The Clinical and Radiological Spectrum of Posterior Reversible Encephalopathy Syndrome: A Retrospective Series of 24 Patients

Department of Neurology, Peking Union Medical College Hospital, Beijing, China.
Journal of neuroimaging: official journal of the American Society of Neuroimaging (Impact Factor: 1.73). 07/2011; 21(3):219-24. DOI: 10.1111/j.1552-6569.2010.00497.x
Source: PubMed


Some patients with posterior reversible encephalopathy syndrome (PRES) present with atypical clinical and neuroimaging findings. The purpose of this study is to review the clinical and neuroimaging findings in patients diagnosed with PRES.
We retrospectively included all patients diagnosed with PRES between January 2005 and March 2009. We reviewed demographic, clinical and laboratory data, neuroimaging findings, and prognosis.
Twenty-four patients were included with a median age of 19 years. The clinical presentations included seizures (91.7%), headache (83.3%), visual disturbance (62.5%), encephalophathy (29.2%), and paralysis (8.3%). Co-morbidities included systemic lupus erythematous (29.2%), kidney disease (20.8%), eclampsia (20.8%), renal artery stenosis (12.5%), Takayasu arteritis (4.2%), Sheehan's syndrome (4.2%), allergic purpura (4.2%), and acute intermittent porphyria (4.2%). Acute elevation of blood pressure was found in 22 patients (91.7%). Ten patients (41.7%) used steroids or immunosuppressants, three (12.5%) had acute renal failure before the symptoms. Atypical neuroimaging features included involvement of the frontal lobe (54.2%), basal ganglia (4.2%) or cortex (8.3%), and irreversible lesions (3/18, 16.7%).
A large proportion of patients with PRES may present with atypical neuroimaging findings. Apart from the known risk factors, such as hypertension, immunosuppressants, and renal failure of various etiologies may be the precipitants of PRES.

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    • "A retrospective study of 36 patients presenting at the Mayo Clinic with PRES demonstrated hypertension with mean systolic blood pressure of 187 mmHg at presentation.[22] Several medications including lenalidomide, dexamethasone, and cyclosporine have been associated with PRES.[192640] In particular, PRES was reported in a 60-year-old woman following treatment with high-dose steroids after an autologous peripheral blood stem cell transplantation for multiple myeloma.[40] "
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    ABSTRACT: The authors describe a unique case of a patient who developed posterior reversible encephalopathy syndrome (PRES) following postoperative treatment of a Chiari I malformation. A 25-year-old female presented with complaints of left upper and lower extremity paresthesias and gait disturbances. A magnetic resonance imaging (MRI) of the brain and cervical spine showed a Chiari I malformation with tonsillar descent beyond the level of the C1 lamina. She underwent a suboccipital craniectomy and C1 laminectomy with cerebellar tonsillar cauterization and duraplasty. Postoperatively, an MRI showed bilateral acute infarcts of the cerebellar vermis. She was initially treated for cerebellar ischemia with hypertensive therapy with a subsequent decline in her neurologic status and generalized tonic-clonic seizure. Further workup showed evidence of PRES. After weaning pressors, the patient had a significant progressive improvement in her mental status. Although the mechanism of PRES remains controversial given its diverse clinical presentation, several theories implicate hypertension and steroid use as causative agents.
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