TY - JOUR
T1 - Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literature
AU - Ferrara, M.
AU - Viesti, Pietro Di
AU - Inchingolo, Vincenzo
AU - Latino, R.
AU - Popolizio, T.
AU - De Cosmo, S.A.
AU - Pugliese, F.
AU - Leone, M.A.
N1 - Export Date: 22 March 2017
Correspondence Address: Ferrara, M.; Neurology Unit, Department of Medicine, Research Center “Casa Sollievo della Sofferenza”, viale Cappuccini, 1, Italy; email: mariangela.ferrara@operapadrepio.it
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PY - 2017
Y1 - 2017
N2 - 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. © 2016 The Author(s)
AB - 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. © 2016 The Author(s)
KW - A typical Posterior Reversible Encephalopathy Syndrome
KW - Clinico-radiological dissociation
KW - Infratentorial vasogenic oedema
KW - Pontine oedema
U2 - 10.1016/j.ensci.2016.11.008
DO - 10.1016/j.ensci.2016.11.008
M3 - Article
VL - 6
SP - 51
EP - 54
JO - eNeurologicalSci
JF - eNeurologicalSci
SN - 2405-6502
ER -