Is decompressive craniectomy necessary in malignant posterior reversible encephalopathy syndrome with brain edema caused uncal herniation? A case report of reversible coma without surgical decompression.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is considered a benign entity and is usually reversible with only medical management, but persistent neurologic deficits and disability or death can occur without adequate treatment. Favorable outcomes have been associated with surgical decompression in malignant-type PRES in which hemorrhagic transformation or brain stem compression has developed. CASE DESCRIPTION: Here we report a case of malignant PRES in a 61-year-old female of Asian descent in which the disease rapidly progressed to coma and a near-fatal condition with uncal herniation caused by severe brain edema; however, this patient achieved a dramatic recovery without surgical decompression. CONCLUSION: After reviewing previous reports regarding malignant PRES, we propose that hemorrhagic transformation is a crucial indicator for surgical decompression and an important prognostic factor in malignant PRES.

Post-partum posterior reversible encephalopathy syndrome requiring decompressive craniectomy: case report and review of the literature.

Posterior reversible encephalopathy syndrome (PRES) is an uncommon but potentially devastating syndrome if not recognized and treated appropriately. As the name implies, recognition of the condition and proper management may reverse the clinical and radiological findings. However, diagnosis is not always straightforward. We present the case of a 24-year-old female who was 4 days post-partum and presented with headache, neck pain, and new-onset seizures. She had undergone epidural anesthesia during labor, and initial imaging was suggestive of intracranial hypotension versus pachymeningitis. Despite initial conservative therapy including anti-epileptic drugs, magnesium therapy, empiric antibiotics, and Trendelenburg positioning, the patient continued to deteriorate. Follow-up imaging was suggestive of PRES with signs of intracranial hypertension. The patient underwent a decompressive suboccipital craniectomy for refractory and severe PRES and later fully recovered. This case highlights the sometimes difficult diagnosis of PRES, possible association with pregnancy, eclampsia/preeclampsia and/or cerebrospinal fluid drainage, and the rare but life-saving need for decompression in severe cases.

Posterior reversible encephalopathy syndrome presenting in the anterior circulation with malignant intracranial hypertension requiring surgical decompression: a case report and literature review.

Posterior reversible encephalopathy syndrome (PRES) is thought to result from endothelial dysfunction and breakdown of the blood-brain barrier with subsequent vasogenic edema. Abrupt hypertension has been identified as one of its risk factors. We present a rare case of PRES in the anterior circulation with sudden onset of left hemiparesis and rapid neurological deterioration on the basis of hypertensive crisis. Due to refractory intracranial hypertension, the patient required emergent right decompressive craniectomy. Further investigations, including a biopsy, revealed an atypical form of PRES. This case illustrates the importance of aggressive medical and early surgical management to prevent permanent neurological deficits.

Life-threatening posterior reversible encephalopathy syndrome in the cerebellum treated by posterior fossa decompression.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological syndrome associated with various clinical conditions, such as headache, encephalopathy, and seizures. It is reversible if a prompt diagnosis is made and treatment undertaken. We report a 52-year-old male with hypertensive crisis. Progressing somnolence and an unresponsive left pupil occurred. MRI revealed an intra-axial hyperintensity of the cerebellum and brainstem and occlusive hydrocephalus suggestive of encephalitis or a tumor. Because of the life-threatening clinical picture, posterior fossa decompression was performed. Histopathology failed to identify any pathology. After decompression, the edema improved immediately. Under life-threatening conditions, a decompressive craniectomy in PRES seems to achieve the same results as supportive treatment.

Severe posterior reversible encephalopathy syndrome resolved with craniectomy.

Posterior reversible encephalopathy syndrome (PRES) has been thought to be a benign disease, but recently severe cases have been reported with increasing recognition. A 3-year-old girl with congenital nephrotic syndrome had rapidly progressed to coma. Computed tomography (CT) of the head showed striking swelling of the brainstem and transtentorial herniation. Emergency decompressive craniectomy was performed. Consecutively, blood pressure was optimally controlled. The patient gradually recovered to the previous state before onset of PRES. Rapid improvement of clinical symptoms and rapid resolution of abnormal findings on serial CT led to diagnosis of PRES. In severe PRES with unstable vital signs, surgical intervention should be considered as well as appropriate blood pressure management.

Posterior reversible encephalopathy syndrome: a case following reversible cerebral vasoconstriction syndrome masquerading as subarachnoid haemorrhage.

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic clinico-radiological diagnosis typically presenting with headache, encephalopathy and visual disturbance accompanied by a unique neuroradiological pattern of symmetrical parieto-occipital vasogenic oedema. Here we present the case of a 51-year-old woman who presented to hospital following a thunderclap headache, initially thought to be secondary to a subarachnoid haemorrhage (SAH). A tiny anterior choroidal artery aneurysm was demonstrated on cerebral angiogram. At surgical clipping, no evidence of haemorrhage was observed. Post-operatively, the patient developed delayed right-sided hemiparesis, managed with aggressive hypertensive treatment, and later, with onset of septicaemia, central visual loss. Computed tomography (CT) brain scans demonstrated oedematous changes within the parieto-occipital regions bilaterally and later areas of infarction. The initial diagnosis of SAH was revised to reversible cerebral vasoconstriction syndrome (RCVS), which gave rise to PRES. To our knowledge, this is the first reported case of RCVS with concomitant PRES and cerebral infarction.

Malignant PRES and RCVS after brain surgery in the early postpartum period.

The management of women with brain tumors in the early post-partum period may be demanding as the patho-physiological changes that occur during pregnancy may also manifest in the early post-partum period. The aim of our paper is to report a case of late-onset post-partum pre-eclampsia after brain tumor surgery, complicated by posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS). Hemicraniectomy and intensive care management were necessary to obtain a favorable neurological outcome. The inherent literature on the subject is also analyzed through a systematic research. This is the first case of supratentorial decompressive hemicraniectomy in post-partum PRES, while there has been only one other case of posterior fossa decompression described in this cohort of patients. PRES and RCVS can complicate the neurosurgical management of women in the postpartum period. A careful evaluation of the clinical presentation is necessary as in some particular cases an aggressive medical and surgical treatment is required to obtain a favorable outcome.

Recurrent posterior reversible encephalopathy syndrome with cerebellar involvement leading to acute hydrocephalus.

Posterior reversible encephalopathy syndrome or PRES is a proposed cliniconeuroradiological entity that is characterized by headache, confusion, seizure, cortical visual disturbances or even blindness and, to a lesser extent, focal neurological signs. The etiology of this entity includes a sudden increase in blood pressure, renal failure, immunosuppressive drugs, infections, and intravenous immunoglobulin (IVIG). Classically, magnetic resonance imaging (MRI) findings show a symmetric reversible vasogenic edema in the parietooccipital lobes. PRES can involve the brainstem and cerebellum and sometimes can leave irreversible lesions but it can also recur, which is a very rare presentation. In this article, we report a case of recurrent PRES with cerebellar involvement associated with non-communicating hydrocephalus in a 2-year-old child with renal failure on peritoneal dialysis after receiving Etoposide for macrophage activation syndrome.

Neuropathological findings from an autopsied case showing posterior reversible encephalopathy syndrome-like neuroradiological findings associated with premedication including tacrolimus for autologous peripheral blood stem cell transplantation.

Posterior reversible encephalopathy syndrome (PRES) is diagnosed based on neuroradiological findings. Typically, PRES is reversible and presents with a good outcome; however, fatal outcomes have been reported. We report an autopsied case showing PRES-like neuroradiological findings associated with premedication including tacrolimus for autologous peripheral blood stem cell transplantation in a 28-year-old woman with a 2-year history of acute myeloid sarcoma/acute myeloid leukemia. Neurological examination revealed disturbed consciousness, muscle weakness in all extremities, and bilaterally diminished tendon reflexes. Brain fluid attenuated inversion recovery MRI showed multiple bilateral hyper-intensity areas in the posterior white matter and left corona radiate. She died of respiratory arrest within 24h after PRES diagnosis. Neuropathological examination revealed diffuse cerebral edema, multiple cerebral hematomas that extended into the lateral ventricles and subarachnoid cavities, and multiple microbleeds predominantly in the inferior surface of the occipital white matter. Microscopic findings revealed paler myelin sheaths, enlargement of the vascular endothelium, leakage of plasma components and red blood cells, and clasmatodendrosis within the occipital white matter. Cerebral herniation and diffuse cerebral edema due to vascular endothelial dysfunction were concluded to be the cause of death. These pathological findings may aid in the pathophysiological recognition of acute-stage PRES.

Pazopanib-associated posterior reversible encephalopathy syndrome with intracerebral haemorrhage.

Pazopanib is a tyrosine kinase receptor antagonist used for renal cell carcinoma and soft tissue sarcoma that inhibits tumour growth and angiogenesis. A common side effect of pazopanib is hypertension. We report a case of a 69-year-old woman with clear cell renal cell carcinoma who developed a large right occipital intracerebral haemorrhage 3 weeks after initiating pazopanib. Although this was initially suspected to be a haemorrhagic metastasis, MRI revealed bi-occipital oedema, supporting a diagnosis of posterior reversible encephalopathy syndrome (PRES). A craniectomy was required. Immunohistochemical stains for renal cell carcinoma antigen, CA IX and PAX8 were negative. This case suggests that PRES and intracerebral haemorrhage may result from pazopanib use and are important complications to consider prior to initiating this agent.

Posterior reversible encephalopathy syndrome following a thoracic discectomy-induced dural leak: case report.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological syndrome characterized by headaches, altered mental status, seizures, and visual disturbances. Classic MRI findings include white matter changes of the parieto-occipital regions. This syndrome has been encountered in myriad medical illnesses, including hypertension, preeclampsia/eclampsia, and immunosuppressive conditions. While the pathogenesis of the disorder is unclear, vasoconstriction and hypoperfusion leading to brain ischemia and vasogenic edema have been implicated as potential mechanisms. The authors present, to the best of their knowledge, the first case of PRES following a thoracic spinal surgery-induced dural leak noted on resection of the fifth rib during a thoracotomy for a T4-5 discectomy. Brain MRI revealed large areas of increased FLAIR and T2 hyperintensity in the superior posterior frontal lobes, superior and medial parietal lobes, and bilateral occipital lobes. Following repair of the CSF leak, the patient's symptoms resolved. Spinal surgeons should be alert to the potentially life-threatening condition of PRES, especially in a hypertensive patient who experiences surgery-induced dural leakage. The development of a severe positional headache with neurological signs is a red flag that suggests the presence of PRES. Prompt attention to the diagnosis and treatment of this condition by repairing the dural leak via surgery or expeditious blood patch increases the likelihood of a favorable outcome.

Hypertensive posterior reversible encephalopathy syndrome causing posterior fossa edema and hydrocephalus.

Posterior reversible encephalopathy syndrome (PRES) is a well characterized entity resulting from the inability of cerebral autoregulation to adequately protect the brain from uncontrolled hypertension. It primarily affects the occipital lobes, but can also involve the structures in the posterior fossa including the brainstem and cerebellum. Treatment usually consists of strict blood pressure control, but more aggressive management may be indicated with acutely worsening neurological status. We present a patient with hypertensive encephalopathy that resulted in hydrocephalus and brainstem compression necessitating surgical decompression requiring ventriculostomy and suboccipital craniectomy. In rare cases, PRES can present with severe brainstem compression requiring emergent posterior fossa decompression. When brainstem signs are present on exam, emergent posterior fossa decompression may be safer than ventriculostomy alone.