A 39-year-old, heavy smoker, left-handed woman, affected by obesity, dyslipidemia and hypertension presented in an emergency hospital, due to the subacute onset of left brachio-crural hemiparesis, and motor aphasia lasting for two days. The patient underwent brain CT scan that showed some periventricular nuanced hypodensities in the right frontoparietal white matter, reported as probably ischemic (Fig. 1.1).
Despite the antiplatelet therapy, hemiplegia and cognitive/behavioral dysfunctions emerged in a few days and the patient was transferred in our Neurological Department. She underwent brain MRI that showed multiple and not ischemic grossly rounded lesions, bilaterally located in the deep frontoparietal white matter, some of which surrounded by vasogenic edema. They appeared hypo-isointense on T1-weighted and hyperintense with peripheral hypo-intensity on T2-weighted images (Fig. 1.2). Some regions of signal restriction associated to intense pseudo nodular enhancement were respectively observable on Apparent Diffusion Coefficient (ADC) map of Diffusion Weighted Imaging (DWI) and after gadolinium injection (Fig. 1.3).
Because of the rapid worsening of symptoms another MRI was executed after nine days, which was also integrated by Dynamic susceptibility contrast (DSC) Perfusion-weighted Imaging (PWI).
New T2 hyperintense lesions had appeared, while others had enlarged (Fig. 1.4). Signal restriction on DWI had disappeared, and perilesional vasogenic edema had increased (Fig. 1.5). Different "open ring” and pseudostratified patterns of enhancement were now observable (Fig. 1.6), although, cerebral blood volume map (CBV) acquired with dynamic susceptibility contrast perfusion MRI showed no increased perfusion, but only a mild hypoperfusion in the right frontal lobe. (Fig. 1.7).
These new imaging findings allowed the diagnosis of a Von Balò’s demyelinating disease (supported also by an increase of cerebrospinal fluid proteins after lumbar puncture and normal cervical spinal MRI).
Corticosteroid therapy with adjunctive plasmapheresis were started with minimal benefit, but the patient didn’t end the whole cycle because of her low compliance. So, she decided to come back home, against doctors’ opinion, with an important disability characterized by left-body hyposthenia, left arm intentional tremor, and alternating moments of strong depression and euphoria.
Nevertheless, we obtained by mutual agreement to follow the patient clinically and with annual MRI follow-up.
MRI, performed 4 years after the onset, showed vasogenic edema and contrast medium uptake disappearance (Fig. 1.8). Fewer and smaller T2-hyperintensities were present in the white matter of the frontal lobes, especially on the right side (Fig. 1.9). Slight bilateral frontal atrophic changes had appeared in the meantime, although clinical improvement was still noted. Milder symptoms such as mood instability, left hyposthenia, intentional left-tremor at upper limb, and some frontal behavioral defects were residual.
Moreover, such residual symptoms anatomically correlated with the right frontal lobe which had been the most hypo-perfused region on PWI during the acute/subacute phase of the disease.
So, we asked ourselves if PWI could be used to improve diagnosis and predict prognosis.