Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literature
Background: Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical-radiological syndrome, usually reversible and with a favorable prognosis, which recognizes a variety of etiologies and clinical patterns and is likely due to an impairment in cerebral blood flow autoregulation. It is typic...
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| Format: | Article |
| Language: | English |
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Elsevier
2017-03-01
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| Series: | eNeurologicalSci |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2405650216300405 |
| _version_ | 1811262936834899968 |
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| author | Mariangela Ferrara Pietro Di Viesti Vincenzo Inchingolo Raffaela Rita Latino Teresa Popolizio Salvatore Angelo De Cosmo Flavia Pugliese Maurizio Angelo Leone |
| author_facet | Mariangela Ferrara Pietro Di Viesti Vincenzo Inchingolo Raffaela Rita Latino Teresa Popolizio Salvatore Angelo De Cosmo Flavia Pugliese Maurizio Angelo Leone |
| author_sort | Mariangela Ferrara |
| collection | DOAJ |
| description | Background: Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical-radiological syndrome, usually reversible and with a favorable prognosis, which recognizes a variety of etiologies and clinical patterns and is likely due to an impairment in cerebral blood flow autoregulation. It is typically characterized by subcortical, predominantly parieto-occipital, vasogenic brain oedema in patients with acute-subacute neurological symptoms. Infratentorial oedema on neuroimaging has been mostly described in association with the typical supratentorial pattern and seldom as isolated.
Case report: We report a case of PRES with isolated pons involvement on MRI. A woman affected by Turner syndrome, epilepsy, slight mental deficiency, obesity and hypothyroidism, experienced a progressive gait and standing impairment, worsening in the last 2 weeks. At admission blood pressure was 220/110 mmHg. Brain MRI showed a wide FLAIR signal hyperintensity on T2-weighted sequences affecting the entire pons, without contrast enhancement. Clonidine, doxazosine, furosemide and telmisartan were effective in restoring normal blood pressure. Pons hyperintensity completely resolved on MRI 3 weeks later, together with return to normal neurological examination.
Conclusions: Though isolated infratentorial involvement in PRES recognizes several causes, hypertension, which is a common feature in Turner syndrome, would have played a key role in our case with solely pons MRI T2-hyperintensity. |
| first_indexed | 2024-04-12T19:35:44Z |
| format | Article |
| id | doaj.art-de1b1fe66d7a40479bd83c01ed58ce3f |
| institution | Directory Open Access Journal |
| issn | 2405-6502 |
| language | English |
| last_indexed | 2024-04-12T19:35:44Z |
| publishDate | 2017-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | eNeurologicalSci |
| spelling | doaj.art-de1b1fe66d7a40479bd83c01ed58ce3f2022-12-22T03:19:14ZengElseviereNeurologicalSci2405-65022017-03-016C515410.1016/j.ensci.2016.11.008Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literatureMariangela Ferrara0Pietro Di Viesti1Vincenzo Inchingolo2Raffaela Rita Latino3Teresa Popolizio4Salvatore Angelo De Cosmo5Flavia Pugliese6Maurizio Angelo Leone7Neurology Unit, Department of Medicine, Research Center “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, ItalyNeurology Unit, Department of Medicine, Research Center “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, ItalyNeurology Unit, Department of Medicine, Research Center “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, ItalyNeurology Unit, Department of Medicine, Research Center “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, ItalyRadiodiagnostics Department, Research Center “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, ItalyEndocrinology Unit, Department of Medicine, Research Center “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, ItalyEndocrinology Unit, Department of Medicine, Research Center “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, ItalyNeurology Unit, Department of Medicine, Research Center “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, ItalyBackground: Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical-radiological syndrome, usually reversible and with a favorable prognosis, which recognizes a variety of etiologies and clinical patterns and is likely due to an impairment in cerebral blood flow autoregulation. It is typically characterized by subcortical, predominantly parieto-occipital, vasogenic brain oedema in patients with acute-subacute neurological symptoms. Infratentorial oedema on neuroimaging has been mostly described in association with the typical supratentorial pattern and seldom as isolated. Case report: We report a case of PRES with isolated pons involvement on MRI. A woman affected by Turner syndrome, epilepsy, slight mental deficiency, obesity and hypothyroidism, experienced a progressive gait and standing impairment, worsening in the last 2 weeks. At admission blood pressure was 220/110 mmHg. Brain MRI showed a wide FLAIR signal hyperintensity on T2-weighted sequences affecting the entire pons, without contrast enhancement. Clonidine, doxazosine, furosemide and telmisartan were effective in restoring normal blood pressure. Pons hyperintensity completely resolved on MRI 3 weeks later, together with return to normal neurological examination. Conclusions: Though isolated infratentorial involvement in PRES recognizes several causes, hypertension, which is a common feature in Turner syndrome, would have played a key role in our case with solely pons MRI T2-hyperintensity.http://www.sciencedirect.com/science/article/pii/S2405650216300405A typical Posterior Reversible Encephalopathy SyndromeInfratentorial vasogenic oedemaPontine oedemaClinico-radiological dissociation |
| spellingShingle | Mariangela Ferrara Pietro Di Viesti Vincenzo Inchingolo Raffaela Rita Latino Teresa Popolizio Salvatore Angelo De Cosmo Flavia Pugliese Maurizio Angelo Leone Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literature eNeurologicalSci A typical Posterior Reversible Encephalopathy Syndrome Infratentorial vasogenic oedema Pontine oedema Clinico-radiological dissociation |
| title | Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literature |
| title_full | Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literature |
| title_fullStr | Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literature |
| title_full_unstemmed | Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literature |
| title_short | Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literature |
| title_sort | isolated pons involvement in posterior reversible encephalopathy syndrome case report and review of the literature |
| topic | A typical Posterior Reversible Encephalopathy Syndrome Infratentorial vasogenic oedema Pontine oedema Clinico-radiological dissociation |
| url | http://www.sciencedirect.com/science/article/pii/S2405650216300405 |
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