Posterior reversible encephalopathy syndrome

Classification according to ICD-10
G93.4	Encephalopathy, unspecified
ICD-10 online (WHO version 2019)

MRI image with hyperintensities especially in the occipital lobe

The Posterior Reversible Encephalopathy Syndrome (PRES) is a rare neurological disorder associated with an edema , especially in the occipital lobe accompanied the brain. This leads to headaches , visual disturbances , epileptic seizures and confusion . The causes have not yet been adequately researched.

course

Over the course of several hours to about 2 days, those affected develop a general disorder of the brain ( encephalopathy ), which can range from mild confusion to severe impaired consciousness. Up to 75% suffer at least one epileptic seizure , which often leads to hospital presentation as a serious symptom. Furthermore, visual disturbances usually occur, which are not always recognized immediately due to the gradual onset. Only about half of those affected suffer from headaches.

Diagnosis

The computer tomography , which is usually carried out at the beginning, remains without any indicative findings in at least half of the patients. In the magnetic resonance imaging of the head, the most pronounced lets edema (especially in the FLAIR or T2-weighted images) represent good. Cerebral edema occurs less often in the lateral or front parts of the brain or in the brain stem. Evidence of complications (diffusion disorders, bleeding) suggests a less favorable course of the disease. In the cerebrospinal fluid an increase of protein is often detected, but no relevant cell count increase. The EEG shows a general change; signs typical of epilepsy may be detectable according to the clinical course. If vascular imaging is carried out with contrast medium, localized enlargements and constrictions of the arteries supplying the brain, especially the posterior cerebral artery , are usually found .

root cause

Various diseases significantly increase the risk of developing a PRES, in particular severe kidney dysfunction , eclampsia , the use of immunosuppressants (especially ciclosporin ) and severe intensive care diseases (e.g. sepsis ). It is assumed that in connection with a significant increase in blood pressure there is a malfunction both in the sealing of the vessel walls ( endothelial function ) and a disruption of the autoregulation of the arteries in the head. As a result, blood fluid escapes from the vessels, an edema. This causes the malfunctions mentioned above.

therapy and progress

The treatment must be carried out in an intensive care unit with invasive measurement of blood pressure and corresponding drug-based lowering of blood pressure with the aim of rapid normalization. Triggering causes should also be treated consistently, as well as epileptic seizures. The administration of magnesium is only considered effective in the case of a magnesium deficiency, glucocorticoids as generally ineffective. No other specific treatments are known.

In about 90% of all cases there is a clinical complete remission of the symptoms within 3–10 days . The cerebral edema has regressed on the MRI. Complications occur in up to 10% of the courses, especially bleeding in the head and occasionally vascular occlusions . This impairs the recovery of those affected and, in individual cases, neurological disorders can remain in the long term.

Individual evidence

1. ↑ ^{a b c d e} Reinhard M, Hetzel A, Meckel S et al .: Posterior reversible encephalopathy syndrome (PRES) or reversible posterior leukoencephalopathy syndrome (RPLS). In: Hufschmidt A, Lücking C, Rauer S et al., Ed. Neurologie compact. 7th edition. Stuttgart: Thieme; 2017. doi: 10.1055 / b-005-143671
2. ^ Eberhardt O .: Hypertensive crisis and posterior reversible encephalopathy syndrome (PRES). In: Advances in Neurology · Psychiatry 2018; 86 (05): 290 - 300. doi: 10.1055 / s-0043-122600

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