Diffusion Tensor Imaging of Microstructural Changes in the Gray and White Matter in Patients With Crigler-Najjar Syndrome Type I

2020 ◽  
Vol 44 (3) ◽  
pp. 393-398 ◽  
Author(s):  
Ahmed Abdel Khalek Abdel Razek ◽  
Saher Ebrahiem Taman ◽  
Mohamed Ezz El Regal ◽  
Ahmed Megahed ◽  
Sherine Elzeny ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
White Matter ◽  
Diffusion Tensor ◽  
Type I ◽  
Syndrome Type ◽  
Microstructural Changes

Diffusion tensor imaging of cerebral white matter in patients with myotonic dystrophy

2005 ◽  
Vol 46 (1) ◽  
pp. 104-109 ◽  
Author(s):  
H. Fukuda ◽  
J. Horiguchi ◽  
C. Ono ◽  
T. Ohshita ◽  
J. Takaba ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
White Matter ◽  
Myotonic Dystrophy ◽  
Normal Control ◽  
Diffusion Tensor ◽  
Age At Onset ◽  
Mean Diffusivity ◽  
Magnetic Resonance Images ◽  
Microstructural Changes ◽  
Control Subjects

Purpose: To determine whether myotonic dystrophy (MyD) patients have diffusion tensor abnormalities suggestive of microstructural changes in normal‐appearing white matter (NAWM). Material and Methods: Conventional and diffusion tensor magnetic resonance images of the brain were obtained in 19 MyD patients and 19 age‐matched normal control subjects. Fractional anisotropy (FA) and mean diffusivity (MD) values were calculated in white matter lesions (WMLs) and NAWM in MyD patients and in the white matter of normal control subjects. Differences between WML and NAWM values and between MyD patient and control subject values were analyzed statistically. Results: Significantly lower FA and higher MD values were found in all regions of interest in the NAWM of MyD patients than in the white matter of control subjects ( P<0.01), as well as significantly lower FA and higher MD values in WMLs than in NAWM of MyD patients ( P<0.05). There was no significant correlation of mean FA or MD values in NAWM with patient age, age at onset, or duration of illness ( P>0.1). Conclusion: Diffusion tensor imaging analysis suggests the presence of diffuse microstructural changes in NAWM of MyD patients that may play an important role in the development of disability.

Diffusion Tensor Imaging of Auditory Pathway in Patients With Crigler-Najjar Syndrome Type I: Correlation With Auditory Brainstem Response

2021 ◽  
pp. 088307382110258
Author(s):  
Ahmed Abdel Khalek Abdel Razek ◽  
Mohamed Ezz El Regal ◽  
Mortada El-Shabrawi ◽  
Mohamed Moustafa Abdeltawwab ◽  
Ahmed Megahed ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
Inferior Colliculus ◽  
Cochlear Nucleus ◽  
Auditory Brainstem Response ◽  
Diffusion Tensor ◽  
Auditory Brainstem ◽  
Type I ◽  
Syndrome Type ◽  
Brainstem Response ◽  
Olivary Nucleus

Aim: To evaluate the role of diffusion tensor imaging of the auditory pathway in patients with Crigler Najjar syndrome type I and its relation to auditory brainstem response. Methods: Prospective study was done including 12 patients with Crigler Najjar syndrome type I and 10 age- and sex-matched controls that underwent diffusion tensor imaging of brain. Mean diffusivity and fractional anisotropy at 4 regions of the brain and brainstem on each side were measured and correlated with the results of auditory brainstem response for patients. Results: There was significantly higher mean diffusivity of cochlear nucleus, superior olivary nucleus, inferior colliculus, and auditory cortex of patients versus controls on both sides for all regions ( P = .001). The fractional anisotropy of cochlear nucleus, superior olivary nucleus, inferior colliculus, and auditory cortex of patients versus controls was significantly lower, with P values of, respectively, .001, .001, .003, and .001 on the right side and .001, .001, .003, and .001 on left side, respectively. Also, a negative correlation was found between the maximum bilirubin level and fractional anisotropy of the left superior olivary nucleus and inferior colliculus of both sides. A positive correlation was found between the mean diffusivity and auditory brainstem response wave latency of the right inferior colliculus and left cochlear nucleus. The fractional anisotropy and auditory brainstem response wave latency of the right superior olivary nucleus, left cochlear nucleus, and inferior colliculus of both sides were negatively correlated. Conclusion: Diffusion tensor imaging can detect microstructural changes in the auditory pathway in Crigler Najjar syndrome type I that can be correlated with auditory brainstem response.

scholarly journals Diffusion tensor imaging findings suggestive of white matter alterations in a canine model of mucopolysaccharidosis type I

2017 ◽  
Vol 31 (1) ◽  
pp. 90-94 ◽  
Author(s):  
Dana M Middleton ◽  
Jonathan Y Li ◽  
Steven D Chen ◽  
Leonard E White ◽  
Patricia Dickson ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
White Matter ◽  
Confidence Intervals ◽  
Brain Region ◽  
Imaging System ◽  
Diffusion Tensor ◽  
Canine Model ◽  
Magnetic Resonance Imaging System ◽  
Type I ◽  
Lysosomal Storage

Purpose We investigated fractional anisotropy (FA) and radial diffusivity (RD) in a canine model of mucopolysaccharidosis (MPS). We hypothesized that canines affected with MPS would exhibit decreased FA and increased RD values when compared to unaffected canines, a trend that has been previously described in humans with white matter diseases. Methods Four unaffected canines and two canines with MPS were euthanized at 18 weeks of age. Their brains were imaged using high-resolution diffusion tensor imaging (DTI) on a 7T small-animal magnetic resonance imaging system. One hundred regions of interest (ROIs) were placed in each of four white matter regions: anterior and posterior regions of the internal capsule (AIC and PIC, respectively) and anterior and posterior regions of the centrum semiovale (ACS and PCS, respectively). For each specimen, average FA and RD values and associated 95% confidence intervals were calculated from 100 ROIs for each brain region. Results For each brain region, the FA values in MPS brains were consistently lower than in unaffected dogs, and the RD values in MPS dogs were consistently higher, supporting our hypothesis. The confidence intervals for affected and unaffected canines did not overlap in any brain region. Conclusion FA and RD values followed the predicted trend in canines affected with MPS, a trend that has been described in humans with lysosomal storage and dysmyelinating diseases. These findings suggest that the canine model parallels MPS in humans, and further indicates that quantitative DTI analysis of such animals may be suitable for future study of disease progression and therapeutic response in MPS.

Brain white matter abnormalities in paediatric Gaucher Type I and Type III using diffusion tensor imaging

2011 ◽  
Vol 34 (2) ◽  
pp. 549-553 ◽  
Author(s):  
Elin Haf Davies ◽  
Kiran K. Seunarine ◽  
Tina Banks ◽  
Chris A. Clark ◽  
Ashok Vellodi
Keyword(s):  
Diffusion Tensor Imaging ◽  
White Matter ◽  
Diffusion Tensor ◽  
Type I ◽  
White Matter Abnormalities ◽  
Type Iii ◽  
Brain White Matter

Abstract WP442: Diffusion Tensor Imaging of White Matter Tracts in Transient Ischemic Attack Patients

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Sana Tariq ◽  
Adrian Tsang ◽  
Jacob Ursenbach ◽  
Naomi-Rose Dutta ◽  
Linda B Andersen ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
White Matter ◽  
Linear Regression ◽  
Transient Ischemic Attack ◽  
Diffusion Tensor ◽  
Memory Function ◽  
Healthy Controls ◽  
Median Score ◽  
Microstructural Changes ◽  
Ischemic Attack

Introduction: Transient ischemic attack (TIA) greatly increases the risk of developing dementia later in life. Measurement of microstructural changes in white matter (WM) using diffusion tensor imaging (DTI) tractography has potential for identifying those at greatest risk of cognitive decline. We hypothesized that patients presenting with TIA have abnormal DTI measures in major WM tracts. Our objective was to determine changes in DTI measures for fractional anisotropy (FA) and medial diffusivity (MD) in frontoparietal (Superior Longitudinal Fasciculus; SF) and medial temporal (Uncinate Fasciculus; UF) WM tracts for TIA patients and healthy controls. Methods: Patients presenting with symptoms of high risk TIA but free of dementia, and healthy volunteer controls were recruited acutely. Structural MRI, inclusive of DTI sequences (31 directions b1000) was performed. FA and MD values were collected in the left and right SLF and UF. Multiple linear regression was performed to determine predictors of DTI values (FA and MD), adjusted for age. Subject’s cognition was screened using Montreal Cognitive Assessment (MoCA). Results: Data from 60 TIA patients (mean age = 68.75 years, SD = 9.39, 45% female) and 33 healthy controls (mean age = 64.97 years, SD = 10.45, 67% female) was analyzed. Linear regression identified that TIA patients have higher FA values in left SLF, F(2, 90) = 9.210, p < .001, R 2 = .170); right SLF, F(2, 90) = 7.154, p = .001, R 2 = .137; and left UF, F(2, 90) = 3.513, p = .034, R 2 = .072, compared to healthy controls. No group changes in MD were observed when corrected for age. TIA patients (median score 24, (interquartile range (IQR) = 5) performed worse than healthy controls (median score = 27, IQR = 4) on the MoCA while controlling for age, F(1, 90) = 7.689, p < .007. Conclusion: TIA patients showed changes in FA of WM tracts related to language and memory function when compared to healthy controls, supporting the presence of incipient microstructural disease. Our results show that measures of microstructural changes using DTI may help identify TIA patients at a greater risk of developing cognitive impairment. Future work aims to identify deterioration of DTI measures over time and their relation to potential vascular and neurodegenerative etiologies.

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