Chorea in the clinical presentation of moyamoya disease: results of surgical revascularization and a proposed clinicopathological correlation

2013 ◽  
Vol 11 (3) ◽  
pp. 313-319 ◽  
Author(s):  
Edward S. Ahn ◽  
R. Michael Scott ◽  
Richard L. Robertson ◽  
Edward R. Smith
Keyword(s):  
Surgical Treatment ◽  
Basal Ganglia ◽  
Moyamoya Disease ◽  
Clinical Presentation ◽  
Disease Stage ◽  
Consecutive Series ◽  
Unilateral Disease ◽  
Collateral Vessels ◽  
Revascularization Surgery

Object Chorea is a movement disorder characterized by brief, irregular, involuntary contractions that appear to flow from 1 muscle to another. There are a limited number of reports in the literature that have linked moyamoya disease and chorea. The authors describe their experience in treating moyamoya disease in patients in whom chorea developed as part of the clinical presentation. Methods The authors conducted a retrospective review of a consecutive series of 316 children who underwent pial synangiosis revascularization for moyamoya disease at the Boston Children's Hospital. Results Of 316 surgically treated patients with moyamoya disease, 10 (3.2%; 6 boys and 4 girls) had chorea as a part of their presentation. The average age at surgical treatment was 9.9 years (range 3.8–17.9 years). All patients had evidence of hypertrophied lenticulostriate collateral vessels through the basal ganglia on preoperative angiography and/or MRI on affected sides. Two patients had cystic lesions in the basal ganglia. Nine patients underwent bilateral craniotomies for pial synangiosis, and 1 patient underwent a single craniotomy for unilateral disease. Follow-up was available in 9 patients (average 50.1 months). The mean duration of chorea was 1.36 years (range 2 days to 4 years), with resolution of symptoms in all patients. One patient developed chorea 3 years after surgical treatment, 4 patients had transient chorea that resolved prior to surgery, and 5 patients experienced resolution of the chorea after surgery (average 13 months). Conclusions The authors describe children with moyamoya disease and chorea as part of their clinical presentation. The data suggest that involvement of the basal ganglia by the hypertrophied collateral vessels contributes to the development of chorea, which can wax or wane depending on disease stage or involution of the vessels after revascularization surgery. In most patients, however, the chorea improves or disappears about 1 year after presentation.

Outcome of repeat revascularization surgery for moyamoya disease after an unsuccessful indirect revascularization

2011 ◽  
Vol 115 (2) ◽  
pp. 328-336 ◽  
Author(s):  
Paritosh Pandey ◽  
Gary K. Steinberg
Keyword(s):  
Mr Imaging ◽  
Moyamoya Disease ◽  
Cerebral Artery ◽  
Repeat Revascularization ◽  
Recipient Vessel ◽  
Indirect Revascularization ◽  
Collateral Vessels ◽  
Revascularization Surgery ◽  
Revascularization Procedures

Object Revascularization for moyamoya disease, either by direct anastomosis or indirect procedures, is an accepted and effective form of treatment for prevention of future ischemic events. Indirect procedures do not provide sufficient collateral vessels in a subset of patients, who then have persistent or new symptoms. Repeat revascularization procedures may be recommended for these patients. Methods Sixteen patients underwent repeat revascularization after undergoing an indirect procedure in the same hemisphere. These patients were included in the study, and a retrospective review of their clinical details, neuroimaging results, surgical details, and outcome was performed. Direct revascularization was the procedure of choice; however, in patients with no acceptable recipient vessel (> 0.6 mm) the authors added a second indirect procedure for further revascularization. Results Over the last 19 years, 16 patients (8 male and 8 female patients, age range 5–48 years, mean 16.7 years, 10 pediatric and 6 adult patients) underwent repeat revascularization for moyamoya disease. Initially all patients presented with ischemic symptoms (4 transient ischemic attacks [TIAs] and 12 strokes; 2 patients had bilateral symptoms). Angiography revealed that 13 patients had bilateral disease, and 3 had unilateral disease. Initial surgery was bilateral encephaloduroarteriosynangiosis (EDAS) in 9, unilateral EDAS alone in 3, unilateral EDAS with contralateral superficial temporal artery–middle cerebral artery (STA-MCA) bypass in 2, bilateral encephalomyosynangiosis (EMS) in 1, and unilateral EMS in 1. Thirteen of the 16 patients continued to have TIAs in the hemisphere ipsilateral to surgery, whereas 1 patient had seizures and cognitive deficit, 1 had asymptomatic infarct on MR imaging, and 1 had visual symptoms. Poor revascularization was seen on angiography studies in all patients. The median duration between the surgeries was 24 months (3 months–10 years). Repeat revascularization was performed in 23 hemispheres (16 patients). Direct revascularization was performed in 14 hemispheres (60.9%): STA-MCA bypass in 10, external carotid artery–MCA vein bypass in 2, occipital artery (OA)–MCA in 1, and OA–posterior cerebral artery in 1 hemisphere. Indirect revascularization was performed for patients without an acceptable recipient vessel, and was done in 9 hemispheres. The procedures included EMS (4 hemispheres), repeat EDAS (2), and omental transposition (3). There was 1 postoperative death in a patient undergoing a high-flow vein graft implantation. None of the other patients experienced any neurological worsening after surgery. Follow-up was available in all patients, ranging from 3 to 144 months (mean 34 months, median 12 months). Of the 15 patients who survived repeat revascularization surgery, 12 (80%) were free from any TIA, stroke, or any other neurological symptoms. Two patients had occasional TIAs, less frequent than before, whereas 1 patient had frequent TIAs and underwent revision of the revascularization. Angiographic studies were available in 11 patients, and showed improved flow in the hemispheres in 10 patients. Follow-up MR imaging performed at 6 months did not reveal a new infarct in any patient. Conclusions Repeat revascularization procedures are effective for patients who are clinically symptomatic and have inadequate collateral vessels following indirect procedures. Although direct procedures are preferred, the choice of procedure depends on the operative findings and the status of donor and recipient vessels.

scholarly journals Clinical features, surgical treatment, and long-term outcome in adult patients with moyamoya disease

2009 ◽  
Vol 111 (5) ◽  
pp. 936-942 ◽  
Author(s):  
Robert M. Starke ◽  
Ricardo J. Komotar ◽  
Zachary L. Hickman ◽  
Yehuda E. Paz ◽  
Angela G. Pugliese ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Clinical Features ◽  
Moyamoya Disease ◽  
Adult Patients ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Baseline Status ◽  
Collateral Vessels

Object The object of this study was to report the clinical features, surgical treatment, and long-term outcomes in adults with moyamoya phenomenon treated at a single institution in the US. Methods Forty-three adult patients with moyamoya disease (mean age 40 ± 11 years [SD], range 18–69 years) were treated with encephaloduroarteriosynangiosis (EDAS). Neurologists examined patients pre- and postoperatively. Follow-up was obtained in person or by structured telephone interview (median 41 months, range 4–126 months). The following outcomes were collected: transient ischemic attack (TIA), infarction, graft collateralization, change in cerebral perfusion, and functional level according to the modified Rankin scale (mRS). Kaplan-Meier estimates of infarction risk were calculated for comparison of surgically treated and contralateral hemispheres. Results The majority of patients were women (65%), were Caucasian (65%), presented with ischemic symptoms (98%), and had bilateral disease (86%). Nineteen patients underwent unilateral and 24 patients bilateral EDAS (67 treated hemispheres). Collateral vessels developed in 50 (98%) of 52 hemispheres for which imaging was available and there was evidence of increased perfusion on SPECT scans in 41 (82%) of the 50 hemispheres evaluated. Periprocedural infarction (< 48 hours) occurred in 3% of the hemispheres treated. In the follow-up period patients experienced 10 TIAs, 6 infarctions, and 1 intracranial hemorrhage. Although the hemisphere selected for surgery was based upon patients' symptoms and severity of pathology, the 5-year infarction-free survival rate was 94% in the surgically treated hemispheres versus < 36% in the untreated hemispheres (p = 0.007). After controlling for age and sex, infarction was 89% less likely to occur in the surgically treated hemispheres than in the contralateral hemispheres (hazard ratio 0.11, 95% CI 0.02–0.56). Thirty-eight (88%) of 43 patients had preserved or improved mRS scores, relative to baseline status. Conclusions In this mixed-race population of North American patients, indirect bypass promoted adequate pial collateral development and increased perfusion in the majority of adult patients with moyamoya disease. Patients had low rates of postoperative TIAs, infarction, and hemorrhage, and the majority of patients had preserved or improved functional status.

scholarly journals Diagnostic Evaluation of 99mTc-TRODAT-1 SPECT in Parkinsonism

2021 ◽  
Vol 9 (1) ◽  
pp. 36-36
Author(s):  
Zahra Babaei Aghdam ◽  
Safa Najmi Tabrizi ◽  
Amin Arasteh ◽  
Mohammad Khalafi ◽  
Morteza Ghojazadeh ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Healthy Subjects ◽  
Clinical Presentation ◽  
Evaluation Study ◽  
Diagnostic Value ◽  
Test Evaluation ◽  
Diagnostic Test Evaluation ◽  
Uptake Pattern ◽  
Spect Images

Background Parkinsonism as a group of movement disorders, exhibit similar clinical presentation. Therefore, clinically differentiating these diseases is difficult. We investigated the diagnostic value of 99m Tc-TRODAT-1 SPECT in this setting. Due to the fact that this modality has some limitations in imaging small organs like the sub-regions of basal ganglia, we also evaluated the use of anatomical MR imaging along with functional SPECT imaging in parkinsonism. Methods This follow-up diagnostic test evaluation study was performed with 40 patients with the clinical presentation of parkinsonism, and 10 healthy subjects as controls. After administration of the radiopharmaceutical, SPECT images were acquired, then co-registered on MRI. Uptake values were evaluated in basal ganglia semi-quantitatively. Results In this study, 99mTc-TRODAT-1 SPECT was able to differentiate essential tremor and healthy subjects from progressive supranuclear palsy (PSP) and Parkinson’s disease (PD) with a sensitivity of 76.47% and specificity of 100% at a cut-off of 0.53; however, findings were not significant in differentiation of PD from PSP (p ˃0.05), and the results were similar in SPECT and co-registered MRI/SPECT images. In evaluation of the uptake pattern in basal ganglia, the lateralization of decreased uptake was only seen in PD; and in PSP, the dysfunction was bilateral in all patients. Conclusion 99mTc-TRODAT-1 SPECT is sensitive and specific in diagnosing basal ganglia dysfunction; however, 99mTc-TRODAT-1 SPECT alone or co-registration on MRI are not adequate in differentiation of the etiologies of basal ganglia dysfunction.

PP151 Comparison Of Patients Undergoing New Technology For Prostate Cancer

2018 ◽  
Vol 34 (S1) ◽  
pp. 125-126
Author(s):  
Deyvid Silva ◽  
Letícia Lazarini ◽  
Araujo Aline ◽  
Evelinda Trindade ◽  
Otavio Becker ◽  
...  
Keyword(s):  
Intensive Care ◽  
Operating Room ◽  
Gleason Score ◽  
Open Surgery ◽  
Sao Paulo ◽  
High Intensity Focused Ultrasound ◽  
Disease Stage ◽  
Consecutive Series ◽  
São Paulo

Introduction:Prostate neoplasia affects more than one million people worldwide. Surgical treatments have evolved from open or video prostatectomy, up to the High Intensity Focused Ultrasound (HIFU) technique. HIFU studies cite less costs and better quality of life during the first year of follow-up. The objective of this study is to describe a consecutive series of eligible patients, with Gleason score 6 and 7, and compare resources used along those three treatment techniques.Methods:A comparative and retrospective study was conducted during the first 2017 semester, at Hospital de Transplantes de São Paulo, São Paulo city, Brazil. Consecutive eligible patients were matched by age, disease stage and profile and Gleason score 6 or 7. Resources used were assessed through medical records review and in- and out-patient visit interviews.Results:A total of 152 patients were followed: 50 underwent open surgery prostatectomy, 50 underwent video prostatectomy and 52 underwent HIFU. Mean age did not differ between groups (66.6, 64.1 and 65.6 years, respectively). All patients were followed for at least three months. The average operating room time was 4.7, 4.1 and 2.3 hours, and the average anesthetic recovery time was 2.0, 1.9 and 2.0 hours, respectively. Average inpatient length of stay was 2.5, 2.7 and 1.5 days, respectively. Postoperatively, nine (18 percent) open surgery patients, and 14 (28 percent) video-prostatectomy patients required an average of one full day of intensive care, compared to only one (2 percent) HIFU patient. During follow-up, the same effectiveness was observed between the groups, none required re-intervention. Thus, considering the 50 percent economy in hours of operating room and of days of hospital stay, as well as 10 times less use of intensive care unit days when the HIFU technique was compared to conventional surgeries, it is estimated the HIFU program allowed 30 percent cost savings.Conclusions:The HIFU program presented effectiveness and savings. The hospital can increase access to care for prostate neoplasia patients.

scholarly journals Clinical Outcome of Hypertensive Uveitis

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Deborah Lewkowicz ◽  
François Willermain ◽  
Lia Judice Relvas ◽  
Dorine Makhoul ◽  
Sarah Janssens ◽  
...  
Keyword(s):  
Intraocular Pressure ◽  
Surgical Treatment ◽  
Clinical Outcome ◽  
Topical Corticosteroid ◽  
Retrospective Review ◽  
Corticosteroid Treatment ◽  
First Episode ◽  
Elevated Intraocular Pressure ◽  
Unilateral Disease

Purpose. To review the clinical outcome of patients with hypertensive uveitis.Methods. Retrospective review of uveitis patients with elevated intraocular pressure (IOP) > 25 mmHg and >1-year follow-up. Data are uveitis type, etiology, viral (VU) and nonviral uveitis (NVU), IOP, and medical and/or surgical treatment.Results. In 61 patients, IOP values are first 32.9 mmHg (SD: 9.0), highest 36.6 mmHg (SD: 9.9), 3 months after the first episode 19.54 mmHg (SD: 9.16), and end of follow-up 15.5 mmHg (SD: 6.24). Patients with VU (n=25) were older (50.6 y/35.7 y,p=0.014) and had more unilateral disease (100%/72.22%  p=0.004) than those with NVU (n=36). Thirty patients (49.2%) had an elevated IOP before topical corticosteroid treatment. Patients with viral uveitis might have higher first elevated IOP (36.0/27.5 mmHg,p=0,008) and maximal IOP (40.28/34.06 mmHg,p=0.0148) but this was not significant when limited to the measurements before the use of topical corticosteroids (p=0.260and 0.160). Glaucoma occurred in 15 patients (24.59%) and was suspected in 11 (18.03%) without difference in viral and nonviral groups (p=0.774).Conclusion. Patients with VU were older and had more unilateral hypertensive uveitis. Glaucoma frequently complicates hypertensive uveitis. Half of the patients had an elevated IOP before topical corticosteroid treatment.

Is “unilateral” moyamoya disease different from moyamoya disease?

1996 ◽  
Vol 85 (5) ◽  
pp. 772-776 ◽  
Author(s):  
Kiyohiro Houkin ◽  
Hiroshi Abe ◽  
Tetsuyuki Yoshimoto ◽  
Akihiro Takahashi
Keyword(s):  
Magnetic Resonance ◽  
Magnetic Resonance Angiography ◽  
Moyamoya Disease ◽  
Familial Occurrence ◽  
Conventional Angiography ◽  
Content Type ◽  
Angiographic Evidence ◽  
Unilateral Moyamoya Disease ◽  
Revascularization Surgery

✓ Whether a diagnosis of moyamoya disease is justified in patients with typical angiographic evidence of moyamoya disease unilaterally and normal angiographic findings contralaterally remains controversial. In this study the authors analyzed longitudinal angiographic change, familial occurrence, and basic fibroblast growth factor (bFGF) concentration in the cerebrospinal fluid (CSF) of patients with “unilateral” moyamoya disease. Over a 10-year period, 10 cases of unilateral moyamoya disease were followed using conventional angiography or magnetic resonance angiography. Basic FGF in CSF, obtained from the subarachnoid space of the cerebral cortex during revascularization surgery, was measured in five cases. Among the 10 cases of unilateral moyamoya disease, only one pediatric case showed obvious signs of progression to typical bilateral disease. The other nine cases (including six adults and three children) remained stable throughout follow-up radiological examinations (magnetic resonance angiography) with a mean observation period of 3.5 years. There was no familial occurrence in these cases of unilateral moyamoya disease. Levels of bFGF, which are high in typical moyamoya disease, were low in these patients. The progression from unilateral moyamoya disease to the typical bilateral form of the disease appears to be infrequent. The low levels of bFGF in the CSF of these patients and the lack of familial occurrence strongly suggest that most cases of unilateral moyamoya disease, especially those found in adults, are distinct from typical bilateral moyamoya disease.

scholarly journals Clinical Features and Treatment of Fibrous Histiocytomas of the Tongue: A Systematic Review

2017 ◽  
Vol 22 (01) ◽  
pp. 094-102 ◽  
Author(s):  
Austin Nguyen ◽  
Adam Vaudreuil ◽  
Paul Haun ◽  
Gabriel Caponetti ◽  
Christopher Huerter
Keyword(s):  
Surgical Treatment ◽  
Clinical Presentation ◽  
Malignant Tumors ◽  
Case Reports ◽  
Data Synthesis ◽  
Excellent Outcome ◽  
Fibrous Histiocytomas ◽  
Pleomorphic Sarcoma ◽  
Level Data

Introduction Benign fibrous histiocytomas are common lesions of the skin that rarely affect the tongue. Such cases are available in the literature exclusively as case reports. Similarly, malignant fibrous histiocytoma, now classified as undifferentiated pleomorphic sarcoma, is exceedingly rare in the tongue and not fully understood. Objectives This study systematically reviews the available literature discussing the clinical and pathological features of malignant and benign fibrous histiocytomas. Data Synthesis A total of 20 cases were included in this review. Patient-level data were extracted from cases to include clinical presentation, workup, treatment, and outcome. Conclusion Benign fibrous histiocytomas are consistent in clinical and histopathologic presentation. Surgical treatment provides excellent outcome, with no recurrence in all excised cases. Malignant tumors have a more aggressive clinical and pathological presentation. Surgical treatment with possible adjuvant radiotherapy resulted in recurrence in 40% of cases (follow-up of 24 months), and death due to disease in 47% of patients (follow-up of 19 months).

Clinical outcome after 450 revascularization procedures for moyamoya disease

2009 ◽  
Vol 111 (5) ◽  
pp. 927-935 ◽  
Author(s):  
Raphael Guzman ◽  
Marco Lee ◽  
Achal Achrol ◽  
Teresa Bell-Stephens ◽  
Michael Kelly ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Clinical Characteristics ◽  
Pediatric Patients ◽  
Consecutive Series ◽  
Modified Rankin Scale ◽  
Surgical Data ◽  
Revascularization Surgery

Object Moyamoya disease (MMD) is a rare cerebrovascular disease mainly described in the Asian literature. To address a lack of data on clinical characteristics and long-term outcomes in the treatment of MMD in North America, the authors analyzed their experience at Stanford University Medical Center. They report on a consecutive series of patients treated for MMD and detail their demographics, clinical characteristics, and long-term surgical outcomes. Methods Data obtained in consecutive series of 329 patients with MMD treated microsurgically by the senior author (G.K.S.) between 1991 and 2008 were analyzed. Demographic, clinical, and surgical data were prospectively gathered and neurological outcomes assessed in postoperative follow-up using the modified Rankin Scale. Association of demographic, clinical, and surgical data with postoperative outcome was assessed by chi-square, uni- and multivariate logistic regression, and Kaplan-Meier survival analyses. Results The authors treated a total of 233 adult patients undergoing 389 procedures (mean age 39.5 years) and 96 pediatric patients undergoing 168 procedures (mean age 10.1 years). Direct revascularization technique was used in 95.1% of adults and 76.2% of pediatric patients. In 264 patients undergoing 450 procedures (mean follow-up 4.9 years), the surgical morbidity rate was 3.5% and the mortality rate was 0.7% per treated hemisphere. The cumulative 5-year risk of perioperative or subsequent stroke or death was 5.5%. Of the 171 patients presenting with a transient ischemic attack, 91.8% were free of transient ischemic attacks at 1 year or later. Overall, there was a significant improvement in quality of life in the cohort as measured using the modified Rankin Scale (p < 0.0001). Conclusions Revascularization surgery in patients with MMD carries a low risk, is effective at preventing future ischemic events, and improves quality of life. Patients in whom symptomatic MMD is diagnosed should be offered revascularization surgery.

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