PB 3 Abnormal height of basilar artery bifurcation in adults with Pompe disease

• Published in: Clinical neurophysiology 2017-10, Vol.128 (10), p.e315-e316
• Main Authors: Hensel, O., Schneider, I., Wieprecht, M., Zierz, S.
• Format: Article
• Language: English
• ISSN: 1388-2457; 1872-8952
• DOI: 10.1016/j.clinph.2017.06.061

The evidence for an involvement of the cerebral arteries in adults with Pompe disease is increasing.Typical changes are dilation, elongation and distension of the vertebrobasilar arteries (Laforêt et al., 2008; Sacconi et al., 2010; Hensel et al., 2014; Montagnese et al., 2016). In 15 adults with Pompe disease (age 57±13years, 8 females) and 12 controls with comparable age and cardiovascular risk factors (age 53±6years, 4 females) the cerebral arteries were examined with a time of flight MR-angiography.The angle of departure of the superior cerebellar artery (SUCA) was measured from the basilar artery (BA) (Figs.1 and 2).In addition, the level of the bifurcation of the BA was evaluated by a Smoker’s criteria based definition (Smoker et al., 1986) (score at or below the dorsum sellae, score 1 within suprasellar cistern, score 2 at the level of third ventricle floor, score 3 indenting and elevating floor of third ventricle). In adults with Pompe disease the angle of SUCA departure was 59° (sum left and right 119°, controls: left 75° and right 83°, sum 159°).Compared with a preliminary MR-angiography five years before (Hensel et al., 2014) the angle of departure decreased from 5 to 11° (n=5 patients).In 11/15 Pompe patients, the bifurcation of the BA was abnormally high (Smoker score 0: 0 patients, score 1: 4 patients, score 2: 5 patients and score 3: 6 patients).In controls this was rarer and less pronounced (Smoker score 0: 6 patients, score 1: 5 patients, score 2: 0 and score 3: 1 patient).The Smoker score was lower among young Pompe patients than in older patients.In 7/15 (47%) of Pompe patients and in 1/12 (8%) of the controls the proximal posterior cerebral artery (PCA) indenting the hypothalamus (arrow in Fig.3).One patient developed a hemorrhage in the thalamus, which was supplied by the displaced superiorly PCA. The results indicate a upwards displacement of the distal basilar artery in adults with Pompe disease.Such upwards displacement in patients with dolichoectasia of other origin is associated with any stroke and subsequently increased dolichoectatic progress (Passero and Rossi, 2008). In our patients the degree of displacement increases with patient age or duration of the disease. These could represent an accelerated destruction of vertebrobasilar arteries in adults with Pompe disease. The abnormal height of basilar bifurcation could represent an early and easy recognizable sign for cerebrovascular involvement as well.