What is Press syndrome, also known as Progressive Encephalopathy with Rigidity and Myoclonus (PERM)?

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From the Research

Press syndrome, also known as Progressive Encephalopathy with Rigidity and Myoclonus (PERM), is not the condition described in the provided evidence, but rather Posterior Reversible Encephalopathy Syndrome (PRES) is the condition characterized by headache, confusion, seizures, and visual disturbances due to brain swelling in the posterior regions. The primary treatment involves addressing the underlying cause, which is often severe hypertension, as noted in the study by 1. Blood pressure should be reduced gradually, typically using intravenous medications like labetalol, nicardipine, or clevidipine in a monitored setting. If seizures occur, they should be managed with antiepileptic drugs such as levetiracetam (500-1000 mg twice daily) or phenytoin (loading dose of 15-20 mg/kg), as suggested by 2. For PRES caused by immunosuppressive medications, these agents should be reduced or discontinued. Some key points to consider in the management of PRES include:

  • Early recognition and prompt treatment are crucial to prevent permanent neurological damage, as emphasized by 3.
  • Frequent neurological evaluations and neuroimaging examinations by computed tomography or magnetic resonance imaging are required for both the diagnosis and assessment of the condition, as noted by 3.
  • PRES develops when sudden changes in blood pressure exceed the brain's autoregulatory capacity, leading to vasogenic edema, particularly in posterior brain regions where autoregulation is less robust, as explained by 4.
  • Most patients recover completely within days to weeks with proper management, though neurological monitoring is essential during this period, as reported by 5. The most critical aspect of managing PRES is early detection and treatment of the underlying cause, which can significantly improve outcomes and reduce the risk of long-term neurological damage, as highlighted by 1.

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This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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