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Ryoko Niwa, Soichi Oya, Takumi Nakamura, Taijun Hana, Toru Matsui. Rapid intracranial pressure drop as a cause for posterior reversible encephalopathy syndrome: Two case reports. 05-Jun-2017;8:103
How to cite this URL: Ryoko Niwa, Soichi Oya, Takumi Nakamura, Taijun Hana, Toru Matsui. Rapid intracranial pressure drop as a cause for posterior reversible encephalopathy syndrome: Two case reports. 05-Jun-2017;8:103. Available from: http://surgicalneurologyint.com/surgicalint-articles/rapid-intracranial-pressure-drop-as-a-cause-for-posterior-reversible-encephalopathy-syndrome-two-case-reports/
Background:Posterior reversible encephalopathy syndrome (PRES) is characterized by reversible edematous lesions on radiological examinations as well as symptoms of altered consciousness and seizures. To date, the underlying mechanism remains largely unknown.
Case Descriptions:Case 1 is a 72-year-old man with a history of hypertension presented with a subarachnoid hemorrhage. Fourteen days after the successful clipping of a ruptured aneurysm; he experienced inadvertent overdrainage via the intraventricular drain. Nine hours later, he started to have seizures followed by disturbances in consciousness. An emergency magnetic resonance imaging showed multiple high-intensity lesions in the frontal, temporal, parietal, and occipital lobes, basal ganglia, brainstem, and cerebellar hemispheres bilaterally, which are compatible with typical magnetic resonance findings in PRES patients. He was treated conservatively and recovered well. Case 2 is a 68-year-old woman with a mild history of hypertension and a ventriculo-peritoneal shunt for obstructive hydrocephalus, who underwent a cysto-peritoneal shunt placement because of an enlarging symptomatic arachnoid cyst. Immediately following surgery, she experienced disturbances in consciousness and developed status epilepticus. Radiological examinations revealed remarkable shrinkage of the arachnoid cyst and multiple edematous lesions, which led us to strongly suspect PRES. With conservative treatment, her symptoms and the radiological abnormalities disappeared.
Conclusion:Based on the previous literature and our cases, we believe that the association between rapid reduction of intracranial pressure (ICP) and the development of PRES should be recognized because most neurosurgical procedures such as craniotomy or cerebrospinal fluid diversion present a potential risk of rapid reduction of ICP.
Keywords: Cerebrospinal fluid, intracranial pressure, lumbar puncture, posterior reversible encephalopathy syndrome
Posterior reversible encephalopathy syndrome (PRES) was proposed by Hinchey et al. in 1996 to describe characteristic radiological changes indicating brain edema, especially distributed in the posterior circulatory area, which is the most apparent on fluid-attenuated inversion recovery (FLAIR) images.[
As the condition gained recognition, additional characteristics have been reported.[
Here, we present two patients who developed PRES immediately after a rapid reduction in intracranial pressure (ICP). We also reviewed the previous literature and propose that a reduction in ICP may be associated with PRES.
A 72-year-old man on regular medication for hypertension was transported to our hospital complaining of sudden headache followed by disturbed consciousness. Upon arrival, he showed an altered mental status with decreased alertness. He could follow commands but could not speak. He had no other focal deficits, such as hemiparesis or cranial nerve palsy. Computed tomography (CT) showed a subarachnoid hemorrhage (SAH) [
(a) Computed tomography (CT) scan upon admission showing a hematoma in the basal cistern. (b) Anteroposterior view of the left internal carotid angiogram revealing an aneurysm at the bifurcation of the internal carotid artery and the posterior communicating artery. (c) CT on day 14 showing ventricular narrowing and low-density area on the left occipital lobe (arrowhead). (d-f) Magnetic resonance (MR) imaging showed fluid-attenuated inversion recovery (FLAIR) images depicting diffuse high-intensity lesions in the bilateral frontal, temporal, parietal, and occipital lobes, and basal ganglia, brainstem, and cerebellum. (g) MR angiography showing no significant vasospasm. (h) MR imaging obtained on day 30 showing a complete resolution of high-intensity lesions.
A 68-year-old woman presented at our outpatient clinic complaining of left hemiparesis. She had a history of well-controlled hypertension and had a VP shunt placed 18 years ago for obstructive hydrocephalus due to midbrain cavernous malformation. CT images showed an enlargement of the right frontotemporal arachnoid cyst [
(a) CT scan demonstrating a large arachnoid cyst on the right frontotemporal region. (b) Immediate postoperative CT revealing a remarkable shrinkage of the cyst. (c) CT obtained 1 day after surgery showing low-density areas in the bilateral occipital lobes. (d) MR FLAIR image on the fourth day after surgery demonstrating diffuse high-intensity areas in the temporal, parietal, and occipital lobes on both sides, and in the right frontal lobe. (e) MR FLAIR image 27 days after surgery showing an almost complete disappearance of lesions
Despite its name containing the term “posterior,” PRES is believed to occur in the posterior circulatory areas as well as in the temporal lobes or frontal lobes,[
Although ICP was not directly measured, a rapid reduction in ICP appeared to occur in both of our cases. In Case 1, radiological clinical signs of PRES presented approximately 6 h after the inadvertent massive CSF drainage, indicating that the rapid decrease in ICP could have been the main trigger of PRES. Similarly, the postoperative CT in Case 2 showed substantial shrinkage of the cyst, suggesting a possible abrupt event that decreased ICP during surgery. Ho et al. proposed that reduction in ICP decreases ventricular size, resulting in mechanical stress to vessels and causing vasoconstriction and vasogenic edema.[
Hammad et al. recently reported a case of PRES secondary to CSF leak and intracranial hypotension, and also reviewed 10 cases of PRES that developed after spinal or epidural tap.[
In Case 2, the cysto-peritoneal shunt for the large arachnoid cyst might have caused an acute drop in ICP that resulted in PRES, a novel situation that has not been reported in the literature, to the best of our knowledge. Theoretically, several neurosurgical procedures are at high risk for unintentional hyperperfusions. Although rare, surgeons should be aware of the possibility of PRES in patients with severe headache, seizure, and disturbances in consciousness that cannot be explained by other medical conditions.
The prognosis of PRES is generally good and almost all patients make full recoveries or return to a normal life with minor deficits.[
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