76 Abstracts ingediend voor het Amsterdam Kindersymposium 2013 ... Pediatric Hematology, Emma Children's Hospital, Academic Medical Centre, Amsterdam.
Cerebral imaging with 7-Tesla MRI in patients with sickle cell disease: a pilot study Veronica van der Land (1), Jaco J. Zwanenburg (2), Bart J. Biemond (3), Jeroen Hendrikse (2), HenkJan M. M. Mutsaerts (4), Marc Engelen (5), Aart J. Nederveen (4), Paul J. Nederkoorn (6), Charles B.L.M. Majoie (4), Peter R. Luijten (2), Karin Fijnvandraat (1) (1) Department of Pediatric Hematology, Emma Children’s Hospital, Academic Medical Centre, Amsterdam (2) Department of Radiology, University Medical Center Utrecht, Utrecht (3) Department of Hematology, Academic Medical Centre, Amsterdam (4) Department of Radiology, Academic Medical Centre, Amsterdam (5) Department of Pediatric Neurology, Academic Medical Centre, Amsterdam (6) Department of Neurology, Academic Medical Centre, Amsterdam
INTRODUCTION Silent cerebral infarction (SCI) is an important complication of sickle cell disease (SCD) and can lead to neurocognitive dysfunction. Typically, SCI’s are white matter lesions of unknown etiology. They are not associated with stenosis of large intracranial vessels. White matter lesions in other conditions (Alzheimer’s disease, hypertension) are considered an aspect of small vessel disease along with other lesions such as microbleeds and microinfarcts. We performed ultra high field MRI (7T) to study the manifestations of small vessel disease in patients with SCD. METHODS
We included 9 patients with SCD (HbSS or HbSβ0) from the Academic Medical Center of Amsterdam without a history of overt stroke and no other neurological deficits. 7T MRI scanning was performed at the University Medical Center Utrecht. We scored number, size and location of cortical infarcts, white matter lesions and microbleeds separately.
RESULTS
Mean age was 23 years (range 19-25 years), four patients were male. Three patients had evidence of cortical infarcts. One of these patients had multiple white matter lesions, mostly located in the frontal lobe (figure). We identified a subcortical micro-infarct in another patient. Surprisingly, we did not find any microbleeds.
CONCLUSION
Despite their young age, we found evidence of SCD related intracerebral lesions in four (44%) patients. The presence of white matter lesions and a subcortical micro-infarct is suggestive of small vessel disease in SCD. The absence of microbleeds however suggests a specific sickle cell related pathophysiological background.
Figure. Multiple white matter lesions (arrows) in a 25-year old patient with sickle cell disease, 7T T2-weighted images
76 Abstracts ingediend voor het Amsterdam Kindersymposium 2013
