scholarly journals A case of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) presenting as post-infectious manifestation of SARS-CoV-2 infection

2021 ◽  
pp. 20210020
Author(s):  
Ishwariya Rajendran ◽  
Madhu Dutta Natarajan ◽  
Pooja Narwani ◽  
Omran Alzouabi ◽  
Khalil Kawafi ◽  
...  
Keyword(s):  
White Matter ◽  
Cerebrovascular Disease ◽  
White Matter Hyperintensities ◽  
Unusual Case ◽  
Autosomal Dominant ◽  
Case Reports ◽  
Imaging Features ◽  
Neurological Manifestations ◽  
Lacunar Infarcts ◽  
Post Infectious

White matter hyperintensities (WMHs) lacunar infarcts and cerebral microbleeds are well-established features associated with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Increasing case reports and series recounts a wide array of neurological manifestations of COVID-19 including acute cerebrovascular disease, encephalopathy, encephalitis and demyelination. Recently association between COVID-19 and CADASIL has been identified. We describe an unusual case of CADASIL diagnosed as a possible post-infectious manifestation of COVID-19 patient with imaging features closely resembling post-infectious encephalomyelitis.

Vanishing White Matter Hyperintensities in CADASIL: A Case Report with Insight into Disease Mechanisms

2020 ◽  
Vol 78 (3) ◽  
pp. 907-910
Author(s):  
Eric Jouvent ◽  
Nassira Alili ◽  
Dominique Hervé ◽  
Hugues Chabriat
Keyword(s):  
White Matter ◽  
White Matter Hyperintensities ◽  
Autosomal Dominant ◽  
Brain Area ◽  
Imaging Features ◽  
Water Influx ◽  
Disease Mechanisms ◽  
The Brain ◽  
Vanishing White Matter ◽  
Insight Into

In a woman with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) followed for 15 years, we observed magnetic resonance imaging white matter hyperintensities that vanished in the anterior temporal poles while the brain volume decreased unexpectedly. These imaging changes were transient and detected when the patient was being treated by valproic acid for stabilizing mood disturbances. This intriguing case supports that mechanisms underlying white matter hyperintensities can vary from one brain area to another and that important modifications of water influx into the brain tissue might be involved in some imaging features of CADASIL.

scholarly journals Parotid Oncocytoma as a Manifestation of Birt-Hogg-Dubé Syndrome

2018 ◽  
Vol 2018 ◽  
pp. 1-7
Author(s):  
Kazuki Yoshida ◽  
Masao Miyagawa ◽  
Teruhito Kido ◽  
Kana Ide ◽  
Yoshifumi Sano ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Autosomal Dominant ◽  
Case Reports ◽  
Imaging Features ◽  
Resonance Imaging ◽  
Rare Disorders ◽  
Pulmonary Cysts ◽  
Dominant Disease ◽  
Rare Autosomal Dominant Disease

Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disease characterized by skin fibrofolliculomas, pulmonary cysts, spontaneous pneumothoraces, and renal cancers. Oncocytomas are benign epithelial tumors that are also rare. Recently, there have been a few case reports of BHD with a parotid oncocytoma that appears to have a BHD phenotype. Here we document the eighth known case and describe the magnetic resonance imaging features of the parotid oncocytoma, which mimicked Warthin’s tumor. Radiologists should be aware of the association between these rare disorders.

scholarly journals Increased interstitial fluid in periventricular and deep white matter hyperintensities in patients with suspected idiopathic normal pressure hydrocephalus

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Alexander Rau ◽  
Marco Reisert ◽  
Elias Kellner ◽  
Jonas A. Hosp ◽  
Horst Urbach ◽  
...  
Keyword(s):  
White Matter ◽  
Water Content ◽  
Normal Pressure Hydrocephalus ◽  
Interstitial Fluid ◽  
White Matter Hyperintensities ◽  
Free Water ◽  
Normal Pressure ◽  
Idiopathic Normal Pressure Hydrocephalus ◽  
Imaging Features ◽  
Deep White Matter

AbstractPeriventricular white matter changes are common in patients with idiopathic normal pressure hydrocephalus (iNPH) and considered to represent focally elevated interstitial fluid. We compared diffusion measures in periventricular hyperintensities in patients with imaging features of iNPH to patients without. The hypothesis is that periventricular hyperintensities in patients with presumed iNPH show higher water content than in patients without imaging features of iNPH. 21 patients with iNPH Radscale 7–12 (“high probability of iNPH”) and 10 patients with iNPH Radscale 2–4 (“low probability of iNPH”) were examined with a neurodegeneration imaging protocol including a diffusion microstructure imaging sequence. Periventricular hyperintensities and deep white matter hyperintensities were segmented and diffusion measures were compared. In patients with imaging features of iNPH, the free water content in periventricular hyperintensities was significantly higher compared to the control group (p = 0.005). This effect was also detectable in deep white matter hyperintensities (p = 0.024). Total brain volumes and total gray or white matter volumes did not differ between the groups. Periventricular cap free water fraction was highly discriminative regarding patients with presumed iNPH and controls with an ROC AUC of 0.933. Quantitative diffusion microstructure imaging shows elevated water content in periventricular hyperintensities in patients with imaging features of iNPH, which could be the imaging correlate for pathologic fluid accumulation and may be used as an imaging biomarker in the future.

Abstract MP057: Subclinical Cerebrovascular Disease is Associated with Worsening Gait and Balance in an Elderly Multi-Ethnic Population: The Northern Manhattan Study (NOMAS)

Circulation ◽  
2017 ◽  
Vol 135 (suppl_1) ◽  
Author(s):  
Joshua Z Willey ◽  
Yeseon Park Moon ◽  
Janet DeRosa ◽  
Erin Kulick ◽  
Sandino Cespedes ◽  
...  
Keyword(s):  
White Matter ◽  
Cerebrovascular Disease ◽  
White Matter Hyperintensities ◽  
Physical Aging ◽  
Disease Risk ◽  
Cardiovascular Disease Risk ◽  
The Elderly ◽  
Brain Regions ◽  
Periventricular White Matter ◽  
Age Related

Introduction: Age-related decline in gait and balance is a contributor to morbidity in the elderly. Subclinical cerebrovascular disease, seen on magnetic resonance imaging (MRI) as white matter hyperintensities (WMH) and silent brain infarcts (SBI), is associated with impaired mobility. Less is known about the association of WMH in specific brain regions and mobility impairment. We hypothesized that anterior WMH volume would be associated with lower scores on the Short Physical Performance Battery (SPPB), a well-validated mobility scale associated with falls and mortality in the elderly. Methods: Participants in the Northern Manhattan Study MRI cohort had the SPPB measured a median of 5 years after enrollment. The SPPB has three domains with a maximum total score of 12: gait speed, chair stands, standing balance. Volumetric distributions for WMH volume across 14 brain regions (brainstem, cerebellum, and bilateral frontal, occipital, temporal, and parietal lobes, and bilateral anterior and posterior periventricular white matter) were determined separately for each hemisphere by combining bimodal image intensity distribution and atlas based methods. Multi-variable linear regression was performed to examine the association between SBI and total and regional (frontal, parietal, occipital, temporal, anterior, posterior, and brainstem) head size-corrected WMH volumes, with the total SPPB score; models were adjusted for cardiovascular disease risk factors, osteo-arthritis, and grip strength. Results: Among 668 stroke-free participants with the SPPB available, mean age at the time of assessment was 74 ±9 years, 37% were male and 70% Hispanic; the mean SPPB score was 8.2 ± 2.9, interquartile range 7-10. Mean total WMHV was 0.55±0.75cc, mean anterior WMH volume 0.18±0.24cc, and 12% of participants had SBI. In multi-variable models, total WMHV was associated with a lower SPPB (beta = -0.3 per SD of logWMH, p=0.004), while SBI was not (beta= -0.12, p=0.7). For regional WMH volumes, only greater anterior periventricular WMHV was associated with SPPB (beta= -0.29 per SD, p=0.009). Conclusions: White matter hyperintensities, especially in the anterior cerebral regions, are associated with a lower SPPB. Prevention of subclinical cerebrovascular disease is a potential target to prevent physical aging in the elderly.

scholarly journals Extensive White Matter Hyperintensities May Increase Brain Volume in Cerebral Autosomal-Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy

Stroke ◽  
2012 ◽  
Vol 43 (12) ◽  
pp. 3252-3257 ◽  
Author(s):  
Ming Yao ◽  
Eric Jouvent ◽  
Marco During ◽  
Ophélia Godin ◽  
Dominique Hervé ◽  
...  
Keyword(s):  
White Matter ◽  
White Matter Hyperintensities ◽  
Autosomal Dominant ◽  
Brain Volume

scholarly journals Spectrum of findings on magnetic resonance imaging of the brain in patients with neurological manifestations of dengue fever

2017 ◽  
Vol 50 (5) ◽  
pp. 285-290 ◽  
Author(s):  
Tejeshwar Singh Jugpal ◽  
Rashmi Dixit ◽  
Anju Garg ◽  
Swati Gupta ◽  
Virendra Jain ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cerebral Cortex ◽  
White Matter ◽  
Basal Ganglia ◽  
Magnetic Resonance ◽  
Dengue Fever ◽  
Imaging Features ◽  
Neurological Manifestations ◽  
Resonance Imaging ◽  
Mri Findings

Abstract Objective: To describe the spectrum of magnetic resonance imaging (MRI) findings in patients with neurological manifestations of dengue. Materials and Methods: We included nine patients with dengue fever (three females and six males; age range, 9–30 years), all of whom presented with neurological manifestations. The MRI examinations, performed in 1.5 T or 3 T scanners, included T1-weighted, T2-weighted, and fluid-attenuated inversion recovery (FLAIR) sequences. Diffusion-weighted imaging with apparent diffusion coefficient mapping was also employed. Fast low-angle shot and susceptibility-weighted gradient-recalled echo sequences, as well as contrast-enhanced T1-weighted scans, were also obtained in order to assess parenchymal enhancement. MRI scans were analyzed for lesion distribution and imaging features. Results: All patients showed areas of altered signal intensity that appeared as hyperintensity on T2-weighted and FLAIR sequences. The most commonly affected site was the basal ganglia-thalamus complex. Other affected sites were the cerebellum, cerebral cortex, white matter, and brainstem. In all cases, we observed patchy areas of restricted diffusion and focal areas of hemorrhage. Conclusion: Dengue encephalitis commonly affects the basal ganglia, thalamus, cerebellum, cerebral cortex, and white matter. Therefore, MRI should be an indispensable part of the evaluation of patients with neurological complications of dengue fever.

scholarly journals Fluid biomarkers of white matter hyperintensities in cerebrovascular disease and neurodegeneration

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Chloe Anastassiadis ◽  
Anna Vasilevskaya ◽  
Melisa Gumus ◽  
Alexandra Santos ◽  
Maria Carmela Tartaglia
Keyword(s):  
White Matter ◽  
Cerebrovascular Disease ◽  
White Matter Hyperintensities

scholarly journals Heterogeneity of White Matter Hyperintensities in Cognitively Impaired Patients With Cerebral Small Vessel Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Tingting Wang ◽  
Aoming Jin ◽  
Ying Fu ◽  
Zaiqiang Zhang ◽  
Shaowu Li ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
White Matter ◽  
White Matter Hyperintensities ◽  
Small Vessel Disease ◽  
Cerebral Small Vessel Disease ◽  
Cognitively Impaired ◽  
Imaging Features ◽  
Vessel Disease

ObjectiveSimilar white matter hyperintensities (WMH) might have different impact on the cognitive outcomes in patients with cerebral small vessel disease (CSVD). This study is to assess the possible factors related to the heterogeneity of WMH in cognitively impaired patients with CVSD.MethodsWe analyzed data from a cohort of patients with CVSD who were recruited consecutively from the Beijing Tiantan Hospital from 2015 to 2020. WMH, lacunes, enlarged perivascular space (ePVS), microbleeds and lacunar infarcts were rated on brain MRI. A score of <26 on the Montreal Cognitive Assessment (MoCA) indicated cognitive impairment. A mismatch was defined as the severity of WMH not matching the severity of cognitive dysfunction. Type-1 mismatch was defined as a mild WMH (Fazekas score = 0-1) associated with cognitive impairment, and type-2 mismatch was defined as a severe WMH (Fazekas score = 5-6) associated with normal cognitive function. Ultrasmall superparamagnetic iron oxide (USPIO)-enhanced SWI on 3-Tesla MRI was used to image the penetrating arteries in basal ganglia to explore the underlying mechanism of this mismatch. Multivariable logistic regression was used to analyze the association between the imaging features and cognitive impairment.ResultsIn 156 patients, 118 (75.6%) had cognitive impairment and 37 (23.7%) showed mismatch. Twenty five (16.0%) had type-1 mismatch and 12 (7.7%) had type-2 mismatch. Regression analysis found that WMH, lacunes, microbleeds and total CSVD scores were associated with cognitive impairment and were independent of vascular risk factors. However, lacunes, microbleeds and total CSVD scores were related to the mismatch between WMH and cognitive impairment (p=0.006, 0.005 and 0.0001, respectively). Specially, age and ePVS in basal ganglia were related to type-1 mismatch (p=0.04 and 0.02, respectively); microbleeds and total CSVD scores were related to type-2 mismatch (p=0.01 and 0.03, respectively). Although the severity of WMH was similar, the injury scores of penetrating arteries were significantly different between those with and without cognitive impairment (p=0.04).ConclusionsHeterogeneity of WMH was present in cognitively impaired patients with CSVD. Conventional imaging features and injury of penetrating arteries may account for such heterogeneity, which can be a hallmark for early identification and prevention of cognitive impairment.

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