Brain injury unmasking Ehlers-Danlos syndromes after trauma: the fiber print.

BACKGROUND: The role of physical trauma in the onset of symptoms in Ehlers-Danlos syndrome (EDS) has never been characterized. We sought to search and describe brain lesions EDS patients also having personal history of physical trauma. We systematically performed brain magnetic resonance imaging in a first cohort of patients with a hypermobility type of EDS which described the onset of their disease or its worsening after a physical trauma. Unexpected yet consistent findings that were thought to be related to the reported traumas led to perform brain imaging in all subsequent patients with similar symptoms regardless of a history of trauma and to search for a prior trauma by active questioning. RESULTS: Fifty-nine patients were recruited and analyzed, among which 53 (89.8%) were women. Overall, 26 (44.1%) reported a personal history of physical trauma. Six signs pertaining to subcortical lesions and affecting white matter tracts were identified. Those included lesions of the reticular formation, the two lenticular nuclei, the corpus callosum and the arcuate fasciculus. Thirty-six patients (61.0%) had at least 5 of the 6 imaging signs. In case of a trauma before 18, patients had significantly more lesions of the reticular formation (100% vs. 50%; p = 0.0035). CONCLUSIONS: Patients with EDS, hypermobility type, were found to have consistent and specific brain lesions involving white matter tracts. Moreover, the record of a physical trauma in a substantial proportion of cases suggests that these lesions could be post-trauma consequences. Therefore, physical trauma could be a triggering factor in EDS.

Three-dimensional dynamic magnetic resonance angiography for the evaluation of radiosurgically treated cerebral arteriovenous malformations.

We assessed the value of three-dimensional (3D) dynamic magnetic resonance angiography (MRA) for the follow-up of patients with radiosurgically treated cerebral arteriovenous malformations (AVMs). Fifty-four patients with cerebral AVMs treated by radiosurgery (RS) were monitored using conventional catheter angiography (CCA) and 3D dynamic MRA with sensitivity encoding based on the parallel imaging. Cerebral AVM was qualitatively classified by two radiologists into one of five categories in terms of residual nidus size and persistence of early draining vein (I, >6 cm; II, 3-6 cm; III, <3 cm; IV, isolated early draining vein; V, complete obliteration). 3D MRA findings showed a good agreement with CCA in 40 cases (kappa=0.62). Of 23 nidus detected on CCA, 3D dynamic MRA showed 14 residual nidus. Of 28 occluded nidus on 3D dynamic MRA, 22 nidus were occluded on CCA. The sensitivity and specificity of 3D dynamic MRA for the detection of residual AVM were 81% and 100%. 3D dynamic MRA after RS may therefore be useful in association with MRI and can be repeated as long as opacification of the nidus or early venous drainage persists, one CCA remaining indispensable to affirm the complete occlusion at the end of follow-up.

Age-related cerebral white matter changes and pulse-wave encephalopathy: observations with three-dimensional MRI.

Our purpose was to investigate leukoaraïosis (LA) using three-dimensional MR imaging combined with advanced image-processing technology to attempt to group signal abnormalities according to their etiology. Coronal T2-weighted fast fluid-attenuated inversion-recovery (FLAIR) sequences and three-dimensional T1-weighted fast spoiled gradient recalled echo sequences were used to examine cerebral white matter changes in 75 elderly people with memory complaint but no dementia. They were otherwise healthy, community-dwelling subjects. Three subtypes of LA were defined on the basis of their shape, geography and extent: the so-called subependymal/subpial LA, perivascular LA and "bands" along long white matter tracts. Subependymal changes were directly contiguous with ventricular spaces. They showed features of "water hammer" lesions with ventricular systematisation and a more frequent location around the frontal horns than around the bodies (P=.0008). The use of cerebrospinal fluid (CSF) contiguity criterion allowed a classification of splenial changes in the subpial group. Conversely, posterior periventricular lesions in the centrum ovale as well as irregular and extensive periventricular lesions were not directly contiguous with CSF spaces. The so-called perivascular changes showed features of small-vessel-associated disease; they surrounded linear CSF-like signals that followed the direction of perforating vessels. Distribution of these perivascular changes appeared heterogeneous (P ranging from .04 to 5.10(-16)). These findings suggest that subependymal/subpial LA and subcortical LA may be separate manifestations of a single underlying pulse-wave encephalopathy.

Spontaneous intracerebral hematoma on diffusion-weighted images: influence of T2-shine-through and T2-blackout effects.

BACKGROUND AND PURPOSE: On diffusion-weighted (DW) images, primary hematomas are initially mainly hyperintense, and then hypointense during the first few days after stroke onset. As in other brain disorders, variations in the T2 relaxation time of the hematoma influence the DW imaging signal intensity. Our aim was to evaluate the contribution of the T2 signal intensity and apparent diffusion coefficient (ADC) changes to signal intensity displayed by DW imaging through the course of hematoma. METHODS: The MR images of 33 patients with primary intracranial hemorrhage were retrospectively reviewed. Variations in T2-weighted echo planar images, DW imaging signal intensity, and apparent diffusion coefficient (ADC) ratios (core of hematoma/contralateral hemisphere) were analyzed according to the putative stages of hematoma, as seen on T1- and T2-weighted images. RESULTS: On both T2-weighted echo planar and DW images, the core of the hematomas was hyperintense at the hyperacute (oxyhemoglobin, n = 11) and late subacute stages (extracellular methemoglobin, n = 4), while being hypointense at the acute (deoxyhemoglobin, n = 11) and early subacute stages (extracellular methemoglobin, n = 7). There was a positive correlation between the signal intensity ratio on T2-weighted echo planar and DW images (r = 0.93, P < .05). ADC ratios were significantly decreased in the whole population and in each of the first three stages of hematoma, without any correlation between DW imaging findings and ADC changes (r = 0.09, P = .6). CONCLUSION: Our results confirm that the core of hematomas is hyperintense on DW images with decreased ADC values at the earliest time point, and may thus mimic arterial stroke on DW images. T2 shine-through and T2 blackout effects contribute to the DW imaging findings of hyperintense and hypointense hematomas, respectively, while ADC values are moderately but consistently decreased during the first three stages of hematoma.

Evaluation of hyperintense vessels on FLAIR MRI for the diagnosis of multiple intracerebral arterial stenoses.

BACKGROUND AND PURPOSE: Hyperintense vessel sign (HVS) on fluid-attenuated inversion recovery (FLAIR) has been described in hyperacute stroke patients with arterial occlusion. We sought to determine whether HVS was more frequent in patients with intracerebral arterial stenoses than in those without stenosis regardless of the presence of a brain infarct. METHODS: In this case-control study (19 symptomatic patients with multiple intracerebral arterial stenoses compared with 19 age-matched asymptomatic patients without stenosis), we looked for HVS (ie, focal or tubular hyperintensities in the subarachnoid space) on FLAIR images. We compared the proportion of HVS-positive patients in the 2 groups and evaluated the concordance between the arterial distribution of stenoses on angiogram and that of HVS on FLAIR. RESULTS: HVS was found in 13 of 19 patients (68%) in the study group and 1 of 19 control patients (5.2%) (P<0.0001). The concordance between the territorial distribution of stenoses on angiogram and HVS on FLAIR was higher for the right and left middle cerebral artery (kappa=0.6 and 0.63, respectively) compared with the right and left anterior cerebral artery (kappa=0.35 and 0.2, respectively). HVSs were observed in 1 of 7 patients with posterior cerebral artery stenoses on angiogram. HVSs were seen equally in patients with acute focal (7 of 10) or diffuse (6 of 9) cerebral involvement. In the 6 HVS-positive patients with acute stroke confirmed by MRI, additional HVSs were observed in a different arterial territory than that of the stroke lesion. CONCLUSIONS: Although their significance remains unclear, multiple HVSs are more frequently observed in symptomatic patients with multiple intracerebral stenoses than in asymptomatic patients without stenosis.