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Sample records for adult moyamoya patients

  1. Adult onset moyamoya disease: Institutional experience

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    Swati Dayanand Chinchure

    2011-01-01

    Full Text Available Moyamoya disease is a progressive steno-occlusive disease of bilateral carotid forks with the formation of fine collateral vascular network and is an angiographic diagnosis. We analyzed case records of 11 patients with "adult-onset moyamoya disease." Six patients presented with intracranial hemorrhage (intracerebral and/or intraventricular and 5 with focal ischemia. Angiography revealed bilateral Internal carotid artery involvement in 8 patients and unilateral involvement in 3. Posterior cerebral artery involvement was seen in 3 patients. Saccular aneurysm involving posterior circulation was seen in only 1 patient. Although rare, adult-onset moyamoya disease should be considered as one of the causes for intracerebral and intraventricular hemorrhage in adults.

  2. Stroke prevention by direct revascularization for patients with adult-onset moyamoya disease presenting with ischemia.

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    Kim, Tackeun; Oh, Chang Wan; Kwon, O-Ki; Hwang, Gyojun; Kim, Jeong Eun; Kang, Hyun-Seung; Cho, Won-Sang; Bang, Jae Seung

    2016-06-01

    OBJECT Moyamoya disease (MMD) is a progressive disease that can cause recurrent stroke. The authors undertook this retrospective case-control study with a large sample size in an attempt to assess the efficacy of direct or combined revascularization surgery for ischemia in adults with MMD. METHODS The authors investigated cases involving patients with moyamoya disease presenting with ischemia who visited Seoul National University Bundang Hospital and Seoul National University Hospital between 2000 and 2014. Among 441 eligible patients, 301 underwent revascularization surgery and 140 were treated conservatively. Variables evaluated included age at diagnosis, sex, surgical record, Suzuki stage, and occurrence of stroke. Patients were stratified into 2 groups based on whether or not they had undergone revascularization surgery. Actuarial 1-, 5-, and 10-year stroke rates were calculated using the life table method. Risk factor analysis for 5-year stroke occurrence was conducted with multivariate regression. RESULTS Of the 441 patients, 301 had been surgically treated (revascularization group) and 140 had not (control group). The mean follow-up durations were 45 and 77 months, respectively. The actuarial 10-year cumulative incidence rate for any kind of stroke was significantly lower in the revascularization group (9.4%) than in the control group (19.6%) (p = 0.041); the relative risk reduction (RRR) was also superior (52.0%) in the revascularization group, and the number needed to treat was 10. The 10-year rate of ischemic stroke was greater (13.3%) in the control group than in the revascularization group (3.9%) (p = 0.019). The RRR for ischemic stroke in the revascularization group was 70.7%, and the number needed to treat was 11. However, the actuarial 1- and 5-year rates of ischemic stroke did not significantly differently between the groups. Overall, revascularization surgery was shown to be an independent protective factor, as revealed by multivariate analysis

  3. Usefulness of CT perfusion imaging in adult moyamoya disease

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    Choi, Bo Bae; Kim, Young Joo; Song, Ha Hun; Kim, Ki Tae [College of Medicine, The Catholic University of Korea, Uijongbu (Korea, Republic of)

    2004-12-01

    The purpose of this study was to evaluate the role of perfusion CT in adult moyamoya disease. The study population consisted of 13 adult moyamoya patients (10 women and 3 men, mean age: 40.4 years) and 11 age-matched normal controls (5 men and 6 women, mean age: 43 years). We retrospectively assessed the perfusion CT scan both visually and by a quantitative regional analysis, and we assessed the relationship between the perfusion CT scan findings and the angiographic findings. The mean relative cerebral blood volume (rCBV) values in moyamoya patients were 8.0% for the MCA area, 6.4% for the PCA area, and 7.7% for the basal ganglia. The rCBV values in the patients were higher than those in the control group with statistical significance (p<0.0001). The time to peak enhancement (TTP) values of the MCA area and the basal ganglia were delayed more than those in the controls; this was statistically significant (p<0.05). Moderate correlation was found between the rCBV in the basal ganglia area and angiographic stage of the basal moyamoya vessels. Perfusion CT demonstrates a statisticaIly significant increase in rCBV in the MCA, PCA and basal ganglia areas and the TTP in the MCA and basal ganglia areas in patients with moyamoya disease. The visual brain perfusion patterns correIate with the extent and severity of the basal moyamoya vessels.

  4. Moyamoya Disease with Coexistent Hypertriglyceridemia in Pediatric Patient

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    Sahni, Deepank; Boucher-Berry, Claudia

    2016-01-01

    Moyamoya disease is a rare chronic and progressive cerebrovascular disease of the arteries of the circle of Willis that can affect children and adults. It has been associated with multiple diseases, including immunologic, like Graves' disease, diabetes mellitus, and SLE. Hyperlipidemia has been recognized in patients with Moyamoya disease with an incidence of 27–37%. However, no case in pediatric patients has been reported of the coexistence of Moyamoya disease and hyperlipidemia. Here we present a case of a 9-year-old female diagnosed with Moyamoya disease after a stroke with incidental finding of familial hypercholesterolemia. This finding will make our patient a very unique case, since there has not been any reporting of Moyamoya disease and hypercholesterolemia association. PMID:27843655

  5. Moyamoya Disease with Coexistent Hypertriglyceridemia in Pediatric Patient

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    Jacqueline Chan

    2016-01-01

    Full Text Available Moyamoya disease is a rare chronic and progressive cerebrovascular disease of the arteries of the circle of Willis that can affect children and adults. It has been associated with multiple diseases, including immunologic, like Graves’ disease, diabetes mellitus, and SLE. Hyperlipidemia has been recognized in patients with Moyamoya disease with an incidence of 27–37%. However, no case in pediatric patients has been reported of the coexistence of Moyamoya disease and hyperlipidemia. Here we present a case of a 9-year-old female diagnosed with Moyamoya disease after a stroke with incidental finding of familial hypercholesterolemia. This finding will make our patient a very unique case, since there has not been any reporting of Moyamoya disease and hypercholesterolemia association.

  6. Moyamoya disease: difference of MR findings between children and adults

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    Kim, Jong Deok; Seo, Chang Hae [College of Medicine, Inje University, Busan Paik Hospital, Busan (Korea, Republic of)

    1995-10-15

    To evaluate whether there are any differences in MR findings between the childhood and the adult moyamoya disease. We compared the brain MR findings in 22 children (13 boys and 9 girls, 2-18 years of age) who had moyamoya disease with 15 adult patients (7 men and 8 women, 19-55 years of age). The MR findings were classified as parenchymal-(infarctions and intracranial hemorrhages) and vascular abnormalities (intracranial vascular patency and moyamoya vessels). The difference in each of these MR findings was analyzed using Chi-square test and Fisher's exact test (two-tailed). Out of 22 children, two children with normal MR finding were excluded from the statistical analysis. Moyamoya diseases were diagnosed angiographically in all adult patients. In children, they were diagnosed by MR imaging, MR angiography(6), and/or conventional cerebral angiography(18). In children, cerebral infarctions were observed in 20 of 22 patients (91%) (cortex 86%, periventricular white matter/centrum semiovale 32%, basal ganglia 10%). In two patients, there was no parenchymal abnormality. Intracranial hemorrhages were not demonstrated in any patients. In adults, intracranial hemorrhages (intracerebral hematoma, intraventricular hemorrhage, alone or combined) were demonstrated in 10 of 15 patients(67%). Cerebral infarctions with or without intracranial hemorrhage were detected in 10 of 15 patients(67%) (cortex 40%, periventricular white matter/centrum semiovale 53%, basal ganglia 20%). The difference in parenchymal abnormalities between the childhood and the adult moyamoya disease was statistically significant ({rho} = 0.000164). There was no significant difference between the two groups with regard to the occlusive changes of the internal carotid and middle cerebral arteries or to moyamoya vessels ({rho} > 0.01). This study could prove the fact that the principal clinical symptoms in the childhood moyamoya disease were due to cerebral infarction and those in the adult cases were

  7. Cognitive Dysfunction Survey of the Japanese Patients with Moyamoya Disease (COSMO-JAPAN Study): study protocol.

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    Takagi, Yasushi; Miyamoto, Susumu

    2015-01-01

    Moyamoya disease is a cerebrovascular occlusive disease characterized by progressive stenosis or by occlusion at the terminal portion of the bilateral internal carotid arteries. The unusual vascular network (moyamoya vessels) at the base of the brain with this disease as collateral channels is developed in this disease. Social independence because of cognitive impairment has recently been recognized as an important unsolved social issue with adult moyamoya disease. The patients with cognitive impairment have difficulty in proving their status because the standard neuroradiological and neuropsychological methods to define cognitive impairment with moyamoya disease are not determined. These patients with cognitive impairment should be supported by social welfare as psychologically handicapped persons. Thus Cognitive Dysfunction Survey of the Japanese Patients with Moyamoya Disease (COSMO-JAPAN study) is planned. In this study, we want to establish a standard finding of the cognitive impairment in patients with moyamoya disease.

  8. Adult hemorrhagic moyamoya disease: The paradoxical role of combined revascularization

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    Vikas C Jha

    2012-01-01

    Full Text Available Background: Moyamoya disease (MMD in adults often manifests with hemorrhage. Combined revascularization in hemorrhagic MMD is controversial as improvement in hemodynamics may be offset by hypervascularity-induced rebleeding. Aim: Long-term outcome assessment of adult patients from non-endemic region with hemorrhagic MMD undergoing combined revascularization. Setting: Tertiary care, academic setting. Materials and Methods: Both Suzuki′s internal carotid artery (ICA grade (1-6 and Mugikura′s posterior cerebral artery (PCA grade (1-4 were applied to 11 patients with hemorrhagic MMD (mean symptom duration 6.11±6.46 months undergoing direct [superficial temporal artery-middle cerebral artery (STA-MCA bypass] and indirect encephalomyosynangiosis (EMSA revascularization. They were clinically graded at follow-up (F/U as: excellent, preoperative symptoms resolved; good, preoperative symptoms resolved, neurological deficits remained; fair, symptom frequency decreased; and poor, symptoms unchanged/worsened. Digital subtraction angiogram/magnetic resonance angiography (DSA/MRA assessed the patency of anastomosis and cerebral hemodynamics as: 0 = non-patent; 1 = patent bypass, STA perfused recipient artery, moyamoya vessels unchanged; and, 2 = patent bypass, STA widely perfused MCA territory, moyamoya vessels diminished. An acetazolamide stimulated single photon emission computed tomography (SPECT study evaluated regional cerebral vascular reserve (RCVR. Results: Angiographic ICA grades were 5 (n=2, 4 (n=2, 3 (n=4, and 2 (n=3, and PCA grades were 1 (n=8 and 3 (n=3. At F/U (mean: 36.55±21.6 months, clinical recovery was excellent in eight and fair in one. Two patients developed delayed re-hemorrhage (in one at a site remote from previous bleed. F/U DSA/MRA (n=6 showed a good caliber, patent anastomosis with collaterals in five patients, and a narrow caliber anastomotic vessel in one patient. SPECT (n=6 revealed improved perfusion in two and normal

  9. [Hemorrhagic Adult Unilateral Moyamoya Disease with Multiple Unruptured Intracranial Aneurysms: A Case Report].

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    Ozaki, Saya; Inoue, Akihiro; Miyazaki, Hajime; Onoue, Shinji; Ichikawa, Haruhisa; Fukumoto, Shinya; Iwata, Shinji; Kohno, Kanehisa

    2016-02-01

    Adult unilateral moyamoya disease with intracranial aneurysm is frequently reported in the literature, but there is much variation in its treatment. In this case report, we describe the time course and treatment regimen of a patient with moyamoya disease and review the literature regarding moyamoya disease with intracranial aneurysm. A 64-year-old man had untreated intracranial aneurysm and unilateral moyamoya disease for 10 years. He presented with sudden-onset right hemiparesis and aphasia due to a subcortical hemorrhage. He was admitted to the local neurosurgical unit, and upon resolution of symptoms, he was admitted to our hospital. A cerebral angiogram revealed the champagne bottleneck sign of the left carotid artery and obliteration of the top of the left intracranial carotid artery with a moyamoya phenomenon. Two unruptured intracranial aneurysms were identified in the anterior communicating artery(Acom A) and the right intracranial carotid artery(C3). We performed superficial temporal artery-middle cerebral artery anastomosis followed by aneurysmal neck clipping of the Acom A aneurysm. Postoperative imaging showed no new ischemic damage and improved cerebral blood flow. Although the patient experienced temporal worsening of aphasia, his function recovered a few months later and he was able to resume his normal daily life activities. The combination of direct bypass surgery and aneurysmal neck clipping might be a therapeutic option for hemorrhagic unilateral moyamoya disease with unruptured intracranial aneurysm.

  10. Computed tomographic evaluation in 8 patient of cerebrovascular moyamoya disease

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    Ko, Young Tae; Lee, Jung Suk; Suh, Soo Jhi; Kim, Soon Yong [School of Medicine, Kyung-Hee University Hospital, Seoul (Korea, Republic of)

    1980-12-15

    CT findings in eight cerebrovascular moyamoya disease were compared with carotid angiographic findings and the results were as follows: 1. The patient's age was ranged from 2 to 49 years. Of eight patients, five were less than 17 years old and the rests were two 23 years and one 49 years of age. 2. Except one 6 years old boy, all of them were female patients. 3. In precontrast CT scan of 8 cases, 6 cases of cortical atrophy, 6 cases of ventricular dilatation and 5 cases of low density area were observed. In postcontrast study the children group shows abnormal contrast enhancement in 3 out of 5 patients but all adult reveal no contrast enhancement. 4. The isodense area in CT were thought to represent good collateral manifested by angiographic moyamoya vascular network and leptomeningeal anastomosis while the low density area in CT appears to poor colleaterals manifested by transdural anastomosis. 5. To evaluate the possibility of this disease, carotid angiography should be performed to the hemiplgic child who shows multiple low density area and abnormal enhancement in CT scan. 6. CT is not only reliable for evaluation of ventricular hemorrhage but also brain damage in patients with moyamoya disease.

  11. The Efficacy of Single Barrel Superficial Temporal Artery-middle Cerebral Artery Bypass in Treatment of Adult Patients with Ischemic-type Moyamoya Disease

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    Ha, Mahnjeong; Choi, Chang Hwa; Lee, Jae Il; Cha, Seung Heon; Lee, Sang Weon

    2016-01-01

    Objective So far, there is no study answering the question of which type of surgical technique is practically the most useful in the treatment of adult patients with ischemic type moyamoya disease (MMD). We evaluated the efficacy of single barrel superficial temporal artery (STA)-middle cerebral artery (MCA) bypass in the treatment of adult patients with ischemic type MMD by retrospectively collecting clinical and radiological data. Materials and Methods A retrospective review identified 31 adult patients who underwent 43 single barrel STA-MCA bypass procedures performed for treatment of ischemic-type MMD between 2006 and 2014. The male to female ratio was 17:14 and the mean age was 41 years (range, 21-65 years). Peri-operative complications, angiographic and clinical outcomes were analyzed retrospectively. Results The permanent neurological morbidity and mortality rates were 2.3% and 0%, respectively. During the observation period of a mean of 35 months (range, 12-73 months), 29 patients (93.5%) had no further cerebrovascular events and transient ischemic attack occurred in two patients (6.5%), resulting in an annual stroke risk of 2.2%. Follow-up computed tomography perfusion (CTP) (mean, 18.4 months after surgery) documented improved cerebral hemodynamics in the revascularized hemispheres (p < 0.001). Post-operative patency was clearly verified in 38 bypasses (88.4%) of 43 bypasses on follow-up imaging (mean, 16.5 months). Conclusion Our results suggest that single barrel STA-MCA bypass with wide dural opening is safe and durable method of cerebral revascularization in adult patients with ischemic type MMD and can be considered as a potential treatment option for adult patients with ischemic type MMD. PMID:27847768

  12. An update on the diagnosis and treatment of adult Moyamoya disease taking into consideration controversial issues.

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    Kim, Jeong Eun; Jeon, Jin Sue

    2014-05-01

    Moyamoya disease (MMD) is characterized by a chronic progressive steno-occlusive disease at the distal portion of the internal carotid artery (ICA) with abnormal Moyamoya vessel (MMV) development without associated diseases. Recent advances in radiologic tests have increased the number of MMD patients. Beyond detection improvement by magnetic resonance angiography (MRA) or cerebral angiography, predicting factors for disease severity, future hemorrhage, clinical outcome, post-op complications, and technical advances in assessing cerebral hemodynamics have been increasingly reported. Although treatment of pediatric MMD is well established, controversy remains over the treatment in adult patients. In particular, there are debates over the disease entity of adult MMD, contralateral progression in adult unilateral MMD, treatment strategy for asymptomatic adult MMD, and the association of MMD with thyroid disease. The purpose of this review is to provide an update on the diagnosis and treatment of adult MMD while addressing controversial issues.

  13. Moyamoya disease: report of three cases in Brazilian patients

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    FRANCO CLÉLIA MARIA RIBEIRO

    1999-01-01

    Full Text Available Moyamoya disease (MMD is a chronic occlusive cerebrovascular disease of unknown etiology reported mainly in the Japanese. Most cases occur in children. The disease is rare in non-Oriental adults manifesting itself mostly as intracerebral hemorrhages. We describe MMD in 2 non-Oriental young adults and one adolescent that developed cerebral infarctions. The adults were medicated with aspirin and no medication was given to the adolescent. All patients did not deteriorate in a follow-up period from 1 to 4 years. Although rare, MMD is an important cause of stroke in young individuals and may well be underreported: only 18 patients have been reported till 1997 in Brazil. Neurologists should include MMD in differential diagnosis of ischemic and hemorrhagic strokes in young adults.

  14. [Somatosensory focal seizures as an onset form in adult Moyamoya syndrome].

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    Molina, C; Alvarez Sabín, J; Bosch, J; Codina Puiggrós, A

    1995-01-01

    Moya-Moya disease is a chronic infrequent vasculopathy. Occasionally such abnormalities are found in association with one of many conditions, in these cases the angiographic abnormality should be termed Moya-Moya syndrome rather than Moya-Moya disease. Although in children the usual manifestations are ischemic events and seizures. This clinical presentation is infrequent in adults. We present a 42-years-old man with a 1-month history of recurrent right sided partial somatosensorial seizures, later he presented a left parietal infarction, the angiographic findings were compatible with moyamoya syndrome.

  15. Moyamoya disease in a patient with hereditary spherocytosis

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    Holz, A.; Woldenberg, R.; Miller, D.; Kalina, P.; Black, K.; Lane, E. [Department of Radiology, North Shore University Hospital, New York University School of Medicine, 300 Community Drive, Manhasset, NY 11030 (United States)

    1998-02-01

    Moyamoya disease (MMD) is a rare cerebral vasculopathy characterized by occlusion of the supraclinoid portion of the internal carotid artery and proximal portions of the anterior and middle cerebral arteries. Patients develop an extensive collateral network of parenchymal, transdural and leptomeningeal vessels to supply the compromised brain. These collateral channels, also known as ``moyamoya vessels,`` may be seen in a number of disorders which lead to intracranial vascular occlusion. We report a case of MMD in a child with hereditary spherocytosis. (orig.) With 4 figs., 5 refs.

  16. Deep hypothermic circulatory arrest for hemiarch replacement in a pediatric patient with moyamoya disease.

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    Kuwajima, Ken; Yoshitani, Kenji; Kato, Shinya; Miyazaki, Atsushi; Kamei, Masataka; Ohnishi, Yoshihiko

    2014-08-01

    Moyamoya disease is a chronic cerebrovascular occlusive disease, occurring predominantly in young populations, that causes cerebral ischemia and hemorrhage. Patients with moyamoya disease are at high risk of neurological complications during cardiac surgery because of perioperative hemodynamic changes. However, there is no established evidence on temperature management during cardiopulmonary bypass. Previous reports described normothermia or mild to moderate hypothermia during cardiopulmonary bypass in patients with moyamoya disease; however, surgical conditions, such as not having enough space to clamp the aorta or a clean surgical field, sometimes force us to use deep hypothermic circuratory arrest. We report a successful case of a pediatric patient with moyamoya disease who underwent deep hypothermic circulatory arrest (18 °C) for hemiarch replacement without neurological complications. Deep hypothermia may be an alternative technique for achieving cerebral protection in the context of moyamoya disease.

  17. Working memory and attention impairment in adult patients with moyamoya disease%成年人烟雾病患者工作记忆及注意力损害的研究

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    方凌凌; 王嵘; 王斌; 万伟庆

    2015-01-01

    目的 探讨成年人烟雾病患者的工作记忆和注意力损害.方法 纳入首都医科大学附属北京天坛医院神经外科2014年4月至9月收住的成人烟雾病患者48例,同时纳入年龄、性别、教育程度、社会背景等相匹配的无脑部疾病48例志愿者为对照组.运用N-back、持续注意力反应测试(SART)评价受试者的视空间工作记忆及持续性注意力.结果 (1)N-back试验:烟雾病组和对照组比较,0-back试验中准确率分别为0.89 ±0.18、0.98 ±0.04,平均反应时间(RT)分别为(572±123)、(466±95) ms;1-back试验中准确率分别为0.55 ±0.23、0.72±0.20,RT分别为(763±232)、(750±202) ms;2-back试验中准确率分别为0.36±0.15、0.53±0.22,RT分别为(714±312)、(865±282)ms;除1-back的RT外,两组的其他指标比较,差异均有统计学意义(P <0.05);(2) SART试验:烟雾病组和对照组比较,击中率分别是0.88±0.19、0.98±0.03,RT分别为(458±112)、(382 ±91)ms,正确拒斥率分别为0.64±0.23、0.76±0.25,差异均有统计学意义(P<0.05).结论 成人烟雾病患者存在视空间工作记忆及持续性注意力的损害.%Objective To investigate the working memory and attention impairment in adult patients with moyamoya disease.Methods Forty-eight adult patients with moyamoya disease admitted to the Department of Neurosurgery,Beijing Tiantan Hospital,Capital Medical University from April 1,2014 to September 15,2014 were enrolled,and the age,sex,education,social background matched 48 normal healthy controls were also enrolled at the same time.N-back and sustaining attention reaction test (SART) were used to assess the visual-spatial working memory and sustained attention of the participants.Results (1) The N-back test:the moyamoya disease group compared with the control group,the accuracy rates in the 0-back test were 0.89 ±0.18 and 0.98 ±0.04 respectively; the mean response times (RT) were 572 ± 123 and 466 ±95 ms respectively; the accuracy

  18. A Recent Update of Clinical and Research Topics Concerning Adult Moyamoya Disease

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    Jeon, Jin Pyeong

    2016-01-01

    A better understanding of moyamoya disease (MMD), such as natural clinical course, surgical outcomes and research, has been obtained. This review article focuses on an giving an update for adult MMD in the Korean population. In this paper, we mainly discuss the results of our domestic investigations including meta-analysis, and related subjects from other countries. PMID:27847564

  19. Onyx embolization of a ruptured aneurysm in a patient with moyamoya disease.

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    Daou, Badih; Chalouhi, Nohra; Tjoumakaris, Stavropoula; Rosenwasser, Robert H; Jabbour, Pascal

    2015-10-01

    We report a woman who presented with an intraparenchymal hemorrhage. Her cerebral angiogram showed a middle cerebral artery (MCA) M1 occlusion with multiple collaterals supplying the distal MCA territory, compatible with moyamoya disease. Also, an associated 8 mm dysplastic distal aneurysm fed by a left-sided P2 perforator was seen, collateral from the posterior cerebral artery. The aneurysm was successfully occluded with Onyx (ev3 Endovascular, Plymouth, MN, USA) embolization. The woman had an uneventful postoperative course. Aneurysm formation in patients with moyamoya disease represents a major hemorrhagic risk. Several treatment strategies exist including endovascular and surgical approaches. Patients with moyamoya disease who present with aneurysmal intracerebral hemorrhage should be treated to prevent rebleeding. Onyx embolization can be an effective treatment of aneurysms that are associated with moyamoya disease and would otherwise be difficult to treat surgically.

  20. Performance of Multi-mode MRI for Adult Patients with Ischemic Moyamoya Disease%成人型缺血性烟雾病综合征的多模式磁共振成像表现

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    李丽艳; 周顺科; 周兆德; 朱道升; 姜永录; 崔文涛

    2014-01-01

    目的:分析成人型缺血性烟雾病综合征的多模式磁共振成像( MRI)表现及其诊断价值。方法收集2012年3月—2014年2月吉林市人民医院诊治的10例成人型缺血性烟雾病综合征患者,回顾分析其MRI平扫、动脉自旋标记法(ASL)脑灌注成像(PWI)、磁敏感成像(SWI)、三维时间飞跃法(3D - TOF)磁共振血管成像(MRA)、匙孔技术四维增强MRA(4D-TRAK-MRA)、MRI增强(CE-MRI)结果。结果 MRI平扫显示双侧大脑前动脉( ACA)、大脑中动脉( MCA)的流空信号消失,基底核区出现烟雾状血管流空信号影以及柔脑膜高信号征;ASL-PWI显示ACA、MCA供血区脑血流量( CBF)减低;SWI显示梗死区缺乏小静脉显影,代偿区柔脑膜周围有多发增粗的小静脉;3D-TOF-MRA直观显示双侧颈内动脉(ICA)末端和ACA、MCA起始端的狭窄或闭塞以及增粗延长的大脑后动脉和脑底部烟雾血管;4D-TRAK-MRA能动态全景显示头颈部血运情况和柔脑膜的侧支循环,对烟雾病综合征诊断分期准确率达90%(9/10)。结论多模式MRI不但可以反映脑实质和颅内血管的情况,而且能提供解剖形态学和功能学两方面的特征,可作为烟雾病综合征筛查、诊断及分期的一种有效的影像学无创检查方法。%Objective To investigate the performance of multi-mode MRI for adult patients with moyamoya disease ( MMD)and its diagnostic value. Methods 10 patients with MMD admitted to Jilin City People's Hospital from March 2012 to February 2014 were selected. The results of MRI plain scan,ASL-PWI,SWI,3D -TOF-MRA,4D -TRAK -MRA and MRI enhanced scan(CE-MRI). Results MRI plain scan showed that flow-void signs of anterior cerebral artery(ACA)and middle cerebral artery( MCA)disappeared,and flow-void signs of moyamoya vessels and leptomeninges high signals appeared in basal ganglia;ASL-PWI showed that the cerebral blood flow( CBF)was reduced

  1. Transient Intraoperative Central Diabetes Insipidus in Moyamoya Patients Undergoing Revascularization Surgery: A Mere Coincidence?

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    Hong, Joe C; Ramos, Emilio; Copeland, Curtis C; Ziv, Keren

    2016-04-15

    We present 2 patients with Moyamoya disease undergoing revascularization surgery who developed transient intraoperative central diabetes insipidus with spontaneous resolution in the immediate postoperative period. We speculate that patients with Moyamoya disease may be predisposed to a transient acute-on-chronic insult to the arginine vasopressin-producing portion of their hypothalamus mediated by anesthetic agents. We describe our management, discuss pertinent literature, and offer possible mechanisms of this transient insult. We hope to improve patient safety by raising awareness of this potentially catastrophic complication.

  2. Moyamoya in Hispanics: not only in Japanese

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    Sarmad Said

    2014-06-01

    Full Text Available Moyamoya disease was first described in 1957 as hypoplasia of the bilateral internal carotid arteries, the characteristic appearance of the associated network of abnormally dilated collateral vessels on angiography was later likened to something hazy, like a puff of cigarette smoke, which, in Japanese, is moyamoya. This paper describes two cases of moyamoya presentations, including moyamoya disease and moyamoya syndrome. Moyamoya may rarely occur in North American Hispanic patients. The presentation can vary significantly and ranges bwtween fulminant outcome and prolonged survival. Awareness about moyamoya and its different presentations may be beneficial for the patients and can improve the outcome.

  3. Moyamoya syndrome associated with neurofibromatosis type I in a pediatric patient

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    Luiz Guilherme Darrigo Júnior

    Full Text Available CONTEXT: Neurofibromatosis type 1 (NF-1 is the most prevalent autosomal dominant genetic disorder among humans. Moyamoya disease is a cerebral vasculopathy that is only rarely observed in association with NF-1, particularly in the pediatric age range. The present study reports an occurrence of this association in an infant. CASE REPORT: An eight-month-old female presented convulsive seizures with clonic movements. The patient suffered an ischemic stroke with hemiparesis. Magnetic resonance imaging revealed radiological findings compatible with moyamoya disease. The diagnosis of NF-1 was made at the age of 20 months. CONCLUSION: Despite the rarity of this association in childhood, children with focal neurological symptoms and a diagnosis of NF-1 deserve to be investigated for moyamoya syndrome.

  4. An institutional experience of 26 patients with Moyamoya disease: A study from Northwest India

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    Trilochan Srivastava

    2014-01-01

    Full Text Available Aim: Moyamoya disease (MMD is a slowly progressive bilateral stenocclusive process of the distal internal carotid and proximal portions of the anterior and middle cerebral arteries and the formation of an abnormal vascular network at the base of the brain. The purpose of this retrospective study was to identify clinical features, salient features, radiological features and yield of diagnostic cerebral angiography in MMD. Materials and Methods: We analyzed the records of 26 patients with MMD evaluated and treated at our institute from August 2010 until March 2013. Diagnosis of MMD was made on the basis of features of angiographic findings. Cerebral angiography showed typically fine network of vessels at the base of the brain with puff of smoke appearance suggestive of MMD. CT angiography (CTA was done in 25 (96.15% patients where as Digital substraction angiography (DSA was done in 18 (69.23% patients. Results: Out of the 26 patients 13 were in the pediatric age group and 13 were adults. At presentation 14 patients had infarcts and 10 patients had hemorrhages. Among the hemorrhagic group 20% had isolated intracerebral hemorrhage (ICH, 50% patients had ICH with intraventricular extension (IVE and 30% patients had primary intraventricular hemorrhage (PIVH. 50 % of the patients had involvement of the posterior circulation. Conclusion: Posterior circulation involvement is frequent in MMD. Though parenchymal bleed with/without intraventricular extension is the usual presentation of hemorrhagic MMD, isolated intraventricular hemorrhage could also be the mode of presentation.

  5. The Application of CTA in the Diagnosis of Moyamoya Disease of Adults%CTA在成人烟雾病诊断中的应用

    Institute of Scientific and Technical Information of China (English)

    吴云军

    2015-01-01

    目的:探讨CTA在成人烟雾病诊断中的应用价值。方法回顾性分析17例烟雾病患者的临床及影像资料。患者均行脑CTA检查,行VR、MPR、MIP、CPR。结果 CTA均能清晰显示发生狭窄、闭塞的颈内动脉、后循环、脑底异常血管网、侧支血管情况。结论 CTA可以清晰显示闭塞及狭窄血管、颅底异常血管网,为诊断烟雾病提供了重要的参考依据。%Objective To study the applied value of CTA in the diagnosis of moyamoya disease of adults. Methods Analysed the clinical and imaging data of 17 patients with moyamoya disease retrospectively. The patients received the cerebral CTA examination, VR, MPR, MIP, CPR. Results CTA can clear display stenosis, occlusion of internal carotid artery, posterior circulation abnormal blood vessels, brain bottom nets, collateral vessels. Conclusion CTA can clear display block and narrow of the blood vessels, the base of abnormal vascular network, provides an important reference for the diagnosis of moyamoya disease.

  6. RNF213 Rare Variants in Slovakian and Czech Moyamoya Disease Patients

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    Kyselová, Kateřina; Viszlayová, Daša; Morimoto, Takaaki; Roubec, Martin; Školoudík, David; Petrovičová, Andrea; Juskanič, Dominik; Strauss, Jozef; Halaj, Marián; Kurray, Peter; Hranai, Marián; Harada, Kouji H.; Inoue, Sumiko; Yoshida, Yukako; Habu, Toshiyuki; Herzig, Roman; Youssefian, Shohab; Koizumi, Akio

    2016-01-01

    RNF213/Mysterin has been identified as a susceptibility gene for moyamoya disease, a cerebrovascular disease characterized by occlusive lesions in the circle of Willis. The p.R4810K (rs112735431) variant is a founder polymorphism that is strongly associated with moyamoya disease in East Asia. Many non-p.R4810K rare variants of RNF213 have been identified in white moyamoya disease patients, although the ethnic mutations have not been investigated in this population. In the present study, we screened for RNF213 variants in 19 Slovakian and Czech moyamoya disease patients. A total of 69 RNF213 coding exons were directly sequenced in 18 probands and one relative who suffered from moyamoya disease in Slovakia and the Czech Republic. We previously reported one proband harboring RNF213 p.D4013N. Results from the present study identified four rare variants other than p.D4013N (p.R4019C, p.E4042K, p.V4146A, and p.W4677L) in four of the patients. P.V4146A was determined to be a novel de novo mutation, and p.R4019C and p.E4042K were identified as double mutations inherited on the same allele. P.W4677L, found in two moyamoya disease patients and an unaffected subject in the same pedigree, was a rare single nucleotide polymorphism. Functional analysis showed that RNF213 p.D4013N, p.R4019C and p.V4146A-transfected human umbilical vein endothelial cells displayed significant lowered migration, and RNF213 p.V4146A significantly reduced tube formation, indicating that these are disease-causing mutations. Results from the present study identified RNF213 rare variants in 22.2% (4/18 probands) of Slovakian and Czech moyamoya disease patients, confirming that RNF213 may also be a major causative gene in a relative large population of white patients. PMID:27736983

  7. Cerebral hemodynamics and metabolism in patients with moyamoya disease not demonstrating either cerebral infarct or hemorrhage on MRI

    Energy Technology Data Exchange (ETDEWEB)

    Kuwabara, Yasuo; Ichiya, Yuichi; Sasaki, Masayuki; Akashi, Yuko; Yoshida, Tsuyoshi; Fukumura, Toshimitsu; Masuda, Kouji; Matsushima, Toshio; Fukui, Masashi [Kyushu Univ., Fukuoka (Japan). Faculty of Medicine

    1995-12-01

    We evaluated the cerebral hemodynamics and metabolism in moyamoya patients who did not demonstrate either cerebral infarct or hemorrhage on MRI. The subjects consisted of 5 patients with moyamoya disease (4 females and one male, aged from 15 to 40 ears). The CBF, OEF and CMRO{sub 2} of the moyamoya patients did not differ from those of the normal control subjects. The CBV did increase significantly in the cerebral cortices and striatum, but not in the cerebellum. The TT was also significantly prolonged in the frontal and parietal regions. The cerebrovascular CO{sub 2} response was markedly impaired in the frontal, temporal and parietal cortices. However, it was relatively preserved in the occipital cortex, thalamus and cerebellum. Thus, the cerebral hemodynamic reserve capacity decreased even in the moyamoya patients not demonstrating either cerebral infarct or hemorrhage on MRI, and it should be considered in the management of these patients. (author).

  8. Unilateral sudden hearing loss: a rare symptom of Moyamoya disease.

    Science.gov (United States)

    Gül, Fatih; Berçin, Sami; Müderris, Togay; Yalçıner, Gökhan; Ünal, Özkan; Kırış, Muzaffer

    2016-01-01

    A 38-year-old female patient experienced a sudden onset of unilateral sensorineural hearing loss due to Moyamoya disease. A detailed summary of audiological and neurological findings indicated that the sudden hearing loss might be due to Moyamoya disease resulting in occlusion of posterior and middle cerebral arteries. Intravenous prednisolone and trimetazidine dihydrochloride may improve hearing thresholds and speech understanding. To our knowledge, this is the first article in the literature reporting a case of sudden hearing loss as the first manifestation of Moyamoya disease in a young adult.

  9. Moyamoya disease:epidemiology,etiology,diagnosis and management

    Institute of Scientific and Technical Information of China (English)

    Zheng Huang; Jizong Zhao

    2014-01-01

    Moyamoya disease as an uncommon cerebrovascular disease has been known for more than 40 years.It is characterized by progressive stenosis of internal carotid artery and its main branches.There is compensatory development of col-lateral vessels,which are termed moyamoya vessels.Ischemic and hemorrhagic stroke can occur in both pediatric and adult patients.Surgical revascularization can improve cerebral hemodynamics and prevent further stroke.In this review,we discuss the epidemiology,etiology,clinical features and surgical treatment of moyamoya disease.

  10. Anestesia em paciente portadora de doença de moyamoya: relato de caso Anestesia en paciente portadora de enfermedad de moyamoya: relato de caso Anesthesia in patient with moyamoya disease: case report

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    Adriano Bechara de Souza Hobaika

    2005-06-01

    , la paciente fue mantenida en normocapnia y normotermia. La extubación fue realizada y la paciente transferida a la sala de recuperación pos-anestésica sin complicaciones. CONCLUSIONES: Este artículo presenta los cuidados anestésicos dados a una paciente portadora de la enfermedad de moyamoya.BACKGROUND AND OBJECTIVES: Moyamoya disease is an uncommon progressive cerebral vasculopathy, more frequently diagnosed among Asian individuals, but which has also been described in Brazil. Moyamoya patients may be submitted to different surgical procedures throughout their lives. Anesthesiologists must understand the pathophysiology of the disease and institute adequate perioperative measures to improve patients' prognosis. CASE REPORT: Female patient, 22 yr-old, chronic renal failure, with moyamoya disease, scheduled for surgical arterial-venous fistula installation. Anesthesia was induced with fentanyl, propofol and atracurium and maintained with sevoflurane. Patient was maintained in normocapnia and normothermia throughout the procedure. Patient was extubated and transferred to the post-anesthetic care unit without complications. CONCLUSIONS: This article describes the anaesthetic care of a moyamoya disease patient.

  11. Anaesthetic management of patients undergoing surgery for Moyamoya disease - our institutional experience

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    Vivek B Sharma

    2014-01-01

    Full Text Available Background: Moyamoya disease (MMD is a chronic cerebrovascular disorder, defined as the progressive stenosis or occlusion of the intracranial vessels. Because of the insecure cerebral circulation, these patients represent an anaesthetic challenge. Literature is scarce on the anaesthetic management of MMD, especially from the Indian subcontinent. The main objective of our study was to evaluate the peri-operative course and outcome of patients undergoing surgery for MMD. Materials and Methods: We analysed available medical records of all patients who underwent revascularisation surgeries for MMD over a period of 10 years (January 2002 to June 2012. Various intra- and post-operative data related to anaesthesia were recorded. The patients for the analysis were divided into two groups: Group 1, paediatrics (<18 years and group 2, adults (≥18 years. Data are presented as number (% or mean ± SD or median (range. Results: There were 36 patients (12 adults and 24 children. Normotension, normovolaemia, normo- or mild-hypercapnia and normothermia were maintained in all patients. No patient developed complications or new neurological deficit in the post-operative period. All patients were discharged with a full Glasgow coma scale (GCS and no new neurologic deficit. Conclusion: Although the incidence of MMD is not high in India, it is an important cause of cerebral stroke in children and adults. Proper pre-operative evaluation is the most effective method to achieve good results. Anaesthetic management of MMD should focus on the maintenance of adequate cerebral blood flow and cerebral perfusion pressure ensuring adequate cerebral oxygenation to avoid ischaemic complications.

  12. Pial synangiosis in patients with moyamoya younger than 2 years of age.

    Science.gov (United States)

    Jackson, Eric M; Lin, Ning; Manjila, Sunil; Scott, R Michael; Smith, Edward R

    2014-04-01

    Object Patients with moyamoya who are younger than 2 years of age represent a therapeutic challenge because of their frequent neurological instability and concomitant anesthetic risks. The authors report their experience with pial synangiosis revascularization in this population. Methods The authors reviewed the clinical and radiographic records of all patients with moyamoya in a consecutive series of patients under 2 years of age, who underwent cerebral revascularization surgery using pial synangiosis at a single institution. Results During a 12-year period (1994-2005), 34 procedures (bilateral in 15 patients, unilateral in 4) were performed in 19 patients younger than 2 years (out of a total of 456 procedures in 240 patients). Eighteen of these patients presented with either stroke or transient ischemic attack. The average age of the 19 patients at first surgery was 1.4 years (range 6 months-1.9 years). Unanticipated staged operations occurred in 3 patients, due to persistent electroencephalographic changes during the initial surgery in 2 cases and due to brain swelling during the procedure requiring ventriculostomy in the other. There were 2 perioperative strokes; both patients had postoperative seizures but made clinical recoveries. The average follow-up was 7 years (range 1-14 years). Long term, at follow-up, 13 patients (68%) were clinically independent for their age, with 8 (42%) having no significant deficit. Late complications included subdural hygroma evacuation (1), additional revascularization procedures performed years later for frontal lobe ischemia (2), late infarction (1), and asymptomatic ischemic change on routine follow-up MRI studies (1). All patients who had both pre- and postoperative angiography demonstrated progression of disease. Conclusions Despite the challenges inherent to this population, the majority of children with moyamoya under the age of 2 years have a good long-term prognosis. The data from this study support the use of pial

  13. Compartment analysis of {sup 123}I-iomazenil brain SPECT in patients with moyamoya disease

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    Kaneta, Tomohiro; Yamazaki, Tetsuro; Takahashi, Shoki; Yamada, Shogo [Tohoku Univ., Sendai (Japan). School of Medicine; Maruoka, Shin; Abe, Yoetsu

    1999-12-01

    We investigated 11 patients with moyamoya disease about {sup 123}I-Iomazenil kinetics in the brain using three-compartment, two-parameter model. The transition rate constant (K1) from the blood to the brain and the binding potential (BP) of the benzodiazepine to the receptors were calculated for every ROI (right and left side of cerebellum, frontal lobe, parietal lobe, occipital lobe and temporal lobe; 10 ROIs a case). The K1 value correlated with BP value significantly, but not so closely (r=0.639). And there is no significant difference in BP valued among low-K1 group (mean (of K1)-S.D.{<=}K1{<=}mean) and high-K1 group (meanmoyamoya disease patients. And we showed a case with IMP/IMZ discrepancy. The nerve cell in the hypoperfused area which has almost normal BP value is ischemic but viable. IMZ-SPECT presents an important information about the viability of the hypoperfused area in moyamoya disease patients' brain. (author)

  14. Retrospective analysis of perioperative factors on outcome of patients undergoing surgery for Moyamoya disease

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    Navneh Samagh

    2015-01-01

    Full Text Available Background: The short term outcome of patients undergoing surgery for Moyamoya disease can be affected by various perioperative factors. However, due to lesser prevalence of this disease in our country, data relating the effect of perioperative factors on the overall neurological outcomes of these patients is lacking. Aims: To analyze the effect of perioperative factors on the duration of postoperative hospital stay in patients undergoing surgery for Moyamoya disease. Settings and Design: It is a retrospective study analyzing various perioperative factors influencing the overall outcome of patients undergoing surgery for Moyamoya disease at a tertiary care centre in North India. Methods and Material: The medical records of all patients who underwent revascularization surgeries for Moyamoya disease from 2007 to till January 2014 were included for retrospective analysis. Various preoperative, intraoperative, and postoperative data were recorded. The data was statistically compared for short and prolonged hospital stay for various perioperative factors. The duration of post operative hospital stay was categorized as short (5 days. Statistical Analysis: Kolmogrov Smirnov test was applied to see the normality of continuous data. The association of various categorically classified data with 2 groups was found using Fisher Exact test. The trends in intraoperative hemodynamics were analysed using 2 way repeated measure Anova test. T-test was used for comparing two group means for various parameters. Results: A total of 15 patients were included in the study. One patient underwent surgery twice on two different occasions. Thirteen patients belonged to paediatric age group (<18 years. The type of anaesthetics used for induction and maintenance had no effect on patient outcome. Mean duration of anaesthesia was 2.45 (1.3-4.0 hours. The mean duration of hospital stay was 5.13 (3-10 days. Most of the parameters did not have significant effect on postoperative

  15. Retrospective analysis of perioperative factors on outcome of patients undergoing surgery for Moyamoya disease

    Science.gov (United States)

    Samagh, Navneh; Bhagat, Hemant; Grover, Vinod K.; Sahni, Neeru; Agarwal, Ashish; Gupta, Sunil K.

    2015-01-01

    Background: The short term outcome of patients undergoing surgery for Moyamoya disease can be affected by various perioperative factors. However, due to lesser prevalence of this disease in our country, data relating the effect of perioperative factors on the overall neurological outcomes of these patients is lacking. Aims: To analyze the effect of perioperative factors on the duration of postoperative hospital stay in patients undergoing surgery for Moyamoya disease. Settings and Design: It is a retrospective study analyzing various perioperative factors influencing the overall outcome of patients undergoing surgery for Moyamoya disease at a tertiary care centre in North India. Methods and Material: The medical records of all patients who underwent revascularization surgeries for Moyamoya disease from 2007 to till January 2014 were included for retrospective analysis. Various preoperative, intraoperative, and postoperative data were recorded. The data was statistically compared for short and prolonged hospital stay for various perioperative factors. The duration of post operative hospital stay was categorized as short (5 days). Statistical Analysis: Kolmogrov Smirnov test was applied to see the normality of continuous data. The association of various categorically classified data with 2 groups was found using Fisher Exact test. The trends in intraoperative hemodynamics were analysed using 2 way repeated measure Anova test. T-test was used for comparing two group means for various parameters. Results: A total of 15 patients were included in the study. One patient underwent surgery twice on two different occasions. Thirteen patients belonged to paediatric age group (<18 years). The type of anaesthetics used for induction and maintenance had no effect on patient outcome. Mean duration of anaesthesia was 2.45 (1.3-4.0) hours. The mean duration of hospital stay was 5.13 (3-10) days. Most of the parameters did not have significant effect on postoperative hospital stay

  16. Moyamoya disease and syndromes: from genetics to clinical management

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    Guey S

    2015-02-01

    Full Text Available Stéphanie Guey,1,3 Elisabeth Tournier-Lasserve,1,2 Dominique Hervé,1,3 Manoelle Kossorotoff4 1Inserm UMR-S1161, Université Paris 7 Denis Diderot, Sorbonne Paris Cité, Paris, France; 2AP-HP, Groupe hospitalier Lariboisière-Saint-Louis, Service de génétique neurovasculaire, Paris, France; 3Service de Neurologie, Centre de Référence des maladies Vasculaires Rares du Cerveau et de l'Œil (CERVCO, Groupe Hospitalier Saint-Louis Lariboisière-Fernand Widal, Assistance Publique-Hôpitaux de Paris, Paris, France; 4Pediatric Neurology Department, French Center for Pediatric Stroke, University Hospital Necker-Enfants Malades, AP-HP Assistance publique-Hôpitaux de Paris, Paris, France Abstract: Moyamoya angiopathy is characterized by a progressive stenosis of the terminal portion of the internal carotid arteries and the development of a network of abnormal collateral vessels. This chronic cerebral angiopathy is observed in children and adults. It mainly leads to brain ischemic events in children, and to ischemic and hemorrhagic events in adults. This is a rare condition, with a marked prevalence gradient between Asian countries and Western countries. Two main nosological entities are identified. On the one hand, moyamoya disease corresponds to isolated moyamoya angiopathy, defined as being “idiopathic” according to the Guidelines of the Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis. This entity is probably multifactorial and polygenic in most patients. On the other hand, moyamoya syndrome is a moyamoya angiopathy associated with an underlying condition and forms a very heterogeneous group with various clinical presentations, various modes of inheritance, and a variable penetrance of the cerebrovascular phenotype. Diagnostic and evaluation techniques rely on magnetic resonance imaging (MRI, magnetic resonance angiography (MRA conventional angiography, and cerebral hemodynamics measurements

  17. Characteristics of CT imaging of adult ischemic moyamoya disease%成年人缺血型烟雾病的CT成像特点

    Institute of Scientific and Technical Information of China (English)

    谢安明; 丁耀军; 李功杰

    2015-01-01

    目的:研究成年人缺血型烟雾病(MMD)的CT影像特点,以帮助临床医生选择治疗方案。方法:回顾性分析20例成年人缺血型MMD患者的CT平扫、CT灌注成像及CT血管成像等影像资料,并参考Suzuki 6级血管分级法,对CT血管成像特点进行相应分级(Ⅰ~Ⅵ级)。结果:①CT平扫检出脑内陈旧性梗死灶13例(占65%),急性脑梗死灶1例(占5%),阴性者6例(占30%);②CT灌注5例(占25%),未发现灌注异常15例(占75%),出现部分脑叶明显低灌注及局限性高灌注;③CT血管成像检出Ⅰ~Ⅵ级分别为1例、2例、5例、6例、4例及2例。结论:成年人缺血型MMD患者脑梗死灶分布以额叶、顶叶皮层或分水岭为主;脑灌注特点表现为正常或血流灌注不均匀,以低灌注为主;血管成像特点以Ⅲ、Ⅳ级为主,属于缺血型MMD中期。%Objective: To study the characteristics of CT imaging of adult ischemic type moyamoya disease, involving CT plain scan, CT perfusion imaging (CTP) and CT angiography (CTA). Methods:A retrospective analysis was made of the imaging data of 20 adult ischemic type moyamoya patients, including CT plain scan, CT perfusion imaging and CT angiography. CT vascular imaging features were graded I-VI with reference to Suzuki vascular grading. Results:Among the 20 patients with adult ischemic type moyamoya , ①CT plain scan: Old infarct lesions occurred in 13 cases(65%), 1 case suffered from acute cerebral infarction(5%), and negative patients totalled 6 (30%);②CT perfusion:5 cases(25%) were normal perfusion, 15 cases(75%) showed obviously low perfusion and local high perfusion;③CT angiography:patients of grade I to VI were respectively 1, 2, 5, 6, 4 and 2. Conclusion:Cerebral infarction lesions associated with adult ischemic type moyamoya disease are distributed mainly in the frontal and parietal cortex, or in watershed regions. Cerebral perfusion is characterized by normal or uneven blood

  18. The role of VEGF and KDR polymorphisms in moyamoya disease and collateral revascularization.

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    Young Seok Park

    Full Text Available We conducted a case-control study to investigate whether vascular endothelial growth factor (VEGF -2578, -1154, -634, and 936 and kinase insert domain containing receptor (KDR -604, 1192, and 1719 polymorphisms are associated with moyamoya disease. Korean patients with moyamoya disease (n = 107, mean age, 20.9±15.9 years; 66.4% female and 243 healthy control subjects (mean age, 23.0±16.1 years; 56.8% female were included. The subjects were divided into pediatric and adult groups. Among the 64 surgical patients, we evaluated collateral vessel formation after 2 years and divided patients into good (collateral grade A or poor (collateral grade B and C groups. The frequencies and distributions of four VEGF (-2578, -1154, -634, and 936 and KDR (-604, 1192, and 1719 polymorphisms were assessed from patients with moyamoya disease and compared to the control group. No differences were observed in VEGF -2578, -1154, -634, and 936 or KDR -604, 1192, and 1719 polymorphisms between the control group and moyamoya disease group. However, we found the -634CC genotype occurred less frequently in the pediatric moyamoya group (p = 0.040 whereas the KDR -604C/1192A/1719T haplotype increased the risk of pediatric moyamoya (p = 0.024. Patients with the CC genotype of VEGF -634 had better collateral vessel formation after surgery. Our results suggest that the VEGF -634G allele is associated with pediatric moyamoya disease and poor collateral vessel formation.

  19. Moyamoya Syndrome: A Window of Moyamoya Disease

    OpenAIRE

    2015-01-01

    Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment con...

  20. Adult Moyamoya disease: 320-Multidetector row CT for evaluation of revascularization in STA–MCA bypasses surgery

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    Tian, Bing; Xu, Bing; Liu, Qi; Hao, Qiang; Lu, Jianping, E-mail: cjr.lujianping@vip.163.com

    2013-12-01

    Purpose: To evaluate the utility of 320-multidetector row whole-brain computed tomography perfusion (WBCTP) and whole-head subtracted dynamic angiography (WHSDCTA) for assessing the revascularization of blood flow after superficial temporal artery (STA) to middle cerebral artery (MCA) bypass surgery in adults with Moyamoya disease (MD) in the short and long term. Patients and methods: 320-multidetector row WBCTP and WHSDCTA were applied in 20 patients with MD before and after surgery (for an average of 3 days and 3 months). The bypass arteries were investigated using WHSDCTA and compared with DSA. The regions of interests (ROIs) in the surgical and mirror sides of the cerebral cortex were drawn on a Vitrea Workstation. Cerebral blood volume (CBV), time to peak (TTP), cerebral blood flow (CBF), mean transit time (MTT), and delay time were recorded. Preoperative and postoperative perfusion parameters in the MCA distribution were compared using the paired t-test. Results: WHSDCTA could clearly demonstrate 24 bypass arteries in 26 arteries for 20 patients, results which were in accordance with the results of digital subtraction angiography (DSA). When comparing preoperative values to those within 3 days after surgery, only TTP and delay time were significantly different (P < 0.05). Values of CBV, TTP, CBF, delay time, and MTT 3 months after surgery were significantly different (P < 0.05) from those of preoperative perfusion. Conclusion: These data suggest that 320-multidetector row WBCTP and WHSDCTA can be used to evaluate the revascularization of blood flow after STA–MCA bypass surgery in patients with MD in the short and long term.

  1. Cerebral infarction following intracranial hemorrhage in pediatric Moyamoya disease - A case report and brief review of literature

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    Soumya Patra

    2012-01-01

    Full Text Available Moyamoya disease is a clinical entity characterized by progressive cerebrovascular occlusion with spontaneous development of a collateral vascular network called Moyamoya vessels. This disease mainly manifests as cerebral ischemia. Intracranial bleeding is another major presentation of patients with Moyamoya disease. We report here a 12-year-old male child who presented with severe headache, vomiting and meningismus. Initial neuroimaging study with noncontrast computed tomography scan revealed fresh intraventricular hemorrhage in right-sided lateral ventricle. Magnetic resonance imaging with angiography of brain was done 5 days later when the child developed right-sided hemiparesis, and the diagnosis of Moyamoya disease was confirmed along with lacunar infarction of right posterior peri and paraventricular area and in the left paraventricular area and centrum semiovale. Simultaneous presence of cerebral infarction along with intraventricular hemorrhage in adult with bleeding-type Moyamoya disease is reported in literature, but it is a rare entity in a child.

  2. Role of CT perfusion imaging in evaluating the effects of multiple burr hole surgery on adult ischemic Moyamoya disease

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    Dai, Dong Wei; Zhao, Wen Yuan; Yang, Zhi Gang; Li, Qiang; Liu, Jian Min [Second Military Medical University, Department of Neurosurgery, Changhai Hospital, Shanghai (China); Zhang, Yong Wei [Second Military Medical University, Department of Neurology, Changhai Hospital, Shanghai (China); Xu, Bing; Ma, Xiao Long; Tian, Bing [Second Military Medical University, Department of Radiology, Changhai Hospital, Shanghai (China)

    2013-12-15

    To evaluate the effects of the multiple burr hole (MBH) revascularization on ischemic type adult Moyamoya disease (MMD) by computed tomography perfusion (CTP). Eighty-six ischemic MMD patients received CTP 1 week before and 3 weeks after MBH operation. Fifty-seven patients received it again at 6 month and underwent digital subtraction angiography (DSA) and mRS follow-up. Cerebral blood flow (CBF), cerebral blood volume (CBV), mean transit time (MTT), time to peak (TTP), and relative values of ischemic symptomatic hemispheres were measured. Differences in pre- and post-surgery perfusion CT values were assessed. There were significant differences of CBF, TTP, and relative time to peak (rTTP) in ischemic hemisphere between 1 week before and 3 weeks after surgery, and no significant difference in relative cerebral blood flow (rCBF), CBV, relative cerebral blood volume (rCBV), MTT, relative mean transit time (rMTT). According to whether there was symptom improvement or not on 3 weeks after MBH, the rTTP value was not statistically significant in the patients whose symptoms were not improved at all on 3 weeks after operation. Six-month follow-up showed that CBF, rCBF, and rCBV values were significantly higher than those before operation. Postoperative MTT, TTP, rMTT, and rTTP values were significantly lower than those before operation. CTP is a sensitive method to obtain functional imaging of cerebral microcirculation, which can be a noninvasive assessment of the abnormalities of intracranial arteries and cerebral perfusion changes in MMD before and after surgery. CBF and TTP map, especially the relative values of TTP, seems to have the capability of being quite sensitive to the presence of altered brain perfusion at early time after indirect revascularization. (orig.)

  3. Use of multidetector CT in the diagnosis of Moyamoya disease

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    Gonzales-de Larrazabal, C.; Bhoey, H.K.; Lim, M.C.L. [Singapore Heart, Stroke and Cancer Centre, Ngee Ann City (Singapore)

    2005-07-01

    This article presents an adult case of moyamoya disease, diagnosed with CT angiography, and discusses the value of using a multidetector CT scan in the diagnosis of the disease. The patient's previous MRA and conventional angiography are used for comparison. (orig.)

  4. [Recurrent hyperperfusion after revascularization surgery for adult moyamoya disease: a case report].

    Science.gov (United States)

    Hayashi, Tomohide; Kashiwazaki, Daina; Akioka, Naoki; Kuwayama, Naoya; Kuroda, Satoshi

    2014-07-01

    Direct bypass surgery for moyamoya disease is quite useful for rapidly improving cerebral hemodynamics and resolving ischemic attacks but may induce hyperperfusion syndrome. In this report, we present a rare case of recurrent hyperperfusion after surgery for moyamoya disease. A 47-year-old woman developed left homonymous hemianopsia and was admitted to our hospital. Magnetic resonance(MR)imaging/angiography revealed definitive moyamoya disease presenting with acute cerebral infarction in the right temporoparietal lobe. She was treated with anti-hypertensive agents because she had severe hypertension. She successfully underwent right superficial temporal artery-middle cerebral artery double anastomosis and indirect bypass. A cerebral blood flow study just after surgery demonstrated marked hyperperfusion in the operated hemisphere. A repeat study 2 days later revealed that the hyperperfusion was dramatically improved. Subsequently, however, she developed a severe ipsilateral headache and focal seizure regardless of the blood pressure control. She complained of the headache for >2 weeks. Follow-up MRI showed a small subcortical hemorrhage in the right frontal lobe. Single-photon emission computed tomography revealed that the hyperperfusion relapsed 9 days after surgery and then gradually disappeared. This study shows that preoperative severe hypertension may induce uncontrollable hyperperfusion after direct bypass surgery for moyamoya disease.

  5. 颅内外直接、间接联合搭桥术治疗成人烟雾病25例临床分析%Clinical analysis of combined direct and indirect extracranial-intracranial bypass in 25 adult patients with Moyamoya disease

    Institute of Scientific and Technical Information of China (English)

    胡福广; 梁朝辉; 王立群; 李国胜; 刁勋; 张皓峰; 马志昭; 田剑光; 赵林

    2016-01-01

    [Abstrict]Objective To explore the key points and clinical value of combined direct and indirect extracranial-in⁃tracranial (EC-IC) bypass in patients with adult moyamoya disease. Methods Retrospective analysis of combined revas⁃cularization surgery in 25 adult patients with moyamoya disease. The frontal branch and parietal branch of the superficial temporal artery (STA) were dissected. Combined revascularization surgery consisted of direct (anastomosis between the su⁃perficial temporal artery and cortical branch of the middle cerebral artery) and indirect (encephalodurogaleosynan-giosis EDAS) surgeries. Clinical status was evaluated using the modified Rankin Scale and NIHSS score at 1 day before, 1 week and 3 months after surgery. Results Thirty lateralities were successfully performed on 25 patients. Postoperative angiogra⁃phy or CTA and cranial computer tomography perfusion imaging(CTP) were conducted to examine the patency of the di⁃rect anastomosis and cerebral blood flow in 23 patientswithin 1 weeks after surgery . The results showed that the anasto⁃motic vascular patency was excellent and the cerebral blood flow increased in parallel to the relief of the patients’s isch⁃emic symptoms. The median mRS scores were 3 (1,3) before surgery, 2 (1,3) 1 week and 1 (0,3) 1 month after surgery.The median mRS scores were significantly improved (Z=15.14, P<0.01). The median NIHSS scores was 5 (4,8) preopera⁃tively and 4(2,7) postoperation 1 week and 3(1,4) 3 months. The median NIHSS scores were also significantly improved (Z=11.36, P<0.01). Unfortunately, two patients had complication and left hemiparesis. One patient complicated with con⁃tralateral hemisphere infarction and the another one complicated with ipsilateral hemispheric hemorrhage after operation. Conclusions Combined revascularization surgery may result in satisfying improvement in clinical, angiographic, and he⁃modynamic states and prevention of recurrent stroke. The stabilized

  6. Investigating Brain Network Characteristics Interrupted by Covert White Matter Injury in Patients with Moyamoya Disease: Insights from Graph Theoretical Analysis.

    Science.gov (United States)

    Kazumata, Ken; Tha, Khin Khin; Narita, Hisashi; Shichinohe, Hideo; Ito, Masaki; Uchino, Haruto; Abumiya, Takeo

    2016-05-01

    Chronic ischemia in adult moyamoya disease (MMD) reduces the integrity of normal-appearing white matter (WM). We investigated whether covert WM impairment alters large-scale brain networks and specific neural circuits associated with neurocognitive dysfunction in MMD. Forty-six participants (control, n = 23; MMD, n = 23) were examined using diffusion tensor imaging and streamline tractography. Structural connectivity among 90 cortical and subcortical brain regions was evaluated using the mean fractional anisotropy along the fiber tracts. Graph theoretical analysis was used to measure network parameters and inter-regional connectivity. Global network parameters were reduced in patients with MMD, including cluster coefficient (controls vs. MMD: 3.62 ± 0.24 vs. 3.26 ± 0.36; P < 0.0001), characteristic path length (controls vs. MMD: 1.20 ± 0.02 vs. 1.17 ± 0.01; P < 0.001), and small-world property (controls vs. MMD: 3.07 ± 0.18 vs. 2.83 ± 0.27; P < 0.001). Reduced pairwise connectivity was found in prefrontal neural circuits within the middle/inferior frontal gyrus; supplementary motor area; and insular, inferior temporal, and dorsal cingulate cortices. Covert WM microstructural changes in patients with MMD alter large-scale brain networks, as well as lateral prefrontal neural circuits. Evaluation of structural connectivity may be useful to assess the severity of chronic ischemic injury from a network perspective.

  7. Moyamoya disease associated with asymptomatic mosaic Turner syndrome: a rare cause of hemorrhagic stroke.

    Science.gov (United States)

    Manjila, Sunil; Miller, Benjamin R; Rao-Frisch, Anitha; Otvos, Balint; Mitchell, Anna; Bambakidis, Nicholas C; De Georgia, Michael A

    2014-01-01

    Moyamoya disease is a rare cerebrovascular anomaly involving the intracranial carotid arteries that can present clinically with either ischemic or hemorrhagic disease. Moyamoya syndrome, indistinguishable from moyamoya disease at presentation, is associated with multiple clinical conditions including neurofibromatosis type 1, autoimmune disease, prior radiation therapy, Down syndrome, and Turner syndrome. We present the first reported case of an adult patient with previously unrecognized mosaic Turner syndrome with acute subarachnoid and intracerebral hemorrhage as the initial manifestation of moyamoya syndrome. A 52-year-old woman was admitted with a subarachnoid hemorrhage with associated flame-shaped intracerebral hemorrhage in the left frontal lobe. Physical examination revealed short stature, pectus excavatum, small fingers, micrognathia, and mild facial dysmorphism. Cerebral angiography showed features consistent with bilateral moyamoya disease, aberrant intrathoracic vessels, and an unruptured 4-mm right superior hypophyseal aneurysm. Genetic analysis confirmed a diagnosis of mosaic Turner syndrome. Our case report is the first documented presentation of adult moyamoya syndrome with subarachnoid and intracerebral hemorrhage as the initial presentation of mosaic Turner syndrome. It illustrates the utility of genetic evaluation in patients with cerebrovascular disease and dysmorphism.

  8. Serial {sup 99m}Tc-HMPAO Brain SPECT for Assessing Perfusion Improvement after DEAS in Moyamoya Patients

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    Lee, Kyung Han; Lee, Sang Hyung; Yeo, Jeong Seok; Kwark, Chul Eun; Chung, June Key; Lee, Myoung Chul; Cho, Byoung Kyu; Koh, Chang Soon [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1994-03-15

    Encephalo-duro-arterio-synangiosis (EDAS) is a relatively new surgical procedure for treatment of childhood moyamoya disease. We assessed regional cerebral perfusion in moyamoya patients before (1.3 mo) and after (6.8 mo) EDAS with {sup 99m}Tc-HMPAO brain SPECT. A total of 21 EDAS operations in 17 moyamoya patients was included. Preoperative CT or MRI showed cerebral infarction in 14 patients and carotid angiography showed Suzuki grade 1 to V stenosis in 6%, 9%, 62%, 12% and 12% of the hemispheres respectively. Preoperative SPECT showed regional hypoperfusion in all patients, bilateral frontal and temporal loves being the most frequently involved site. 4 X 4 pixel sized ROIs were applied on the frontotemmporal cortex in 3 slice averaged transverse tomographic images. An index of regional perfusion was measured as; PI (%)=average FT activity/average cerebellar activity X 100 Pre-EDAS ipsilateral PI ranged from 23.7 to 98.4% (mean:74.3 +- 17%) and increased significantly after operation (81.4 +- 17%, p<0.001). Individual post-EDAS PI improved in 15/21 cases, showed no significant change in 5 and was slightly aggravated in 1. The amount of clinical improvement XCI) was graded with a scale of 0 to 4 based on frequency and severity of TIA attacks. When patients were grouped according to pre-EDAS PI, group II (PI 70approx89) showed a significantly higher CI (3.3) compared to group I (PI<70, 1.57) of group III (PI>90, 0.5) (p<0.001). The amount of perfusion improvement (PI) showed significant correlation with CI (r-0.42, p=0.04). PI did not, however, correlate with the amount of neovascularization assessed angiographically in 8 patients. Serial HMPAO SPECT is an useful noninvasive study for assessing perfusion improvement after EDAS in childhood moyamoya patients.

  9. Management of distal choroidal artery aneurysms in patients with moyamoya disease: report of three cases and review of the literature.

    Science.gov (United States)

    He, Kangmin; Zhu, Wei; Chen, Liang; Mao, Ying

    2013-08-12

    Prevention of rebleeding plays an important role in the treatment of hemorrhagic moyamoya disease, because rebleeding results in high mortality and morbidity. We discuss possible treatment for patients with moyamoya disease accompanied with distal choroidal artery aneurysms and review the literature to summarize clinical treatment and mechanisms. The cases of three male patients who suffered from intraventricular hemorrhage are presented. Computed tomography (CT) and digital subtractive angiography (DSA) revealed that bleeding was believed to be caused by ruptured aneurysms originating from distal choroidal artery aneurysms. Two patients successfully underwent superficial temporal artery (STA)-middle cerebral artery (MCA) bypass combined with encephalo-duro-myo-synangiosis (EDMS) and the obliteration of the aneurysm. The follow-up DSA or CT scan demonstrated that the aneurysms completely disappeared with the patency of the reconstructed artery. Neither of the patients experienced rebleeding during the follow-up period (up to 34 months). Given conservative treatment, the third patient experienced recurrent hemorrhages 4 months after the first ictus. This study describes treatment for moyamoya disease accompanied with distal choroidal artery aneurysms. Our experience suggests that cerebral revascularization combined with obliteration of the complicated distal aneurysm in the same session is a possible treatment.

  10. Delayed cerebral ischemia associated with reversible cerebral vasoconstriction in a patient with Moyamoya disease with intraventricular hemorrhage: Case report.

    Science.gov (United States)

    Matsuoka, Go; Kubota, Yuichi; Okada, Yoshikazu

    2015-06-01

    We describe a case of cerebral infarctions caused by transient vasoconstrictions in the posterior circulation 2 weeks after intraventricular hemorrhage without subarachnoid hemorrhage in a 35-year-old patient with Moyamoya disease. To our knowledge, this is the first case report where diffuse segmental vasoconstrictions of the basilar and posterior cerebral arteries were recognized after intraventricular hemorrhage in Moyamoya disease. The patient complained of severe and acute-onset headache 14 days after the intraventricular hemorrhage, which had a different character and severity from the one she complained of at the onset of intraventricular hemorrhage. Finally, headache disappeared within 1 month and vasoconstriction resolved in 2 months. Reversible cerebral vasoconstriction syndrome was under consideration for the etiology of her condition because of the "thunderclap" characteristics of the headache and the delayed timing of occurrence of the vasoconstriction. This case report informs and alerts neurologists, neurosurgeons and neuroradiologists who observe and treat patients with Moyamoya disease that vasoconstriction in the posterior circulation may occur after intraventricular hemorrhage in these patients.

  11. A patient with Moyamoya-like vessels after radiation therapy for a tumor in the basal ganglia

    Energy Technology Data Exchange (ETDEWEB)

    Ishiyama, Koichi; Tomura, Noriaki; Kato, Koki; Takahashi, Satoshi; Watarai, Jiro; Sasajima, Toshio; Mizoi, Kazuo [Akita Univ. (Japan). School of Medicine

    2001-10-01

    A patient with Moyamoya-like vessels after radiation therapy for treatment of a tumor in the basal ganglia is reported. He was diagnosed as Down syndrome at birth. He had a tumor in the left basal ganglionic region at 12 years of the age. The tumor increased in size at age 14. He underwent cerebral angiography, which did not show a stenosis nor occlusion of the internal carotid artery, anterior cerebral artery, nor the middle cerebral artery. He received radiation therapy with a total dose of 56 Gy. He presented a dressing apraxia at age 19. MRI showed cerebral infarction in the left temporo-occipital region. Right internal carotid angiography revealed a severe stenosis of the internal carotid artery and anterior cerebral artery as well as a severe stenosis of the middle cerebral artery on the right side. Moyamoya-like vessels were seen in the basal ganglionic region. Left internal carotid angiography also showed a stenosis of the internal carotid artery and anterior cerebral artery as well as a severe stenosis of the middle cerebral artery on the left side. Moyamoya-like vessels were seen in the basal ganglionic region. Leptomeningeal anastomose and transdural anastomose were bilaterally seen. These arterial occlusion and stenotic phenomenon corresponded to a previous radiation field. These Moyamoya-like vessels with arterial stenosis and occlusion were thought to be due to radiation-induced vasculopathy, because a previous cerebral angiography showed a normal caliber of cerebral arteries. This patient showed that patients with radiation therapy in their early childhood should be carefully observed considering the possibility of the phenomenon. (author)

  12. Surgical treatment of adult moyamoya disease with combined STA-MCA bypass and EDAS: demonstration of technique in video presentation.

    Science.gov (United States)

    Li, Yiping; Cikla, Ulas; Baggott, Christopher; Yilmaz, Tevfik; Chao, Clifford; Baskaya, Mustafa K

    2015-01-01

    The natural history of Moyamoya disease (MD) is variable. Moyamoya disease in children mostly presents with progressive ischemic complications and in adulthood it tends to present with hemorrhage. Surgical strategies depend on augmenting cerebral blood flow to the anterior cerebral circulation. Revascularization is the mainstay treatment for MD. We introduce a 31 year old female with a history of MD and leftsided revascularization as a child, who presented with headache, confusion, and worsening left sided weakness. Cranial computed tomography (CT) showed a right sided putaminal hemorrhage. Cerebral angiogram with selective external runs revealed MD, hypoplastic parietal branch of the superficial temporal artery (STA) on the right side and previous revascularization on the left side. Thus, our decision was made to perform a bypass with the frontal branch of the STA to an M3-M4 segment of the middle cerebral artery and an encephaloduroarteriomyosynangiosis with the parietal branch of the STA. The patient's postoperative course was uneventful. Postoperative CT angiogram confirmed patency of bypass. This technique provides immediate revascularization and seems to provide risk reduction for ischemic and hemorrhagic stroke. We review the natural history and surgical treatment of MD along with a case presentation in which surgical technique is demonstrated in video.

  13. Ivy signs on FLAIR images before and after STA-MCA anastomosis in patients with Moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Ideguchi, Reiko; Enokizono, Mikako; Uetani, Masataka (Dept. of Radiological Sciences, Nagasaki Univ. Graduate School of Biomedical Sciences, Nagasaki (Japan)), e-mail: qqtt37gd9@forest.ocn.ne.jp; Morikawa, Minoru (Dept. of Radiology, Nagasaki Univ. School of Medicine, Nagasaki (Japan)); Ogawa, Yoji (Dept. of Radiology, Hanwa Daini Senboku Hospital, Osaka (Japan)); Nagata, Izumi (Dept. of Neurosurgery, Nagasaki Univ. School of Medicine, Nagasaki (Japan))

    2011-04-15

    Background: Leptomeningeal high signal intensity (ivy sign) on fluid-attenuated inversion-recovery (FLAIR) MR imaging is one of the features of Moyamoya disease. However, the correlation between ivy sign and cerebral perfusion status has not been fully evaluated. Purpose: To characterize ivy sign on FLAIR images in Moyamoya disease and compare this finding with hemodynamic alterations on perfusion single-photon emission CT (SPECT) obtained before and after bypass surgery. Material and Methods: Sixteen patients with angiographically confirmed Moyamoya disease who underwent superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis were included in the study. The presence of ivy sign on FLAIR images was classified as 'negative', 'minimal' and 'positive'. We evaluated the relationship between ivy sign and findings of SPECT, including cerebral vascular reserve (CVR) before and after surgery. Results: Minimal or positive ivy sign was seen in 13 (81%) of 16 patients, and 21 (66%) of 32 hemispheres. CVR in the areas with positive or minimal ivy sign was lower than that in the areas with negative ivy sign. After STA-MCA anastomosis, ivy sign disappeared or decreased in all 21 hemispheres demonstrating ivy sign. SPECT demonstrated apparent hemodynamic improvement in areas demonstrating disappearance or decrease of ivy sign. Conclusion: Ivy sign on FLAIR image is seen in areas with decreased cerebral perfusion. The sign is useful for non-invasive assessment of cerebral hemodynamic status before and after surgery

  14. Brain protective effect of dexmedetomidine on perioperative patients in temporal muscle sticking for moyamoya disease

    Directory of Open Access Journals (Sweden)

    Jun CHEN

    2014-06-01

    Full Text Available Objective To study the application of dexmedetomidine in patients undergoing temporal muscle sticking for moyamoya disease (MMD, and to discuss the possible mechanism of brain protection. Methods Sixty patients undergoing temporal muscle sticking operation were randomly divided into 2 groups: dexmedetomidine group (Group D, N = 30 and control group (Group S, N = 30, respectively receiving dexmedetomidine intravenous infusion (0.60 μ g/kg and the same dose of normal saline before anesthesia induction. In Group D, dexemdetomidine was administered continuously (intravenous pumping with the dose of 0.40 μg/(kg·h perioperatively, while the same dose of normal saline was administered in Group S. Heart rate (HR and mean arterial pressure (MAP were recorded at T0 (before administration, T1 (before tracheal intubation, T2 (1 min after intubation, T3 (at the moment of skin incision, T4 (before extubation and T5 (1 min after extubation respectively. Total amount of propofol, remifentanil and patients' recovery conditions after anesthesia were also recorded. Glutamine and malondialdehyde (MDA were measured and compared before and after operation. Results In group D, HR and MAP decreased significantly at T2-4 compared with T0 (P < 0.05, for all. Total amount of propofol, remifentanil in Group D was lower than that in Group S (P < 0.05, for all and the patients' recovery conditions in Group D after anesthesia was better than Group S (P = 0.000, for all, without occurence of shivering, cough and dysphoria (P < 0.05, for all. Glutamine and MDA increased after anesthesia compared with that before anesthesia in both 2 groups (P < 0.05, for all, however, it was higher in Group S than that in Group D (P < 0.05, for all. Conclusions Dexmedetomidine can provide stable hemodynamic condition during the anesthesia with patients undergoing temporal muscle sticking for moyamoya disease, and has a positive effect on improving both the outcome of the operation and

  15. Increased prevalence of autoimmune disease in patients with unilateral compared with bilateral moyamoya disease.

    Science.gov (United States)

    Chen, Jian-Bin; Liu, Yi; Zhou, Liang-Xue; Sun, Hong; He, Min; You, Chao

    2016-05-01

    OBJECT This study explored whether there were differences between the autoimmune disease prevalence rates in unilateral and bilateral moyamoya disease (MMD). METHODS The authors performed a retrospective review of data obtained from the medical records of their hospital, analyzing and comparing the clinical characteristics and prevalence rates of all autoimmune diseases that were associated with unilateral and bilateral MMD in their hospital from January 1995 to October 2014. RESULTS Three hundred sixteen patients with bilateral MMD and 68 with unilateral MMD were identified. The results indicated that patients with unilateral MMD were more likely to be female than were patients with bilateral MMD (67.6% vs 51.3%, p = 0.014, odds ratio [OR] 1.99). Overall, non-autoimmune comorbidities tended to be more prevalent in the unilateral MMD cases than in the bilateral MMD cases (17.6% vs 9.8%, p = 0.063, OR 1.97, chi-square test). Autoimmune thyroid disease and other autoimmune diseases also tended to be more prevalent in the unilateral MMD cases than in the bilateral MMD cases (19.1% vs 10.8%, p = 0.056, OR 1.96 and 8.8% vs 3.5%, p = 0.092, OR 2.77, respectively, chi-square test). The overall autoimmune disease prevalence in the unilateral MMD cases was significantly higher than in the bilateral MMD cases (26.5% vs 13.6%, p = 0.008, OR 2.29, 95% CI 1.22-4.28, chi-square test). Multiple logistic regression analysis showed that autoimmune disease was more likely to be associated with unilateral than with bilateral MMD (p = 0.039, OR 10.91, 95% CI 1.13-105.25). CONCLUSIONS This study indicated a higher overall autoimmune disease prevalence in unilateral than in bilateral MMD. Unilateral MMD may be more associated with autoimmune disease than bilateral MMD. Different pathogenetic mechanisms may underlie moyamoya vessel formation in unilateral and bilateral MMD.

  16. Relationship between hemorrhagic types and patterns of the collateral circulation in adult patients with hemorrhagic moyamoya disease%成人出血型烟雾病的出血类型与侧枝循环模式的关系初探

    Institute of Scientific and Technical Information of China (English)

    王戈鹰; 张桂运; 潘友贵; 韩洪杰; 何育生; 靳令经; 聂志余

    2014-01-01

    目的:探讨成人出血型烟雾病的出血类型与侧枝循环模式的关系。方法对同济大学附属同济医院2005年6月至2013年4月经DSA检查确诊的35例成人出血型烟雾病患者进行回顾性分析,根据头颅CT检查将颅内出血分为脑实质出血、脑室出血和蛛网膜下腔出血3种类型,其涉及的侧枝循环模式分为:第Ⅰ型前组:扩张的眼动脉分支筛前、后动脉及镰前动脉穿过颅底供应额叶皮质;第Ⅰ型后组:大脑后动脉皮层支→大脑前动脉和大脑中动脉皮层支;第Ⅱ型:由扩张和延长的脉络膜前动脉(anterior choroidal artery, AChA)和/或开放的后交通动脉(posterior communicating artery,PComA)→大脑后动脉→胼周后动脉和脉络膜后动脉→大脑前、中动脉深穿支;第Ⅲ型前组:脑膜中动脉和颞浅动脉→大脑前动脉和大脑中动脉供血区的代偿;第Ⅲ型后组:枕动脉→大脑后动脉供血区的代偿。分别使用Fisher′s确切概率法和交叉列联表比较三种出血类型组间和组内侧枝循环模式构成比的差异。结果该组出血型烟雾病患者存在2种或2种以上模式的侧枝循环占90.7%,不同出血部位组间侧枝循环代偿模式构成比有具有统计学差异(χ2=18.70,P=0.011),即脑实质出血、脑室出血和蛛网膜下腔出血各亚组侧枝循环的优势模式分别为第Ⅲ型前组、第Ⅱ型和第I型前组。结论第Ⅲ型前组、第Ⅱ型和第I型前组侧支循环模式分别可能是导致成人出血型烟雾病脑实质出血、脑室出血和蛛网膜下腔出血的出血原因之一。%Objective Purpose The study was designed to investigate the relationship between hemorrhage types and patterns of the collateral circulation of hemorrhagic moyamoya disease in adults. Methods Thirty-five patients with adult DSA-confirmed hemorrhagic MMD in Tongji Hospital of Tongji University between June 2005 and November 2013

  17. A study on measurement of the regional cerebral blood flow using autoradiographic method in moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Sasaki, Tomohiro; Kiya, Katsuzo; Yuki, Kiyoshi; Kawamoto, Hitoshi; Mizoue, Tatsuya; Kiura, Yoshihiro; Uozumi, Tohru [Hiroshima Prefectural Hospital (Japan); Ikawa, Fusao

    1997-11-01

    Development of Autoradiographic method (ARG) has provided measurement of cerebral blood flow in moyamoya disease. We evaluate a cerebral vasodilatory capacity (CVC) for moyamoya disease using ARG method. We used 5 patients with moyamoya disease as a candidate for measurement of the cerebral blood flow (CBF) who admitted to Hiroshima Prefectural Hospital during the past one year. There were 3 patients in an adult age and 2 patients in a young age. We tried to measure the regional CBF (rCBF) using ARG method which was a easy way to estimate the rCBF on SPECT. The CVC was calculated from the difference of the rCBF between resting SPECT and Diamox-loading SPECT. Results were as follows; Reactivity of cerebral vessels to CO{sub 2} loading and CVC weakened in moyamoya disease. The rCBF and CVC in the territories of anterior and middle cerebral arteries reduced in comparison with those in the area supplied by the posterior cerebral artery. The CVC at the treated side with surgical reconstruction recovered somewhat in an adult type. From these results, measurement of CBF using ARG method seems to be useful for evaluation of the CVC in moyamoya disease. (author)

  18. Prehospital antiplatelet use and functional status on admission of patients with non-haemorrhagic moyamoya disease: a nationwide retrospective cohort study (J-ASPECT study)

    Science.gov (United States)

    Onozuka, Daisuke; Hagihara, Akihito; Nishimura, Kunihiro; Kada, Akiko; Nakagawara, Jyoji; Ogasawara, Kuniaki; Ono, Junichi; Shiokawa, Yoshiaki; Aruga, Toru; Miyachi, Shigeru; Nagata, Izumi; Toyoda, Kazunori; Matsuda, Shinya; Suzuki, Akifumi; Kataoka, Hiroharu; Nakamura, Fumiaki; Kamitani, Satoru; Nishimura, Ataru; Kurogi, Ryota; Sayama, Tetsuro; Iihara, Koji

    2016-01-01

    Objectives To elucidate the association between antiplatelet use in patients with non-haemorrhagic moyamoya disease before hospital admission and good functional status on admission in Japan. Design Retrospective, multicentre, non-randomised, observational study. Setting Nationwide registry data in Japan. Participants A total of 1925 patients with non-haemorrhagic moyamoya disease admitted between 1 April 2012 and 31 March 2014 in Japan. Main outcome measure We performed propensity score-matched analysis to examine the association between prehospital antiplatelet use and no significant disability on hospital admission, as defined by a modified Rankin Scale score of 0 or 1. Results Propensity-matched patients who received prehospital antiplatelet drugs were associated with a good outcome on hospital admission (OR adjusted for all covariates, 3.82; 95% CI 1.22 to 11.99) compared with those who did not receive antiplatelet drugs prior to hospital admission. Conclusions Prehospital antiplatelet use was significantly associated with good functional status on hospital admission among patients with non-haemorrhagic moyamoya disease in Japan. Our results suggest that prehospital antiplatelet use should be considered when evaluating outcomes of patients with non-haemorrhagic moyamoya disease. PMID:27008684

  19. Analyses of the angiographic findings in cases of childhood moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Satoh, S.; Shibuya, H.; Suzuki, S.; Matsushima, Y.

    1988-04-01

    Cerebral angiograms of 34 patients with childhood moyamoya disease have been analysed, this analysis having revealed the following findings. The blood flow to abnormal net-like vessels (ANV) in the base of the brain is mainly supplied from the internal carotid artery (ICA) in the early stage of this disease. In a later stage, however the blood supply is mainly from the posterior cerebral artery (PCA). Though, no remarkable change was found in the volumes of ANV from the early to later stages of the disease, the leptomeningeal collaterals, the most prominent anastomoses in moyamoya disease, had a tendency to decrease during the later stage with the development of PCA stenosis. Eighteen occlusive PCA lesions were found in the proximal portion of the PCA in eight cases (44%), and in the distal in nine cases (50%). The incidence of aneurysm (1.5%) and intracranial bleeding was low as compared to cases of adult moyamoya disease.

  20. Moyamoya Disease with Peripheral Pulmonary Artery Stenoses and Coronary Artery Fistulae

    Directory of Open Access Journals (Sweden)

    Lindsay Reardon

    2009-01-01

    Full Text Available Moyamoya is a progressive disorder of the cerebral vasculature. Our report describes a rare case of Moyamoya disease with distal peripheral pulmonary artery stenoses and coronary fistulae in a 12-year-old Caucasian female patient.

  1. Axillary brachial plexus blockade in moyamoya disease?

    Directory of Open Access Journals (Sweden)

    Saban Yalcin

    2011-01-01

    Full Text Available Moyamoya disease is characterized by steno-occlusive changes of the intracranial internal carotid arteries. Cerebral blood flow and metabolism are strictly impaired. The goal in perioperative anaesthetic management is to preserve the stability between oxygen supply and demand in the brain. Peripheral nerve blockade allows excellent neurological status monitoring and maintains haemodynamic stability which is very important in this patient group. Herein, we present an axillary brachial plexus blockade in a moyamoya patient operated for radius fracture.

  2. Assessment of the hemodynamic changes after EDAS combined with bifrontal EGS in pediatric patients with moyamoya disease

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    Song, Yoo Sung; Kim, Yu Kyeong; Lee, Jae Sung; Kim, Seung Ki; Lee, Dong Soo; Chung, June Key; Lee, Myung Chul [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of)

    2007-07-01

    To assess the effect of encephaloduroarteriosynangiosis (EDAS) with or without bifrontal encephalogaleosynangiosis (EGS) in children with moyamoya disease, we evaluated cerebral hemodynamic changes using brain Tc-99m HMPAO SPECT. Total 34 pediatric patients (M: F=12:22, mean age;93 yrs) enrolled. Bypass surgery for both hemispheres (EDAS with EGS on one side, and EDAS on the other side) in 25 patients, unilateral EDAS with EGS in 7, and unilateral EDAS only in 2 were underwent. Perfusion SPECT before surgery, and 4 to 18 months after final surgery were done. The vascular territories for ICA, MCA and the brain regions for the frontal, parietal, temporal, and the occipital cortices were determined using standard ROls based on K-SPAM. Additionally, medial frontal cortex was selected to assess the effect of EGS. Basal/acetazolamide challenged cerebral blood flow (CBF), and cerebral vascular index (CVRI) were determined using normalized regional brain uptake to the cerebellum. 24 patients became symptom free, and 6 were improved but having some residual symptoms at the last follow up period. The other 3 were worsened after operation. Overall basal/acetazolamide stress CBF and CVRI for each brain region after surgery were increased, however, only the changes of CVRI were significant. Meanwhile, the improvement of CBF or CVRI in the brain regions ipsilateral to the hemisphere having EDAS with EGS was not significantly different when compared with those for the brain regions with EDAS only. Also, the hemodynamic improvement for the mesial frontal cortex in patients after EDAS with EGS was not significant, and showed no difference with those in patient with EDAS only. Quantitative perfusion SPECT demonstrated the hemodynamic improvement after EDAS with or without EGS in pediatric moyamoya disease. Cerebrovascular reserve showed meaningful improvement after surgery, implicating the effect of vascular anastomosis in ischemic areas.

  3. 经颅多普勒超声(TCD)检测3例烟雾病(Moyamoya Disease)患者的报告%Transcranial Doppler (TCD) detected 3 Cases of Moyamoya Disease Patients Report

    Institute of Scientific and Technical Information of China (English)

    刘春林; 刘秀珍

    2013-01-01

    烟雾病(Moyamoya disease,MMD)是一种病因未明的慢性进展性颅内动脉闭塞性疾病,DSA一直作为烟雾病临床诊断的金标准,但其有创性及价格昂贵的特点,限制了在长期随访观察中的应用[1]。 TCD在诊断被检动脉狭窄或闭塞方面已经被证实具有很好的敏感性和特异性,其无创性、廉价、操作简便的特点可使其反复检查或连续监测,适用于实时评价颅内血管的血流速度和频谱形态,可见TCD对超早期的烟雾病的筛查有非常重要的意义,也可为患者的病情、预后、治疗效果提供有力的依据。%Moyamoya disease (MMD) is a kind of unknown etiology of chronic progressive intracranial artery occlusive disease, DSA as the gold standard for clinical diagnosis of Moyamoya disease, but it has the characteristics of the invasive and expensive, limits the application in long-term fol ow-up observation. TCD in the diagnosis of checked artery stenosis or occlusion has been proved to have good sensitivity and specificity, the characteristics of its non-invasive, cheap, easy to operate, can make its double checking or continuous monitoring, is suitable for the real-time evaluation of intracranial vascular blood flow velocity and spectrum shape, visible TCD in super early screening of moyamoya disease have very important sense, can also be used for the patient to provide powerful basis, prognosis and therapeutic ef ect.

  4. A Pleural Solitary Fibrous Tumor, Multiple Gastrointestinal Stromal Tumors, Moyamoya Disease, and Hyperparathyroidism in a Patient Associated with NF1

    Directory of Open Access Journals (Sweden)

    Yoko Yamamoto

    2015-01-01

    Full Text Available Neurofibromatosis type 1 (NF1, also called von Recklinghausen’s disease, is a multisystemic disease caused by an alteration of the NF1 gene, a tumor suppressor located on the long arm of chromosome 17 (17q11.2. Loss of the gene function, due to a point mutation, leads to an increase in cell proliferation and the development of several tumors. We report a 60-year-old female patient manifesting hypercalcemia due to hyperparathyroidism, a solitary fibrous tumor (SFT of the pleura, multiple gastrointestinal stromal tumors (GISTs, and moyamoya disease associated with NF1. The SFT and GISTs were removed by staged operations. Then, hypercalcemia was successfully controlled after resection of the parathyroid adenoma. Based on a literature review, these combinations have never been reported, and the relevant literature is briefly discussed.

  5. Moyamoya disease and pregnancy: case reports and criteria for successful vaginal delivery

    OpenAIRE

    2015-01-01

    Key Clinical Message Based on our experience with seven deliveries (five cesarean and two vaginal deliveries) in five women with Moyamoya disease, we discussed the appropriate method of delivery and anesthesia for patients with Moyamoya disease. In certain conditions, women with Moyamoya disease can successfully undergo vaginal delivery.

  6. Intraoperative dexmedetomidine and postoperative cerebral hyperperfusion syndrome in patients who underwent superficial temporal artery-middle cerebral artery anastomosis for moyamoya disease

    Science.gov (United States)

    Seo, Hyungseok; Ryu, Ho-Geol; Son, Je Do; Kim, Jeong-Soo; Ha, Eun Jin; Kim, Jeong-Eun; Park, Hee-Pyoung

    2016-01-01

    Abstract Dexmedetomidine, a selective α2-agonist, reduces cerebral blood flow and has neuroprotective effects against cerebral ischemia/reperfusion injury in experimental animals. We examined whether intraoperative dexmedetomidine would reduce the incidence of postoperative cerebral hyperperfusion syndrome (CHS) after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis in patients with moyamoya disease. The electronic medical records of 117 moyamoya patients who underwent STA-MCA anastomosis were reviewed retrospectively. The patients were divided into 2 groups: 48 patients received intraoperative dexmedetomidine (Group D), while 69 patients did not (Group ND). The incidence (primary outcome), onset, and duration of postoperative CHS were noted. The incidence of postoperative CHS was 45.8% and 40.6% in groups D and ND, respectively (P = 0.708). The duration of postoperative CHS was shorter in group D than in group ND (median [Q1–Q3], 5 [3–7] vs 8 [5–10] days, P = 0.021). There was no significant difference in the onset of CHS between group D and group ND (0 [0–2] vs 1 [0–3] days, P = 0.226). In conclusion, intraoperative dexmedetomidine did not reduce the incidence of postoperative CHS, although it reduced the duration of CHS, in patients who had undergone direct revascularization surgery for moyamoya disease. PMID:28033272

  7. Childhood moyamoya disease: hemodynamic MRI

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    Tzika, A.A. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Robertson, R.L. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Barnes, P.D. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Vajapeyam, S. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Burrows, P.E. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Treves, S.T. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Scott, R.M. l [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States)

    1997-09-01

    Background. Childhood moyamoya disease is a rare progressive cerebrovascular disease. Objective. To evaluate cerebral hemodynamics using dynamic Gd-DTPA-enhanced imaging in children with moyamoya disease. Materials and methods. Eight children (2-11 years of age) with the clinical and angiographic findings typical of moyamoya disease, before and/or after surgical intervention (pial synangiosis), underwent conventional MR imaging (MRI) and hemodynamic MR imaging (HMRI). HMRI used a spoiled gradient-echo with low flip angle (10 deg) and long TE (TR/TE = 24/15 ms) to minimize T 1 effects and emphasize T 2{sup *} weighting. Raw and calculated hemodynamic images were reviewed. Three-dimensional time-of-flight MR angiography (MRA) and perfusion brain single photon emission computed tomography (SPECT) were also performed. Results. Abnormal hemodynamic maps resulting from vascular stenosis or occlusion and basal collaterals were observed in six patient studies. HMRI depicted perfusion dynamics of affected cerebrovascular territories, detected cortical perfusion deficits, and complemented conventional MRI and MRA. HMRI findings were consistent with those of catheter angiography and perfusion SPECT. Conclusion. Our preliminary experience suggests that HMRI may be of value in the preoperative and postoperative evaluation of surgical interventions in moyamoya disease. (orig.). With 4 figs., 3 tabs.

  8. Moyamoya disease: Experience with direct and indirect revascularization in 70 patients from a nonendemic region

    Directory of Open Access Journals (Sweden)

    Nishanth Sadashiva

    2016-01-01

    Conclusion: Both the combined and indirect revascularization procedures are effective in treating MMD. Pediatric patients had a better clinical improvement after surgery than the adult patients . Patients undergoing combined revascularization had a better clinical status compared to those who only underwent indirect revascularization. Combined revascularization surgery should be the surgical strategy in all age groups as it is feasible in a significant proportion of pediatric patients too.

  9. 成人烟雾病颅内出血部位及原N的MSCTA分析%MSCTA analysis of intracranial bleeding site and causes in adults moyamoya disease

    Institute of Scientific and Technical Information of China (English)

    王立莎; 朱磊; 张悦; 李光; 王勇

    2013-01-01

    Objective; To investigate the locations and causes of intracranial hemorrhage in adult moyamoya dis ease by the use of MSCTA technology. Methods:Clinical data and MSCTA materials of 30 moyamoya patients at the age of 30 or above were retrospectively analyzed. Results:ln this group,there were 6 cases of cerebral lobar hemorrhage,15 cases of thalamus and basal ganglia region hemorrhage,5 cases of intraventricular hemorrhage,and 4 cases of subarachnoid hemor rhage. MSCTA manifestations:① All cases of this group showed different degree of stenosis and occlusion above the internal carotid bifurcation;20 cases showed stenosis and occlusion in bilateral middle cerebral artery and 10 cases unilateral;25 caebral artery and 3 cases unilateral cases showed stenosis in superficial temporal artery and 3 cases unilateral. ②Abnormal vascular network developed around circle of Willis and basal ganglia region in 28 cases. ③Development of collateral circula tion: dilatation of posterior cerebral artery with formation of collateral with leptomeningeal branches in 22 cases; dilatation of anterior communicating artery supplied from the contralateral anterior cerebral artery in 10 case;dilatation of ophthalmic ar teries in 5 caseidilalation of external carotid artery branches forming anastomosis with cerebral pial arteris in 8 cases. ④ de velopment of cerebral aneurysm in 11 cases. ⑤Relationship between cerebral hemorrhage and collateral circulation: we can see the abnormal vascular network developed in all cases (15 cases of thalamus and basal ganglia area hemorrhage and 5 ca ses of ventricular hemorrhage). In 6 cases of cerebral lobar hemorrhage we can see rich piamater anastomosis branches,one of which showed that the bleeding in occipital lobe was due to the rupture of posterior cerebral artery aneurysm. Conclusion: Intracranial hemorrhage in adult moyamoya disease is mainly located in thalamus, basal ganglia region and ventricle. Abnormal vascular network,dilatation of

  10. Homozygosity for moyamoya disease risk allele leads to moyamoya disease with extracranial systemic and pulmonary vasculopathy.

    Science.gov (United States)

    Fukushima, Hiroyuki; Takenouchi, Toshiki; Kosaki, Kenjiro

    2016-09-01

    Moyamoya disease is characterized by diffuse distal intracranial stenosis. Recently, RNF213 has been identified as a susceptibility gene in the development of this condition. Pulmonary hypertension is a rare progressive vasculopathy with an unknown etiology. The co-occurrence of pulmonary hypertension and Moyamoya disease has been described in four patients; however, whether this co-occurrence represents a chance association or a common vascular pathology has remained unknown. Here, we report two unrelated male patients who presented during their childhood with dyspnea on exertion. Systemic vascular imaging studies revealed the presence of pulmonary hypertension and Moyamoya disease in both patients. Medical exome sequencing revealed that both patients had a homozygous mutation for p.Arg4810Lys in RNF213. We suggest that homozygosity in RNF213 may lead to a novel entity involving the brain and lung. Interestingly, when present in a heterozygous state, this mutation causes a classic cerebral vascular disease, Moyamoya disease. In the homozygous state, the exact same mutation led to Moyamoya disease with extracranial systemic vasculopathy in at least two patients. From a clinical standpoint, cerebrovascular or pulmonary vascular investigations may be warranted in patients with pulmonary hypertension or Moyamoya disease, respectively. © 2016 Wiley Periodicals, Inc.

  11. Moyamoya disease: diagnosis with three-dimensional CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Tsuchiya, K. (Dept. of Radiology, National Defense Medical Coll., Saitama (Japan) Dept. of Radiology, Kyorin Univ. School of Medicine, Tokyo (Japan)); Makita, K. (Dept. of Radiology, National Defense Medical Coll., Saitama (Japan) Dept. of Radiology, Social Health Insurance Medical Center, Tokyo (Japan)); Furui, S. (Dept. of Radiology, National Defense Medical Coll., Saitama (Japan) Dept. of Diagnostic Radiology, Toranomon Kyosai Hospital, Tokyo (Japan))

    1994-08-01

    Our purpose was to assess the value of three-dimensional (3D) CT angiography in the diagnosis of moyamoya disease. We studied seven patients with moyamoya disease proved by conventional angiography. Three-dimensional (3D) CT angiography was performed using rapid sequence or helical (spiral) scanning in conjunction with a bolus injection of intravenous contrast medium. All seven patients could be diagnosed as having moyamoya disease on the basis of the following 3D CT angiographic findings: poor visualisation of the main trunks and/or major branches of anterior and middle cerebral arteries (7 patients); dilated leptomeningeal anastomotic channels from the posterior cerebral arteries (4); and demonstration of ''moyamoya vessels'' in the basal ganglia (2). Although conventional angiography remains the principal imaging technique for demonstrating anatomical changes in detail, less invasive 3D CT angiography provides a solid means of diagnosing moyamoya disease when it is suspected on CT, MRI, or clinical grounds. (orig.)

  12. Asymptomatic moyamoya disease: literature review and ongoing AMORE study.

    Science.gov (United States)

    Kuroda, Satoshi

    2015-01-01

    Recent development of a non-invasive magnetic resonance examination has increased the opportunity to identify asymptomatic patients with moyamoya disease who have experienced no cerebrovascular events. However, their clinical features, prognosis, and treatment strategy are still unclear because of small number of subjects and short follow-up periods. Therefore, we have designed Asymptomatic Moyamoya Registry (AMORE) study in Japan. The objectives of this nation-wide, multi-center prospective study are to clarify long-term prognosis of asymptomatic patients with moyamoya disease and to determine the risk factors that cause ischemic and hemorrhagic stroke in them. In this article, we review the published data on asymptomatic moyamoya disease and report the on-going multi-center prospective cohort study, AMORE study. We would like to emphasize the importance to determine the clinical features, prognosis, and treatment strategies of asymptomatic moyamoya disease in very near future.

  13. Diffuse and uncontrolled vascular smooth muscle cell proliferation in rapidly progressing pediatric moyamoya disease.

    Science.gov (United States)

    Reid, Amy J; Bhattacharjee, Meenakshi B; Regalado, Ellen S; Milewicz, Allen L; El-Hakam, Lisa M; Dauser, Robert C; Milewicz, Dianna M

    2010-09-01

    Moyamoya disease is a rare stroke syndrome of unknown etiology resulting from stenosis or occlusion of the supraclinoid internal carotid artery (ICA) in association with an abnormal vascular network in the basal ganglia. Although the highest incidence of moyamoya disease is in pediatric patients, pathology reports have been primarily limited to adult samples and describe occlusive fibrocellular lesions in the intimae of affected arteries. We describe the case of a young girl with primary moyamoya disease who presented at 18 months of age with right hemiparesis following an ischemic stroke. Angiography showed stenosis of the distal left ICA, left middle cerebral artery, and right ICA. An emergent left-sided dural inversion was performed. Recurrent strokes and alternating hemiplegia necessitated a right dural inversion 6 months later. Nonetheless, her aggressive disease proved uniquely refractory to surgical revascularization, and she succumbed to recurrent strokes and neurological deterioration at 2.5 years of age. Pathological specimens revealed a striking bilateral occlusion of the anterior carotid circulation resulting from intimal proliferation of smooth muscle cells (SMCs). Most strikingly, the ascending aorta and the superior mesenteric artery demonstrated similar intimal proliferation, along with SMC proliferation in the media. The systemic pathology involving multiple arteries in this extremely young child, the first case of its kind available for autopsy, suggests that globally uncontrolled SMC proliferation, in the absence of environmental risk factors and likely resulting from an underlying genetic alteration, may be a primary etiologic event leading to moyamoya disease.

  14. Collateral circulation characteristics of CT angiography imaging of adult ischemic type moyamoya disease%成人烟雾病侧支循环的CT血管成像特点

    Institute of Scientific and Technical Information of China (English)

    谢安明; 丁耀军; 李功杰

    2016-01-01

    目的:探讨烟雾病侧支循环形成的CT血管成像特点。方法:收集在医院就诊的120例烟雾病患者影像资料,所有患者均行CT血管成像(CTA),根据侧支循环代偿途径的不同将其分为Ⅰ组(15例),Ⅱ组(53例),Ⅲ组(38例),Ⅳ组(14例)。采用Lightspeed VCT对所有烟雾病患者行头颅CTA检查,评价4组患者的临床表现、烟雾病血管代偿模式以及脑底异常血管网情况。结果:Ⅰ组15例全部为缺血型烟雾病;Ⅱ组53例中缺血型烟雾病8例,出血型烟雾病45例;Ⅲ组38例中缺血型烟雾病7例,出血型烟雾病31例;Ⅳ组14例全部为出血型烟雾病。结论:缺血型烟雾病血管网以迂曲扩张或正常为主,而出血型烟雾病血管网以狭窄为主。烟雾病的类型与侧支循环血管代偿特点有密切关联。%Objective:To explore the characteristics of collateral circulation of moyamoya disease in CT angiography imaging.Methods: Data of 120 moyamoya disease patients diagnosed by the 94th hospital were collected. All the patients underwent CT angiography imaging and were divided into groups according to compensatory ways of collateral circulation. Lightspeed VCT was used in all patients to conduct CTA check, and assessed the clinical performance of 4 groups. Results: Group 1 consisted of 15 cases (12.5%) of ischemic type moyamoya disease. Group 2 had 53 cases (44.2%), 8 cases were ischemic type moyamoya disease, and 45 cases were beeding type group moyamoya disease. Group 3 had 38 cases (31.7%), who showed moyamoya vessel formation in the bottom of the brain, 7 of these cases were ischemic moyamoya disease, and 31 were bleeding type moyamoya disease. Group 4 had 14 cases (11.6%), all of whom belonged to bleeding type moyamoya disease, characterized by ophthalmic artery, temporal artery, middle meningeal artery, occipital artery communicating with terminal cortex intracranial vascular.Conclusion: Compensatory characteristics

  15. Visualization of periventricular collaterals in moyamoya disease with flow-sensitive black-blood magnetic resonance angiography: preliminary experience.

    Science.gov (United States)

    Funaki, Takeshi; Fushimi, Yasutaka; Takahashi, Jun C; Takagi, Yasushi; Araki, Yoshio; Yoshida, Kazumichi; Kikuchi, Takayuki; Miyamoto, Susumu

    2015-01-01

    Fragile abnormal collaterals in moyamoya disease, known as "moyamoya vessels," have rarely been defined. While flow-sensitive black-blood magnetic resonance angiography (FSBB-MRA) is a promising technique for visualizing perforating arteries, as of this writing no other reports exist regarding its application to moyamoya disease. Six adults with moyamoya disease underwent FSBB-MRA. It depicted abnormal collaterals as extended lenticulostriate, thalamic perforating, or choroidal arteries, which were all connected to the medullary or insular artery in the periventricular area and supplied the cortex. This preliminary case series illustrates the potential for FSBB-MRA to reveal abnormal moyamoya vessels, which could be reasonably defined as periventricular collaterals.

  16. Moyamoya disease: a review of the literature.

    Science.gov (United States)

    Hertza, Jeremy; Loughan, Ashlee; Perna, Robert; Davis, Andrew S; Segraves, Kelly; Tiberi, Nina L

    2014-01-01

    Moyamoya disease is a rare progressive cerebrovascular disorder that can be congenital or acquired and involves progressive stenosis and occlusion of cerebral arteries. The brain through compensatory angeogenesis then attempts to compensate for ischemia by producing a local network of tiny blood vessels, which appear cloud-like on angiograms. Consistent with multifocal or diffuse areas of ischemia, people with moyamoya often have multiple areas of cognitive impairment. A thorough literature review of the neuropsychological profile in individuals diagnosed with moyamoya disease is provided. Medical and neuropsychological/neurobehavioral data of a middle-aged woman with moyamoya disease is also described. The patient (MA) suffered an embolic shower with insult to both cerebral hemispheres. Neuropsychological results suggested a multifocal decline, with both cortical and subcortical involvement. Data were consistent with impairments in attention, concentration, executive skills, processing speed, and memory encoding and retrieval, with relatively spared aspects of memory and language skills. This case study supports the literature and provides an additional example of the neuropsychological profile and deficit pattern of an individual with moyamoya following an embolic stroke shower.

  17. Role of magnetic resonance perfusion studies in moyamoya disease

    Directory of Open Access Journals (Sweden)

    Khanna Paritosh

    2004-04-01

    Full Text Available Moyamoya disease, Japanese for ′puff of smoke′, is a rare disease that presents most commonly with recurrent TIAs (transient ischemic attacks / stroke in childhood. Ischemic symptoms in patients with moyamoya disease are usually due to hemodynamically-mediated perfusion failure. Identification of abnormal tissue perfusion is an important aspect of the evaluation of these patients. We present the radiological features including the Magnetic Resonance (MR Perfusion findings illustrating the hemodynamic changes of cerebral ischemia in a case of moyamoya disease.

  18. Clinical studies of cerebral circulation using single photon emission computed tomography, 2; Evaluation of cerebral blood flow after acetazolamide loading on moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Uno, Toshiro [Gifu Univ. (Japan). Faculty of Medicine

    1993-09-01

    To evaluate cerebral blood flow (CBF) in patients with moyamoya disease, single photon emission computed tomography (SPECT) was performed using acetazolamide-activated {sup 133}Xe inhalation method. In the present investigation, 15 patients were subjected: 6 pediatric cases with the mean age of 10.8 years and 9 adult cases with the mean age of 44.6 years. The regional CBF (rCBF) was measured in the territory of anterior cerebral artery (ACA), middle cerebral artery (MCA), posterior cerebral artery (PCA), basal ganglia, and cerebellum. Cerebrovascular acetazolamide reactivity was evaluated from the rCBF measured before and after administration of acetazolamide. Namely, cerebrovascular acetazolamide reactivity was expressed as %CBF and calculated as follows: %CBF=100 x (CBF after acetazolamide administration-CBF at rest)/CBF at rest. rCBF in adult patients was decreased in the cerebral hemisphere, while that in childhood was significantly decreased in the territory of ACA. The %CBF after acetazolamide loading was decreased in the territory of ACA and MCA in both adult and childhood. When cerebral %CBF was compared to the cerebellar %CBF, the ratio of cerebral %CBF and cerebellar %CBF resulted in markedly lower in childhood than adult. rCBF and cerebrovascular acetazolamide reactivity were also measured before and after extracranial and intracranial (EC-IC) bypass surgery in three pediatric moyamoya patients. Although rCBF was increased immediately after EC-IC bypass surgery, the cerebrovascular acetazolamide reactivity remained blunted. These results meant that in the pediatric moyamoya patients cerebrovascular acetazolamide reactivity is more blunted than adult moyamoya patients. Also, the cerebral vessels in moyamoya disease were considered to be dilated to their limitation by the blood supplied through the EC-IC bypass and not to be expandable any more by acetazolamide. (author) 45 refs.

  19. "Moya-moya' disease caused by cranial trauma.

    Science.gov (United States)

    Fernandez-Alvarez, E; Pineda, M; Royo, C; Manzanares, R

    1979-01-01

    A case of "moya-moya" disease of a 12-year-old boy is reported. The clinical history started at 3 years 2 months after cranial trauma. The patient developed mental retardation, hemiparesis and seizures.

  20. Moyamoya vasculopathy in a child after hemolytic uremic syndrome: a possible etiopathogenesis.

    Science.gov (United States)

    Singla, M; John, E; Hidalgo, G; Grewal, D; Macmillan, C

    2008-04-01

    Moyamoya disease is a cerebral vasculopathy of unknown etiology frequently seen in the Asian population. We report a case of moyamoya vasculopathy in an African-American child who had renal failure followed by cerebral ischemia. Our patient presented with hemolytic uremic syndrome (HUS) and renal failure, and later developed seizures. We believe that in this patient HUS led to the pathogenesis of moyamoya disease. We suggest that patients with HUS who develop any neurological symptoms should be investigated for moyamoya vasculopathy for early diagnosis and treatment.

  1. Moyamoya disease; Die Moyamoya-Erkrankung

    Energy Technology Data Exchange (ETDEWEB)

    Papanagiotou, P.; Grunwald, I.Q.; Politi, M.; Roth, C.; Reith, W. [Universitaetsklinikum des Saarlandes Homburg/Saar (Germany). Klinik fuer Diagnostische und Interventionelle Neuroradiologie

    2005-05-01

    Moyamoya disease is an idiopathic progressive vasculopathy characterized by slowly progressive narrowing involving the distal internal carotid artery and the proximal circle of Willis. Collateral vessels develop to compensate for the slowly progressive stenosis. These enlarged collaterals appear as a ''puff of smoke'' on angiography, which gives the disease its name. This review discusses the clinical manifestation, imaging findings, and angiographic features. (orig.) [German] Die Moyamoya-Erkrankung ist eine idiopathische progressive Vaskulopathie, die durch eine langsam progrediente Stenosierung der distalen A. carotis interna und der proximalen Arterien des Circulus arteriosus Wilisii charakterisiert wird. Kollaterale Gefaesse hypertrophieren, um die progressive Stenosierung auszugleichen. In der Angiographie zeigen diese Kollateralen ein ''nebelartiges'' Erscheinungsbild, was der Erkrankung ihren Namen gab. In diesem Review werden das klinische Bild, die Bildgebung sowie die angiographischen Eigenschaften der Erkrankung diskutiert. (orig.)

  2. MOYAMOYA INDUCED ACUTE PARAPLEGIA IN A CHILD WITH EPILEPSY

    Directory of Open Access Journals (Sweden)

    MM. Taghdiri

    2009-04-01

    Full Text Available ObjectiveMoyamoya disease (MMD is a chronic, occlusive, cerebrovascular disorder of unknown  pathogenesis, characterized by progressive stenosis of the bilateral supraclinoid internal carotid arteries, with concomitant formation of tortuous arterial collateral vessels at the base of the brain, which reconstitute distal branches of the cerebral circulation. In Japanese, "Moyamoya" means "hazy puff of smoke" and refers to the angiographic appearance of the abnormal network of vessels that develop at the base of the brain and basal ganglia to supply a collateral route of blood flow. We report here the case of Moyamoya disease in a 5 year-old girl with normal mentality with a one year history of epilepsy, with Todd's paralysis. This condition is rare and most patients are diagnosed in childhood. With this report we aim to underscore the possibility that a usual neurological sign could be associated with unusual neurological disorders.Keywords:Moyamoya disease, Todd's paralysis, Epilepsy

  3. Clinical features of the primary intraventricular haemorrhagic type moyamoya disease%原发性脑室出血型Moyamoya病21例临床分析

    Institute of Scientific and Technical Information of China (English)

    陈骅; 罗良生; 张健; 林忠; 王东; 廖萍; 吴有志; 吴鸣

    2011-01-01

    Objective To investigate the relationship among non-hypertensive primary in-traventricular hemorrhage? Moyamoya disease and angiographic changes> we reviewed the clinical data of 21 adult patients with primary intraventricular haemorrhagic type moyamoya disease. Methods CT, DSA and clinical data of primary intraventricular haemorrhagic type moyamoya disease were reviewed. Results 21 patients in 25 non - hypertensive primary intraventricular hemorrhage were diagnosed as Moyamoya Disease. Moyamoya disease associated with basilar artery aneurysm was comfirmed in three patients. Angiographic dilatation and branch extension of the anterior choroidal artery were found in each haemorrhagic hemisphere. All patients received initial treatment obtained good results. Conclusion Moyamoya disease is the major cause of a-dult non-hypertensive primary intraventricular hemorrhage. Non-hypertensive adults with primary intraventricular hemorrhage should be conventionally inspected for moyamoya disease by DSA. Adult non-hypertensive primary intraventricular hemorrhage and angiographic dilatation and branch extension of the anterior choroidal artery is closely related. And the latter can be used as a predictor of the former.%目的 通过回顾性分析我院10年来收治的21例原发性脑室出血型Moyamoya病患者的临床资料,探讨非高血压性原发性脑室出血与Moyamoya病及其影像学特征的关系.方法 对我院神经外科10年来确诊的原发性脑室出血Moyamoya病患者的CT、DSA和临床治疗进行回顾性分析.结果 25例非高血压性原发性脑室出血患者中DSA证实21例为Moyamoya病.3例合并基底动脉动脉瘤.所有患者出血侧均存在脉络膜前动脉异常扩张扭曲及异常分支.该组患者首次治疗均获得良好结果.结论 Moyamoya病是成人非高血压性原发性脑室出血的主要病因,对成人非高血压性原发性脑室出血应常规行DSA明确是否存在Moyamoya病.成人非高血压性原发

  4. Efficacy of extracranial-intracranial revascularization for non-moyamoya steno-occlusive cerebrovascular disease in a series of 66 patients.

    Science.gov (United States)

    Gu, Yuxiang; Ni, Wei; Jiang, Hanqiang; Ning, Gang; Xu, Bin; Tian, Yanlong; Xu, Feng; Liao, Yujun; Song, Donglei; Mao, Ying

    2012-10-01

    Although there is uncertainty about whether extracranial-intracranial arterial bypass is useful for the treatment of steno-occlusive cerebrovascular disease in general, there is some argument for its continued use in particular patients. In the present study, we evaluated the efficacy of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis combined with encephalo-duro-myo-synangiosis (EDMS) in the treatment of non-moyamoya steno-occlusive cerebrovascular disease by retrospectively reviewing clinical and radiological data from 66 patients treated between January 2006 and April 2011. Forty-six double STA-MCA anastomoses and 20 single anastomoses were completed, and all remained patent in the perioperative phase, as confirmed by CT angiography. Postoperative CT perfusion imaging demonstrated immediate improvement in perfusion in the revascularized hemisphere. On discharge, 50 of the 66 patients (75.8%) had an improved National Institutes of Health Stroke Scale (NIHSS) score relative to preoperative values. After at least 6 months of follow-up, 54 (88.5%) patients had improved NIHSS scores relative to discharge values. CT perfusion imaging showed significant improvement compared to post-operative images. Follow-up digital subtraction angiography confirmed that 95 of 96 (99%) anastomoses remained patent. There was no significant difference between the single and double bypasses in terms of either clinical or hemodynamic outcomes on the revascularized side. STA-MCA combined with EDMS was effective for the treatment of non-moyamoya steno-occlusive cerebrovascular disease with hemodynamic impairment. Hemodynamic evaluation before and after surgery may help identify patients in need of a bypass and may be useful for predicting outcome.

  5. Usefulness of 3D TOF MR angiography using MTC and TONE in the follow-up of Moyamoya disease after cerebrovascular reconstruction

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    Miyazawa, Takahito; Arimoto, Hirohiko; Kaji, Tatsumi; Shima, Katsuji [National Defence Medical Coll., Tokorozawa, Saitama (Japan); Tsuchiya, Kazuhiro

    1997-09-01

    We studied patients with Moyamoya disease with or without indirect cerebrovascular reconstruction by means of 3D time-of-flight MR Angiography (3D TOF MRA) using MTC (magnetization transfer contrast) and TONE (tilted optimized non-saturating excitation). Twelve patients (6 males and 6 females, 6 children and 6 adults) with Moyamoya disease were investigated. Indirect cerebrovascular reconstruction (EDAS or EMAS) had been performed in 17 cerebral hemispheres of 12 patients. Imaging was performed on a 1.5T unit (Magnetom Vision, Siemens, Germany) using parameters as follows: TR/TE=39/7.0 msec, flip angle (mean) =20 degrees, slab thickness=70 mm, partitions=70, matrix=192 x 512, FOV=23O x 230 mm. In conclusion, 3D TOF MRA thus obtained was useful for depictions of steno-occlusive changes and also the development of collateral blood vessels after indirect vascular reconstruction. In addition, both the degree of development and distribution of Moyamoya vessels were clearly depicted on the MRA source images. We believe that 3D TOF MRA, using MTC and TONE, can be a substitute for invasive cerebral angiography for follow-up patients with Moyamoya disease. (author)

  6. Down syndrome and moyamoya: clinical presentation and surgical management.

    Science.gov (United States)

    See, Alfred P; Ropper, Alexander E; Underberg, Daniel L; Robertson, Richard L; Scott, R Michael; Smith, Edward R

    2015-07-01

    OBJECT Moyamoya can cause cerebral ischemia and stroke in Down syndrome (DS) patients. In this study, the authors defined a surgically treated population of patients with DS and moyamoya and compared their clinical presentation, response to surgical treatment, and long-term prognosis with those of the general population of patients with moyamoya but without DS. METHODS This study was a retrospective review of a consecutive operative series of moyamoya patients with DS treated at Boston Children's Hospital from 1985 through 2012. RESULTS Thirty-two patients, average age 9.7 years (range 1.8-29.3 years), underwent surgery for moyamoya in association with DS. The majority presented with ischemic symptoms (87% stroke, 42% transient ischemic attacks). Twenty-four patients (75%) had congenital heart disease. Nineteen patients (59%) had bilateral moyamoya on presentation, and 13 presented with unilateral disease, of which 2 progressed to surgery on the opposite side at a later date. Patients were followed for a median of 7.5 years (1-20.2 years) after surgery, with no patients lost to follow-up. Follow-up arteriography demonstrated Matsushima Grade A collaterals in 29 of 39 (74%) hemispheres, Grade B in 5 (13%), and Grade C in 5 (13%). Complications included postoperative strokes in 2 patients, which occurred within 48 hours of surgery in both; one of these patients had arm weakness and the other confusion (both had recovered completely at follow-up). Seizures occurred in 5 patients perioperatively, including one who had a new seizure disorder related to hypocalcemia. CONCLUSIONS Moyamoya disease is a cause of stroke in patients with DS. Both the incidence of preoperative stroke (87% vs 67%) and the average age at diagnosis for children under age 21 (8.4 vs 6.5 years) were greater in patients with DS and moyamoya than in the general moyamoya surgical population, suggesting a possible delay in reaching a correct diagnosis of the cause of cerebral ischemia in the DS patient

  7. Clinical significance of posterior cerebral artery stenosis/occlusion in moyamoya disease

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    Kuroda, Satoshi; Ishikawa, Tatsuya; Iwasaki, Yoshinobu [Hokkaido Univ., Sapporo (Japan). Graduate School of Medicine; Houkin, Kiyohiro [Sapporo Medical Univ. (Japan)

    2002-12-01

    The present study was aimed at clarifying the clinical significance of posterior cerebral artery (PCA) stenosis/occlusion in pediatric and adult moyamoya disease. This study included a total of 132 patients (52 children and 80 adults) who were diagnosed as by cerebral angiography having moyamoya disease. CT or MRI was performed to examine the location of cerebral infarction in all subjects. Cerebral blood flow and vasoreactivity to acetazolamide were measured in 80 patients before surgery, using single photon emission computed tomography (SPECT). Three-dimensional MR angiography (3D-MRA) was repeated in 32 pediatric patients after surgery in order to clarify the natural course of the PCA stenosis/occlusion. Of 264 sides in 132 patients, PCA stenosis/occlusion was observed in 50 sides of 40 patients (30.3%). Its incidence was significantly higher in ischemic-type patients than in hemorrhagic-type and asymptomatic patients, and was higher in patients in the advanced stage of the disease. The hemisphere ipsilateral to PCA stenosis/occlusion had higher incidence of ischemic symptoms, cerebral infarction, and impaired cerebral hemodynamics. Transient ischemic attack (TIA) (hemianopsia) or cerebral infarction in the occipital lobe was noted in 4 (10%) of 40 patients during follow-up periods after bypass surgery for anterior circulation. Of 32 pediatric patients, none showed progression of PCA stenosis on 3D-MRA during follow-up periods. The present study showed that the involvement of PCA could increase the risk of TIA and/or cerebral infarction in both anterior and posterior circulation areas, suggesting that the PCA plays an important collateral role in moyamoya disease. (author)

  8. Local Vasogenic Edema without Cerebral Hyperperfusion after Direct Revascularization Surgery for Moyamoya Disease.

    Science.gov (United States)

    Sakata, Hiroyuki; Fujimura, Miki; Mugikura, Shunji; Sato, Kenichi; Tominaga, Teiji

    2015-07-01

    Superficial temporal artery-middle cerebral artery anastomosis is generally used as the standard surgical treatment for moyamoya disease to prevent cerebral ischemic attacks. Although the main potential complications associated with this treatment are cerebral hyperperfusion and ischemia, the adverse impacts of revascularization surgery remain unclear. Of the 142 consecutive surgeries for moyamoya disease at our hospital from 2008, we herein presented 2 cases of adult-onset moyamoya disease that manifested local vasogenic edema at the site of anastomosis without cerebral hyperperfusion; 1 in a 31-year-old woman presented with transient ischemic attack and the other in a 22-year-old man manifested as minor completed stroke. Both patients underwent superficial temporal artery-middle cerebral artery anastomosis, resulting in the formation of a reversible high-signal-intensity lesion at the site of anastomosis on T2-weighted images along with an increase in apparent diffusion coefficient values, whereas diffusion-weighted images showed no changes. Neither hyperperfusion nor hypoperfusion, as assessed by single-photon emission computed tomography with N-isopropyl[123I]-p-iodoamphetamine, was observed postoperatively. In light of the increased risk of the further progression of vasogenic edema to intracerebral hemorrhage, these patients were treated with prophylactic blood pressure lowering and the intravenous infusion of a free radical scavenger. They did not have any further cerebrovascular events during the follow-up period. Regional vasogenic edema without cerebral hyperperfusion, possibly due to cerebral ischemia/reperfusion injury, may be another novel entity that needs to be considered as a potential complication after extracranial-intracranial bypass for moyamoya disease. Strict postoperative management should be used to avoid hemorrhagic transformation.

  9. Recent progress in hemorrhagic moyamoya disease.

    Science.gov (United States)

    Wan, Ming; Duan, Lian

    2015-04-01

    Moyamoya disease (MMD) is a chronic progressive cerebrovascular disease, which can be divided into three types: ischemic, hemorrhagic, and asymptomatic. Hemorrhagic MMD has attracted considerable attention due to its distinctive imaging features and the controversy over the treatment. This report presents a comprehensive review of the literature on hemorrhagic MMD, focusing on the epidemiological characteristics, etiology and pathogenesis, imaging features, predictors of hemorrhage, and treatment options and their efficacy of hemorrhagic MMD. Hemorrhagic MMD mainly occurs in adult patients in Asian countries, and many factors may contribute to the etiology and development of this disease. Hemorrhagic MMD has two major imaging features: the dilatation and abnormal branching of anterior choroidal artery or posterior communicating artery, and multiple microbleeds, which may predict subsequent hemorrhage. The treatment for hemorrhagic MMD is not standardized, and large sample prospective randomized clinical trials may help to determine which method is better. In hemorrhagic MMD patients, more attention should be paid to cognitive function and quality of life, and these assessments should be included in the evaluation of effectiveness of treatment modalities.

  10. Vascular collateralization along ventriculoperitoneal shunt catheters in moyamoya disease.

    Science.gov (United States)

    Singla, Amit; Lin, Ning; Ho, Allen L; Scott, R Michael; Smith, Edward R

    2013-06-01

    Surgically created openings such as bur holes can serve as avenues for the development of collateral blood supply to the brain in patients with moyamoya disease. When such collateralization occurs through preexisting shunt catheter sites, the potential exists for perioperative stroke if these vessels are damaged during revision of a ventricular catheter for shunt malfunction. In this paper the authors report on a series of patients with a history of ventriculoperitoneal (VP) shunts who later developed moyamoya disease and were found to have spontaneous transdural collateral vessels at ventricular catheter sites readily visualized on diagnostic angiography. A consecutive surgical series of 412 patients with moyamoya disease treated at Boston Children's Hospital from 1990 to 2010 were reviewed to identify patients with concomitant moyamoya and a VP shunt. The clinical records and angiograms of these patients were reviewed to determine the extent of bur hole collaterals through the shunt site. Three patients were identified who had VP shunts placed for hydrocephalus and subsequently developed moyamoya disease. All 3 patients demonstrated spontaneous transdural collaterals at the ventricular catheter bur hole, as confirmed by angiography during the workup for moyamoya disease. No patients required subsequent revision of their ventricular catheters following the diagnosis of moyamoya. All patients have remained stroke free and clinically stable following pial synangiosis. Although the association of moyamoya and shunted hydrocephalus is rare, it may present a significant potential problem for the neurosurgeon treating a shunt malfunction in this patient population, because shunt bur holes may become entry sites for the ingrowth of significant cortical transdural collateral blood supply to the underlying brain. Shunt revision might therefore be associated with an increased risk of postoperative stroke or operative-site hemorrhage in this population if this

  11. Functional Cortical and Cerebellar Reorganization in a Case of Moyamoya Disease

    Science.gov (United States)

    Calabrò, Rocco S.; Bramanti, Placido; Baglieri, Annalisa; Corallo, Francesco; De Luca, Rosaria; De Salvo, Simona

    2015-01-01

    Background: Functional studies have been previous reported in stroke patients, but no studies of functional magnetic resonance imaging have been performed in Moyamoya disease. Objective: To assess the cortical and cerebellar reorganization in a moyamoya patient. Methods: We reported a case of a patient suffering from moyamoya disease, undergoing a neuropsychological assessment, a neurocognitive rehabilitative treatment, an electroencephalogram evaluation, and a functional magnetic resonance imaging examination. Results: The subject showed a cognitive impairment, a slow electroencephalogram activity, and the ipsi- and controlateral motor cortex and cerebellar functional magnetic resonance imaging activation. Conclusions: This is the first functional magnetic resonance imaging case study reported in moyamoya disease. We showed a cortical reorganization, which could play an important role in clinical evaluation and motor recovery. The cerebellar activation, showed after cognitive and motor rehabilitation, could support the idea that the cerebellum contains several cognitive-related subregions involved in different functional networks in moyamoya disease. PMID:25852976

  12. [Unilateral moyamoya disease associated with acrofacial vitiligo in a 13-year-old patient--case report].

    Science.gov (United States)

    Vranjesević, D; Jović, N; Milovanović, D; Djukić, A

    1994-01-01

    Cerebrovascular Moyamoya disease (MMD) is a clinical entity characterized by stenotic or occlusive lesions around the terminal portions of the internal carotid arteries (ICA) and the formation of abnormal vascular networks at the base or convexity. This condition is sporadic, and there are no pathological explanations for this cause. A case with unilateral juvenile MMD is reported. A 13-year-old girl with sudden episode of left limbs weakness, accompanied by transient dysphasia and seizures attack, was admitted to our hospital for further investigation. Computed tomography scanning showed an area of localized brain swelling in the right capsular-thalamic region. Angiographic findings demonstra-ted unilateral involvement. Cerebral panangiography reve-aled a high grade stenosis of the right supraclinoid ICA and a peculiar network (moyamoya phenomenon) formed by dilated basal collateral perforating arteries and produced a cloudy image resembling "a puff of smoke". Left carotid angiogram showed a collateral circulation through the anterior communicant artery and apportioned to the right cerebral hemisphere. The vertebrobasilar system was normal. An ultrasoud Doppler study demonstrated a severely decreased blood flow gradient (0.1 l/min) through the right hypoplastic ICA, while the left common and the left ICA showed remarkable increased blood flow, partly due to abondant collateral vessels. Acquired, non-progressive vitiligo with areas of complete pigment loss appeared on flexor wrists, extensor distal extremities, superior eyelids and especially on joints regions (elbow, knee and small joints of the hands and fret). This association has not been reported previosly.

  13. Moyamoya disease: diagnostic accuracy of MRI.

    Science.gov (United States)

    Yamada, I; Suzuki, S; Matsushima, Y

    1995-07-01

    Our purpose was to evaluate the diagnostic accuracy of MRI in moyamoya disease. We studied 30 patients with this disease, comparing MRI and angiographic findings. The diagnostic value of MRI was evaluated for occlusive lesions, collateral vessels, and parenchymal lesions. In all patients bilateral occlusion or stenosis of the supraclinoid internal carotid artery and proximal anterior and middle cerebral arteries was clearly shown by MRI, and staging of the extent of occlusion agreed with angiographic staging in 44 (73%) of 60 arteries. MRI, particularly coronal images, clearly showed basal cerebral moyamoya vessels in 54 hemispheres, and 45 of a total of 71 large leptomeningeal and transdural collateral vessels were identified. MRI also showed parenchymal lesions in 48 (80%) hemispheres, and the extent of occlusion in the anterior and posterior circulations respectively correlated with white matter and cortical and/or subcortical infarcts.

  14. Moyamoya syndrome precipitated by cranial irradiation for craniopharyngioma in children.

    Science.gov (United States)

    Lee, Hyun Seok; Seol, Ho Jun; Kong, Doo-Sik; Shin, Hyung Jin

    2011-12-01

    Recently, combination of surgery and radiation therapy (RT) has been recommended in the treatment of craniopharyngioma. RT could be associated with late complications, including vasculopathy. We report two cases of the moyamoya syndrome seen in children with craniopharyngioma who received RT after surgical resection. Thirty-five patients in pediatric age with craniopharyngioma were surgically treated. Fifteen out of 35 patients underwent surgical resection followed by RT or gamma knife surgery. Two of the 15 were found to have symptoms of transient ischemic attack and were diagnosed as moyamoya syndrome through the cerebral angiography. Age at RT was 4 and 13 years, respectively. The latent period for development of the moyamoya syndrome was 27 months and 3 years, respectively, after RT. The RT dose of both patients was 54 Gy. These two patients received bilateral encephaloduroarteriosynangiosis procedures. We report here these two cases of radiation-induced moyamoya syndrome in pediatric craniopharyngioma. Pediatric patients with craniopharyngioma who received RT should be reminded, during follow-up, about the risk of development of the moyamoya syndrome.

  15. Moyamoya Disease Mimicking Encephalitis

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    Maryam Khalesi

    2014-09-01

    Full Text Available Moyamoya disease is a rare vaso-occlusive illness with an unknown etiology characterized by stenosis of the internal carotid arteries with spontaneous development of a collateral vascular network. A 15-month-old girl was referred to the emergency ward of Imam Reza Hospital due to decreased level of consciousness, focal seizures and fever during the previous 24 hours with an impression of encephalitis. Physical examination revealed left side hemiparesis; however brain CT-Scan did not show any significant lesions. Initial therapy with vancomycin, ceftriaxone and acyclovir was administered. CSF analysis did not show any abnormality and the blood as well as CSF cultures results were negative. Brain MRI showed hyperintensity at right frontal and parietal regions, suggesting vascular lesion. Magnetic resonance angiography (MRA showed bilaterally multiple torsions in vessels at the basal ganglia consistent with moyamoya vessels. In all children exhibiting encephalitis, vascular events such as moyamoya disease should be considered. Brain MRI is a critical tool for this purpose. Common causes of encephalitis such as herpes simplex should also be ruled out.

  16. Psychomotor Delay, a Possible Rare Presentation of Moyamoya Disease

    Directory of Open Access Journals (Sweden)

    M.R. Ashrafi

    2011-06-01

    Full Text Available Moyamoya disease is a rare, chronic cerebrovascular occlusive disease of unknown etiology. It is characterized by progressive stenosis of the arteries of the circle of Willis leading to ischemicstrokes in young people and cerebral hemorrhage, which is more frequent in adults. Secondarily,an abnormal network of fine collateral vessels arises at the base of the brain. The term moyamoyarefers to the angiographic appearance of the cerebral vasculature. We present such a disease in an 18-month-old Iranian girl with global developmental delay, which is a very rare presentationof moyamoya disease. She was diagnosed by magnetic resonance imaging (MRI and magnetic resonance angiography (MRA.

  17. Assessment of Cerebral Hemodynamic Changes in Pediatric Patients with Moyamoya Disease Using Probabilistic Maps on Analysis of Basal/Acetazolamide Stress Brain Perfusion SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ho Young; Lee, Jae Sung; Kim, Seung Ki; Wang, Kyu Chang; Cho, Byung Kyu; Chung, June Key; Lee, Myung Chul; Lee, Dong Soo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2008-06-15

    To evaluate the hemodynamic changes and the predictive factors of the clinical outcome in pediatric patients with moyamoya disease, we analyzed pre/post basal/acetazolamide stress brain perfusion SPECT with automated volume of interest (VOIs) method. Total fifty six (M:F=33:24, age 6.7{+-}3.2 years) pediatric patients with moyamoya disease, who underwent basal/acetazolamide stress brain perfusion SPECT within 6 before and after revascularization surgery (encephalo-duro-arterio-synangiosis (EDAS) with frontal encephalo-galeo-synangiosis (EGS) and EDAS only followed on contralateral hemisphere), and followed-up more than 6 months after post-operative SPECT, were included. A mean follow-up period after post-operative SPECT was 33{+-}21 months. Each patient's SPECT image was spatially normalized to Korean template with the SPM2. For the regional count normalization, the count of pons was used as a reference region. The basal/acetazolamide-stressed cerebral blood flow (CBF), the cerebral vascular reserve index (CVRI), and the extent of area with significantly decreased basal/acetazolamide- stressed rCBF than age-matched normal control were evaluated on both medial frontal, frontal, parietal, occipital lobes, and whole brain in each patient's images. The post-operative clinical outcome was assigned as good, poor according to the presence of transient ischemic attacks and/or fixed neurological deficits by pediatric neurosurgeon. In a paired t-test, basal/acetazolamide-stressed rCBF and the CVRI were significantly improved after revascularization (p<0.05). The significant difference in the pre-operative basal/acetazolamide-stressed rCBF and the CVRI between the hemispheres where EDAS with frontal EGS was performed and their contralateral counterparts where EDAS only was done disappeared after operation (p<0.05). In an independent student t-test, the pre-operative basal rCBF in the medial frontal gyrus, the post-operative CVRI in the frontal lobe and the parietal

  18. 'Moyamoya' disease accompanied by low-density areas in the occipital regions on CT

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    Kobayashi, Akira; Handa, Hajime; Yonekawa, Yasuhiro; Taki, Waro; Miyake, Hidenori; Nakatani, Hideyuki (Kyoto Univ. (Japan). Faculty of Medicine)

    1983-10-01

    The CT, angiographic, and clinical symptoms of 33 patients with ''Moyamoya'' disease were examined in order to evaluate the hemodynamic role of the vertebro-basilar system. 1) CT of 4 adults and 12 children showed a low-density area in the occipital region. Besides, all of those areas were accompanied by such abnormal findings as an abnormal, low-density and contrast enhancement in the other cerebrum. 2) The angiographic findings revealed that 16 patients belonged to the IIIrd and IVth stages, according to Suzuki's classification. 3) All of those 16 patients developed infarctions, which was considered to be a rather graver ischemic sign than TIA, convulsion, or hemorrhage. 4) In addition, Krypton-81m single-photon-emission CT demonstrated that, in ''Moyamoya'' disease, the vertebro-basilar blood actually supplied the carotid territory. These findings indicate that the posterior cerebtal artery plays an important role in the collateral circulation in ''Moyamoya'' disease.

  19. Middle cerebral artery stenosis associated with moyamoya pattern collateralization

    Directory of Open Access Journals (Sweden)

    Randall Edgell

    2010-11-01

    Full Text Available Background and Purpose: Moyamoya disease is a well described phenomenon presenting with terminal internal carotid artery occlusion and rete pattern of collateralization around the occlusion. The development of moyamoya-like collaterals secondary to isolated middle cerebral artery stenosis or occlusion and the natural history of this entity in Caucasians have not been well described. Methods: Cerebral angiograms and CT angiograms performed between August 2004 and August of 2006 demonstrating moyamoya collateralization at a single US center were retrospectively reviewed. All cases of middle cerebral artery stenosis associated with a rete pattern of collateralization were included in this series. Demographic, clinical, and angiographic data were obtained. Results: There were 3 cases of middle cerebral artery stenosis associated with a moyamoya pattern of collateralization. The average age of the patients was 36 years old, 2 were male, and all were Caucasian. All patients presented with ischemic symptoms. The average degree of stenosis was 91%. No stenosis was seen in the supraclinoid internal carotid arteries or elsewhere in the intracranial vasculature. Conclusion: We describe a moyamoya-like pattern of anastomosis associated with isolated severe middle cerebral artery stenosis or occlusion in Caucasians.

  20. Staging of moyamoya disease by perfusion SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Kuwabara, Yasuo [Kyushu Univ., Fukuoka (Japan). Hospital; Matsushima, Toshio; Fukui, Masashi

    2001-04-01

    Staging of moyamoya disease, based on angiography and PET have already been established. The authors have established staging of moyamoya disease based on perfusion SPECT, that can be summarized as follows: Stage I, no abnormality is seen at rest or after acetazolamide loading; Stage II, no abnormality is seen at rest, however, a decreased response (blood flow increase rate: <15%) is seen to acetazolamide loading (a, a decreased response is seen only in the frontal lobe; b, a decreased response is seen in regions other than the frontal lobe; and c, a decreased response is seen throughout the cerebrum); Stage III, localized decrease in blood flow (blood flow decrease compared with peripheral tissue: {>=}15%) and marked decrease in response to acetazolamide (blood flow increase rate: <5%) are seen at rest. In Stage III, CT and MRI show no abnormal findings or only mild lesions of the white matter; and Stage IV, multiple decreases in blood flow are seen at rest, and CT and MRI reveal infarctions and severe atrophy at the same sites. The above staging does not require determination of cerebral blood flow, and thus it can be used in children, in whom cerebral blood flow determination is difficult. The authors performed 99m-Tc ECD perfusion SPECT in 25 patients with moyamoya disease for the staging, and compared staging based on angiography with staging based on perfusion SPECT. The results did not show a correlation between the 2 staging methods. A problem inherent in the staging of moyamoya disease based on perfusion SPECT is that the relationship between cerebral blood flow and cerebral radioactivity concentrations may differ depending on the drug used to determine cerebral blood flow. Thus, although the present staging system does not depend on any specific radioactive drug to determine cerebral blood flow, further investigation is necessary to identify a more appropriate drug than those in current use. (K.H.)

  1. Development of moyamoya disease in pregnancy and puerperium: case report.

    Science.gov (United States)

    Akamatsu, Yosuke; Fujimura, Miki; Uenohara, Hiroshi; Shimizu, Hiroaki; Tominaga, Teiji

    2014-01-01

    Progressive moyamoya disease in pregnancy and puerperium has not been reported previously. Here, we present a 39-year-old woman who had been found to have moderate stenosis of right middle cerebral artery (MCA) 4 years prior to her pregnancy, finally suffering minor completed stroke due to progressive moyamoya disease at the early postpartum period. Three days after cesarean section without any complication, she developed cerebral infraction at right hemisphere, when magnetic resonance angiography indicated apparent progression of the proximal MCA stenosis. Catheter angiography demonstrated nearly occlusion of the right terminal internal carotid artery (ICA) and the development of an abnormal vascular network at the base of the brain as well as MCA stenosis, indicating a definitive diagnosis of moyamoya disease with unilateral involvement. The patient underwent superficial temporal artery-middle cerebral artery anastomosis 1 month after the onset of stroke, and she did not manifest as further neurological events during the follow-up period of 2 years. Moyamoya disease could newly develop in pregnancy and puerperium, which should be noted as a pitfall of the management of moyamoya disease with pregnancy.

  2. 37例成年烟雾病患者甲状腺功能的临床分析%Clinical Analysis of 37 Cases of Adult Patients with Thyroid Function Smoke

    Institute of Scientific and Technical Information of China (English)

    王亮

    2015-01-01

    目的:分析研究成年发病的烟雾病患者甲状腺功能和其临床特点。方法将本院2012年3月~2014年3月期间收治的37例烟雾病卒中患者,62例非烟雾病卒中患者和90例对照者的甲状腺功能进行比较,分析研究成年烟雾病的临床特点。结果烟雾病卒中组男13例,女24例,其比例为1:1.9,与另外两组比较,烟雾病卒中抗甲状腺微粒体抗体(anti-TPO)水平明显增高。每组间抗甲状腺蛋白抗体(anti-TG)和亚临床甲状腺疾病无明显差异。结论成年烟雾病卒中患者anti-TPO水平升高和FT3下降,且出血型较为常见,烟雾病的发病机制可能和anti-TPO有关。%Objective To analyze the incidence of smog in adult patients with thyroid function and clinical characteristics.Methods The hospital in March 2012 - March 2014 period 37 cases were treated stroke patients with Moyamoya disease, 62 cases of non-stroke patients with Moyamoya disease and 90 cases of thyroid function controls were compared, analyzed the clinical characteristics of adult moyamoya disease .Results Moyamoya disease stroke group 13 males and 24 females, a ratio of 1:1.9, compared with the other two groups, moyamoya disease stroke antithyroid microsomal antibodies (anti-TPO) levels were significantly increased.Conclusion Adult moyamoya disease stroke patients with elevated anti-TPO levels and FT3 decreased,and the blood is more common in the pathogenesis of moyamoya disease may and anti-TPO related.

  3. Surgical management of moyamoya disease: a review.

    Science.gov (United States)

    Baaj, Ali A; Agazzi, Siviero; Sayed, Zafar A; Toledo, Maria; Spetzler, Robert F; van Loveren, Harry

    2009-04-01

    Moyamoya disease (MMD) is a progressive, occlusive disease of the distal internal carotid arteries associated with secondary stenosis of the circle of Willis. Symptoms include ischemic infarcts in children and hemorrhages in adults. Bypass of the stenotic vessel(s) is the primary surgical treatment modality for MMD. Superficial temporal artery-to-middle cerebral artery bypass is the most common direct bypass method. Indirect techniques rely on the approximation of vascularized tissue to the cerebral cortex to promote neoangiogenesis. This tissue may be in the form of muscle, pericranium, dura, or even omentum. This review highlights the surgical options available for the treatment of MMD.

  4. MOYAMOYA INDUCED ACUTE PARAPLEGIA IN A CHILD WITH EPILEPSY

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    MM. Taghdiri

    2009-01-01

    Full Text Available ObjectiveMoyamoya disease (MMD is a chronic, occlusive, cerebrovascular disorder of unknown pathogenesis, characterized by progressive stenosis of the bilateral supraclinoid internal carotid arteries, with concomitant formation of tortuous arterial collateral vessels at the base of the brain, which reconstitute distal branches of the cerebral circulation. In Japanese, "Moyamoya" means "hazy puff of smoke" and refers to the angiographic appearance of the abnormal network of vessels that develop at the base of the brain and basal ganglia to supply a collateral route of blood flow. We report here the case of Moyamoya disease in a 5 year-old girl with normal mentality with a one year history of epilepsy, with Todd's paralysis. This condition is rare and most patients are diagnosed in childhood. With this report we aim to underscore the possibility that a usual neurological sign could be associated with unusual neurological disorders.

  5. Presentation of moyamoya disease with occipital hemorrhage: a case report

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    Serkan Demir

    2012-12-01

    Full Text Available Moyamoya disease is a cerebrovascular disease which is characterized with stenosis and occlusions at the distal part of internal carotid artery and at the proximal part of anterior and middle cerebral arteries. It rarely causes temporary or recurrent hemiparesis due to intracranial hemorrhage while symptoms like headache, convulsion, nystagmus, aphasia and ataxia may also occur. In this paper, we present a case of Moyamoya disease which was diagnosed with a 23 year old female patient who was admitted to our emergency department with headache, nausea and vomiting complaints and whose radiological findings showed occipital lobe hemorrhage.

  6. Moyamoya disease: diagnostic accuracy of MRI

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    Yamada, I. [Dept. of Radiology, Faculty of Medicine, Tokyo Medical and Dental Univ. (Japan); Suzuki, [Dept. of Radiology, Faculty of Medicine, Tokyo Medical and Dental Univ. (Japan); Matsushima, Y. [Dept. of Neurosurgery, Faculty of Medicine, Tokyo Medical and Dental Univ. (Japan)

    1995-07-01

    MRI may be employed to investigate moyamoya disease, since it provides vascular information without use of contrast medium. We reported the usefulness and limitations of MR angiography (MRA) in moyamoya disease. To our knowledge, no report has appeared dealing with the diagnostic accuracy of MRI in a large number of cases of moyamoya disease, although MRI is used more commonly than MRA. We therefore undertook to evaluate the accuracy of MRI in moyamoya disease. (orig.)

  7. Smith-Magenis syndrome and Moyamoya disease in a patient with del(17)(p11.2p13.1).

    Science.gov (United States)

    Girirajan, Santhosh; Mendoza-Londono, Roberto; Vlangos, Christopher N; Dupuis, Lucie; Nowak, Norma J; Bunyan, David J; Hatchwell, Eli; Elsea, Sarah H

    2007-05-01

    Chromosomal rearrangements causing microdeletions and microduplications are a major cause of congenital malformation and mental retardation. Because they are not visible by routine chromosome analysis, high resolution whole-genome technologies are required for the detection and diagnosis of small chromosomal abnormalities. Recently, array-comparative genomic hybridization (aCGH) and multiplex ligation-dependent probe amplification (MLPA) have been useful tools for the identification and mapping of deletions and duplications at higher resolution and throughput. Smith-Magenis syndrome (SMS) is a multiple congenital anomalies/mental retardation syndrome caused by deletion or mutation of the retinoic acid induced 1 (RAI1) gene and is often associated with a chromosome 17p11.2 deletion. We report here on the clinical and molecular analysis of a 10-year-old girl with SMS and moyamoya disease (occlusion of the circle of Willis). We have employed a combination of aCGH, FISH, and MLPA to characterize an approximately 6.3 Mb deletion spanning chromosome region 17p11.2-p13.1 in this patient, with the proximal breakpoint within the RAI1 gene. Further, investigation of the genomic architecture at the breakpoint intervals of this large deletion documented the presence of palindromic repeat elements that could potentially form recombination substrates leading to unequal crossover.

  8. Varied presentations of moyamoya disease in a tertiary care hospital of north-east India

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    Papori Borah

    2014-01-01

    Full Text Available Introduction: Moyamoya disease is a chronic progressive cerebrovascular disorder, characterized by stenosis or occlusion of bilateral internal carotid arteries (ICAs, anterior cerebral arteries (ACAs and middle cerebral arteries (MCAs, accompanied by a collateral network of vessels formed at the base of the brain. Ischemia and intracranial hemorrhage are the common typical manifestations. However moyamoya disease has been associated with atypical presentations like headache, seizures and involuntary movements. Although frequently reported from Asian countries like Japan, China and Korea, only few studies reported on clinical manifestations of moyamoya disease from India. Objectives: To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India. Material and Methods: Relevant investigations were done to rule out other causes of moyamoya syndrome. Results: We report 6 cases of moyamoya disease with varied presentations from a tertiary care referral government hospital. Case 1, 2 and 6 presented with alternating hemiparesis. Case 3 had amaurosis fugax. Case 4 had history suggestive of ischemic stroke and presented with hemichorea. Case 4 had focal seizure as the only manifestation. Cases 4 and 5 notably had stenosis of posterior cerebral artery (PCA in addition to stenosis of bilateral ICAs, ACAs and MCAs. Conclusion: Owing to its low incidence in India, moyamoya disease is easily overlooked as a possible diagnosis. However, because of its progressive nature, it is imperative to diagnose this disease early and offer surgical treatment to the patients.

  9. Steal phenomenon through the anterior communicating artery in Moyamoya disease

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    Lim, Soo Mee [Ewha Womans University, Department of Radiology, Mok-dong Hospital, College of Medicine, Seoul (Korea); Chae, Eun Jin; Kim, Min Yeong; Kim, Sang Joon; Choi, Choong Gon; Pyun, Hae Wook; Suh, Dae Chul [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea); Kim, Jae Kyun [Seoul Veterans Hospital, Department of Radiology, Seoul (Korea); Ahn, Jae Sung; Ra, Young-Shin [University of Ulsan, Asan Medical Center, Department of Neurosurgery, College of Medicine, Seoul (Korea); Kim, Jong-Uk; Hahm, Kyung Don [University of Ulsan, Asan Medical Center, Department of Anesthesiology, College of Medicine, Seoul (Korea)

    2007-01-15

    Branch occlusion of the anterior cerebral artery (ACA) is regarded as a part of Moyamoya disease. The purpose of this study is to define the ACA steal phenomenon (SP) in Moyamoya disease and to evaluate temporal changes according to the disease progression. From 139 Moyamoya patients we defined ACASP as narrowing of the ipsilateral A1-2 junction while preserving the anterior communicating artery and supplying the contralateral ACA cortical branches with the development of leptomeningeal collaterals by the ipsilateral middle cerebral artery into the hypoperfused ipsilateral ACA territory. Direction of the steal related to the stage in both hemispheres by Suzuki classification was statistically analyzed using the binomial test based on binomial distribution. Follow-ups of ACASP were evaluated in five patients. We identified ACASP in 13 (9%) patients (male:female=7:6, mean age 18 years, range: 2-58 years) of the 139 study patients. The presenting pattern was ischemic in 12 and hemorrhagic in one. The direction of SP occurred from the hemisphere in the lower to the higher stage of Suzuki classification (two-tail P value=0.0002). After revascularization surgery, ACASP disappeared or diminished. ACASP may occur in bilaterally different stages of Moyamoya disease as a transient self-adaptive process. It regresses after revascularization surgery. (orig.)

  10. Moyamoya disease : a review.

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    Gosalakkal J

    2002-01-01

    Full Text Available Moyamoya is probably an inherited vasocclusive disease first described in Japan and now reported from all over the world. Genetic linkage studies and study of the factors possibly involved in its pathogenesis have shed new light on this disease. There is some suggestion that the pathogenesis may vary between races. A number of revascularization procedures have been devised. There is still controversy regarding the timing and the type of surgery. Functional MRI and PET scanning may provide more objective criterion on the outcome of these procedures. Neuropsychological studies may also be of use in judging the benefits of surgery. In this review, some of the recent advances in the study of the genetics and pathogenesis of the disease are reviewed and the clinical manifestation and the role of surgery are discussed. Relative lack of literature from outside Japan and the Korean peninsula has hampered the understanding of the disease in other countries.

  11. A Case of Moyamoya-like Vessels with History of Brain Radiation

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    M Zare

    2004-08-01

    Full Text Available A 13 years old boy underwenta surgical operation of craniopharyngioma which followed by postoperative radiation therapy. Eighteen monthes later he was admitted to the neurological ward due to sudden sensory aphasia. Brain CT showed infarction of left hemisphere. Since no other predisposing factor was present, the cause of stroke in this patient can be related to Moyamoya - like disease after radiation. Keywords: Moyamoya, Stroke, Aphasia, Craniopharyngioma, Radiation Therapy

  12. Clinical manifestations and cerebral angiographic findings of moyamoya disease

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Objective To study the clinical features and angiographic findings of moyamoya disease (MMD) as well as their relationship. Methods A total of 22 MMD patients received routine digital substraction angiography (DSA). The clinical manifestations and angiographic findings were analyzed. Results Clinical manifestations varied and each patient often had multiple symptoms,including cerebral infarction in 9 patients with an average age of 23.6 (13-39 years) and cerebral hemorrhage in 7 patients with an average age...

  13. Indirect revascularization surgery for moyamoya disease in children and its special considerations

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    Kyu-Chang Wang

    2012-11-01

    Full Text Available Moyamoya disease (MMD is the most common pediatric cerebrovascular disease in Far Eastern countries. In children, MMD frequently manifests as ischemic symptomatology. Cerebral perfusion gradually decreases as the disease progresses, which often leads to cerebral infarction. The benefits of revascularization surgery, whether direct or indirect, have been well established in MMD patients with ischemic symptoms. In adults, the increase in cerebral blood flow achieved with indirect revascularization is often unsatisfactory, and direct revascularization is usually feasible. In children, however, direct revascularization is frequently technically not feasible, whereas the response to indirect revascularization is excellent, although 1 or 2 weeks are required for stabilization of symptoms. The authors describe surgical procedures and perioperative care in indirect revascularization for MMD. In addition, special considerations with regard to very young patients, patients with recent cerebral infarction, and patients with hyperthyroidism are discussed.

  14. MR angiography is a great boon for children with moyamoya disease

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    Ueda, Takashi; Wakisaka, Shinichiro; Kodama, Takao; Watanabe, Katsushi (Miyazaki Medical Coll., Kiyotake (Japan))

    1993-04-01

    Magnetic resonance angiography (MRA) films of 15 pediatric patients with moyamoya disease diagnosed with conventional angiography and of 11 children with suspected moyamoya disease were reviewed to evaluate the ability of MRA to demonstrate vascular abnormalities. We performed MRA with a superconductive 1.5-tesla MR system which employs a 3D-field echo technique based on the time-of-flight (TOF) effect. The typical MRA findings of moyamoya disease were; stenosis or obstruction of the terminal segment of the internal carotid arteries and the proximal segments of the anterior and middle cerebral arteries, plus well-developed basal moyamoya vessels, ophthalmic arteries, posterior cerebral arteries and leptomeningeal anastomoses. Retrograde filling through the superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis was visible, but fine vessels through the encephalo-myo-synangiosis were rarely seen. Stenosis of the artery was over-diagnosed because of the exaggerated turbulent flow seen in MRA. The natural course of asymptomatic, early stage, unilateral and hemorrhagic types of moyamoya disease and its pathophysiology are still unknown. MRA is a much better way to obtain anatomical and physiological information about the vascular structure and blood flow without any stress to the patient than is conventional angiography. Therefore, it promises to become a valuable alternative to conventional angiography not only for the diagnosis of moyamoya disease but also for treatment planning. (author).

  15. Endovascular treatment of basilar tip aneurysms associated with moyamoya disease

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    Arita, K.; Kurisu, K.; Ohba, S.; Shibukawa, M.; Kiura, H.; Sakamoto, S. [Department of Neurosurgery, Graduate School of Biomedical Science, Hiroshima University, 1-2-3 Kasumi, Minami-Ku, 734-8551, Hiroshima (Japan); Uozumi, T. [Hibino Hospital, Hiroshima (Japan); Nakahara, T. [Division of Neuroendovascular Treatment, Mazda Hospital, Hiroshima (Japan)

    2003-07-01

    We report the efficacy and safety of endovascular treatment of basilar tip aneurysms (BTA) in five patients with moyamoya disease. The patients underwent intra-aneurysmal embolisation with detachable platinum coils. Three BTA presented with subarachnoid haemorrhage (SAH); the other two were asymptomatic. In four cases, one embolisation procedure produced >95% angiographic obliteration of the aneurysm. In the other patient, 80-90% obliteration was achieved initially, but due to growth of the residual aneurysm, the procedure was repeated 7 months later. Two patients experienced transient oculomotor paresis as a procedure-related complication. Mean follow-up was 43.6{+-}34.0 months (range 8-92 months). One patient died of putaminal haemorrhage unrelated to the aneurysm 15 months after embolisation. The other four had no subsequent SAH and survived without sequelae. Endovascular embolisation using detachable platinum coils proved to be a safe and efficient treatment modality for BTA associated with moyamoya disease. (orig.)

  16. An evaluation of the cerebral hemodynamics in moyamoya disease with acetazolamide (Diamox) [sup 99m]Tc-HMPAO SPECT

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    Qin-Yi, Gao; Kuwabara, Yasuo; Ichiya, Yuichi (Kyushu Univ., Fukuoka (Japan). Faculty of Medicine) (and others)

    1994-08-01

    We studied the cerebral hemodynamics using Diamox [sup 99m]Tc-HMPAO SPECT in 9 patients with moyamoya disease, consisting of 4 children and 5 adults. Diamox [sup 99m]Tc-HMPO SPECT studies were performed by the double injection method. Hypoperfusion areas were scored by a visual inspection as follows: ++, definite positive; +, probable positive; -, negative. Diamox test was interpreted as positive when the score increased over one degree. In the resting state, 6 out of 9 patients showed definite or probable positive hypoperfusion, while all of 9 patients showed a similar finding after Diamox injection. The Diamox test was positive in 6 out of 9 patients. It was positive in all 3 patients who showed a negative hypoperfusion in the resting state. The regional cerebrovascular response to Diamox was most severely impaired in the frontal region. However, it was relatively well preserved in the cerebellum and thalamus according to the semiquantiative analysis. Thus, the Diamox HOPAO SPECT was considered to be useful in evaluating the hemodynamics in patients with moyamoya disease. (author).

  17. Diagnosis of moyamoya disease on magnetic resonance imaging: are flow voids in the basal ganglia an essential criterion for definitive diagnosis?

    Science.gov (United States)

    Mikami, Takeshi; Sugino, Toshiya; Ohtaki, Shunya; Houkin, Kiyohiro; Mikuni, Nobuhiro

    2013-08-01

    Flow voids in the basal ganglia cannot always be recognized on magnetic resonance imaging, even in patients with typical moyamoya disease. In this report, flow voids in the basal ganglia and cisternal flow voids of the sylvian valley were evaluated in patients with moyamoya disease, and their diagnostic value was verified. A total of 41 consecutive patients with moyamoya disease were included in this analysis. The number of flow voids in the basal ganglia and the sylvian valley were counted on each side by 3 observers. Then the numbers of flow voids were compared between the patients with moyamoya disease and controls. The patients with moyamoya disease had a significantly higher mean number of flow voids in the basal ganglia and the sylvian valley (P moyamoya disease. Comparative analysis using the area under the receiver operating curve indicated that the evaluation of flow voids in the sylvian valley was significantly superior method to that in the basal ganglia (P moyamoya disease was 6. Based on these findings, we recommend a definitive diagnosis of moyamoya disease should include assessment for abnormal vessels around the terminal portions of the internal carotid arteries.

  18. Fatal outcome in a Hispanic woman with moyamoya syndrome and Graves’ disease

    Science.gov (United States)

    Suthakar, Perin; Farmand, Farbod

    2016-01-01

    Summary We describe the case of a young Hispanic female who presented with thyrotoxicosis with seizures and ischemic stroke. She was diagnosed with a rare vasculopathy – moyamoya syndrome. After starting antithyroid therapy, her neurologic symptoms did not improve. Acute neurosurgical intervention had relieved her symptoms in the immediate post-operative period after re-anastomosis surgery. However, 2 post-operative days later, she was found to be in status epilepticus and in hyperthyroid state. She quickly deteriorated clinically and had expired a few days afterward. This is the second case in literature of a fatality in a patient with moyamoya syndrome and Graves’ disease. However, unlike the other case report, our patient had undergone successful revascularization surgery. We believe her underlying non-euthyroid state had potentiated her clinical deterioration. Case studies have shown positive correlation between uncontrolled hyperthyroidism and stroke-like symptoms in moyamoya syndrome. Mostly all patients with these two disease processes become symptomatic in marked hyperthyroid states. Thus, it may be either fluctuations in baseline thyroid function or thyrotoxicosis that potentiate otherwise asymptomatic moyamoya vasculopathy. Learning points: Awareness of the association between Graves’ disease and moyamoya syndrome in younger patients presenting with stroke-like symptoms. Obtaining euthyroid states before undergoing revascularization surgery may protect the patient from perioperative mortality and morbidity. Although moyamoya disease is usually thought to be genetically associated, there are reports that thyroid antibodies may play a role in its pathogenesis and have an autoimmune link. Fluctuations in baseline thyroid function for patients with known Graves’ disease may be a potentiating factor in exacerbating moyamoya vasculopathy. PMID:27857839

  19. Moyamoya: Report of a Pediatric Case

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    P. Karimzadeh

    2006-05-01

    Full Text Available Moyamoya (a Japanese term, meaning ‘hazy things’ was first described by Takeuchi in 1963. Two forms of this disease have been distinguished: 1-Primary moyamoya, or moyamoya dis-ease, with a strong hereditary predisposition and girls are more frequently affected. 2-Secondary moyamoya, or moyamoya syndrome, which is caused by a variety of underlying dis-eases. The Japanese scientists have classified moyamoya into four types: hemorrhagic, epileptic, infarct, and transient ischemic attack. Herein, we introduce an 8-years-old girl with the chief complaint of speech disorder. In her physical examination, we detected expressive aphasia and right-sided central facial palsy. After a few days, right hemiplegia and cortical blindness appeared as well. Gradually she was totally unable to move and was transferred to the ICU because of loss of consciousness. MRI showed diffuse hyper signal lesions in the left temporoparietal and bilateral occipital area. MRA showed narrowing of the internal carotid artery and abnormal collaterals (moyamoya vessels. After indirect bypass surgery (EDAS, she is now able to sit, walk, run and speak. There are rare angiographically proven moyamoya cases. To our knowledge this was the first EDAS in Iran and a rare case of moyamoya with a dramatic response to operation.

  20. Iodine-123 IMP SPECT before and after by-pass surgery in a patient with occlusion of left anterior and middle cerebral arteries with basal abnormal telangiectasis (unilateral Moyamoya disease)

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    Honda, Norinari; Machida, Kikuo; Takishima, Teruo; Kaizu, Hiroyuki; Sugimoto, Eiichi

    1987-09-01

    A case of left anterior and middle cerebral arterial occlusion with angiographic features similar to Moyamoya disease was reported. IMP SPECT of the patient revealed the success of by-pass surgery clearly. The patient complained of transient right hemiparesis with aphasia 4 times. The cerebral arteriography disclosed occlusions of left anterior and middle cerebral arteries at their proximal portions. Right internal carotid and its branches were normal. I-123 IMP SPECT study showed hypoperfusion in left temporal lobe, basal ganglia with incomplete reperfusion on the delayed (4 hours after injection) SPECT images. After the superficial temporal-middle cerebral artery anastomosis, I-123 IMP SPECT showed improvement of the brain blood flow. I-123 IMP SPECT was very useful in detecting the ischemic areas and evaluating the revascularizing surgery in this case.

  1. Endovascular therapy of ruptured distal anterior choroidal artery aneurysm associated with moyamoya pattern collateralization secondary to middle cerebral artery occlusion

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    Hidenori Oishi

    2013-01-01

    Full Text Available We report a case of a ruptured distal anterior choroidal artery (AChoA aneurysm associated with moyamoya pattern collateralization secondary to the middle cerebral artery occlusion. Patient was successfully treated with the coil embolization of the distal AChoA. This case supports the feasibility and efficacy of the endovascular therapy for the distal AChoA aneurysms in patients with MCA occlusion with moyamoya pattern collateralization.

  2. Leptomeningeal contrast enhancement in moyamoya: its potential role in postoperative assessment of circulation through the bypass

    Energy Technology Data Exchange (ETDEWEB)

    Komiyama, M.; Nakajima, H.; Nishikawa, M.; Yasui, T. [Dept. of Neurosurgery, Osaka City General Hospital (Japan); Kitano, S.; Sakamoto, H. [Dept. of Paediatric Neurosurgery, Osaka City General Hospital (Japan)

    2001-01-01

    Leptomeningeal contrast enhancement (LMCE) is one of the MRI features of moyamoya. Its clinical significance, however, is not elucidated. Our purpose was to characterise LMCE on MRI and to evaluate its role in the assessment of circulation through a surgically established bypass in moyamoya. We studied 16 patients with idiopathic moyamoya (seven males, nine females, includingt four children, aged 7 to 54 years, mean 24 years) who underwent T1-weighted MRI before and after intravenous contrast medium. The presence of LMCE, its intensity and anatomical distribution, catheter angiographic findings, and relation of LMCE to the bypass surgery were assessed. More LMCE was seen in the cerebrum in most patients with moyamoya than in normal controls. LMCE in the brain stem and cerebellum was minimal, similar to that seen in the controls. LMCE was less prominent following surgery than before operation or in patients who did not undergo surgery. In three patients examined both before and after operation LMCE became less prominent following bypass surgery. As LMCE becomes less prominent after ''effective'' bypass surgery, this may be used for evaluation of effectiveness of surgery in moyamoya. (orig.)

  3. The diagnostic accuracy of time-of-flight MR angiography in diagnosing Moyamoya disease

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    Shono, Tadahisa; Ikezaki, Kiyonobu; Matsushima, Toshio; Inoue, Tooru; Fujii, Kiyotaka; Fukui, Masashi [Kyushu Univ., Fukuoka (Japan). Faculty of Medicine

    1995-09-01

    Conventional cerebral angiography is essential for achieving the diagnosis of Moyamoya disease. It is, however, an invasive examination, especially for the pediatric patients. In this paper, we report on the results of an investigation as to whether MRA is able to accurately visualize the steno-occlusive changes in the carotid fork and the basal moyamoya vessels less invasively. Twenty patients among them, 16 children who were diagnosed as having Moyamoya disease with conventional angiography underwent MRI and MRA using a 1.5T MR unit (Signa, G.E.). Angiographically, 37 sides were determined to be in stage 3. MRA was performed using two-dimensional time-of-flight (2D-TOF), 3D-TOF, and gadolinium (Gd)-enhanced 3D-TOF methods. The 3D-TOF MRAs showed almost equal sensitivity to that of conventional angiography for the detection of steno-occlusive changes in the carotid fork. However, it was difficult to visualize the moyamoya vessels clearly. Further, 3D-TOF, 2D-TOF, and Gd-enhanced 3D-TOF methods depicted the moyamoya vessels in 65%, 79%, and 80% of the examined sides, respectively. In contrast, MRI visualized the moyamoya vessels as multiple flow voids in the basal ganglia in 38 of 40 sides (95%). Based on the above findings, it was thus concluded that approximately 90% of the stage 3 Moyamoya disease cases could be diagnosed by a combination of 3D-TOF MRA and MRI without the use of conventional angiography. (author).

  4. Diagnosis of moyamoya disease using 3-T MRI and MRA: value of cisternal moyamoya vessels

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    Sawada, Takeshi; Yamamoto, Akira; Okada, Tomohisa; Kanagaki, Mitsunori; Kasahara, Seiko; Togashi, Kaori [Kyoto University, Department of Diagnostic Imaging and Nuclear Medicine, Graduate School of Medicine, Kyoto (Japan); Miki, Yukio [Osaka City University, Department of Radiology, Graduate School of Medicine, Osaka (Japan); Kikuta, Ken-ichiro [Fukui University, Division of Neurosurgery, Department of Sensory and Locomotor Medicine, Faculty of Medical Sciences, Fukui (Japan); Miyamoto, Susumu; Takahashi, Jun C. [Kyoto University, Department of Neurosurgery, Graduate School of Medicine, Kyoto (Japan); Fukuyama, Hidenao [Kyoto University, Human Brain Research Center, Graduate School of Medicine, Kyoto (Japan)

    2012-10-15

    The purpose of this study was to propose new magnetic resonance (MR) criteria of diagnosing moyamoya disease (MMD) from cisternal moyamoya vessels (MMVs) on 3-T magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and compare the diagnostic accuracy of the existing MR criteria and the proposed MR criteria. Participants comprised 20 consecutive patients with MMD (4 males, 16 females) diagnosed clinically using conventional angiography and 20 controls (13 male and 7 female arteriosclerosis patients). In these participants, 3-T MRI/MRA was evaluated by the existing MR criteria, which use MMVs in the basal ganglia, and the proposed MR criteria, which use cisternal MMVs, and then these two criteria were statistically compared by McNemar's test. Diagnostic accuracy was 62.5% with the existing MR criteria and 97.5% with the proposed MR criteria. The proposed MR criteria was more sensitive (1.00) than the existing MR criteria (0.45), but less specific (0.95) than the existing MR criteria (1.00). The proposed MR criteria using cisternal MMVs showed significantly higher diagnostic accuracy than the existing MR criteria. We believe that our proposed MR criteria will be beneficial for diagnosing MMD. (orig.)

  5. Characterization of inpatient moyamoya in the United States: 1988-2004

    Directory of Open Access Journals (Sweden)

    Darrin J Lee

    2011-07-01

    Full Text Available Background and Purpose: Moyamoya disease has been classically described by the Asian experience, yet clinical aspects of moyamoya phenomena in the United States remain vastly undefined. The multifocal occlusive arterial disorder may be linked with numerous conditions; however, later stages of this syndrome share common vascular pathophysiology. This study is aimed at characterizing inpatient moyamoya cases in the United States over a broad time span.Methods: A comprehensive analysis of the Nationwide Inpatient Sample of the Healthcare Cost and Utilization Project (Releases 1-13, 1988-2004 based on ICD-9-CM code 437.5 was performed. Annual percentages and trends analyses were conducted for demographic variables, admission characteristics, co-morbidities, and procedures.Results: 2247 admissions of moyamoya cases were analyzed from a wide geographic distribution throughout the United States between 1988 and 2004. Age at admission varied considerably (mean 29.6 ± 18.5 years, affecting women more frequently than men (61.9%. Various racial groups were identified (35.4% White, 19.7% African American, 5.6% Hispanic, 8.3% Asian or Pacific Islander, 1.4% Native American. Admissions were typically emergent (38.8% or urgent (18.7%, although elective admissions occurred (24.4%. Aside from moyamoya, sickle cell disease was diagnosed in 13.6%, ischemic stroke in 20.7%, intracerebral hemorrhage in 7.4%, TIA in 3.4%, and subarachnoid hemorrhage in 3.1%. Cerebral angiography was performed in 24.9% while extracranial-intracranial bypass was done in 8.4% of patients. Conclusions: Moyamoya in the United States is a heterogeneous condition affecting individuals of all ages across a diverse racial spectrum and wide geographic distribution. Further recognition of moyamoya syndrome may facilitate ongoing research and future therapeutic approaches.

  6. Leptomeningeal high signal intensity (ivy sign) on fluid-attenuated inversion-recovery (FLAIR) MR images in moyamoya disease

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    Fujiwara, Hirokazu [Department of Diagnostic Radiology, School of Medicine, Keio University, Tokyo 1608582 (Japan)]. E-mail: hirokazu_fujiwara@ybb.ne.jp; Momoshima, Suketaka [Department of Diagnostic Radiology, School of Medicine, Keio University, Tokyo 1608582 (Japan); Kuribayashi, Sachio [Department of Diagnostic Radiology, School of Medicine, Keio University, Tokyo 1608582 (Japan)

    2005-08-01

    Purpose: There are a few reports on leptomeningeal high signal intensity (LMHI: ivy sign) on fluid-attenuated inversion-recovery (FLAIR) images in moyamoya disease, but the feature of this finding has not been completely understood. The purpose of this study was to characterize LMHI on FLAIR images in moyamoya disease and to assess usefulness of this finding in the diagnosis of moyamoya disease in conventional MR imaging. Material and methods: MR imaging of 28 patients with moyamoya disease was retrospectively reviewed. The grade of LMHI on FLAIR images was classified as 'absent,' 'minimal,' 'moderate' and 'marked.' Fifty-four hemispheres of 28 patients (2 patients had unilateral disease) were assessed for the frequency of visualization and distribution of LMHI. The correlations between LMHI on FLAIR images, moyamoya vessels on T1- and T2-weighted images and MR angiography findings were also analyzed. Results: Moderate and marked LMHI was seen in 31 out of 54 hemispheres (57%). LMHI was seen more prominently in the frontal and parietal lobes than in the temporal and occipital lobes. Although there was a tendency for LMHI on FLAIR images to be prominent in groups with moderate and marked moyamoya vessels on T1- and T2-weighted images, there was no significant correlation. More prominent LMHI was observed in the hemispheres in which cortical branches of the middle cerebral arteries were poorly visualized on MR angiography. Conclusion: Leptomeningeal high signal intensity (ivy sign) on FLAIR images is predominantly seen in the frontal and parietal lobes. Because this sign can be seen in patients with unremarkable moyamoya vessels, LMHI is a useful sign in conventional MR imaging for the diagnosis of moyamoya disease.

  7. A rare case of moyamoya disease in a 20-year-old Puerto Rican female U.S. soldier.

    Science.gov (United States)

    Busey, Blake; Berry-Cabán, Cristóbal S; Hoedebecke, Kyle; Barts, Rachel N

    2014-12-01

    Moyamoya disease is a progressive, occlusive pathology involving the cerebral vasculature with particular involvement of the circle of Willis and its tributaries. The cause of moyamoya disease is unknown, but is believed to be hereditary. Females 20 to 39 years old with moyamoya represent 0.5% of all acute cerebral ischemia and infarcts with risk factors including smoking, estrogen-containing birth control use, coagulopathy, neoplasm, and congenital malformation. This case reports on a 20-year-old Puerto Rican female U.S. soldier with a 1-year history of migraine headaches with worsening right retro-orbital pain, blurred vision, and photophobia. The patient had minimal unilateral neurological deficits despite evidence of significant cerebral infarction on non-contrast computed tomography. Other neuroimaging findings were consistent with moyamoya disease with confirmation via cerebral angiography. This case details the process of diagnosis and treatment as well as discussing its incidence, identification, and treatment options.

  8. Germline mutation of CBL is associated with moyamoya disease in a child with juvenile myelomonocytic leukemia and Noonan syndrome-like disorder.

    Science.gov (United States)

    Hyakuna, Nobuyuki; Muramatsu, Hideki; Higa, Takeshi; Chinen, Yasutsugu; Wang, Xinan; Kojima, Seiji

    2015-03-01

    Germline mutations in CBL have been identified in patients with Noonan syndrome-like phenotypes, while juvenile myelomonocytic leukemia (JMML) harbors duplication of a germline CBL, resulting in acquired isodisomy. The association between moyamoya disease and Noonan syndrome carrying a PTPN11 mutation has recently been reported. We present a patient with JMML who developed moyamoya disease and neovascular glaucoma. Our patient exhibited a Noonan syndrome-like phenotype. Genetic analysis revealed acquired isodisomy and a germline heterozygous mutation in CBL. This is a rare case of CBL mutation associated with moyamoya disease. Prolonged RAS pathway signaling may cause disruption of cerebrovascular development.

  9. Fluctuating Hemiparesis Secondary to Moyamoya Phenomenon in a Child with Down Syndrome: a case report.

    Science.gov (United States)

    Rison, Richard A

    2008-10-15

    Moyamoya phenomenon is a term used to describe extensive collateralization of the circle of Willis arteries associated with severe unilateral or bilateral internal carotid artery stenosis or occlusion in the presence of certain conditions. Down syndrome is among these conditions. A case is reported of a young girl with Down syndrome who presented with fluctuating right-sided weakness and facial droop found to have cerebral ischemia. Subsequent investigations disclosed characteristic "puff of smoke" patterns on angiographic studies consistent with moyamoya phenomenon. The patient was initially treated with aspirin and eventually underwent an encephalomyosynangiosis. This young patient with Down syndrome and moyamoya phenomenon serves as a reminder of the association between these two conditions.

  10. Angiographic profile in childhood moyamoya disease; A study of 8 caucasian Indian children

    Energy Technology Data Exchange (ETDEWEB)

    Jayakumar, P.N.; Arya, B.Y.T.; Vasudev, M.K. (National Inst. of Mental Health and Neuro Sciences, Bangalore (India). Dept. of Neuroradiology)

    1991-11-01

    The cerebral angiograms of 8 patients with childhood moyamoya disease showed that the common findings were stenosis/occlusion of the supraclinoid internal carotid artery and the proximal segments of the anterior and middle cerebral arteries and basal moyamoya. The volume of basal moyamoya and its collateral supply depended upon the stage of the disease. Leptomeningeal collaterals were frequent in the later stages. Stenotic lesions in the posterior circulation were seen in a majority (75%) of patients. A feature unique to the study was evidence of intracranial small-vessel disease and stenotic cervical internal carotid artery in half of the cases. The disease in the ethnic caucasian Indians seems largely similar to the classical disease frequently reported in the Japanese literature. (orig.).

  11. Angiographic profile in childhood moyamoya disease. A study of 8 Caucasian Indian children.

    Science.gov (United States)

    Jayakumar, P N; Arya, B Y; Vasudev, M K

    1991-11-01

    The cerebral angiograms of 8 patients with childhood moyamoya disease showed that the common findings were stenosis/occlusion of the supraclinoid internal carotid artery and the proximal segments of the anterior and middle cerebral arteries and basal moyamoya. The volume of basal moyamoya and its collateral supply depended upon the stage of the disease. Leptomeningeal collaterals were frequent in the later stages. Stenotic lesions in the posterior circulation were seen in a majority (75%) of patients. A feature unique to the study was evidence of intracranial small-vessel disease and stenotic cervical internal carotid artery in half of the cases. The disease in the ethnic caucasian Indians seems largely similar to the classical disease frequently reported in the Japanese literature.

  12. Moyamoya syndrome: impaired hemodynamics on ECD SPECT after EEG controlled hyperventilation

    Energy Technology Data Exchange (ETDEWEB)

    Matheja, P.; Weckesser, M.; Franzius, C.; Loettgen, J.; Schober, O. [Muenster Univ. (Germany). Dept. of Nuclear Medicine; Debus, O.; Kurlemann, G. [Dept. of Neuropediatrics, Muenster Univ. (Germany)

    2002-02-01

    Background and purpose: Ischemic symptoms in children with Moyamoya syndrome are typically provoked by hyperventilation (HV) and are accompanied by the ''re-build-up'' phenomenon in EEG. The value of scintigraphic detection of HV-provoked perfusion deficits remains to be elucidated. Patients and methods: In seven children with Moyamoya syndrome regional cerebral blood flow was assessed by {sup 99m}Tc-ethyl-cysteine-dimer (ECD) single photon emission computed tomography (SPECT) after HV and under baseline conditions to identify ischemia prone regions. Results: Regional marked hypoperfusion after HV was found in all patients. Predominant perfusion deficits were detected in the frontal lobes. Conclusion: ECD SPECT is a potential tool for the preoperative evaluation of cerebral hemodynamics and for monitoring angiosurgical therapies in Moyamoya disease. (orig.)

  13. Selective and superselective angiography of pediatric moyamoya disease angioarchitecture: the anterior circulation.

    Science.gov (United States)

    Baltsavias, Gerasimos; Valavanis, Anton; Filipce, Venko; Khan, Nadia

    2014-01-01

    The angioarchitecture of the so-called moyamoya vessels in children has not been explicitly analyzed. We aimed to investigate the precise anatomy of the vascular anastomotic networks in patients with childhood moyamoya disease. Six children diagnosed with moyamoya disease for the first time underwent an angiographic investigation with selective and superselective injections. We recorded the arterial branches feeding the moyamoya anastomotic networks, their connections and the recipient vessels. Depending on the level of the steno-occlusive lesion, the feeding vessels included the medial striate arteries, the perforators of the choroidal segment of the carotid, the uncal artery, the medial and lateral branches of the intraventricular segment of the anterior choroidal artery, perforators of the communicating segment, the superior hypophyseal arteries, the prechiasmal branches of the ophthalmic artery, the ethmoidal arteries and the dural branches of the cavernous carotid. Through connections, which are described, the recipient vessels were the lateral striate arteries and the middle cerebral, the medial striate arteries and the anterior cerebral, medullary arteries around the ventricular system, anterior temporal branches of the middle cerebral, orbitofrontal and frontopolar branches of the anterior cerebral, as well as other cortical branches of the anterior and middle cerebral territories. The use of high quality selective and superselective angiography enabled us to clearly demonstrate for the first time aspects of the microangiographic anatomy of the moyamoya anastomotic network previously only vaguely or incompletely described.

  14. Selective and superselective angiography of pediatric moyamoya disease angioarchitecture in the posterior circulation.

    Science.gov (United States)

    Baltsavias, Gerasimos; Khan, Nadia; Filipce, Venko; Valavanis, Anton

    2014-01-01

    The anastomotic network of the posterior circulation in children with moyamoya disease has not been analyzed. We aimed to investigate the angiographic anatomy of this unique vascular network in patients with childhood moyamoya disease. Selective and superselective injections of the posterior circulation were performed in six children with newly diagnosed moyamoya disease. The arterial branches feeding the moyamoya anastomotic network, their connections and the recipient vessels were demonstrated. Depending on the level of the steno-occlusive lesion, the feeding vessels were the thalamoperforators, the posterior choroidals, the splenic artery, parietoccipital artery, other cortical posterior cerebral artery (PCA) branches, the dural branch of the PCA, the premamillary artery and other posterior communicating artery perforators. Through connections, which are described, the recipient vessels were the striate and medullary arteries, other thalamic arteries with or without medullary extensions, the pericallosal artery, medial parietoccipital cortical branches of the PCA and the anterior choroidal artery. High quality selective and superselective angiography helped in demonstrating the angiographic anatomy of the moyamoya posterior anastomotic network previously either vaguely or incompletely described, as well as connections within the posterior circulation but also its relevance as a collateral to the anterior circulation.

  15. Moyamoya disease: Clinical findings and radiological approach; Klinische Symptomatik und bildgebende Diagnostik bei der Moyamoya-Erkrankung

    Energy Technology Data Exchange (ETDEWEB)

    Christopoulos, G.; Coblenz, G.; Wedell, E.; Schmitt, R. [Herz- und Gefaessklinik GmbH, Bad Neustadt a.d. Saale (Germany). Inst. fuer Diagnostische und Interventionelle Radiologie; Ziegler, V.; Griewing, B. [Neurologische Klinik GmbH, Bad Neustadt a.d. Saale (Germany)

    2003-07-01

    Objective: Presentation of typical imaging findings and diagnostic approaches in patients suspected of moyamoya disease. Materials and methods: Two female patients (24 resp. 44 years old) presenting with equivocal neurological symptoms (headache, recurrent monoparesis, choreiformic ataxia, grand mal seizure) were examined by means of colour-coded ultrasound, selective catheter-based angiography (DSA), CT and MRI including MR angiography. Results: Cranial CT and MRI showed normal findings in one patient, and focal cerebral infarctions of different age in the other. In both cases, coulour-coded ultrasound was indicative in detecting occlusive disease of the internal carotid arteries. Both, MR angiography as well as catheter-based angiography revealed the pathology of the culprid vessel, in addition catheter-based angiography was more accurate in depicting moyamoya collaterals at the skull base. Conclusion: In moyamoya disease coulour-coded ultrasound is diagnostic for the lesion of the internal carotid artery, MRI resp. MRA depict the vascular and parenchymal lesion, whereas catheter-based DSA by providing information about the main vessel and collateral system allows the staging of the disease. (orig.) [German] Ziel: Praesentation der charakteristischen bildgebenden Befunde und des Diagnosealgorithmus beim Verdacht auf eine Moyamoya-Erkrankung. Material und Methode: Zwei 24 bzw. 44 Jahre alte Patientinnen mit unspezifischen neurologischen Symptomen (Zephalgien, rezidivierende Monoparesen, choreiforme Bewegungsstoerungen bzw. Grand-mal-Anfall) wurden mit den bildgebenden Verfahren der Doppler-/Duplex-Sonographie, der selektiven DSA, der CT und MRT einschliesslich MR-Angiographie untersucht. Ergebnisse: Die kranielle CT und MRT erbrachten bei der einen Patientin einen unauffaelligen Befund, bei der anderen Patientin fokale Infarktareale unterschiedlichen Alters. In beiden Faellen war die Doppler-/Duplex-Sonographie mit dem Nachweis stenosierter bzw. okkludierter Aa

  16. MR angiography in moyamoya disease under the different magnetic field strengths

    Energy Technology Data Exchange (ETDEWEB)

    Mizoguchi, Masahiro; Matsushima, Toshio; Ikezaki, Kiyonobu; Inoue, Tooru; Fukui, Masashi; Hasuo, Kanehiro [Kyushu Univ., Fukuoka (Japan). Faculty of Medicine; Nakayama, Taku

    1995-06-01

    MR angiography (MRA) has been developing rapidly as a clinically useful modality. Currently, MRA studies are performed with the range of 0.5 to 1.5T MR units. We compared the MRA findings obtained under different magnetic field strengths in eight patients (16 sides) with moyamoya disease in order to evaluate their accuracy in comparison with the conventional cerebral angiographic findings on these individuals. 3D-TOF MRA with 1.5T demonstrated almost the same steno-occlusive changes of the main trunk of Willis` circle as did conventional cerebral angiography. However, 0.5T MRA tended to overestimate these changes as occlusive lesions. Basal moyamoya vessels were detected by 1.5T 3D-TOF MRA in 12 of 16 sides (75%) and in 7 of 16 sides (44%) by 0.5T 3D-TOF MRA. We could diagnose moyamoya disease in 50% of the patients with 1.5T and in 25% of them with 0.5T 3D-TOF MRA alone. In combination with other imaging methods, such as 2D-TOF and PC, the rate of diagnoses increased to up to 75% and 38% with 1.5T and 0.5T MR units, respectively. MRA with higher magnetic field strength had greater sensitivity and could detect more accurately two different vascular abnormalities such as steno-occlusive changes and basal moyamoya vessels in moyamoya disease. One third of our patients, however, could be diagnosed as having moyamoya disease with 0.5T MRA alone. (author).

  17. Moyamoya disease and sagittal sinus thrombosis in a child with Down's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Del-Rio Camacho, G.; Leal Orozco, A.; Camino Lopez, M.; Ruiz-Moreno, M. [Dept. of Paediatrics, Fundacion Jimenez Diaz, Madrid (Spain); Perez-Higueras, A.; Al-Assir, I. [Dept. of Neuroradiology, Fundacion Jimenez Diaz, Madrid (Spain)

    2001-02-01

    A girl with Down's syndrome, moyamoya disease and sagittal sinus thrombosis is described. She was diagnosed after acute neurological deterioration by MRI and angiography. Recombinant tissue plasminogen activator (r-TPA) was injected locally to recanalise the thrombus. The patient's condition significantly improved and she was discharged. After 2 years of follow-up the child remains asymptomatic. Moyamoya syndrome and cerebral venous thrombosis should not be overlooked as a cause of acute neurological deterioration in a child with Down's syndrome. MRA appears to be a safe and accurate alternative to traditional angiography for the diagnosis of moyamoya disease. Local fibrinolysis with r-TPA is the treatment of choice for cerebral venous thrombosis due to its safety and efficacy. (orig.)

  18. The Rare Association of Moyamoya Disease and Cerebral Arteriovenous Malformations: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Wu, Te Chang [Chi-Mei Foundation Hospital, Tainan (China); Guo, Wan Yuo; Wu, Hsiu Mei; Chang, Feng Chi; Shiau, Cheng Ying; Chung, Wen Yuh [Taipei Veterans General Hospital, Taipei (China)

    2008-07-15

    A 36-year-old man was diagnosed with a right temporal lobe grade II cerebral arteriovenous malformation (cAVM) and was treated with radiosurgery. At nine months after the cAVM radiosurgery, the patient began to develop bilateral focal narrowing at the M1 segments of the bilateral middle cerebral arteries. The narrowing progressively deteriorated as was demonstrated on longitudinal serial follow- up MR imaging. X-ray angiography performed at 51 months after radiosurgery confirmed that the cAVM was cured and a diagnosis of moyamoya disease. To the best of our knowledge, this is the first case of cAVM-associated moyamoya disease that developed after radiosurgery. Given the chronological sequence of disease development and radiation dose distribution of radiosurgery, it is proposed that humoral or unknown predisposing factors, rather than direct radiation effects, are the cause of moyamoya disease associated with cAVM.

  19. Focal time-to-peak changes on perfusion MRI in children with Moyamoya disease: correlation with conventional angiography

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Hyun Seok (Dept. of Radiology, Yonsei Univ. College of Medicine, Seoul (Korea, Republic of); Dept. of Radiology, Seoul St Mary' s Hospital, College of Medicine, The Catholic Univ. of Korea, Seoul (Korea, Republic of)); Kim, Dong-Seok; Shim, Kyu-Won (Dept. of Neurosurgery, Yonsei Univ. College of Medicine, Seoul (Korea, Republic of)); Kim, Jinna; Kim, Eun Soo; Lee, Seung-Koo (Dept. of Radiology, Yonsei Univ. College of Medicine, Seoul (Korea, Republic of)), email: slee@yuhs.ac

    2011-07-15

    Background: Moyamoya disease is a chronic progressive steno-occlusion of the distal internal carotid arteries with unknown etiology. As the classical presentation of childhood Moyamoya disease is ischemic stroke, cerebral hemodynamic evaluation is important for patient selection for surgery to prevent recurrent ischemic attacks. Perfusion MR imaging has been applied to evaluate cerebral hemodynamics. Purpose: To correlate the 'basal time-to-peak preservation sign', 'auto-synangiosis sign', and 'posterior involvement sign' on time-to-peak map of perfusion MRI with catheter angiography. Material and Methods: Thirty-four children (6.91 +- 3.08 years) with Moyamoya disease who underwent both perfusion-weighted MRI and catheter angiography were enrolled in this study. Given catheter angiography as a reference standard, basal time-to-peak preservation sign, auto-synangiosis sign, and posterior involvement sign were evaluated on time-to-peak maps. Results: The basal time-to-peak preservation sign was accurate for the diagnosis of childhood Moyamoya disease; both sensitivity and specificity were 100%. The auto-synangiosis sign showed lower sensitivity (65%), however, with an acceptable specificity (98%). The posterior involvement sign showed lower sensitivity (61%) but had an acceptable specificity (96%). Conclusion: The basal time-to-peak preservation sign may be a universal finding in childhood Moyamoya disease. The auto-synangiosis and posterior involvement sign may be useful in determining transdural collateral status and posterior circulation involvement in childhood Moyamoya disease

  20. Assessment of Cerebral Hemodynamics by 64-Row VCT Perfusion Imaging in Patients with Moya-Moya Disease%64排VCT脑灌注成像对烟雾病的血流动力学研究

    Institute of Scientific and Technical Information of China (English)

    谢安明; 丁耀军

    2014-01-01

    Objective To investigate the changes in cerebral hemodynamics (cerebral blood flow volume,blood capacity,mean transit time and time to peak)using 64-row VCT perfusion im-aging,and to provide imaging evidence for clinical intervention.Methods Thirty patients with moya-moya disease aged 13-49 years (average age 33.8 years,14 males,16 females)were recruited in this study.Cerebral perfusion imaging was performed using a GE LightSpeed VCT scanner at a rate of 5 mL/second and a dose of 50 mL.The scan delay time was 5 seconds and scan time was 45 seconds.Post-processing was conducted with Perfusion 4 software to analyze cerebral blood flow volume,blood capacity,mean transit time and time to peak.Results Of the 30 patients,9 had is-chemic type moya-moya disease and 21 had hemorrhagic type moya-moya disease.The 64-row VCT perfusion imaging showed increased regional cerebral blood flow in 3 patients,decreased re-gional cerebral blood flow in 5,increased regional cerebral blood volume in 3,normal cerebral blood volume in 27,shortened mean transit time and time to peak in 3,and prolonged mean transit time and time to peak in 8.Conclusion Most patients with ischemic type moya-moya disease are characterized by regional hypoperfusion,while patients with hemorrhagic type moya-moya disease are characterized by normal or regional hyperperfusion.The mean transit time and time to peakare sensitive indicators of changes in cerebral hemodynamics.%目的:通过对烟雾病患者进行64排VCT脑灌注成像,了解其脑血流动力学变化情况,包括脑血流量、脑血容量、平均通过时间及达峰时间,为临床干预治疗提供影像学依据。方法30例烟雾病患者,男性14例,女性16例,年龄最大为49岁,最小为13岁,平均年龄为33.8岁。利用 GE公司生产的 LightSpeed VCT螺旋扫描机对烟雾病患者进行灌注扫描,速率5 mL·s-1、剂量50 mL、扫描延迟5 s、扫描时间45 s,然后用该公司配备的 Perfu-sion4

  1. 烟雾病合并甲状腺功能亢进症患者的临床分析%Clinical analysis of patients with moyamoya disease complicating hyperthyroidism

    Institute of Scientific and Technical Information of China (English)

    李德生; 刘鹏; 暴向阳; 杨伟中; 张正善; 咸鹏; 杨日淼; 段炼

    2011-01-01

    Objective To investigate the clinical features, pathogenesis and the effect of surgical and medical treatment of patients with moyamoya disease complicating hyperthyroidism. Methods The clinical data of 11 patients with moyamoya disease complicating hyperthyroidism were analyzed retrospec-tively, 10 of them were diagnosed by digital substraction angiography ( DSA), and 1 was diagnosed by magnetic resonance angiography. All the patients met the diagnostic criteria of hyperthyroidism. After con-trolling hyperthyroidism, 8 patients underwent encephalo-duro-arterio-synangiosis, the other 3 were only given drugs to control hyperthyroidism. Results ①Ten of the 11 patients (8 females) showed thyroid dysfunction, with increased 3,5,3-triiodothyronine (T3) and thyroxine (T4), and the decreased thyroid stimulating hormone (TSH). ②Nine of the 11 patients showed cerebral infarction, 1 showed intraventricu-lar hemorrhage and 1 did not found any abnormality in imaging findings. Ten patients had bilateral lesions and 1 had unilateral lesions. ③Eleven patients were followed-up for 11-102 months. Except 1 patient left mental decline and slow reaction, the symptoms of others were relieved or disappeared after the treatment. After encephalo-duro-arterio-synangiosis, 8 patients did not have any surgical complications, 6 of them con-ducted postoperative angiography (10-26 months) and all showed a great deal of compensatory blood supply from extracranial to intracranial arteries. Conclusion Moyamoya disease complicating hyperthyroidism is mostly occured in female patients and their clinical symptoms are mainly cerebral infarction. The pathogenesis of moyamoya disease complicating hyperthyroidism remains unclear. It may be associated with a variety of ge-netic and immune factors. After controlling hyperthyroidism, surgical treatment can establish effective collat-eral circulation and reduce the risk of recurrence of stroke.%目的 探讨烟雾病合并甲状腺功能亢进症(简

  2. Simultaneous occurrence of diabetic ketoacidosis, thyroid storm, and multiple cerebral infarctions due to Moyamoya disease.

    Science.gov (United States)

    Noh, Byoungho H; Cho, Sang-Won; Ahn, Sung Yeon

    2016-02-01

    Diabetic ketoacidosis (DKA) is one of the precipitating factors that can evoke a thyroid storm. Thyroid storm may cause cerebral ischemia in Moyamoya disease, which can coexist in patients with Graves' disease. A 16-year-old girl complaining of dizziness and palpitations visited the emergency department and was diagnosed with DKA combined with hyperthyroidism. A thyroid storm occurred 6 h after the start of DKA management. Her Burch and Wartofsky score was 65 points. Right hemiplegia developed during the thyroid storm, and brain magnetic resonance (MR) diffusion-weighted images revealed multiple acute infarcts in both hemispheres. MR angiography showed stenosis of both distal internal carotid arteries and both M1 portions of the middle cerebral arteries, consistent with Moyamoya disease. After acute management for the thyroid storm with methimazole, Lugol solution and hydrocortisone, the patient's neurological symptoms completely resolved within 1 month, and free T4 level normalized within 2 months. Thyroid storm may trigger cerebral ischemia in Moyamoya disease and lead to rapid progression of cerebrovascular occlusive disease. As a simultaneous occurrence of DKA, thyroid storm and cerebrovascular accident in Moyamoya disease highly elevates morbidity and mortality, prompt recognition and management are critical to save the patient's life.

  3. Periventricular anastomosis in moyamoya disease: detecting fragile collateral vessels with MR angiography.

    Science.gov (United States)

    Funaki, Takeshi; Takahashi, Jun C; Yoshida, Kazumichi; Takagi, Yasushi; Fushimi, Yasutaka; Kikuchi, Takayuki; Mineharu, Yohei; Okada, Tomohisa; Morimoto, Takaaki; Miyamoto, Susumu

    2016-06-01

    OBJECT The authors' aim in this paper was to determine whether periventricular anastomosis, a novel term for the abnormal collateral vessels typical of moyamoya disease, is reliably measured with MR angiography and is associated with intracranial hemorrhage. METHODS This cross-sectional study sampled consecutive patients with moyamoya disease or moyamoya syndrome at a single institution. Periventricular anastomoses were detected using MR angiography images reformatted as sliding-thin-slab maximum-intensity-projection coronal images and were scored according to 3 subtypes: lenticulostriate, thalamic, and choroidal types. The association between periventricular anastomosis and hemorrhagic presentation at onset was evaluated using multivariate analyses. RESULTS Of 136 eligible patients, 122 were analyzed. Eighteen (14.8%) patients presented with intracranial hemorrhage with neurological symptoms at onset. Intra- and interrater agreement for rating of the periventricular anastomosis score was good (κw = 0.65 and 0.70, respectively). The prevalence of hemorrhagic presentation increased with the periventricular anastomosis score: 2.8% for Score 0, 8.8% for Score 1, 18.9% for Score 2, and 46.7% for Score 3 (p anastomosis score (p anastomosis detected with MR angiography can be scored with good intra- and interrater reliability and is associated with hemorrhagic presentation at onset in moyamoya disease. The clinical utility of periventricular anastomosis as a predictor for hemorrhage should be validated in further prospective studies.

  4. Hemodynamic changes after vascular reconstructive surgery for moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Kuyama, Hideyuki; Niimi, Hitohisa; Kawauchi, Masamitsu; Tanimoto, Takaho; Murota, Takenobu; Kinugasa, Kazushi; Asari, Shoji; Nishimoto, Akira; Nagao, Seigo.

    1988-06-01

    The regional cerebral blood flow (rCBF) was measured in eight juvenile cases with moyamoya disease before and after vascular reconstructive surgery consisting either of encephalo-duro-arterio-synangiosis (EDAS) or of encephalo-duro-arterio-myo-synangiosis (EDAMS). The rCBF was measured quantitatively using the inert-gas washout technique with xenon-133 and single-photon-emission computed tomography. A patient with a completed stroke showed a low value of the mean hemispheric blood flow on the lesion side. There was no significant difference in the mean hemispheric blood flow between the two hemispheres in the patients with a transient ischemic attack. The variation in the rCBF versus the mean hemispheric blood flow was studied before and after the operation. The preoperative rCBF was relatively high in the basal ganglia and occipital regions and low in the frontal region. One month after the operation, the rCBF in each region was not significantly changed in comparison with the preoperative rCBF. Six months after the operation, however, the rCBF was significantly reduced in the basal ganglia and significantly increased in the temporal region. On the other hand, the rCBF was almost identical with the mean hemispheric blood flow in the frontal and occipital regions. These results may be explained by the angiographic findings, which showed a diminution in the moyamoya vessels and a good filling of the middle cerebral artery area via the external carotid artery on the postoperative angiogram. Vascular reconstructive surgery for moyamoya disease in children may, therefore, be thought to be an effective procedure for smoothing the cerebral-blood-flow distribution.

  5. Brain perfusion SPECT for therapeutic evaluation of patients with moyamoya disease after surgery%SPECT脑血流灌注显像对烟雾病患者手术疗效的评价意义

    Institute of Scientific and Technical Information of China (English)

    王璐; 陈雪红; 冯建中

    2015-01-01

    目的 探讨SPECT脑血流灌注显像评估烟雾病(MMD)搭桥手术后rCBF改善情况的价值.方法 回顾性分析13例MMD患者[儿童5例,年龄3~8岁;成人8例,年龄45 ~ 55岁]治疗前后SPECT脑血流灌注显像的影像学资料,以同期因偶发头痛行脑灌注显像的年龄匹配者(儿童、成人各10例)为对照组.以两样本或配对t检验对2组的rCBF及病例组手术前后rCBF进行比较分析.手术后的血流恢复以DSA、MRI随访情况作参照.结果 病例组中SPECT显像目测分析示11/13的患者术后血流灌注减低区较术前有明显改善;与DSA符合率为8/11,与MRI符合率为9/11.对照组和病例组术前rCBF分别为(51.19±1.63)和(38.83±2.22) ml·100 g-1·min-1(t=-18.465,P<0.05);病例组术前、术后的rCBF分别为(38.83±2.22)和(45.03±2.63) ml·100 g-1·min-1(t=-11.707,P<0.05);病例组术后与对照组rCBF差异有统计学意义[(51.19±1.63) ml· 100 g-1·min-1;t=-8.313,P<0.05];对照组中儿童和成人的rCBF分别为(51.81±1.82)和(50.55±1.18) ml· 100 g-1·min-1,两者间差异无统计学意义(t=0.081,P>0.05).结论 SPECT脑血流灌注显像能够较好地反映MMD手术前后缺血脑组织的血流量变化,对MMD搭桥手术治疗的疗效评价具有一定价值.%Objective To investigate the value of SPECT imaging in the evaluation of rCBF improvement after bypass surgery in patients with moyamoya disease (MMD).Methods Retrospective analysis was conducted in 13 patients with MMD (5 children aged 3-8 years and 8 adults aged 45-55 years).The SPECT cerebral perfusion imaging data before and after operation were analyzed.Age matched subjects who underwent cerebral perfusion imaging for accidental headache (10 children,10 adults) were taken as control group.Two-sample t test or paired t test was used to compare rCBF of different groups.The brain DSA and MRI results during follow-up were taken as the reference of perfusion improvement.Results Based on visual judgment

  6. Bilateral Moyamoya Disease in a 2-Year-Old Pakistani Male Treated with Bilateral Encephaloduroarteriosynangiosis: A Positive Outcome.

    Science.gov (United States)

    Magsi, Shahvaiz; Khoja, Adeel; Rameez, Mansoor Ali Merchant; Khan, Ariba; Ishaque, Noman

    2016-01-01

    Background. We present a rare case of bilateral moyamoya disease presenting as multiple strokes and neurological deficits, treated with the neurosurgical procedure, encephaloduroarteriosynangiosis (EDAS), in a 2-year-old male Pakistani minor. A positive outcome was achieved and the patient recovered fully. Case Summary. Our patient presented with a history of seizures and multiple episodes of hemiparesis (on and off weakness) at the age of 2 years. He had a delayed speech development and could not speak more than a few words. He had a slight slurring of speech too. He was diagnosed with bilateral moyamoya disease on Computed Tomography Angiography (CTA). Bilateral EDAS was done in the same year, after which his symptoms improved and patient had moderate functional recovery. Conclusion. A rare disease, moyamoya has been left unexplored in Pakistan; physicians and surgeons when dealing with cases in the pediatric population presenting with symptoms of stroke, signs of generalized weakness, and seizures should consider moyamoya disease as a possibility. Furthermore, this case demonstrates the effectiveness of EDAS procedure for the treatment of moyamoya disease.

  7. Bilateral Moyamoya Disease in a 2-Year-Old Pakistani Male Treated with Bilateral Encephaloduroarteriosynangiosis: A Positive Outcome

    Directory of Open Access Journals (Sweden)

    Shahvaiz Magsi

    2016-01-01

    Full Text Available Background. We present a rare case of bilateral moyamoya disease presenting as multiple strokes and neurological deficits, treated with the neurosurgical procedure, encephaloduroarteriosynangiosis (EDAS, in a 2-year-old male Pakistani minor. A positive outcome was achieved and the patient recovered fully. Case Summary. Our patient presented with a history of seizures and multiple episodes of hemiparesis (on and off weakness at the age of 2 years. He had a delayed speech development and could not speak more than a few words. He had a slight slurring of speech too. He was diagnosed with bilateral moyamoya disease on Computed Tomography Angiography (CTA. Bilateral EDAS was done in the same year, after which his symptoms improved and patient had moderate functional recovery. Conclusion. A rare disease, moyamoya has been left unexplored in Pakistan; physicians and surgeons when dealing with cases in the pediatric population presenting with symptoms of stroke, signs of generalized weakness, and seizures should consider moyamoya disease as a possibility. Furthermore, this case demonstrates the effectiveness of EDAS procedure for the treatment of moyamoya disease.

  8. [A case of akin moyamoya disease associated with type-I diabetes mellitus managed by extracranial-intracranial bypass].

    Science.gov (United States)

    Akamatsu, Yosuke; Fujimura, Miki; Sakata, Hiroyuki; Endo, Hidenori; Itabashi, Ryo; Tominaga, Teiji

    2015-03-01

    Moyamoya disease is characterized by idiopathic steno-occlusion at the terminal portion of the internal carotid artery with concomitant abnormal vascular networks that can lead to transient ischemic attacks and hemorrhagic stroke with symptoms of headache, confusion, dizziness, ataxia, seizure, and cognitive and personality changes. Because these symptoms also occur in patients with type 1 diabetes mellitus(T1DM), patients with both diseases might go unnoticed and without the less common diagnosis of akin moyamoya disease, accurate diagnosis and treatment could be delayed. Here, we report the case of a 32-year-old woman with past history of T1DM for 26 years presenting with right amaurosis, which was diagnosed as akin moyamoya disease even though she had suffered right incomplete hemiparesis 2 years ago. She underwent superficial temporal artery-middle cerebral artery anastomosis with pial synangiosis in the left hemisphere without complication. She had no cerebrovascular events postoperatively. Although akin moyamoya disease associated with T1DM is rare in Japan, we recommend that clinicians consider the coexistence of both diseases when evaluating patients with T1DM who have neurologic signs or symptoms and not overlook the possibility of cerebrovascular diseases, such as akin moyamoya disease.

  9. Downregulation of Securin by the variant RNF213 R4810K (rs112735431, G>A) reduces angiogenic activity of induced pluripotent stem cell-derived vascular endothelial cells from moyamoya patients

    Energy Technology Data Exchange (ETDEWEB)

    Hitomi, Toshiaki [Department of Health and Environmental Sciences, Kyoto University, Kyoto (Japan); Habu, Toshiyuki [Radiation Biology Center, Kyoto University, Kyoto (Japan); Kobayashi, Hatasu; Okuda, Hiroko; Harada, Kouji H. [Department of Health and Environmental Sciences, Kyoto University, Kyoto (Japan); Osafune, Kenji [Center for iPS Cell Research and Application (CiRA), Kyoto University, Kyoto (Japan); Taura, Daisuke; Sone, Masakatsu [Department of Medicine and Clinical Science, Kyoto University, Kyoto (Japan); Asaka, Isao; Ameku, Tomonaga; Watanabe, Akira; Kasahara, Tomoko; Sudo, Tomomi; Shiota, Fumihiko [Center for iPS Cell Research and Application (CiRA), Kyoto University, Kyoto (Japan); Hashikata, Hirokuni; Takagi, Yasushi [Department of Neurosurgery, Kyoto University,Kyoto (Japan); Morito, Daisuke [Faculty of Life Sciences, Kyoto Sangyo University, Kyoto (Japan); Miyamoto, Susumu [Department of Neurosurgery, Kyoto University,Kyoto (Japan); Nakao, Kazuwa [Department of Medicine and Clinical Science, Kyoto University, Kyoto (Japan); Koizumi, Akio, E-mail: koizumi.akio.5v@kyoto-u.ac.jp [Department of Health and Environmental Sciences, Kyoto University, Kyoto (Japan)

    2013-08-16

    Highlights: •Angiogenic activities were reduced in iPSECs from MMD patients. •Many mitosis-regulated genes were downregulated in iPSECs from MMD patients. •RNF213 R4810K downregulated Securin and inhibited angiogenic activity. •Securin suppression by siRNA reduced angiogenic activities of iPSECs and HUVECs. -- Abstract: Moyamoya disease (MMD) is a cerebrovascular disease characterized by occlusive lesions in the circle of Willis. The RNF213 R4810K polymorphism increases susceptibility to MMD. Induced pluripotent stem cells (iPSCs) were established from unaffected fibroblast donors with wild-type RNF213 alleles, and from carriers/patients with one or two RNF213 R4810K alleles. Angiogenic activities of iPSC-derived vascular endothelial cells (iPSECs) from patients and carriers were lower (49.0 ± 19.4%) than from wild-type subjects (p < 0.01). Gene expression profiles in iPSECs showed that Securin was down-regulated (p < 0.01) in carriers and patients. Overexpression of RNF213 R4810K downregulated Securin, inhibited angiogenic activity (36.0 ± 16.9%) and proliferation of humanumbilical vein endothelial cells (HUVECs) while overexpression of RNF213 wild type did not. Securin expression was downregulated using RNA interference techniques, which reduced the level of tube formation in iPSECs and HUVECs without inhibition of proliferation. RNF213 R4810K reduced angiogenic activities of iPSECs from patients with MMD, suggesting that it is a promising in vitro model for MMD.

  10. Proposal for a Prospective Registry for Moyamoya Disease in Japan

    Science.gov (United States)

    KAZUMATA, Ken; ITO, Masaki; UCHINO, Haruto; NISHIHARA, Hiroshi; HOUKIN, Kiyohiro

    2017-01-01

    The number of clinical research papers published worldwide on moyamoya disease (MMD) has increased recently. However, the majority of the literature comprises retrospective single-center studies collecting data on small numbers of patients. Several multi-center studies are ongoing in Japan; however, the current data are insufficient for comprehensively outlining the various characteristics of MMD. To enhance our knowledge on epidemiologic, vascular, and genetic aspects of MMD, a prospective multicenter registry will be established in Japan that will help to streamline clinical research as well as improve clinical treatments and long-term outcomes. Patients with MMD or secondary moyamoya syndrome referred to the participating centers will be invited to the registry. Demographic and physiological parameters, along with neuroimaging data will be collected chronologically. Clinical events, including neurological, medical, and surgical interventions will be recorded. Whole blood samples will be collected. Extra- and intracranial vascular tissue, and/or cerebrospinal fluid will also be collected from patients who undergo surgical revascularization. These biospecimens will be stored at the repositories and utilized for genome-wide association studies for identifying genetic variants, as well as tissue-specific proteomic, and/or molecular analyses. Ethics approval will be obtained at all facilities collecting biospecimens. The registry will provide descriptive statistics on functional outcomes, surgical techniques used, medications, and neurological events stratified according to patients’ clinical characteristics. We expect this study to provide novel insights in the management of MMD patients and design better therapies. PMID:28070115

  11. Computed tomographic findings of moyamoya disease

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    Yang, DaL Mo; Choi, Woo Suk; Ryu, Kyung Nam; Lee, Sun Wha; Yoon, Yup [Kyung Hee University Hospital, Seoul (Korea, Republic of)

    1991-01-15

    We retrospectively reviewed the CT scans and angiograms of 17 cases with angiographically-proved moyamoya disease from April 1987 to October 1989. The results were as follows. The age distribution ranged from 9 to 48. Eight patients were less than 20 years old. Nine cases were male and 8 cases were female. On postcontrast CT scan, curvilinear, tortuous vessels were well dilineated at the circle of willis and basal ganglia in 4 cases, and contrast enhancement in 7 cases. These findings are most likely to represent parenchymal and leptomeningeal collateral vessels in angiography. On angiography, bilateral supraclinoid internal carotid artery (ICA), proximal middle cerebral artery (MI), and proximal anterior cerebral artery (AI) were involved in 13 cases. The prognosis were poor in one case with bilateral ICA, MI, AI and posterior cerebral artery (PCA) involvement. One case had a pseudoaneurysm which was seen at the left anterior choroidal artery branch, and measured about 3 x 2 mm in size. One case was accompanied by a dural arteriovenous malformation (AVM)

  12. Intracranial Aneurysms in Sickle-Cell Disease Are Associated With the Hemoglobin SS Genotype But Not With Moyamoya Syndrome

    DEFF Research Database (Denmark)

    Birkeland, Peter; Gardner, Kate; Kesse-Adu, Rachel

    2016-01-01

    BACKGROUND AND PURPOSE: Intracranial aneurysms and aneurysmal subarachnoid hemorrhage may occur more frequently in sickle-cell disease (SCD), and this could be related to the sickle genotype and moyamoya syndrome seen in SCD. METHODS: Records from a total of 1002 patients with SCD attending 2...... had imaging data; the prevalence was significantly higher in patients with HbSS genotype compared with other sickle genotypes with the highest prevalence (15%) observed in women in the age group 30 to 39 years. Fifty-one HbSS patients had a moyamoya vasculopathy, but only 3 of these had concomitant...

  13. Headache and focal neurologic signs following exposure to spicy aroma as an initial presentation of moyamoya disease

    Directory of Open Access Journals (Sweden)

    Bilal A. Siddiqui, A.B.

    2014-12-01

    Full Text Available Moyamoya disease is a condition of the cerebrovascular system that involves stenosis of the intracranial internal carotid arteries as well as their proximal branches, often leading to stroke in affected patients. Here we describe the case of a patient with headache and focal neurologic signs following exposure to a spicy aroma, who initially had a negative vascular work-up and a preliminary diagnosis of a complex migraine syndrome. She subsequently developed infarction of the left frontal lobe, and imaging studies revealed the diagnosis of moyamoya disease. She was treated with an encephalodurosynangiosis procedure, with notable improvement. This case highlights the importance of considering moyamoya disease in the differential diagnosis of patients presenting with headaches with aura.

  14. Progress on Complications of Direct Bypass for Moyamoya Disease

    Science.gov (United States)

    Yu, Jinlu; Shi, Lei; Guo, Yunbao; Xu, Baofeng; Xu, Kan

    2016-01-01

    Moyamoya disease (MMD) involves progressive occlusion of the intracranial internal carotid artery resulting in formation of moyamoya-like vessels at the base of the brain. It can be characterized by hemorrhage or ischemia. Direct vascular bypass is the main and most effective treatment of MMD. However, patients with MMD differ from those with normal cerebral vessels. MMD patients have unstable intracranial artery hemodynamics and a poor blood flow reserve; therefore, during the direct bypass of superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis, perioperative risk factors and anesthesia can affect the hemodynamics of these patients. When brain tissue cannot tolerate a high blood flow rate, it becomes prone to hyperperfusion syndrome, which leads to neurological function defects and can even cause intracranial hemorrhage in severe cases. The brain tissue is prone to infarction when hemodynamic equilibrium is affected. In addition, bypass vessels become susceptible to occlusion or atrophy when blood resistance increases. Even compression of the temporalis affects bypass vessels. Because the STA is used in MMD surgery, the scalp becomes ischemic and is likely to develop necrosis and infection. These complications of MMD surgery are difficult to manage and are not well understood. To date, no systematic studies of the complications that occur after direct bypass in MMD have been performed, and reported complications are hidden among various case studies; therefore, this paper presents a review and summary of the literature in PubMed on the complications of direct bypass in MMD. PMID:27499690

  15. Progress on Complications of Direct Bypass for Moyamoya Disease.

    Science.gov (United States)

    Yu, Jinlu; Shi, Lei; Guo, Yunbao; Xu, Baofeng; Xu, Kan

    2016-01-01

    Moyamoya disease (MMD) involves progressive occlusion of the intracranial internal carotid artery resulting in formation of moyamoya-like vessels at the base of the brain. It can be characterized by hemorrhage or ischemia. Direct vascular bypass is the main and most effective treatment of MMD. However, patients with MMD differ from those with normal cerebral vessels. MMD patients have unstable intracranial artery hemodynamics and a poor blood flow reserve; therefore, during the direct bypass of superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis, perioperative risk factors and anesthesia can affect the hemodynamics of these patients. When brain tissue cannot tolerate a high blood flow rate, it becomes prone to hyperperfusion syndrome, which leads to neurological function defects and can even cause intracranial hemorrhage in severe cases. The brain tissue is prone to infarction when hemodynamic equilibrium is affected. In addition, bypass vessels become susceptible to occlusion or atrophy when blood resistance increases. Even compression of the temporalis affects bypass vessels. Because the STA is used in MMD surgery, the scalp becomes ischemic and is likely to develop necrosis and infection. These complications of MMD surgery are difficult to manage and are not well understood. To date, no systematic studies of the complications that occur after direct bypass in MMD have been performed, and reported complications are hidden among various case studies; therefore, this paper presents a review and summary of the literature in PubMed on the complications of direct bypass in MMD.

  16. Studies of Japanese cases with cerebral basal rete mirabile (Moyamoya disease). Investigation of cerebrovascular hemodynamics

    Energy Technology Data Exchange (ETDEWEB)

    Ueta, Kiyotaka

    1988-04-01

    Eight children under 15 years of age and 8 adults with basal cerebral rate (so called Moyamoya disease) were examined. Regional cerebral blood flow (rCBF) was measured in 12 of these patients under normocapneic, hypercapneic, hypocapneic and hypotensive states by a /sup 133/Xe-intracarotid injection method using a gamma camera. Patients with neurological deficits due to completed stroke (CS) had angiographically poor normograde vascularization of cortical branches of the middle cerebral artery (MCA) and poorly developed collateral circulation. Hemispheric cerebral blood flow (HCBF) in the resting state tended to decrease according to the severity of neurological deficits, but was within the normal range in patients with no neurological deficits. However, focal reduction of rCBF was seen in half of the patients with transient ischemic attacks. Development of leptomeningeal anastomosis of MCA territories from the posterior cerebral artery seemed to have a more important role than basal rete in maintaining cerebral blood flow. There was a significant reduction of the HCBF under the hypocaneic state, but no significant increase under the hypercapneic state. The lack of a CBF response to increased arterial CO/sub 2/ tension seemed to be due to maximum dilatation of cerebral arterioles. (author) 61 refs.

  17. Anlysis on Clinical Manifestation Characteristics of 43 Moyamoya Disease Patients%43例烟雾病的临床分析

    Institute of Scientific and Technical Information of China (English)

    雒东江; 艾林; 张明宇

    2012-01-01

    目的 通过全脑血管造影(DSA)诊断及分析43例烟雾病(Moyamoya)病的临床表现和特征.方法 分析北京天坛医院神经放射中心2009年2-9月经DSA确诊的43例Moyamoya病患者的临床资料及影像学资料.结果 43例患者年龄5~45岁,其中15岁以下11例,平均年龄27.5岁;脑卒中是本组Moyamoya病患者最常见的临床表现,发生率为76.7%(33例);儿童及青少年患者以缺血性脑卒中发病为主,而成年患者以出血性脑卒中发病为主;患者均有颈内动脉或其分支狭窄、闭塞和Moyamoya血管形成.结论 对于无脑血管病危险因素的短暂性脑缺血发作(TIA)或脑卒中患者,尤其有卒中史或反复发作者则要考虑Moyamoya病的可能;DSA是诊断Moyamoya病和对其搭桥手术后进行疗效观察的最直接检查手段.

  18. 烟雾病与免疫炎症%Moyamoya disease and immune inflammation

    Institute of Scientific and Technical Information of China (English)

    陈祥; 徐运

    2016-01-01

    烟雾病是一种慢性进展性脑血管病,主要表现为双侧颈内动脉进行性闭塞并伴有脑底代偿增生的异常血管网形成。烟雾病的病理学机制尚不清楚。越来越多的证据表明免疫炎症可能在其发生和发展过程中发挥着重要作用。烟雾病患者可见多种炎性因子和免疫蛋白表达异常。文章就近年来有关免疫炎症在烟雾病中的可能作用机制进行了综述。%Moyamoya disease is a chronic progressive cerebrovascular disease. Its main manifestation is bilateral internal carotid artery progressive stenosis w ith abnormal vascular netw ork formation of the compensatory hyperplasia in brain base. The pathological mechanism of moyamoya disease is not clear. Grow ing evidence has suggested that immune inflammation may play an important role in its occurrence and development process. Abnormal expression of various inflammatory cytokines and immune proteins can be observed in patients w ith moyamoya disease. This article review s the possible mechanism of immune inflammation in moyamoya disease in recent years.

  19. T2 shortening in childhood moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Takanashi, J. [Department of Pediatrics, Faculty of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba-shi 260 (Japan); Sugita, K. [Department of Pediatrics, Faculty of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba-shi 260 (Japan); Tanabe, Y. [Division of Neurology, Chiba Children`s Hospital, 579-1 Heta, Midori-ku, Chiba-shi 266 (Japan); Ito, C. [Division of Neurosurgery, Chiba Children`s Hospital, 579-1 Heta, Midori-ku, Chiba-shi 266 (Japan); Date, H. [Division of Neurosurgery, Chiba Children`s Hospital, 579-1 Heta, Midori-ku, Chiba-shi 266 (Japan); Niimi, H. [Department of Pediatrics, Faculty of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba-shi 260 (Japan)

    1996-05-01

    We examined T2 shortening in six children with infarcts due to moyamoya disease to clarify whether there are characteristic patterns of T2 shortening in the deep grey and white matter. Profound T2 shortening in the deep grey and white matter was observed in the acute stage of infarct in two cases, which changed to high intensity in the chronic stage; in this stage no T2 shortening was demonstrated in any case. Neither haemorrhagic infarction nor calcification was seen on CT or MRI. There could be longitudinally different T2 shortening patterns between infarcts due to moyamoya disease and other disorders. (orig.). With 2 figs., 1 tab.

  20. Charactics of cerebrovescular lesion,collateral circulation in Moyamoya syndrome secondary to intracranial atherosclerotic disease%动脉粥样硬化性大脑中动脉狭窄继发Moyamoya 综合征的脑血管病变及侧支循环分布模式

    Institute of Scientific and Technical Information of China (English)

    徐宁; 张千; 李先锋

    2014-01-01

    Objective To investigate the charactics of cerebrovescular lesion,collateral circulation in Moyamoya syndrome secondary to intracranial atherosclerotic disease.Methods 26 subjects were identified by digital subtraction angiography between August 2006 and December 2013.Evaluating the correlation between moyamoya vessels ,intracranial arterial lesions and the degree of leptomeningeal anastomosis.Results 1 7 pa-tients had moyamoya vessels.The presence of moyamoya vessels was associated with ipsilateral or contralateral anterior cerebral artery stenosis to the occluded MCA (P =0.004)and poor development of anterior leptomen-ingeal anastomosis (P =0.012).It was also associated with vascular lesions involving more than one intracrani-al branch vessel in patients with moyamoya syndrome secondary to atherosclerosis.Conclusions Moyamoya vessels might be associated with poorly developed leptomeningeal collateral vessels.%目的:探讨动脉粥样硬化性大脑中动脉狭窄继发 moyamoya 综合征的脑血管病变及侧支循环分布模式。方法收集2010年8月~2013年12月经 DSA 证实动脉粥样硬化性大脑中动脉狭窄患者26例。评估 moyamoya 血管网建立与颅内血管病变及软脑膜侧支循环开放度之间的关系。结果26例患者中 moy-amoya 综合征组17例,非 moyamoya 综合征组9例。moyamoya 综合征组4例患者在 MCA 闭塞侧 A1段表现为细小或缺如,而非 moyamoya 综合征组9例 A1段表现正常。moyamoya 综合征组11例在 MCA 一侧或对侧的 ACA 上发现动脉粥样硬化性狭窄或闭塞病变,而非 moyamoya 综合征组未发现上述病变(P =0.004)。非 moyamoya 综合征组所有患者均显示自 ACA 到达 M1或 M2节段足够的软脑膜侧支循环,而 moy-amoya 综合征组仅有8例(P =0.012)。结论动脉粥样硬化所致的 moyamoya 血管网重建可能与软脑膜侧支循环建立不佳有关。

  1. Síndrome de moya-moya y falciformia. Reporte de un caso

    Directory of Open Access Journals (Sweden)

    Marlon Martínez Barros

    2013-10-01

    Full Text Available Title: Moya moya syndrome and falciformia. Report of a case.ResumenLa Enfermedad de Moya-Moya es una vasculopatía oclusiva progresiva, no inflamatoria, no ateroesclerótica, que afecta los grandes vasos del polígono de Willis. En el presente artículo se reporta un caso de un síndrome de Moya- Moya asociado a Anemia de Células Falciformes, quien presentaba ataques isquémicos transitorios con frecuencia, el diagnostico se basa en la clínica del paciente y los hallazgos angiográficos, fue revascularizado mediante encefalomiosinangiosis, procedimiento con el cual mejora notablemente su calidad de vida y cesa la aparición de ataques isquémicos transitorios. Se concluye que se trata de un caso de síndrome de Moya-Moya dado que concomitantemente este paciente presentó Enfermedad de Células Falciformes. (DUAZARY 2012 No. 2, 176 - 180AbstractMoya-Moya Disease is a progressive occlusive vascular, non-inflammatory, non-atherosclerotic, which affects the large vessels of the circle of Willis. In this article we report a case of Moya-Moya syndrome associated with Sickle Cell Anemia, who had transient ischemic attacks often the diagnosis is based on the patient’s clinical and angiographic findings was revascularized by encefalomiosinangiosis, process by which greatly improves their quality of life and cease the occurrence of transient ischemic attacks. We conclude that it is a case of Moya-Moya Syndrome since the patient had concomitant Sickle Cell Disease.Keywords: Moya-Moya Syndrome; transient ischemic attacks; Sickle Cell Anemia. (MeSH. 

  2. The scintigraphic appearance of childhood moyamoya disease on cerebral perfusion imaging

    Energy Technology Data Exchange (ETDEWEB)

    Miller, J.H. [Department of Radiology, Childrens Hospital Los Angeles, Los Angeles, CA (United States)]|[University of Southern California School of Medicine, Los Angeles (United States); Khonsary, A. [Department of Radiology, Childrens Hospital Los Angeles, Los Angeles, CA (United States)]|[University of Southern California School of Medicine, Los Angeles (United States); Raffel, C. [Department of Surgery, Childrens Hospital Los Angeles (United States)]|[University of Southern California School of Medicine, Los Angeles (United States)

    1996-12-01

    Objective. This study was performed to evaluate the role of single photon emission computed tomography (SPECT) perfusion imaging in the evaluation of patients with moyamoya disease. Materials and methods. Five patients (four female, one male) were studied utilizing iodine-123 iodoamphetamine or technetium-99m hexamethylpropyleneamine oxime SPECT. The data were reconstructed into axial, coronal and sagittal sections for review, and compared with CT, MR and/or angiographic images. Results. All five patients had significant perfusion defects. These areas of vascular compromise were seen to cross normal vascular territories, and were greater in number and extent than seen on anatomic sectional imaging. Conclusion. Patients with moyamoya disease have a recognizable pattern of scintigraphic perfusion deficits which should be identified by pediatric imaging physicians. SPECT perfusion studies should be performed in conjunction with other imaging modalities (CT, MR or angiography). (orig.). With 3 figs.

  3. Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines. A review.

    Science.gov (United States)

    Smith, Edward R; Scott, R Michael

    2012-04-01

    Moyamoya is a progressive arteriopathy of unknown origin affecting the branches of the internal carotid artery (ICA). The arteriopathy can present as an isolated medical condition, affecting both sides of the brain ("moyamoya disease") or can be unilateral or found in association with systemic disorders ("moyamoya syndrome"). The ischemia resulting from luminal narrowing predisposes children to transient ischemic attacks and stroke-the primary presentations of affected patients. Although it is rare-affecting 1 in 1 million children in the US-moyamoya is implicated in 6% of all childhood strokes. Diagnosis is defined by characteristic findings on arteriograms, including stenosis of the branches of the ICA and a pathognomonic spray of small collateral vessels in this region, descriptively likened to a "puff of smoke" ("moyamoya" in Japanese). Treatment is predicated on restoration of cerebral blood flow by surgical revascularization. The rarity of this disorder has limited research and the development of evidence-based clinical management. While acknowledging these limitations, in this article the authors aim to summarize current studies of pediatric moyamoya, with the objective of providing a framework for construction of evidence-based guidelines for treatment. The compilation of current data in these guidelines should serve as a resource to aid pediatric neurosurgeons in their role as advocates for providing appropriate care to affected children.

  4. Ocular Features and Visual Outcome in Children with Moyamoya Disease and Moyamoya Syndrome: A Case Series.

    Science.gov (United States)

    John, Deepa; Muthusamy, Karthik; Bandla, Bhavagna; Sudhakar, Sniya Valsa; Thomas, Maya

    2016-05-01

    Moya Moya Disease (MMD) is characterised by idiopathic vasculopathy affecting the terminal internal carotid arteries resulting in the formation of extensive collaterals at the base of the brain, leptomeninges and parenchymal regions with resultant infarcts and bleeds. Four children presented with clinico-radiological features suggestive of Moyamoya disease/syndrome. This includes global developmental delay, recurrent seizures, transient ischaemic attacks and impaired vision. The first patient had vision of 6/15 in both eyes with bilateral optic disc pallor. Second case also had bilateral optic disc pallor with arteriolar attenuation, but had vision of perception of light only in both eyes. The third child had vision of 6/60 with alternate divergent squint and clinical features suggestive of Neurofibromatosis 1 (NF 1). Fourth patient presented with poor fixation in both eyes with bilateral total cataract. He underwent bilateral cataract surgery with intraocular lens implantation and vision improved to 2/60 with good fixation. We also describe their medical and neurosurgical interventions in this report.

  5. Moyamoya syndrome associated with γ knife surgery for cerebral arteriovenous malformation: case report.

    Science.gov (United States)

    Uozumi, Yoichi; Sumitomo, Masaki; Maruwaka, Mikio; Araki, Yoshio; Izumi, Takashi; Miyachi, Shigeru; Kato, Takenori; Hasegawa, Toshinori; Kida, Yoshihisa; Okamoto, Sho; Wakabayashi, Toshihiko

    2012-01-01

    A 30-year-old female developed moyamoya syndrome after gamma knife surgery (GKS) for cerebral arteriovenous malformation (AVM), and was treated with bypass surgery. She suffered from flittering scotoma, right transient hemianopsia, and headache for 1 year. Cerebral angiography revealed a Spetzler-Martin grade III AVM located in the left occipital lobe. After staged embolization, GKS was performed with a minimum dose of 20 Gy to the periphery of the nidus at the 50% isodose level of the maximum target dose. Gradual nidus regression was achieved, and the clinical symptoms disappeared completely. However, at 30 months after GKS, the patient suffered transient ischemic attack. Cerebral angiography showed left middle cerebral artery occlusion with moyamoya vessels. The patient underwent direct and indirect bypass surgery. After surgery, the patient was free from ischemic symptoms. Chronic inflammation and long-term changes in expression of cytokines and growth factors after GKS may have triggered this case.

  6. [Moyamoya disease in a girl with Down syndrome. Report of one case].

    Science.gov (United States)

    Hernández Ch, Marta; Huete L, Isidro; Concha G, María-José; Méndez C, José I; Sánchez D, Nicolás; Cuéllar G, María-Carolina; Retamal R, Eva; Mesa L, Tomás

    2009-08-01

    Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. It can be primary or secondary to genetic syndromes such as Down syndrome. We report a seven year-old girl with a Down syndrome that presented with a disturbance of consciousness, seizures and a right hemiparesia at the age of five. Magnetic resonance imaging showed old cortical ischemic lesions in both cerebral hemispheres and a recent infarction in the territory of the left middle cerebral artery. Brain angiography showed a proximal stenosis of both medial cerebral arteries and a net of collateral vessels, consistent with the diagnosis of moyamoya syndrome. The patient had also an antithrombin III deficiency. Aspirin was indicated and a surgical correction was recommended. However, prior to the procedure, the patient had a new infarction in the territory of the right middle cerebral artery, which caused a severe disability.

  7. Localized 1H-MR spectroscopy in moyamoya disease before and after revascularization surgery

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Soo Mee; Choi, Hye Young; Suh, Jung Soo [Ewha Womans University Hospital, Seoul (Korea, Republic of); Lee, Jung Hee; Lim, Keun Ho; Suh, Dae Chul; Lee, Ho Kyu; Lim, Tae Hwan; Ra, Young Shin [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    2003-06-01

    To evaluate, using localized proton magnetic resonance spectroscopy (1H-MRS), the cerebral metabolic change apparent after revascularization surgery in patients with moyamoya disease. Sixteen children with moyamoya disease and eight age-matched normal controls underwent MR imaging, MR angiography, conventional angiography, and {sup 99m}Tc- ECD SPECT. Frontal white matter and the basal ganglia of both hemispheres were subjected to localized {sup 1}H-MRS, and after revascularization surgery, four patients underwent follow-up {sup 1}H-MRS. Decreased NAA/Cr ratios (1.35{+-}0.14 in patients vs. 1.55{+-}0.24 in controls) and Cho/Cr ratios (0.96{+-}0.13 in patients vs. 1.10{+-}0.11 in controls) were observed in frontal white matter. After revascularization surgery, NAA/Cr and Cho/Cr ratios in this region increased. In the basal ganglia, there is no abnormal metabolic ratios. Localized 1H-MRS revealed abnormal metabolic change in both hemispheres of children with moyamoya disease. Because of its non-invasive nature, {sup 1}H-MRS is potentially useful for the preoperative evaluation of metabolic abnormalities and their postoperative monitoring.

  8. Moyamoya Syndrome Associated With Hereditary Spherocytosis: An Emerging Clinical Entity.

    Science.gov (United States)

    Gait-Carr, Eleanor; Connolly, Daniel J A; King, David

    2017-04-01

    Moyamoya syndrome is an unusual cerebrovascular disorder, which has rarely been reported in association with hereditary spherocytosis. We present the case of a 6-year-old boy with hereditary spherocytosis who was diagnosed with Moyamoya syndrome following a stroke. We discuss why these conditions may coexist and briefly outline the management of such children.

  9. Diagnosis of moyamoya disease: international standard and regional differences.

    Science.gov (United States)

    Fujimura, Miki; Tominaga, Teiji

    2015-01-01

    Moyamoya disease is a chronic, occlusive cerebrovascular disease with unknown etiology characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. These diagnostic criteria of the moyamoya disease, stated by the Research Committee on Spontaneous Occlusion of the Circle of Willis (moyamoya disease) in Japan, are well established and generally accepted as the definition of this rare entity. On the contrary to the diagnosis of definitive moyamoya disease, there is some confusion in the terminology and understanding of quasi-moyamoya disease; moyamoya disease in association with various disease entities, such as atherosclerosis, autoimmune diseases, Down syndrome, etc. Although the clinical management is not affected by these semantic distinctions, terminological confusion may interfere with the international collaboration of the clinical investigation of these rare conditions. In this article, we sought to review the international standard and regional differences in the diagnosis of moyamoya disease and quasi-moyamoya disease.

  10. Intellectual Ability and Executive Function in Pediatric Moyamoya Vasculopathy

    Science.gov (United States)

    Williams, Tricia S.; Westmacott, Robyn; Dlamini, Nomazulu; Granite, Leeor; Dirks, Peter; Askalan, Rand; MacGregor, Daune; Moharir, Mahendranath; Deveber, Gabrielle

    2012-01-01

    Aim: Moyamoya vasculopathy is characterized by progressive stenosis of the major arteries of the Circle of Willis, resulting in compromised cerebral blood flow and increased risk of stroke. The objectives of the current study were to examine intellectual and executive functioning of children with moyamoya and to evaluate the impact of moyamoya…

  11. Development of a de novo arteriovenous malformation after bilateral revascularization surgery in a child with moyamoya disease.

    Science.gov (United States)

    Fujimura, Miki; Kimura, Naoto; Ezura, Masayuki; Niizuma, Kuniyasu; Uenohara, Hiroshi; Tominaga, Teiji

    2014-06-01

    The development of a de novo arteriovenous malformation (AVM) in patients with moyamoya disease is extremely rare. A 14-year-old girl developed an AVM in the right occipital lobe during the 4-year postoperative period following successful bilateral revascularization surgeries. She suffered a transient ischemic attack with hemodynamic compromise of the bilateral hemispheres at the age of 10 years. Results of an initial examination by 1.5-T MRI and MR angiography satisfied the diagnostic criteria of moyamoya disease but failed to detect any vascular malformation. Bilateral direct and indirect revascularization surgeries in the anterior circulation relieved her symptoms, and she underwent MRI and MR angiography follow-up every year after surgery. Serial T2-weighted MRI revealed the gradual appearance of flow voids in the right occipital lobe during the follow-up period. Magnetic resonance angiography ultimately indicated the development of an AVM 4 years after these surgeries when catheter angiography confirmed the diagnosis of an AVM in the right occipital lobe. The AVM remained asymptomatic, and the patient remained free of cerebrovascular events during the time she was observed by the authors. Acquired AVM in moyamoya disease is extremely rare, with only 3 pediatric cases including the present case being reported in the literature. The development of a de novo AVM in a postoperative patient with moyamoya disease appears to be unique, and this case may provide insight into the dynamic pathology of AVMs.

  12. Multi-delay arterial spin labeling perfusion MRI in moyamoya disease-comparison with CT perfusion imaging

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Rui [Chinese Academy of Sciences, State Key Laboratory of Brain and Cognitive Science, Beijing MRI Center for Brain Research, Institute of Biophysics, Beijing (China); University of Chinese Academy of Sciences, Graduate School, Beijing (China); UCLA-Beijing Joint Center for Advanced Brain Imaging, Beijing (China); UCLA-Beijing Joint Center for Advanced Brain Imaging, Los Angeles, CA (United States); Yu, Songlin [Capital Medical University, Department of Neurosurgery, Beijing Tiantan Hospital, Beijing (China); University of California Los Angeles, Department of Neurology, Los Angeles (United States); Alger, Jeffry R.; Wang, Danny J.J. [University of California Los Angeles, Department of Neurology, Los Angeles (United States); UCLA-Beijing Joint Center for Advanced Brain Imaging, Beijing (China); UCLA-Beijing Joint Center for Advanced Brain Imaging, Los Angeles, CA (United States); Zuo, Zhentao; Wang, Bo [Chinese Academy of Sciences, State Key Laboratory of Brain and Cognitive Science, Beijing MRI Center for Brain Research, Institute of Biophysics, Beijing (China); Chen, Juan [Beijing Hospital, Department of Radiology, Beijing (China); Wang, Rong; Zhao, Jizong [Capital Medical University, Department of Neurosurgery, Beijing Tiantan Hospital, Beijing (China); An, Jing [Siemens Shenzhen Magnetic Resonance Ltd, Shenzhen (China); Xue, Rong [Chinese Academy of Sciences, State Key Laboratory of Brain and Cognitive Science, Beijing MRI Center for Brain Research, Institute of Biophysics, Beijing (China); UCLA-Beijing Joint Center for Advanced Brain Imaging, Beijing (China); UCLA-Beijing Joint Center for Advanced Brain Imaging, Los Angeles, CA (United States)

    2014-05-15

    To present a multi-delay pseudo-continuous ASL (pCASL) protocol that offers simultaneous measurements of cerebral blood flow (CBF) and arterial transit time (ATT), and to study correlations between multi-delay pCASL and CT perfusion in moyamoya disease. A 4 post-labeling delay (PLD) pCASL protocol was applied on 17 patients with moyamoya disease who also underwent CT perfusion imaging. ATT was estimated using the multi-delay protocol and included in the calculation of CBF. ASL and CT perfusion images were rated for lesion severity/conspicuity. Pearson correlation coefficients were calculated across voxels between the two modalities in grey and white matter of each subject respectively and between normalized mean values of ASL and CT perfusion measures in major vascular territories. Significant associations between ASL and CT perfusion were detected using subjective ratings, voxel-wise analysis in grey and white matter and region of interest (ROI)-based analysis of normalized mean perfusion. The correlation between ASL CBF and CT perfusion was improved using the multi-delay pCASL protocol compared to CBF acquired at a single PLD of 2 s (P < 0.05). There is a correlation between perfusion data from ASL and CT perfusion imaging in patients with moyamoya disease. Multi-delay ASL can improve CBF quantification, which could be a prognostic imaging biomarker in patients with moyamoya disease. (orig.)

  13. Moyamoya disease associated with antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Mahmut Abuhandan

    2011-12-01

    Full Text Available Moyamoya (MMD is a disease that often involves the vascular structures of anterior cerebral circulation, particularly the proximal segments of anterior and middle cerebral arteries. The etiology of the disease is unknown. MMD often presents with cerebral ischemia and rarely with cerebral hemorrhage. The pathology is termed Moyamoya syndrome (MMS when the pathological cerebral angiography findings are accompanied by meningitis, neurofibromatosis, neoplasm, Down syndrome or polycystic kidney disease. Autoimmune diseases including Graves’ disease, Behcet’s disease and antiphospholipid syndrome might also lead to the development of MMS. In this manuscript, we presented an interesting case of MMD associated with antiphospholipid syndrome, which is quite a rare cause of acute cerebral infarction in childhood

  14. Resolution of bilateral moyamoya associated collateral vessel aneurysms: Rationale for endovascular versus surgical intervention

    Directory of Open Access Journals (Sweden)

    Sepideh Amin-Hanjani

    2014-01-01

    Full Text Available Background: Management of aneurysms associated with deep collateral vessels in moyamoya disease is challenging both from an endovascular and a surgical standpoint. Difficulties with access or localization, and compromise of the collateral circulation with subsequent ischemia are the primary concerns, making direct obliteration potentially unfeasible or risky. Alternatively, superficial temporal artery-middle cerebral artery bypass is another potential strategy for resolution of these aneurysms. Case Description: Presented are the findings and management for a patient with moyamoya disease and bilateral deep collateral vessel aneurysms, successfully treated with endovascular obliteration following a right-sided hemorrhage and subsequently with bypass for an unruptured but growing contralateral aneurysm. Conclusions: A rationale and approach to management is outlined, as derived from review of the current literature and the illustrative case with bilateral collateral vessel aneurysms.

  15. Cystic fibrosis lung disease in adult patients.

    Science.gov (United States)

    Vender, Robert L

    2008-04-01

    As the longevity of all patients with cystic fibrosis (CF) continues to increase (median 2005 survival=36.8 years), more adult patients will be receiving their medical care from nonpediatric adult-care providers. Cystic fibrosis remains a fatal disease, with more than 80% of patients dying after the age of 18 years, and most deaths resulting from pulmonary disease. The changing epidemiology requires adult-care providers to become knowledgeable and competent in the clinical management of adults with CF. Physicians must understand the influence of specific genotype on phenotypic disease presentation and severity, the pathogenic factors determining lung disease onset and progression, the impact of comorbid disease factors such as CF-related diabetes and malnutrition upon lung disease severity, and the currently approved or standard accepted therapies used for chronic management of CF lung disease. This knowledge is critical to help alleviate morbidity and improve mortality for the rapidly expanding population of adults with CF.

  16. Collateral vessels in moyamoya disease : comparison of MR and MRA with conventional angiography

    Energy Technology Data Exchange (ETDEWEB)

    Shim, Joo Eun; Yoon, Dae Young; Yi, Jeong Geun; Kim, Ho Chul; Choi, Chul Sun; Bae, Sang Hoon [Hallym University College of Medicine, Choonchun (Korea, Republic of)

    1998-01-01

    To determine the value of magnetic resonance imaging (MR) and magnetic resonance angiography (MRA) in assessing collateral vessels of moyamoya disease. Twenty-four patients with moyamoya disease who underwent MR, 3D TOF MRA, and conventional angiography participated in this study. Two radiologists working independently and with no knowledge of the angiographic findings, interpreted the MR and MRA images. To determine the presence of parenchymal and leptomeningeal collaterals (48 hemispheres) and transdural collaterals (38 hemispheres in 19 patients were depicted by angiography of the external carotid), the findings were compared with those of angiography. Parenchymal, leptomeningeal, and transdural collaterals were depicted by conventional angiography in 34 (71%), 32 (67%), and 11 (29%) hemispheres respectively. The sensitivity and specificity of MR/MRA for collateral vessels were 79.1/88.1 % for parenchymal collaterals, 72.1/88.1 % for leptomeningeal collaterals, and 0.1/18.1 % for transdural collaterals, respectively. Respective sensitivity and specificity of MR/MRA were 88.94/94.1% for leptomeningeal collaterals, and 18.93/55.1 % for transdural collaterals, when the prominent posterior cerebral and external carotid artery were regarded as secondary signs of leptomeningeal and transdural collateral vessels. In moyamoya disease, MR and MRA are useful imaging modalities for the assessment of collateral vessels. The prominent posterior cerebral artery and external carotid artery can be useful secondary signs of leptomeningeal and transdural collateral vessels. (author). 18 refs., 2 figs.

  17. Patient education for adults with rheumatoid arthritis

    NARCIS (Netherlands)

    Riemsma, R.P.; Kirwan, J.R.; Taal, E.; Rasker, H.J.J.

    2009-01-01

    Patient education shows short-term benefits for adults with rheumatoid arthritis. The purpose was to examine the effectiveness of patient education interventions on health status (pain, functional disability, psychological well-being and disease activity) in patients with rheumatoid arthritis (RA).

  18. Headache associated with moyamoya disease: a case story and literature review.

    Science.gov (United States)

    Zach, Victor; Bezov, David; Lipton, Richard B; Ashina, Sait

    2010-02-01

    Headache associated with moyamoya disease (HAMD) is common in moyamoya disease. However, the characteristics and classification of HAMD are largely unknown. We present a case of a 39-year-old woman with HAMD. To characterize and classify the features of this syndrome, the patient was asked to complete a 4-month diagnostic headache diary. There was a total of 15 ictal days. All episodes were without aura. The headache was more commonly pressing (10/15), mild to moderate in severity (14/15), unchanged by physical activity (11/15), and associated with photophobia (10/15). The International Headache Society Classification was utilized to determine that eight episodes met criteria for probable migraine without aura, while seven episodes met criteria for probable frequent episodic tension-type headache. We identified four other case reports of HAMD with partial descriptions of the characteristics. When combined with our patient, the median age was 34 years old (range 6-49, SD 16). Four were female, while the patient with cluster headache was male. The median time from headache onset to diagnosis with moyamoya disease was 9.5 months (range 0-192, SD 88.0). Headaches were described as migraine with aura in two of five cases, hemiplegic migraine in one of five, and cluster headache in one of five. The highest intensity was described as severe in three of three cases, in which headache intensity was reported. Meanwhile, nausea, vomiting, and photophobia were present in two of three cases, where these features were reported, while nausea without vomiting was seen in one of three cases. In all five cases, patients had other neurological symptoms, such as paresis, seizures, visual disturbances, dysarthria, allodynia, ptosis, and unilateral restless leg syndrome. In conclusion, HAMD can present as migraine without aura. It can be the first presenting symptom of moyamoya disease. The headache features are not diagnostic; hence, early neurovascular imaging should be considered in

  19. Diagnosis of Adult Patients with Cystic Fibrosis.

    Science.gov (United States)

    Nick, Jerry A; Nichols, David P

    2016-03-01

    The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis.

  20. Refractory High Intracranial Pressure following Intraventricular Hemorrhage due to Moyamoya Disease in a Pregnant Caucasian Woman

    Science.gov (United States)

    Montiel, Virginie; Grandin, Cécile; Goffette, Pierre; Fomekong, Edward; Hantson, Philippe

    2009-01-01

    Intraventricular hemorrhage during pregnancy is usually followed by a poor recovery. When caused by moyamoya disease, ischemic or hemorrhagic episodes may complicate the management of high intracranial pressure. A 26-year-old Caucasian woman presented with generalized seizures and a Glasgow Coma Score (GCS) of 3 during the 36th week of pregnancy. The fetus was delivered by caesarean section. The brain CT in the mother revealed bilateral intraventricular hemorrhage, a callosal hematoma, hydrocephalus and right frontal ischemia. Refractory high intracranial pressure developed and required bilateral ventricular drainage and intensive care treatment with barbiturates and hypothermia. Magnetic resonance imaging and cerebral angiography revealed a moyamoya syndrome with rupture of the abnormal collateral vascular network as the cause of the hemorrhage. Intracranial pressure could only be controlled after the surgical removal of the clots after a large opening of the right ventricle. Despite an initially low GCS, this patient made a good functional recovery at one year follow-up. Management of refractory high intracranial pressure following moyamoya related intraventricular bleeding should require optimal removal of ventricular clots and appropriate control of cerebral hemodynamics to avoid ischemic or hemorrhagic complications. PMID:20508823

  1. Refractory High Intracranial Pressure following Intraventricular Hemorrhage due to Moyamoya Disease in a Pregnant Caucasian Woman

    Directory of Open Access Journals (Sweden)

    Virginie Montiel

    2009-02-01

    Full Text Available Intraventricular hemorrhage during pregnancy is usually followed by a poor recovery. When caused by moyamoya disease, ischemic or hemorrhagic episodes may complicate the management of high intracranial pressure. A 26-year-old Caucasian woman presented with generalized seizures and a Glasgow Coma Score (GCS of 3 during the 36th week of pregnancy. The fetus was delivered by caesarean section. The brain CT in the mother revealed bilateral intraventricular hemorrhage, a callosal hematoma, hydrocephalus and right frontal ischemia. Refractory high intracranial pressure developed and required bilateral ventricular drainage and intensive care treatment with barbiturates and hypothermia. Magnetic resonance imaging and cerebral angiography revealed a moyamoya syndrome with rupture of the abnormal collateral vascular network as the cause of the hemorrhage. Intracranial pressure could only be controlled after the surgical removal of the clots after a large opening of the right ventricle. Despite an initially low GCS, this patient made a good functional recovery at one year follow-up. Management of refractory high intracranial pressure following moyamoya related intraventricular bleeding should require optimal removal of ventricular clots and appropriate control of cerebral hemodynamics to avoid ischemic or hemorrhagic complications.

  2. Moyamoya Syndrome Associated with Henoch-Schönlein Purpura

    Science.gov (United States)

    SHIARI, Reza; TABATABAEI NODUSHAN, Seyed Mohamad Hossein; MOHEBBI, Mohamad Mahdi; KARIMZADEH, Parvaneh; JAVADZADEH, Mohsen

    2016-01-01

    Some reports have shown the association between Moyamoya syndrome and autoimmune diseases. Herewith, we present a 3.5 yr old girl with Henoch- Schönleinpurpura (HSP) who was treated with steroids because of sever colicky abdominal pain. However, central nervous system manifestations such as headache, ataxia and vision impairment developed during 6 months of her outpatient follow-up. More evaluation using MRA revealed intracranial stenosis of internal carotid artery and arterial collaterals that were in favor of Moyamoya syndrome. To our knowledge, this is the first report of Moyamoya syndrome following henoch-schönleinpurpura. PMID:27843469

  3. Moyamoya Disease Clinical Course and Severity in Childhood

    Directory of Open Access Journals (Sweden)

    Ayse Kacar Bayram

    2016-01-01

    Full Text Available Aim: Moyamoya disease (MMD is a rare, progressive and oclusive cerebrovascular disorder, predominantly affecting the terminal segment of the internal carotid arteries (ICA and its main branches. The purpose of this study is to evaluate the clinical course and severity of MMD in pediatric patients. Material and Method: We examined 5 consecutive pediatric patients with MMD, focusing on clinical and radiological features, the therapy and outcome over the 58-month follow-up period. Results: The study population consisted of 3 boys and 2 girls. The mean age at diagnosis of patients was 7.2 ± 3.4 years (age range: 3-10 years. The mean duration of follow-up was 30.4 ± 17.4 months (follow-up interval: 12-58 months. Neurological findings at presentation included: motor deficit in 4 patients (80.0%, epileptic seizures in 2 patients (40.0%, movement disorders in 3 patients (60.0%, and headache in 1 patients (20.0%. There was areas of infarction on brain MRI in all patients. Angiographic findings included: internal carotid artery stenosis in all patients, anterior cerebral artery stenosis in 3 patients, middle cerebral artery stenosis in 3 patients, posterior cerebral artery stenosis in 2 patients, and vertebral artery stenosis in 1 patient. Enoxaparine therapy was started to all patients. Subdural hematoma developed in 1 patient during follow-up. Cerebral infarctions recurred despite medical treatment in 4 patients. Discussion: Although this disease is rare, it is an important cause of pediatric stroke. MMD shows different clinical course and disease severity in childhood. Early diagnosis and appropriate treatment are crucial.

  4. A preliminary study of SPECT-MRI fusion imaging by different machine in patients with moyamoya disease%SPECT-MRI异机融合图像在烟雾病中的应用价值初探

    Institute of Scientific and Technical Information of China (English)

    张林悦; 弓健; 郭斌; 唐勇进; 尚靖杰; 徐浩

    2016-01-01

    目的:分析烟雾病(MMD)的单光子发射计算机断层成像(SPECT)、磁共振成像(MRI)图像及SPECT-MRI异机融合图像,探讨SPECT-MRI异机融合图像在烟雾病中的应用价值.方法:回顾性分析符合烟雾病诊断标准的5例MMD患者的99m Tc-ECD脑血流灌注SPECT显像及头颅MRI检查并影像资料,总结其SPECT-MRI影像的特征性表现.结果:SPECT显示额、颞、顶、枕叶放射性稀疏缺损区,脑血流灌注减少;MRI可清晰显示MMD患者的脑实质损害表现(脑梗死、脑出血、脑软化及脑萎缩);SPECT-MRI融合图像显示3例SPECT显示脑血流灌注减低区和MRI显示脑实质损害区是同一部位,SPECT-MRI二者病灶显示匹配;2例SPECT显示脑血流灌注减低区,而相应区域的MRI显示脑实质正常,SPECT-MRI二者病灶显示不匹配.结论:SPECT-MRI融合图像能直观显示烟雾病患者脑实质解剖上的损害及脑血流灌注情况.%Aim:The images of SPECT,MRI,and the SPECT-MRI fusion imaging of Moyamoya dis-ease (MMD)were analyzed and the values of SPECT-MRI fusion imaging in MMD were investigated. Methods:Five cases of patients with MMD were reviewed,which were in accordance with the diagnostic standard.The images of SPECT and MRI and their characteristics of SPECT-MRI fusion imaging wene analyzed.Results:SPECT can show radioactive sparseness areas in frontal lobe,temporal lobe,parietal lobe,occipital lobe.These areas indicate a reduction of cerebral perfusion;MRI can show the lesions clearly in brain parenchymal,such as the cerebral infarction,hemorrhage,encephalomalacia,brain atro-phy.SPECT-MRI fusion imaging shows that the regions of cerebral blood flow perfusion defect in SPECT are the same with the regions of brain damage showed in MRI in three patients,and these lesions match. However,the regions of cerebral blood flow perfusion defect showed in SPECT are normal in MRI, SPECT-MRI shows that these lesions do not match.Conclusion:SPECT-MRI fusion imaging can show

  5. Fanconi anemia associated with moyamoya disease in Saudi Arabia

    OpenAIRE

    Al-Hawsawi, Zakaria M.; Al-Zaid, Mohamed A.; Barnawi, Ashwaq I.; Yassine, Saadeddine M.

    2015-01-01

    We report a 10-year-old Saudi girl who has Fanconi anemia (FA) and was admitted due to acute hemiplegia, of the right side. She had a previous attack of left side hemiplegia that resolved spontaneously. The brain magnetic resonance angiography showed a cerebrovascular pattern of moyamoya disease. She underwent partially matched related donor stem cell transplantation (SCT), but unfortunately died 3 months later with post SCT complications. The association of moyamoya disease with FA is uncomm...

  6. Clinical and experimental pathology of Moyamoya disease

    Institute of Scientific and Technical Information of China (English)

    饶明俐; 张海鸥; 刘群; 张淑琴; 胡林森; 邓方

    2003-01-01

    Objective To investigate the etiology, pathology, and mechanism of pathogenesis of Moyamoya disease.Methods A total of 15 human autopsies were analyzed. In addition, in order to create an animal model of the disease, 21 Japanese rabbits were divided randomly into two groups and subjected to injections of horse serum either intravenously or locally in the area of the sympathetic ganglia. Pathological and immunohistochemical characteristics were observed.Results The pathological features of the autopsies and the animal models both involved intima hyperplasia and stenosis or even occlusion of the lumen in the terminal ends of the internal carotid artery and the anterior and middle cerebral arteries. Disconnections or even breakages of the inner layer of the lumen were also observed, without an obvious inflammatory response. Hyperplasic smooth muscle cells of the medial membrane had extended inward through broken portions of the internal elastic lamina, with intima cell hyperplasia resulting in lumen stenosis. The hyperplastic vascular walls were positive for IgG and IgM.Conclusions The etiology of Moyamoya disease may involve allergic angiitis. A possible mechanism is that proximal portions of the circle of Willis first develop chronic stenosis or occlusion, leading to compensatory small vessel proliferation, which perforates into the cerebral parenchyma.

  7. Neurologic complications of cerebral angiography in childhood moyamoya syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Robertson, R.L.; Chavali, R.V.; Robson, C.D.; Barnes, P.D.; Burrows, P.E. [Department of Radiology, Children`s Hospital Medical Center, Boston, MA (United States); Eldredge, E.A. [Department of Anesthesia, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States); Scott, R.M. [Department of Neurosurgery, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States)

    1998-11-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher`s exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. (orig.) With 8 tabs., 37 refs.

  8. The relevance of hemodynamic factors to perioperative ischemic complications in childhood moyamoya disease.

    Science.gov (United States)

    Iwama, T; Hashimoto, N; Yonekawa, Y

    1996-06-01

    Of 124 children younger than 15 years who underwent surgery for moyamoya disease, 21 (16.9%) experienced perioperative ischemic complications that could not be unequivocally attributed to the surgery. Eleven of the 21 patients experienced infarctions, and 10 experienced reversible ischemic neurological deficits without new lesions, as revealed by computed tomographic scans. An examination of the patients' perioperative clinical and laboratory data revealed that the mean values of intra- and postoperative minimum arterial carbon dioxide pressure, maximum arterial carbon dioxide pressure, and mean arterial pressure were similar in patients with and without ischemic complications. However, in patients with perioperative complications, the incidence of preoperative transient ischemic attacks (TIAs) and intra- and postoperative hypercapnia (maximum arterial carbon dioxide pressure > 45 mm Hg) was significantly higher. In addition, 7 of the 11 perioperative infarctions occurred in patients with frequent preoperative TIAs and intra- and postoperative hypercapnia. Cerebral blood flow studies with preoperative acetazolamide loading showed that the new infarctions were located in areas in which the cerebral blood flow had been compromised. Our results suggest that the occurrence of frequent preoperative TIA is an important indicator of the instability of the cerebral hemodynamics and of the risk of perioperative ischemic complications. To prevent these complications, preoperative management aimed at stabilizing the hemodynamic status is very important. Children who have moyamoya disease and who experience frequent preoperative TIAs are at risk for ischemic brain damage caused by hypercapnia as well as hypocapnia and hypotension. The establishment and maintenance of normocapnia with normotension are highly desirable for the perioperative management of moyamoya disease in children.

  9. MRI and MRA for diagnosis of moyamoya disease in children%儿童烟雾病MRI和MRA的诊断价值

    Institute of Scientific and Technical Information of China (English)

    王凡

    2008-01-01

    Objective To evaluate MRI and MRA in the diagnosis of moyamoya disease in children. Methods MRI and MRA findings and clinical materials of 19 patients with moyamoya disease in children were retrospectively analyzed. While 3D TOP was used for MRA. Enhanced MRA was carried out in 7 cases. Results MRI showed ①moyamoya vessels,bilateral(19cases) ;②0the cortical and subcortical infarction and softening lesions(14 cases) ;and③local brain atrophy(10 cases). M RA showed:Stenosis or occlusion at the terminus of the siphon portions of internal carotid arteries(ICA) and anterior cerebral arteries or middle cerebral arteries (ACA or MCA) ,and abnormal vascular networks at the base of brain were noted by MRA. Conclusion MRI and MRA are effective and noninvasive methods for the diagnosis of moyamoya disease. MRI combined with MRA may be used for the diagnoses of moyamoya disease.%目的 探讨MRI和MRA对儿童烟雾病(Moyamoya)的诊断价值.方法 分析19例烟雾病患儿的MRI、MRA表现和临床资料.MRA采用3D TOP法,7例行增强MRA.结果 MRI表现为①Moyamoya血管(双侧、19例);②大脑半球皮质和/或皮质下梗死、软化灶(14例);③局部脑萎缩(10例).MRA表现为颈内动脉虹吸部末端和大脑前或中动脉近端狭窄或闭塞,并有脑底部异常血管网形成.结论 MRI和MRA是诊断烟雾病有效非创伤性的检查方法.MRI与MRA相结合可作为儿童烟雾病诊断的首选方法.

  10. The value of hyperintense vessel signs on fluid-attenuated inversion recovery imaging for assessing the patterns of collateral blood flow in adult moyamoya disease%液体衰减反转恢复成像-高信号血管征评估成年型烟雾病颅内侧支血流模式的价值

    Institute of Scientific and Technical Information of China (English)

    刘文华; 黄显军; 李永坤; 朱武生; 马敏敏; 徐格林; 刘新峰

    2012-01-01

    目的 探讨MRI-液体衰减反转恢复(FLAIR)序列-高信号血管征(hyperintense vessel signs,HVS)评估烟雾病患者颅内侧支血流模式的价值.方法 从南京卒中注册系统中提取2008年8月至2011年1月经脑血管造影诊断,且实施了MRI-FLAIR序列检查的41例非脑出血性成年型烟雾病患者临床资料.根据HVS在大脑中动脉供血区域分布部位的不同,将其分布模式分为0~3级:0 级是指缺乏HVS;l级是指HVS主要分布在颞叶脑沟和外侧裂区域;2级是指HVS主要分布在顶、额叶脑沟和外侧裂区域;3级是指HVS分布于1级和2级联合的区域.另外,依据脑血管造影结果,将继发于血管狭窄和闭塞的颅内侧支血流模式分为1~3型:第1型为通过病变血管或其周围的新生血管形成的顺向性残余血流;第2型为通过软膜血管形成的逆向性血流;第3型为第1和第2型联合组成的混合性血流.分析烟雾病患者颅内侧支血流模式与HVS分布之间的关系.结果 在41例非出血性成年型烟雾病患者中,3例为单侧病变,38例为双侧血管病变,故存在烟雾血管的半球数为79侧;因3例患者双侧大脑半球表现HVS缺如,故存在HVS的半球数为73侧,HVS阳性率达92.4%(73/79).重要的是,1级HVS对应的均是缓慢的顺向性血流(7/7);2级HVS主要对应的是缓慢的逆向性软脑膜血流(95.0%,19/20);3级主要对应的是缓慢的混合性血流(84.8%,39/46).且随着HVS分布从颞叶脑沟转移至额、顶叶脑沟,侧支血流的方向由顺向转变为逆向,此转变趋势有统计学意义.结论 不同分布部位的HVS反映了不同模式的侧支血流,HVS能无创评估成年型烟雾病患者颅内侧支血流方向.%Objective To investigate the value of hyperintense vessel signs (HVS) on fluidattenuated inversion recovery (FLAIR) sequence for assessing the patterns of collateral blood flow in adult moyamoya disease (MMD).Methods Forty-one adult patients

  11. Analysis of the Results of Antinuclear Antibody Spectrum for Patients of Moyamoya%402例烟雾病抗核抗体谱检测结果分析

    Institute of Scientific and Technical Information of China (English)

    田曙光; 曾利军; 陈建魁; 于农; 宋世平; 尹秀云; 黄媛; 金欣; 左向华; 杜宇

    2011-01-01

    Objective: Look for the occurrence of moyamoya disease with autoimmune of certain links. Methods: 402 cases of moyamoya disease spectrum antinuclear antibod respectively. Results: 112 cases of anti-nuclear antibody spectrum result was positive disease, the positive rate was 27.86%, and the percentage of anti-dsDNA, anti-CB, ant B, anti Jo-1, anti-RO-52, anti-ScL-70, anti-RIB, anti-PM-sd, anti-nRNP, anti-PCr anti-M2 antibodies were 16.07%, 1.79%, 5.36%, 17.86%, 7.14%, 5.36%, 13.39%, 5.3< 11.61%, 6.25% and 8.93%, 8.04%, respectively. Conclusion: Autoimmune disease nu moyamoya disease.%目的:探讨烟雾病的发生与自身免疫性疾病是否存在一定的联系.方法:对402例烟雾病抗核抗体谱检测结果进行回顾性分析.结果:402例烟雾病确诊病例中,有112位患者抗核抗体谱检测结果中存在阳性结果,阳性率为27.86%.其中,抗dsDNA、抗CB、抗Sm、抗SS-A、抗SS-B、抗Jo-1、抗RO-52、抗ScL-70、抗RIB、抗PM -sd、抗nRNP、抗PCNA、抗Nuc、抗HI、抗M2抗体在阳性病例中所占百分比分别为16.07%、1.79%、5.36%、17.86%、7.14%、5.36%、13.39%、5.36%、7.14%、4.46%、2.68%、11.61%、6.25%、8.93%和8.04%.结论:烟雾病患者中抗核抗体阳性率较高,自身免疫性疾病可能是烟雾病发生的重要诱因之一.

  12. Surgical excision of heterotopic ossification of hip in a rare case of Moyamoya disease with extra articular ankylosis

    Directory of Open Access Journals (Sweden)

    Dhanasekararaja Palanisami

    2012-01-01

    Full Text Available We report a case of isolated ossification of iliopsoas with ankylosis of the left hip in a 27-year-old female. The patient was diagnosed to have Moyamoya disease, a rare chronic occlusive disorder of cerebrovascular circulation following an acute onset of hemiplegia. The patient presented 9 months later to us with ankylosis of left hip which was successfully treated by surgical excision of the heterotopic bone and there was no recurrence at the end of 5 years. A review of literature failed to reveal a similar case with isolated and complete ossification of iliopsoas muscle associated with Moyamoya disease which required surgical intervention. Surgical excision resulted in dramatic improvement in the quality of life. Surgical excision of neurogenic type of heterotopic ossification is a very successful procedure and timely intervention after maturity of mass is very important to prevent the onset of secondary complications and to avoid recurrence.

  13. Treatment of Moyamoya disease by multipoint skull drilling for indirect revascularization combined with mobilization of autologous bone marrow stem cells.

    Science.gov (United States)

    Wu, R; Su, N; Zhang, Z; Jia, F

    2015-07-06

    This study discusses the clinical efficacy of multipoint skull drilling for indirect revascularization combined with mobilization of autologous bone marrow stem cells and use of simvastatin in the treatment of moyamoya disease. Seventy-eight patients [control group (group A), 39 patients; experimental group (group B), 39 patients] with moyamoya disease were selected. Group A underwent indirect revascularization, and group B, in addition to indirect revascularization, received alternate subcutaneous injections from day 7 post-surgery. The number and differentiation of the mobilized bone marrow stem cells were detected by the proportion of hematopoietic progenitor cell (HPCs) in mononuclear cells (MNCs) in the peripheral blood. There was no statistical difference in the BI (80.2 ± 13.7) and NIHSS (6.7 ± 2.3) scores between the groups before treatment (P > 0.05). The CSS score of group B was 13.5 ± 0.6 and there was a statistical significance compared to group A (18.2 ± 0.8) (P 0.05) and the proportions of CD34+ CDl33+ cells in MNCs in peripheral blood in groups A and B at 30 days after surgery were significantly higher than those before surgery (P moyamoya disease by multipoint skull drilling for indirect revascularization combined with mobilization of autologous bone marrow stem cells and simvastatin is a safe and effective method as it can promote recovery of neurological functions and improve patients' daily living abilities and quality of life.

  14. A study on cerebral hemodynamic analysis of moyamoya disease by using perfusion MRI

    Science.gov (United States)

    Dong, Kyung-Rae; Goo, Eun-Hoe; Lee, Jae-Seung; Chung, Woon-Kwan

    2013-10-01

    This study examined the clinical applications of perfusion magnetic resonance imaging (MRI) in patients with moyamoya disease (MMD). Twenty-two patients with moyamoya disease (9 men and 13 women) with a mean age of 9.3 years (range: 4-22 years) were enrolled in this study. Perfusion MRI was performed by scanning the patients7.5 cm upward from the base of the cerebellum before their being process for post-treatment. The scan led to the acquisition of the following four map images: the cerebral blood volume (CBV), the cerebral blood flow (CBF), the mean transit time (MTT) for the contrast medium, and the time to peak (TTP) for the contrast medium. The lesions were assessed using the CBV, the CBF, the MTT and the TTP maps of perfusion MRI; the MTT and the TTP were measured in the lesion areas, as well as in the normal and the symmetric areas. Perfusion defects were recognizable in all four perfusion MRI maps, and the MTT and the TTP showed a conspicuous delay in the parts where perfusion defects were recognized. The MTT and the TTP images of perfusion MRI reflected a significant correlation between the degrees of stenosis and occlusion in the posterior cerebral artery (PCA), as well as the development of collateral vessels. The four perfusion MRI maps could be used to predict the degrees of stenosis and occlusion in the posterior circulation, as well as the development of the collateral vessels, which enabled a hemodynamic evaluation of the parts with perfusion defects. Overall, perfusion MRI is useful for the diagnosis and the treatment of moyamoya disease and can be applied to clinical practice.

  15. MR imaging of ischemic parenchymal lesions in moyamoya disease of children

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Whal; Kim, In One; Kim, Woo Sun; Yeon, Kyung Mo; Han, Man Chung [Seoul National Univ. College of Medicine, the Institute of Radiation Medicine and Neuroscience Research Institute, SNUMRC, Seoul (Korea, Republic of); Cho, Byung Kyu; Wang, Kyu Chang; Hwang, Yong Seung [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-12-01

    To determine by means of MR imaging the ischemic status of parenchymal lesions in moyamoya disease. Ninety-two MR images in 50 children wiht moyamoya disease were retrospectively reviewed. Ischemic parenchymal lesions were categorized according to the signal intensities of cortex and subcortical white matter. We also analyzed enhancement patterns, time sequential changes in the lesions, and the Prognosis for each patient, according to lesion type. Among one hundred and seventeen parenchymal abnormalities, 89 gyral lesions were seen in 43 children (86%), predominantly in the frontal area (33.1%). Cortical parenchymal lesions were categorized as either type I-intermediate to high signal intensity (SI) on both T2 weighted (T2WI) and proton density images (PDI), and associated with low SI of the subcortical white matter;type II-high SI on T2WI and PDI, without low SI of the subcortical white matter;or type III-high SI on T2WI and iso SI on PDI. Thirty-three lesions were type I, ten were type II, and 43 were type III. Time sequential changes from type I to type II, and then to type III, were observed. The prognoses of patients with a type-I lesion were better than those of patients whose lesions were type II or III. Type I lesions presented with abnormal low signal intensity in the subcortical white matter, as seen on T2WI Images. This was the characteristic and earliest finding of ischemic parenchymal lesions in moyamoya disease; sequential MR images showed that type-I lesions progressed to type II or III.

  16. Tuberous sclerosis complex and polycystic kidney disease contiguous gene syndrome with Moyamoya disease.

    Science.gov (United States)

    Lai, Jonathan; Modi, Lopa; Ramai, Daryl; Tortora, Matthew

    2017-04-01

    Tuberous sclerosis complex (TSC) and autosomal dominant polycystic kidney disease (ADPKD) are two diseases sharing close genetic loci on chromosome 16. Due to contiguous gene syndrome, also known as contiguous gene deletion syndrome, the proximity of TSC2 and PKD1 genes increases the risk of co-deletion resulting in a shared clinical presentation. Furthermore, Moyamoya disease (MMD) is a rare vaso-occlusive disease in the circle of Willis. We present the first case of TSC2/PKD1 contiguous gene syndrome in a patient with MMD along with detailed histopathologic, radiologic, and cytogenetic analyses. We also highlight the clinical presentation and surgical complications in this case.

  17. Doença cerebrovascular tipo "moyamoya"

    Directory of Open Access Journals (Sweden)

    Reynaldo A. Brandt

    1976-09-01

    Full Text Available É relatado o caso de um paciente masculino, branco, 34 anos, que apresentou hemorragia meníngea sem sinais de comprometimento cerebral. A carotidoangiografia direita mostrou as alterações típicas do "moyamoya", com estenose da artéria carótida interna ao nível do sifão, acima da origem da artéria comunicante posterior; há uma rede vascular angiomatosa anormal projetando-se na base do cérebro, da qual nascem as artérias cerebrais anterior e média, sendo as artérias comunicante e cerebral posterior hipertrofiadas e de trajeto tortuoso; há anastomoses meningo-corticiais, sendo as artérias meníngeas hipertrofiadas. A carotidoangiografia esquerda mostrou hipertrofia das artérias carótidas comum e interna, com contrastação de ambas as artérias cerebrais anteriores e médias a partir deste lado; a artéria comunicante anterior é do tipo fetal e há a presença anômala da artéria cerebral anterior mediana do corpo caloso. Estes dados apoiam a teoria segundo a qual a doença constitui anomalia do desenvolvimento vascular encefálico, não tendo sido encontrado outro caso na literatura com hipertrofia vicariante da artéria carótida interna contralateral à da lesão típica do "moyamoya".

  18. Arterial spin-labeling MR imaging in moyamoya disease compared with clinical assessments and other MR imaging finings

    Energy Technology Data Exchange (ETDEWEB)

    Noguchi, Tomoyuki, E-mail: tnogucci@radiol.med.kyushu-u.ac.jp [Department of Radiology, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Kawashima, Masatou [Department of Neurosurgery, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Nishihara, Masashi; Hirai, Tetsuyoshi [Department of Radiology, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Matsushima, Toshio [Department of Neurosurgery, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Irie, Hiroyuki [Department of Radiology, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan)

    2013-12-01

    Purpose: Our purpose was to identify the causal factors for the perfusion distribution obtained with ASL-MRI by comparing ASL-MRI with clinical information and other MRI findings in moyamoya disease. Methods: Seventy-one patients with moyamoya disease underwent ASL-MRI and other MRI, including fluid-attenuated inversion recovery imaging (FLAIR) and three-dimensional time-of-flight magnetic resonance angiography (MRA) on 3.0-Tesla MRI system. Cerebral blood flow (CBF) values (ASL values) for the cerebral hemispheres (142 sides) were measured on CBF maps generated by ASL-MRI. Relationships between the ASL values and the following 9 factors were assessed: sex, family history, revascularization surgery, age at MR exam, age at onset, the steno-occlusive severity on MRA (MRA score), degree of basal collaterals, degree of leptomeningeal high signal intensity seen on FLAIR, and size of ischemic or hemorrhagic cerebrovascular accident lesion (CVA score). Results: Patients with a family history had significantly higher ASL values than those without such a history. There were significant negative correlations between ASL values and age at MR exam, MRA score, and CVA score. Conclusions: ASL-MRI may have cause-and-effect or mutual associations with family history, current patient age, size of CVA lesion, and intracranial arterial steno-occlusive severity in Moyamoya disease.

  19. Chronic subdural hematoma associated with moyamoya phenomenon after radiotherapy for medulloblastoma; A case report

    Energy Technology Data Exchange (ETDEWEB)

    Fuse, Takahisa; Takagi, Takuji; Fukushima, Tsuneyuki; Mizuno, Shiroh; Hashimoto, Nobukazu; Suzuki, Osamu (Nagoya City Higashi General Hospital (Japan))

    1994-04-01

    A 9-year-old boy had been diagnosed at the age of 9 months as having a cerebellar medulloblastoma and had received 40 Gy of radiation therapy to the brain after removal of the tumor. Cerebral angiography at the time of initial diagnosis did not show any evidence of occlusive disease involving the internal carotid circulation. At the age of 6 years, the patient developed generalized seizures. On examination, he was drowsy and had right hemiparesis. CT scan demonstrated a low-density area in the left frontal lobe. Cerebral angiography showed a marked narrowing of the bilateral internal carotid arteries with moyamoya vessels. The patient was treated medically with aspirin (100 mg/day) and anticonvulsants. His neurological deficits improved gradually. At the age of 8 years, there was no recurrence of the tumor although a slight left subdural hematoma was seen on CT scan. On August 10, 1993, at the age of 9 years, he was admitted for treatment of a developing subdural hematoma. MRI showed a chronic subdural hematoma with thick outer and inner membranes. Cerebral angiography showed occlusion of the left internal carotid artery which fed the right frontal lobe through moyamoya vessels, marked narrowing of the right internal carotid artery distal to the ophthalmic artery, moyamoya vessels at the base, and cortical revascularization througth the ophthalmic, posterior cerebral and middle meningeal arteries. Trepanation and aspiration of the hematoma were performed. The outer membrane of the hematoma was about 2 mm thick and the hematoma cavity was filled with a partially organized hematoma. In this case, we speculate that development of the chronic subdural hematoma involved the following factors: (1) transdural external-internal carotid anastomosis after radiation-induced cerebrovasculopathy; (2) repeated mild head trauma due to gait disturbance after removal of the cerebellar tumor; and (3) administration of acetylsalicylic acid. (author).

  20. Combined extracranial-intracranial bypass surgery with stent-assisted coil embolization for moyamoya disease with a ruptured wide-necked basilar trunk aneurysm: a case report.

    Science.gov (United States)

    Jiang, Hanqiang; Ni, Wei; Lei, Yu; Li, Yanjiang; Gu, Yuxiang

    2015-01-01

    A ruptured wide-necked basilar trunk aneurysm is uncommon in patients with moyamoya disease. The optimal treatment is unclear. We report a safe and beneficial treatment modality for moyamoya disease with aneurysms located in the posterior circulation. A 37-year-old man presenting with subarachnoid hemorrhage was admitted to our hospital. Emergent cerebral angiography demonstrated moyamoya disease associated with a wide-necked basilar trunk aneurysm. We performed bilateral extracranial-intracranial bypass surgeries prior to stent-assisted coil embolization of the aneurysm after the acute phase. No complication occurred and the patient was discharged with no neurological deficit. Follow-up digital subtraction angiography (DSA) performed 6 months after the surgery showed that all the anastomosises were patent and bilateral collateral vascular compensation was fully established with no recanalization of the basilar trunk aneurysm post embolization.We also found that high-flow bypass did not contribute to cerebral revascularization as imagined despite the good patency. Combined extracranial-intracranial bypass surgery with endovascular treatment proved to be an efficient therapeutic modality for moyamoya disease with aneurysms located in the posterior circulation. High-flow bypass surgery was not essential due to the inefficiency and the high risk of postoperative cerebral hyperperfusion syndrome.

  1. Autoimmune lymphoproliferative disorder in an adult patient

    Directory of Open Access Journals (Sweden)

    N K Desai

    2011-01-01

    Full Text Available A 50-year-old male patient presented with fever, epistaxis and multiple lymphadenopathy since 15 days. In the light of the above presentation a complete workup was initiated to exclude common conditions like tuberculosis, acquired immunodeficiency syndrome, lymphoid malignancy and sarcoidosis. After excluding common conditions a biopsy of cervical lymph node demonstrated reactive lymphadenitis with paracortical hyperplasia. Immunohistochemistry demonstrated double negative lymphocytes (CD4-, CD8-. A diagnosis of autoimmune lymphoproliferative disorder syndrome (ALPS (probable was made and patient was started on 1 mg/kg of steroids. Patient showed a dramatic improvement with respect to general wellbeing, fever and regression of lymphadenopathy. This entity of ALPS has been recently identified and classified; most of the reports are from the pediatric population. To the best of our knowledge ours is one of the few cases of this entity being reported in an adult patient from India.

  2. MRI and N-isopropyl(I-123)p-iodoamphetamine SPECT findings in cases of moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Suto, Yuji; Kato, Terumi; Ohta, Yoshio (Tottori Univ., Yonago (Japan). School of Medicine); Caner, B.E.

    1993-07-01

    Six patients with moyamoya disease underwent magnetic resonance imaging (MRI), and in 4 of 6 cases, MRI findings were compared with N-isopropyl(I-123)p-iodoamphetamine (IMP) single-photon emission computed tomography (SPECT) brain scans to determine if there was a correlation between the morphology, as seen on MRI, and cerebral perfusion and/or metabolic changes detected by IMP-SPECT. MRI clearly revealed abnormalities secondary to moyamoya disease: nonvisualization of a signal void flow sign in the arteries, moyamoya vessels, and cerebral atrophy. All the perfusion abnormalities of the cortex, except a small one, observed on MR images were also detected on IMP-SPECT images, but small white matter abnormalities demonstrated on MR images could not be revealed by IMP-SPECT. Interestingly, in 2 patients, 2 additional cortical defects that were not observed on MR images were revealed by IMP-SPECT. Moreover, 3 areas with perfusion defects were larger on the IMP-SPECT scans than on the MR images. The cortical defects observed on the IMP-SPECT images but not on the MR images may reflect mild ischemia and/or certain metabolic abnormalities that lead to low tracer accumulation. Overall, IMP-SPECT and MRI may play complementary roles in the evaluation of this disease. (author).

  3. Ab interno trabeculectomy in the adult patient.

    Science.gov (United States)

    SooHoo, Jeffrey R; Seibold, Leonard K; Kahook, Malik Y

    2015-01-01

    Glaucoma is a potentially blinding disease that affects millions of people worldwide. The mainstay of treatment is lowering of intraocular pressure (IOP) through the use of medications, laser and/or incisional surgery. The trabecular meshwork (TM) is thought to be the site of significant resistance to aqueous outflow in open angle glaucoma. Theoretically, an incision through TM or TM removal should decrease this resistance and lead to a significant reduction in IOP. This approach, commonly referred to as goniotomy or trabeculotomy, has been validated in the pediatric population and has been associated with long-term IOP control. In adults, however, removal of TM tissue has been historically associated with more limited and short-lived success. More recent evidence, reveals that even adult patients may benefit significantly from removal of diseased TM tissue and can lead to a significant reduction in IOP that is long-lasting and safe. In this review, we discuss current evidence and techniques for ab interno trabeculectomy using various devices in the adult patient.

  4. Recent advances in moyamoya disease: pathophysiology and treatment.

    Science.gov (United States)

    Kronenburg, Annick; Braun, Kees P J; van der Zwan, Albert; Klijn, Catharina J M

    2014-01-01

    Moyamoya disease is a progressive intracranial arteriopathy characterized by bilateral stenosis of the distal portion of the internal carotid artery and the proximal anterior and middle cerebral arteries, resulting in transient ischemic attacks or strokes. The pathogenesis of moyamoya disease remains unresolved, but recent advances have suggested exciting new insights into a genetic contribution as well as into other pathophysiological mechanisms. Treatment that may halt progression of the disease or even reverse the intracranial arteriopathy is yet to be found. There are strong indications that neurosurgical intervention, through direct, indirect, or combined revascularization surgery, can reduce the risk of ischemic stroke and possibly also cognitive dysfunction by improving cerebral perfusion, although randomized clinical trials have not been performed. Many questions regarding the indication for and timing of surgery remain unanswered. In this review, we discuss recent developments in the pathogenesis and treatment of moyamoya disease.

  5. Cranial CT of the unconscious adult patient

    Energy Technology Data Exchange (ETDEWEB)

    Harden, S.P. [Department of Radiology, Southampton General Hospital, Southampton (United Kingdom); Dey, C. [Department of Radiology, Southampton General Hospital, Southampton (United Kingdom); Gawne-Cain, M.L. [Department of Radiology, Southampton General Hospital, Southampton (United Kingdom)]. E-mail: mary.gawne@suht.swest.nhs.uk

    2007-05-15

    Unconscious patients are frequently referred to radiology departments for computed tomography (CT) of the brain. The objectives of these examinations are to define the underlying cause of impaired consciousness and in some cases to determine the severity of associated brain injury. There is often little history available to guide the clinician or radiologist, particularly in patients referred from the casualty department. In this review, we present the typical CT appearances of adult patients presenting with loss of consciousness for CT examination. We focus on the most common abnormalities that are identified in everyday radiological practice, emphasize important diagnostic signs that may enable a confident diagnosis to be made and discuss when further imaging may be warranted.

  6. Imaging of disease progression in a case of idiopathic moyamoya

    Directory of Open Access Journals (Sweden)

    A. Chacko

    2013-08-01

    Full Text Available Moyamoya is a rare cerebrovascular disease characterised by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. We report on the progression of the disease in a 3-year-old boy, confirmed to have idiopathic Moyamoya disease, over 6 ensuing years, using serial magnetic resonance imaging with MR angiography. Comparison is also made with conventional angiography at the last visit. Characteristic imaging appearances of asymmetric narrowing of the internal carotid arteries (especially the supra-clinoid portion with numerous collateral vessels around the brainstem especially within the ambient and quadrigeminal plate cisterns are demonstrated with progression.

  7. Clinical and imaging characteristics of Moyamoya disease: report of forty-one cases%41例成人烟雾病的临床特点及血管影像学分析

    Institute of Scientific and Technical Information of China (English)

    张萍; 梅翠红; 陈黛琪; 田代实; 李倩; 骆翔

    2011-01-01

    Objective To analyze the clinical and digital subtraction angiography ( DSA ) features of adult patients with Moyamoya disease. Methods 41 patients who were diagnosed as Moyamoya disease wereinvestigated. The clinical representation and DSA imagines were observed. Results This disease primarily affected youth and middle-ages, with an average age of 40 years. No conspicuous difference between male and female. The forms of disease onset were all acute cerebral accidents. 19. 5% was ischemic type, while 80. 5% was hemorrhage type. DSA could find vascular stenosis or occlusion in the circle of willis and collateral circulation formed to compensate. Aneurysm formed in some cases. Conclusion Moyamoya disease should be considered when stroke recurrently attacks in youth and middle-ages, especially without risk factors. DSA should be performed in order to identify diagnosis.%目的 总结成人烟雾病的临床及血管影像学特点.方法 回顾性分析41例成人烟雾病的临床表现及影像学资料,以观察其临床及影像学特点.结果 本组患者多为青壮年起病,平均起病年龄40岁左右,无明显性别差异;均以脑卒中形式急性起病,缺血性卒中占19.5%,出血性卒中占80.5%;DSA表现为Willis环附近动脉狭窄或闭塞,脑底烟雾状血管网、侧枝循环及动脉瘤形成.结论 青壮年反复发作的脑卒中,尤其是无高血压病及动脉硬化证据时,应警惕烟雾病的可能,需行全脑DSA检查以明确诊断.

  8. [A Case of Ruptured Peripheral Cerebral Aneurysm at Abnormal Vessels Associated with Middle Cerebral Artery Stenosis:Similarity to Moyamoya Disease].

    Science.gov (United States)

    Miyazaki, Hajime; Kohno, Kanehisa; Tanaka, Hideo; Fukumoto, Shinya; Ichikawa, Haruhisa; Onoue, Shinji; Fumoto, Noriyuki; Ozaki, Saya; Maeda, Toshiharu

    2016-04-01

    We report a case of ruptured peripheral cerebral aneurysm at abnormal vessels associated with severe stenosis at the middle cerebral artery (MCA). A 66-year-old woman was admitted at our hospital with headache on foot. Computed tomography (CT) showed intracerebral hemorrhage in the left fronto-basal area. Three-dimensional-CT and conventional angiogram revealed abnormal vessels, which were similar to those seen in moyamoya disease, with a small enhancement close to the hematoma. On day 11, subsequent cerebral angiogram demonstrated an aneurysm at the peripheral portion of an abnormal vessel arising from the left A2. On day 17, soon after the diagnosis of the ruptured aneurysm was made (while still at the subacute stage), we operated on the aneurysm. Superficial temporal artery (STA)-MCA anastomosis was also performed to preserve cerebral blood flow and reduce hemodynamic stress. Several days after the operation, she had transient aphasia due to hyperperfusion of the MCA territory, but eventually recovered with no neurological deficit at discharge. Follow-up study revealed revascularization from the branches of the external carotid artery as well as the STA. On admission, we initially thought that this patient had abnormal vessels associated with arteriosclerotic MCA stenosis. However, the postoperative clinical course as well as the histopathological specimens of both the abnormal artery with the aneurysm and the STA revealed similar findings to those of moyamoya disease. Although this case did not satisfy the criteria for moyamoya disease, it is conceivable that a single arterial occlusive lesion associated with moyamoya-like vessels might develop in the same mechanism with that of moyamoya disease.

  9. Role of Ring Finger Protein 213 in Moyamoya Disease

    Science.gov (United States)

    Ma, Yong-Gang; Zhang, Qian; Yu, Le-Bao; Zhao, Ji-Zong

    2016-01-01

    Objective: The aim of this study was to help people comprehensively understand the research advances related to ring finger protein 213 (RNF213) in moyamoya disease (MMD) and to understand the disease at the molecular level to provide a new perspective of the diagnosis of the disease. Data Sources: This review was based on data in articles published between 2005 and 2015 that were retrieved from the PubMed database. The search terms included RNF213, MMD, intracranial major artery stenosis/occlusion (ICASO), genotype, phenotype, mutant and variants, and the combinations of these terms. Study Selection: Articles related to MMD and RNF213 were selected for review, and we also reviewed publications related to ICASO. Results: RNF213 is not only associated with MMD but also associated with intracranial major artery stenosis. In addition, RNF213 variants exhibit apparent ethnic diversity; specifically, the c.14576G>A variant is mainly detected in Korean, Chinese, and Japanese populations, particularly the latter population. The genotypes of RNF213 correlate with the phenotypes of MMD; for example, the homozygous c.14576G>A variant is associated with early-onset, severe symptoms, and an unfavorable prognosis. Furthermore, the RNF213 c.14576G>A variant should be considered during the diagnosis of MMD because no patients with quasi-MMD have been reported to carry the RNF213 c.14576G>A variant whereas 66 of 78 patients with definite MMD have been found to carry this variant. Conclusions: The growing literature demonstrates that MMD is primarily caused by the synergy of genetic and environmental factors, and unknown genetic modifiers might play roles in the etiology of MMD. Further research should be conducted to clarify the pathogenic mechanism of MMD. PMID:27748344

  10. Síndrome de Down e Moyamoya: estudo através de metanálise Moyamoya and Down syndrome: study conducted by meta-analysis

    Directory of Open Access Journals (Sweden)

    Paulo Alves Junqueira

    2002-06-01

    Full Text Available Apresentamos o estudo clínico-epidemiológico de dois pacientes e metanálise (período 1977-2000 sobre a comorbidade entre síndrome de Down (SD e síndrome de "moyamoya" (SMM. Entre os 42 pacientes catalogados no presente estudo, a metanálise permitiu verificar: maior número de publicações de pesquisadores do Japão e Estados Unidos, seguidos pelo Brasil e Itália; predomínio do acidente vascular cerebral (AVC em lactentes e pré-escolares; sintomatologia inaugural, hemiparesia (78,6%, distúrbio da fala (26,2%; predomínio de infarto isquêmico (76,2%; episódios isquêmicos recorrentes (62%; comprometimento bilateral (83,3%. Esta análise permitiu concluir que, na investigação clínico-neurológica de pacientes com SD e episódios de hemiparesia aguda, a SMM deve ser incluída como diagnóstico mais provável .We present a clinic-epidemiological study of two patients and meta-analysis (period 1977-2000 of the co-morbidity of the Down syndrome (DS and moyamoya syndrome (MMS. Among the 42 patients listed in this survey, meta-analysis permitted to find the highest number of publications by researchers from Japan and United States, followed by Brazil and Italy; prevalence of cerebrovascular disease in suckling and pre school children; first symptomatology was hemiparesis (78.6%, speech disorders (26.2%; ischemic infarction (76.2%; recurring ischemic episodes (62%; bilateral impairment (83.3%. This analysis led to the conclusion that in the clinic-neurological investigation of DS patients with acute hemiparesis episodes, MMS should be included as the most probable diagnosis.

  11. Cardiac arrhythmias in adult patients with asthma

    DEFF Research Database (Denmark)

    Warnier, Miriam J; Rutten, Frans H; Kors, Jan A;

    2012-01-01

    OBJECTIVE: The pathogenesis of cardiac arrhythmias in asthma patients has not been fully elucidated. Adverse drug effects, particularly those of β2-mimetics, may play a role. The aim of this study was to determine whether asthma is associated with the risk of cardiac arrhythmias and electrocardio......OBJECTIVE: The pathogenesis of cardiac arrhythmias in asthma patients has not been fully elucidated. Adverse drug effects, particularly those of β2-mimetics, may play a role. The aim of this study was to determine whether asthma is associated with the risk of cardiac arrhythmias...... and electrocardiographic characteristics of arrhythmogenicity (ECG) and to explore the role of β2-mimetics. METHODS: A cross-sectional study was conducted among 158 adult patients with a diagnosis of asthma and 6303 participants without asthma from the cohort of the Utrecht Health Project-an ongoing, longitudinal, primary...... or flutter). Secondary outcomes were tachycardia, bradycardia, PVC, atrial fibrillation or flutter, mean heart rate, mean corrected QT (QTc) interval length, and prolonged QTc interval. RESULTS: Tachycardia and PVCs were more prevalent in patients with asthma (3% and 4%, respectively) than those without...

  12. Surgical treatment of moyamoya disease: operative technique for encephalo-duro-arterio-myo-synangiosis, its follow-up, clinical results, and angiograms.

    Science.gov (United States)

    Kinugasa, K; Mandai, S; Kamata, I; Sugiu, K; Ohmoto, T

    1993-04-01

    Moyamoya syndrome is defined as the development of collateral anastomosis pathways at the base of the brain, associated with chronic progressive stenosis of the carotid fork. Both reconstructive vascular surgery and conservative strategies are used to treat this syndrome, but the latter cannot prevent the disease from progressing. We describe the procedure of encephalo-duro-arterio-myo-synangiosis (EDAMS), and report the results in 17 patients (28 sides) who underwent EDAMS. The clinical symptoms of moyamoya disease include transient ischemic attacks, reversible ischemic neurological deficits, stroke, seizures, Gerstmann's syndrome, involuntary movements, or mental retardation resulting from the lack of cerebral blood flow. The clinical results of EDAMS were poor in one patient, fair in two, good in five, excellent in eight, and fair on one side and excellent on the other side in one patient. Postoperative angiograms showed widespread collateral circulation on the ischemic brain surface in patients undergoing EDAMS.

  13. Moyamoya的间接血运重建%Indirect Revascularization for Moyamoya

    Institute of Scientific and Technical Information of China (English)

    赵文元; 刘建民

    2011-01-01

    @@ 由于Moyamoya病与Moyamoya综合征的自然病程相近,在手术策略上并无特殊差异,因此常笼统将二者统称为Moyamoya.根据临床表现和发病年龄的不同,Moyamoya可分为缺血型Moyamoya、出血型Moyamoya,或儿童Moyamoya、成人型Moyamoya.

  14. MRI and MRA signs of moyamoya disease%烟雾病的MRI和MRA征象

    Institute of Scientific and Technical Information of China (English)

    许秋霞; 詹浩辉; 程鹏

    2013-01-01

    Objective:By analyzing signs of 27 cases of MRI and MRA images of moyamoya disease (MMD),to further improve the diagnosis level of moyamoya disease.Methods:Via retrospective analysis of 27 patients with cerebral MRI and MRA check and MMD patients' image data which confirmed to MMD diagnosis standard enacted in 1997 by the Japanese Moyamoya Disease Research Council,summing up the MRI and MRA characteristic signs.Results:MRI could clearly show substantive damage of MMD patients with cerebral infarction,cerebral hemorrhage,cerebral softening,or brain atrophy,and low signal vascular network of anomaly expansion and circuitous of basement department of brain were visible:5 cases in T1WI and FLAIR saw leptomeninges signs; 3 D-TOF MRA directly showed the ICA end and (or) ACA,MCA near-end occlusion or stenosis,smoke shape arteriolar and cerebral collateral circulation; CE-MRA displayed collateral vessels more clearly; MRI signs of brain damage and MRA signs of vascular stenosis or occlusion condition were not completely consistent,which had close relation to cerebral collateral circulation angiogenesis.Conclusion:MRA sign of moyamoya disease has characteristics,so do MRI signs of smoke shape empty vessels shadow and leptomeninges but not as intuitive as MRA.MRI signs of brain damage have close relationship with MRA signs of collateral circulation,MRI and MRA could make clear MMD diagnosis.%目的:分析27例烟雾病(moyamoya disease,MMD)的MRI和MRA征象,以进一步提高对烟雾病的诊断水平.方法:回顾性分析27例经颅脑MRI及MRA检查并符合日本烟雾病研究委员会1997年制定的烟雾病诊断标准的MMD患者的影像资料,总结其MRI和MRA的特征性表现.结果:MRI可清晰显示MMD患者的脑梗死、脑出血、脑软化、脑萎缩等脑实质损害表现,亦可见脑基底部异常扩张迂曲的低信号血管网;5例于T1WI和FLAIR见柔脑膜征;3D-TOF MRA直接显示了颈内动脉末端和/(或)大脑前中动脉近端闭塞

  15. 21例烟雾病的临床表现及双源FlashCT应用价值分析%Analysis of the clinical manifestation of 21 cases of moyamoya disease and application value of double source flashCT

    Institute of Scientific and Technical Information of China (English)

    孟连英; 李锋; 杨文兵

    2015-01-01

    目的:分析烟雾病的临床表现及影像学表现,探讨双源FlashCT血管造影及其后处理技术在烟雾病诊断中的应用价值。方法对本院2013年1月~2014年7月收治的21例烟雾病患者的临床特点及影像检查资料进行回顾性分析。结果①烟雾病发病年龄在17~42岁左右,40岁以前占90豫,临床表现以缺血为主,肢体无力和偏瘫最为常见。②CT提示:脑梗死13例,脑出血6例,脑出血合并脑梗死2例。 CT血管造影及DSA示双侧或单侧颈内动脉、大脑前动脉及大脑中动脉管腔狭窄或闭塞,脑基底部异常血管网形成,如烟雾状。结论对于儿童及青年人反复出现脑缺血发作、脑出血、脑梗死,应高度怀疑烟雾病,CT血管造影可作为首选检查方法,临床怀疑病例及早行DSA检查。%Objective To analyze the clinical manifestation and imaging manifestation of moyamoya disease, and to ex-plore the application value of double source FlashCT angiography and its post-processing technique in the diagnosis of moyamoya disease. Methods Clinical characteristics and data of imaging examination of 21 patients with moyamoya disease who were admitted to our hospital from January 2013 to July 2014 were retrospectively analyzed. Results①Age of onset of moyamoya disease was around 17-42 years old, and patients younger than 40 years old accounted for 90% of the total. The clinical manifestation was mainly ischemia, and weakness of limbs and hemiplegia were the most commonly seen. ②CT indicated that 13 patients were cerebral infarction, 6 patients were cerebral bleeding, and 2 pa-tients were cerebral bleeding complicated with cerebral infarction. CT angiography and DSA showed stenosis or block-ing of bilateral or unilateral internal carotid artery, anterior cerebral artery and middle cerebral artery, and showed the formation of abnormal vessel network at the base of the brain like smoke. Conclusion For children and adults

  16. 成人Moyamoya病的临床表现和影像学特征%Clinical Manifestations and Iconographic Features in Adult Patients with Moyamoya Disease

    Institute of Scientific and Technical Information of China (English)

    张天宇; 詹青

    2007-01-01

    目的 总结成人Moyamoya病(烟雾病)的临床表现特点.比较MRA与DSA在Moyamoya病诊断中的价值.方法 对经DSA或MRA确诊的8例成人Moyamoya病患者的临床及影像学资料进行回顾性分析.结果 脑卒中是本组Moyamoya病患者最常见的临床表现,缺血性卒中与出血性卒中发病各半.MRA与DSA检查显示所有患者均有颈内动脉或其分支狭窄或闭塞及脑底异常血管网形成,两者结果相符合.结论 对年轻无基础疾病的脑血管病患者,尤其反复TIA或卒中或头痛患者,应常规行MRI和MRA检查.DSA可清晰显示颅内、外侧枝循环状况,更有助于手术适应证的选择与预后评价.

  17. CT Perfusion and MR Perfusion Weighted Imaging in Evaluating the Effect of Vascular Reconstruction on Short-term Cerebral Blood Flow in Patients with Moyamoya Disease%CT灌注与MR灌注加权成像评价烟雾病血管重建术对烟雾病患者短期脑血流的影响

    Institute of Scientific and Technical Information of China (English)

    崔若棣; 易明岗

    2015-01-01

    Objective To study CT perfusion (CTP) and MR perfusion (PWI) weighted imaging in evaluating the effect of vascular reconstruction on short-term cerebral blood flow in patients with moyamoya disease.Methods 47 patients with unilateral vascular reconstruction confirmed by DSA in the hospital were selected as the research objects and all received vascular reconstruction. All the patients received CTP and PWI examination before and after operation to obtain parameters, such as cerebral blood volume (CBV), cerebral blood flow (CBF), mean transit time (MTT) and time to peak (TTP). The preoperative and postoperative distribution of cerebral middle artery (MCA) and ipsilateral cerebellar perfusion parameters in control part (rCBF, rCBV, rMTT and rTTP) were compared as well as the differences in preoperative and postoperative relative value of CTP and PWI perfusion parameters.Results Postoperative CT perfusion image showed that CBF, CBV, MTT and TTP in the right distribution area of MCA significantly changed, compared with those before operation. Postoperative PWI perfusion image showed that CBF, CBV, MTT and TTP significantly significantly changed, compared with those before operation. After operation, rCBF and rCBV values in the distribution area of operation side MCA and the control ares were significantly higher than those before operation (P<0.05) while rMTT and rTTP were significantly lower than those before operation (P<0.05).Conclusion Both of CT perfusion and MR perfusion weighted imaging are the effective observation methods to observe the changes of cerebral blood flow in patients with moyamoya disease, which can be used in clinic.%目的:研究CT灌注(CTP)与MR灌注(PWI)加权成像评价烟雾病血管重建术对烟雾病患者短期脑血流的影响。方法选取我院47例经DSA证实的单侧血管重建术患者为研究对象,均行血管重建术治疗,术前术后接受CTP、PWI检查,获得脑血容量(CBV)、脑血流量(CBF)、平均通

  18. Moyamoya Disease – a Vasculopahty and an Uncommon Cause of Recurrent Cerebrovascular Accidents

    Directory of Open Access Journals (Sweden)

    Yasmin S Hamirani

    2008-09-01

    Full Text Available

    Moyamoya disease is a very rare chronic cerebrovascular disease of unknown etiology characterized by recurrent ischemic or hemorrhagic strokes. Initially diagnosed in Japan and named after finding puff of smoke like collateral blood vessels around the occluded blood vessels of circle of Willis. With increase awareness this disease is now diagnosed more often. Medical and surgical treatment have been used to treat the disease, with surgical treatment been mostly experimental. Special attention should be given to the surgical treatment which has shown to have an edge over the medical treatment in some clinical trials especially in young patients with recurrent strokes to prevent progressive cognitive decline and to improve their quality of life. In our patient, who is a young man, the diagnosis was picked up late and when surgical evaluation was performed, it was considered to be fruitless with findings of nonviable brain tissue on MRI imaging.

  19. Dietary intake and nutritional status in cancer patients: comparing adults and older adults

    Directory of Open Access Journals (Sweden)

    Henyse Gómez Valiente da Silva

    2014-04-01

    Full Text Available Objective: Evaluate the nutrient intake and nutritional status of food in cancer patients admitted to a university hospital, with comparison of adult and older adult age category Methods: Cross-sectional study. This study involved cancer patients admitted to a hospital in 2010. Dietary habits were collected using a Brazilian food frequency questionnaire. Participants were divided in two groups: adults or older adults and in 4-cancer category: hematologic, lung, gastrointestinal and others. Body Mass Index evaluated nutritional status. Results: A total of 86 patients with a mean age of 56.5 years, with 55% males and 42% older adults were evaluated. The older adult category had a higher frequency of being underweight (24.4% vs 16.3%, p < 0.01 and a lower frequency of being overweight (7% vs. 15.1%, p < 0.01 than adults. Both, adult and older adults had a high frequency of smoking, alcohol consumption and physical inactivity. The older adults had lower consumption of calories, intake of iron and folic acid. Inadequacy of vitamin intake was observed in both groups; respectively, 52%, 43%, 95%, 76% and 88% for Vitamin A, C, D, E and folic acid. The older adults had a higher folic acid and calcium inadequacy than the adults (97% vs 82%, p <0.01; 88% vs 72%, p < 0.01. There was no association of micronutrient intake with cancer, nor with nutritional status. Conclusion: The food intake, macro and micronutrients ingestion is insufficient among cancer individuals. Food intake of older adults was inferior, when compared to the adult category. There was a high prevalence of BMI excess in the adult group and a worst nutritional status in the older adult category.

  20. Voxel Based Analysis of Surgical Revascularization for Moyamoya Disease: Pre- and Postoperative SPECT Studies.

    Directory of Open Access Journals (Sweden)

    Yasutaka Fushimi

    Full Text Available Moyamoya disease (MMD is a chronic, progressive, cerebrovascular occlusive disease that causes abnormal enlargement of collateral pathways (moyamoya vessels in the region of the basal ganglia and thalamus. Cerebral revascularization procedures remain the preferred treatment for patients with MMD, improving the compromised cerebral blood flow (CBF. However, voxel based analysis (VBA of revascularization surgery for MMD based on data from pre- and postoperative data has not been established. The latest algorithm called as Diffeomorphic Anatomical Registration Through Exponentiated Lie Algebra (DARTEL has been introduced for VBA as the function of statistical parametric mapping (SPM8, and improved registration has been achieved by SPM8 with DARTEL. In this study, VBA was conducted to evaluate pre- and postoperative single photon emission computed tomography (SPECT images for MMD by SPM8 with DARTEL algorithm, and the results were compared with those from SPM8 without DARTEL (a conventional method. Thirty-two patients with MMD who underwent superficial temporal artery-middle cerebral artery (STA-MCA bypass surgery as the first surgery were included and all patients underwent pre- and postoperative 3D T1-weighted imaging and SPECT. Pre- and postoperative SPECT images were registered to 3D T1-weighted images, then VBA was conducted. Postoperative SPECT showed more statistically increased CBF areas in the bypassed side cerebral hemisphere by using SPM8 with DARTEL (58,989 voxels; P<0.001, and increased ratio of CBF after operation was less than 15%. Meanwhile, postoperative SPECT showed less CBF increased areas by SPM8 without DARTEL. In conclusion, VBA was conducted for patients with MMD, and SPM8 with DARTEL revealed that postoperative SPECT showed statistically significant CBF increases over a relatively large area and with at most 15% increase ratio.

  1. 应用单光子发射断层显像评价合并大脑后动脉病变的烟雾病患者脑血流灌注%Evaluation of cerebral perfusion in moyamoya diseased patients with posterior cerebral artery lesions by single photon emission tomography examination

    Institute of Scientific and Technical Information of China (English)

    王莉; 段炼; 杨伟中; 龙亚红; 暴向阳; 赵峰; 咸鹏

    2013-01-01

    目的 分析合并大脑后动脉(PCA)病变的烟雾病患者脑血流异常灌注情况.方法 共纳入27例合并PCA病变的烟雾病患者,根据DSA检查结果,判定27例患者54侧的半球的铃木分期,及54支PCA的病变程度.27例患者均接受单光子发射断层显像术(SPECT)检查,记录额叶、顶叶、颞叶、枕叶、基底核区3~4个感兴趣区的平均局部脑血流量(rCBF),用各个脑区rCBF与小脑的rCBF的比值作为各个脑区rCBF的相对值.判定PCA病变程度、不同铃木分期对各脑叶rCBF相对值的影响. 结果 ①随着PCA病变程度的加重,额叶、顶叶、颞叶、枕叶、基底核区的rCBF相对值均逐渐下降,差异具有统计学意义,均P<0.05;其中顶叶轻度PCA病变的rCBF相对值(74±6)高于中度(63±9)、重度病变(60±17) PCA的rCBF相对值,差异有统计学意义;枕叶轻度PCA病变的rCBF相对值(72±7)高于中度(65±9)、重度病变(60±15) PCA的rCBF相对值,差异有统计学意义.②不同铃木分期的额叶、顶叶、颞叶、枕叶、基底核区rCBF相对值比较,差异均无统计学意义,P>0.05. 结论 各脑叶的rCBF相对值随着PCA病变程度加重而下降,但与铃木分期的病变程度无关.脑血管后循环病变可能是影响烟雾病患者脑血流灌注水平的重要因素.%Objective To analyze the abnormal cerebral blood flow perfusion in moyamoya diseased patients with posterior cerebral artery ( PCA) lesions. Methods A total of 27 moyamoya diseased patients with posterior cerebral artery lesions were enrolled in the study. According to the digital subtract an-giography (DSA) results, the Suzuki s stages of 27 patients ( 54 hemispheres) were determined and the PCA grading in all the 27 patients were identified. All the patients accepted single photon emission tomography ( SPECT) examination, and the mean value of three or four regions of interest was calculated in five regions ( frontal, parietal, temporal, and occipital

  2. Presumed Perinatal Stroke in a Child with Down Syndrome and Moyamoya Disease

    Science.gov (United States)

    Pysden, Karen; Fallon, Penny; Moorthy, Bhagavatheswaran; Ganesan, Vijeya

    2010-01-01

    Moyamoya disease describes a cerebral arteriopathy characterized by stenosis or occlusion of the terminal internal carotid and/or the proximal middle cerebral arteries. We report a female child with trisomy 21 and bilateral moyamoya disease who presented, unusually, with a presumed perinatal cerebral infarct. The clinical, radiological, and…

  3. Moyamoya disease presenting as acute onset cortical blindness: a case report

    Directory of Open Access Journals (Sweden)

    Maniram Dudi

    2016-03-01

    Full Text Available We report a case where acute onset cortical blindness is the mode of presentation in Moyamoya disease. Cortical blindness is very rare presenting symptom of Moyamoya disease. Progressive visual loss and homonymous anopsia has been described previously, but this case had acute visual loss.

  4. Moyamoya disease presenting as acute onset cortical blindness: a case report

    OpenAIRE

    Maniram Dudi; Rajeev Bansal; Trilochan Srivastava; Sardana R.V.

    2016-01-01

    We report a case where acute onset cortical blindness is the mode of presentation in Moyamoya disease. Cortical blindness is very rare presenting symptom of Moyamoya disease. Progressive visual loss and homonymous anopsia has been described previously, but this case had acute visual loss.

  5. Moyamoya disease associated with an anterior inferior cerebellar artery arising from a persistent trigeminal artery

    Energy Technology Data Exchange (ETDEWEB)

    Uchino, A.; Sawada, A.; Takase, Y.; Kudo, S. [Department of Radiology, Saga Medical School, 5-1-1, Nabeshima, Saga, 849-8501 (Japan); Koizumi, T. [Department of Neurosurgery, Saga Medical School, 5-1-1, Nabeshima, Saga, 849-8501 (Japan)

    2002-07-01

    The authors present a case of moyamoya disease associated with a persistent trigeminal artery from which the anterior inferior cerebellar artery arose. We reviewed previously reported cases of moyamoya disease associated with persistent carotid-basilar arterial anastomosis and investigated the embryology of this rare arterial variation. (orig.)

  6. Unilateral moyamoya disease associated with cerebellar arteriovenous malformation:one case report

    Institute of Scientific and Technical Information of China (English)

    DENG Zheng-hai; WANG Shuo; LI Zhong; ZHAO Ji-zong

    2008-01-01

    @@ As a non-atherosclerotic chronic cerebral vasculopathy,moyamoya disease is characterized by progressive steno-occlusion of the arteries of the circle of Willis with a developed collateral vascular network mainly at the cerebral base.1 And it is named moyamoya disease because of a characteristic "puff-of-smoke" angiographic appearance.

  7. Treatment strategies for aneurysms associated with moyamoya disease.

    Science.gov (United States)

    Zhang, Lei; Xu, Kan; Zhang, Yandong; Wang, Xin; Yu, Jinlu

    2015-01-01

    The treatment of aneurysms associated with moyamoya disease (MMD) is difficult for neurosurgeons, and little is known of strategy options. This report constitutes a comprehensive review of the literature. We summarize the known treatments and their clinical outcomes according to the site of the aneurysm: in major arteries, peripheral arteries, moyamoya vessels, meningeal arteries, or at the site of anastomosis. The literature review indicates that the treatment of MMD-associated aneurysms varies according to the site of the aneurysm and its hemodynamic characteristics. In particular, the treatment for basilar tip aneurysms remains challenging, since both endovascular embolization and direct clipping are difficult. The potential risk for ischemia should be considered in selecting endovascular or surgical approaches. Revascularization surgery, which is important for the treatment of MMD, also determines the clinical treatment outcome of aneurysms associated with MMD.

  8. The relationship between cerebral infarction on MR and angiographic findings in moyamoya disease: significance of the posterior circulation

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Eun Ja; Song, Soon Young [College of Medicine, Kwangdong Univ., Koyang (Korea, Republic of); Yu, Won Jong; Jung, So Lyung; Chung, Bong Gak; Kag, Si Won [College of Medicine, The Catholic Univ. of Korea, Seoul (Korea, Republic of); Kim, Man Deuk [College of Medicine, Pochon CHA Univ., Pochon (Korea, Republic of)

    2002-06-01

    To investigate the relationship between changes in the posterior and anterior circulation, as seen at angiography, and the frequency and extent of cerebral infarction revealed by MR imaging in moyamoya disease. This study involved 34 patients (22 females and 12 males, aged 2-52 years) in whom cerebral angiography revealed the presence of moyamoya disease (bilateral; unilateral= 24:10; total hemispheres=58) and who also underwent brain MR imaging. To evaluate the angiographic findings, we applied each angiographic staging system to the anterior and posterior circulation. Leptomeningeal collateral circulation from the cortical branches of the posterior cerebral artery (PCA) was also assigned one of four grades. At MR imaging, areas of cerebral cortical or subcortical infarction in the hemisphere were divided into six zones. White matter and basal ganglionic infarction, ventricular dilatation, cortical atrophy, and hemorrhagic lesions were also evaluated. To demonstrate the statistical significance of the relationship between the angiographic and the MR findings, both the Mantel-Haenszel chi-square test for trend and the chi-square test were used. The degree of steno-occlusive PCA change correlated significantly with the internal carotid artery (ICA) stage (p<0.0001). As PCA stages advanced, the degree of leptomeningeal collaterals from the PCA decreased significantly (P<0.0001), but ICA stages were not significant (p>0.05). The prevalence of infarction showed significant correlation with the degree of steno-occlusive change in both the ICA and PCA. The degree of cerebral ischemia in moyamoya patients increased proportionally with the severity of PCA stenosis rather than with that of steno-occlusive lesins of the anterior circulation. Infarctions tended to be distributed in the anterior part of the hemisphere at PCA state I or II, while in more advanced PCA lesions, they were also found posteriorly, especially in the territories of the posterior middle cerebral artery

  9. Ivy sign in mildly symptomatic β-thalassemia intermedia, with development of moyamoya disease.

    Science.gov (United States)

    El Beltagi, Ahmed H; El-Sheikh, Ahmed; El-Saif, Reem; Norbash, Alexander

    2014-02-01

    Cerebrovascular occlusive disease with secondary proliferative angiogenesis can be idiopathic as a standalone disease state, known as moyamoya disease, or it may develop secondary to different disease entities, such as chronic hemoglobinopathies, in which case it is known as moyamoya syndrome. Although moyamoya syndrome has been well described with sickle cell anemia, its association with other hemoglobinopathies is rarely reported. We describe a 16-year-old girl with β-thalassemia intermedia who presented with recurrent headaches and focal seizures non-responsive to medical treatment. Magnetic resonance imaging showed diffuse intrasulcal bright signal intensity on fluid-attenuated inversion recovery and leptomeningeal enhancement previously termed the "ivy sign", and her magnetic resonance angiography was consistent with bilateral moyamoya disease. The literature describing and explaining the pathogenesis of the "ivy sign" and its relationship to moyamoya disease was reviewed.

  10. Clinical features and surgical efficacy analysis of subarachnoid hemorrhagic moyamoya disease%蛛网膜下腔出血型烟雾病的临床特征和手术疗效分析

    Institute of Scientific and Technical Information of China (English)

    万明; 韩聪; 咸鹏; 杨伟中; 李德生; 段炼

    2015-01-01

    Objective To investigate the clinical features of subarachnoid hemorrhagic moyamoya disease and the therapeutic effect of encephalo-duro-arterio-synangiosis (EDAS). Methods The clinical and imaging data of 38 patients with subarachnoid hemorrhagic moyamoya disease admitted to the Department of Neurosurgery,the 307th Hospital of PLA from January 2002 to April 2013 were analyzed retrospectively. Thirty-five patients underwent unilateral or bilateral EDAS (64-sides underwent EDAS,4 patients with aneurysms underwent endovascular embolization first),and 3 patients did not undergo any surgery. Results (1)Subarachnoid hemorrhagic moyamoya disease accounted for 10. 8%(38/353)of all the hemorrhagic moyamoya disease admitted in hospital over the same period,including 37 adults and 1 child. The male to female ratio was 1∶3. 22 (9/29),and the age of onset was 12 to 59 years. The mean age of patients was 39 ± 11 years. Four patients were combined with aneurysms. There were no significant differences in the distribution of Suzuki stage,anterior choroidal artery dilatation and posterior communicating artery dilatation in the remaining 34 patients without aneurysms between the bleeding sides and non-bleeding sides (P>0.05). (2 ) The patients were followed up for 13 -125 months (mean 51 ± 27 months ),two patients had rebleeding,one of them was intraventricular hemorrhage,the other was parenchymal hemorrhage. The postoperative modified Rankin score (mRS)was significantly lower in 35 patients whom were treated with EDAS. Compared with before surgery,there was significant difference (P<0. 05). The re-examination of positron emission tomography (PET)for 16 patients at 3 to 19 months after surgery showed that among the 23 surgically treated hemispheres,the cerebral metabolisms of 17 hemispheres were improved after surgery, and 6 did not have any change after surgery. The re-examination of whole brain digital subtraction angiography (DSA)at 5 to 30 months after surgery in 13

  11. Multiple prograssive intracranial arterial occlusions or Moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Prevo, R.L.; Straalen, A.M. van (Medisch Spectrum Twente, Enschede (Netherlands). Department of Radiology); Geelen, J.A.G. (Medisch Spectrum Twente, Enschede (Netherlands). Department of Neurology)

    A case history is presented of a 21-year-old white female with a clinical diagnosis of an intracranial hemorrhage. CT scanning revealed a hemorrhage in the parenchyma just above the lateral ventricle. Despite her excellent neurological status angiographic findings were severe and compatible with Moyamoya desease. On angiography, leptomeningeal anastomoses and rete mirabile anastomoses were present. The vertebrobasilary system was involved as well. (Author). 23 refs.; 5 figs.

  12. The effectiveness of O2 administration for transient ischemic attacks in moyamoya disease in children.

    Science.gov (United States)

    Fujiwara, J; Nakahara, S; Enomoto, T; Nakata, Y; Takita, H

    1996-02-01

    Moyamoya disease is a cerebrovascular obstructive disease of unknown etiology. The rebuild-up phenomenon, slowing of waves on electroencephalogram (EEG) seen after cessation of hyperventilation (HV), is one of the characteristic phenomena of the disease and is thought to be related to a development of its symptoms. Therefore, we investigated the mechanism involved in the rebuild-up phenomenon to clarify the mechanism of development of transient ischemic attack (TIA) in moyamoya disease. Ten patients with moyamoya disease were studied; they ranged in age from 7 to 17 years. The power spectra of the EEGs in the occipital region were obtained with a Berg Fourier EEG analyzer for quantitative analysis. Arterial blood gas change (pH, PaO2, PaCO2), respiratory pattern (abdominal and nasal), tidal volume and respiratory rate were analyzed simultaneously every 30 s-1 min before, during, and after HV. The slow wave power spectrum (rebuild-up) increased and symptoms of TIA developed as a result of the sharp decrease in PaO2 (PaO2 60.5 +/- 15.4 mmHg) after cessation of HV. Based on the fact that hypoxemia was playing a main role, 100% oxygen was administered at a rate of 0.5 l/min in 4 cases where the rebuild-up phenomenon was clear. The EEG power spectra and arterial blood gas were analyzed during rebuild-up with and without O2 administration. The effectiveness of O2 administration at the beginning of rebuild-up as measure to prevent the symptoms was checked by a recovery rate of slow wave power percentage, a recovery time of slow wave power percent and by clinical observation. The recovery rates were 11.8 +/- 4.2%/min and 5.5 +/- 4.0%/min with and without O2 inhalation, respectively (P < 0.001). Recovery times of slow wave power percentage were 4.3 +/- 1.8 min and 8.1 +/- 1.2 min with and without O2 inhalation, respectively (P < 0.01). Thus, oxygen administration soon after the cessation of HV was shown to be effective in eliminating the rebuild-up phenomenon and hence in

  13. Noninvasive method for mapping CVR in moyamoya disease using ASL-MRI

    Energy Technology Data Exchange (ETDEWEB)

    Noguchi, Tomoyuki, E-mail: tnogucci@radiol.med.kyushu-u.ac.jp [Department of Radiology, National center for Global Health and Medicine, 1-21-1, Toyama, Shinjuku-ku, Tokyo 162-8655 (Japan); Department of Radiology, Faculty of Medicine and Graduate School of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Kawashima, Masatou, E-mail: m996kawa@cc.saga-u.ac.jp [Department of Neurosugery, Faculty of Medicine and Graduate School of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Nishihara, Masashi, E-mail: nishiham@cc.saga-u.ac.jp [Department of Radiology, Faculty of Medicine and Graduate School of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Egashira, Yoshiaki, E-mail: bunta1974@yahoo.co.jp [Department of Radiology, Faculty of Medicine and Graduate School of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Azama, Shinya, E-mail: azama-ssam-71257@b2.bunbun.ne.jp [Department of Radiology, Faculty of Medicine and Graduate School of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Irie, Hiroyuki, E-mail: irie@cc.saga-u.ac.jp [Department of Radiology, Faculty of Medicine and Graduate School of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan)

    2015-06-15

    Highlights: • A clinical use of a pulse-wave-synchronized ASL-MRI (pulsy ASL-MRI) was proposed. • Resting IMP-SPECT correlated with pulsy ASL-MRI in moyamoya disease. • ASL-MRI could measure cerebrovascular reserve noninvasively in moyamoya disease. - Abstract: Purpose: To project a noninvasive method for mapping cerebrovascular reserve (CVR) in moyamoya disease (MMD) using ASL-MRI. Methods: 16 MMD patients underwent cerebral blood flow (CBF) examinations by standard ASL-MRI, pulse-wave-synchronized ASL-MRI (pulsy ASL-MRI) which tagged the arterial blood coincident with a peak of a pulse wave, and single photon emission computed tomography (SPECT) imagings with iodine-123-N-isopropyl-p-iodoamphetamine in the resting (rest-IMP) and after acetazolamide challenge (ACZ-IMP). Hemispheric 32-sided cerebral blood flow (CBF) values were measured with normalized CBF maps created from standard ASL-MRI (standard-ASL value), pulsy ASL-MRI (pulsy-ASL value), rest-IMP (rest-IMP value), and ACZ-IMP (ACZ-IMP value). CVR based on rest-IMP and ACZ-IMP values (IMP-CVR) was calculated. ASL-CVR was also calculated on the basis of corrected standard-ASL values and pulsy-ASL values, which were adjusted to the ACZ-IMP values and rest-IMP values, respectively, by the least-squares method. We assessed the relationships between rest-IMP values and pulsy-ASL values, ACZ-IMP values and standard-ASL values, and IMP-CVR and ASL-CVR. Results: Significant relationships were observed between rest-IMP values and pulsy-ASL values (correlation coefficient (r = 0.557, p < 0.01)), ACZ-IMP values and standard-ASL values (r = 0.825, p < 0.01), and IMP-CVR and ASL-CVR (r = 0.736, p < 0.01). Conclusions: ASL-MRI is equivalent to SPECT and that it might serve as a noninvasive method for mapping CVR in MMD.

  14. Approximate Quantification in Young, Healthy Older Adults', and Alzheimer Patients

    Science.gov (United States)

    Gandini, Delphine; Lemaire, Patrick; Michel, Bernard Francois

    2009-01-01

    Forty young adults, 40 healthy older adults, and 39 probable AD patients were asked to estimate small (e.g., 25) and large (e.g., 60) collections of dots in a choice condition and in two no-choice conditions. Participants could choose between benchmark and anchoring strategies on each collection of dots in the choice condition and were required to…

  15. Physical and psychosocial challenges in adult hemophilia patients with inhibitors

    Directory of Open Access Journals (Sweden)

    duTreil S

    2014-07-01

    Full Text Available Sue duTreil Louisiana Center for Bleeding and Clotting Disorders, Tulane University Health Sciences Center, New Orleans, LA, USA Abstract: Numerous challenges confront adult hemophilia patients with inhibitors, including difficulty in controlling bleeding episodes, deterioration of joints, arthritic pain, physical disability, emotional turmoil, and social issues. High-intensity treatment regimens often used in the treatment of patients with inhibitors also impose significant scheduling, economic, and emotional demands on patients and their families or primary caregivers. A comprehensive multidisciplinary assessment of the physical, emotional, and social status of adult hemophilia patients with inhibitors is essential for the development of treatment strategies that can be individualized to address the complex needs of these patients. Keywords: adult hemophilia patients with inhibitors, adherence, physical challenges, psychosocial challenges, health-related quality of life

  16. Superficial temporal artery-middle cerebral artery bypass combined with encephalo-duro-myo-synangiosis in treating moyamoya disease: surgical techniques, indications and midterm follow-up results

    Institute of Scientific and Technical Information of China (English)

    XU Bin; SONG Dong-lei; MAO Ying; GU Yu-xiang; XU Hong; LIAO Yu-jun; LIU Chuang-hong; ZHOU Liang-fu

    2012-01-01

    Background Surgical interventions for moyamoya disease include direct and indirect revascularizations.This study aimed to evaluate the therapeutic effect of superficial temporal artery-middle cerebral artery bypass combined with an indirect revascularization procedure,encephalo-duro-myo-synangiosis,in the treatment of moyamoya disease.Methods From October 2005 to November 2009,we performed this combined revascularization procedure in 111 patients with different types and stages of moyamoya disease.The superficial temporal artery,middle meningeal artery and the deep temporal artery were evaluated for individualized surgical planning in these cases.The integrity of the deep temporal artery and the middle meningeal artery network,and the pre-existing spontaneous anastomoses of the distal branches of the external carotid artery with the cortical arteries were well preserved.The mean follow-up time was 72.5 months,all clinical and radiological data were retrospectively reviewed.Results A total of 198 stomas were performed in 122 hemispheres,all remaining patent until the last follow-up.The encephalo-duro-myo-synangiosis resulted in extensive anastomoses of the deep temporal artery (100%),the middle meningeal artery (90.9%),and the sphenopalatine artery (39.8%) with the cortical arteries,respectitvely.The superficial temporal artery,deep temporal artery,and the middle meningeal artery were significantly thickened in 88 patients as determined by digital subtraction angiography at follow-up.The relative cerebral blood flow increased significantly within one week after the operation.At 6 months post the operation,the relative cerebral blood flow was further increased by 15.5% from the gradual formation of anastomoses as a result of indirect revascularization.Transient ischemic attacks were effectively reduced or totally arrested.The neurological deficits significantly improved in 37 patients,with the National Institutes of Health Stroke Scale scores lowered by 2

  17. CO2BOLD assessment of moyamoya syndrome: Validation with single photon emission computed tomography and positron emission tomography imaging

    Science.gov (United States)

    Pellaton, Alain; Bijlenga, Philippe; Bouchez, Laurie; Cuvinciuc, Victor; Barnaure, Isabelle; Garibotto, Valentina; Lövblad, Karl-Olof; Haller, Sven

    2016-01-01

    AIM To compare the assessment of cerebrovascular reserve (CVR) using CO2BOLD magnetic resonance imaging (MRI) vs positron emission tomography (PET) and single photon emission computed tomography (SPECT) as reference standard. METHODS Ten consecutive patients (8 women, mean age of 41 ± 26 years) with moyamoya syndrome underwent 14 pre-surgical evaluations for external-internal carotid artery bypass surgery. CVR was assessed using CO2BOLD and PET (4)/SPECT (11) with a maximum interval of 36 d, and evaluated by two experienced neuroradiologists. RESULTS The inter-rater agreement was 0.81 for SPECT (excellent), 0.43 for PET (fair) and 0.7 for CO2BOLD (good). In 9/14 cases, there was a correspondence between CO2BOLD and PET/SPECT. In 4/14 cases, CVR was over-estimated in CO2BOLD, while in 1/14 case, CVR was underestimated in CO2BOLD. The sensitivity of CO2BOLD was 86% and a specificity of 43%. CONCLUSION CO2BOLD can be used for pre-surgical assessment of CVR in patients with moyamoya syndrome and combines the advantages of absent irradiation, high availability of MRI and assessment of brain parenchyma, cerebral vessels and surrogate CVR in one stop. PMID:27928470

  18. The metabolic consequences of thyroxine replacement in adult hypopituitary patients

    DEFF Research Database (Denmark)

    Filipsson Nyström, Helena; Feldt-Rasmussen, Ulla; Kourides, Ione;

    2012-01-01

    The metabolic consequences of thyroxine replacement in patients with central hypothyroidism (CH) need to be evaluated. The aim was to examine the outcome of thyroxine replacement in CH. Adult hypopituitary patients (n = 1595) with and without CH from KIMS (Pfizer International Metabolic Database...

  19. Moyamoya disease in a child with previous acute necrotizing encephalopathy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Taik-Kun; Cha, Sang Hoon; Chung, Kyoo Byung; Kim, Jung Hyuck; Kim, Baek Hyun; Chung, Hwan Hoon [Department of Diagnostic Radiology, Korea University College of Medicine, Ansan Hospital, 516 Kojan-Dong, Ansan City, Kyungki-Do 425-020 (Korea); Eun, Baik-Lin [Department of Pediatrics, Korea University College of Medicine, Seoul (Korea)

    2003-09-01

    A previously healthy 24-day-old boy presented with a 2-day history of fever and had a convulsion on the day of admission. MRI showed abnormal signal in the thalami, caudate nuclei and central white matter. Acute necrotising encephalopathy was diagnosed, other causes having been excluded after biochemical and haematological analysis of blood, urine and CSF. He recovered, but with spastic quadriparesis. At the age of 28 months, he suffered sudden deterioration of consciousness and motor weakness of his right limbs. MRI was consistent with an acute cerebrovascular accident. Angiography showed bilateral middle cerebral artery stenosis or frank occlusion with numerous lenticulostriate collateral vessels consistent with moyamoya disease. (orig.)

  20. Moyamoya disease in children%儿童烟雾病

    Institute of Scientific and Technical Information of China (English)

    王华

    2008-01-01

    烟雾病(moyamoya disease,MMD)又称为脑底异常血管网病,最早由13本学者Suzuki等提出,moyamoya一词为13文“烟雾”之意。MMD是以颈内动脉虹吸部及大脑前、中动脉起始部进行性狭窄或闭塞,以及颅底软脑膜穿通动脉形成细小密集的吻合血管网为特征的脑血管疾病。

  1. Co-morbidity in adult haemophilia patients

    NARCIS (Netherlands)

    Fransen van de Putte, D.E.

    2012-01-01

    Haemophilia is an X-linked inherited bleeding disorder, caused by a deficiency of clotting factor VIII or IX. Due to the availability of treatment with clotting factor concentrates, life expectancy of haemophilia patients is now approaching that of the general population. Haemophilia patients are th

  2. Crisis homes for adult psychiatric patients

    DEFF Research Database (Denmark)

    Aagaard, Jørgen; Freiesleben, Michael; Foldager, Leslie

    2008-01-01

    INTRODUCTION: Inspired by the Crisis Home programme in Madison, we have adapted and evaluated the programme at the Community Mental Health (CMH) Centre in Tønder, Denmark. MATERIAL AND METHODS: Procedures and schedules from the Crisis Home programme were applied in this open trial. Questionnaire...... data concerning satisfaction with the stay and registration data concerning the admissions and bed days two years before and two years after the first stay were obtained. RESULTS: During four years, 52 different patients had a total of 187 stays in a crisis home. Twenty (38.5%) of the patients were...... attached to the ACT team. The average duration of the stays was 4.0 days. The number of readmissions and bed days after the first stay showed a significant downward tendency for the subgroup of patients with a more severe mental disorder, but not for the whole group. The patients, the crisis homes families...

  3. Doença cerebrovascular tipo "moyamoya" Cerebrovascular "moyamoya" disease: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Reynaldo A. Brandt

    1976-09-01

    Full Text Available É relatado o caso de um paciente masculino, branco, 34 anos, que apresentou hemorragia meníngea sem sinais de comprometimento cerebral. A carotidoangiografia direita mostrou as alterações típicas do "moyamoya", com estenose da artéria carótida interna ao nível do sifão, acima da origem da artéria comunicante posterior; há uma rede vascular angiomatosa anormal projetando-se na base do cérebro, da qual nascem as artérias cerebrais anterior e média, sendo as artérias comunicante e cerebral posterior hipertrofiadas e de trajeto tortuoso; há anastomoses meningo-corticiais, sendo as artérias meníngeas hipertrofiadas. A carotidoangiografia esquerda mostrou hipertrofia das artérias carótidas comum e interna, com contrastação de ambas as artérias cerebrais anteriores e médias a partir deste lado; a artéria comunicante anterior é do tipo fetal e há a presença anômala da artéria cerebral anterior mediana do corpo caloso. Estes dados apoiam a teoria segundo a qual a doença constitui anomalia do desenvolvimento vascular encefálico, não tendo sido encontrado outro caso na literatura com hipertrofia vicariante da artéria carótida interna contralateral à da lesão típica do "moyamoya".The case of a 34-year-old Caucasian male with subarachnoid hemorrahge is reported. The right carotid arteriogram shows the typical internal carotid artery occlusion at the syphon, with an abnormal vascular network at the base of the brain; the right anterior and middle cerebral arteries fill from the vascular network and through meningocortical anastomoses; the right posterior communicating and cerebral arteries are tortuous and hypertrophic. The left carotid arteriogram shows hypertrophy of the common and internal carotid arteries; both anterior and middle cerebral arteries fill from this side; the anterior communicating artery is thick (foetal type and a median anterior cerebral artery to the corpus callosum is present. It is the authors

  4. Acne in the adult female patient: a practical approach.

    Science.gov (United States)

    Kamangar, Faranak; Shinkai, Kanade

    2012-10-01

    Acne vulgaris is a common reason why adult women present to dermatologists and can be a clinical challenge to treat. It may also be an important sign of an underlying endocrine disease such as Polycystic Ovary Syndrome (PCOS). Although standard acne therapies can be successfully used to treat acne in adult female patients, hormonal treatment is a safe and effective therapeutic option that may provide an opportunity to better target acne in this population, even when other systemic therapies have failed. In this article, a practical approach to the adult female patient with acne will be reviewed to enhance the dermatologist's ability to use hormonal acne therapies and to better identify and evaluate patients with acne in the setting of a possible endocrine disorder.

  5. Hematological abnormalities in adult patients with Down's syndrome.

    LENUS (Irish Health Repository)

    McLean, S

    2012-02-01

    BACKGROUND: There is a paucity of data regarding hematological abnormalities in adults with Down\\'s syndrome (DS). AIMS: We aimed to characterize hematological abnormalities in adult patients with DS and determine their long-term significance. METHODS: We retrospectively studied a cohort of nine DS patients referred to the adult hematology service in our institution between May 2001 and April 2008. Data collected were: full blood count (FBC), comorbidities, investigations performed, duration of follow-up and outcome to most recent follow-up. RESULTS: Median follow-up was 26 months (9-71). Of the nine patients, two had myelodysplastic syndrome (MDS) at presentation. Of these, one progressed, with increasing marrow failure, and requiring support with transfusions and gCSF. The remaining eight patients, with a variety of hematological abnormalities including leukopenia, macrocytosis, and thrombocytopenia, had persistently abnormal FBCs. However there was no evidence of progression, and no patient has evolved to acute myeloid leukemia (AML). CONCLUSIONS: MDS is a complication of DS and may require supportive therapy. However, minor hematological abnormalities are common in adult DS patients, and may not signify underlying marrow disease.

  6. Lifetime Increased Risk of Adult Onset Atopic Dermatitis in Adolescent and Adult Patients with Food Allergy

    Directory of Open Access Journals (Sweden)

    Hsu-Sheng Yu

    2016-12-01

    Full Text Available Food allergy can result in life-threatening anaphylaxis. Atopic dermatitis (AD causes intense itching and impaired quality of life. Previous studies have shown that patients with classical early-onset AD tend to develop food allergy and that 10% of adults with food allergies have concomitant AD. However, it is not known whether late-onset food allergy leads to adult-onset AD, a recently recognized disease entity. Using an initial cohort of one-million subjects, this study retrospectively followed-up 2851 patients with food allergy (age > 12 years for 14 years and compared them with 11,404 matched controls. While 2.8% (81 of the 2851 food allergy patients developed AD, only 2.0% (227 of the 11,404 controls developed AD. Multivariate regression analysis showed that food allergy patients were more likely to develop AD (adjusted hazard ratio = 2.49, p < 0.0001. Controls had a 1.99% risk of developing AD, while food allergy patients had a significantly higher risk (7.18% and 3.46% for patients with ≥3 and <3 food allergy claims, respectively of developing adult-onset AD. This is the first study to describe the chronological and dose-dependent associations between food allergy in adolescence and the development of adult-onset AD.

  7. Pyridoxine deficiency in adult patients with status epilepticus.

    Science.gov (United States)

    Dave, Hina N; Eugene Ramsay, Richard; Khan, Fawad; Sabharwal, Vivek; Irland, Megan

    2015-11-01

    An 8-year-old girl treated at our facility for superrefractory status epilepticus was found to have a low pyridoxine level at 5 μg/L. After starting pyridoxine supplementation, improvement in the EEG for a 24-hour period was seen. We decided to look at the pyridoxine levels in adult patients admitted with status epilepticus. We reviewed the records on patients admitted to the neurological ICU for status epilepticus (SE). Eighty-one adult patients were identified with documented pyridoxine levels. For comparison purposes, we looked at pyridoxine levels in outpatients with epilepsy (n=132). Reported normal pyridoxine range is >10 ng/mL. All but six patients admitted for SE had low normal or undetectable pyridoxine levels. A selective pyridoxine deficiency was seen in 94% of patients with status epilepticus (compared to 39.4% in the outpatients) which leads us to believe that there is a relationship between status epilepticus and pyridoxine levels.

  8. Body Composition in Adult Patients with Thalassemia Major

    Directory of Open Access Journals (Sweden)

    Marianna Vlychou

    2016-01-01

    Full Text Available Objective. To assess body composition in adult male and female patients with thalassemia major by dual-energy X-ray absorptiometry (DXA and to compare the findings with a group of healthy age-matched controls. Methods. Our study group included sixty-two patients (27 males, mean age 36 years, and 35 females, mean age 36.4 years and fifteen age-matched healthy controls. All patients had an established diagnosis of thalassemia major and followed a regular blood transfusion scheme since childhood and chelation treatment. Fat, lean, and bone mineral density (BMD were assessed with dual-energy X-ray absorptiometry. Ferritin levels and body mass index of all patients and controls were also recorded. Student t-test and Wilcoxon test were performed and statistical significance was set at p<0.05. Results. BMD and whole body lean mass are lower in both male and female adult patients compared with controls (p<0.01 in both groups, whereas whole body fat mass was found to have no statistically significant difference compared to controls. Regional trunk fat around the abdomen was found to be lower in male patients compared to controls (p=0.02. Conclusion. Severe bone loss and diminished lean mass are expected in adult male and female patients with thalassemia major. Fat changes seem to affect mainly male patients.

  9. Case Report: A case report of Moyamoya disease in a 36 year old African American woman [v1; ref status: indexed, http://f1000r.es/4tx

    Directory of Open Access Journals (Sweden)

    Rohit Kumar Gudepu

    2014-12-01

    Full Text Available Moyamoya is a rare idiopathic progressive vaso-occlusive disease characterized by irreversible condition of main blood vessels to the brain as they enter into the skull. We present a case of 36 year old African American female presenting to the Out Patient Clinic with headache which were on and off for 4-6 months and did not relieve on routine medical therapy. It was associated with weakness on right side for last few days. The patient was investigated with CT Angiogram, diagnosed as Moyamoya disease and operated. She has been followed up for the last 5 years and the patient has not complained of any headaches or focal neurological symptoms.

  10. Doença de Moyamoya e anestesia com sevoflurano fora do centro cirúrgico: relato de caso Enfermedad de Moyamoya y anestesia con sevoflurano fuera del centro quirúrgico: relato de caso Moyamoya disease and sevoflurane anesthesia outside the surgery center: case report

    Directory of Open Access Journals (Sweden)

    Sheila Braga Machado

    2002-06-01

    causa de accidente vascular cerebral en individuos jóvenes. El objetivo de este relato es presentar el caso de un paciente con enfermedad de Moyamoya que fue sometido a anestesia general con sevoflurano para procedimiento diagnóstico fuera del centro quirúrgico. RELATO DEL CASO: Niño con 13 años, estado físico ASA IV, portador de enfermedad de Moyamoya con secuela neurológica después de tres accidentes vasculares cerebrales, insuficiencia renal crónica e hipertensión arterial sistémica, sometido a endoscopia digestiva alta. En decúbito dorsal y después monitorización, se realizó inducción inalatoria por la cánula de traqueostomía con sevoflurano (aumento gradual de la concentración inspiratoria hasta 6% y mezcla de oxígeno/óxido nitroso a 50%. Un catéter venoso fue introducido para infusión de solución glucosada a 5%. Fue realizada ventilación controlada manual, siendo la manutención de la anestesia hecha con sevoflurano a 4% y mezcla de oxígeno/óxido nitroso a 50%. Al final del procedimiento los agentes anestésicos fueron descontinuados simultáneamente y fue administrado oxígeno a 100%. La anestesia fue satisfactoria, con buena estabilidad hemodinámica, sin ocurrencia de complicaciones durante el procedimiento y con un precoz despertar. CONCLUSIONES: El sevoflurano puede ofrecer nuevas perspectivas para la anestesia inhalatoria en pacientes con enfermedad neurológica que realizan procedimiento ambulatorial, ya que permite buena estabilidad hemodinamica y despertar precoz, preservando la fisiología cerebral.BACKGROUND AND OBJECTIVES: Moyamoya disease is a progressive cerebrovascular disorder implying anesthetic challenges due to patients’ poor brain perfusion, in addition to being a major cause for stroke in young people. This report aimed at describing a case of Moyamoya’s disease in a patient submitted to general anesthesia with sevoflurane for a diagnostic procedure outside the surgery center. CASE REPORT: Male child, 13 years

  11. Movement disorders in adult patients with classical galactosemia.

    Science.gov (United States)

    Rubio-Agusti, Ignacio; Carecchio, Miryam; Bhatia, Kailash P; Kojovic, Maja; Parees, Isabel; Chandrashekar, Hoskote S; Footitt, Emma J; Burke, Derek; Edwards, Mark J; Lachmann, Robin H L; Murphy, Elaine

    2013-06-01

    Classical galactosemia is an autosomal recessive inborn error of metabolism leading to toxic accumulation of galactose and derived metabolites. It presents with acute systemic complications in the newborn. Galactose restriction resolves these symptoms, but long-term complications, such as premature ovarian failure and neurological problems including motor dysfunction, may occur despite adequate treatment. The objective of the current study was to determine the frequency and phenotype of motor problems in adult patients with classical galactosemia. In this cross-sectional study, adult patients with a biochemically confirmed diagnosis of galactosemia attending our clinic were assessed with an interview and neurological examination and their notes retrospectively reviewed. Patients were classified according to the presence/absence of motor dysfunction on examination. Patients with motor dysfunction were further categorized according to the presence/absence of reported motor symptoms. Forty-seven patients were included. Thirty-one patients showed evidence of motor dysfunction including: tremor (23 patients), dystonia (23 patients), cerebellar signs (6 patients), and pyramidal signs (4 patients). Tremor and dystonia were often combined (16 patients). Thirteen patients reported motor symptoms, with 8 describing progressive worsening. Symptomatic treatment was effective in 4 of 5 patients. Nonmotor neurological features (cognitive, psychiatric, and speech disorders) and premature ovarian failure were more frequent in patients with motor dysfunction. Motor dysfunction is a common complication of classical galactosemia, with tremor and dystonia the most frequent findings. Up to one third of patients report motor symptoms and may benefit from appropriate treatment. Progressive worsening is not uncommon and may suggest ongoing brain damage in a subset of patients.

  12. Milrinone for cardiac dysfunction in critically ill adult patients

    DEFF Research Database (Denmark)

    Koster, Geert; Bekema, Hanneke J; Wetterslev, Jørn;

    2016-01-01

    INTRODUCTION: Milrinone is an inotrope widely used for treatment of cardiac failure. Because previous meta-analyses had methodological flaws, we decided to conduct a systematic review of the effect of milrinone in critically ill adult patients with cardiac dysfunction. METHODS: This systematic...... review was performed according to The Cochrane Handbook for Systematic Reviews of Interventions. Searches were conducted until November 2015. Patients with cardiac dysfunction were included. The primary outcome was serious adverse events (SAE) including mortality at maximum follow-up. The risk of bias...... analyses displayed statistical and/or clinical heterogeneity of patients, interventions, comparators, outcomes, and/or settings and all featured missing data. DISCUSSION: The current evidence on the use of milrinone in critically ill adult patients with cardiac dysfunction suffers from considerable risks...

  13. Ocular disorders in adult leukemia patients in Nigeria

    Directory of Open Access Journals (Sweden)

    Omoti Afekhide

    2010-01-01

    Full Text Available Context: Leukemias may present with, or be associated with ocular disorders. Aims: To determine the rates of ophthalmic disorders in adult patients with leukemia. Settings and Design: A prospective study of ocular disorders in adult patients with leukemia at the University of Benin Teaching Hospital, Benin City, Nigeria, between July 2004 and June 2008 was conducted. Methods and Materials: The patients were interviewed and examined by the authors and the ocular findings were recorded. Statistical analysis was performed using Instat GraphPad™ v2.05a statistical package software. The means, standard deviation, and the Kruskal-Wallis non parametric test were performed. Results: Forty-seven patients with leukemias were seen. Nineteen patients (40.4% had CLL, 14(29.8% had CML, 9(19.1% had AML and 5(10.6% had ALL. Seven patients (14.9% had ocular disorders due to leukemia. The ocular disorders due to the leukemia were proptosis in two patients (4.3%, retinopathy in one patient (2.1%, conjunctival infiltration in one patient (2.1%, periorbital edema in one patient (2.1%, retinal detachment in one patient (2.1%, and subconjunctival hemorrhage in one patient (2.1%. There was no significant difference in rate of the ocular disorders in the various types of leukemia (Kruskal-Wallis KW= 4.019; corrected for ties. P=0.2595. One patient (2.1% was blind from bilateral exudative retinal detachment while 1 patient (2.1% had monocular blindness from mature cataract. Conclusions: Ophthalmic disorders that are potentially blinding occur in leukemias. Ophthalmic evaluation is needed in these patients for early identification and treatment of blinding conditions.

  14. Home Parenteral Nutrition in Adult Patients With Chronic Intestinal Failure

    DEFF Research Database (Denmark)

    Brandt, Christopher Filtenborg; Tribler, Siri; Hvistendahl, Mark

    2017-01-01

    BACKGROUND/AIMS: Catheter-related complications (CRCs) cause mortality and morbidity in patients dependent on parenteral support at home (HPN) due to intestinal failure (IF). This study describes the incidences of CRCs in an adult IF cohort over 40 years. It illustrates the evolution and conseque...

  15. Focal epilepsies in adult patients attending two epilepsy centers

    DEFF Research Database (Denmark)

    Gilioli, Isabella; Vignoli, Aglaia; Visani, Elisa

    2012-01-01

    PURPOSE: To classify the grade of antiepileptic drug (AED) resistance in a cohort of patients with focal epilepsies, to recognize the risk factors for AED resistance, and to estimate the helpfulness of "new-generation" AEDs. METHODS: We included 1,155 adults with focal epilepsies who were observe...

  16. Prevalence of Peptostreptococcus micros morphotypes in patients with adult periodontitis

    NARCIS (Netherlands)

    van Dalen, PJ; van Winkelhoff, AJ; van Steenbergen, TJM

    1998-01-01

    The prevalence of the smooth and rough colonial morphotypes of Peptostreptococcus micros was examined with culture technique in 123 patients with adult periodontitis (age 24-68 years). Of all subgingival samples, 91% contained the smooth morphotype of P. micros. The smooth morphotype constituted a m

  17. Patient-specific FDG dosimetry for adult males, adult females, and very low birth weight infants

    Science.gov (United States)

    Niven, Erin

    Fluorodeoxyglucose is the most commonly used radiopharmaceutical in Positron Emission Tomography, with applications in neurology, cardiology, and oncology. Despite its routine use worldwide, the radiation absorbed dose estimates from FDG have been based primarily on data obtained from two dogs studied in 1977 and 11 adults (most likely males) studied in 1982. In addition, the dose estimates calculated for FDG have been centered on the adult male, with little or no mention of variations in the dose estimates due to sex, age, height, weight, nationality, diet, or pathological condition. Through an extensive investigation into the Medical Internal Radiation Dose schema for calculating absorbed doses, I have developed a simple patient-specific equation; this equation incorporates the parameters necessary for alterations to the mathematical values of the human model to produce an estimate more representative of the individual under consideration. I have used this method to determine the range of absorbed doses to FDG from the collection of a large quantity of biological data obtained in adult males, adult females, and very low birth weight infants. Therefore, a more accurate quantification of the dose to humans from FDG has been completed. My results show that per unit administered activity, the absorbed dose from FDG is higher for infants compared to adults, and the dose for adult women is higher than for adult men. Given an injected activity of approximately 3.7 MBq kg-1, the doses for adult men, adult women, and full-term newborns would be on the order of 5.5, 7.1, and 2.8 mSv, respectively. These absorbed doses are comparable to the doses received from other nuclear medicine procedures.

  18. Comparison of effects of transcranial Doppler sonography and transcranial color Doppler ultrasonography on diagnosis of Moyamoya disease with that of DSA%经颅多普勒及彩色经颅多普勒超声与DSA对烟雾病诊断的比较分析

    Institute of Scientific and Technical Information of China (English)

    张小征; 莫雪红; 华莎; 杨铭; 陈刚; 马廉亭

    2011-01-01

    Objectives To summarize the experiences in diagnosing Moyamoya disease by transcranial color Doppler ultrasonography (TCCD) and to evaluate the clinical value of TCCD to noninvasive diagnosis of Moyamoya disease. Methods Of 137 patients with Moyamoa disease diagnosed as by DSA, 24 received transcranial Doppler (TCD) examination and 113 did TCCD. The outcomes of TCD and TCCD examination were compared to that DSA which was performed in all the patients. Results The diagnosis of Moyamoya disease was definitely made by TCD in 22, Moyamoya disease was highly suspected in 1 and Moyamoya disease was not detected in 1 of the 24 patients with Moyamoya disease receiving TCD. The positive detected rate of Moyamoya disease by TCD was 95.8%. The diagnosis of Moyamoya disease was definitely made by TCCD in 102, Moyamoya disease was highly suspected in 8 and TCCD examination failed of success in 3 because there were not bone windows through which the sound passed. Conclusions The vessels in the brain bases and changes in their hemodynamics can dynamically be observed in real time by TCD and TCCD. TCCD is more helpful to estimating the occlusion and light stenosis of the intracranial vessels and it is of higher value to diagnosing Moyamoya disease than TCD because it is more sensitive to the blood flow in the stenosed vessels. TCD and TCCD, which are noninvasive methods, should be firstly selected for diagnosing and following up in the patients with Moyamoya disease.%目的 探讨经颅多普勒(TCD)及彩色经颅多普勒超声(TCCD)对烟雾病诊断的临床应用价值.方法 采用TCD及TCCD探测137例烟雾病患者颅内血管血流动力学的改变,并与其DSA结果比较分析.结果 TCD探测24例,明确诊断22例,高度怀疑1例,阴性者1例,与DSA比较诊断符合率为96%.TCCD探测113例,明确诊断102例,高度怀疑8例,因无透声窗检测失败3例,与DSA比较诊断符合率为97%.结论 TCD及TCCD均能实时动态地观察颅底血管血流动力

  19. Amelogenesis imperfecta - lifelong management. Restorative management of the adult patient.

    Science.gov (United States)

    Patel, M; McDonnell, S T; Iram, S; Chan, M F W-Y

    2013-11-08

    The biggest challenge restorative dentists face in rehabilitating patients with amelogenesis imperfecta (AI) is trying to restore aesthetics, function and occlusal stability while keeping the treatment as conservative as possible. The goals of treatment should be to prolong the life of the patient's own teeth and avoid or delay the need for extractions and subsequent replacement with conventional fixed, removable or implant retained prostheses. In order to achieve these goals a stepwise approach to treatment planning is required starting with the most conservative but aesthetically acceptable treatment. This article discusses the management of AI and presents the various treatment options available for restoring the adult patient who presents to the dentist with AI.

  20. Optimal serum phenylalanine for adult patients with phenylketonuria.

    Science.gov (United States)

    Okano, Yoshiyuki; Nagasaka, Hironori

    2013-12-01

    High serum phenylalanine in adult patients with phenylketonuria (PKU) causes neuropsychological and psychosocial problems that can be resolved by phenylalanine-restricted diet. Therefore, PKU patients must continue to adhere to phenylalanine-restricted diet for life, although the optimal serum phenylalanine level in later life has yet to be established. The purpose of this review was to establish the optimal serum phenylalanine level in later life of PKU patients. We evaluated oxidative stress status, nitric oxide metabolism, cholesterol-derived oxysterols, vitamin D and bone status, and magnetic resonance imaging (MRI) in adult PKU patients according to serum phenylalanine level. Oxidative stress increased markedly at serum phenylalanine of 700-800 μmol/L. Serum phenylalanine higher than 700-850 μmol/L correlated with the disturbance of nitric oxide regulatory system. Adult PKU patients had poor vitamin D status and exhibited predominance of bone resorption over bone formation. In the brain, the levels of 24S-hydroxycholesterol, a marker of brain cholesterol elimination, were low at serum phenylalanine levels exceeding 650 μmol/L. MRI studies showed high signal intensity in deep white matter on T2-weighted and FLAIR images of PKU patients with serum phenylalanine greater than 500 μmol/L, with decreased apparent diffusion coefficients. Changes in most parameters covering the entire body organs in adult PKU were almost acceptable below 700-800 μmol/L of phenylalanine level. However, the optimal serum phenylalanine level should be 500 μmol/L or less in later life for the brain to be safe.

  1. Pancreatic exocrine insufficiency in critically ill adult patients.

    Science.gov (United States)

    Ma, Lijie; Liu, Yuhao; Lu, Zhifeng; Zhao, Li; Wang, Sheng

    2016-03-01

    Pancreatic exocrine insufficiency is usually present in patients with pancreatic diseases. Surprisingly, recent studies indicated that patients with critical illness often suffer from pancreatic injury due to non-specific reasons other than pancreatic diseases, and pancreatic exocrine insufficiency is also commonly observed in critically ill adult patients without preexisting pancreatic diseases. It is well known that malnutrition is the main clinical consequence of pancreatic exocrine insufficiency, thus, the high incidence of pancreatic exocrine insufficiency is most likely to be an important contributor of malnutrition which is a frequent problem associated with detrimental clinical outcomes in critically ill patients admitted into intensive care unit. In order to prevent pancreatic exocrine insufficiency due to primary pancreatic diseases, pancreatic enzyme replacement therapy is indispensable to treat indigestion, malabsorption and nutritional deficiency. Similarly, pancreatic enzyme supplementation has the potential to be an adjuvant therapy in critically ill patients with enteral nutrition therapy, which may be helpful to improve the nutritional status and the prognosis of critically ill patients by reducing the occurrence of malnutrition. Here, we reviewed the diagnostic methods of pancreatic exocrine function, the epidemiology and risk factors of pancreatic exocrine insufficiency, and potential treatment strategies for pancreatic exocrine insufficiency in critically ill adult patients.

  2. RNF213 Mutations in an Ethnically Diverse Population with Moyamoya Disease

    Science.gov (United States)

    Cecchi, Alana C.; Guo, Dongchuan; Ren, Zhao; Flynn, Kelly; Santos-Cortez, Regie Lyn P.; Leal, Suzanne M.; Wang, Gao T.; Regalado, Ellen S.; Steinberg, Gary K.; Shendure, Jay; Bamshad, Michael J.; Grotta, James C.; Nickerson, Deborah A.; Pannu, Hariyadarshi; Milewicz, Dianna M.

    2015-01-01

    Background and Purpose Moyamoya disease (MMD) is a rare, genetically heterogeneous cerebrovascular disease resulting from occlusion of the distal internal carotid arteries. A variant in the Ring Finger 213 gene (RNF213), altering arginine at position 4810 (p.R4810K), is associated with MMD in Asian populations. However, there is a lack of data on the role of RNF213 in MMD patients of additional ethnicities and diasporic Asian populations. We investigate the contribution of RNF213 alterations to MMD in an ethnically diverse population based in the United States (U.S). Methods We initially sequenced RNF213 exons 43, 44, 45 (encoding the eponymous RING finger domain), and exon 60 (encoding p.R4810K), in 86 ethnically diverse patients with MMD. Comprehensive exome sequencing data from 24 additional MMD patients was then analyzed to globally identify RNF213 variants. Segregation of variants with MMD and other vascular diseases was assessed in families. Results RNF213 p.R4810K was identified in 56% (9/16) of MMD patients of Asian descent, and not in 94 patients of non-Asian descent. 3.6% (4/110) of patients had variants in the exons encoding the RING finger domain. Seven additional variants were identified in 29% (7/24) of MMD patients who underwent exome sequencing. Segregation analysis supported an association with MMD for two variants, and a lack of association with disease for one variant. Conclusions These results confirm that alterations in RNF213 predispose patients of diverse ethnicities to MMD, and that the p.R4810K variant predisposes individuals of Asian descent in the U.S. to MMD. PMID:25278557

  3. Perfusion MRI abnormalities in the absence of diffusion changes in a case of moyamoya-like syndrome in neurofibromatosis type 1

    Energy Technology Data Exchange (ETDEWEB)

    El-Koussy, Marwan; Kiefer, Claus; Schroth, Gerhard [Department of Neuroradiology, University of Bern, Inselspital, Freiburgstrasse 4, 3010 Bern (Switzerland); Loevblad, Karl-Olof [Department of Neuroradiology, University of Bern, Inselspital, Freiburgstrasse 4, 3010 Bern (Switzerland); Neuroradiology, Service de Radiodiagnostic, Hopital Cantonal Universitaire HUG, 24 rue Micheli-du-Crest, 1211 Geneva 11 (Switzerland); Steinlin, Maja [Department of Neuropediatrics, University of Bern, Inselspital, Freiburgstrasse 4, 3010 Bern (Switzerland)

    2002-11-01

    We report on a 12-year-old boy with neurofibromatosis type 1 who suffered a transient ischemic attack. Angiography revealed occlusion of intracranial arteries, moyamoya vessels and leptomeningeal collaterals. The conventional T2-weighted and the diffusion-weighted MRI images demonstrated no pathology. Dynamic first-pass postgadolinium T2* perfusion-weighted MRI depicted altered hemodynamics in the vascular territory of the left middle cerebral artery, which defined this region as ischemic tissue at risk. The patient suffered a repeat transient ischemic attack5 days later. (orig.)

  4. ACG Clinical Guideline: Nutrition Therapy in the Adult Hospitalized Patient.

    Science.gov (United States)

    McClave, Stephen A; DiBaise, John K; Mullin, Gerard E; Martindale, Robert G

    2016-03-01

    The value of nutrition therapy for the adult hospitalized patient is derived from the outcome benefits achieved by the delivery of early enteral feeding. Nutritional assessment should identify those patients at high nutritional risk, determined by both disease severity and nutritional status. For such patients if they are unable to maintain volitional intake, enteral access should be attained and enteral nutrition (EN) initiated within 24-48 h of admission. Orogastric or nasogastric feeding is most appropriate when starting EN, switching to post-pyloric or deep jejunal feeding only in those patients who are intolerant of gastric feeds or at high risk for aspiration. Percutaneous access should be used for those patients anticipated to require EN for >4 weeks. Patients receiving EN should be monitored for risk of aspiration, tolerance, and adequacy of feeding (determined by percent of goal calories and protein delivered). Intentional permissive underfeeding (and even trophic feeding) is appropriate temporarily for certain subsets of hospitalized patients. Although a standard polymeric formula should be used routinely in most patients, an immune-modulating formula (with arginine and fish oil) should be reserved for patients who have had major surgery in a surgical ICU setting. Adequacy of nutrition therapy is enhanced by establishing nurse-driven enteral feeding protocols, increasing delivery by volume-based or top-down feeding strategies, minimizing interruptions, and eliminating the practice of gastric residual volumes. Parenteral nutrition should be used in patients at high nutritional risk when EN is not feasible or after the first week of hospitalization if EN is not sufficient. Because of their knowledge base and skill set, the gastroenterologist endoscopist is an asset to the Nutrition Support Team and should participate in providing optimal nutrition therapy to the hospitalized adult patient.

  5. Movement disorders associated with moyamoya disease: a report of 4 new cases and a review of literatures.

    Science.gov (United States)

    Baik, Jong Sam; Lee, Myung Sik

    2010-07-30

    The aim of this study was to define the clinical characteristics of patients who developed movement disorders in association with moyamoya disease (MMD). Using PubMed and medical records of our hospital from 1985 to 2008, we searched for patients who developed movement disorders in association with MMD. This study included 38 patients described in previous studies and 4 patients found in the medical records. The onset of movement disorders was thought to be sudden. In 13 patients, the movement disorders were precipitated by hyperventilation or emotional stress. Twenty-seven of the 42 patients developed chorea, 4 patients developed dystonia, and 4 developed a mixture of both. The movement disorders of the remaining 7 patients were described as dyskinesia. A third of the 42 patients developed bilateral movement disorders, and their mean age was younger than that of those with unilateral movement disorders. In 37 of the 42 patients, brain imaging studies showed ischemic lesions, but the remaining 5 patients showed no parenchymal lesions. Cerebral perfusion studies showed hypoperfusion in the basal ganglia and in the cerebral cortical areas. Most patients improved whether they were treated or not. MMD must be included in the differential diagnosis of the sudden onset of dyskinesias, particularly chorea and focal dystonia. Even in patients with no parenchymal lesion in brain imaging studies, cerebral angiography and cerebral blood perfusion studies must be performed, if they develop a sudden onset or recurrent movement disorders preceded by emotional stress or hyperventilation.

  6. An Attempt to evaluate cerebral hemodynamics before and after neurosurgical treatment of Moyamoya disease by factor analysis

    Energy Technology Data Exchange (ETDEWEB)

    Nakazawa, Keiji; Ishii, Katsumi; Sakai, Fumihiko; Matsubayashi, Takashi (Kitasato Univ., Sagamihara, Kanagawa (Japan). School of Medicine)

    1991-09-01

    We made factor analysis on the data obtained by the radionuclide angiography to evaluate cerebral hemodynamics before and after neurosurgical treatment in a patient with Moyamoya disease (bilateral occlusion of internal carotid arteries). Tracer used was {sup 99m}Tc-RBC or {sup 99m}TcHSA. Before the neurosurgical treatment, the cerebral factor image obtained by the factor analysis showed defects in the right and the left frontal cerebral regions, and the venous factor image showed abnormal accumulations in the right and the left frontal cerebral regions. After Encephalo-Myo-Synangiosis and burr hole operations, defects in the right and the left frontal cerebral regions on the cerebral factor image were improved and abnormal accumulations in the right and the left frontal cerebral regions on the venous factor image disappeared. (author).

  7. Dysnatremias and Survival in Adult Burn Patients: A Retrospective Analysis

    Science.gov (United States)

    2013-01-11

    DOI: 10.1159/000346206 Dysnatremias and Survival in Adult Burn Patients: A Retrospective Analysis Ian J. Stewart a Benjamin D. Morrow a... mortality rate of 4.3%. On Cox proportional hazard regres- sion age, %TBSA, ISS, and AKIN stage were found to be sig- nificant predictors of mortality ...hypernatremia [1–4] and hyponatremia [1, 4, 5] have been examined in the hospital setting and have been found to be significantly associated with mortality

  8. Giant Primary Mature Retroperitoneal Teratomain in Adult Male Patient

    OpenAIRE

    R. Ebrahimian; D. Tasa

    2015-01-01

    Introduction: Teratomas are congenital tumors consisting of derivatives from the ectoderm, endoderm and mesoderm germ cell layers. A teratoma is considered to be a non-seminomatous germ cell tumor and is typically located in either the sacrococcygeal region or in the gonads. Giant retroperitoneal teratomas in adults are even rarer, with only a few cases previously described in the literature. Case report: A 35-year-old male patient with severe nausea and vomiting was taken to the emergency wa...

  9. Interventional radiology procedures in adult patients who underwent liver transplantation

    Institute of Scientific and Technical Information of China (English)

    Roberto Miraglia; Luigi Maruzzelli; Settimo Caruso; Mariapina Milazzo; Gianluca Marrone; Giuseppe Mamone; Vincenzo Carollo; Salvatore Gruttadauria; Angelo Luca; Bruno Gridelli

    2009-01-01

    Interventional radiology has acquired a key role in every liver transplantation (LT) program by treating the majority of vascular and non-vascular post-transplant complications, improving graft and patient survival and avoiding, in the majority of cases, surgical revision and/or re-transplantation. The aim of this paper is to review indications, technical consideration, results achievable and potential complications of interventional radiology procedures after deceased donor LT and living related adult LT.

  10. Moyamoya disease manifested as multiple simultaneous intracerebral hemorrhages: A case report and literature review

    Science.gov (United States)

    Yu, Jinlu; Yuan, Yongjie; Li, Wei; Xu, Kan

    2016-01-01

    Multiple simultaneous intracerebral hemorrhages (MSIH) caused by Moyamoya disease (MMD) is extremely rare. To date, the clinical manifestations, imaging characteristics and mechanism of MMD-induced MSIH have not yet been elucidated. In order to improve the understanding on such cases, the present study described a rare case of MSIH caused by MMD. A 40-year-old female patient with no history of hypertension or diabetes mellitus experienced a sudden headache followed by coma. Cranial computed tomography (CT) examination revealed MSIH in the left frontal area, temporal lobe and basal ganglia. CT angiography and digital subtraction angiography examinations revealed typical characteristics of MMD. Subsequent to excluding disorders of the blood system and blood coagulation, we concluded that the present case of MSIH was caused by MMD. Hematoma evacuation and decompressive craniectomy were performed with satisfactory results. In addition, after reviewing previous MSIH cases in the literature, potential mechanisms of MMD-mediated MSIH were considered. In conclusion, MMD should be considered as a possible cause of MSIH during diagnosis and treatment. MMD can lead to pathological changes in the fragility of small arteries; therefore, rupture and hemorrhage at one site may induce a transient increase in blood pressure, causing the rupture of small arteries at other sites, and thus leading to MSIH. Hematoma evacuation and decompression should be conducted in selective cases of MMD-induced MSIH in order to achieve a good prognosis. PMID:27588064

  11. Stratification of cerebral hemodynamics of child moyamoya disease using CBF SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Nakagawara, Jyoji; Saito, Norihiro; Osato, Toshiaki; Kamiyama, Kenji; Takeda, Rihei; Nakamura, Hirohiko [Nakamura Memorial Hospital, Sapporo (Japan)

    2002-12-01

    In order to make sure the stratification of cerebral hemodynamics of child moyamoya disease, we evaluated {sup 123}I-IMP SPECT before and after surgical revascularization. The aim of this paper is to establish the semiquantitative parameters that can be applied to estimate severity of hemodynamic cerebral ischemia instead of quantitative parameters. Quantitative studies using IMP-autoradiography (ARG) method were performed on thirteen patients to measure resting regional cerebral blood flow (rCBF), and vascular reserve (rVR): (Diamox-activated rCBF/resting rCBF-1) x 100%. Semiquantitative parameters were calculated from the ratio of region of interest (ROI) counts in the anterior cerebral artery (ACA) and middle cerebral artery (MCA) territory to the dominant cerebellum counts (ROI/Ce ratio) at resting and Diamox-activated conditions. From the quantitative study, both mean resting rCBF less than 40 ml/100 g/min and rVR less than 10% could indicate stage 2 hemodynamic cerebral ischemia. The prediction of stage 2 ischemia using semiquantitative parameters (resting ROI/Ce ratio less than 0.9 and Diamox-activated ROI/Ce ratio less than 0.85 in the MCA territory) was not statistically different comparison to the diagnosis using quantitative parameters (using Fisher exact test<0.0001, Sensitivity and specificity were 87.5% and 90.9%, respectively). The ROI/Ce ratio can be utilized as simple parameters instead of quantitative parameters. (author)

  12. Vaccination of Adult Patients with Systemic Lupus Erythematosus in Portugal

    Science.gov (United States)

    Moraes-Fontes, Maria Francisca; Antunes, Ana Margarida; Gruner, Heidi; Riso, Nuno

    2016-01-01

    In the wake of the Portuguese vaccination program 50th anniversary it seems appropriate to review vaccination in patients with systemic lupus erythematosus. Controversial issues as regards the association between autoimmune diseases, infections, and vaccines are discussed as well as vaccine safety and efficacy issues as regards chronic immunosuppressant (IS) drug therapy. After a brief overview of national policies, specific recommendations are made as regards vaccination for adult patients with SLE with a particular focus on current IS therapy and unmet needs. PMID:27069477

  13. Vaccination of Adult Patients with Systemic Lupus Erythematosus in Portugal

    Directory of Open Access Journals (Sweden)

    Maria Francisca Moraes-Fontes

    2016-01-01

    Full Text Available In the wake of the Portuguese vaccination program 50th anniversary it seems appropriate to review vaccination in patients with systemic lupus erythematosus. Controversial issues as regards the association between autoimmune diseases, infections, and vaccines are discussed as well as vaccine safety and efficacy issues as regards chronic immunosuppressant (IS drug therapy. After a brief overview of national policies, specific recommendations are made as regards vaccination for adult patients with SLE with a particular focus on current IS therapy and unmet needs.

  14. Stepping to stability and fall prevention in adult psychiatric patients.

    Science.gov (United States)

    Emory, Sara L; Silva, Susan G; Christopher, Eric J; Edwards, Pamela B; Wahl, Leanne E

    2011-12-01

    Fall prevention is a major area of concern in inpatient settings. This article reports on the feasibility of implementing a daily exercise program that features line dancing to promote stability, balance, and flexibility in adult psychiatric patients and describes the impact of that program. Six hundred sixty-five patient charts drawn from before and after the practice change were reviewed. The fall rate after the introduction of line dancing was 2.8% compared with 3.2% before implementation. In a setting that treats both men and women of many ages and with varying levels of mobility, line dancing offers a viable approach to exercise in a secure setting.

  15. [Nursing diagnosis in adult patients with acute myeloid leukemia].

    Science.gov (United States)

    de Souza, Luccas Melo; Gorini, Maria Isabel Pinto Coelho

    2006-09-01

    This case study aimed at identifying Nursing Diagnosis (ND) in adult patients with Acute Myeloid Leukemia, with the purpose of contributing to the Systematization of Nurse Care. Interviews and observation were used for data collection, in addition to Nursing Process application. During the three months of data collection, other NDs were obtained by searching the files of the 6 patients. The 32 ND found in this study were grouped according to Maslow's hierarchy of needs. Out of these 32 ND, 15 corresponded to changes in Physiological Needs, and 10 to changes in Protection and Safety Needs.

  16. [The definition of the medical clown's role with adult patients].

    Science.gov (United States)

    Scheyer, Rachel; Nuttman-Shwartz, Orit; Ziyoni, Herzel

    2008-01-01

    In recent years, the healthcare system has grown increasingly aware of the need to develop and adopt new models and intervention methods aimed at improving patients' quality of life. As part of this perception, medical clowns have been integrated into hospitals, primarily in work with children. Recently, there have been attempts to integrate clowns into work with adult patients in emergency rooms, but this intervention method has not yet been systematically implemented and studied. This article describes and examines the definition of the medical clown's role as an intervention strategy with adult outpatients suffering from chronic and life-threatening illnesses. The study is qualitative and based on a content analysis of the documentation of the work of two medical clowns over two years. The dominant theme arising from this analysis involves the definition of the clown's role within the medical space of the hospital and includes perspectives on his integration into the hospital's multidisciplinary medical staff and his impact on the staff and on patients and their families. The findings indicate that, from the clowns' point of view, integrated medical clowns as part of the medical team, would contribute to the functioning of both patients and staff. This is in accord with additional studies conducted recently in medical centers around the world. Since this is a pioneering study, there is room to further probe and research the medical clown's contribution to assisting and improving patients' and staff's quality of life and to develop ways of increasing his integration and professionalism.

  17. Triage in an adult emergency service: patient satisfaction

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    Pollyane Liliane Silva

    2016-06-01

    Full Text Available Abstract OBJECTIVE Assess the degree of patient satisfaction with triage in the adult emergency service of a public hospital. METHOD Exploratory, descriptive, cross-sectional study with a quantitative approach. Three hundred patients were interviewed and the data were analyzed using descriptive statistics based on sociodemographic variables and those related to patient satisfaction. RESULTS There was a predominance of women, with elementary education and a mean age of 41 years. Most of the interviewees reported being satisfied in regard to the following items: timely service, embracement, trust, environment (comfort, cleanliness and signage, humanization (courtesy, respect, and interest, timely referral/scheduling of appointments and care expectations. CONCLUSION There was a high level of patient satisfaction, evidenced by the strong association of user satisfaction with the items investigated.

  18. Factors related to orthodontic treatment time in adult patients

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    Ana Camila Esteves de Oliveira Melo

    2013-10-01

    Full Text Available INTRODUCTION: The length of time that it takes an orthodontist to treat adult patients varies widely. OBJECTIVE: The aim of this study was to investigate how different variables influence treatment time. METHODS: Seventy clinical case reports of successfully treated adult patients were examined. The patients were selected from 4,723 records held by three experienced orthodontists. The influence exerted by the following variables on treatment time was assessed: age, sex, facial pattern, severity of malocclusion (measured by the PAR index, sagittal relationship of canines, type of brackets (ceramic or metal, tooth extractions, missed appointments and orthodontic appliance issues/breakages, the latter being the dependent variable. Assessment was performed by multiple linear regression analysis, followed by the stepwise method with P < 0.05. RESULTS: The number of times a patient missed their appointment (no-show (R² = 14.4%, p < 0.0001 and the number of appliance issues/breakages (R² = 29.71%, p = 0.0037 significantly affected variability in treatment time, and these two variables together can predict 43.75% (R² total of the overall variability in treatment time. Other factors, such as canine relationship at the beginning of treatment, bracket type (metal or ceramic, tooth extractions, age at start of treatment, severity of the initial malocclusion, sex and facial pattern had no significant bearing on treatment time. CONCLUSIONS: The duration of orthodontic treatment in adults, when performed by experienced orthodontists, is mainly influenced by factors related to patient compliance. However, several factors which were not included in this study may contribute to variability in orthodontic treatment time.

  19. Whole-brain CT perfusion and CT angiography assessment of Moyamoya disease before and after surgical revascularization: preliminary study with 256-slice CT.

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    Jun Zhang

    Full Text Available BACKGROUND/AIMS: The 256-slice CT enables the entire brain to be scanned in a single examination. We evaluated the application of 256-slice whole-brain CT perfusion (CTP in determining graft patency as well as investigating cerebral hemodynamic changes in Moyamoya disease before and after surgical revascularization. METHODS: Thirty-nine cases of Moyamoya disease were evaluated before and after surgical revascularization with 256-slice CT. Whole-brain perfusion images and dynamic 3D CT angiographic images generated from perfusion source data were obtained in all patients. Cerebral blood flow (CBF, cerebral blood volume (CBV, time to peak (TTP and mean transit time (MTT of one hemisphere in the region of middle cerebral artery (MCA distribution and contralateral mirroring areas were measured. Relative CTP values (rCBF, rCBV, rTTP, rMTT were also obtained. Differences in pre- and post- operation perfusion CT values were assessed with paired t test or matched-pairs signed-ranks test. RESULTS: Preoperative CBF, MTT and TTP of potential surgical side were significantly different from those of contralateral side (P<0.01 for all. All graft patencies were displayed using the 3D-CTA images. Postoperative CBF, rCBF and rCBV values of surgical side in the region of MCA were significantly higher than those before operation (P<0.01 for all. Postoperative MTT, TTP, rMTT and rTTP values of the surgical side in the region of MCA were significantly lower than those before operation (P<0.05 for all. CONCLUSION: The 256-slice whole-brain CTP can be used to evaluate cerebral hemodynamic changes in Moyamoya disease before and after surgery and the 3D-CTA is useful for assessing the abnormalities of intracranial arteries and graft patencies.

  20. Paramedic assessment of pain in the cognitively impaired adult patient

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    Lord Bill

    2009-10-01

    Full Text Available Abstract Background Paramedics are often a first point of contact for people experiencing pain in the community. Wherever possible the patient's self report of pain should be sought to guide the assessment and management of this complaint. Communication difficulty or disability such as cognitive impairment associated with dementia may limit the patient's ability to report their pain experience, and this has the potential to affect the quality of care. The primary objective of this study was to systematically locate evidence relating to the use of pain assessment tools that have been validated for use with cognitively impaired adults and to identify those that have been recommended for use by paramedics. Methods A systematic search of health databases for evidence relating to the use of pain assessment tools that have been validated for use with cognitively impaired adults was undertaken using specific search criteria. An extended search included position statements and clinical practice guidelines developed by health agencies to identify evidence-based recommendations regarding pain assessment in older adults. Results Two systematic reviews met study inclusion criteria. Weaknesses in tools evaluated by these studies limited their application in assessing pain in the population of interest. Only one tool was designed to assess pain in acute care settings. No tools were located that are designed for paramedic use. Conclusion The reviews of pain assessment tools found that the majority were developed to assess chronic pain in aged care, hospital or hospice settings. An analysis of the characteristics of these pain assessment tools identified attributes that may limit their use in paramedic practice. One tool - the Abbey Pain Scale - may have application in paramedic assessment of pain, but clinical evaluation is required to validate this tool in the paramedic practice setting. Further research is recommended to evaluate the Abbey Pain Scale and to

  1. Usefulness and limitation of MR imaging and MR angiography in diagnosis of juvenile moyamoya disease

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    Aoki, Takeshi; Matsuzawa, Hitoshi; Houkin, Kiyohiro; Kamiyama, Hiroyasu; Abe, Hiroshi; Miyasaka, Kazuo (Hokkaido Univ., Sapporo (Japan). School of Medicine); Saito, Hisatoshi

    1993-04-01

    Magnetic resonance (MR) images and MR angiograms (MRA) were studied in 20 childhood cases of moyamoya disease. Both MRI and MRA successfully demonstrated moyamoya vessels in the basal ganglia in all cases, with a positive but not definite correlation to the conventional angiographic findings. MRI depicted the stenotic and occlusive lesions of the carotid fork and horizontal portion of the middle cerebral artery effectively. MRA demonstrated some lesions which even MRI failed to prove, but it tended to overestimate the lesions. Post-operative state of collateral flow and the patency of EC-IC bypass graft could be evaluated as accurately with MRA as with conventional angiography, although MRA was limited in spatial resolution and evaluation of flow direction. In conclusion, MRI and MRA were considered to be useful in the diagnosis of moyamoya disease in stages 3 and 4, but less effective in the evaluation of its angiographical stage. (author).

  2. Evaluation of surgical outcome with pre-and post-operative rest/acetazolamide Tc-99m HMPAO SPECT in children with moyamoya disease

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    Lee, D. S.; Wang, K. C.; Cho, B. K.; Chung, J. K.; Lee, M. C. [College of Medicine, Seoul National Univ., Seoul (Korea, Republic of); Hyun, I. Y. [College of Medicine, Inha Univ., Inchon (Korea, Republic of)

    1998-08-01

    This study was performed to evaluate the outcome of encephalo-duro-arterio-synangiosis(EDAS) surgery with rest/acetazolamide Tc-99m HMPAO SPECT in moyamoya disease. Rest/acetazolamide subtraction SPECT with consecutive acquisition were done before and 2 months after 21 EDAS surgeries in 18 patients. Perfusion decrease was graded visually for 14 areas of each hemisphere as 0 (normal) to 3 (defect) using 4 point scoring system. Postopertive rest perfusion or perfusion reserve was compared with preoperative ones. Among 294 areas of 21 hemispheres, rest perfusion abnormality was found in 97 areas of 15 hemispheres. Decrease of perfusion reserve was found in 146 areas of 18 hemispheres. Six hemispheres having normal rest perfusion and 12 of 15 hemispheres having rest perfusion abnormality showed reserve decrease. Three having rest perfusion defect did not change after acetazolamide in preoperative SPECT. After operation, 16 patients (89%) demonstrated clinical improvement. Fifteen among 18 hemispheres (83%) with decreased reserve improved. Rest perfusion abnormality improved in 6 among the 15 hemispheres (40%). The areas having rest perfusion and/or reserve decrease improved in 87 among 146 areas (60%). Decrease of reserve improved in 85% (68/80). However, areas without reserve decrease also improved in 29% (19/66). The better was preoperative rest perfusion in involved areas or the more decreased vascular reserve, the more improved perfusion and reserve after operation. We conclude that assessment of perfusion and perfusion reserve using rest/acetazolamide brain perfusion SPECT predict the surgical outcome in patients with moyamoya disease.

  3. Characteristic distribution of stroke localization due to moyamoya disease and its mechanisms%烟雾病脑卒中部位的分布特征及其机制

    Institute of Scientific and Technical Information of China (English)

    胡琦; 康慧聪; 陈琳; 许峰; 刘晓艳; 李巷; 朱遂强

    2009-01-01

    目的 分析烟雾病脑卒中的好发部位,提高对烟雾病的认识,以减少漏诊.方法 分析我院神经内科1998年1月至2008年12月的32例烟雾病患者,对其脑卒中部位进行总结分类,并计算各构成比.结果 32例病例中男性14例,女性18例,男女比例为1:1.28,发病年龄7~47岁,缺血性脑卒中5例(15.6%),平均年龄24岁,出血性脑卒中21例(65.6%),平均年龄33岁,同时患缺血性脑卒中和出血性脑卒中者6例(18.8%),平均年龄32岁.出现梗死部位共20处,常见部位依次为额顶叶12次(60%)、颞枕叶4次(20%)及基底节区4次(20%),发生出血共28次,常见部位依次为脑室旁11次(42.8%)、脑室11次(39.2%)、颞枕叶3次(10.8%)及蛛网膜下腔2次(7.2%),小脑和脑干无脑卒中发生.结论 成人烟雾病多以颅内出血发病;无脑卒中危险因素成人出现脑室或脑室旁出血、额顶叶梗死以及青少年颅内出现缺血性病变合并癫痫者需高度警惕烟雾病.%Objective To clinically analyze the frequent stroke localizations of moyamoya disease in order to improve our cognition toward it and reduce missed diagnosis. Methods All 32 patients were prospectively analyzed over the past 10 years in our hospital. Results The ratio of female to male was 1.28 and their age of onset ranged from 7 to 47 years old. Mean age of 5 ischemic stroke patients (15.6%) was 24 and mean age of 21 haemorrhagic stroke patients (65.6%) was 33 while mean age of 6 mixed type stroke patients (18. 8% ) was 32. The frequent ischemic stroke localizations were frontoparietal lobe (60%),temporo-occipital lobe (20%), and periventricular zone (20%). The frequent haemorrhagic stroke iocalizations were periventricular zone (42.8%), ventricle (39.2%), temporo-occipital lobe (10. 8%) and subarachnoid space (7.2%). No cerebellum and brain stem stroke occurred. Conclusion Adult patients usually develop intracranial hemorrhage. Moyamoya disease should be considered severely when adult

  4. Analysis of Adult Trauma Patients Admitted to Emergency Department

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    Sema Puskulluoglu

    2015-09-01

    Full Text Available Purpose: Trauma is one of the most common reason of admissions to emergency departments. In this study, it was aimed to determine the demographic characteristics, etiology, morbidity and mortality rates and prognosis of adult trauma patients admitted to our emergency department (ED. Materials and Methods: Patients over the age of 18 years, who admitted to ED between 01 March 2011 and 31 August 2011 were included in this retrospective study. Patient examination cards, hospitalization files and records entered with ICD 10 codes to hospital automation system were analyzed. Patients with inaccessible data were excluded from the study. Results: During the study period, total number of 110495 patients admitted to ED, and 13585 (12,29% of them admitted with trauma. Simple extremity injuries (38,28% and falls (31,7% were most common etiological factors. Glasgow coma scales of 99,71% of the patients were between 13 and 15. Of the patients with trauma, 9,6% had a Computed Tomography (CT scan, and 84,5% of CT scans were evaluated as normal, and cranial CT was the most requested one. Only 6% of the patients were hospitalized, and 0,9% of the trauma patients died. Falls from height in females and traffic accidents in males were the leading causes of death. Conclusion: Most of the patients with simple traumas admitted to ED can be discharged from the hospital with a complete history and careful examination. The rate of unnecessary medical tests, loss of time and waste money should be reduced, and the time and labor allocated to severe patients can be increased by this way. [Cukurova Med J 2015; 40(3.000: 569-579

  5. An Adult Patient with Fontan Physiology: A TEE Perspective

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    Edward Gologorsky

    2012-01-01

    Full Text Available Fontan and Baudet described in 1971 the separation of the pulmonary and systemic circulations resulting in univentricular physiology. The evolution of the Fontan procedure, most notably the substitution of right atrial-to-pulmonary artery anastomosis with cavopulmonary connections, resulted in significantly improved late outcomes. Many patients survive well into adulthood and are able to lead productive lives. While ideally under medical care at specialized centers for adult congenital cardiac pathology, these patients may present to the outside hospitals for emergency surgery, electrophysiologic interventions, and pregnancy. This presentation presents a “train of thought,” linking the TEE images to the perioperative physiologic considerations faced by an anesthesiologist caring for a patient with Fontan circulation in the perioperative settings. Relevant effects of mechanical ventilation on pulmonary vascular resistance, pulmonary blood flow and cardiac preload, presence of coagulopathy and thromboembolic potential, danger of abrupt changes of systemic vascular resistance and systemic venous return are discussed.

  6. A rare agent of spondylodiscitis in adult patient: Salmonella enteritidis

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    Bilgehan Aygen

    2012-03-01

    Full Text Available Salmonella infections are a public health problem in Turkey,as all over the world. Salmonella spp. can causevery different infections such as gastroenteritis, typhoidparatyphoidfever, bacteremia, local metastatic infectionsand chronic carriage. Salmonella spondylodiscitis occursrarely in the adult population. In this case report, we havepresented a 66 years old female patient followed with thediagnosis of rheumatoid arthritis and treated with prednisolone.The patient had a new diagnosis of Salmonellaenteritidis and we aimed to discuss similar cases by theculture of lumbar empyema culture ampiciline, cefotaxime,trimethoprim/sulfamethoxazole, ciprofloxacin was revealedthe presence of resistant S.enteritidis. The patienthas received ciprofloxacin 2x200 mg per day for 3 weeksas intravenous. And patient was discharged with advice ofusing ciprofloxacin as per oral long three months.

  7. Thyroid gland diseases in adult patients with diabetes mellitus.

    Science.gov (United States)

    Vondra, K; Vrbikova, J; Dvorakova, K

    2005-12-01

    This review concerns the relation between most frequent thyroid gland diseases and diabetes mellitus in adult patients. Special attention is paid to autoimmune thyroiditis, Graves' disease, thyroid autoimmunity in pregnant diabetic women, and iodine metabolism. We focused on mechanisms leading to coexistence of both endocrine disorders, and on distinctions in the prevalence, diagnosis, clinical course and treatment of thyroid diseases in diabetic patients. The prevalence of thyroid diseases in diabetic patients is 2-3 times higher than in nondiabetic subjects; it raises with age, and is strongly influenced by female gender and autoimmune diabetes. Clinical relevance of thyroid diseases, especially in diabetic patients, significantly increases if it is associated with deteriorated function, which always cause a number problems with metabolic compensation of diabetes. Most serious consequences are increased frequency of hypoglycaemia in hypothyroidism and development of potentially life-threatening ketoacidosis in thyrotoxicosis. In spite of that, little attention is paid to the diagnosis of thyroid diseases in diabetics, as they are diagnosed in only about half of the patients. At the end, we provide recommendations for the thyroid disease screening and diagnosis in patients with diabetes mellitus based on our experience.

  8. A rare presentation of stroke in young age ( moyamoya disease

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    Amal F Radwan

    2013-01-01

    Full Text Available A 37-year-old hypertensive housewife presented with a sudden onset of left-sided hemiplegia, hemianaesthesia, dysarthria and urinary incontinence. The condition was preceded by recurrent attacks of motor neurological deficits over a 3-year duration. She reported a history of a fall from a height at the age of 10, which was followed by a hearing deficit and a history of two caesarean sections after eclampsia. The blood pressure was 170/100 mmHg. Laboratory investigations revealed hyperglycaemia (fasting glucose 306 mg/dl and normal kidney function tests. The computed tomography scans revealed old multiple bilateral cerebral infarcts with recent intracranial haemorrhage in the right parietal region. The inflammatory markers (ESR and CRP and immune profile (ANA, anti-ds DNA and ANCA were found to be normal. Cerebral angiography revealed a complete occlusion of the intracranial parts of both internal carotid arteries at their supraclinoid segments along with the proximal parts of the anterior cerebral artery and middle cerebral artery, with collaterals from the posterior circulation. Consequently, the diagnosis of moyamoya disease with the collaterals was confirmed. Antihypertensive medications and insulin were administered. Cerebral dehydration measures were undertaken with partial improvement. A superficial temporal artery-middle cerebral artery bypass operation was performed with some postoperative improvement. One month later, she suffered a new stroke with severe impairment of the level of consciousness; the computed tomography scans revealed a large recent cerebral infarct, her condition deteriorated rapidly and she died shortly thereafter.

  9. Unexplained lymphadenopathies: autoimmune lymphoproliferative syndrome in an adult patient.

    Science.gov (United States)

    Leal-Seabra, Fatima; Costa, Gonçalo Sarmento; Coelho, Henrique Pereira; Oliveira, Agripino

    2016-12-15

    Autoimmune lymphoproliferative syndrome (ALPS) is characterised by massive enlargement of the lymphoid organs, autoimmune cytopenias and a predisposition to develop lymphoid malignancies. The basic defect is a disturbance of the lymphocyte apoptosis, and a high number of circulating TCRab CD3(+)CD4(-)CD8(-) T-cells (double-negative T cells (DNT cells)). We describe a case of a 41-year-old man with fever, hepatosplenomegaly, multiple lymphadenopathy, autoimmune haemolytic anaemia and severe thrombocytopenia. Peripheral blood immunophenotyping revealed elevation of the characteristic DNT cells in 8% and high levels of interleukin 10. Histopathological analysis of lymph nodes showed lymphadenitis with paracortical hyperplasia. It was assumed as a probable diagnosis of ALPS, and the procedure was to medicate the patient with steroids. As a result, a significant clinical improvement was achieved, and he has been in remission for 2 years. To our knowledge, this is the first case reported in a Portuguese adult patient.

  10. Dense Persistent Pupillary Membrane in an Adult Patient

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    Yesim Altay

    2014-06-01

    Full Text Available Persistent pupillary membranes (PPM are congenital abnormalities which results from an incomplete involution of tunica vasculosa lentis and are rarely seen in adults. A thirty-year old man applied to the hospital with the complaint of uncommon-looking pupils and progressive blurring of vision in the left eye. On examination, uncorrected visual acuity (Snellen were 20/100 in the right eye and 20/640 in the left eye with amblyopia. On biomicroscopic examination, there were bilateral dense PPM and cataract in the left eye. Visual field analysis of right and left eyes showed great narrowing of visual fields. We present our case in order to emphasize that analysis of visual field of patients with PPM is as important as central vision when planning its treatment. For planning treatment of patients with PPM, visual impairment, size of pupillary opening, and visual field analysis should be considered.

  11. Comparing postural balance among older adults and Parkinson's disease patients

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    Isabela Andrelino de Almeida

    Full Text Available ABSTRACT The objective of this study was to compare postural balance among healthy older adults and Parkinson's disease (PD patients during one-legged stance balance. We recruited 36 individuals of both sexes and divided them into two groups: healthy older adults (HG, and individuals with PD (PG. All the participants were assessed through a single-leg balance test, with eyes open, during 30 seconds (30 seconds of rest across trials on a force platform. Balance parameters were computed from mean across trials to quantify postural control: center of pressure (COP area and mean velocity in both directions of movement, anterior-posterior and medial-lateral. Significant differences between-group were reported for area of COP (P=0.002 and mean velocity in anterior-posterior direction (P=0.037, where poor postural control was related to PD patients rather than to healthy individuals. One-legged stance balance was a sensitive task used to discriminate poor postural control in Parkinson individuals.

  12. The Efficacy of Surgical Treatment for the Secondary Prevention of Stroke in Symptomatic Moyamoya Disease

    Science.gov (United States)

    Qian, Cong; Yu, Xiaobo; Li, Jianru; Chen, Jingyin; Wang, Lin; Chen, Gao

    2015-01-01

    Abstract The treatment of moyamoya disease (MMD) is controversial and often depends on the doctor's experience. In addition, the choice of surgical procedure to treat MMD can differ in many ways. In this study, we performed a meta-analysis to determine whether surgical treatment of MMD is superior to conservative treatment and to provide evidence for the selection of an appropriate surgical treatment. The human case–control studies regarding the association of MMD treatment were systematically identified through online databases (PubMed, Web of Science, Elsevier Science Direct, and Springer Link). Inclusion and exclusion criteria were defined for the eligible studies. The fixed-effects model was performed when homogeneity was indicated. Alternatively, the random-effects model was utilized. This meta-analysis included 16 studies. Surgical treatment significantly reduced the risk of stroke (odds ratio (OR) of 0.17, 95% confidence interval (CI), 0.12–0.26, P < 0.01). A subgroup analysis showed that surgical treatment was more beneficial to hemorrhagic MMD (OR of 0.23, 95% CI, 0.15–0.38, P < 0.01), but there was no significant difference between surgical treatment and conservative treatment on ischemic MMD treatment (OR of 0.45, 95% CI, 0.15–1.29, P = 0.14). Further analysis indicated that compared to direct bypass surgery, indirect bypass surgery had a lower efficacy on secondary stroke risk reduction (OR of 1.79, 95% CI, 1.14–2.82, P = 0.01), while no significant difference was detected for perioperative complications. Surgery is an effective treatment for symptomatic MMD patients, and direct bypass surgery may bring more benefits for these patients. PMID:26656359

  13. Dystonia an unusual presentation in pediatric moyamoya disease: Imaging findings of a case

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    Suresh Kumar

    2016-01-01

    Full Text Available Moyamoya disease (MMD is a rare cerebrovascular disease characterized by idiopathic occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels along anterior or posterior circulation. We present an unusual case of MMD presenting with generalized dystonia as the predominant manifestation.

  14. Demonstration of cerebral perfusion abnormalities in moyamoya disease using susceptibility perfusion- and diffusion-weighted MRI

    Energy Technology Data Exchange (ETDEWEB)

    Adams, W.M.; Laitt, R.D. [Department of Neuroradiology, Central Manchester Healthcare Trust, Oxford Road, Manchester M13 9WL (United Kingdom); Li, K.L.; Jackson, A. [Department of Diagnostic Radiology, University of Manchester, Manchester M13 9PT (United Kingdom); Sherrington, C.R.; Talbot, P. [Department of Neurology, Central Manchester Healthcare Trust, Oxford Road, Manchester M13 9WL (United Kingdom)

    1999-02-01

    We describe the use of diffusion-weighted imaging and perfusion MRI using a contrast-medium bolus in the preoperative investigation for young man presenting with a cerebral ischaemic episode as a manifestation of moyamoya disease. (orig.) With 6 figs., 21 refs.

  15. Wavelet analysis of cerebral oxygenation oscillations in the screening of Moyamoya disease.

    Science.gov (United States)

    He, Ying; Jiang, Pengjun; Han, Shanshan; Wang, Rong; Li, Yue; Teng, Yichao; Gao, Tianxin

    2014-01-01

    Near-infrared spectroscopy (NIRS) was used to investigate the cerebral oxygenation of Moyamoya and healthy subjects. Continuous recordings of NIRS signals for 20 min (20 min signals) were obtained from 17 healthy subjects (age: 37.4 ± 11.3) and 17 Moyamoya subjects (age: 40.1 ± 11.2). Spectral analysis based on wavelet transformation identified five frequency intervals (I, 0.0095 Hz to 0.02 Hz; II, 0.02 Hz to 0.06 Hz; III, 0.06 Hz to 0.15 Hz; IV, 0.15 Hz to 0.40 Hz; and V, 0.40 Hz to 2.00 Hz) in the 20 min signals and three frequency intervals (III, 0.06 Hz to 0.15 Hz; IV, 0.15 Hz to 0.40 Hz; and V, 0.40 Hz to 2.00 Hz) in the 3 min signals (the first 3 min signals were continuously extracted from the 20 min signals). Significant differences (p Moyamoya disease. As a potential screening method for Moyamoya disease, the simple threshold method exhibited 73.5% accuracy.

  16. Respiratory pattern in an adult population of dystrophic patients.

    Science.gov (United States)

    D'Angelo, M G; Romei, M; Lo Mauro, A; Marchi, E; Gandossini, S; Bonato, S; Comi, G P; Magri, F; Turconi, A C; Pedotti, A; Bresolin, N; Aliverti, A

    2011-07-15

    We studied respiratory function and Chest Wall kinematics in a large population of adult patients affected by slow course muscular dystrophies such as Limb-Girdle Muscular Dystrophy (LGMD, n=38), Becker Muscular Dystrophy (BMD, n=20) and Facio-Scapulo Humeral Dystrophy (FSHD, n=30), through standard spirometry and through the Optoelectronic Plethysmography, to measure the thoraco-abdominal motion during Quiet Breathing and Slow Vital Capacity maneuvers. Within the restrictive pulmonary syndrome characterizing LGMD and FSHD, several different thoraco-abdominal patterns compared to those of healthy subjects were present in the more advanced stages of the disease. These differences were present in the seated position, during the execution of a maximal maneuver such as Slow Vital Capacity. A global respiratory (both inspiratory and expiratory) muscle involvement was more pronounced in the LGMD and FSHD than in the BMD patients, and a significant reduction of abdominal contribution in wheelchair bound patients was observed. In conclusion, OEP technique is able to reveal mild initial modifications in the respiratory muscles in FSHD and LGMD patients, which could be helpful for functional and new therapeutic strategy evaluation.

  17. ‘Smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adult

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    Imtiaz Ismail

    2015-06-01

    Full Text Available Moyamoya disease is a rare neurological condition that affects children and adults of all ages. It is characterized by chronic, progressive stenosis of the circle of Willis that ultimately leads to the development of extensive collateral vessels. Presenting symptoms are usually due to cerebral ischemia or hemorrhage. The Japanese term moyamoya (meaning puffy or obscure was coined to describe the characteristic ‘smoke in the air’ appearance of these vessels on cerebral angiography. Moyamoya has the highest recorded incidence in Japan (0.28 per 100,000. In the west it is an extremely rare condition with an overall incidence of (0.086 per 100,000 in the Western United States. Etiology for the most part is unknown; however, genetic susceptibility related to RNF213 gene on chromosome 17q25.3 has been suggested. Moyamoya is being diagnosed more frequently in all races with varying clinical manifestations. Moyamoya disease is a rare progressive neurologic condition characterized by occlusion of the cerebral circulation with extensive collaterals recruitment in children and adults. Distinguished radiological findings confirm the diagnosis. Early recognition and swift institution of therapy is vital in order to minimize neurological deficits. We present the case of a 19-year-old African American female who presented with left-sided parastheia, weakness, and headache for 2 days duration.

  18. Cervical column morphology in adult patients with obstructive sleep apnoea.

    Science.gov (United States)

    Sonnesen, Liselotte; Petri, Niels; Kjaer, Inger; Svanholt, Palle

    2008-10-01

    Cervical column morphology was examined in adult patients with obstructive sleep apnoea (OSA) and compared with the cervical morphology of an adult control group with neutral occlusion, normal craniofacial morphology, and no history of sleep apnoea. The sleep apnoea group consisted of 91 patients, 16 females aged 29-59 years (mean 49.4 years) and 75 males aged 27-65 years (mean 49.0 years). All patients were diagnosed with OSA by overnight polysomnography. The control group consisted of 21 subjects, 15 females aged 23-40 years (mean 29.2 years) and 6 males aged 25-44 years (mean 32.8 years). From each individual, a visual assessment of the cervical column was performed on the radiograph. Differences in the cervical column morphology, between the genders and the groups were assessed by Fisher's exact test and the effect of age by logistic regression analysis. In the OSA group, 46.2 per cent had fusion anomalies of the cervical column and 5.5 per cent a posterior arch deficiency. Fusion anomalies occurred in 26.4 per cent as fusions between two cervical vertebrae. Block fusions occurred in 12.1 per cent and occipitalization in 14.3 per cent. A posterior arch deficiency occurred in 2.2 per cent as a partial cleft of C1 and in 3.3 per cent as dehiscence of C3 and C4. No statistical gender differences were found in the occurrence of morphological characteristics of the cervical column. The fusion anomalies of the cervical column occurred significantly more often in the OSA group. The results indicate that the morphological deviations of the upper cervical vertebrae play a role in the phenotypical subdivision and diagnosis of OSA.

  19. Adult growth hormone (GH)-deficient patients demonstrate heterogeneity between childhood onset and adult onset before and during human GH treatment. Adult Growth Hormone Deficiency Study Group

    DEFF Research Database (Denmark)

    Attanasio, A F; Lamberts, S W; Matranga, A M

    1997-01-01

    -deficient adult patients have been compared. The first 6 months comprised randomized, double-blind treatment with GH or placebo, then all patients were GH-treated for a further 12 months. At baseline the height, body weight, body mass index, lean body mass, and waist/hip ratio of AO patients were significantly (P...

  20. Clinical characteristics of intermediate uveitis in adult Turkish patients

    Institute of Scientific and Technical Information of China (English)

    Esra; Kardes; Betul; Ilkay; Sezgin; Akcay; Kansu; Bozkurt; Cihan; Unlu; Gurkan; Erdogan; Gulunay; Akcali

    2015-01-01

    ·AIM: To describe the clinical characteristics of Turkish patients with intermediate uveitis(IU) and to investigate the effect of clinical findings and complications on final visual acuity(VA).·METHODS: We retrospectively analyzed the medical records of patients with IU who had at least 6mo of follow-up and were older than 16 y.· RESULTS: A total of 78 eyes of 45 patients were included in the study and the mean follow-up period was19.4mo. The mean age at the time of presentation was42.9s. Systemic disease associations were found in17.7% of cases; sarcoidosis(8.8%) and multiple sclerosis(6.6%) were the most common diseases. Recurrence rate(odds ratio=45.53; 95%CI: 2.181-950.58), vitritis equals to or more than 3+ cells(odds ratio =57.456; 95%CI: 4.154-794.79) and presenting with VA less than 20/40(odds ratio =43.81; 95% CI: 2.184-878.71) were also found as high risk factors for poor final VA. At the last follow-up examination, 67.9% of eyes had VA of 20/40 or better.·CONCLUSION: IU is frequently seen at the beginning of the fourth decade of life. The disease is most commonly idiopathic in adult Turkish patients. Patients with severe vitritis at presentation and patients with frequent recurrences are at high risk for poor visual outcome.

  1. Osteoporosis in adult Sri Lankan inflammatory bowel disease patients

    Institute of Scientific and Technical Information of China (English)

    Arjuna Priyadarsin de Silva; Aranjan Lionel Karunanayake; Thalahitiya Gamaralalage Iruka Dissanayaka; Anuradha Supun Dassanayake; Hewa Kattadi Kankanamgae Tilak Duminda; Arunasalam Pathmeswaran; Ananda Rajitha Wickramasinghe; Hithanadura Janaka de Silva

    2009-01-01

    AIM: To determine if inflammatory bowel disease (IBD) is a risk factor for osteoporosis in adult Sri Lankans. METHODS: We identified eligible subjects from among consecutive patients diagnosed with IBD who attended our outpatient clinic. We included only patients aged between 20 and 70 years. Patients who were pregnant, had significant comorbidity, or were on calcium supplements or treatment for osteoporosis within the past 6 mo, were excluded. Healthy, age- and sex-matched controls were also recruited, in a control to patient ratio of 3:1. Both groups were screened for osteoporosis using peripheral dual energy X-ray absorptiometry scanning. RESULTS: The study population consisted of 111 IBD patients (male:female = 43:68; mean age 42.5 years) and 333 controls (male:female = 129:204; mean age 43.8 years). The occurrence of osteoporosis among IBD patients (13.5%) was significantly higher than among controls (4.5%) ( P = 0.001). The frequency of osteoporosis was not significantly different between ulcerative colitis (14.45%) and Crohn's disease (10.7%). However, on multivariate analysis, only age ( P = 0.001), menopause ( P = 0.024) and use of systemic steroids ( P < 0.001) were found to be associated independently with the occurrence of osteoporosis, while IBD, severity of disease, number of relapses, duration of illness or treatment other than systemic steroids were not. CONCLUSION: IBD does not appear to be an independent risk factor for the occurrence of osteoporosis in this population. However, the use of systemic steroids was a risk factor.

  2. CT perfusion assessment of Moyamoya syndrome before and after direct revascularization (superficial temporal artery to middle cerebral artery bypass)

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Yueqin [Hospital of Qingdao University, Department of Radiology, Qingdao (China); Hospital of Jining Medical College, CT Department, Jining (China); Xu, Wenjian [Hospital of Qingdao University, Department of Radiology, Qingdao (China); Guo, Xiang; Shi, Zhitao; Sun, Zhanguo; Wang, Jiehuan [Hospital of Jining Medical College, CT Department, Jining (China); Gao, Lingyun [Hospital of Jining Medical College, MR Department, Jining (China); Jin, Feng [Hospital of Jining Medical College, Department of Neurosurgery, Jining (China); Chen, Weijian; Yang, Yunjun [Hospital of Wenzhou Medical University, Department of Radiology, Wenzhou (China)

    2016-01-15

    To evaluate the utility of CT perfusion (CTP) for the assessment of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis in patients with Moyamoya syndrome (MMS). Twenty-four consecutive MMS patients, who underwent unilateral STA-MCA bypass surgery, received CTP before and after surgery. The relative perfusion parameter values of surgical hemispheres before treatment were compared with post-treatment values. All patients underwent CT angiography (CTA) before and after surgery in order to confirm the patency of bypass. The follow-up CTA after surgery clearly demonstrated 20 (20/24, 83.3 %) bypass arteries, whereas four (16.7 %) bypass arteries were occluded or very small. Postoperative rMTT and rTTP values (P < 0.05) of the surgical side were significantly lower than pre-operation. In patients (n = 20) with bypass patency, postoperative rCBF, rMTT and rTTP values (P < 0.05) of the surgical side were significantly improved. However, the differences of all parameters were not significant (P > 0.05) in the patients (n = 4) without bypass patency after revascularization. This study demonstrates that CTP can provide a crucial quantitative assessment of cerebral haemodynamic changes in MMS before and after STA-MCA anastomosis. (orig.)

  3. [Three Cases of Moyamoya Disease with a History of Kawasaki Disease].

    Science.gov (United States)

    Kawasaki, Toshinari; Arakawa, Yoshiki; Sugino, Toshiya; Mitsuhara, Takafumi; Funaki, Takeshi; Kikuchi, Takayuki; Koyanagi, Masaomi; Yoshida, Kazumichi; Kunieda, Takeharu; Takahashi, Jun C; Takagi, Yasushi; Miyamoto, Susumu

    2015-11-01

    Here, we report three cases of moyamoya disease with a history of Kawasaki disease. A 33-year-old man was found to have stenotic lesions of the internal carotid arteries(ICAs)on both sides at a nearby hospital where he visited complaining of headache and lisping. He had received immunoglobulin therapy for Kawasaki disease at the ages of 1, 2, and 6 years. MRI showed only a chronic ischemic lesion in the white matter. Angiography showed occlusion at the terminal portion of the ICAs on both sides. He was diagnosed with moyamoya disease, but as he had no symptoms and preserved cerebral blood flow (CBF), he was kept under observation. An 8-year-old boy was diagnosed with moyamoya disease and underwent right encephaloduroarteriosynangiosis at a nearby hospital. He had received immunoglobulin therapy for Kawasaki disease at the age of 1 year. His ischemic symptoms worsened. Although MRI detected no apparent ischemic lesion, angiography revealed severe stenosis at the terminal portions of the ICAs on both sides, and 123I-IMP SPECT showed CBF impairment. Bilateral direct bypass was performed. His father was subsequently also diagnosed with moyamoya disease. A 4-year-old girl with epilepsy was diagnosed with moyamoya disease at a nearby hospital. She had been treated with aspirin for Kawasaki disease at the age of 1 year. MRI detected no remarkable ischemic lesions, but angiography revealed mild stenosis at the terminal portions of the ICAs on both sides. Five months later, her ischemic symptoms were worsening with progressing stenotic lesions, and she underwent bilateral direct bypass.

  4. Orthodontics in the adult patient, with special reference to the periodontally compromised patient.

    Science.gov (United States)

    Johal, A; Ide, M

    1999-04-01

    There is increasing demand from adult patients for orthodontic treatment, either purely for aesthetics, to improve aesthetics or function following previous disease, or to facilitate the stabilization, restoration or replacement of teeth. Orthodontics may have a major role in the rehabilitation of patients suffering the effects of advanced periodontal disease, but there are a number of important factors to be considered in the management of such patients if the optimal outcome is to be obtained. This paper summarizes important aspects of treatment and the potential complications and how to avoid them.

  5. Enfermedad de Moyamoya y embarazo gemelar. Presentación de un caso y revisión de literatura

    Directory of Open Access Journals (Sweden)

    Airam Amoroso

    2012-03-01

    Full Text Available La enfermedad de Moyamoya es una vasculopatía oclusiva cerebral progresiva, caracterizada por estenosis u oclusión de la porción supraclinoidea de la arteria carótida interna, principalmente de la arteria cerebral media y de la arteria cerebral anterior. Su aspecto angiográfico semeja el humo de cigarrillo que en japonés significa “moyamoya”. Presentamos el caso de una paciente femenina de 28 años quien presentó cefalea y crisis epilépticas focales desde los 5 años de edad y embarazo gemelar de alto riesgo a los 16 años, que ameritó cesárea segmentaria; Panangiografía que revela imagen semejante a humo de cigarrillo consistente con Enfermedad de Moyamoya. Moyamoya disease and twin pregnancy. Case report and literature revision Moyamoya disease is a progressive cerebral occlusive vasculopathy characterized by stenosis or occlusion of the supraclinoid portion of the internal carotid artery, mainly the middle cerebral artery and anterior cerebral artery. The angiographic appearance resembles “cigarette’s smoke” which means in Japanese ";moyamoya";. We report the case of a 28 years old female who presented headache and focal seizures since 5 years old and a high-risk twin pregnancy at 16 years old, that required cesarean section; panangiography reveals a similar picture of “cigarette’s smoke” consistent with Moyamoya disease.

  6. Humidification during mechanical ventilation in the adult patient.

    Science.gov (United States)

    Al Ashry, Haitham S; Modrykamien, Ariel M

    2014-01-01

    Humidification of inhaled gases has been standard of care in mechanical ventilation for a long period of time. More than a century ago, a variety of reports described important airway damage by applying dry gases during artificial ventilation. Consequently, respiratory care providers have been utilizing external humidifiers to compensate for the lack of natural humidification mechanisms when the upper airway is bypassed. Particularly, active and passive humidification devices have rapidly evolved. Sophisticated systems composed of reservoirs, wires, heating devices, and other elements have become part of our usual armamentarium in the intensive care unit. Therefore, basic knowledge of the mechanisms of action of each of these devices, as well as their advantages and disadvantages, becomes a necessity for the respiratory care and intensive care practitioner. In this paper, we review current methods of airway humidification during invasive mechanical ventilation of adult patients. We describe a variety of devices and describe the eventual applications according to specific clinical conditions.

  7. Humidification during Mechanical Ventilation in the Adult Patient

    Directory of Open Access Journals (Sweden)

    Haitham S. Al Ashry

    2014-01-01

    Full Text Available Humidification of inhaled gases has been standard of care in mechanical ventilation for a long period of time. More than a century ago, a variety of reports described important airway damage by applying dry gases during artificial ventilation. Consequently, respiratory care providers have been utilizing external humidifiers to compensate for the lack of natural humidification mechanisms when the upper airway is bypassed. Particularly, active and passive humidification devices have rapidly evolved. Sophisticated systems composed of reservoirs, wires, heating devices, and other elements have become part of our usual armamentarium in the intensive care unit. Therefore, basic knowledge of the mechanisms of action of each of these devices, as well as their advantages and disadvantages, becomes a necessity for the respiratory care and intensive care practitioner. In this paper, we review current methods of airway humidification during invasive mechanical ventilation of adult patients. We describe a variety of devices and describe the eventual applications according to specific clinical conditions.

  8. EPIGLOTTIS MICROFLORA OF ADULT PATIENTS WITH ACUTE EPIGLOTTITIS

    Directory of Open Access Journals (Sweden)

    Golovko NA

    2016-12-01

    Full Text Available Introduction. Nowadays acute infectious-inflammatory processes of upper respiratory tract, including acute epiglottitis retain a high proportion among human pathology. In the literature acute epiglottitis is allocated into an independent nosology as severe acute phlegmonous bacterial inflammation of the epiglottis and hypopharynx. There are currently no clear guidelines on how to classify an acute epiglottitis, as well as protocols for patients at various stages of the pathological process. According to common belief, Haemophilus influenzae type -B (Haemophilus influenza type b (Hib is the most common cause of epiglottitis. At present, the main etiological role in the genesis of acute epiglottitis in children belongs to haemophilus influenzae. In adults the causes of the disease are beta hemolytic streptococci groups A, B, pneumococcus, Klebsiella, Pseudomonas, Staphylococcus aureus, herpes simplex virus (type 1 and parainfluenza, and others.The aim of this work is to study: the mucosal microflora of the epiglottis in adult patients with acute epiglottitis and to study sensitivity of certain isolates to antimicrobial agents. Material & methods. 86 adult patients with acute epiglottitis were observed: 36 with abscess form of epiglottitis and 50 - with infiltrative. Microbiological analysis of mucosal swab samples taken from hypopharynx were conducted by the conventional technology: for seeding solid or liquid nutrient medium, followed by allocation of isolith and its microscopic, biochemical and serological identification. Microorganisms were classified according to schemes of Bergy. Antimicrobial susceptibility of each strain was determined in accordance with the guidelines. We used discs with antibacterial drugs. The availability of sensitive and resistant strains of microorganisms to antibiotics was assessed. A mucous membrane of the epiglottis was analyzed through microbiological investigation in 86 patients with acute epiglottitis. As a

  9. Migraine pain location in adult patients from eastern India

    Directory of Open Access Journals (Sweden)

    Chakravarty Ambar

    2008-01-01

    Full Text Available Background: Sparse literature documenting the location of pain at the onset of migraine attacks and during established headaches is available. Objectives: A prospective study (2003-05 on 800 adult migraine patients (International Classifications of Headache Disorders (ICHD, 2:1.1, 1.2.1 and 1.6.1 was conducted to document (a sites of onset of pain and (b location of pain during established attacks (in> 50% occasions through semistructured interviews. Results: Demography: N = 800; M:F = 144:656 (1:4.56; age, 16-42 years (mean, 26 years; duration of migraine, 1-18 years (mean, 6.8 years. 87% of the subjects were ethnic Bengalis from the eastern Indian state of West Bengal, Calcutta being the capital city. Migraine types (on the basis of> 50% headache spells: N = 800; 1.1:668 (83.5%; 1.2.1:18 (2.25%; 1.6.1:114 (14.25%. Location of pain at onset: Unilateral onset was present in 41.38% of the patients; of these, 53.17% had eye pain; 8.16%, frontal pain and 38.67%, temporal pain. In 32.25% of the patients, bilateral/central location of pain, mostly bitemporal or at vertex was noted. Cervico-occipital pain onset was noted in 26.43% patients (predominantly occipital, 14.68%; predominantly cervical, 11.75%. Location of established headaches: In 47.4% of the patients, with unilateral ocular or temporal onset, pain remained at the same site. Pain became hemicranial in 32.9%. In most patients, unilateral frontal onset pain (55.5% became bilateral or holocranial. Most bilateral ocular (69.4% and temporal onset (69.7% pains remained at the same location. However, most bifrontal (55.6% and vertex onset (56.9% pains subsequently became holocranial. Most occipital pains at onset became holocranial (45.3%, but cervical pains subsequently became either hemicranial (38.3% or holocranial (36.2%. Conclusions: This study documents location of pain at the onset and during established headaches in migraine patients largely from a specific ethnic group. Migraine with

  10. Moyamoya病相关眼部症状的临床分析%Clinical analysis of ocular presentations in moyamoya disease

    Institute of Scientific and Technical Information of China (English)

    张朝贵; 万金城; 瞿昌华; 杨华; 王朝华

    2015-01-01

    目的:探讨与Moyamoya病相关的眼部临床表现、发生机制及其临床意义。方法回顾性分析37例有眼部症状的Moyamoya病患者的临床资料。结果37例有眼部症状的Moyamoya病患者,单纯以眼部症状首发者8例,眼部症状与其它神经系统症状同时出现的29例;患者眼部症状主要表现为:一过性黑朦、偏盲、视力下降甚至丧失、视野缺损、瞳孔直接/间接对光反射消失、瞳孔大小异常、上睑下垂、眼球运动障碍、眼球疼痛。结论 Moyamoya病眼部症状多样,神经科医生和眼科医生均应掌握Moyamoya病的临床表现及其特点,对于有眼部症状的患者应及时做全面的检查,以免漏诊和误诊。%OBJECTIVE To explore the clinical manifestations and mechanism of ocular symptoms related to moyamoya disease. METHODS The clinical information was analyzed retrospectively in 37 moyamoya disease. RE-SULTS There were 8 patients examined to simply perform ocular manifestations, while 29 patients had ocular mani-festations accompanied by other neurologic symptoms. These ocular signs included amaurosis fugax, hemianopia, de-cline even loss of the visual ability, visual field defect, pupil direct/indirect light reflex, pupil size abnormal, ptosis, ocular dyskinesia and eye pain. CONCLUSIONS The symptoms of moyamoya disease are multiple, which required neurologic physician and ophthalmologist be proficient in the onset characteristics and clinical manifestation. The patients must be diagnosed and treated as soon as possible to avoid misdiagnosis and missed diagnosis, especially for patients with ocular signs.

  11. Relationship between perceived sleep and polysomnography in older adult patients

    Science.gov (United States)

    dos Santos Silva, Mayra; Bazzana, Caroline Moreira; de Souza, Altay Lino; Ramos, Luiz Roberto; Tufik, Sergio; Lucchesi, Lígia M.; Lopes, Guiomar Silva

    2015-01-01

    Background and aims Aging is a multifactorial process that elicits changes in the duration and quality of sleep. Polysomnography is considered to be the standard examination for the analysis of sleep and consists of the simultaneous recording of selected physiological variables during sleep. Objective The objective of this study was to use polysomnography to compare sleep reported by senior citizens. Methods We selected 40 patients, both male and female, with ages ranging from 64 to 89 years from the Center for the Study of Aging at the Federal University of São Paulo. Patients answered questions about sleep on the Comprehensive Geriatric Assessment and underwent polysomnography. Results The results were compared, and agreement between perceived sleep and polysomnography was found in several areas. There was an association between difficulty sleeping and sleep onset latency (p=0.015), waking up at night with sleep onset latency (p=0.005), total sleep time with daytime sleepiness (0.005) and snoring (0.027), sleep efficiency with sleepiness (0.004), snoring (0.033) and pause in breathing (p=0.024), awakenings with snoring (p=0.012) and sleep apnea with pauses in breathing (p=0.001). Conclusion These results suggest that the older adult population have a good perception of their sleep. The questionnaires aimed at this population should be used as an alternative to polysomnography. PMID:26483948

  12. Experiences of adult patients hearing loss postlingually with Cochlear Implant

    Directory of Open Access Journals (Sweden)

    Teresa María Lizcano Tejado

    2013-09-01

    Full Text Available Hearing loss is a significant public health problem. The incidence is difficult to establish because of the lack of data in people under age three, but is estimated about 1 per thousand for severe and profound hearing loss.A cochlear implant (CI is a device that converts sounds into electrical energy that triggers a sensation of hearing. The IC is indicated in patients with severe bilateral sensorineural hearing loss with null or poor benefit use of hearing aids.The general objective of this project is to understand the experiences of adult patients with severe-profound sensorineural hearing loss with IC postlingually throughout the implementation process.A personal vision of those implemented will allow us to learn how to face the possibility to hear and interact with their environment, applying this information to improve health care provided to them and identifying those areas where such assistance should be improved. Also allow us to compare the initial expectations and have been achieved, creating realistic expectations for future candidates.For its development we have designed a qualitative study, based on the principles and procedures of grounded theory, semistructured interviews, participant observation and discussion groups.The data will be analyzed using the software Nudist ViVo 9.

  13. Staphylococcus aureus isolated from tonsillectomized adult patients with recurrent tonsillitis.

    Science.gov (United States)

    Katkowska, Marta; Garbacz, Katarzyna; Stromkowski, Józef

    2017-01-01

    The aim of this study was to analyze the prevalence and antibiotic resistance of Staphylococcus aureus strains from 118 tonsillectomized adults due to recurrent tonsillitis (RT). The study included strains isolated from the tonsillar surface prior to tonsillectomy, recovered from the tonsillar core at the time of surgery, and from the posterior throat 2-4 weeks after the procedure. Susceptibility of isolates to 19 antibiotics was tested in line with the Clinical and Laboratory Standards Institute recommendations. Irrespective of the stage, the most commonly isolated bacteria were gram-positive cocci, and among them S. aureus. The tonsillar core was the most common site of S. aureus isolation (30.5%), followed by the tonsillar surface (10.8%) and the posterior pharynx (5.9%). This difference turned out to be statistically significant (p aureus seems to be the most common pathogen isolated from patients tonsillectomized due to RT. Staphylococcal isolates associated with RT are present mostly within the tonsillar core and susceptible to most antibiotics. They are typically isolated from patients between 21 and 30 years of age. Tonsillectomy results in less frequent isolation of S. aureus strains.

  14. Surgical management of 143 patients with adult primary retroperitoneal tumor

    Institute of Scientific and Technical Information of China (English)

    Yuan-Hong Xu; Ke-Jian Guo; Ren-Xuan Guo; Chun-Lin Ge; Yu-Lin Tian; San-Guang He

    2007-01-01

    AIM: To analyze the surgical management of adult primary retroperitoneal tumors (APRT) and the factors influencing the outcome after operation.METHODS: Data of 143 cases of APRT from 1990 to 2003 in the First Affiliated Hospital of China Medical University were evaluated retrospectively.RESULTS: A total of 143 cases of APRT were treated surgically. Among them, 122 (85.3%) underwent complete resection, 16 (11.2%) incomplete resection,and 3 (3%) surgical biopsies. Twenty-nine (20.2%)underwent tumor resection plus multiple organ resections. Ninety-five malignant cases were followed up for 1 mo to 5 years. The 1-year, 3-year, and 5-year survival rates of the patients subject to complete resection was 94.9%, 76.6% and 34.3% and that of patients with incomplete resection was 80.4%, 6.7%,and 0%, respectively (P < 0.001). The Cox multi-various regression analysis showed the completeness of tumor,sex and histological type were associated closely with local recurrence.CONCLUSION: Sufficient preoperative preparation and complete tumor resection play important roles in reducing recurrence and improving survival.

  15. [Acute hepatitis in a patient with adult onset Still disease].

    Science.gov (United States)

    Gallo, M; Calvanese, A; Oscuro, F; Gallo, A; Caso, P; Annibale, E; Farinato, N

    1997-04-01

    Liver abnormalities in the course of Adult Onset Still's Disease (AOSD), both in form of hepatomegaly and elevation of hepatic enzymes, have been reported in up to three-quarts of the affected patients. These abnormalities may reflect disease activity or may be induced by drugs. Only in a few of this patients a liver biopsy was performed. However liver histology has shown, generally, non specific abnormalities or even normal pictures. We have recently observed a 47-year-old woman with a febrile illness started five months before, who after pertinent investigation was diagnosed as AOSD (according to criteria of Yamaguchi et al.). Apart from laboratory findings characteristic of an inflammatory disease, in absence of drug therapies the biochemical data showed raised levels of aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and aminoglutamil transferase. Serological tests for either viral hepatitis viruses (HAV, HBV, HCV) or other viruses were negative. Ultrasonographic examination of gallbladder and bile ducts did not find gallstones or other abnormalities. A liver biopsy was performed, which histopathologic examination showed moderate fatty methamorphosis with focal areas of hepatocellular swelling with minimal necrosis, mild Kuppfer cell hyperplasia, portal and sinusoidal infiltrates of mononuclear cells. This picture consisted with the diagnosis of an acute unspecific reactive hepatitis.

  16. Metabolic aspects of adult patients with nonalcoholic fatty liver disease

    Science.gov (United States)

    Abenavoli, Ludovico; Milic, Natasa; Di Renzo, Laura; Preveden, Tomislav; Medić-Stojanoska, Milica; De Lorenzo, Antonino

    2016-01-01

    Nonalcoholic fatty liver disease (NAFLD) is a major cause of chronic liver disease and it encompasses a spectrum from simple steatosis to steatohepatitis, fibrosis, or cirrhosis. The mechanisms involved in the occurrence of NAFLD and its progression are probably due to a metabolic profile expressed within the context of a genetic predisposition and is associated with a higher energy intake. The metabolic syndrome (MS) is a cluster of metabolic alterations associated with an increased risk for the development of cardiovascular diseases and diabetes. NAFLD patients have more than one feature of the MS, and now they are considered the hepatic components of the MS. Several scientific advances in understanding the association between NAFLD and MS have identified insulin resistance (IR) as the key aspect in the pathophysiology of both diseases. In the multi parallel hits theory of NAFLD pathogenesis, IR was described to be central in the predisposition of hepatocytes to be susceptible to other multiple pathogenetic factors. The recent knowledge gained from these advances can be applied clinically in the prevention and management of NAFLD and its associated metabolic changes. The present review analyses the current literature and highlights the new evidence on the metabolic aspects in the adult patients with NAFLD. PMID:27610012

  17. Patient knowledge and pulmonary medication adherence in adult patients with cystic fibrosis

    Science.gov (United States)

    Lin, Ann Hsu-An; Kendrick, Jennifer G; Wilcox, Pearce G; Quon, Bradley S

    2017-01-01

    Background and objectives Patient knowledge of lung function (ie, forced expiratory volume in 1 s [FEV1]% predicted) and the intended benefits of their prescribed pulmonary medications might play an important role in medication adherence, but this relationship has not been examined previously in patients with cystic fibrosis (CF). Methods All patients diagnosed with CF and without prior lung transplantation were invited to complete knowledge and self-reported medication adherence questionnaires during routine outpatient visits to the Adult CF Clinic, St Paul’s Hospital, Vancouver, Canada from June 2013 to August 2014. Results A total of 142 out of 167 (85%) consecutive adults attending CF clinic completed patient knowledge and medication adherence survey questionnaires. Sixty-four percent of the patients recalled their last FEV1% predicted value within 5%, and 70% knew the intended benefits of all their prescribed medications. Self-reported adherence rates were highest for inhaled antibiotics (81%), azithromycin (87%), and dornase alpha (76%) and lowest for hypertonic saline (47%). Individuals who knew their FEV1% predicted value within 5% were more likely to self-report adherence to dornase alpha (84% vs 62%, P=0.06) and inhaled antibiotics (88% vs 64%, P=0.06) compared to those who did not, but these associations were not statistically significant. There were no significant associations observed between patient knowledge of intended medication benefits and self-reported medication adherence. Conclusion Contrary to our hypothesis, disease- and treatment-related knowledge was not associated with self-reported medication adherence. This suggests other barriers to medication adherence should be targeted in future studies aiming to improve medication adherence in adults with CF.

  18. Asymmetric periflexural exanthema: A report in an adult patient

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    Zawar V

    2003-11-01

    Full Text Available Asymmetric periflexural exanthem (APE is a distinctive exanthem, probably viral in origin. It is largely a disease of childhood and is uncommon in adults. We report an adult man presenting with the typical clinical findings of APE.

  19. Clinical features of adult patients with Eisenmenger syndrome associated with different types of congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    陈果

    2013-01-01

    Objective To explore the clinical features and hemodynamics of adult patients with Eisenmenger syndrome in different types of congenital heart diseases (CHD) .Methods Patients with Eisenmenger syndrome with different types of CHD diagnosed by right heart

  20. 改良间接血管重建治疗缺血性烟雾病%Modified indirect revascularization for the treatment of ischemic moyamoya disease

    Institute of Scientific and Technical Information of China (English)

    路华; 宋海; 黄金; 杨智勇; 王进昆; 李向新; 王嘉沪

    2012-01-01

    目的 探讨改良脑-硬膜-动脉融合术(EDAS)治疗缺血性烟雾病的疗效.方法回顾性分析21例缺血性烟雾病病人的临床资料,其中单侧缺血性烟雾病6例,双侧缺血性烟雾病15例,病人经CTA、MRA或DSA检查证实.行双侧改良EDAS术12例,单侧改良EDAS术9例.结果 本组手术均顺利.硬脑膜内层病理检查提示:存在炎症反应15例,未见异常6例.术后出现硬脑膜下积液1例.16例病人随访3~12个月,无短暂性脑缺血发作和脑卒中发生;行DSA复查13例,颅底异常血管明显减少10例,无明显变化3例.结论 改良EDAS术安全、微创,治疗缺血性烟雾病近期效果良好,远期疗效有待于进一步随访.%Objective To discuss the therapeutic efficacy of modified encephaloduroarteriosynangiosis (EDAS) for the treatment of ischemic moyamoya disease. Methods Clinical data of 21 patients with ischemic moyamoya disease were analyzed retrospectively, including unilateral lesion in 6 cases and bilateral lesions in 15, which were verified by CTA, MRA or DSA. The bilateral modified EDAS was performed in 12 patients and unilateral modified EDAS in 9. Results The surgical procedures were all successful. The pathological examination of inner dural layers indicated inflammatory response in 15 cases and no abnormality in 6. Subdural effusion occurred in 1 case after surgery. Sixteen patients were followed up for 3 to 12 months, and no transient ischemic attack or cerebral stroke occurred. DSA reexamination were performed in 13 cases, and the abnormal vessels of skull base significantly reduced in 10 cases and no obvious change was seen in 3. Conclusions Modified EDAS is safe and minimally invasive, and its short-term effect is good for the ischemic moyamoya disease, but its long-term outcome needs to be observed further.

  1. Adult attachment status and psychological disorder: the predictive validity of adult attachment in violent, personality-disordered patients

    OpenAIRE

    McGauley, G. A.

    2011-01-01

    This thesis explores the role of attachment in a group of violent, personality-disordered patients in a high secure hospital. The research examines the mental representation of attachment and the perception of parenting, as assessed by the Adult Attachment Interview and the Parental Bonding Instrument, in this patient group and examines whether these differ from those of non-violent psychiatric patients. A prospective study examines whether the attachment measures predict change across a rang...

  2. Intensified microbiological investigations in adult patients admitted to hospital with lower respiratory tract infections

    DEFF Research Database (Denmark)

    Korsgaard, Jens; Rasmussen, TR; Sommer, T;

    2002-01-01

    September 1st 1997 to May 31st 1998 and were compared with a control group from the preceding year. A total of 67 adult patients were included in the study group and they were compared with 122 adult patients in the control group. The study group underwent fibre-optic bronchoscopy (FOB) with bronchoalveolar...

  3. Warming up Improves Speech Production in Patients with Adult Onset Myotonic Dystrophy

    Science.gov (United States)

    de Swart, B.J.M.; van Engelen, B.G.M.; Maassen, B.A.M.

    2007-01-01

    This investigation was conducted to study whether warming up decreases myotonia (muscle stiffness) during speech production or causes adverse effects due to fatigue or exhaustion caused by intensive speech activity in patients with adult onset myotonic dystrophy. Thirty patients with adult onset myotonic dystrophy (MD) and ten healthy controls…

  4. Laparoscopic Splenectomy in a Child with Moyamoya Syndrome, Hereditary Spherocytosis, and Interstitial Lung Disease: A Mere Coincidence or Partnership Based on Genetic Similarities

    Directory of Open Access Journals (Sweden)

    Kasra Karvandian

    2011-01-01

    Full Text Available A case of moyamoya syndrome and spherocytosis with concurrent interstitial lung disease who underwent laparoscopic splenectomy is being reported. A theory regarding their coexistence is being forwarded together with their anesthetic management. According to our search, this is the fourth case of moyamoya syndrome and the first case with an associated interstitial lung disease in a 10-year-old child.

  5. The Efficacy of Surgical Treatment for the Secondary Prevention of Stroke in Symptomatic Moyamoya Disease: A Meta-Analysis.

    Science.gov (United States)

    Qian, Cong; Yu, Xiaobo; Li, Jianru; Chen, Jingyin; Wang, Lin; Chen, Gao

    2015-12-01

    The treatment of moyamoya disease (MMD) is controversial and often depends on the doctor's experience. In addition, the choice of surgical procedure to treat MMD can differ in many ways. In this study, we performed a meta-analysis to determine whether surgical treatment of MMD is superior to conservative treatment and to provide evidence for the selection of an appropriate surgical treatment.The human case-control studies regarding the association of MMD treatment were systematically identified through online databases (PubMed, Web of Science, Elsevier Science Direct, and Springer Link). Inclusion and exclusion criteria were defined for the eligible studies. The fixed-effects model was performed when homogeneity was indicated. Alternatively, the random-effects model was utilized.This meta-analysis included 16 studies. Surgical treatment significantly reduced the risk of stroke (odds ratio (OR) of 0.17, 95% confidence interval (CI), 0.12-0.26, P direct bypass surgery, indirect bypass surgery had a lower efficacy on secondary stroke risk reduction (OR of 1.79, 95% CI, 1.14-2.82, P = 0.01), while no significant difference was detected for perioperative complications.Surgery is an effective treatment for symptomatic MMD patients, and direct bypass surgery may bring more benefits for these patients.

  6. Von Willebrand factor and coagulation factor VIII in Moyamoya disease associated with Graves' disease: A case report

    Science.gov (United States)

    Ren, Shou-Chen; Gao, Bao-Qin; Yang, Wei-Li; Feng, Wei-Xin; Xu, Jian; Li, Shao-Wu; Wang, Yong-Jun

    2016-01-01

    The present study reported the case of a Chinese boy who was diagnosed with Moyamoya disease (MMD) associated with Graves' disease (GD). An overactivation of von Willebrand factor (vWF) and coagulation factor VIII (FVIII) was identified in the plasma of the patient. Thiamazole and metoprolol treatment was thus administrated. After 2 months of treatment, the patient's thyroid function returned to normal and the neurological symptoms improved gradually. At the same time, the activities of vWF and FVIII were depressed. During the 20-month follow-up, information regarding the neurological symptoms, cerebrovascular imaging, thyroid function, thyroid autoantibodies and coagulation parameters was collected. High levels of thyroid autoantibodies persisted throughout the follow-up period, while other coagulation parameters remained in the normal range. In conclusion, considering the vital role of vWF and FVIII in vascular diseases, it is hypothesized that these two factors may serve an important role in the occurrence of GD associated with MMD. PMID:27882137

  7. Giant Primary Mature Retroperitoneal Teratomain in Adult Male Patient

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    R. Ebrahimian

    2015-07-01

    Full Text Available Introduction: Teratomas are congenital tumors consisting of derivatives from the ectoderm, endoderm and mesoderm germ cell layers. A teratoma is considered to be a non-seminomatous germ cell tumor and is typically located in either the sacrococcygeal region or in the gonads. Giant retroperitoneal teratomas in adults are even rarer, with only a few cases previously described in the literature. Case report: A 35-year-old male patient with severe nausea and vomiting was taken to the emergency ward of Hamadan Be’sat Hospital. He had not been feeling well, and had suffering from abdominal pain for a month. A physical examination showed some concretion in the right side of his abdomen. A CT scan of his abdomen and pelvis with IV and oral contrast re-vealed that the concretion was formed by aggregates of solid, cystic, and calcareous compo-nents. It compressed stomach and caused the rotation of the stomach around its longitudinal axis. Conclusion: Following the diagnosis, we performed a laparotomy and respected a concretion with dimension 20?25?22cm. Interestingly, we found out all mature tissues within the con-cretion in the pathology examination of an adequate sample (such as trachea, bone, GI lu-men…. (Sci J Hamadan Univ Med Sci 2015; 22 (2: 165-169

  8. Localized Tetanus in an Adult Patient: Case Report

    Science.gov (United States)

    Gulamhussein, Mohamed Amirali; Li, Yueyang; Guha, Abhijit

    2016-01-01

    Introduction: Tetanus is a severe and potentially fatal infection caused by the bacterium Clostridium tetani. Of all the cases described in literature, generalized tetanus is by far the most common presentation, but it may also present as neonatal tetanus, cephalic tetanus, and localized tetanus, the latter two being much rarer. In this case report, we present the rare form of this disease, i.e., localized tetanus in an adult male with a history of minimal trauma as well as a late, unusual mode of presentation. Case Report: A 35-year-old Caucasian male presented with an acutely painful, swollen right thumb associated with a small superficial collection on the dorsal aspect of the base of the thumb. A formal wound exploration and washout were carried out in theater, however, at the time of tourniquet inflation, the right hand went into a carpopedal spasm and remained in that position until an infusion of a muscle relaxant was given. The findings were consistent with a case of localized tetanus. The patient was treated with human immunoglobulin and tetanus toxoid and safely discharged home 48 h later without any complications. Conclusion: This case report emphasizes the importance of the recognition of a rare form of this fatal infectious disease, which may present with prodromal symptoms before the generalized form shows its clinical effects. Moreover, the astute clinician should be aware of the variable presentations of this infectious disease, with early identification greatly reducing the associated risks of morbidity and mortality. PMID:28164065

  9. HETEROTOPIC OSSIFICATION OF HIP IN A RARE CASE OF MOYAMOYA DISEASE: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Anita

    2015-04-01

    Full Text Available A case of extensive ossification around the left hip joint involving lesser trochanter of the femur leading to ankylosis of left hip joint in a 60 years male is being reported. The diagnosis of moyamoya disease was made , which is a rare form of occlusive cerebrovasc ular disorder. Occlusion of an artery may present with Transient Ischemic Attacks , headaches , stroke and seizures. Surgical management is the only option in these cases. This 60 years male had an acute onset hemiplegia 4 ½ years back and at present came wit h complaints of pain and swelling over the left hip. Surgical excision was done and histopathological examination revealed extensive ossification of skeletal muscle. A diagnosis of Moyamoya disease complicated with heterotopic ossification was made. There was no recurrence after 8 months of follow up.

  10. Role of L-asparaginase in acute lymphoblastic leukemia: focus on adult patients

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    Rytting ME

    2012-06-01

    Full Text Available Michael E RyttingDepartment of Pediatrics and Leukemia, University of Texas MD Anderson Cancer Center, Houston, TX, USAAbstract: Asparaginase preparations deplete asparagine in acute lymphoblastic leukemia (ALL blasts. Asparaginase in its various forms is an important component of treatment regimens for pediatric ALL. Recently, interest and use of asparaginase in adult patients with ALL has increased, particularly in young adults. There is much less information on asparaginase use and toxicity in adult compared with pediatric populations. This review surveys prior published studies of the three most commonly used asparagine preparations as used in adult patients, and discusses important toxicities encountered in adult patients who receive asparaginase preparations.Keywords: asparaginase, leukemia, adults, children

  11. 颅骨多处钻孔硬膜翻转并骨膜贴敷术治疗烟雾病%Therapeutic effect of multiple burr - hole operation combined with dural inversion and periosteal synangiosis for moyamoya disease

    Institute of Scientific and Technical Information of China (English)

    赵旭; 王志刚; 李卫国; 曲春城; 孟庆虎; 王成伟

    2012-01-01

    目的 初步探讨应用颅骨多处钻孔硬膜翻转并骨膜贴敷术治疗烟雾病的疗效.方法 对25例烟雾病患者行颅骨多处钻孔硬膜翻转并骨膜贴敷术,术后随访3个月-3年.结果 20例缺血型患者术前原有症状术后均好转,5例出血型患者术后未发生再出血.9例患者行DSA复查,造影结果显示38个骨孔均有新生血管生长,缺血区血运改善.手术并发症包括硬膜下血肿1例、暂时性神经功能障碍5例、发热1例.结论 单独应用颅骨多处钻孔硬膜翻转并骨膜贴敷术治疗烟雾病是简单、安全、有效的.%Objective To explore the therapeutic effect of multiple burr - hole operation combined with dural inversion and periosteal synangiosis for moyamoya disease.Methods The multiple burr - hole operation combined with dural inversion and periosteal synangiosis was performed on 25 patients with moyamoya disease,which were followed up for three months to three years.Results The clinical symptoms of the 20 patients with ischemic moyamoya disease gradually improved after the operations.There was no postoperative rebleeding in the 5 patients with hemorrhagic moyamoya disease.Nine patients were reexamined by digital subtraction angiogram (DSA).The postoperative DSA results revealed there was apparent neovascularization in all the 38 burr - holes and the revascularization of the ischemic cerebral tissue was obtained. The postoperative complications.included one case of subdural hematoma,5 cases of postoperative temporary neurological deficits and 1 case of fever.Conclusion Multiple burr- hole operation combined with dural inversion and periosteal synangiosis is a simple,safe and effective treatment method for movamova disease as a sole treatment without supplementary revascularization procedures.

  12. Appreciation of music in adult patients with cochlear implants: a patient questionnaire.

    Science.gov (United States)

    Mirza, S; Douglas, S A; Lindsey, P; Hildreth, T; Hawthorne, M

    2003-06-01

    Many cochlear implant candidates express hopes of enjoying music following implantation. Our aim was to assess the appreciation of music after cochlear implantation in adult patients. Thirty-five out of 45 cochlear implantees (78%) from the North East Programme responded to a questionnaire. Only 16 out of 35 patients (46%) listened to music after implantation. Enjoyment of music on a self-assessment scale was graded a mean of 8.7/10 before deafness but only 2.6/10 after implantation. Listening to music after implantation was more likely in younger patients, those with higher speech perception scores and those with a shorter length of deafness, but was not found to be related to gender, type of implant, processing strategy, time since implant or music enjoyment before becoming deaf. Appreciation of music after cochlear implantation is disappointingly low. Future developments in implant technology should strive to improve satisfaction with music listening.

  13. [Angiographic moyamoya in a female with glycogenosis type IA - a case report focusing therapeutic management: bilateral encephalo-duro-arterio-myo-synangiosis (EDAMS)].

    Science.gov (United States)

    Weiss, C; Witt, T N; Fesl, G; Geisler, J; Tonn, J C

    2010-03-01

    Angiographic Moyamoya is a rare cerebrovascular disease most frequent in asia. Its characateristics are recurrent ischemic attacks due to progressive occlusion of ICA branches. Angiography reveals fine arterial collateralisation reminding of ascending smoke ("moyamoya" in japanese). Neurosurgical treatment strategies include direct and indirect reanastomosation procedures. Randomised trials for comparison of clinical outcome and long term survival remain missing. A 23 years old female with glycogenosis type IA was first diagnosed bilateral angiographic moyamoya with bilateral proximal stenosis of ICA after transient ischemic attack (TIA). Coincidence of both rare diseases moyamoya and glycogenosis has previously been reported in three cases, so that this metabolic dysfunction presumably is a true risk factor for moyamoya. In our case, excellent angiographic and functional results were achieved by bilateral, consecutive Enzephalo-Duro-Arterio-Myo-Synangiosis (EDAMS).

  14. Latanoprost systemic exposure in pediatric and adult patients with glaucoma

    DEFF Research Database (Denmark)

    Raber, Susan; Courtney, Rachel; Maeda-Chubachi, Tomoko;

    2011-01-01

    To evaluate short-term safety and steady-state systemic pharmacokinetics (PK) of latanoprost acid in pediatric subjects with glaucoma or ocular hypertension who received the adult latanoprost dose.......To evaluate short-term safety and steady-state systemic pharmacokinetics (PK) of latanoprost acid in pediatric subjects with glaucoma or ocular hypertension who received the adult latanoprost dose....

  15. Experimental study of the pathogenesis of moyamoya disease: histological changes in the arterial wall caused by immunological reactions in monkeys.

    Directory of Open Access Journals (Sweden)

    Terai Y

    2003-10-01

    Full Text Available Moyamoya disease is a progressive vascular disorder of unknown etiology. Theories of inflammatory and immunologic mechanisms have been proposed as the pathogeneses. We have designed a new method of administering N-acetylmuramyl-L-alanyl-D-isoglutamine (MDP for experimental induction of moyamoya disease using an intravascular interventional technique combined with rod-shaped embolic materials made from lactic acid-glycolic acid copolymer. The embolic materials containing MDP were repeatedly injected into the right internal carotid artery of monkeys in the embolic group. Intravenous injections of MDP solution alone were performed in the intravenous group. Histological examination of the arteries demonstrated reduplication and lamination of the internal elastic laminae, which corresponded with findings of moyamoya disease in both groups. These histological changes occurred not only in the intracranial arteries on the embolization side, but also in the contralateral intracranial and even extracranial arteries. The changes were more prominent in the intravenous group than in the embolic group. We conclude that the systemic humoral factors induced by MDP in this study may be important in the pathogeneses of moyamoya disease. Our observations suggest that moyamoya disease is a systemic vascular disease and has an etiologic factor affecting both intracranial and extracranial arteries

  16. Motivation of adult female patients seeking orthodontic treatment: an application of Q-methodology

    Directory of Open Access Journals (Sweden)

    Tang X

    2015-02-01

    Full Text Available Xia Tang,1 Jiaxin Cai,1 Beibei Lin,1 Linjie Yao,2 Feiou Lin3 1School of Stomatology, 2Department of Pedodontics, 3Department of Orthodontics, School of Stomatology, Wenzhou Medical University, Lu Cheng District, People’s Republic of China Background: Motivation is the impetus for patients to seek orthodontic treatment, affecting adherence, treatment outcomes, and satisfaction. The aim of this study was to assess the motivation of adult female patients seeking orthodontic treatment, and classify the patients according to their motivations.Methods: This study used Q-methodology as the main tool. Q-samples were collected and categorized (35 items. Forty-two adult female patients were interviewed before treatment as the P-sample, and their responses were categorized into the Q-methodology grid. Participants were asked to rank-order a set of 35 statements (Q-sample from “agree most” to “disagree most” (Q-sorting. The finished Q-grids were analyzed using PQ method 2.35.Results: Four main factors were identified based on how adult female patients ranked statements: factor 1, patients who focus on their self-perception of their appearance; factor 2, patients who are concerned about the esthetics and function of their teeth; factor 3, patients who are easily influenced by others; factor 4, patients who want to improve their confidence and avoid negative thoughts caused by their teeth. The remaining patients who had other views did not match any of the above four groups.Conclusion: The motivations of adult female patients seeking orthodontic treatment are complex. This study found that most adult female patients fell into one of four typical factor groups. Our findings may improve the adherence of adult female patients by developing a more ideal treatment program. Keywords: adult female patients, orthodontic treatment, Q-methodology

  17. First autopsy analysis of a neovascularized arterial network induced by indirect bypass surgery for moyamoya disease: case report.

    Science.gov (United States)

    Mukawa, Maki; Nariai, Tadashi; Inaji, Motoki; Tamada, Natsumi; Maehara, Taketoshi; Matsushima, Yoshiharu; Ohno, Kikuo; Negi, Mariko; Kobayashi, Daisuke

    2016-05-01

    The object of this study was to analyze the pathology of collateral vessels newly induced by indirect bypass surgery for moyamoya disease (MMD). An autopsy analysis was conducted on a 39-year-old woman with MMD who had died of a brainstem infarction. The patient had undergone bilateral indirect bypass surgeries 22 years earlier. Sufficient revascularization via bilateral external carotid arterial systems was confirmed by cerebral angiography before her death. Macroscopic observation of the operative areas revealed countless meandering vessels on the internal surface of the dura mater connected with small vessels on the brain surface and in the subpial brain tissue. Notably, microscopic analysis of these vessels revealed the characteristic 3-layer structure of an arterial wall. This autopsy analysis was the first to confirm that indirect bypass surgery had induced the formation of a new arterial network (arteriogenesis) and that this network had been maintained for more than 20 years to compensate for the chronic cerebral ischemia caused by the MMD.

  18. Mandibular fractures: a comparative analysis between young and adult patients in the southeast region of Turkey

    Directory of Open Access Journals (Sweden)

    Serhat Atilgan

    2010-02-01

    Full Text Available OBJECTIVE: The purpose of this study was to review and compare the differences between mandibular fractures in young and adult patients. MATERIAL AND METHODS: Patients treated at the Oral and Maxillofacial Department of Dicle University during a five-year period between 2000 and 2005 were retrospectively evaluated with respect to age groups, gender, etiology, localization and type of fractures, treatment methods and complications. RESULTS: 532 patients were included in the study, 370 (70% males and 162 (30% females, with a total of 744 mandibular fractures. The mean age of young patients was 10, with a male-female ratio of 2:1. The mean age of adult patients was 28, with a male-female ratio of 3:1. The most common causes of injury were falls (65% in young patients and traffic accidents (38% in adults. The most common fracture sites were the symphysis (35% and condyle (36% in young patients, and the symphysis in adults (36%. Mandibular fractures were generally treated by arch bar and maxillomandibular fixation in both young (67% and adult (39% patients, and 43% of the adult patients were treated by open reduction and internal fixation. CONCLUSION: There was a similar gender, monthly and type of treatment distribution in both young and adult patients in the southeast region of Turkey. However, there were differences regarding age, etiology and fracture site. These findings between young and adult patients are broadly similar to those from other studies. Analysis of small differences may be an important factor in assessing educational and socioeconomic environments.

  19. Anxiety and depression in adult patients with celiac disease on a gluten-free diet

    Institute of Scientific and Technical Information of China (English)

    Winfried; Huser; Karl-Heinz; Janke; Bodo; Klump; Michael; Gregor; Andreas; Hinz

    2010-01-01

    AIM: To compare anxiety and depression levels in adult patients with celiac disease (CD) on a gluten-free diet (GFD) with controls.METHODS: The levels of anxiety, depression and of a probable anxiety or depressive disorder were assessed by the Hospital Anxiety and Depression Scale in 441 adult patients with CD recruited by the German Celiac Society, in 235 age-and sex-matched patients with inflammatory bowel disease (IBD) in remission or with slight disease activity, and in 441 adult persons of a representa...

  20. Patient-reported outcomes in adult survivors with single-ventricle physiology

    DEFF Research Database (Denmark)

    Overgaard, Dorthe; Schrader, Anne-Marie; Lisby, Karen H

    2011-01-01

    Objectives: Data on patient-reported outcomes (PROs) in patients with single-ventricle physiology (SVP) are scarce. We sought (1) to describe the perceived health status, quality of life, symptoms of anxiety and depression, and sense of coherence in adult survivors with SVP, (2) to compare PROs...... across functional classes, and (3) to compare PROs between patients and controls. Methods: A case-control study in two adult congenital heart programmes with 62 adult survivors with SVP were matched to 172 healthy controls. A wide range of PROs were measured using validated questionnaires. The treating...

  1. Molecular analysis of RNF213 gene for moyamoya disease in the Chinese Han population.

    Directory of Open Access Journals (Sweden)

    Zhiyuan Wu

    Full Text Available BACKGROUND: Moyamoya disease (MMD is an uncommon cerebrovascular disorder characterized by progressive occlusion of the internal carotid artery causing cerebral ischemia and hemorrhage. Genetic factors in the etiology and pathogenesis of MMD are being increasingly recognized. Previous studies have shown that the RNF213 gene was related to MMD susceptibility in the Japanese population. However, there is no large scale study of the association between this gene and MMD in the Chinese Han population. Thus we designed this case-control study to validate the R4810K mutation and to define the further spectrum of RNF213 mutations in Han Chinese. METHODOLOGY/PRINCIPAL FINDINGS: Genotyping of the R4810K mutation in the RNF213 gene was performed in 170 MMD cases and 507 controls from a Chinese Han population. The R4810K mutation was identified in 22 of 170 MMD cases (13%, including 21 heterozygotes and a single familial homozygote. Two of the 507 controls (0.4% were heterozygous R4810K carriers. The R4810K mutation greatly increased the risk for MMD (OR = 36.7, 95% CI: 8.6~156.6, P = 6.1 E-15. The allele frequency of R4810K was significantly different between patients with ischemia and hemorrhage (OR = 5.4, 95% CI: 1.8~16.1, P = 0.001. Genomic sequencing covering RNF213 exon 40 to exon 68 also identified eight other non-R4810K variants; P4007R, Q4367L, A4399T, T4586P, L4631V, E4950D, A5021V and M5136I. Among them A4399T polymorphism was found in 28/170 cases (16.5% and 45/507 controls (8.9% and was associated with MMD (OR = 2.0, 95% CI: 1.2~3.3, P = 0.004, especially with hemorrhage (OR = 2.8, 95% CI: 1.2~6.5, P = 0.014. CONCLUSIONS: RNF213 mutations are associated with MMD susceptibility in Han Chinese. The ischemic type MMD is particularly related to the R4810K mutation. However, A4399T is also a susceptible variant for MMD, primarily associated with hemorrhage. Identification of novel variants in the RNF213 gene further highlights the genetic

  2. Relationship between perceived sleep and polysomnography in older adult patients

    Directory of Open Access Journals (Sweden)

    Mayra dos Santos Silva

    2015-04-01

    Conclusion: These results suggest that the older adult population have a good perception of their sleep. The questionnaires aimed at this population should be used as an alternative to polysomnography.

  3. Loss of Olfactory Function and Nutritional Status in Vital Older Adults and Geriatric Patients

    NARCIS (Netherlands)

    Toussaint, N.; Roon, de M.; Campen, van J.P.C.M.; Kremer, S.; Boesveldt, S.

    2015-01-01

    The aim of this cross-sectional study was to assess the association of olfactory function and nutritional status in vital older adults and geriatric patients. Three hundred forty-five vital (mean age 67.1 years) and 138 geriatric older adults (mean age 80.9 years) were included. Nutritional status w

  4. Seizure detection in adult ICU patients based on changes in EEG synchronization likelihood

    NARCIS (Netherlands)

    Slooter, A. J. C.; Vriens, E. M.; Spijkstra, J. J.; Girbes, A. R. J.; van Huffelen, A. C.; Stam, C. J.

    2006-01-01

    Introduction: Seizures are common in Intensive Care Unit (ICU) patients, and may increase neuronal injury. Purpose: To explore the possible value of synchronization likelihood (SL) for the automatic detection of seizures in adult ICU patients. Methods: We included EEGs from ICU patients with a varie

  5. Oral Impacts on Quality of Life in Adult Patients with Class I, II and III Malocclusion

    OpenAIRE

    Javed, Omair; Bernabé, Eduardo

    2016-01-01

    PURPOSE: To compare the social impact of malocclusion on quality of life between adult patients with Angle Class I, II and III malocclusion.MATERIALS AND METHODS: A total of 222 adult patients (139, 42 and 41 with Angle Class I, II and III malocclusion, respectively) were recruited voluntarily from those attending the Orthodontic Clinic of Khyber College of Dentistry in Pesh awar, Pakistan. Participants were asked to complete the Urdu version of the short form of the Oral Health Impact Profil...

  6. Cardiovascular effects of growth hormone in adult hemodialysis patients: results from a randomized controlled trial

    DEFF Research Database (Denmark)

    Køber, Lars; Rustom, Rana; Wiedmann, Jonas;

    2010-01-01

    The high morbidity and mortality rates in hemodialysis (HD) patients are due, at least in part, to their increased risk for cardiovascular diseases (CVD). This prospective study evaluated the effect of growth hormone (GH) on a number of CVD risk markers in adult patients on HD.......The high morbidity and mortality rates in hemodialysis (HD) patients are due, at least in part, to their increased risk for cardiovascular diseases (CVD). This prospective study evaluated the effect of growth hormone (GH) on a number of CVD risk markers in adult patients on HD....

  7. The adult cystic fibrosis patient with abdominal pain: what the radiologist needs to know

    Energy Technology Data Exchange (ETDEWEB)

    Liong, S.Y.; Awad, D. [Department of Radiology, University Hospital of South Manchester, Wythenshawe Hospital, Manchester (United Kingdom); Jones, A.M. [Department of Respiratory Medicine, University Hospital of South Manchester, Wythenshawe Hospital, Manchester (United Kingdom); Manchester Academic Health Science Centre, University of Manchester, Manchester (United Kingdom); Sukumar, S.A., E-mail: Sathi.Sukumar@uhsm.nhs.u [Department of Radiology, University Hospital of South Manchester, Wythenshawe Hospital, Manchester (United Kingdom)

    2011-02-15

    As the life expectancy of cystic fibrosis (CF) patients continues to increase, abdominal manifestations of CF are increasingly being encountered by clinicians and radiologists. Imaging plays an important role in the evaluation of adult CF patients with abdominal pain as a cause is often not discernable clinically. Accurate diagnosis is crucial in these patients as some causes may be managed conservatively, whilst others may require surgical intervention. In this review, we describe clinical presentation, imaging findings, and management of adult CF patients presenting with abdominal pain.

  8. Structural myocardial involvement in adult patients with type 1 myotonic dystrophy

    Directory of Open Access Journals (Sweden)

    Upinder K. Dhand

    2013-03-01

    Full Text Available Myotonic dystrophy type 1 (DM1 is the commonest muscular dystrophy in adults, affecting multiple organs in addition to skeletal muscles. Cardiac conduction system abnormalities are well recognized as an important component of DM1 phenotype; however, primary structural myocardial abnormalities, which may predispose these patients to congestive heart failure, are not as well characterized. We reviewed the retrospective analysis of the clinical and echocardiographic findings in adult patients with DM1. Among 27 patients (16 male; age 19-61 years with DM1, the echocardiogram (ECHO was abnormal in 10 (37% including one of 6 patients (16% with congenital myotonic dystrophy. Reduced left ventricular ejection fraction (LVEF ≤50% was noted in 5, diastolic dysfunction in 4, left atrial dilatation in 3, left ventricular hypertrophy in 2, apical hypokinesia in 1 and mitral valve prolapse in 3 patients. One patient had paradoxical septal movement in the setting of left bundle branch block. Echocardiographic abnormalities significantly correlated with older age; however, patients with systolic dysfunction on echocardiogram ranged in age from 27 to 52 years including 2 patients aged 27 and 34 years. We can conclude that echocardiographic abnormalities are frequent in adult patients with DM1. The incidence is similar in the classical and congenital type of DM1. Overall, echocardiographic abnormalities in DM1 correlate with increasing age; however, reduced LVEF is observed even at young age. Cardiac assessment and monitoring in adult patients with DM1 should include evaluation for primary myocardial involvement.

  9. Influence of Marital Status on the Quality of Life of Chinese Adult Patients with Epilepsy

    Science.gov (United States)

    Wang, Fu-Li; Gu, Xiang-Min; Hao, Bao-Yun; Wang, Shan; Chen, Ze-Jie; Ding, Cheng-Yun

    2017-01-01

    Background: Epilepsy is a chronic disorder characterized by recurrent seizures and has significant psychological and social consequence for everyday living. Epilepsy affects various aspects of ones’ social life. The present study aimed to investigate the influence of marital status on the quality of life of adult Chinese patients with epilepsy. Methods: This study surveyed 805 Chinese adults who have been clinically diagnosed with epilepsy for longer than 1 year in 11 hospitals in Beijing. In this survey, 532 (66.1%) participants were married. All of them completed the case report form with enquiries on demographic data, social factors, and illness. The marriage status of adult epileptic quality of life was the dependent variable, and demographic data and clinical data were independent variables, analyzed through the multiple linear regression analysis methods. The patients’ quality of life was assessed using the Quality of Life in patients with Epilepsy-31 items (QOLIE-31) questionnaire, the Patient Health Questionnaire-9 items (PHQ-9), and the Generalized Anxiety Disorder-7 items (GAD-7). Results: The PHQ-9 and GAD-7 scores in the unmarried group (PHQ-9 = 6.0 and GAD-7 = 5.0) were significantly higher than that of the married group (PHQ-9 = 4.0 and GAD-7 =3.0). The scores of married adult patients with epilepsy on QOLIE (61.8 ± 15.3) and social function (70.9 ± 22.7) were higher than the scores of the unmarried patients aged between 20 and 44 years. The scores of married adult epileptics on the QOLIE (58.4 ± 14.6) and the energy/fatigue (62.1 ± 20.4) were higher than the scores of the unmarried patients (QOLIE = 58.4 ± 14.6 and the energy/fatigue = 62.1 ± 20.4) aged between 45 and 59 years. For the adult epilepsy patients, depression, anxiety, seizures within the last year, disease course, medical expense category, and marriage* age are negatively correlated with the quality of life. Occupation, educational level, and average monthly income are closely

  10. Association of apolipoprotein E gene polymorphism with small-vessel lesions and stroke type in moyamoya disease: a preliminary study.

    Science.gov (United States)

    Jang, Dong-Kyu; Huh, Pil Woo; Lee, Kwan-Sung

    2016-06-01

    OBJECT The present study was conducted to investigate whether microbleeds or microinfarcts are associated with apolipoprotein E (APOE) gene polymorphisms in patients with moyamoya disease (MMD), and if so, whetherAPOE gene polymorphisms are also associated with stroke type in patients with MMD. METHODS This cross-sectional, multicenter study included 86 consecutive patients with MMD who underwent T2*-weighted gradient echo or susceptibility-weighted MR imaging and 83 healthy control volunteers. Baseline clinical and radiological characteristics were recorded at diagnosis, and inter- and intragroup differences in the APOE genotypes were assessed. Multivariate binary logistic regression models were used to determine the association factors for small-vessel lesions (SVLs) and hemorrhagic presentation in patients with MMD. RESULTS There was no difference in APOE gene polymorphism and the incidence of SVLs between patients with MMD and healthy controls (p > 0.05). In the MMD group, 7 (8.1%) patients had microbleeds and 32 (37.2%) patients had microinfarcts. Microbleeds were more frequently identified in patients with hemorrhagic-type than in nonhemorrhagictype MMD (p = 0.003). APOE genotypes differed according to the presence of microbleeds (p = 0.024). APOE ε2 or ε4 carriers also experienced microbleeds more frequently than APOE ε3/ε3 carriers (p = 0.013). In the multivariate regression analysis in patients with MMD, microbleeds were significantly related to APOE ε2 or ε4 carrier status (OR 7.86; 95% CI1.20-51.62; p = 0.032) and cerebral aneurysm (OR 17.31; 95% CI 2.09-143.57; p = 0.008). Microinfarcts were independently associated with hypertension (OR 3.01; 95% CI 1.05-7.86; p = 0.007). Hemorrhagic presentation was markedly associated with microbleeds (OR 10.63; 95% CI 1.11-102.0; p = 0.041). CONCLUSIONS These preliminary results did not show a difference in APOE gene polymorphisms between patients with MMD and healthy persons. However, they imply that APOE

  11. Comparison of biological characteristics of marrow mesenchymal stem cells in hepatitis B patients and normal adults

    Institute of Scientific and Technical Information of China (English)

    Liang Peng; Hua Li; Lin Gu; Xiao-Mou Peng; Yang-Su Huang; Zhi-Liang Gao

    2007-01-01

    AIM: To establish a culture system of marrow mesenchymal stem cells (MSCs) from hepatitis B patients and normal adults and to compare their biological characteristics.METHODS: MSCs were isolated from bone marrow in 34 male hepatitis B patients and 15 male normal adults and cultivated in vitro. Their biological characteristics including surface markers, shapes and appearances, growth curves, first passage time and passage generations were compared.RESULTS: Cultivation achievement ratio of hepatitis B patients was lower than that of normal adults, no statistical significance (82.35% vs 100%, P >0.05). Compared with MSCs of normal adults, MSCs of hepatitis B patients presented a statistical lower growth curve, longer first passage time (13.0 ± 1.6 d vs 11.4 ± 1.5 d, P < 0.05), fewer passaging generation numbers (10.5 ± 1.4 generations vs 12.3±1.7 generations, P < 0.05), though both shared same appearances, shapes and surface markers. MSCs in hepatitis B patients would expand, spread out and age more easily and there were more refractive particles in the cytoplasm.CONCLUSION: MSCs from hepatitis B patients can be cultured in vitro. Although their appearance, shape and surface marker are similar to those of MSCs from normal adults, there are differences in their biological characteristics.

  12. Enfermedad de Moyamoya: Reporte de caso y revisión de la literatura

    OpenAIRE

    Ruiz, Víctor Hugo; Hoyos Pulgarín, Julián Andrés

    2012-01-01

    Mujer de 46 años que ingreso por cefalea súbita, hemiparesia izquierda y disartria, con tomografía axial computarizada cerebral simple que mostró hemorragia frontoparietal derecha drenada a ventrículos y arteriografía con patrón Moyamoya. Reingreso 3 meses después con deterioro del estado de consciencia y tomografía axial computarizada cerebral simple que mostró hemorragia intraparenquimatosa temporoparietal izquierda con extensión intraventricular y desviación de la línea media, se manejo en...

  13. Neoplastic Disorders in 100 Patients with Adult Celiac Disease

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    HUGH J Freeman

    1996-01-01

    Full Text Available Previous reports have suggested that the incidence of some neoplastic disorders, particularly malignant lymphoma, is increased in patients with celiac disease. In this study, the type and number of neoplastic disorders detected in 100 consecutive celiac disease patients were explored. Sixty-five patients were initially diagnosed with celiac disease before, and 35 after, age 60 years. Ten elderly celiac patients had lymphoma or small intestinal adenocarcinoma. Although the overall incidence of malignant lymphoma was 8%, similar to that in other centres, the incidence in elderly celiac patients was 23% in this study. Celiac disease was detected before or after the diagnosis of lymphoma or small intestinal adenocarcinoma. In some patients, epithelial lymphocytosis was evident in the gastric, colonic or biliary tract epithelium. In addition, other immune-mediated disorders, dermatitis herpetiformis and autoimmune thyroiditis, were common. Finally, other malignant disorders of the esophagus, stomach and colon were not detected.

  14. Reference values for frequency volume chart and uroflowmetry parameters in adolescent and adult enuresis patients

    NARCIS (Netherlands)

    Hofmeester, Ilse; Brinker, Astrid E.; Steffens, Martijn G.; Mulder, Zwaan; van Capelle, Jan Willem; Feitz, Wout F.J.; Blanker, Marco H.

    2017-01-01

    Aims: Reference values of Frequency Volume Chart (FVC) and uroflowmetry parameters for adolescent and adult enuresis patients are lacking. In this study, we aim to describe those parameters, in order to interpret findings from FVCs and uroflowmetries in those patients. Methods: Retrospective, descri

  15. Presentations and outcome analysis of 205 adult patients with Henoch-Schnlein purpura nephritis

    Institute of Scientific and Technical Information of China (English)

    宣萍

    2014-01-01

    Objective To analyze the clinical,pathological data and outcomes of the adult patients with HenochSchnlein purpura nephritis(HSPN).Methods The data of 205 HSPN patients who were diagnosed in Kidney Disease Center of the First Affiliated Hospital of Zhejiang University betwee Jan 2004 and May 2013 were collected and analyzed in different groups.Results The average

  16. Predictors of Death in Contemporary Adult Patients with Eisenmenger Syndrome

    DEFF Research Database (Denmark)

    Kempny, Aleksander; Hjortshøj, Cristel S; Gu, Hong;

    2017-01-01

    syndrome). The majority had a post-tricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pre-tricuspid lesion (n=, 12.7%). Over a median follow-up of 3.1years [IQR 1.4-5.9], allowing for 4361.6 patient-years observation, 278 patients died and six and six underwent...

  17. Persistent Airflow Obstruction in Young Adult Asthma Patients

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    Kiyoshi Sekiya

    2012-01-01

    Conclusions: In this study, patients not undergoing treatment for asthma were examined. History of childhood asthma and smoking history may be the risk factors for persistent airway obstruction in the asthma patients with mild subjective symptoms. Tests on the bronchodilator change in FEV1 should be performed in patients with history of childhood asthma and smoking history, even if they have only mild subjective symptoms.

  18. Cognitive deficits in adult patients with brain tumours.

    NARCIS (Netherlands)

    Taphoorn, M.J.B.; Klein, M.

    2004-01-01

    Cognitive function, with survival and response on brain imaging, is increasingly regarded as an important outcome measure in patients with brain tumours. This measure provides us with information on a patient's clinical situation and adverse treatment effects. Radiotherapy has been regarded as the m

  19. Long-term outcome of correction of tetralogy of Fallot in 56 adult patients

    Institute of Scientific and Technical Information of China (English)

    ZHENG Da-wei; SHAO Guo-feng; FENG Qiang; NI Yi-ming

    2013-01-01

    Background Although most patients with tetralogy of Fallot undergo radical repair during infancy and childhood,patients that remain undiagnosed and untreated until adulthood can still be treated.This study aimed to evaluate longterm outcomes of adult patients with tetralogy of Fallot who were treated surgically,and to determine the predictors of postoperative pulmonary regurgitation.Methods Fifty-six adult patients underwent complete surgical repair.Forty-three patients (76.8%) required a transannular patch.Systolic,diastolic,and mean pressure in the main pulmonary artery were measured after repair.Results The early mortality rate was 3.6%.The 16-year survival rate was (84.4±11.5)%.Late echocardiography revealed 41 patients with transannular patch who had pulmonary regurgitation,consisting of mild pulmonary regurgitation in 28 patients,moderate in eight,and severe regurgitation in five patients.In addition,there was right ventricular outflow tract stenosis in nine patients,moderate/severe tricuspid valve regurgitation in seven,and residual ventricular septal defect in five.Logistic regression analysis demonstrated that the mean pulmonary pressure measured just after repair predicted late pulmonary regurgitation.Conclusions The long-term survival of surgically treated adult patients with tetralogy of Fallot is acceptable.The mean pressure >20 mmHg in the main pulmonary artery measured right after surgical repair may be a feasible reference to time the reconstruction of the pulmonary valve.

  20. Lichen Striatus in a UVB Treated Adult Psoriasis Patient

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    Pınar Özuğuz

    2014-06-01

    Full Text Available Lichen striatus is a self-limited lichenoid eruption that follows Blaschko’s lines. It is particularly common in children and rarely seen in adults. Cases related to atopic diathesis are common, besides it is reported among family members. Viral infections, vaccines and trauma related cases are also present. Various environmental stimuli in the setting of genetic predisposition may play a role. We herein, reported a forty eight year-old woman who developed lichen striatus eruptions in her arm and trunk while she was receiving UVB treatment with maintenance dose for psoriasis. Phototherapy might act as a stimulant with its immunosuppressive and traumatic effects.

  1. Borderline typical symptoms in adult patients with attention deficit/hyperactivity disorder.

    Science.gov (United States)

    Philipsen, Alexandra; Feige, Bernd; Hesslinger, Bernd; Scheel, Corinna; Ebert, Dieter; Matthies, Swantje; Limberger, Matthias F; Kleindienst, Nikolaus; Bohus, Martin; Lieb, Klaus

    2009-05-01

    Adult attention deficit/hyperactivity disorder (ADHD) and borderline personality disorder (BPD) share several clinical features, e.g. emotional lability and impulsivity. This study aimed to delineate differences and similarities between ADHD and BPD with respect to borderline typical symptomatology and gender specifics. Borderline symptomatology was assessed in 60 adult patients with ADHD with the borderline symptom list (BSL) and compared to both 60 gender- and age-matched BPD patients and control subjects. The BSL is a standardized instrument including 95 items on 7 subscales (self-perception, affect regulation, self-destruction, dysphoria, loneliness, intrusions and hostility). Adult ADHD patients showed significantly higher BSL total scores and all of the seven subscales compared to healthy controls (p ADHD and BPD patients were found with respect to self-destruction (d = 1.12) and affect dysregulation (d = 0.90), whereas the smallest difference was found with respect to loneliness (d = 0.36). In females, the BSL subscales "loneliness" and "hostility" did not differentiate between BPD and ADHD. Borderline typical symptoms are common in adult patients with ADHD but seem to be less pronounced than in patients with BPD. Females with ADHD and BPD share more clinical features than males. However, symptoms of self-destruction and affect dysregulation appear to be more severe in BPD patients.

  2. SURGICAL TREATMENT OF ADULT PATIENTS WITH CONGENITAL CLUBFOOT IN SEVERITY DEGREE

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    A. A. Mukhamadeev

    2012-01-01

    Full Text Available There were treated 48 patients (adolescents and adults with severe congenital clubfoot in Saratov Research Institute of Traumatology and Orthopedics. 43 patients had the age ranged from 16 to 30, 5 patients had the age over 30. 19 patients had bilateral clubfoot, 29 patients had unilateral clubfoot including 19 on right side and 10 left side. To improve treatment results and to prevent deformity recurrence we developed two-staged surgery for congenital clubfoot treatment in adults. Cartilages of talo-navicular, talo-calcanean, cuneo-talar joints are deleted and deformity is corrected with external fixator on I stage. Surgeon performs external fixator resetting, autobone grafting of these joints with compressive arthrodesis on second stage. This method results in full foot reconstruction, good functional outcome, full weight-bearing and improves patient life’s quality.

  3. Assessment of adult patients with hypernatremia: A single center experience

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    Ercan Gündüz

    2015-09-01

    Full Text Available Objective: In the present study, determination of symptoms, clinical characteristics, prevalence and recovery rates was aimed in patients who applied to the emergency service and diagnosed with hypernatremia. Methods: Patients who applied to Dicle University Medical School Emergency Service during January 2013-December 2014 and whose serum Na>148 mEq/L were included in the study. The study was conducted retrospectively. Results: Hypernatremia prevalence was determined as 0.21% in the cases who applied to the emergency service. The average age in all patients was 69±22 and the median age was 72 years. The average hospitalization period was 13.3±10.9 days. The mortality rate was 75.7% and male gender domination (56% was determined in patients who developed mortality. When mortality and recovery groups were compared statistically; significant difference was determined (p<0.05 in terms of hospitalization period, glucose, urea, creatinine and calcium averages. The complaints of our patients who applied to the emergency service were changes in consciousness (92.7%, oral intake disorder (83.4% and fever (48.6% based on frequency order. The accompanying comorbid states were cerebrovascular illness (36.9%, Dementia/Alzheimer (32.4% and hypertension (28.9% based on frequency order. Conclusion: Consequently, hypernatremia is a fluid-electrolyte disorder progressing with high mortality and could be observed in older patients and in patients whose oral intake is defective and who have cerebrovascular illness and dementia.

  4. Autistic-Like Traits in Adult Patients with Mood Disorders and Schizophrenia

    OpenAIRE

    Junko Matsuo; Yoko Kamio; Hidetoshi Takahashi; Miho Ota; Toshiya Teraishi; Hiroaki Hori; Anna Nagashima; Reiko Takei; Teruhiko Higuchi; Nobutaka Motohashi; Hiroshi Kunugi

    2015-01-01

    Autism spectrum disorder often co-occurs with other psychiatric disorders. Although a high prevalence of autistic-like traits/symptoms has been identified in the pediatric psychiatric population of normal intelligence, there are no reports from adult psychiatric population. This study examined whether there is a greater prevalence of autistic-like traits/symptoms in patients with adult-onset psychiatric disorders such as major depressive disorder (MDD), bipolar disorder, or schizophrenia, and...

  5. Ingestion and Pharyngeal Trauma Causing Secondary Retropharyngeal Abscess in Five Adult Patients

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    Sudhir B. Sharma

    2012-01-01

    Full Text Available Retropharyngeal abscess most commonly occurs in children. When present in adults the clinical features may not be typical, and associated immunosuppression or local trauma can be part of the presentation. We present a case series of five adult patients who developed foreign body ingestion trauma associated retropharyngeal abscess. The unusual pearls of each case, along with their outcomes, are discussed. Pertinent information for the emergency medicine physician regarding retropharyngeal abscess is presented as well.

  6. [Acute cerebral ischemia: an unusual clinical presentation of isolated left ventricular noncompaction in an adult patient].

    Science.gov (United States)

    Fiorencis, Andrea; Quadretti, Laura; Bacich, Daniela; Chiodi, Elisabetta; Mele, Donato; Fiorencis, Roberto

    2013-01-01

    Isolated left ventricular noncompaction in adults is uncommon. The most frequent clinical manifestations are heart failure due to left ventricular systolic dysfunction and supraventricular and ventricular arrhythmias, which may be sustained and associated with sudden death. Thromboembolic complications are also possible. We report the case of an adult patient with isolated left ventricular noncompaction who came to our observation because of acute cerebral ischemia, an initial presentation of the disease only rarely described.

  7. Paradoxical Embolization in an Adult Cystic Fibrosis Patient

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    Nabil M Al Lawati

    2007-01-01

    Full Text Available Cystic fibrosis patients with an implantable venous access device (IVAD and a patent foramen ovale (PFO are at an increased risk of developing paradoxical embolism. A 33-year-old patient who had a cerebrovascular accident in the above setting is described. She had been anticoagulated because she had thrombosis of the tip of the indwelling catheter, and her PFO was closed percutaneuosly followed by replacement of her IVAD. She made a full neurological recovery. Echocardiography and prophylactic closure of the PFO, when present, as primary prevention for paradoxical embolism may be warranted in cystic fibrosis patients before placement of an IVAD.

  8. Optimizing Survival Outcomes For Adult Patients With Nontraumatic Cardiac Arrest.

    Science.gov (United States)

    Jung, Julianna

    2016-10-01

    Patient survival after cardiac arrest can be improved significantly with prompt and effective resuscitative care. This systematic review analyzes the basic life support factors that improve survival outcome, including chest compression technique and rapid defibrillation of shockable rhythms. For patients who are successfully resuscitated, comprehensive postresuscitation care is essential. Targeted temperature management is recommended for all patients who remain comatose, in addition to careful monitoring of oxygenation, hemodynamics, and cardiac rhythm. Management of cardiac arrest in circumstances such as pregnancy, pulmonary embolism, opioid overdose and other toxicologic causes, hypothermia, and coronary ischemia are also reviewed.

  9. Mutations in Smooth Muscle Alpha-Actin (ACTA2) Cause Coronary Artery Disease, Stroke, and Moyamoya Disease, Along with Thoracic Aortic Disease

    Science.gov (United States)

    Guo, Dong-Chuan; Papke, Christina L.; Tran-Fadulu, Van; Regalado, Ellen S.; Avidan, Nili; Johnson, Ralph Jay; Kim, Dong H.; Pannu, Hariyadarshi; Willing, Marcia C.; Sparks, Elizabeth; Pyeritz, Reed E.; Singh, Michael N.; Dalman, Ronald L.; Grotta, James C.; Marian, Ali J.; Boerwinkle, Eric A.; Frazier, Lorraine Q.; LeMaire, Scott A.; Coselli, Joseph S.; Estrera, Anthony L.; Safi, Hazim J.; Veeraraghavan, Sudha; Muzny, Donna M.; Wheeler, David A.; Willerson, James T.; Yu, Robert K.; Shete, Sanjay S.; Scherer, Steven E.; Raman, C.S.; Buja, L. Maximilian; Milewicz, Dianna M.

    2009-01-01

    The vascular smooth muscle cell (SMC)-specific isoform of α-actin (ACTA2) is a major component of the contractile apparatus in SMCs located throughout the arterial system. Heterozygous ACTA2 mutations cause familial thoracic aortic aneurysms and dissections (TAAD), but only half of mutation carriers have aortic disease. Linkage analysis and association studies of individuals in 20 families with ACTA2 mutations indicate that mutation carriers can have a diversity of vascular diseases, including premature onset of coronary artery disease (CAD) and premature ischemic strokes (including Moyamoya disease [MMD]), as well as previously defined TAAD. Sequencing of DNA from patients with nonfamilial TAAD and from premature-onset CAD patients independently identified ACTA2 mutations in these patients and premature onset strokes in family members with ACTA2 mutations. Vascular pathology and analysis of explanted SMCs and myofibroblasts from patients harboring ACTA2 suggested that increased proliferation of SMCs contributed to occlusive diseases. These results indicate that heterozygous ACTA2 mutations predispose patients to a variety of diffuse and diverse vascular diseases, including TAAD, premature CAD, ischemic strokes, and MMD. These data demonstrate that diffuse vascular diseases resulting from either occluded or enlarged arteries can be caused by mutations in a single gene and have direct implications for clinical management and research on familial vascular diseases. PMID:19409525

  10. Sabril® registry 5-year results: Characteristics of adult patients treated with vigabatrin.

    Science.gov (United States)

    Krauss, Gregory; Faught, Edward; Foroozan, Rod; Pellock, John M; Sergott, Robert C; Shields, W Donald; Ziemann, Adam; Dribinsky, Yekaterina; Lee, Deborah; Torri, Sarah; Othman, Feisal; Isojarvi, Jouko

    2016-03-01

    Vigabatrin (Sabril®), approved in the US in 2009, is currently indicated as adjunctive therapy for refractory complex partial seizures (rCPS) in patients ≥ 10 years old who have responded inadequately to several alternative treatments and as monotherapy for infantile spasms (IS) in patients 1 month to 2 years of age. Because of reports of vision loss following vigabatrin exposure, FDA approval required a risk evaluation mitigation strategy (REMS) program. Vigabatrin is only available in the US through Support, Help, And Resources for Epilepsy (SHARE), which includes a mandated registry. This article describes 5 years of demographic and treatment exposure data from adult patients (≥ 17 years old) in the US treated with vigabatrin and monitored in the ongoing Sabril® registry. Registry participation is mandatory for all US Sabril® prescribers and patients. A benefit-risk assessment must be documented by the physician for a patient to progress to maintenance therapy, defined as 1 month of vigabatrin treatment for patients with IS and 3 months for patients with rCPS. Ophthalmologic assessments must be documented during and after completion of therapy. As of August 26, 2014, a total of 6823 patients were enrolled in the registry, of which 1200 were adults at enrollment. Of these patients, 1031 (86%) were naïve to vigabatrin. The majority of adult patients (n=783, 65%) had previously been prescribed ≥ 4 AEDs, and 719 (60%) were receiving ≥ 3 concomitant AEDs at vigabatrin initiation. Prescribers submitted an initial ophthalmological assessment form for 863 patients; an ophthalmologic exam was not completed for 300 (35%) patients and thus, were considered exempted from vision testing. Of these patients, 128 (43%) were exempted for neurologic disabilities. Clinicians discontinued treatment in 8 patients because of visual field deficits (VFD) (5 patients naïve to vigabatrin and 3 patients previously exposed). Based on Kaplan-Meier survival estimates, it is

  11. Congenital tracheoesophageal fistula: A rare and late presentation in adult patient

    Directory of Open Access Journals (Sweden)

    Waseem M Hajjar

    2012-01-01

    Full Text Available Congenital H-type tracheoesophageal fistula (TEF in adults is a rare presentation and can test the diagnostic acumen of a surgeon, endoscopist, and the radiologist. These undetected fistulas may present as chronic lung disease of unknown origin because repeated aspirations can lead to recurrent lung infections and bronchiectasis. Congenital TEFs should be considered in the diagnosis of infants and young adults with recurrent respiratory distress and/or infections. Here, we present the successful management of this rare case in an adult patient.

  12. Nail involvement in adult patients with plaque-type psoriasis: prevalence and clinical features

    OpenAIRE

    Schons,Karen Regina Rosso; Beber,André Avelino Costa; Beck, Maristela de Oliveira; Monticielo, Odirlei André

    2015-01-01

    Abstract BACKGROUND: Psoriasis is a disease of worldwide distribution with a prevalence of 1 to 3%. Nail psoriasis is estimated in 50% of patients with psoriasis, and in the presence of joint involvement, it can reach 80%. OBJECTIVE: To study the nail changes - and their clinical implications - presented by patients with psoriasis vulgaris under surveillance in a university hospital from the south of Brazil. METHODS: his cross-sectional study evaluated 65 adult patients from January 2012 to M...

  13. 甲状腺功能亢进与moya moya综合征的研究进展%Research Progess in Hyperthyroidism and Moyamoya Syndrome

    Institute of Scientific and Technical Information of China (English)

    肖伏龙; 高培毅

    2015-01-01

    与moyamoya病相比,moyamoya综合征尚未形成典型的异常血管网,moyamoya综合征中颅内动脉狭窄可能是moyamoya病的一个亚型或早期表现,且与moyamoya病存在相同的病理生理学机制。甲状腺功能亢进相关的moyamoya综合征的病理生理学机制包括自身免疫性破坏、血流动力学异常、动脉粥样硬化及交感神经兴奋性增加等,诊断和治疗上,应密切关注甲状腺素水平的变化。本文对甲状腺功能亢进相关的moyamoya综合征的病理生理学机制及临床诊断治疗中应注意的问题做一综述。%The abnormal vascular network found in moyamoya syndrome is different from that in moyamoya disease. Stenosis in intracranial vessels found in moyamoya syndrome may be a subtype or an early stage of moyamoya disease. The pathophysiology in moyamoya syndrome may have some common ground with moyamoya disease. The pathophysiology in moyamoya syndrome associated with hyperthyroidism includes autoimmune disorder, abnormal hemodynamic, arteriosclerosis and increased sympathetic activation. Changes in thyroxin level should be concerned in clinical practice. In this review, we summarize the pathophysiology of moyamoya syndrome associated with hyperthyroidism and some points in clinical diagnosis and treatment.

  14. Factors affecting ventriculoperitoneal shunt survival in adult patients

    Directory of Open Access Journals (Sweden)

    Farid Khan

    2015-01-01

    Conclusions: Patients with increased age, prolonged hospital stay, GCS score of less than 13, extra-ventricular drains in situ, or excision of brain tumors were more likely to experience early shunt malfunction.

  15. Giardia Assemblages A and B in Diarrheic Patients: A Comparative Study in Egyptian Children and Adults.

    Science.gov (United States)

    El Basha, Noussa R; Zaki, Mayssa M; Hassanin, Omayma M; Rehan, Mohamed K; Omran, Dalia

    2016-02-01

    Giardia duodenalis is considered the most common intestinal parasite in humans worldwide. Children are especially affected, with more severe consequences than adults. The present study was designed to determine the distribution of assemblages A and B Giardia infection in children and adults, with the use of light microscopy and polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) as diagnostic procedures, and to investigate its associations with clinical and epidemiological data collected from children and adult groups. This cross-sectional study was conducted from October 2012 to October 2013 by collecting fecal samples from 200 children and 200 adults complaining of diarrhea. Samples were subjected to parasitological examination by direct wet smear and formol-ether methods. Genotyping of G. doudenalis samples was conducted by PCR-RFLP analysis. Giardia duodenalis infection caused by assemblages A and B was identified in 60 samples, 34 from children and 26 from adults. Assemblage B was detected in 38 patients (63.34%), and assemblage A was detected in 22 patients (36.66%). Assemblage A was significantly more frequent in children with age range 2-8 yr, and assemblage B was higher in children with age range 6-16 yr old. Diarrhea frequency/day and recurrences per month affected patients infected with assemblage A (P value Giardia assemblages A and B were identified in children and adults, assemblage A infected younger children more frequently and was more closely related to severe clinical manifestations than assemblage B.

  16. Patient satisfaction and ethnic identity among American Indian older adults.

    Science.gov (United States)

    Garroutte, Eva Marie; Kunovich, Robert M; Jacobsen, Clemma; Goldberg, Jack

    2004-12-01

    Work in the field of culturally competent medical care draws on studies showing that minority Americans often report lower satisfaction with care than White Americans and recommends that providers should adapt care to patients' cultural needs. However, empirical evidence in support of cultural competence models is limited by reliance upon measurements of racial rather than ethnic identity and also by a near-total neglect of American Indians. This project explored the relationship between ethnic identity and satisfaction using survey data collected from 115 chronically ill American Indian patients >or=50 years at a Cherokee Nation clinic. Satisfaction scores were high overall and comparable to those found in the general population. Nevertheless, analysis using hierarchical linear modeling showed that patients' self-rated American Indian ethnic identity was significantly associated with satisfaction. Specifically, patients who rated themselves high on the measure of American Indian ethnic identity reported reduced scores on satisfaction with health care providers' social skill and attentiveness, as compared to those who rated themselves lower. Significant associations remained after controlling for patients' sex, age, education, marital status, self-reported health, wait time, and number of previous visits. There were no significant associations between patients' American Indian ethnic identity and satisfaction with provider's technical skill and shared decision-making. Likewise, there were no significant associations between satisfaction and a separate measure of White American ethnic identity, although a suggestive trend was observed for satisfaction with provider's social skill. Our findings demonstrate the importance of including measures of ethnic identity in studies of medical satisfaction in racial minority populations. They support the importance of adapting care to patient's cultural needs, and they highlight the particular significance of interpersonal

  17. Monitoring of the Adult Patient on Venoarterial Extracorporeal Membrane Oxygenation

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    Mabel Chung

    2014-01-01

    Full Text Available Venoarterial extracorporeal membrane oxygenation (VA ECMO provides mechanical support to the patient with cardiac or cardiopulmonary failure. This paper reviews the physiology of VA ECMO including the determinants of ECMO flow and gas exchange. The efficacy of this therapy may be determined by assessing patient hemodynamics and device flow, overall gas exchange support, markers of adequate oxygen delivery, and pulsatility of the arterial blood pressure waveform.

  18. Analysis of adult patients with epilepsy in the age continuum

    Directory of Open Access Journals (Sweden)

    V. A. Karlov

    2016-01-01

    Full Text Available Objective: to study the presence of comorbidities and to investigate quality of life in patients with main types of epilepsy in relation to age.Patients and methods. The investigation enrolled 150 patients aged 17 to 64 years with idiopathic, cryptogenic, and symptomatic types of epilepsy. The investigators studied the presence of sleep disorders using the questionnaires designed by the Somnology Center, Ministry of Health of Russia, and that of daytime sleepiness by the Epworth Sleepiness Scale, determined the level of anxiety and depression by the Hospital Anxiety and Depression Scale, and assessed the severity of seizures by the National Hospital Seizure Severity Scale. Quality of life was investigated using the QOLIE-89 questionnaire. A cluster analysis was carried out to divide all the patients into three age groups (mean age, 27.3; 30.7; and 37.7 years.Results and discussion. A positive relationship was obtained between some indicators of sleep disorders, level of anxiety, and age. There were significant differences between the above three age groups in the following indicators: sleep disorders; daytime sleepiness; level of anxiety, severity of seizures, and scores of the QOLIE-89 questionnaire (p<0.001. Since the patients suffer from different basic types of epilepsy (cryptogenic, idiopathic, and symptomatic, the findings testify to age as an independent factor that modifies quality of life in epileptic patients.

  19. [Perspectives in the management of congenital heart defects in adult patients].

    Science.gov (United States)

    Hartyánszky, István; Varga, Sándor; Havasi, Kálmán; Babik, Barna; Katona, Márta; Bogáts, Gábor

    2015-01-18

    Due to improving results in congenital heart surgery, the number of adult patients with congenital heart defect is increasing. The question is: what kind of problems can be managed in this patient-group? The authors review the different problems of management of congenital heart defects in adults based on national and international literature data. Simple defects recognised in adults, postoperative residual problems, changing of small grafts and valves, correction of primary or operated coarctation aortae can be usually managed without problems. A very close follow-up is necessary to establish the correct period for heart transplantation in patients with transposition of great arteries with Senning/Mustard operation, and univentricular heart corrected with "Fontan-circulation" type surgical procedure. The authors conclude that although the number of patients increases, only a few congenital heart diseases may cause problems. It seems important (1) to monitor asymptomatic patient who underwent operation (Fallot-IV, Ross procedure, etc.), (2) follow up regularly patients who underwent Senning/Mustard procedure (magnetic resonance imaging, echocardiography, brain natriuretic peptide measurement), (3) define the proper period of preparation for heart transplantation of patients with a univentricular heart, with special attention to the possibility of multiorgan (lung, liver, etc.) failure. Due to the improvement of foetal diagnosis of congenital heart defects, the number of patients with complex congenital heart defects is decreasing. The standard management of these patients could be primary heart transplantation in infancy.

  20. Split-liver transplantation in the high-MELD adult patient: are we being too cautious?

    Science.gov (United States)

    Nadalin, Silvio; Schaffer, Randolph; Fruehauf, Nils

    2009-07-01

    The fear that patients with high-mathematical model for end stage liver disease (MELD) score may not be suitable candidates for segmental grafts because of their need for greater liver mass has continued to push the transplant community toward the use of whole LT (WLT) in preference to split LT (SLT). In order to define the outcome of segmental liver transplantation in a better manner in high-MELD patients (score > or =26), we queried the UNOS registry for graft and patient survival results according to MELD score in adult patients receiving WLT and SLT in the United States from the inception of MELD allocation (February 27, 2002) through March 9, 2007. A total of 316 adult patients received a SLT as compared with 20 778 WLTs. Patient and graft survival rates at 6 and 12 months were comparable for all MELD ranges, including the 'high-MELD' recipients (e.g. at MELD score 31-35, patients' and grafts' survival rates at 12 months was 87.5% in SLT group vs. 84.4% and 76.7% in WLT group respectively). The results even at higher MELD scores (i.e. >35) were more than acceptable. In conclusion, patient and graft survival rates for SLT in high-MELD adult patients are comparable to the same for WLT.

  1. Moyamoya disease: CT scan study of a Brazilian-born Japanese girl

    Directory of Open Access Journals (Sweden)

    Guilberto Minguetti

    1981-03-01

    Full Text Available Moyamoya é uma doença cérebro-vascular de etiologia desconhecida que aparece predominantemente nos indivíduos de raça japonesa. Poucos são os relatos de casos desta doença cujos sintomas principais são decorrentes de infartos cerebrais múltiplos, convulsões e hemorragia subaracnoide. Os achados principais nos casos de moyamoya são os angiográficos que se caracterizam por oclusão parcial ou total das carótidas, malformações angiomatosas, circulação colateral proeminente na região dos gânglios da base e anastomoses trans-durais múltiplas. O caso aqui descrito apresenta, além do estudo angiográfico, avaliação genética e tomografia computadorizada cerebral (TCC. O cariotipo levado a efeito na paciente e em suas duas irmãs não apresenta qualquer anormalidade. A TCC, contudo, mostra claramente um padrão compatível com infartos cerebrais múltiplos antigos e a consequente repercussão de tais lesões no cérebro da paciente.

  2. [Post-radiotherapy stenosis of the supraclinoid internal carotid artery. Moyamoya network].

    Science.gov (United States)

    Benoit, P; Destée, A; Verier, A; Giraldon, J M; Warot, P

    1985-01-01

    A 36 year-old caucasian woman was operated and then irradiated for a pituitary adenoma. Two years later, a left anterior temporal lobectomy for a grade I astrocytoma was performed. Four years later, she experienced right hemiparesis and aphasia. CT scan showed a left temporo-occipital low density area. A left carotid angiogram showed a narrowing of the left carotid artery beginning in the lower part of the siphon and progressing to a complete supraclinoid occlusion. There was a collateral circulation of the Moyamoya type. Radiation-induced narrowing or occlusion of the intracranial internal carotid artery is an infrequent finding. Most cases appear in young subjects, several years after a high dose of radiation therapy (30 to 60 grays). Some cases may show a network of the Moyamoya type. Usually, the absence of vascular abnormalities prior to radiation cannot be demonstrated. In our case, as in 3 other cases of the literature, the intracranial vessels were of normal appearance before irradiation. The vascular lesions can thus be considered as acquired and secondary to radiation therapy.

  3. A new horizon of moyamoya disease and associated health risks explored through RNF213.

    Science.gov (United States)

    Koizumi, Akio; Kobayashi, Hatasu; Hitomi, Toshiaki; Harada, Kouji H; Habu, Toshiyuki; Youssefian, Shohab

    2016-03-01

    The cerebrovascular disorder moyamoya disease (MMD) was first described in 1957 in Japan, and is typically considered to be an Asian-specific disease. However, it is globally recognized as one of the major causes of childhood stroke. Although several monogenic diseases are known to be complicated by Moyamoya angiopathy, the ring finger protein 213 gene (RNF213) was identified as a susceptibility gene for MMD. RNF213 is unusual, because (1) it induces MMD with no other recognizable phenotypes, (2) the RNF213 p.R4810K variant is an Asian founder mutation common to Japanese, Korean and Chinese with carrier rates of 0.5-2% of the general population but a low penetrance, and (3) it encodes a relatively largest proteins with a dual AAA+ ATPase and E3 Ligase activities. In this review, we focus on the genetics and genetic epidemiology of RNF213, the pathology of RNF213 R4810K, and the molecular functions of RNF213, and also address the public health contributions to current unresolved issues of MMD. We also emphasize the importance of a more updated definition for MMD, of qualified cohort studies based on genetic epidemiology and an awareness of the ethical issues associated with genetic testing of carriers.

  4. Patient safety in the rehabilitation of the adult with an amputation.

    Science.gov (United States)

    Latlief, Gail; Elnitsky, Christine; Hart-Hughes, Stephanie; Phillips, Samuel L; Adams-Koss, Laurel; Kent, Robert; Highsmith, M Jason

    2012-05-01

    This article reviews and summarizes the literature on patient safety issues in the rehabilitation of adults with an amputation. Safety issues in the following areas are discussed; the prosthesis, falls, wound care, pain, and treatment of complex patients. Specific recommendations for further research and implementation strategies to prevent injury and improve safety are also provided. Communication between interdisciplinary team members and patient and caregiver education are crucial to executing a safe treatment plan. The multidisciplinary rehabilitation team members should feel comfortable discussing safety issues with patients and be able to recommend preventive approaches to patients as appropriate.

  5. Current status of revascularization surgery for Moyamoya disease: special consideration for its 'internal carotid-external carotid (IC-EC) conversion' as the physiological reorganization system.

    Science.gov (United States)

    Fujimura, Miki; Tominaga, Teiji

    2015-01-01

    Moyamoya disease is a chronic cerebrovascular disease with unknown etiology, which is characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network formation at the base of the brain. Moyamoya disease is known to have unique and dynamic nature to convert the vascular supply for the brain from internal carotid (IC) system to the external carotid (EC) system, as indicated by Suzuki's angiographic staging established in 1969. Insufficiency of this 'IC-EC conversion system' may result in cerebral ischemia, as well as in intracranial hemorrhage from inadequate collateral vascular network, both of which represent the clinical presentation of moyamoya disease. Therefore, surgical revascularization by extracranial-intracranial bypass is the preferred procedure for moyamoya disease to complement 'IC-EC conversion' and thus to avoid cerebral infarction and/or intracranial hemorrhage. Long-term outcome of revascularization surgery for moyamoya disease is favorable, but rapid increase in cerebral blood flow on the affected hemisphere could temporarily cause unfavorable phenomenon such as cerebral hyperperfusion syndrome. We would review the current status of revascularization surgery for moyamoya disease based on its basic pathology, and sought to discuss the significance of measuring cerebral blood flow in the acute stage and intensive perioperative management.

  6. Symptoms and aetiology of delirium: a comparison of elderly and adult patients.

    Science.gov (United States)

    Grover, S; Agarwal, M; Sharma, A; Mattoo, S K; Avasthi, A; Chakrabarti, S; Malhotra, S; Kulhara, P; Bas, D

    2013-06-01

    OBJECTIVE. To compare the symptoms of delirium as assessed by the Delirium Rating Scale-Revised-98 (DRS-R-98) and associated aetiologies in adult and elderly patients seen in a consultation-liaison service. METHODS. A total of 321 consecutive patients with a DSM-IV-TR diagnosis of delirium were assessed on the DRS-R-98 and a study-specific aetiology checklist. RESULTS. Of the 321 patients, 245 (76%) aged 18 to 64 years formed the adult group, while 76 (24%) formed the elderly group (≥ 65 years). The prevalence and severity of various symptoms of delirium as assessed using the DRS-R-98 were similar across the 2 groups, except for the adult group having statistically higher prevalence and severity scores for thought process abnormalities and lability of affect. For both groups and the whole sample, factor analysis yielded a 3-factor model for the phenomenology. In the 2 groups, the DRS-R-98 item loadings showed subtle differences across various factors. The 2 groups were similar for the mean number of aetiologies associated with delirium, the mean number being 3. However, the 2 groups differed with respect to hepatic derangement, substance intoxication, withdrawal, and postpartum causes being more common in the adult group, in contrast lung disease and cardiac abnormalities were more common in the elderly group. CONCLUSION. Adult and elderly patients with delirium are similar with respect to the distribution of various symptoms, motor subtypes, and associated aetiologies.

  7. The level of specialist assessment of adult asthma is influenced by patient age

    DEFF Research Database (Denmark)

    Porsbjerg, C; Sverrild, A; Stensen, L;

    2014-01-01

    is sparse. AIM: To examine the impact of patient age on the type and proportion of diagnostic tests performed in patients undergoing specialist assessment for asthma. METHODS: Data from a clinical population consisting of all patients consecutively referred over a 12 months period to a specialist clinic...... adults, and were more frequently smokers. However, a regression analysis showed that older age was associated with a lower likelihood of diagnostic assessment with a reversibility test, a bronchial challenge test, or measurement of exhaled NO, independently of a known diagnosis of asthma, smoking habits......BACKGROUND: Late onset asthma is associated with more severe disease and higher morbidity than in younger asthma patients. This may in part relate to under recognition of asthma in older adults, but evidence on the impact of patient age on diagnostic assessment of asthma in a specialist setting...

  8. Health-related quality of life in adult dermatitis patients stratified by filaggrin genotype

    DEFF Research Database (Denmark)

    Heede, Nina G; Thyssen, Jacob P; Thuesen, Betina H;

    2016-01-01

    BACKGROUND: Information concerning health-related quality of life (HRQoL) and comorbidities of adult dermatitis patients stratified by loss-of-function mutations in the filaggrin gene (FLG) is limited. OBJECTIVE: To investigate HRQoL, skin symptoms and comorbidities in adult FLG mutation carriers......, as measured by use of the Dermatology Life Quality Index (DLQI), no association with self-reported anxiety or depression was identified. Notably, the highest median DLQI score, reflecting greater impairment, was reported by patients with both FLG mutations and atopic dermatitis. Overall, 19.7% of patients....... METHODS: This cross-sectional study included patients diagnosed with atopic dermatitis and/or hand eczema (n = 520). Patients completed questionnaires about dermatitis, skin symptoms, HRQoL, and comorbidities, including actinic keratosis, and atopic and mental disorders. RESULTS: FLG mutations (R501X...

  9. Demographic characteristics of epilepsy patients and antiepileptic drug utilization in adult patients: Results of a cross-sectional survey

    Directory of Open Access Journals (Sweden)

    Sanket Newale

    2016-01-01

    Conclusion: Epilepsy is common in adult patients. Hypertension is the most common comorbidity. Levetiracetam is the most commonly used AED across all studied age groups and both genders. Control of epilepsy with current treatment is satisfactory with no major adverse events.

  10. Modern treatment of adult short bowel syndrome patients

    DEFF Research Database (Denmark)

    Efsen, E; Jeppesen, P B

    2011-01-01

    By definition, intestinal failure prevails when oral compensation is no longer feasible and parenteral support is necessary to maintain nutritional equilibrium. In the past, conventional treatment has mainly focused on "making the most of what the short bowel syndrome patient still had" by optimi......By definition, intestinal failure prevails when oral compensation is no longer feasible and parenteral support is necessary to maintain nutritional equilibrium. In the past, conventional treatment has mainly focused on "making the most of what the short bowel syndrome patient still had...

  11. Adult patients' adjustability to orthodontic appliances. Part I: a comparison between Labial, Lingual, and Invisalign™.

    Science.gov (United States)

    Shalish, Miri; Cooper-Kazaz, Rena; Ivgi, Inbal; Canetti, Laura; Tsur, Boaz; Bachar, Eytan; Chaushu, Stella

    2012-12-01

    This prospective study examined the adult patient's perception of recovery after insertion of three types of orthodontic appliances: Buccal, Lingual and Invisalign. The sample consisted of sixty-eight adult patients (45 females and 23 males) who comprised three groups: 28 Buccal, 19 Lingual, and 21 Invisalign patients. After appliance insertion, patients completed a Health-Related Quality of Life questionnaire daily for the first week and again on day 14, in order to assess patients' perception of pain and analgesic consumption. In addition, four areas of dysfunction were assessed: oral dysfunction, eating disturbances, general activity parameters, and oral symptoms. Lingual appliance was associated with more severe pain and analgesic consumption, the greatest oral and general dysfunction, and the most difficult and longest recovery. The Invisalign patients complained of relatively high levels of pain in the first days after insertion; however this group was characterized by the lowest level of oral symptoms and by a similar level of general activity disturbances and oral dysfunction compared to the Buccal appliance. Many Lingual and some Buccal patients did not reach a full recovery from their eating difficulties by the end of the study period. The present study provides information to adult patients and clinicians assisting them in choosing the most appropriate treatment modality in relation to Health-Related Quality of Life parameters.

  12. Simultaneous premaxillary repositioning and cheiloplasty in adult patients with unrepaired bilateral cleft lip and palate.

    Science.gov (United States)

    Lee, Ui-Lyong; Cho, Jun-Bum; Choung, Pill-Hoon

    2013-03-01

    Primary cheiloplasty in adult patients with unrepaired complete bilateral cleft lip and palate is quite challenging due to severe premaxillary anterior projection. To get the best repair results, the author carried out repositioning of the premaxilla and repair of the lip deformity in a single stage. Positive results for the primary lip repair and appropriate repositioning of the premaxilla were achieved. No avascular necrosis of the premaxilla was observed. Repositioning of the premaxilla and repair of the lip deformity in a single operation appears to be a reliable method for treating adult patients with previously unrepaired or poorly repaired bilateral cleft lip and palate.

  13. Corticosteroids for all adult patients with community-acquired pneumonia?

    OpenAIRE

    Ger Rijkers; Simone Spoorenberg; Stefan Vestjens; Werner Albrich

    2015-01-01

    Corticosteroid therapy as adjunctive treatment in community-acquired pneumonia (CAP) is a promising but controversial subject. The potentially beneficial effect of corticosteroids is based on the ability of steroids to dampen an excessive inflammatory response that often occurs in patients with CAP. This excessive inflammatory response can cause damage to the lungs and other organs, and is associated with poor outcome.

  14. Intravenous lipids in adult intensive care unit patients.

    Science.gov (United States)

    Hecker, Matthias; Mayer, Konstantin

    2015-01-01

    Malnutrition of critically ill patients is a widespread phenomenon in intensive care units (ICUs) worldwide. Lipid emulsions (LEs) are able to provide sufficient caloric support and essential fatty acids to correct the energy deficit and improve outcome. Furthermore, components of LEs might impact cell and organ function in an ICU setting. All currently available LEs for parenteral use are effective in providing energy and possess a good safety profile. Nevertheless, soybean oil-based LEs have been associated with an elevated risk of adverse outcomes, possibly due to their high content of omega-6 fatty acids. More newly developed emulsions partially replace soybean oil with medium-chain triglycerides, fish oil or olive oil in various combinations to reduce its negative effects on immune function and inflammation. The majority of experimental studies and smaller clinical trials provide initial evidence for a beneficial impact of these modern LEs on critically ill patients. However, large, well-designed clinical trials are needed to evaluate which LE offers the greatest advantages concerning clinical outcome. Lipid emulsions (LEs) are a powerful source of energy that can help to adjust the caloric deficit of intensive care unit (ICU) patients. LEs possess various biological activities, but their subsequent impact on critically ill patients awaits further investigations.

  15. Effectiveness of sulpiride in adult patients with schizophrenia.

    Science.gov (United States)

    Lai, Edward Chia-Cheng; Chang, Chia-Hsien; Kao Yang, Yea-Huei; Lin, Swu-Jane; Lin, Chia-Yin

    2013-05-01

    The objective of this study is to compare the effectiveness among sulpiride, risperidone, olanzapine, and haloperidol by evaluating the persistence of drug use. A retrospective cohort study was conducted by analyzing the National Health Insurance Research Database of Taiwan. Patients with schizophrenia aged 18-65 years and newly prescribed with a single oral antipsychotic medication between years 2003 and 2008 were included. The primary outcome was the persistence of antipsychotic agents by calculating the treatment duration till treatment changed. All defined treatment changes were also analyzed separately, including discontinuation, switching, augmentation, and hospitalization. A total of 1324 eligible patients were included, with an average age of 36 years old and approximately 45% of them were female. The most prevalent antipsychotic use was risperidone (42.1%), followed by sulpiride (36.0%), haloperidol (14.2%), and olanzapine (7.7%). After adjusting for patient demographics, mental illness characteristics, and propensity score, the Cox regression models found that the risk of nonpersistence was significantly higher in patients receiving risperidone (hazard ratio [HR], 1.22; 95% CI, 1.06-1.40), haloperidol (HR, 1.98; 95% CI, 1.63-2.40), and olanzapine (HR, 1.34; 95% CI, 1.07-1.68), as compared with sulpiride, suggesting the effectiveness of sulpiride was better than the other 3 antipsychotics. Therefore, this study would provide strong grounds for a properly conducted randomized controlled trial of the clinical- and cost-effectiveness of sulpiride vs atypical antipsychotics.

  16. Home Parenteral Nutrition in Adult Patients With Chronic Intestinal Failure

    DEFF Research Database (Denmark)

    Brandt, Christopher Filtenborg; Hvistendahl, Mark; Naimi, Rahim M

    2017-01-01

    in treating IF with home parenteral nutrition (HPN), this study documents the HPN evolution and describes the demographics and outcome in one of the world's largest single-center cohorts. METHODS: We included patients with IF discharged with HPN from 1970-2010. Data were extracted according to European...

  17. Analgesia for terminally ill adult patients. Preserve quality of life.

    Science.gov (United States)

    2011-11-01

    Adequate pain management is crucial in maintaining the best possible quality of life for terminally ill patients. This article examines pain management in the palliative care setting, based on a review of the literature using the standard Prescrire methodology. Accurate pain evaluation, preferably by the patient, is essential for guiding treatment decisions. Some causes of pain are amenable to specific treatments. The expected benefits and harms of the various treatment options and procedures must be weighed on a case by case basis. Quality of life should always be the first priority. The World Health Organization has developed a "three-step analgesic ladder", based on the use of increasingly potent analgesics: step I analgesics include paracetamol and nonsteroidal anti-inflammatory drugs (NSAIDs); codeine is the standard step II analgesic; and morphine is the standard step III analgesic. Fentanyl is an alternative to morphine. The daily morphine dose must be determined for each patient. Morphine titration starts with oral doses given every 4 hours, but additional doses can be taken every hour if necessary. Total consumption is then used to calculate the dose required the following day. A sustained-release product can be used to reduce the number of doses required when a consistently effective daily dose has been established. When patients are unable to take morphine orally, it can be given by subcutaneous injection, and by subcutaneous or intravenous infusion. Pumps allow the patient to self-administer morphine on demand. Fentanyl transdermal patches are another option for stable pain. Immediate-release oral forms and injections are useful for preventing or treating breakthrough pain. If morphine requirements increase during treatment, the most likely explanations are exacerbations of pain or an excessively long interval between doses. Pharmacological tolerance and psychological dependence are rare during palliative care. In case of renal failure, the morphine

  18. Validity of the Adult ADHD Self-Report Scale (ASRS) as a screener for adult ADHD in treatment seeking substance use disorder patients

    NARCIS (Netherlands)

    van de Glind, Geurt; van den Brink, Wim; Koeter, Maarten W. J.; Carpentier, Pieter-Jan; van Emmerik-van Oortmerssen, Katelijne; Kaye, Sharlene; Skutle, Arvid; Bu, Eli-Torild H.; Franck, Johan; Konstenius, Maija; Moggi, Franz; Dom, Geert; Verspreet, Sofie; Demetrovics, Zsolt; Kapitany-Foeveny, Mate; Fatseas, Melina; Auriacombe, Marc; Schillinger, Arild; Seitz, Andrea; Johnson, Brian; Faraone, Stephen V.; Ramos-Quiroga, J. Antoni; Casas, Miguel; Allsop, Steve; Carruthers, Susan; Bartar, Csaba; Schoevers, Robert A.; Levin, Frances R.

    2013-01-01

    Background: To detect attention deficit hyperactivity disorder (ADHD) in treatment seeking substance use disorders (SUD) patients, a valid screening instrument is needed. Objectives: To test the performance of the Adult ADHD Self-Report Scale V 1.1(ASRS) for adult ADHD in an international sample of

  19. 多层螺旋CT血管成像容积重建技术在Moyamoya病中的诊断价值%The diagnostic value of multi-slice computed tomographic angiography with volume rendering for Moyamoya disease

    Institute of Scientific and Technical Information of China (English)

    林伯法; 奚玉平; 曹国全

    2008-01-01

    Objective To evaluate the diagnostic value of multi-slice thrce-dimensional computed tomographic angiography(MS-CTA) with volume rendering(VR) for Moyamoya disease.Methods MS-CTA of 25 patients with Moyamoya disease verified by DSA were restrospectively analyzed.Source images were got by GE Lightspeed pro scanner.VR was adopted to reconstruct 3D images in all cases.Results Of 25 CT precontrast,13,12 cases showed infarction and hemorrhage respectively,while 11 cases had dialated vessels in thalamus-basal ganglia region on CT.Accuracy of stenosis or occlusion of the bifurcation of ICA, proximal portion of the ACA or MCA was 88.0% (22/25) by MS-CTA.MS-CTA overestimated the degree of stenosis,MS-CTA misdiagnosed 3 cases(12.0%)with stenosis to occlusion.MS-CTA showed fewer small Moyamoya vessels than DSA.MS-CTA only demonstrated 6 cases(25.0%)with collateral vessels.Conclusion CT is still the conventional method for detecting secondary lesions to Moyamoya disease.CT and MS-CTA can accurately diagnose Moyamoya disease.%目的 探讨多层螺旋CT血管造影(MS-CTA)容积重建(VR)技术在Moyamoya病中的诊断价值.方法 对经DSA证实的25例Moyamoya患者的MS-CTA影像资料进行回顾性分析,使用GE Lightspeed pro 16层螺旋CT扫描仪获得原始图像,所有病例均采用VR技术对图像进行三维重建.结果 25例CT平扫患者中,脑梗死13例,脑出血12例,其中11例可见丘脑一基底节区有扩张的血管;MS-CTA VR重建显示Moyamoya病颈内动脉(ICA)末端、大脑中动脉(MCA)或大脑前动脉(ACA)近端闭塞或狭窄的准确率为88.0%(22/25).与DSA相比,MS-CTA VR重建高估动脉狭窄程度,将ICA狭窄误诊为闭塞3例(12.0%);在显示Moyamoya血管数目及分支方面,MS-CTA VR重建显示细小分支数目较少;在显示侧支循环方面,MS-CTA VR重建仅显示6例(25.0%).结论 CT平扫仍是发现Moyamoya病继发病变的常规方法,与MS-CTA VR重建相结合可正确诊断Moyamoya病.

  20. Comparison of human growth hormone products' cost in pediatric and adult patients. A budgetary impact model.

    Science.gov (United States)

    Bazalo, Gary R; Joshi, Ashish V; Germak, John

    2007-09-01

    We assessed the economic impact to the United States payer of recombinant human growth hormone (rhGH) utilization, comparing the relative dosage efficiency of marketed pen-based and vial-based products in a pediatric and in an adult population. A budgetary impact model calculated drug costs based on product waste and cost. Waste was the difference between prescribed dose, based on patient weight, and actual delivered dose, based on dosing increments and maximum deliverable dose for pens and a fixed-percent waste as derived from the literature for vials. Annual wholesale acquisition costs were calculated based upon total milligrams delivered, using a daily dose of 0.03 mg/kg for pediatric patients and 0.016 mg/kg for adults. Total annual drug costs were compared for two scenarios: 1) a product mix based on national market share and 2) restricting use to the product with lowest waste. Based on the literature, waste for each vial product was 23 percent. Among individual pens, waste was highest for Humatrope 24 mg (19.5 percent pediatric, 14.3 percent adult) and lowest for Norditropin Nordi-Flex 5 mg (1.1 percent pediatric, 1 percent adult). Restricting use to the brand with least waste (Norditropin), compared to national product share mix, resulted in a 10.2 percent reduction in annual pediatric patient cost from $19,026 to $17,089 and an 8 percent reduction in annual adult patient cost from $24,099 to $22,161. We concluded that pen delivery systems result in less waste than vial and syringe. Considering all approved delivery systems, Norditropin resulted in the least product waste and lower annual patient cost for both pediatric and adult populations.

  1. Enterocolitis without diarrhoea in an adult patient: a clinical dilemma.

    Science.gov (United States)

    Vaidya, Gaurang Nandkishor; Sharma, Amit; Khorasani-Zadeh, Arman; John, Savio

    2014-03-04

    Campylobacter jejuni is one of the most common causes of bloody diarrhoea in the USA. We report a case of a young woman who presented with a clinical picture reminiscent of acute appendicitis. Ultrasonography and CT of the abdomen performed subsequently revealed evidence of colitis. Quite unexpectedly, she had no symptoms of diarrhoea and the stool Gram stain and culture were negative. Nevertheless, due to high clinical suspicion of infectious colitis, appendectomy was deferred. Blood culture was later reported positive for Campylobacter species and the patient responded to quinolones. With this case report we try to highlight one of the unusual presentations of C jejuni infection, closely mimicking acute appendicitis in the absence of classical symptoms of bacterial enteritis. In such cases, a high index of suspicion, astute history taking skills and the proper use of imaging studies can save the patient from the surgical knife.

  2. Vaccination recommendations for adult patients with autoimmune inflammatory rheumatic diseases

    OpenAIRE

    Bühler, Silja; Eperon, Gilles; Ribi, Camillo; Kyburz, Diego; van Gompel, Fons; Visser, Leo G.; Siegrist, Claire-Anne; Hatz, Christoph

    2015-01-01

    BACKGROUND The number of individuals with autoimmune inflammatory rheumatic diseases (AIIRDs) treated with immunosuppressive drugs is increasing steadily. The variety of immunosuppressive drugs and, in particular, biological therapies is also rising. The immunosuppressants, as well as the AIIRD itself, increase the risk of infection in this population. Thus, preventing infections by means of vaccination is of utmost importance. New Swiss vaccination recommendations for AIIRD patients were in...

  3. HIV Risk Reduction Among Young Adult Chronic Psychiatric Patients

    Science.gov (United States)

    1990-08-28

    test was subsequently licensed in 1985, Even though HlV -antibodles were nearly universal in AIDS patients, the antibodies serve no apparent protective...for Disease Control, 1989; Confronting AIDS Directions for Public Health. Health Care, and Research. 1986), Although some temporizing drug therapy...sharing of needles by Intravenous drug users (IVDA), and transplacental spread, The most dangerous types of sexual contact are anal and vaginal

  4. Effectiveness of Sulpiride in Adult Patients With Schizophrenia

    OpenAIRE

    2012-01-01

    The objective of this study is to compare the effectiveness among sulpiride, risperidone, olanzapine, and haloperidol by evaluating the persistence of drug use. A retrospective cohort study was conducted by analyzing the National Health Insurance Research Database of Taiwan. Patients with schizophrenia aged 18–65 years and newly prescribed with a single oral antipsychotic medication between years 2003 and 2008 were included. The primary outcome was the persistence of antipsychotic agents by c...

  5. Treatment of adult short bowel syndrome patients with teduglutide

    DEFF Research Database (Denmark)

    Nørholk, Lærke Marijke; Holst, Jens Juul; Jeppesen, Palle Bekker

    2012-01-01

    INTRODUCTION: Parenteral support is lifesaving in short bowel syndrome patients with intestinal failure (SBS-IF), who are unable to compensate for their malabsorption by metabolic or pharmacologic adaptation. Mutually, the symptoms of SBS-IF and the inconveniences and complications in relation...... support were obtained. EXPERT OPINION: Teduglutide seems to be safe and well-tolerated and demonstrates restoration of structural and functional integrity of the remaining intestine with significant intestinotrophic and proabsorptive effects, facilitating a reduction in diarrhea and an equivalent...

  6. Is the medical home for adult patients with sickle cell disease a reality or an illusion?

    Science.gov (United States)

    Ballas, Samir K; Vichinsky, Elliott P

    2015-01-01

    Recently, the patient-centered medical home (PCMH) emerged as a viable method to improve delivery of medical care. Due to all the promotion about the effectiveness of the PCMH, patients with sickle cell disease, their families and the community hoped that this could be a possible solution to the problems that arise in the treatment of adult patients with sickle cell disease. Review of the literature and review of the criteria for the establishment of a PCMH show that the PCMH is not an ideal model for patients with sickle cell disease because finding a personal physician, which is the first criteria of a functional PCMH, is a major problem in the process of transitioning the care of patients with sickle cell disease from pediatrics to adult care. Moreover, garnering hospital support to defray the initial costs to establish a PCMH for adults with sickle cell disease is unlikely given the already high costs of care for patients with sickle cell disease. Moreover, recent studies have shown insufficient evidence to determine the presumed beneficial effects of the PCMH, especially in patients with chronic disease.

  7. Loss of olfactory function and nutritional status in vital older adults and geriatric patients.

    Science.gov (United States)

    Toussaint, Nicole; de Roon, Margot; van Campen, Jos P C M; Kremer, Stefanie; Boesveldt, Sanne

    2015-03-01

    The aim of this cross-sectional study was to assess the association of olfactory function and nutritional status in vital older adults and geriatric patients. Three hundred forty-five vital (mean age 67.1 years) and 138 geriatric older adults (mean age 80.9 years) were included. Nutritional status was assessed using the mini nutritional assessment-short form. The Sniffin' Sticks was used to measure olfactory function. Eleven percentage of the vital older adults were at risk of malnutrition, whereas 60% of the geriatric participants were malnourished or at risk. Only 2% of the vital older adults were anosmic, compared with 46% of the geriatric participants. Linear regression demonstrated a significant association (P = 0.015) between olfactory function and nutritional status in the geriatric subjects. However, this association became insignificant after adjustment for confounders. Both crude and adjusted analysis in the vital older adults did not show a significant association. The results indicate that, in both groups of elderly, there is no direct relation between olfactory function and nutritional status. We suggest that a decline in olfactory function may still be considered as one of the risk-factors for malnutrition in geriatric patients-once co-occurring with other mental and/or physical problems that are more likely to occur in those patients experience.

  8. The moyamoya disease susceptibility variant RNF213 R4810K (rs112735431) induces genomic instability by mitotic abnormality

    Energy Technology Data Exchange (ETDEWEB)

    Hitomi, Toshiaki [Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Habu, Toshiyuki [Radiation Biology Center, Kyoto University, Kyoto (Japan); Kobayashi, Hatasu; Okuda, Hiroko; Harada, Kouji H. [Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Osafune, Kenji [Center for iPS Cell Research and Application (CiRA), Kyoto University, Kyoto (Japan); Taura, Daisuke; Sone, Masakatsu [Department of Medicine and Clinical Science, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Asaka, Isao; Ameku, Tomonaga; Watanabe, Akira; Kasahara, Tomoko; Sudo, Tomomi; Shiota, Fumihiko [Center for iPS Cell Research and Application (CiRA), Kyoto University, Kyoto (Japan); Hashikata, Hirokuni; Takagi, Yasushi [Department of Neurosurgery, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Morito, Daisuke [Faculty of Life Sciences, Kyoto Sangyo University, Kyoto (Japan); Miyamoto, Susumu [Department of Neurosurgery, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Nakao, Kazuwa [Department of Medicine and Clinical Science, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Koizumi, Akio, E-mail: koizumi.akio.5v@kyoto-u.ac.jp [Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto University, Kyoto (Japan)

    2013-10-04

    Highlights: •Overexpression of RNF213 R4810K inhibited cell proliferation. •Overexpression of RNF213 R4810K had the time of mitosis 4-fold and mitotic failure. •R4810K formed a complex with MAD2 more readily than wild-type. •iPSECs from the MMD patients had elevated mitotic failure compared from the control. •RNF213 R4810K induced mitotic abnormality and increased risk of aneuploidy. -- Abstract: Moyamoya disease (MMD) is a cerebrovascular disease characterized by occlusive lesions in the Circle of Willis. The RNF213 R4810K polymorphism increases susceptibility to MMD. In the present study, we characterized phenotypes caused by overexpression of RNF213 wild type and R4810K variant in the cell cycle to investigate the mechanism of proliferation inhibition. Overexpression of RNF213 R4810K in HeLa cells inhibited cell proliferation and extended the time of mitosis 4-fold. Ablation of spindle checkpoint by depletion of mitotic arrest deficiency 2 (MAD2) did not shorten the time of mitosis. Mitotic morphology in HeLa cells revealed that MAD2 colocalized with RNF213 R4810K. Immunoprecipitation revealed an RNF213/MAD2 complex: R4810K formed a complex with MAD2 more readily than RNF213 wild-type. Desynchronized localization of MAD2 was observed more frequently during mitosis in fibroblasts from patients (n = 3, 61.0 ± 8.2%) compared with wild-type subjects (n = 6, 13.1 ± 7.7%; p < 0.01). Aneuploidy was observed more frequently in fibroblasts (p < 0.01) and induced pluripotent stem cells (iPSCs) (p < 0.03) from patients than from wild-type subjects. Vascular endothelial cells differentiated from iPSCs (iPSECs) of patients and an unaffected carrier had a longer time from prometaphase to metaphase than those from controls (p < 0.05). iPSECs from the patients and unaffected carrier had significantly increased mitotic failure rates compared with controls (p < 0.05). Thus, RNF213 R4810K induced mitotic abnormalities and increased risk of genomic instability.

  9. Upper and lower urinary tract outcomes in adult myelomeningocele patients: a systematic review.

    Directory of Open Access Journals (Sweden)

    Paul W Veenboer

    Full Text Available BACKGROUND: The introduction of sophisticated treatment of bladder dysfunction and hydrocephalus allows the majority of SB patients to survive into adulthood. However, no systematic review on urological outcome in adult SB patients is available and no follow-up schemes exist. OBJECTIVES: To systematically summarize the evidence on outcome of urinary tract functioning in adult SB patients. METHODS: A literature search in PubMed and Embase databases was done. Only papers published in the last 25 years describing patients with open SB with a mean age >18 years were included. We focused on finding differences in the treatment strategies, e.g., clean intermittent catheterization and antimuscarinic drugs versus early urinary diversion, with regard to long-term renal and bladder outcomes. RESULTS: A total of 13 articles and 5 meeting abstracts on urinary tract status of adult SB patients were found describing a total of 1564 patients with a mean age of 26.1 years (range 3-74 years, with a few patients <18 years. All were retrospective cohort studies with relatively small and heterogeneous samples with inconsistent reporting of outcome; this precluded the pooling of data and meta-analysis. Total continence was achieved in 449/1192 (37.7%; range 8-85% patients. Neurological level of the lesion and hydrocephalus were associated with incontinence. Renal function was studied in 1128 adult patients. In 290/1128 (25.7%; range 3-81.8% patients some degree of renal damage was found and end-stage renal disease was seen in 12/958 (1.3% patients. Detrusor-sphincter dyssynergy and detrusor-overactivity acted as adverse prognostic factors for the development of renal damage. CONCLUSIONS: These findings should outline follow-up schedules for SB patients, which do not yet exist. Since renal and bladder deterioration continues beyond adolescence, follow-up of these individuals is needed. We recommend standardization in reporting the outcome of urinary tract function in

  10. Patient Priority-Directed Decision Making and Care for Older Adults with Multiple Chronic Conditions.

    Science.gov (United States)

    Tinetti, Mary E; Esterson, Jessica; Ferris, Rosie; Posner, Philip; Blaum, Caroline S

    2016-05-01

    Older adults with multiple conditions receive care that is often fragmented, burdensome, and of unclear benefit. An advisory group of patients, caregivers, clinicians, health system engineers, health care system leaders, payers, and others identified three modifiable contributors to this fragmented, burdensome care: decision making and care focused on diseases, not patients; inadequate delineation of roles and responsibilities and accountability among clinicians; and lack of attention to what matters to patients and caregivers (ie, their health outcome goals and care preferences). The advisory group identified patient priority-directed care as a feasible, sustainable approach to addressing these modifiable factors.

  11. Treatment of Acute Myeloid Leukemia in Adolescent and Young Adult Patients

    Directory of Open Access Journals (Sweden)

    Guldane Cengiz Seval

    2015-03-01

    Full Text Available The objectives of this review were to discuss standard and investigational treatment strategies for adolescent and young adult with acute myeloid leukemia, excluding acute promyelocytic leukemia. Acute myeloid leukemia (AML in adolescent and young adult patients (AYAs may need a different type of therapy than those currently used in children and older patients. As soon as AML is diagnosed, AYA patient should be offered to participate in well-designed clinical trials. The standard treatment approach for AYAs with AML is remission induction chemotherapy with an anthracycline/cytarabine combination, followed by either consolidation chemotherapy or stem cell transplantation, depending on the ability of the patient to tolerate intensive treatment and cytogenetic features. Presently, continuing progress of novel drugs targeting specific pathways in acute leukemia may bring AML treatment into a new era.

  12. Successful Non-Invasive treatment of stricturing fibrosing colonopathy in an adult patient

    Directory of Open Access Journals (Sweden)

    Terheggen G

    2011-09-01

    Full Text Available Abstract Objective Fibrosing colonopathy (FC is a rare entity associated with cystic fibrosis (CF. Until now, patients with stricturing FC have usually been treated surgically. In this instance, we aimed at avoiding surgery by applying a new conservative approach. Methods Case report on an adult with CF who developed persistent abdominal pain due to a non-passable stricture in the right transverse colon. Histology confirmed fibrosing colonopathy. Results Initially we treated the patient with prednisolone pulse therapy and additive antibiotic therapy. For maintenance therapy we administered budesonide. The patient underwent clinical, laboratory and endoscopic follow-up over a three-year period. The stricture healed and was easy to pass. A relapse in the cecum at the ileocecal valve again improved under steroid and antibiotic therapy. Conclusions We present a novel therapeutic approach for advanced stricturing FC in an adult patient which successfully avoided surgery (right hemicolectomy over a three year follow up.

  13. Comparative Study of Early Maladaptive Schemas in Rheumatoid Arthrits Patients and Normal Adults

    Directory of Open Access Journals (Sweden)

    Fatemeh Rezaei

    2014-01-01

    Full Text Available Early maladaptive schemas (EMSs designed to ases early distres. EMSs are at he core of personality pathology and psychological distres. The main objective of this study was to find out he diferences betwen rheumatoid arthrits (RA patients and normal adults on EMSs. 10 RA patients and 10 normal adults completed Young’s Schema Questionaire developed by Jefery Young (198. The results showed that data was subjected to statistical analysis; T- test showed that the RA patients reported a signifcantly greater severity of early maladaptive schemata than the normal subjects. This study sugested that a remarkable amount of RA patients may sufer from EMSs which have an efect on their pain situation. These findings confirm those pieces of evidence indicating the psychological treatments included in multidisciplinary programs for this disorder.

  14. 鲁西南地区烟雾病临床特点及其外科治疗策略%Clinical characteristics of Moyamoya disease in the southwest of Shandong Province and its surgical treatment strategies

    Institute of Scientific and Technical Information of China (English)

    冯嵩; 马辉福; 张浩; 孔令胜; 靳峰

    2015-01-01

    目的 探讨鲁西南地区烟雾病临床特点和外科治疗经验.方法 对自2011年3月至2013年12月济宁医学院附属医院神经外科收治的96例烟雾病患者临床资料进行回顾性分析,并将分析结果与日韩及国内相关报道分析比较.结果 鲁西南地区烟雾病在发病年龄、性别比及临床表现方面具有特殊性,与日韩及国内相关报道不完全一致.具体表现为:女性多于男性(与日韩相似);有0~10岁及31~50岁2个发病高峰期(与日韩相似);但发病年龄更集中(与国内报道相似)且儿童发病率(12.5%)明显低于日韩;临床表现以缺血型为主(80.2%),但短暂脑缺血发作(TIA)的发生率高(高于日韩及国内报道),癫痫和脑梗死的发生率低(低于日韩及国内报道),脑出血的发生率(19.8%)与日本及国内报道相似,明显低于韩国.经颅内外血管重建术治疗后大部分患者的临床症状得到改善,与日韩及国内相关报道的治疗效果无明显差异.结论 鲁西南地区的烟雾病临床特点具有特殊性,其发病机制可能与日韩存在差异;颅内外血管重建术治疗烟雾病临床效果显著,是治疗烟雾病的有效方法.%Objective To explore the clinical characteristics of patients with Moyamoya disease in the southwest of Shandong province and evaluate the efficacy of cerebral revascularization treatment in it.Methods Clinical data of 96 patients with Moyamoya disease,admitted to our hospital form March 2011 to December 2013,were analyzed retrospectively and compared with those in Japan,South Korea and Inland.Results The clinical characteristics of patients with Moyamoya disease in the southwest of Shandong province were similar with that in Japan,South Korea and Inland,but enjoyed their own particularity:the age distribution showed two peaks (aged 0-10 and aged 31-50) and the sex ratio (female>male) was similar to those among Japanese and Korean patients,but the age distribution was

  15. The Impact of Loneliness on Quality of Life and Patient Satisfaction Among Older, Sicker Adults

    OpenAIRE

    Shirley Musich PhD; Shaohung S. Wang PhD; Kevin Hawkins PhD; Charlotte S. Yeh MA, FACEP

    2015-01-01

    Objective: This study estimated prevalence rates of loneliness, identified characteristics associated with loneliness, and estimated the impact of loneliness on quality of life (QOL) and patient satisfaction. Method: Surveys were mailed to 15,500 adults eligible for care management programs. Loneliness was measured using the University of California Los Angeles (UCLA) three-item scale, and QOL using Veteran’s RAND 12-item (VR-12) survey. Patient satisfaction was measured on a 10-point scale. ...

  16. Effects of chondroitin sulfate and glucosamine in adult patients with Kaschin-Beck disease

    DEFF Research Database (Denmark)

    Zhang, Ya-xu; Dong, Wei; Liu, Hui;

    2010-01-01

    The purpose is to investigate the effects of chondroitin sulfate and glucosamine on adult patients with Kaschin-Beck disease (KBD). A total of 80 patients, aged over 40 years, were randomized into two groups receiving either 1,600 mg oral mixture of chondroitin sulfate and glucosamine or placebo......). But the overall mean change in joint space was significant between the two groups (P glucosamine might play a protective role in preserving articular cartilage and provide...

  17. Clinical effectiveness of first and repeat influenza vaccination in adult and elderly diabetic patients

    NARCIS (Netherlands)

    Looijmans-Van den Akker, I.; Verheij, T.J.M.; Buskens, E.; Nichol, K.L.; Rutten, G.E.H.M.; Hak, E.

    2006-01-01

    OBJECTIVE: Influenza vaccine uptake remains low among the high-risk group of patients with diabetes, partly because of conflicting evidence regarding its potential benefits. We assessed the clinical effectiveness of influenza vaccination in adults with diabetes and specifically examined potential mo

  18. Reduced corticosteroid use in adult patients with primary immune thrombocytopenia receiving romiplostim

    NARCIS (Netherlands)

    M. Michel (Marc); P.A.W. te Boekhorst (Peter); A. Janssens (Andre); I. Pabinger-Fasching (Ingrid); M.A. Sanz (Miguel Angel); K. Nie (Kun); G. Kreuzbauer (Georg)

    2011-01-01

    textabstractAdult patients with primary immune thrombocytopenia requiring first-line treatment typically receive corticosteroids, which are associated with low response rates and many potential side effects. In a retrospective analysis of two 6-month, placebo-controlled, phase III trials, corticoste

  19. The relationships among separation anxiety disorder, adult attachment style and agoraphobia in patients with panic disorder.

    Science.gov (United States)

    Pini, Stefano; Abelli, Marianna; Troisi, Alfonso; Siracusano, Alberto; Cassano, Giovanni B; Shear, Katherine M; Baldwin, David

    2014-12-01

    Epidemiological studies indicate that separation anxiety disorder occurs more frequently in adults than children. It is unclear whether the presence of adult separation anxiety disorder (ASAD) is a manifestation of anxious attachment, or a form of agoraphobia, or a specific condition with clinically significant consequences. We conducted a study to examine these questions. A sample of 141 adult outpatients with panic disorder participated in the study. Participants completed standardized measures of separation anxiety, attachment style, agoraphobia, panic disorder severity and quality of life. Patients with ASAD (49.5% of our sample) had greater panic symptom severity and more impairment in quality of life than those without separation anxiety. We found a greater rate of symptoms suggestive of anxious attachment among panic patients with ASAD compared to those without ASAD. However, the relationship between ASAD and attachment style is not strong, and adult ASAD occurs in some patients who report secure attachment style. Similarly, there is little evidence for the idea that separation anxiety disorder is a form of agoraphobia. Factor analysis shows clear differentiation of agoraphobic and separation anxiety symptoms. Our data corroborate the notion that ASAD is a distinct condition associated with impairment in quality of life and needs to be better recognized and treated in patients with panic disorder.

  20. Fractures in pituitary adenoma patients from the Dutch National Registry of Growth Hormone Treatment in Adults

    NARCIS (Netherlands)

    N.C. Van Varsseveld; C.C. van Bunderen (Christa); A.A.M. Franken (Anton); H.P.F. Koppeschaar (Hans); A-J. van der Lely (Aart-Jan); M.L. Drent (Madeleine)

    2016-01-01

    textabstractPurpose: The effects of growth hormone (GH) replacement therapy on fracture risk in adult GH deficient (GHD) patients with different etiologies of pituitary GHD are not well known, due to limited data. The aim of this study was to investigate characteristics and fracture occurrence at st

  1. A computerized algorithm for arousal detection in healthy adults and patients with Parkinson disease

    DEFF Research Database (Denmark)

    Sørensen, Gertrud Laura; Jennum, Poul; Kempfner, Jacob

    2012-01-01

    polysomnographic (PSG) recordings from a total of 24 subjects. Eight of the subjects were diagnosed with Parkinson disease (PD) and the rest (16) were healthy adults in various ages. The performance of the algorithm was validated in 3 settings: testing on the 8 patients with PD using the leave-one-out method...

  2. Myotonia and flaccid dysarthria in patients with adult onset myotonic dystrophy.

    NARCIS (Netherlands)

    Swart, B.J.M. de; Engelen, B.G.M. van; Kerkhof, J.P. van de; Maassen, B.A.M.

    2004-01-01

    BACKGROUND: Myotonia and weakness are the most important components of dysarthric speech in myotonic dystrophy. OBJECTIVE: To specify and quantify possible defects in speech execution in patients with adult onset myotonic dystrophy. METHODS: Studies on speech production were done on 30 mildly affect

  3. An Examination of Intimate Partner Violence and Psychological Stressors in Adult Abortion Patients

    Science.gov (United States)

    Ely, Gretchen E.; Otis, Melanie D.

    2011-01-01

    The purpose of this article is to describe an exploratory study examining the relationship between intimate partner violence and psychological stressors in a sample of 188 adult abortion patients. Results indicate the almost 15% of respondents report a history of abuse by the coconceiving partner. In addition, women who reported having had one or…

  4. Application value of SPECT-CT fusion imaging in moyamoya disease revascularization:a clinical study%SPECT与CT影像融合在烟雾病血管重建术中的应用价值

    Institute of Scientific and Technical Information of China (English)

    齐辉; 赵雷; 吴广源; 郑思阳; 黄达; 尹卫; 杨红杰; 贾少微

    2014-01-01

    目的:探讨烟雾病直接血管重建术前应用SPECT脑灌注成像与同机CT影像融合确定脑缺血灶体表位置并以此设计手术入路的应用价值。方法回顾性分析13例成年缺血型烟雾病颞浅动脉-大脑中动脉旁路移植术患者的临床资料,术前将单光子发射体层摄影( SPECT)脑灌注成像与同机CT影像融合,将皮质缺血灶在体表定位,以缺血灶为中心设计手术入路。术后随访6~12个月,观察术后临床症状的改善以及术后脑灌注改善情况。结果围手术期有1例脑高灌注综合征,其余无其他围手术期并发症发生。随访1个月时症状改善优秀者4例,良好者5例,一般者为4例,差者为0;随访6~12个月时症状改善优秀者9例,良好者4例,一般者为0,差者为0。术后SPECT脑灌注成像视觉分析提示全部患者脑灌注较术前明显改善;定量分析:目标缺血灶术前术后试验前各病灶/全脑计数比值(Fb)值差异有统计学意义[(2.13±1.06)%比(4.13±2.09)%, P50%,提示手术疗效显著。患者术后头部CT血管成像均可见颞浅动脉向颅内供血,术后半年复查头部MRI无新增梗死灶。结论SPECT脑灌注成像与同机CT影像融合的方法用于颞浅动脉-大脑中动脉旁路移植术的手术入路设计,有助于提高手术疗效,降低手术风险。%Objective To investigate the application value of identification of the scalp surface locations of cerebral ischemia lesions before direct revascularization for moyamoya disease and to design surgical approaches according to this by using the fusion of single photon emission computed tomography ( SPECT) cerebral perfusion imaging with CT imaging. Methods The clinical data of 13 adult patients with ischemic-type moyamoya disease underwent superficial temporal artery-middle cerebral artery bypass surgery were analyzed retrospectively. SPECT cerebral perfusion imaging was fused with CT imaging of the same machine before procedure

  5. Routine radiography does not have a role in the diagnostic evaluation of ambulatory adult febrile neutropenic cancer patients

    NARCIS (Netherlands)

    Nijhuis, CSMO; Gietema, JA; Vellenga, E; Daenen, SMGJ; De Bont, ESJM; Kamps, WA; Groen, HJM; van der Jagt, EJ; van der Graaf, WTA

    2003-01-01

    Cancer patients treated with chemotherapy are susceptible to bacterial infections. When an adult patient presents with febrile neutropenia. standard diagnostic care includes physical examination, laboratory diagnostics, chest X-ray (CXR) and sinus radiography. However, the yield of routine radiograp

  6. Pathology of nondiabetic glomerular disease among adult Iraqi patients from a single center

    Directory of Open Access Journals (Sweden)

    Hashim Al-Saedi Ali

    2009-01-01

    Full Text Available Almost all forms of glomerular diseases have been reported in diabetics. In a recent series, 12% of those with type I and 27% of those with type II diabetes were found to have non diabetic renal disease. We studied 80 adult diabetic Iraqi patients who were diagnosed with glo-merular disease on native kidney biopsies from January 2000 to April 2008. Membranoproliferative GN was seen in 32 patients (40%, Focal and Segmental glomerulosclerosis in 16 patients (20%, Membranous nephropathy in 20 patients (25%, Minimal change disease in 8 patients (10%, Renal amyloidosis in 4 patients (5%. In conclusion Membranoproliferative GN was the most common histological diagnosis in our diabetic patients undergoing renal biopsy.

  7. CT diagnosis of suspected acute appendicitis in adult patients

    Energy Technology Data Exchange (ETDEWEB)

    Yamase, Hiroshi; Sahashi, Kiyomi; Kawai, Masayuki; Kishida, Yoshihiko; Sumida, Kei; Kawamura, Ken-ichi [Gifu Syakaihoken Hospital (Japan)

    1998-06-01

    In order to assess the CT diagnosis of suspected acute appendicitis, we performed abdominal contrasted CT measurements in 77 patients from 20 to 86 years old, and of 50 men and 27 women from June 1993 to June 1996. The surgical findings were compared with the preoperative CT findings. By the preoperative CT imaging, we can know the degree and the position of inflammation in appendix vermiformis and the degree and the spread of periappendicular inflammation in the case of appendicitis, and can make a differential diagnosis of diverticulitis or gynecological diseases from appendicitis. It is important to make a preoperative diagnosis by the objectively excellent abdominal CT imaging and to avoid unnecessary surgery. (K.H.)

  8. Intrusion of incisors in adult patients with marginal bone loss.

    Science.gov (United States)

    Melsen, B; Agerbaek, N; Markenstam, G

    1989-09-01

    Elongated and spaced incisors are common problems in patients suffering from severe periodontal disease. Thirty patients characterized by marginal bone loss and deep overbite were treated by intrusion of incisors. Three different methods for intrusion were applied: (1) J hooks and extraoral high-pull headgear, (2) utility arches, (3) intrusion bent into a loop in a 0.17 x 0.25-inch wire, and (4) base arch as described by Burstone. The intrusion was evaluated from the displacement of the apex, incision, and the center of resistance of the most prominent or elongated central incisor. Change in the marginal bone level and the amount of root resorption were evaluated on standardized intraoral radiographs. The pockets were assessed by standardized probing and the clinical crown length was measured on study casts. The results showed that the true intrusion of the center of resistance varied from 0 to 3.5 mm and was most pronounced when intrusion was performed with a base arch. The clinical crown length was generally reduced by 0.5 to 1.0 mm. The marginal bone level approached the cementoenamel junction in all but six cases. All cases demonstrated root resorption varying from 1 to 3 mm. The total amount of alveolar support--that is, the calculated area of the alveolar wall--was unaltered or increased in 19 of the 30 cases. The dependency of the results on the oral hygiene, the force distribution, and the perioral function was evaluated in relation to the individual cases. It was obvious that intrusion was best performed when (1) forces were low (5 to 15 gm per tooth) with the line of action of the force passing through or close to the center of resistance, (2) the gingiva status was healthy, and (3) no interference with perioral function was present.

  9. Psychiatric stigma in treatment seeking adults with personality problems: evidence from a sample of 214 patients.

    Directory of Open Access Journals (Sweden)

    Kirsten eCatthoor

    2015-07-01

    Full Text Available Stigmatization is a major hindrance in adult psychiatric patients with Axis-I diagnoses, as shown consistently in most studies. Significantly fewer studies on the emergence of psychiatric stigma in adult patients with personality disorders exist, although the resulting evidence is conclusive. Some authors consider patients with personality disorders at risk for severe stigmatization because of intense difficulties during interpersonal contact, even in a psychotherapeutic relationship. The aim of this study was primarily the assessment of pre-existing stigma in patients referred for intensive treatment for personality disorders. The study enrolled 214 patients admitted to the adult department of a highly specialized mental health care institute offering psychotherapy for patients with severe and complex personality pathology. All patients underwent a standard assessment with self-report questionnaires and a semi-structured interview to measure Axis II personality disorders. The Stigma Consciousness Questionnaire (SCQ and the Perceived Devaluation-Discrimination Questionnaire (DDQ, both validated instruments, were used to measure perceived and actual experiences of stigma. Independent sample t-tests were used to investigate differences in the mean total stigma scores for patients both with and without a personality disorder. One-way ANOVA’s were performed to assess the differences between having a borderline personality disorder, another personality disorder, or no personality disorder diagnosis.Multiple regression main effect analyses were conducted in order to explore the impact of the different personality disorder diagnosis on the level of stigma. The mean scores across all patient groups were consistent with rather low stigma. No differences were found for patients with or without a personality disorder diagnosis. Level of stigma in general was not associated with an accumulating number of personality disorders.

  10. Altered aiming movements in Parkinson's disease patients and elderly adults as a function of delays in movement onset

    NARCIS (Netherlands)

    Romero, D.H.; Gemmert, A.W.A. van; Adler, C.H.; Bekkering, H.; Stelmach, G.E.

    2003-01-01

    This study investigated the effect of lengthening the time the hand remains immobilized on an aiming movement performed by Parkinson's disease (PD) patients and elderly adults, and whether visual information could compensate for the effects of delay. In Experiment One, PD patients and elderly adults

  11. Anemia among adult HIV patients in Ethiopia: a hospital-based cross-sectional study

    Science.gov (United States)

    Melese, Hermela; Wassie, Molla Mesele; Woldie, Haile; Tadesse, Abilo; Mesfin, Nebiyu

    2017-01-01

    Background Anemia is a major public health problem in HIV patients around the world. It has a negative effect on the quality of life of HIV patients and progression of the HIV disease. In the sub-Saharan African setting, including Ethiopia where both HIV infection and under-nutrition are expected to be high, there is a paucity of data on the matter. This study was aimed to reveal the magnitude and factors associated with anemia among adult HIV patients in Debre-Tabor Hospital, northwest Ethiopia. Methods A hospital-based cross-sectional study was used among adult HIV patients in Debre-Tabor Hospital from April 1 to May 30, 2015. The diagnosis of anemia was made following the 2011 World Health Organization recommendation on hemoglobin cut-off points. Univariable and multivariable logistic regression was carried out to assess factors associated with anemia. Results A total of 377 patients’ charts were reviewed. Most of the participants (n=237, 62.9%) were taking antiretroviral treatment (ART). The overall prevalence of anemia was 23% (95% CI: 19.1, 27.6). Being ART-naïve (adjusted odds ratio [AOR]: 3.37; 95% CI: 1.59, 7.14), having treatment history with anti-tuberculosis (TB) drug (AOR: 3.2; 95% CI: 1.19, 8.67), taking zidovudine (ZDV)-containing ART regimen (AOR: 2.14; 95% CI: 1.03, 4.57), and having recent CD4+ T-lymphocytes count of HIV patients. Conclusion and recommendation Anemia continues to be a major co-morbidity among adult HIV patients in Ethiopia. Adult HIV patients who are taking ZDV-containing ART, with a history of TB treatment, have a low CD4+T-lymphocytes count and are ART-naïve should be carefully screened and treated for anemia. PMID:28243151

  12. Prevalence of alcohol problems among adult somatic in-patients in Naples

    DEFF Research Database (Denmark)

    Rambaldi, A; Gluud, C; Belli, A;

    1995-01-01

    The aim of this cross-sectional study was to determine the prevalence of alcohol problems among adult somatic in-patients in urban hospitals of Naples. The patients were screened with a structured questionnaire regarding life style. After discharge, the patient records were examined and the hospi......The aim of this cross-sectional study was to determine the prevalence of alcohol problems among adult somatic in-patients in urban hospitals of Naples. The patients were screened with a structured questionnaire regarding life style. After discharge, the patient records were examined...... and the hospital discharge diagnoses were registered. A patient was considered having an alcohol problem if one or more of the following criteria were fulfilled: (1) a Michigan Alcoholism Screening Test score at or above five; (2) a self-reported daily consumption for at least 2 years of at least 60 g of ethanol...... for males and 36 g for females; (3) an alcohol-related discharge diagnosis. The prevalence of patients with alcohol problems was significantly (P

  13. Subclinical anaemia of chronic disease in adult patients with cystic fibrosis.

    LENUS (Irish Health Repository)

    O'connor, T M

    2012-02-03

    Patients with chronic hypoxaemia develop secondary polycythaemia that improves oxygen-carrying capacity. Therefore, normal haemoglobin and haematocrit values in the presence of chronic arterial hypoxaemia in cystic fibrosis constitute \\'relative anaemia\\'. We sought to determine the cause of this relative anaemia in patients with cystic fibrosis. We studied haematological indices and oxygen saturation in healthy volunteers (n=17) and in adult patients with cystic fibrosis (n=15). Patients with cystic fibrosis had lower resting arterial oxygen saturation when compared with normal volunteers (P<0.0001), and exercise led to a greater reduction in arterial oxygen saturation (P<0.0001). However, haemoglobin and haematocrit values in patients with cystic fibrosis did not significantly differ from normal volunteers. Serum iron (P=0.002), transferrin (P=0.02), and total iron-binding capacity (P=0.01) were lower in patients with cystic fibrosis. There were no significant differences in serum ferritin, percentage iron saturation, serum erythropoietin or red cell volume between the groups. The data presented demonstrate a characteristic picture of anaemia of chronic disease in adult patients with cystic fibrosis, except for normal haemoglobin and haematocrit values. Normal haemoglobin and haematocrit values in patients with cystic fibrosis appear to represent a combination of the effects of arterial hypoxaemia promoting polycythaemia, counterbalanced by chronic inflammation promoting anaemia of chronic disease.

  14. Clinical and MRI findings of cerebellar agenesis in two living adult patients

    Directory of Open Access Journals (Sweden)

    Fazil Mustafa Gelal

    2016-01-01

    Full Text Available Cerebellar agenesis (CA is an extremely rare entity. We present two adult patients with CA. The 61-year-old man had ataxia, dysarthria, abnormalities in cerebellar tests, severe cognitive impairment, and moderate mental retardation. The 26-year-old woman had dysmetria, dysdiadochokinesia, and dysarthria as well as mild cognitive impairment and mild mental retardation. Magnetic resonance imaging (MRI showed complete absence of the cerebellum with small residual vermis. Brainstem was hypoplastic and structures above tentorium were normal. Supratentorial white matter bundles were unaffected in diffusion tensor tractography. Only few adult patients with CA have so far been published. These cases show that patients with CA present with a variety of developmental, clinical, and mental abnormalities; and emphasize the role of the cerebellum in normal motor, language, and mental development.

  15. Gender differences among young adult cancer patients: a study of blogs.

    Science.gov (United States)

    Kim, Bora; Gillham, David

    2015-01-01

    The Internet has increasing relevance and practical use as a tool to support cancer care. For example, health support Web sites are now widely used to connect specific groups of patients who may otherwise have remained isolated, and understanding their health-related online behaviors will help in the development of more effective health support Web sites. This article examined blogs written by young adults affected by cancer and in particular examined the gender differences in these blog entries through content analysis. The results showed there is little difference in blog content between genders. This suggests that the blog environment could lessen the gender-typical behaviors often expected by society and may provide an outlet for young adult cancer patients to more freely share their cancer-related experiences, at the same time providing an opportunity for social connection. This is particularly significant for male patients who are known to inhibit their emotions as well as the expression of their health concerns.

  16. Theory of mind deficit in adult patients with congenital heart disease.

    Science.gov (United States)

    Chiavarino, Claudia; Bianchino, Claudia; Brach-Prever, Silvia; Riggi, Chiara; Palumbo, Luigi; Bara, Bruno G; Bosco, Francesca M

    2015-10-01

    This article provides the first assessment of theory of mind, that is, the ability to reason about mental states, in adult patients with congenital heart disease. Patients with congenital heart disease and matched healthy controls were administered classical theory of mind tasks and a semi-structured interview which provides a multidimensional evaluation of theory of mind (Theory of Mind Assessment Scale). The patients with congenital heart disease performed worse than the controls on the Theory of Mind Assessment Scale, whereas they did as well as the control group on the classical theory-of-mind tasks. These findings provide the first evidence that adults with congenital heart disease may display specific impairments in theory of mind.

  17. Concomitant therapies (glucocorticoids and sex hormones) in adult patients with growth hormone deficiency.

    Science.gov (United States)

    Scaroni, C; Ceccato, F; Rizzati, S; Mantero, F

    2008-09-01

    Adult-onset GH deficiency (GHD), mostly due to organic lesions of the pituitary-hypothalamic region, is frequently associated with multiple anterior pituitary deficiencies that need long-term substitutive treatment. The GH-IGF-I axis may play an important role in modulating peripheral metabolism of hormones (adrenal, thyroid, and sex hormones) and these interactions may have clinically significant implications on the phenotypes of adult GHD patients and on the effects of the combined replacement hormonal treatment of this condition. By accelerating the peripheral metabolism of cortisol, GH therapy may precipitate adrenal insufficiency in susceptible hypopituitary patients; estrogen replacement blunts the response to GH in women whereas in men with androgen substitution the responsivity increases over time. Endocrinologists should be mindful of these phenomena when starting patients with hypopituitarism on GH replacement therapy.

  18. THE MEANING OF INSULIN PUMPE THERAPY TO ADULT PATIENTS WITH TYPE 1 DIABETES

    DEFF Research Database (Denmark)

    Nissen, Heidi; Aagaard, Hanne

    and meaningfulness in relation to diabetes self-care and self-management. The bolus guide, as a rather new feature, seem to play an important role. Conclusion: Based on The Shifting Perspectives Model of Chronic Illness, we concluded that a well established insulin pump therapy lead to changing the patients......Background: Insulin pump therapy is an increasing field. Studies have documented a clinical relevant decrease in HbA1c, especially among adults type 1 diabetes patients with initial high HbA1c. However, only few studies investigate the lived experience with and the meaning of the insulin pump...... therapy in adulthood. Aim: The study explore the lived experiences and the meaning of insulin pump therapy in adulthood. Method: The study is based on a phenomenological – hermeneutic approach. Four adult type 1 diabetes patients were interviewed about their insulin pump therapy. The interviews were based...

  19. MRI in adult patients with aortic coarctation: diagnosis and follow-up.

    Science.gov (United States)

    Shepherd, B; Abbas, A; McParland, P; Fitzsimmons, S; Shambrook, J; Peebles, C; Brown, I; Harden, S

    2015-04-01

    Aortic coarctation is a disease that usually presents in infancy; however, a proportion of patients present for the first time in adulthood. These lesions generally require repair with either surgery or interventional techniques. The success of these techniques means that increasing numbers of patients are presenting for follow-up imaging in adulthood, whether their coarctation was initially repaired in infancy or as adults. Thus, the adult presenting to the radiologist for assessment of possible coarctation or follow-up of coarctation repair is not an uncommon scenario. In this review, we present details of the MRI protocols and MRI findings in these patients so that a confident and accurate assessment can be made.

  20. Combined duplication of the colon and vermiform appendix in an adult patient

    Institute of Scientific and Technical Information of China (English)

    Sahin Kabay; Mehmet Yucel; Faik Yaylak; Alper Hacioglu; Mustafa C Algin; Esra G Olgun; Levent Sahin; Tayfun Aydin

    2008-01-01

    Combined duplication of the colon and vermiform appendix is one of the rare congenital anomalities of the alimentary tract. Only a few cases have been reported in the adult population. A 28-year-old man presented to the clinic with a mass in the right flank. Imaging showed only a hydronephrotic atrophic kidney. The final diagnosis was only available at exploration. Combined duplication of the tubular colon and vermiform appendix was confirmed histopathologically. The patient was treated with nephrectomy and complete resection of the duplicated colon and vermiform appendix. The patient recovered uneventfully, and has done well for the past year. This is believed to be one of the first reports of combined duplication of the tubular colon and vermiform appendix as a cause of hydronephrotic atrophic kidney in an adult patient.

  1. New Guidelines for Assessment of Malnutrition in Adults: Obese Critically Ill Patients.

    Science.gov (United States)

    Mauldin, Kasuen; O'Leary-Kelley, Colleen

    2015-08-01

    Recently released recommendations for detection and documentation of malnutrition in adults in clinical practice define 3 types of malnutrition: starvation related, acute disease or injury related, and chronic disease related. The first 2 are more easily recognized, but the third may be more often unnoticed, particularly in obese patients. Critical care patients tend to be at high risk for malnutrition and thus require a thorough nutritional assessment. Compared with patients of earlier times, intensive care unit patients today tend to be older, have more complex medical and comorbid conditions, and often are obese. Missed or delayed detection of malnutrition in these patients may contribute to increases in hospital morbidity and longer hospital stays. Critical care nurses are in a prime position to screen patients at risk for malnutrition and to work with members of the interprofessional team in implementing nutritional intervention plans.

  2. Importance of measuring Bone Mineral Density in Adult Coeliac Disease Patients

    Directory of Open Access Journals (Sweden)

    Dr. DI Hobday

    2007-07-01

    Full Text Available This study was conducted with an aim to confirm the presence of osteoporosis in patients proven to be suffering from Coeliac Disease and compare present practice in the hospital against the guidelines suggested in the published literature. Information was obtained by retrospective analysis by reviewing notes of 73 patients with coeliac disease, who are registered in the database of a busy Gastroenterology Department of Sunderland Royal Hospital. Of the total of 73 patients, 54 patients underwent a DEXA scan at diagnosis and the Osteoporosis (WHO defined criteria of T score below 2.5 SD the mean adult was detected in 15(27.3% of them. 6 of the 15 patients detected to have osteoporosis were less than 53 years of age. Osteoporosis is common in patients with coeliac disease, and need regular monitoring for osteoporosis, as they are at significant risk of developing it.

  3. Comprehensive Orthodontic Treatment of Adult Patient with Cleft Lip and Palate

    Directory of Open Access Journals (Sweden)

    Noemí Leiva Villagra

    2014-01-01

    Full Text Available The aim of the paper is to present full orthodontic treatment of an operated cleft lip adult patient. Case Report. An 18-year-old patient consulted for severe crowded teeth. He comes from a poor family. At that time he already had four operations (velum, palate, lip, and myringotomy. Treatment included maxillary expansion, tooth extraction, and fixed orthodontic, as well as kinesiology and speech therapy treatment. A multidisciplinary approach allowed us to achieve successfully an excellent result for this patient and gave him a harmonic smile and an optimal function without orthognathic surgery. Two years after treatment, occlusion remains stable.

  4. Nebulised dornase alfa versus placebo or hypertonic saline in adult critically ill patients

    DEFF Research Database (Denmark)

    Claudius, Casper; Perner, Anders; Møller, Morten Hylander

    2015-01-01

    BACKGROUND: Nebulised dornase alfa is used off-label in critically ill patients. We aimed to assess the benefits and harms of nebulised dornase alfa versus placebo, no prophylaxis, or hypertonic saline on patient-important outcome measures in adult critically ill patients. METHODS: We performed...... a systematic review with meta-analysis and trial sequential analysis (TSA) using the Cochrane Collaboration methodology. Eligible trials were randomised clinical trials comparing nebulised dornase alfa with placebo, no prophylaxis, or hypertonic saline. The predefined outcome measures were all-cause mortality...

  5. Surgical repair of chronic complete hamstring tendon rupture in the adult patient.

    Science.gov (United States)

    Cross, M J; Vandersluis, R; Wood, D; Banff, M

    1998-01-01

    Complete rupture of the hamstring tendons in the adult is a rare injury. This report discusses complete rupture of the hamstring tendons in nine patients treated by late operative repair. All patients were referred from outside centers for a second opinion after failed nonoperative treatment. The diagnosis was made quite easily on clinical grounds and was confirmed at surgery. Surgical treatment in all cases consisted of reattachment of the hamstring tendons to the origin on the ischium, and in all cases it was necessary to perform neurolysis of the sciatic nerve. Good results were achieved in all cases, at follow-up all patients were satisfied with the surgery.

  6. Radiotherapy in Gorlin Syndrome: Can It Be Safe and Effective in Adult Patients?

    Science.gov (United States)

    Baker, Sarah; Joseph, Kurian; Tai, Patricia

    2016-01-01

    Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare autosomal dominant disorder with multiple manifestations including early onset of cutaneous basal cell carcinomas (BCCs). Radiotherapy has traditionally been contraindicated due to reports of BCC induction. We describe here a patient treated successfully with radiotherapy with no tumour induction at 57 months of follow-up. A comprehensive literature review of radiotherapy outcomes in patients with Gorlin syndrome suggests radiotherapy may be a feasible treatment option for adult patients with treatment refractory lesions or surgical contraindication.

  7. Comprehensive orthodontic treatment of adult patient with cleft lip and palate.

    Science.gov (United States)

    Leiva Villagra, Noemí; Muñoz Domon, Miguel; Véliz Méndez, Sebastian

    2014-01-01

    The aim of the paper is to present full orthodontic treatment of an operated cleft lip adult patient. Case Report. An 18-year-old patient consulted for severe crowded teeth. He comes from a poor family. At that time he already had four operations (velum, palate, lip, and myringotomy). Treatment included maxillary expansion, tooth extraction, and fixed orthodontic, as well as kinesiology and speech therapy treatment. A multidisciplinary approach allowed us to achieve successfully an excellent result for this patient and gave him a harmonic smile and an optimal function without orthognathic surgery. Two years after treatment, occlusion remains stable.

  8. The safe practice of CT coronary angiography in adult patients in UK imaging departments.

    Science.gov (United States)

    Harden, S P; Bull, R K; Bury, R W; Castellano, E A; Clayton, B; Hamilton, M C K; Morgan-Hughes, G J; O'Regan, D; Padley, S P G; Roditi, G H; Roobottom, C A; Stirrup, J; Nicol, E D

    2016-08-01

    Computed tomography coronary angiography is increasingly used in imaging departments in the investigation of patients with chest pain and suspected coronary artery disease. Due to the routine use of heart rate controlling medication and the potential for very high radiation doses during these scans, there is a need for guidance on best practice for departments performing this examination, so the patient can be assured of a good quality scan and outcome in a safe environment. This article is a summary of the document on 'Standards of practice of computed tomography coronary angiography (CTCA) in adult patients' published by the Royal College of Radiologists (RCR) in December 2014.

  9. [Angiodysplasia of moya-moya type disclosed by choreic unvoluntary abnormal movements during oral contraception. Apropos of 2 cases].

    Science.gov (United States)

    Pelletier, J; Cabanot, C; Lévrier, O; Thuillier, J N; Chérif, A A

    1997-07-01

    Two seventeen year-old women, developed acute onset left choreic movements following two months and two weeks use of oral contraceptives. Left hemiparesia appeared a few days later, while involuntary movements discontinued. Cranial CT scan and MRI showed bilateral ischemic lesion in the frontal region for the first case and isolated lesion in the right centrum ovale for the second. Angiography showed nearly complete obstruction of the terminal portion of the internal carotid artery with an outline Moya-Moya network. After discontinuing oral contraceptives, there has been no relapse of neurologic dysfunction for more than three years for the first case and twelve months for the second one. The role of perfusion insufficiency in limb-shaking carotid transient ischemic attack is discussed and the possible relations between oral contraceptives, chorea and angiographic features resembling Moya-Moya disease are evaluated.

  10. Moyamoya disease-associated protein mysterin/RNF213 is a novel AAA+ ATPase, which dynamically changes its oligomeric state

    Science.gov (United States)

    Morito, Daisuke; Nishikawa, Kouki; Hoseki, Jun; Kitamura, Akira; Kotani, Yuri; Kiso, Kazumi; Kinjo, Masataka; Fujiyoshi, Yoshinori; Nagata, Kazuhiro

    2014-03-01

    Moyamoya disease is an idiopathic human cerebrovascular disorder that is characterized by progressive stenosis and abnormal collateral vessels. We recently identified mysterin/RNF213 as its first susceptibility gene, which encodes a 591-kDa protein containing enzymatically active P-loop ATPase and ubiquitin ligase domains and is involved in proper vascular development in zebrafish. Here we demonstrate that mysterin further contains two tandem AAA+ ATPase modules and forms huge ring-shaped oligomeric complex. AAA+ ATPases are known to generally mediate various biophysical and mechanical processes with the characteristic ring-shaped structure. Fluorescence correlation spectroscopy and biochemical evaluation suggested that mysterin dynamically changes its oligomeric forms through ATP/ADP binding and hydrolysis cycles. Thus, the moyamoya disease-associated gene product is a unique protein that functions as ubiquitin ligase and AAA+ ATPase, which possibly contributes to vascular development through mechanical processes in the cell.

  11. Epidemiology of adult-onset hydrocephalus: institutional experience with 2001 patients.

    Science.gov (United States)

    Bir, Shyamal C; Patra, Devi Prasad; Maiti, Tanmoy K; Sun, Hai; Guthikonda, Bharat; Notarianni, Christina; Nanda, Anil

    2016-09-01

    OBJECTIVE Adult-onset hydrocephalus is not commonly discussed in the literature, especially regarding its demographic distribution. In contrast to pediatric hydrocephalus, which is related to a primary CSF pathway defect, its development in adults is often secondary to other pathologies. In this study, the authors investigated the epidemiology of adult-onset hydrocephalus as it pertains to different etiologies and in reference to age, sex, and race distributions. METHODS The authors retrospectively reviewed the clinical notes of 2001 patients with adult-onset hydrocephalus who presented to Louisiana State University Health Sciences Center within a 25-year span. Significant differences between the groups were analyzed by a chi-square test; p hydrocephalus in this population was 77 ± 30 per year, with a significant increase in incidence in the past decade (55 ± 3 [1990-2003] vs 102 ± 6 [2004-2015]; p Hydrocephalus in a majority of the patients had a vascular etiology (45.5%) or was a result of a tumor (30.2%). The incidence of hydrocephalus in different age groups varied according to various pathologies. The incidence was significantly higher in males with normal-pressure hydrocephalus (p = 0.03) or head injury (p = 0.01) and higher in females with pseudotumor cerebri (p hydrocephalus was significantly higher in Caucasian patients (p = 0.0002) than in those of any other race. CONCLUSIONS Knowledge of the demographic variations in adult-onset hydrocephalus is helpful in achieving better risk stratification and better managing the disease in patients. For general applicability, these results should be validated in a large-scale meta-analysis based on a national population database.

  12. CLINICAL, BIOCHEMICAL AND HISTOPATHOLOGICAL PROFILE OF ADULT NEPHROTIC SYNDROME PATIENTS IN A TERTIARY CARE HOSPITAL

    Directory of Open Access Journals (Sweden)

    Krishnamoorthy

    2015-09-01

    Full Text Available BACKGROUND: The nephrotic syndrome is recognized as an independent entity of renal disease for over half a century . 1 Causes of nephrotic syndrome varies with age, time period, geographical location and race. In children, minimal change nephrotic syndrome is the commonest 2 ; however, membranous nephropathy is most frequent in adults . 3 As it commonly affects the younger age group and is associated with high morbidity and mortality, there is a need to understand and diagnose the disease at an early stage. Hence, this study has been done to identify the clinical presentation, biochemical parameters and histopathology associated with nephrotic syndrome in adults and its subtypes. OBJECTIVE: To study the clinical, biochemical and histopathological profile of patients with Adult Nephrotic Syndrome admitted in our tertiary care hospital. METHODS: Prospective study of 100 patients with Adult Nephrotic Syndrome admitt ed in our tertiary care hospital were screened with facial puffiness and pedal edema. They were tested for urine proteinuria, urine protein creatinine ratio or 24 hour urine protein estimation. Later renal biopsy was done for all patients to stratify the subtypes. RESULTS: In this study, males were predominantly affected. Most common presenting complaints were facial puffiness and pedal edema. Systolic BP was increased in 96% of patients and diastolic BP was elevated in 50% of patients. Serum LDL and TGL were elevated in nephrotic syndrome. In young patients less than 40 years Focal Segmental Glomerulosclerosis (FSGS is the commonest type, then Membrano Proliferative Glomerulo Nephritis (MPGN and Minimal Change Disease (MCD. In individuals more than 40 years, membranous nephropathy was predominantly seen followed by FSGS. CONCLUSION: There is a changing trend in primary nephrotic syndrome and FSGS was found to be the commonest subtype. Male preponderance was noticed and also FSGS was found to be more common in younger adults. Most

  13. Nutritional status and nosocomial infections among adult elective surgery patients in a Mexican tertiary care hospital.

    Directory of Open Access Journals (Sweden)

    Judith Rodríguez-García

    Full Text Available Controversy exists as to whether obesity constitutes a risk-factor or a protective-factor for the development of nosocomial Infection (NI. According to the obesity-paradox, there is evidence that moderate obesity is a protective-factor. In Mexico few studies have focused on the nutritional status (NS distribution in the hospital setting.The aim of this study was to estimate the distribution of NS and the prevalence of nosocomial infection NI among adult elective surgery (ES patients and to compare the clinical and anthropometric characteristics and length of stays (LOS between obese and non-obese patients and between patients with and without NI.We conducted a cross-sectional study with a sample (n = 82 adult ES patients (21-59 years old who were recruited from a tertiary-care hospital. The prevalences of each NS category and NI were estimated, the assessments were compared between groups (Mann-Whitney, Chi-squared or the Fisher's-exact-test, and the association between preoperative risk-factors and NI was evaluated using odds ratios.The distribution of subjects by NS category was: underweight (3.66%, normal-weight (28.05%, overweight (35.36%, and obese (32.93%. The prevalence of NI was 14.63%. The LOS was longer (p<0.001 for the patients who developed NI. The percentages of NI were: 33.3% in underweight, 18.52% in obese, 17.39% in normal-weight, and 6.90% in overweight patients.The prevalence of overweight and obesity in adult ES patients is high. The highest prevalence of NI occurred in the underweight and obese patients. The presence of NI considerably increased the LOS, resulting in higher medical care costs.

  14. Social determinants and health-related dimensions of quality of life in adult patients with haemophilia.

    Science.gov (United States)

    Dolatkhah, R; Fakhari, A; Pezeshki, M Z; Shabanlouei, R; Tavassoli, N; Gholchin, M

    2014-05-01

    The availability of safe and effective factor replacement therapies, in persons with haemophilia (PWH), has in some countries answered the basic need for treatment of these patients. The findings suggest that adult patients who have always been on prophylaxis reported significantly better physical functioning, and thus better quality of life. This study is designed to evaluate the QoL in adult PWH, by focusing on social determinants of QoL and their relationship with health-related dimensions, in Tabriz, Iran. The survey instrument was a self-report 36 items questionnaire, 'A36 Hemofilia - QoL', which is a disease-specific questionnaire for the assessment of the health-related QoL in adults living with haemophilia. A total of 100 haemophilia A and B patients, aged over 17 years participated in this study within 1 year. QoL total score was 71.88 (±26.89 SD). Patients who treat in our Hemophilia Treatment Center, had better QoL score (P = 0.000), and education has a significant impact on the social aspects of QoL (P = 0.18). The QoL was very poor in urban area in contrast to patients who lived in the city (54.45 vs. 74.21 respectively). Single patients have a better QoL than married patients (76.56 vs. 68.50 respectively). Our results showed that low education and lack of awareness of the diseases among PWH lead to reduce of QoL and more disease complications. More and wider treatment and psychological care for improving quality of life of these patients are seriously recommended.

  15. Adjuvant chemotherapy in adult medulloblastoma: is it an option for average-risk patients?

    Science.gov (United States)

    Franceschi, E; Bartolotti, M; Paccapelo, A; Marucci, G; Agati, R; Volpin, L; Danieli, D; Ghimenton, C; Gardiman, M P; Sturiale, C; Poggi, R; Mascarin, M; Balestrini, D; Masotto, B; Brandes, A A

    2016-06-01

    The standard treatment in children with average-risk medulloblastoma (MB) is reduced-dose radiotherapy (RT) followed by chemotherapy. However, in adults, there is no agreement on the use of adjuvant chemotherapy. We performed a retrospective analysis of adult MB patients with average-risk disease, defined as no postsurgical residual (or ≤1.5 cm(2)) and no metastatic disease (M0). Main inclusion criteria were: age >16 years, post-surgical treatment with craniospinal irradiation with or without adjuvant chemotherapy (cisplatin and etoposide ± cyclophosphamide). From 1988 to 2012 were accrued 43 average-risk MB patients treated with surgery and adjuvant RT. Fifteen (34.9 %) patients received also chemotherapy: 7 before RT, 5 after RT, and 3 before and after RT. Reasons to administer chemotherapy were presence of residual disease (even if ≤1.5 cm) and delay in RT. After a median follow up time of 10 years (range: 8-13), median survival was 18 years (95 % CI 9-28) in patients who receive RT alone, and was not reached in patients treated with RT plus chemotherapy. The survival rates at 5, 10 and 15 years were 100 %, 78.6 % (95 % CI 60.0-97.2 %) and 60.2 % (95 % CI 36.9-83.5 %), in patients treated with RT alone, and 100, 100 and 100 %, in patients treated with RT plus chemotherapy (p = 0.079). Our findings suggest a role for adjuvant chemotherapy in the treatment of average-risk MB adult patients. Further improvements might drive to add chemotherapy in average-risk setting with less favourable biological signatures (i.e., non-WNT group).

  16. Celiac Disease in Adult Patients: Specific Autoantibodies in the Diagnosis, Monitoring, and Screening

    Directory of Open Access Journals (Sweden)

    Evagelia Trigoni

    2014-01-01

    Full Text Available The increasing prevalence of celiac disease (CD, especially in adults, its atypical clinical presentation, and the strict, lifelong adherence to gluten-free diet (GFD as the only option for healthy state create an imperative need for noninvasive methods that can effectively diagnose CD and monitor GFD. Aim. Evaluation of anti-endomysium (EmA and anti-tissue transglutaminase IgA (tTG-A antibodies in CD diagnosis, GFD monitoring, and first degree relatives screening in CD adult patients. Methods. 70 newly diagnosed Greek adult patients, 70 controls, and 47 first degree relatives were tested for the presence of EmA and tTG-A. The CD patients were monitored during a 3-year period. Results. EmA predictive ability for CD diagnosis was slightly better compared to tTG-A (P=0.043. EmA could assess compliance with GFD already from the beginning of the diet, while both EmA and tTG-A had an equal ability to discriminate between strictly and partially compliant patients after the first semester and so on. Screening of first degree relatives resulted in the identification of 2 undiagnosed CD cases. Conclusions. Both EmA and tTG-A are suitable markers in the CD diagnosis, in the screening of CD among first degree relatives, having also an equal performance in the long term monitoring.

  17. Celiac disease in adult patients: specific autoantibodies in the diagnosis, monitoring, and screening.

    Science.gov (United States)

    Trigoni, Evagelia; Tsirogianni, Alexandra; Pipi, Elena; Mantzaris, Gerassimos; Papasteriades, Chryssa

    2014-01-01

    The increasing prevalence of celiac disease (CD), especially in adults, its atypical clinical presentation, and the strict, lifelong adherence to gluten-free diet (GFD) as the only option for healthy state create an imperative need for noninvasive methods that can effectively diagnose CD and monitor GFD. Aim. Evaluation of anti-endomysium (EmA) and anti-tissue transglutaminase IgA (tTG-A) antibodies in CD diagnosis, GFD monitoring, and first degree relatives screening in CD adult patients. Methods. 70 newly diagnosed Greek adult patients, 70 controls, and 47 first degree relatives were tested for the presence of EmA and tTG-A. The CD patients were monitored during a 3-year period. Results. EmA predictive ability for CD diagnosis was slightly better compared to tTG-A (P = 0.043). EmA could assess compliance with GFD already from the beginning of the diet, while both EmA and tTG-A had an equal ability to discriminate between strictly and partially compliant patients after the first semester and so on. Screening of first degree relatives resulted in the identification of 2 undiagnosed CD cases. Conclusions. Both EmA and tTG-A are suitable markers in the CD diagnosis, in the screening of CD among first degree relatives, having also an equal performance in the long term monitoring.

  18. Visual and SPM analysis of regional cerebral glucose metabolism in adult patients with neurofibromatosis

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Joon Kee; An, Young Sil; Hong, Seon Pyo; Joh, Chul Woo; Yoon, Seok Nam [Ajou University, School of Medicine, Suwon (Korea, Republic of)

    2005-07-01

    We evaluated the regional cerebral glucose metabolism in adult patients with neurofibromatosis (NF) using visual and SPM analysis, and compared with MRI findings. A total of 11 adult patients with NF type I were prospectively included in the study. All patients underwent F-18 FDG PET and brain MRI within 2 month of each other. All hypometabolic areas on PET were determined visually by 2 nuclear medicine physician and compared with MRI findings. SPM analysis was done using 42 normal controls with p = 0.005. Seven of 11 PET images showed 10 hypometabolic areas and 4 of 11 MRIs showed 6 areas of signal change brain parenchyma. Hypometabolic areas were bilateral thalamus (n=5), left temporal cortex (n=4) and dentate nucleus (n=1). In only 2 lesions (thalamus and dentate nucleus), hypometabolic foci were consistently related to signal change on MRI. SPM analysis revealed significantly decreased area in bilateral thalamus and left temporal cortex. F-18 FDG PET revealed significant hypometabolism in bilateral thalamus and left temporal cortex in adult patients with NF, and it might be helpful in understanding developmental abnormality of NF.

  19. Health-related quality of life outcome of adult patients after otoplasty.

    Science.gov (United States)

    Schwentner, Ilona; Schmutzhard, Joachim; Deibl, Martina; Sprinzl, Georg M

    2006-07-01

    Prominent ears are relatively frequent. Decreased self-esteem, increased anxiety, behavioral problems and social avoidance may result from disfigurement. In modern medicine it is becoming increasingly important to measure the benefit of surgical intervention by its impact on the patient's Quality of Life (QOL). Our study was performed in a retrospective manner at our institution including 40 adult patients with prominent ears. The Glasgow Benefit Inventory (GBI), a reproducible, valid and responsive questionnaire, was the basis of the used inquiry for detecting the changes in HRQOL after otoplasty. We showed an improvement in GBI subscores after intervention. There was no difference in GBI subscores between men and women. The follow-up time as well as critical life event and chronic concomitant disease have no influence on the GBI results. Otoplasty has a positive impact on the HRQOL of adult patients with prominent ears. The importance of this benefit is not only of individual nature. In times of healthcare economization, an amelioration of QOL followed by increased productivity at work of the individual is an important argument to justify health care expenditures. We believe that otoplasty is an appropriate therapy for selected adult patients burdened by prominent ears.

  20. Statin therapy reduces the likelihood of suboptimal blood pressure control among Ugandan adult diabetic patients

    Science.gov (United States)

    Lumu, William; Kampiire, Leaticia; Akabwai, George Patrick; Kiggundu, Daniel Ssekikubo; Kibirige, Davis

    2017-01-01

    Background Hypertension is one of the recognized risk factors of cardiovascular diseases in adult diabetic patients. High prevalence of suboptimal blood pressure (BP) control has been well documented in the majority of studies assessing BP control in diabetic patients in sub-Saharan Africa. In Uganda, there is a dearth of similar studies. This study evaluated the prevalence and correlates of suboptimal BP control in an adult diabetic population in Uganda. Patients and methods This was a cross-sectional study that enrolled 425 eligible ambulatory adult diabetic patients attending three urban diabetic outpatient clinics over 11 months. Data about their sociodemographic characteristics and clinical history were collected using pre-tested questionnaires. Suboptimal BP control was defined according to the 2015 American Diabetes Association standards of diabetes care guideline as BP levels ≥140/90 mmHg. Results The mean age of the study participants was 52.2±14.4 years, with the majority being females (283, 66.9%). Suboptimal BP control was documented in 192 (45.3%) study participants and was independently associated with the study site (private hospitals; odds ratio 2.01, 95% confidence interval 1.18–3.43, P=0.01) and use of statin therapy (odds ratio 0.5, 95% confidence interval 0.26–0.96, P=0.037). Conclusion Suboptimal BP control was highly prevalent in this study population. Strategies to improve optimal BP control, especially in the private hospitals, and the use of statin therapy should be encouraged in adult diabetic patients.

  1. Iron Overload Assessment in Adult Thalassaemic Patients Using MRI T2

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    Azita Azarkeivan

    2009-01-01

    Full Text Available "nd anemia in our country. Blood transfusion is the continual treatment of this disease. But transfusion causes a serious side effect which is iron overload in vital organs such as the heart, liver and the endocrine system. Accumulated iron in these organs may cause high risk secondary problems which threaten the patients' life. Early assessment of the iron overload in vital organs and applying for early treatment can be beneficial for increasing life quality in these patients. Assessment of cardiac and hepatic iron overload using MRI T2 technique and comparing it with serum ferritin level was the goal of this study. "nMaterials and Methods: The referred thalassaemic patients to Zafar adult thalassaemia clinic were the population of this study. Serum ferritin test was carried out for all these patients. Cardiac and hepatic iron overload assessment of these patients was performed in Noor medical imaging center using MRI T2 technique. The iron overload results of all patients were classified as normal, mild, moderate and severe. We compared them with the patients’ clinical parameters, especially the serum ferritin level. Results were analyzed by SPSS software. "nResults: 700 adult patients with the mean age of 25.76 (SD±10.4 were studied in this research project. There were 360 males (51.4% and 340 (48.6% females enrolled in this study. Among them, there were 502 (71.7% major thalassaemia, 158 (22.6% intermediate thalassaemia, 7 alpha thalassaemia, 9 sickle cell anemia and 10 hemochromatosis patients. The mean of serum ferritin level was 2327.6 mg/dl (SD±2095.8. Classified results of cardiac iron overload assessment were normal in 366 (66.5% patients, mild in 44 (8% patients, moderate in 64 (11.6% patients, and severe in 76 (13.8%patients. The classified results of hepatic iron overload assessment were: normal in 122 (22.2% patients, mild in 137 (25% patients, moderate in 235 (42.8% patients and severe in 55 (10% patients. Iron overload

  2. Assessment and monitoring of treatment response in adult ADHD patients: current perspectives

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    Ramsay JR

    2017-01-01

    Full Text Available J Russell Ramsay Adult ADHD Treatment & Research Program, Perelman School of Medicine, University of Pennsylvania Philadelphia, PA, USA Abstract: Attention-deficit/hyperactivity disorder (ADHD is a neurodevelopmental syndrome that emerges in childhood or early adolescence and persists into adulthood for a majority of individuals. There are many other adults with ADHD who may not seek out evaluation and treatment until adulthood, having been able to “get by” before struggling with inattention, hyperactivity, and/or impulsivity in adulthood, in addition to facing the associated features of disorganization, poor time management, and procrastination among many others. A lifetime diagnosis of ADHD is associated with a wide range of life impairments, which makes a comprehensive and accurate diagnostic assessment essential in order to obtain appropriate treatment. Moreover, while there are effective medical and psychosocial treatments for ADHD, it is important to be able to track treatment response in order to evaluate whether adjustments in specific interventions are needed or referrals for adjunctive treatments and supports are indicated to facilitate optimal therapeutic outcomes. The goal of this article is to provide a clinically useful review of the various measures that practicing clinicians can use to aid in the diagnostic assessment and monitoring of psychosocial and medical treatment of ADHD in adult patients. This review includes various structured interviews, screening scales, adult ADHD symptom inventories, measures of associated features of ADHD, as well as ratings of impairment and functioning which can be adapted to clinicians’ practice needs in order to track treatment progress and optimize treatments for adults with ADHD. Keywords: adult attention-deficit/hyperactivity disorder, adult ADHD, pharmacotherapy, psychosocial treatment, symptoms, functional impairments, executive functions

  3. Management of asthma in adults: do the patients get what they need--and want?

    DEFF Research Database (Denmark)

    Backer, V; Ulrik, Charlotte Suppli; Harving, H;

    2007-01-01

    Suboptimal asthma control may be caused by a combination of factors, such as nonadherence to guidelines, lack of compliance, and poor asthma education. The aim was to assess patients' knowledge of asthma and different management strategies, including patients' attitudes toward involvement...... in treatment decisions. The participants (n=509) were recruited from all parts of Denmark through a web-based panel (Zapera Danmarkspanel). A questionnaire concerning asthma knowledge, compliance, and treatment was fulfilled through the Internet. Among the participating adult asthmatic patients, signs...... function measured and peak flow monitoring was reported by 5%. Written action plans were provided for 12% of patients and 50% had had their inhaler technique checked. Although 59% of patients were instructed to adjust their controller therapy if needed, only 23% reported that they had done so...

  4. Campylobacter enteritis in adult patients with acute diarrhea from 2005 to 2009 in Beijing, China

    Institute of Scientific and Technical Information of China (English)

    CHEN Jie; SUN Xin-ting; ZENG Zheng; YU Yan-yan

    2011-01-01

    Background There has been a marked global increase in the incidence of human Campylobacter enteritis in recent years. This study investigated the epidemiological and clinical features of Campylobacter enteritis in adult patients suffering from acute diarrhea.Methods This was a retrospective review of Campylobacter enteritis in adult patients with acute diarrhea presenting at Beijing University First Hospital, Beijing, China, in the summer and autumn (April to October) of 2005 to 2009. The data collected included the species of campylobacter identified, and the age, gender, clinical manifestations and results of laboratory test on stool samples collected from the patients. Campylobacter sensitivity tests to various antimicrobial agents were conducted on 80 specimens. Chi-square tests were applied using SPSS13.0 software and a two-sided P value of <0.05 was considered statistically significant.Results Campylobacter spp. isolated from the stool specimens of 142 patients with diarrhea represented 14.9% of all the cases examined. C. jejuni was identified in 127 patients (89.4%) and C. coli in 15 others (10.6%). The infection incidence was highest in the age range of 21-30 years which comprised 21.7% of the total cases examined. Most cases of diarrhea (46 patients) occurred in June. Watery diarrhea (97.2%), abdominal pain (72.5%) and fever (64.8%) were the most common manifestations of enteric campylobacteriosis. Only four patients (2.8%) had bloody diarrhea. The antimicrobial resistance rates were: cefoperazone (100%), levofloxacin (61.3%), gentamicin (12.5%), erythromycin (6.3%), and azithromycin (2.5%).Conclusions Campylobacter was prevalent among adults with acute diarrhea from 2005 to 2009 in Beijing, China. The large number of those afflicted by the disease warrants the commission of a large multicenter study to determine the extent of enteric campylobacteriosis in this region.

  5. Peptic ulcer as a risk factor for postherpetic neuralgia in adult patients with herpes zoster.

    Science.gov (United States)

    Chen, Jen-Yin; Lan, Kuo-Mao; Sheu, Ming-Jen; Tseng, Su-Feng; Weng, Shih-Feng; Hu, Miao-Lin

    2015-02-01

    Postherpetic neuralgia is the most common complication of herpes zoster. Identifying predictors for postherpetic neuralgia may help physicians screen herpes zoster patients at risk of postherpetic neuralgia and undertake preventive strategies. Peptic ulcer has been linked to immunological dysfunctions and malnutrition, both of which are predictors of postherpetic neuralgia. The aim of this retrospective case-control study was to determine whether adult herpes zoster patients with peptic ulcer were at greater risk of postherpetic neuralgia. Adult zoster patients without postherpetic neuralgia and postherpetic neuralgia patients were automatically selected from a medical center's electronic database using herpes zoster/postherpetic neuralgia ICD-9 codes supported with inclusion and exclusion criteria. Consequently, medical record review was performed to validate the diagnostic codes and all pertaining data including peptic ulcer, Helicobacter pylori (H. pylori) infection and ulcerogenic medications. Because no standard pain intensity measurement exists, opioid usage was used as a proxy measurement for moderate to severe pain. In total, 410 zoster patients without postherpetic neuralgia and 115 postherpetic neuralgia patients were included. Multivariate logistic regressions identified 60 years of age and older, peptic ulcer and greater acute herpetic pain as independent predictors for postherpetic neuralgia. Among etiologies of peptic ulcer, H. pylori infection and usage of non-selective nonsteroidal anti-inflammatory drugs were significantly associated with the increased risk of postherpetic neuralgia; conversely, other etiologies were not significantly associated with the postherpetic neuralgia risk. In conclusion, 60 years of age and older, peptic ulcer and greater acute herpetic pain are independent predictors for postherpetic neuralgia in adult herpes zoster patients.

  6. A systematic review of music therapy practice and outcomes with acute adult psychiatric in-patients.

    Directory of Open Access Journals (Sweden)

    Catherine Carr

    Full Text Available There is an emerging evidence base for the use of music therapy in the treatment of severe mental illness. Whilst different models of music therapy have been developed in mental health care, none have specifically accounted for the features and context of acute in-patient settings. This review aimed to identify how music therapy is provided for acute adult psychiatric in-patients and what outcomes have been reported.A systematic review using medical, psychological and music therapy databases. Papers describing music therapy with acute adult psychiatric in-patients were included. Analysis utilised narrative synthesis.98 papers were identified, of which 35 reported research findings. Open group work and active music making for nonverbal expression alongside verbal reflection was emphasised. Aims were engagement, communication and interpersonal relationships focusing upon immediate areas of need rather than longer term insight. The short stay, patient diversity and institutional structure influenced delivery and resulted in a focus on single sessions, high session frequency, more therapist direction, flexible use of musical activities, predictable musical structures, and clear realistic goals. Outcome studies suggested effectiveness in addressing a range of symptoms, but were limited by methodological shortcomings and small sample sizes. Studies with significant positive effects all used active musical participation with a degree of structure and were delivered in four or more sessions.No single clearly defined model exists for music therapy with adults in acute psychiatric in-patient settings, and described models are not conclusive. Greater frequency of therapy, active structured music making with verbal discussion, consistency of contact and boundaries, an emphasis on building a therapeutic relationship and building patient resources may be of particular importance. Further research is required to develop specific music therapy models for this

  7. [Clinical study of 32 patients with adult Philadelphia chromosome-positive acute lymphoblastic leukemia].

    Science.gov (United States)

    Chen, Xiao-Yun; Zheng, Yong-Liang; Chen, Yi-Jian

    2014-12-01

    This study was aimed to evaluate the efficacy and safety of imatinib in the treatment of patients with adult Ph chromosome-positive acute lymphoblastic leukemia (Ph(+)ALL). A total of 32 diagnosed adult Ph(+)ALL patients from July 2007 to February 2014 in our hospital were retrospectively analyzed and were divided into two groups: imatinib plus chemotherapy group and traditional chemotherapy group. The differences between two groups were analysed in disease-free survival time (DFS), overall survival time (OS) and toxicity. The G banding technigue was used to analyse the karyotype, and the flow cytometry was applyed to detect the immune markers on surface of cells. The results showed that all patients expressed B cell and hematopietic stem/progenitor cell immune markers, out of them 21 patients (65.6%) were with myeloid antigens, 27 patients with simple Ph (+) phenotype and 5 patients with additional chromosome abnormality. The DFS and OS of the imatinib group were statistically longer than those of the traditional chemotherapy group (14.3 ± 4.7 months vs 10.7 ± 3.8 months) (P 0.05)). It is concluded that the all cases of adult Ph(+)ALL are with B cell phenotype and express hematopietic stem/progenitor cell antigen. They often accompanied by expression of myeloid antigens and additonal chromosome abnormality in genetics. The combination of imatinib with chemotherapy can prolong remission time and survival time for patients of non-hematopietic stem cell transplantation on the basis of no notably increasing the toxic effects.

  8. Experiences from Cochlear Implantation and Auditory Brainstem Implantation in Adults and Children : Electrophysiological Measurements, Hearing Outcomes and Patient Satisfaction

    OpenAIRE

    Lundin, Karin

    2016-01-01

    Cochlear implants (CIs) and auditory brainstem implants (ABIs) are prostheses for hearing used in patients with profound hearing impairment. A CI requires an operational cochlear nerve to function in contrast to an ABI. ABIs were initially designed for adult patients with neurofibromatosis type 2 (NF2), suffering from bilateral vestibular schwannomas. Now ABIs are also used for patients, both adults and children, with congenital cochlear malformations, cochlear nerve hypoplasia/aplasia, and c...

  9. Distinct quasispecies characteristics and positive selection within the core gene in chronic hepatitis B virus infected child and adult patients.

    Science.gov (United States)

    Haijun, Deng; Yong, Huang; Ailong, Huang; Quanxin, Long

    2015-05-01

    There are significant differences in clinical characteristics between chronic hepatitis B virus infected (CHB) child and adult patients. Viral quasispecies characteristics are associated with its pathogenic properties. For hepatitis B virus (HBV), its core region is the main immune recognition region for its enriched epitopes. In our study, we discuss the quasispecies characteristics and positive selection within core gene within chronic HBV infected child and adult patients. By analyzing 170 core gene sequences from child CHB patients and 121 core genes sequences from adult CHB patients, quasispecies characteristics were described by sequence complexity, diversity, non-synonymous substitution ratio (dN) and synonymous substitution ratios (dS). In addition, positive selection sites were also determined by bioinformatics tools. Then, all these parameters were compared between child and adult CHB patient groups. Compared with child patients, adult patients with CHB showed distinct quasispecies characteristics within the core region, had a higher sequence complexity and diversity and more positive selection sites, suggesting that the adult CHB patients had a higher immune selection pressure on the HBV core gene. Reduced selection pressure on the HBV core gene in hepatitis B e antigen (HBeAg)-positive CHB patients than HBeAg negative CHB patients were observed in both adult and child patient groups. The majority of the screened positive selection sites lay within human leukocyte antigens (HLA)-restricted epitopes. In conclusion, this study analyzed the quasispecies characteristics discrepancy between child and adult patients with CHB, and revealed the possible reason for the distinct clinical characteristics in the perspective of population genetics.

  10. Anemia among adult HIV patients in Ethiopia: a hospital-based cross-sectional study

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    Melese H

    2017-02-01

    Full Text Available Hermela Melese,1 Molla Mesele Wassie,2 Haile Woldie,2 Abilo Tadesse,3 Nebiyu Mesfin3 1HIV Follow-up Care Clinic, Debre-Tabor Hospital, Debre‑Tabor, 2Department of Human Nutrition, Institute of Public Health, 3Department of Internal Medicine, School of Medicine, College of Medicine and Health Sciences, University of Gondar, Gondar, Ethiopia Background: Anemia is a major public health problem in HIV patients around the world. It has a negative effect on the quality of life of HIV patients and progression of the HIV disease. In the sub-Saharan African setting, including Ethiopia where both HIV infection and under-nutrition are expected to be high, there is a paucity of data on the matter. This study was aimed to reveal the magnitude and factors associated with anemia among adult HIV patients in Debre-Tabor Hospital, northwest Ethiopia. Methods: A hospital-based cross-sectional study was used among adult HIV patients in Debre-Tabor Hospital from April 1 to May 30, 2015. The diagnosis of anemia was made following the 2011 World Health Organization recommendation on hemoglobin cut-off points. Univariable and multivariable logistic regression was carried out to assess factors associated with anemia. Results: A total of 377 patients’ charts were reviewed. Most of the participants (n=237, 62.9% were taking antiretroviral treatment (ART. The overall prevalence of anemia was 23% (95% CI: 19.1, 27.6. Being ART-naïve (adjusted odds ratio [AOR]: 3.37; 95% CI: 1.59, 7.14, having treatment history with anti-tuberculosis (TB drug (AOR: 3.2; 95% CI: 1.19, 8.67, taking zidovudine (ZDV-containing ART regimen (AOR: 2.14; 95% CI: 1.03, 4.57, and having recent CD4+ T-lymphocytes count of <200 cells/µL (AOR: 2.13; 95% CI: 1.04, 4.36 were associated with occurrence of anemia among adult HIV patients. Conclusion and recommendation: Anemia continues to be a major co-morbidity among adult HIV patients in Ethiopia. Adult HIV patients who are taking ZDV-containing ART

  11. Movement disorders in adult surviving patients with maple syrup urine disease.

    Science.gov (United States)

    Carecchio, Miryam; Schneider, Susanne A; Chan, Heidi; Lachmann, Robin; Lee, Philip J; Murphy, Elaine; Bhatia, Kailash P

    2011-06-01

    Maple syrup urine disease is a rare metabolic disorder caused by mutations in the branched-chain α-keto acid dehydrogenase complex gene. Patients generally present early in life with a toxic encephalopathy because of the accumulation of the branched-chain amino acids leucine, isoleucine, and valine and the corresponding ketoacids. Movement disorders in maple syrup urine disease have typically been described during decompensation episodes or at presentation in the context of a toxic encephalopathy, with complete resolution after appropriate dietary treatment. Movement disorders in patients surviving childhood are not well documented. We assessed 17 adult patients with maple syrup urine disease (mean age, 27.5 years) with a special focus on movement disorders. Twelve (70.6%) had a movement disorder on clinical examination, mainly tremor and dystonia or a combination of both. Parkinsonism and simple motor tics were also observed. Pyramidal signs were present in 11 patients (64.7%), and a spastic-dystonic gait was observed in 6 patients (35.2%). In summary, movement disorders are common in treated adult patients with maple syrup urine disease, and careful neurological examination is advisable to identify those who may benefit from specific therapy. © 2011 Movement Disorder Society.

  12. Clinical and hepatic evaluation in adult dengue patients: a prospective two-month cohort study

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    Ricardo Tristão-Sá

    2012-12-01

    Full Text Available INTRODUCTION: To analyze the liver dysfunction and evolution of signs and symptoms in adult dengue patients during a two-month follow-up period. METHODS: A prospective cohort study was conducted in Campos dos Goytacazes, Rio de Janeiro, Brazil, from January to July, 2008. The evolution of laboratory and clinical manifestations of 90 adult dengue patients was evaluated in five scheduled visits within a two-month follow-up period. Twenty controls were enrolled for the analysis of liver function. Patients with hepatitis B, hepatitis C, those known to be human immunodeficiency virus (HIV seropositive and pregnant women were excluded from the study. RESULTS: At the end of the second month following diagnosis, we observed that symptoms persisted in 33.3% (30/90 of dengue patients. We also observed that, 57.7% (15/26 of the symptoms persisted at the end of the second month. The most persistent symptoms were arthralgia, fatigue, weakness, adynamia, anorexia, taste alteration, and hair loss. Prior dengue virus (DENV infection did not predispose patients to a longer duration of symptoms. Among hepatic functions, transaminases had the most remarkable elevation and in some cases remained elevated up to the second month after the disease onset. Alanine aminotransferase (ALT levels overcame aspartate aminotransferase (AST during the convalescent period. Male patients were more severely affected than females. CONCLUSIONS: Dengue fever may present a wide number of symptoms and elevated liver transaminases at the end of the second month.

  13. Methimazole-Induced Goitrogenesis in an Adult Patient With the Syndrome of Resistance to Thyroid Hormone

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    Kathleen Glymph DO

    2014-10-01

    Full Text Available Patients with the syndrome of resistance to thyroid hormone (RTH have clinical (tachycardia and anxiety and biochemical (elevated thyroid hormones level features of hyperthyroidism. Based on previous reports in pediatric patients with the RTH, antithyroid treatment in these patients is not indicated. Clinical and biochemical sequel of antithyroid therapy in an adult patient with RTH was not previously reported. A 63-year-old African American female with history of RTH was treated with a therapy consisting of methimazole 15 mg daily and atenolol. Methimazole treatment resulted in reduction in thyroid hormone level while the patient’s TSH increased with a peak of 24.88 mIU/L. Having achieved biochemical euthyroidism, the patient developed thyroid gland enlargement associated with progressive symptoms of dysphagia and dyspnea. Examination demonstrated globally enlarged firm thyroid gland with areas of nodularity in both lobes. A computed tomography of the neck showed enlarged thyroid gland with extension around bilateral sternocleidomastoid muscles and compression onto the trachea. Methimazole therapy was discontinued and patient was treated just on atenolol. Over 12 months following discontinuation of methimazole, the patient experienced marked clinical and radiographic improvement of the goiter size associated with TSH reduction to 1.26 mIU/L and modest free thyroxine increase as expected in RTH. It seems appealing to treat patients with the RTH with antithyroid medications. However, in these patients decrease in thyroid hormone levels will stimulate TSH production, which can, in turn, predispose to goiter formation. Our report supports prior observations in children with RTH that treatment with methimazole is not indicated in adult patients with RTH.

  14. Clinical and laboratory characteristics of acute community-acquired urinary tract infections in adult hospitalised patients.

    Science.gov (United States)

    Piljic, Dilista; Piljic, Dragan; Ahmetagic, Sead; Ljuca, Farid; Porobic Jahic, Humera

    2010-02-01

    Urinary tract infections (UTI) cause a great number of morbidity and mortality. These infections are serious complications in pregnancy, patients with diabetes, polycystic kidneys disease, sickle cell anaemia, kidney transplant and in patients with functional or structural anomalies of the urinary tract. The aim of this investigation was to determine a dominant causative agents of UTI and some of the clinical and laboratory characteristics of acute community-acquired UTI in adult hospitalised patients. We studied 200 adult patients with acute community-acquired UTI hospitalised in the Clinic for Infectious Diseases Tuzla from January 2006 to December 2007. The patients were divided into two groups: a group of patients with E. coli UTI (147) and a group of patients with non-E. coli UTI (53). In these two groups, the symptoms and signs of illness, blood test and urine analysis results were analysed. Our results have shown that the patients with E. coli UTI frequently had fever higher than 38,5 degrees C (p<0,0001), chills (p=0,0349), headache (p=0,0499), cloudy urine (p<0,0001), proteinuria (p=0,0011) and positive nitrite-test (p=0,0002). The patients with non-E. coli UTI frequently had fever lower than 38,5 degrees C (p<0,0001) and urine specific gravity <1015 (p=0,0012). There was no significant difference in blood test results between patients with E. coli and non-E. coli UTI. These clinical and laboratory findings can lead us to early etiological diagnosis of these UTI before urine culture detection of causative agents, which takes several days. Early etiological diagnosis of the E. coli and non-E. coli UTI is necessary for an urgent administration of appropriate empirical antibiotic treatment. This is very important in prevention of irreversible kidney damage, prolonged treatment, complications, as well as recidives and chronicity of the illness.

  15. Use of the adult attachment projective picture system in psychodynamic psychotherapy with a severely traumatized patient.

    Science.gov (United States)

    George, Carol; Buchheim, Anna

    2014-01-01

    The following case study is presented to facilitate an understanding of how the attachment information evident from Adult Attachment Projective Picture System (AAP) assessment can be integrated into a psychodynamic perspective in making therapeutic recommendations that integrate an attachment perspective. The Adult Attachment Projective Picture System (AAP) is a valid representational measure of internal representations of attachment based on the analysis of a set of free response picture stimuli designed to systematically activate the attachment system (George and West, 2012). The AAP provides a fruitful diagnostic tool for psychodynamic-oriented clinicians to identify attachment-based deficits and resources for an individual patient in therapy. This paper considers the use of the AAP with a traumatized patient in an inpatient setting and uses a case study to illustrate the components of the AAP that are particularly relevant to a psychodynamic conceptualization. The paper discusses also attachment-based recommendations for intervention.

  16. Temporomandibular disorders and psychological status in adult patients with a deep bite

    DEFF Research Database (Denmark)

    Sonnesen, Liselotte; Svensson, Peter

    2008-01-01

    Temporomandibular disorders (TMDs) and psychological status were examined in adult patients with a deep bite and compared with an adult age- and gender-matched control group with neutral occlusion. The deep bite group consisted of 20 females (mean age 30.3 years) and 10 males (mean age 33.1 years......). The control group comprised 20 females (mean age 29.4 years) and 10 males (mean age 34.2 years). TMD examination, according to the Rese