scholarly journals Longitudinal anterior-to-posterior shift of collateral channels in patients with moyamoya disease: an implication for its hemorrhagic onset

2019 ◽  
Vol 130 (3) ◽  
pp. 884-890 ◽  
Author(s):  
Shusuke Yamamoto ◽  
Satoshi Hori ◽  
Daina Kashiwazaki ◽  
Naoki Akioka ◽  
Naoya Kuwayama ◽  
...  
Keyword(s):  
Disease Progression ◽  
Moyamoya Disease ◽  
Staging System ◽  
Stage 4 ◽  
Advanced Stages ◽  
Lenticulostriate Artery ◽  
Moyamoya Vessel ◽  
Ischemic Attack ◽  
Adult Moyamoya Disease ◽  
The Impact

OBJECTIVEThis study aimed to assess longitudinal changes in the collateral channels originating from the lenticulostriate artery (LSA), posterior communicating artery (PCoA), and anterior and posterior choroidal arteries (AChA and PChA, respectively) during disease progression and/or aging. The impact of collateral channels on onset type was also examined.METHODSThis study included 71 involved hemispheres in 41 patients with moyamoya disease. The disease was categorized into 6 stages according to Suzuki’s angiographic staging system. The degree of development of each moyamoya vessel was categorized into 3 grades.RESULTSThe LSA started to dilate in stage 2, showed the most prominent development in stage 3, and decreased in more advanced stages (p < 0.001). The AChA most notably developed in stage 3 and gradually shrank (p = 0.04). The PCoA started to dilate in stage 3 and showed the most prominent development in stage 4 (p = 0.03). The PChA started to dilate in stage 3 and showed the most prominent development in stages 4 to 5 (p < 0.001). Patient age was negatively related to LSA development (p = 0.01, R = 0.30) and was positively associated with the abnormal dilation and extension of the PCoA (p = 0.02, R = 0.28) and PChA (p < 0.001, R = 0.45). The PCoA, AChA, and PChA more distinctly developed in hemispheres with intracerebral or intraventricular hemorrhage than in hemispheres with ischemic stroke or transient ischemic attack (p < 0.001, p = 0.03, and p = 0.03, respectively).CONCLUSIONSThis study suggests that the collateral channels through moyamoya vessels longitudinally shift from the anterior to posterior component during disease progression and aging, which may be closely related to the onset of hemorrhagic stroke in adult moyamoya disease.

scholarly journals Incidence and Clinical Features of Disease Progression in Adult Moyamoya Disease

Stroke ◽  
2005 ◽  
Vol 36 (10) ◽  
pp. 2148-2153 ◽  
Author(s):  
Satoshi Kuroda ◽  
Tatsuya Ishikawa ◽  
Kiyohiro Houkin ◽  
Rina Nanba ◽  
Masaaki Hokari ◽  
...  
Keyword(s):  
Disease Progression ◽  
Clinical Features ◽  
Moyamoya Disease ◽  
Adult Moyamoya Disease

scholarly journals The Impact of Moyamoya Disease and RNF213 Mutations on the Spectrum of Plasma Protein and MicroRNA

2019 ◽  
Vol 8 (10) ◽  
pp. 1648 ◽  
Author(s):  
Ming-Jen Lee ◽  
Shannon Fallen ◽  
Yong Zhou ◽  
David Baxter ◽  
Kelsey Scherler ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Plasma Protein ◽  
Ring Finger ◽  
Ring Finger Protein ◽  
Absolute Quantitation ◽  
Small Vessels ◽  
Finger Protein ◽  
Isobaric Tags ◽  
Ischemic Attack ◽  
The Impact

Moyamoya disease (MMD) is a rare cerebrovascular disorder characterized by occlusion of bilateral internal carotid and intracerebral arteries with the compensatory growth of fragile small vessels. MMD patients develop recurrent infarctions in the basal ganglia and subcortical regions. Symptoms include transient ischemic attack or stroke, seizures, and headaches, which may occur suddenly or in a stepwise progression. Mutations in Ring Finger Protein 213 (RNF213), a Zinc ring finger protein, have been identified in some MMD patients but the etiology of MMD is still largely unknown. To gain insight into the pathophysiology of MMD, we characterized the impact of the RNF213 mutations on plasma protein and RNA profiles. Isobaric tags for relative and absolute quantitation and proximity extension assay were used to characterize the plasma proteome. Next generation sequencing-based small RNAseq was used to analyze the cell-free small RNAs in whole plasma and RNA encapsulated in extracellular vesicles. The changes of miRNAs and proteins identified are associated with signaling processes including angiogenesis and immune activities which may reflect the pathology and progression of MMD.

scholarly journals Intracranial Hemodynamic Changes During Adult Moyamoya Disease Progression

2008 ◽  
Vol 4 (2) ◽  
pp. 67 ◽  
Author(s):  
Hyun-Jeong Kwag ◽  
Dong-Wook Jeong ◽  
Suk Hoon Lee ◽  
Dae Hyun Kim ◽  
Jei Kim
Keyword(s):  
Disease Progression ◽  
Moyamoya Disease ◽  
Hemodynamic Changes ◽  
Adult Moyamoya Disease

Assessment of the difference in posterior circulation involvement between pediatric and adult patients with moyamoya disease

2013 ◽  
Vol 119 (4) ◽  
pp. 961-965 ◽  
Author(s):  
Tomohito Hishikawa ◽  
Koji Tokunaga ◽  
Kenji Sugiu ◽  
Isao Date
Keyword(s):  
Moyamoya Disease ◽  
Pediatric Patients ◽  
Clinical Manifestations ◽  
Staging System ◽  
Posterior Circulation ◽  
Adult Patients ◽  
Mri Findings ◽  
Significant Difference ◽  
Advanced Stages ◽  
The Difference

Object There is no description of the change in the posterior cerebral artery (PCA) in the diagnostic criteria of moyamoya disease (MMD). However, PCAs are often involved in the clinical setting, and an understanding of the significance of PCA lesions is therefore of great importance when evaluating the disease progression and predicting prognosis. The aim of this study was to assess the difference in posterior circulation involvement in pediatric and adult patients with MMD. Methods The records of 120 consecutive patients with MMD were reviewed. The clinical manifestations at diagnosis were evaluated on the basis of symptoms and CT and MRI findings. The degree of steno-occlusive internal carotid artery (ICA) lesions and the existence of steno-occlusive PCA lesions were evaluated by observing a total of 240 ICAs and PCAs on angiography. Angiographic correlation between anterior and posterior circulation was assessed in pediatric and adult patients with MMD. Results Seventeen (26%) of 66 pediatric patients and 18 (33%) of 54 adult patients exhibited steno-occlusive PCA lesions. There was no significant difference in the prevalence of PCA lesions between pediatric and adult patients with MMD (p = 0.36). The prevalence of infarction in pediatric and adult patients with PCA involvement was significantly higher than that in pediatric and adult patients without PCA involvement (p = 0.0003 and p = 0.003, respectively). There was no significant difference in the distribution of infarction areas between pediatric and adult patients with PCA involvement (p = 0.62). On the basis of the staging system used, steno-occlusive lesions in ICAs ipsilateral to PCAs with lesions were in significantly advanced stages compared with lesions in ICAs ipsilateral to PCAs without lesions in both pediatric and adult cases (p < 0.0001 and p = 0.0008, respectively). Pediatric patients had less advanced steno-occlusive lesions in ICAs ipsilateral to PCAs with lesions compared with adults (p < 0.05). Conclusions The clinical significance of posterior circulation involvement in MMD was similar between pediatric and adult patients. The only significant difference was that less advanced ICA lesions could complicate posterior circulation involvement in pediatric patients.

scholarly journals Combined structural and diffusion tensor imaging detection of ischemic injury in moyamoya disease: relation to disease advancement and cerebral hypoperfusion

2020 ◽  
pp. 1-10
Author(s):  
Ken Kazumata ◽  
Kikutaro Tokairin ◽  
Masaki Ito ◽  
Haruto Uchino ◽  
Taku Sugiyama ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
Moyamoya Disease ◽  
Diffusion Tensor ◽  
Arterial Occlusion ◽  
Structural Mri ◽  
Neural Substrates ◽  
Cerebral Hypoperfusion ◽  
Advanced Stage ◽  
Advanced Stages ◽  
Adult Moyamoya Disease

OBJECTIVEThe microstructural integrity of gray and white matter is decreased in adult moyamoya disease, suggesting covert ischemic injury as a mechanism of cognitive dysfunction. Establishing a microstructural brain imaging marker is critical for monitoring cognitive outcomes following surgical interventions. The authors of the present study determined the pathophysiological basis of altered microstructural brain injury in relation to advanced arterial occlusion, cerebral hypoperfusion, and cognitive function.METHODSThe authors examined 58 patients without apparent brain lesions and 30 healthy controls by using structural MRI, as well as diffusion tensor imaging (DTI). Arterial occlusion in each hemisphere was classified as early or advanced stage based on MRA and posterior cerebral artery (PCA) involvement. Regional cerebral blood flow (rCBF) was measured with N-isopropyl-p-[123I]-iodoamphetamine SPECT. Furthermore, cognitive performance was examined using the Wechsler Adult Intelligence Scale, Third Edition and the Trail Making Test (TMT). Both voxel- and region of interest–based analyses were performed for groupwise comparisons, as well as correlation analysis, using parameters such as cognitive test scores; gray matter volume; fractional anisotropy (FA) of association fiber tracts, including the inferior frontooccipital fasciculus (IFOF) and superior longitudinal fasciculus (SLF); PCA involvement; and rCBF.RESULTSCompared to the early stages, advanced stages of arterial occlusion in the left hemisphere were associated with a lower Performance IQ (p = 0.031), decreased anterior cingulate volumes (p = 0.0001, uncorrected), and lower FA in the IFOF, cingulum, and forceps major (all p < 0.01, all uncorrected). There was no significant difference in rCBF between the early and the advanced stage. In patients with an advanced stage, PCA involvement was correlated with a significantly lower Full Scale IQ (p = 0.036), cingulate volume (p < 0.01, uncorrected), and FA of the left SLF (p = 0.0002, uncorrected) compared to those with an intact PCA. The rCBF was positively correlated with FA of the SLF, IFOF, and forceps major (r > 0.34, p < 0.05). Global gray matter volumes were moderately correlated with TMT part A (r = 0.40, p = 0.003). FA values in the left SLF were moderately associated with processing speed (r = 0.40, p = 0.002).CONCLUSIONSAlthough hemodynamic compensation may mask cerebral ischemia in advanced stages of adult moyamoya disease, the disease progression is detrimental to gray and white matter microstructure as well as cognition. In particular, additional PCA involvement in advanced disease stages may impair key neural substrates such as the cingulum and SLF. Thus, combined structural MRI and DTI are potentially useful for tracking the neural integrity of key neural substrates associated with cognitive function and detecting subtle anatomical changes associated with persistent ischemia, as well as disease progression.

Challenges in management of gastrointestinal cancers in pregnancies: A report of three cases

2021 ◽  
pp. 1753495X2098704
Author(s):  
Serene Thain ◽  
Jess McMicking ◽  
Julien de Naurois ◽  
Catherine Nelson-Piercy
Keyword(s):  
Disease Progression ◽  
Gastrointestinal Cancer ◽  
Gastrointestinal Cancers ◽  
Gastrointestinal Malignancies ◽  
Diagnosis And Management ◽  
Advanced Stages ◽  
Physiological Symptoms ◽  
The Impact ◽  
In Pregnancy ◽  
Diagnostic Investigations

Gastrointestinal cancer occurs in approximately 1 in 13,000 pregnancies, making up 4% of malignancies detected in pregnancy. It is a complex and challenging condition to diagnose and manage and is often only detected in its more advanced stages. This is partly due to symptoms of gastrointestinal cancer being incorrectly attributed to physiological symptoms of pregnancy, as well as concerns about the safety of diagnostic investigations in pregnancy, both of which may delay diagnosis and lead to disease progression. Challenges in management also arise from under-treatment in pregnancy due to concerns about the impact of surgery or chemotherapy on the pregnancy. We present here three cases of gastrointestinal cancer diagnosed in pregnancy in our centre and discuss the challenges and pitfalls one may encounter in the diagnosis and management of gastrointestinal malignancies in pregnancy.

The need for a revised staging system of metastatic (M) colorectal cancer (CRC): Evidence from a national perspective on survival following surgically treated (HPX) liver metastases

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 4099-4099
Author(s):  
E. Morris ◽  
J. Thomas ◽  
D. Forman ◽  
P. Quirke ◽  
B. Cottier ◽  
...  
Keyword(s):  
Lymph Node ◽  
Staging System ◽  
Population Based ◽  
Curative Intent ◽  
Kaplan Meier ◽  
Stage 4 ◽  
Presentation Methods ◽  
National Perspective ◽  
The Impact ◽  
Better Than

4099 Background: AJCC V.6 (2002) places all patients with MCRC beyond the lymph node basin of the primary tumor in a homogenous Stage 4. Patients with inoperable hepatic MCRC can be made operable with curative intent with chemotherapy yet remaining in Stage 4. Via the linkage of routine health datasets across England this population-based study sought to determine the impact of HPX for MCRC on stage-matched survival at initial presentation. Methods: All patients between 1998–2001 undergoing surgery for CRC in England were identified via the national-linked cancer registry HES dataset. All care episodes in the 3 years following initial colorectal surgery were examined to determine the frequency of subsequent HPX. Kaplan-Meier curves and log- rank tests were used to examine 5-year survival following HPX for MCRC compared to all Stage 3 and Stage 4 at presentation. Survival was calculated from the date of resection of each patient's primary colorectal tumor. Results: 68,307 individuals were identified as undergoing surgery for primary CRC over the study period. 20,298 were Stage 3 at presentation. 1,483 (2.2%) subsequently underwent HPX <3 years of their colorectal operation. 55 patients died within 30 days of HPX (mortality rate: 3.7%). Crude 5-year survival of patients who underwent HPX was 41.6% (95%CI 39.0–44.1%) from time of initial colectomy. This survival rate was significantly better than that for both Stage 3 (38.6% (95%CI 37.9%-39.2%, P<0.01) and 4 (6.1% 95%CI 5.3–6.9%, P<0.01) overall. Conclusions: 5-year survival following HPX for MCRC is better than that seen overall for all Stage 3 patients (with MCRC confined to the regional draining lymph node basin) following initial colectomy. Our data support the hypothesis that all MCRC that is potentially resectable with curative intent should be stratified within Stage 3, and Stage 4 should only contain those MCRC patients for whom surgery is not an option. If further evidence emerges to support this theory then a revision of the current staging system will be required. [Table: see text]

scholarly journals Treatment of HIV-associated facial lipoatrophy: impact on infection progression assessed by viral load and CD4 count

2013 ◽  
Vol 88 (4) ◽  
pp. 570-577 ◽  
Author(s):  
Flávia Machado Gonçalves Soares ◽  
Izelda Maria Carvalho Costa
Keyword(s):  
Viral Load ◽  
Disease Progression ◽  
Statistical Significance ◽  
Undetectable Viral Load ◽  
Fat Distribution ◽  
Cd4 Count ◽  
Before And After ◽  
Facial Lipoatrophy ◽  
A Prospective Study ◽  
The Impact

BACKGROUND: HIV/AIDS-Associated Lipodystrophy Syndrome includes changes in body fat distribution, with or without metabolic changes. The loss of fat from the face, called facial lipoatrophy, is one of the most stigmatizing signs of the syndrome.OBJECTIVES:To evaluate the effect of FL treatment using polymethylmethacrylate (PMMA) implants on disease progression, assessed by viral load and CD4 cell count.METHODS: This was a prospective study of 44 patients treated from July 2009 to December 2010. Male and female patients, aged over 18 years, with clinically detectable FL and who had never been treated were included in the study. PMMA implantation was done to fill atrophic areas. Laboratory tests were conducted to measure viral load and CD4 count before and after treatment.RESULTS: Of the 44 patients, 72.72% were male and 27.27% female, mean age of 44.38 years. Before treatment, 82% of patients had undetectable viral load, which increased to 88.6% after treatment, but without statistical significance (p = 0.67). CD4 count before treatment ranged from 209 to 1293, averaging 493.97. After treatment, the average increased to 548.61. The increase in CD4 count after treatment was statistically significant with p = 0.02.CONCLUSION: The treatment of FL with PMMA implants showed a statistically significant increase in CD4 count after treatment, revealing the impact of FL treatment on disease progression. Viral load before and after treatment did not vary significantly.

scholarly journals Natural History of NAFLD

2021 ◽  
Vol 10 (6) ◽  
pp. 1161
Author(s):  
Raluca Pais ◽  
Thomas Maurel
Keyword(s):  
Liver Disease ◽  
Natural History ◽  
Disease Progression ◽  
Specific Treatment ◽  
Alcoholic Fatty Liver ◽  
Individual Level ◽  
Close Relationship ◽  
History Of ◽  
The Individual ◽  
The Impact

The epidemiology and the current burden of chronic liver disease are changing globally, with non-alcoholic fatty liver disease (NAFLD) becoming the most frequent cause of liver disease in close relationship with the global epidemics of obesity, type 2 diabetes and metabolic syndrome. The clinical phenotypes of NAFLD are very heterogeneous in relationship with multiple pathways involved in the disease progression. In the absence of a specific treatment for non-alcoholic steatohepatitis (NASH), it is important to understand the natural history of the disease, to identify and to optimize the control of factors that are involved in disease progression. In this paper we propose a critical analysis of factors that are involved in the progression of the liver damage and the occurrence of extra-hepatic complications (cardiovascular diseases, extra hepatic cancer) in patients with NAFLD. We also briefly discuss the impact of the heterogeneity of the clinical phenotype of NAFLD on the clinical practice globally and at the individual level.

scholarly journals The impact of mental health on COVID 19 disease progression: Case report

2021 ◽  
pp. 102543
Author(s):  
Amine Bouabdallaoui ◽  
Salma Taouihar ◽  
Ghizlane El Aidouni ◽  
Mohamed Aabdi ◽  
Rajae Alkouh ◽  
...  
Keyword(s):  
Mental Health ◽  
Case Report ◽  
Disease Progression ◽  
The Impact
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