Posterior reversible encephalopathy syndrome
|Posterior reversible encephalopathy syndrome|
|Synonyms||Reversible posterior leukoencephalopathy syndrome (RPLS)|
|Posterior reversible encephalopathy syndrome visible on magnetic resonance imaging as multiple cortico-subcortical areas of T2-weighted hyperintense (white) signal involving the occipital and parietal lobes bilaterally and pons.|
Posterior reversible encephalopathy syndrome (PRES) is a syndrome characterized by headache, confusion, seizures and visual loss. It may occur due to a number of causes, predominantly malignant hypertension, eclampsia and some medical treatments. On magnetic resonance imaging (MRI) of the brain, areas of edema (swelling) are seen. The symptoms tend to resolve after a period of time, although visual changes sometimes remain. It was first described in 1996.
Signs and symptoms
Typical symptoms of PRES, listed according to prevalence, include: altered mental status (encephalopathy), seizure, and headache. Less commonly there may be visual disturbances, focal neurologic signs, and status epilepticus.
Several factors appear to play a role in the pathogenesis of PRES, including medications that suppress the function of the immune system (immunosuppressive medications), kidney failure, eclampsia, severe high blood pressure, systemic lupus erythematosus, and postpartum hemorrhage.
The diagnosis is typically made clinically with magnetic resonance imaging of the brain often revealing hyperintensities on T2-weighed imaging. Three patterns have been described: superior frontal sulcus, dominant parieto-occipital, and holohemispheric watershed.
The treatment of PRES dependent on its cause. Anti-epileptic medication may also be appropriate.
Many cases resolve within 1–2 weeks of controlling blood pressure and eliminating the inciting factor. However some cases may persist with permanent neurologic impairment in the form of visual changes and seizures among others. Though uncommon, death may occur with progressive swelling of the brain (cerebral edema), compression of the brainstem, increased intracranial pressure, or a bleed in the brain (intracerebral hemorrhage). PRES may recur in about 5-10% of cases; this occurs more commonly in cases precipitated by hypertension as opposed to other factors (medications, etc.).
The number cases of PRES that occur each year is not known. It may be somewhat more common in females.
- Garg RK (January 2001). "Posterior leukoencephalopathy syndrome". Postgrad Med J. 77 (903): 24–8. doi:10.1136/pmj.77.903.24. PMC 1741870. PMID 11123390.
- Pula JH, Eggenberger E (November 2008). "Posterior reversible encephalopathy syndrome". Curr Opin Ophthalmol. 19 (6): 479–84. doi:10.1097/ICU.0b013e3283129746. PMID 18854692.
- Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, Pessin M, Lamy C, Mas J, Caplan L (1996). "A reversible posterior leukoencephalopathy syndrome". N Engl J Med. 334 (8): 494–500. doi:10.1056/NEJM199602223340803. PMID 8559202.
- Rajasekhar, A.; George, T. J. (November 2007). "Gemcitabine-Induced Reversible Posterior Leukoencephalopathy Syndrome: A Case Report and Review of the Literature". The Oncologist. 12 (11): 1332–1335. doi:10.1634/theoncologist.12-11-1332. PMID 18055853.
- Fugate, Jennifer E.; Rabinstein, Alejandro A. (2015-09-01). "Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions". The Lancet Neurology. 14 (9): 914–925. doi:10.1016/S1474-4422(15)00111-8. ISSN 1474-4465. PMID 26184985.
- Kur, JK; Esdaile, JM (November 2006). "Posterior reversible encephalopathy syndrome--an underrecognized manifestation of systemic lupus erythematosus". The Journal of Rheumatology. 33 (11): 2178–83. PMID 16960925.
- Lio, Chon-Fu; Lee, Ying-Hua; Chan, Hung-Yen; Yu, Chang-Ching; Peng, Nan-Jing; Chan, Hung-Pin (2017-04-01). "Posterior reversible encephalopathy syndrome in a postpartum hemorrhagic woman without hypertension: A case report". Medicine. 96 (16): e6690. doi:10.1097/MD.0000000000006690. ISSN 1536-5964. PMC 5406100. PMID 28422884.
- Peter P, George A. Posterior reversible encephalopathy syndrome and the pediatric population. J Pediatr Neurosci 2012;7:136-8.