Injuries of neural tracts in a patient with CADASIL: a diffusion tensor imaging study

Abstract

BackgroundWe report a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), who showed injuries of the neural tracts, which was demonstrated by diffusion tensor tractography (DTT).Case presentationA 64-year-old male patient and seven age-matched control volunteers were recruited. Since approximately 1.5 years ago, he had felt mild weakness of the right arm and was diagnosed as CADASIL by the finding of the exon 11 mutation of the NOTCH3 gene approximately 10 months ago. T2-weighted and FLAIR brain MRI images obtained at admission showed high signal intensity lesions in the subcortical gray matter and periventricular white matter. He showed mild quadriparesis, mild dysarthria, mild cognitive impairment, and emotional problems. Diffusion tensor imaging was performed and nine neural tracts (corticospinal tract, corticobulbar tract, corticofugal tract from the supplementary motor area, corticofugal tract from the premotor cortex, thalmoprefrontal tract [TPT] to the dorsolateral prefrontal cortex, TPT to the ventrolateral prefrontal cortex, TPT to the orbitoprefrontal cortex, fornix, and cingulum) were reconstructed. Fractional anisotropy (FA), mean diffusivity (MD), and tract volume of each neural tract were measured. All neural tracts except for the left fornix showed at least one more abnormality in terms of DTT parameters (decrement of FA, increment of MD, or decrement of tract volume).ConclusionWe demonstrated injuries of the neural tracts in a patient with CADASIL. It appears that clinical manifestations in this patient were related to injuries of the neural tracts.

Diffusion Tensor Imaging to Map Brain Microstructural Changes in CADASIL

“…Accordingly, the present study reveals that as compared to a group of healthy controls, patients with CADASIL show widespread significant changes of the DTI‐derived indices in WM tracts. This in line with the observation in a single case of CADASIL examined with tractography . The results of this between group analysis in consideration of the above neuropathological descriptions justifies the assumption that the DTI changes we observed correspond to the microstructural damage in CADASIL …”

Diffusion-Tensor-Tractography-Based Diagnosis for Injury of Corticospinal Tract in a Patient with Hemiplegia Following Traumatic Brain Injury

“…In Tract 2, the left CST showed a discontinuation to the cerebral cortex and a disconnection below the lower pons compared to Tract 1 (Figure 2B,Ca). As in previous studies, DTT parameter values (FA, MD, and TV) indicating a deviation of more or less than two standard deviations(SDs) compared with control subjects were defined as abnormal [9,10]. The majority of the left CST exhibited lower FA and TV values than SDs and higher MD values than two SDs.…”

Altered Default Mode Network Is Associated With Cognitive Impairment in CADASIL as Revealed by Multimodal Neuroimaging

“…Diffusion tensor imaging (DTI) studies have demonstrated widespread white matter lesions associated with cognitive deficits in CADASIL ( 13 – 15 ). Moreover, a case study using diffusion tensor tractography indicated that neural tract injuries were mainly located in the frontal lobe in a patient with CADASIL ( 16 ). Furthermore, a recent 18 F-2-fluoro-2-deoxy- d -glucose PET ( 18 F-FDG PET) study showed that decreased metabolism in the limbic lobe, including the hippocampus and PHC, was positively associated with a cognitive score in patients with CADASIL ( 17 ).…”