1. San Giovanni Rotondo (FG), Italy
A case of Posterior Reversible Encephalopathy Syndrome with isolated pons involvement in a patient with Turner Syndrome
Ferrara Mariangela¹, Di Viesti Pietro¹, Inchingolo Vincenzo,¹ Latino Raffaella¹, Popolizio Teresa², Leone Maurizio¹
Neurology Unit¹, Radiology and Medical Imaging Department², IRCCS “Casa Sollievo della Sofferenza”.
San Giovanni Rotondo (FG), Italy
Conclusions:
Rapid increase in blood pressure exceeding cerebral blood flow autoregulation, and possibly, an individual pattern of sympathetic innervation of blood vessel in vertebrobasilar circulation, may play a key role in brainstem type PRES.
References:
1 Fugate Jennifer E. et al: ”Posterior reversibile encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions” Lancet Neurol 2015; 14:914-25
2 Gao Bo, Liang Hui, Liu Feng-li, Lv Cui : “Isolated pons variant of posterior reversibile encephalopathy syndrome complicated with ischemic stroke in a young patient: association or coincidence?” Neurological Sciences 2014; 35:1646
3 Liang Hui, Li Deqiang, Xu Ziqi, Luo Benyan :” Isolated pons variant of posterior reversibile encephalopathy syndrome complicated with ischemic stroke in a young patient” Neurological Sciences 2012; 34:1082
Introduction:
Posterior Reversible Encephalopathy Syndrome (PRES) is characterized by a reversible subcortical vasogenic brain oedema, predominantly involving the bilateral parieto-occipital regions, in patients with acute-subacute neurological symptoms. It is caused by a variety of factors, the commonest being hypertension. PRES is generally reversible, both radiologically and clinically, and has a favorable prognosis (1).
Methods:
We report a case of a brainstem variant of PRES with isolated pons involvement, rarely described in Literature (2, 3).
Results:
A 28 years–old woman, affected by Turner syndrome, epilepsy, slight mental deficiency, obesity and hypothyroidism, presented with a progressive gait and standing impairment, worsening in the last 2 weeks. At admission blood pressure was 220/110 mmHg.
Neurological examination showed brisk reflexes, Babinski sign, inability at standing and marching. She underwent full blood examination (homocisteine, folate, B12 vitamin, vanilmandelic acid and chromogranine A levels, antiviral, bacterial, trombophilic and autoimmune screening), lumbar puncture, abdominal, renal arteries and cardiac echographies, thoracic aorta MRI, all normal. A brain MRI showed a wide FLAIR hyperintense area affecting the entire pons, without contrast enhancement (FIG.1).
Clonidine, doxazosine and furosemide were effective in restoring normal blood pressure within 10 days and pons hyperintensity was completely resolved on MRI 3 weeks later (FIG.2), together with return to normal neurological examination.
FIG.1
FIG.2