ABSTRACT
Introduction: The resection of brainstem cerebral cavernous malformations (CCM) harbors the risk of damaging the corticospinal tract (CST) and other major tracts. Hence, visualization of eloquent fiber tracts supports pre- and intraoperative planning. However, diffusion tensor imaging fiber tracking at brainstem level suffers from distortion due to field inhomogeneities and eddy currents by steep diffusion gradients. Research question: This study aims to analyze the effect of distortion correction for CST tractography in brainstem CCM patients. Material and methods: 25 patients who underwent resection of brainstem CCM were enrolled, 24 suffered from hemorrhage. We performed an anatomically based tractography of the CST with a mean minimal fractional anisotropy of 0.22 ± 0.04 before and after cranial distortion correction (CDC). Accuracy was measured by anatomical plausibility and aberrant fibers. Results: CDC led to a more precise CST tractography, further approximating its assumed anatomical localization in all cases. CDC resulted in a significantly more ventral location of the CST of 1.5 ± 0.6 mm (6.1 ± 2.7 mm before CDC vs. 4.6 ± 2.1 mm after CDC; p < .0001) as measured by the distance to the basilar artery and of 1.7 ± 0.6 mm (8.9 ± 2.7 mm vs. 7.2 ± 2.1 mm; p < .0001) in relation to the clivus. Aberrant fibers were reduced by CDC in 44% of cases. We found a mean difference in CST volume of 0.6 ± 0.8 ccm. We could not detect motor deficits after resection of irregular fibers. Discussion and conclusion: CDC effectively corrects tractography for distortion at brainstem level, especially in patients suffering from brainstem CCM, further approximating its actual anatomical localization.
ABSTRACT
In patients with spinal muscular atrophy (SMA) headache after intrathecal administration of nusinersen is usually attributed to post-lumbar puncture syndrome. However, lumbar puncture opening pressure (LOP) has also been reported to be increased in children with SMA, both before and after treatment with nusinersen, although symptoms associated with increased LOP were not observed. We report to our knowledge the first case of symptomatic intracranial hypertension in an adult SMA patient. This 21-year-old man suffered from headache and vomiting followed by visual disturbances after the 12th injection of nusinersen. Bilateral papilledema was recognized ophthalmologically. MRI of the head showed signs of intracranial hypertension and additionally arachnoid cysts but not hydrocephalus. Symptoms resolved after 8 weeks of treatment with repeated lumbar punctures and acetazolamide. This case raises the possibility of intracranial hypertension as a complication of nusinersen therapy although arachnoid cysts represent another risk factor for intracranial hypertension. We recommend that patients suffering from headache after nusinersen injections should not only be questioned and examined for symptoms suggestive of post-lumbar puncture syndrome, but also intracranial hypertension.
