Discovery of asymptomatic moyamoya arteriopathy in pediatric syndromic populations: radiographic and clinical progression

2011 ◽  
Vol 31 (6) ◽  
pp. E6 ◽  
Author(s):  
Ning Lin ◽  
Lissa Baird ◽  
McKenzie Koss ◽  
Kimberly E. Kopecky ◽  
Evelyne Gone ◽  
...  

Object Limited data exist to guide management of incidentally discovered pediatric moyamoya. Best exemplified in the setting of unilateral moyamoya, in which the unaffected side is monitored, this phenomenon also occurs in populations undergoing routine surveillance of the cerebral vasculature for other conditions, such as sickle cell disease (SCD) or neurofibromatosis Type 1 (NF1). The authors present their experience with specific syndromic moyamoya populations to better characterize the natural history of radiographic and clinical progression in patients with asymptomatic moyamoya. Methods The authors performed a retrospective review of the clinical database of the neurosurgery department at Children's Hospital Boston, including both nonoperative referrals and a consecutive series of 418 patients who underwent surgical revascularization for moyamoya disease between 1988 and 2010. Results Within the period of time studied, 83 patients were asymptomatic at the time of radiographic diagnosis of moyamoya, while also having either unilateral moyamoya or moyamoya in association with either SCD or NF1. The mean age at presentation was 9.1 years (range 1–21 years), and there were 49 female (59%) and 34 male (41%) patients. The mean follow-up duration was 5.4 ± 3.8 years (mean ± SD), with 45 patients (54%) demonstrating radiographic progression and 37 (45%) becoming symptomatic within this period. Patients with SCD had the highest incidence of both radiographic (15 patients [75%]) and clinical (13 patients [65%]) progression, followed by NF1 (20 patients [59%] with radiographic progression and 15 patients [44%] with clinical progression) and patients with unilateral moyamoya (10 patients [35%] with radiographic progression and 9 patients [31%] with clinical progression). Conclusions Radiographic progression occurred in the majority of asymptomatic patients and generally heralded subsequent clinical symptoms. These data demonstrate that moyamoya is a progressive disorder, even in asymptomatic populations, and support the rationale of early surgical intervention to minimize morbidity from stroke.

Stroke ◽  
2012 ◽  
Vol 43 (suppl_1) ◽  
Author(s):  
Ning Lin ◽  
Lissa Baird ◽  
R. Michael Scott ◽  
Edward R Smith

Introduction: Limited data exists to guide management of incidentally discovered pediatric moyamoya. Best exemplified in the setting of unilateral moyamoya, in which the unaffected side is monitored, this phenomenon also occurs in populations undergoing routine surveillance of the cerebral vasculature for other conditions, such as sickle cell disease (SCD) or neurofibromatosis type I (NF1). Here we present our experience with specific syndromic moyamoya populations to better characterize the natural history of radiographic and clinical progression in asymptomatic moyamoya. Methods: Retrospective review of a consecutive series of 418 patients who underwent surgical revascularization for moyamoya disease at a single institution from 1988-2010. Results: Of 418 patients, 83 were asymptomatic at the time of radiographic diagnosis of moyamoya, while also having either unilateral moyamoya or moyamoya in association with either SCD or NF1. Average age at presentation was 9.1 years (range 1-21), with 47 females (60%) and 31 males (40%). Mean follow-up was 5.4 years (SD +/-3.8), with 45 patients (54%) demonstrating radiographic progression and 37 patients (44.6%) becoming symptomatic within this period. The time interval between radiographic progression from the diagnosis of syndromic disease, to evidence of arteriopathy, to signs of slow cortical blood flow, and to stroke was 5.8 years (SD +/-4.7), 0.7 years (SD +/-1.1), and 0.3 years (SD +/-0.5), respectively. SCD patients had the highest incidence of both radiographic (n=15, 75%) and clinical (n=13, 65%) progression, followed by NF1 (n=20, 59% radiographic, n=15, 44% clinical) and unilateral patients (n=10, 34.5% radiographic, n=9, 31%). Transient ischemic attacks were the most frequent symptoms during follow-up (29 patients, 35%), and 10 patients (12%) developed clinical stroke. Overall 49 patients (59%) underwent surgical treatment, and the time between arteriopathy to surgery was 1.1 years (SD +/-1.2). Conclusion: Radiographic progression occurred in the majority of asymptomatic patients and generally heralded subsequent clinical symptoms. SCD or NF patients with asymptomatic moyamoya are more likely to progress and require treatment than unilateral moyamoya patients without a syndromic disease. These data demonstrate that moyamoya is a progressive disorder, even in asymptomatic populations, and supports the rationale of early surgical intervention to minimize morbidity from stroke.


Author(s):  
Yu Lei ◽  
Xiaobo Huang ◽  
Bamu SiLang ◽  
YunPing Lan ◽  
Jianli Lu ◽  
...  

AbstractCoronavirus disease 2019 (COVID-19), caused by SARS-CoV-2, has rapidly spread throughout China, but the clinical characteristics of Tibetan patients living in the Qinghai-Tibetan plateau are unknown. We aimed to investigate the epidemiological, clinical, laboratory and radiological characteristics of these patients. We included 67 Tibetan patients with confirmed SARS-CoV-2 infection. The patients were divided into two groups based on the presence of clinical symptoms at admission, with 31 and 36 patients in the symptomatic and asymptomatic groups, respectively. The epidemiological, clinical, laboratory and radiological characteristics were extracted and analysed. No patient had a history of exposure to COVID-19 patients from Wuhan or had travelled to Wuhan. The mean age of Tibetan patients was 39.3 years and 59% of the patients were male. Seven patients presented with fever on admission and lymphocytopenia was present in 20 patients. 47 patients had abnormal chest CTs at admission instead of stating that 20 were unchanged. Lactate dehydrogenase levels were increased in 31 patients. Seven patients progressed to severe COVID-19; however, after treatment, their condition was stable. No patients died. Of the 36 asymptomatic patients, the mean age was younger than the symptomatic group (34.4±17.3vs 44.9±18.1 years, P=0.02). Lymphocyte count and prealbumin levels were higher in the asymptomatic group than the group with clinical symptoms (1.6±0.5 vs 1.3±0.6 and 241.8±68.2 vs 191.9±60.3, respectively; P<0.05). Imported cases of COVID-19 in Tibetan patients were generally mild in this high-altitude area. Absence of fever or radiologic abnormalities on initial presentation were common.


2020 ◽  
Author(s):  
Yu lei ◽  
xiaobo huang ◽  
silang bamu ◽  
yunping lan ◽  
jianli lu ◽  
...  

Abstract Background: Coronavirus disease 2019 (COVID-19), caused by SARS-CoV-2, has rapidly spread throughout China, but the clinical characteristics of Tibetan patients living in the Qinghai-Tibetan plateau are unknown. We aimed to investigate the epidemiological, clinical, laboratory and radiological characteristics of these patients. Methods: We included 67 Tibetan patients with confirmed SARS-CoV-2 infection. The patients were divided into two groups based on the presence of clinical symptoms at admission, with 31 and 36 patients in the symptomatic and asymptomatic groups, respectively. The epidemiological, clinical, laboratory and radiological characteristics were extracted and analysed. Results: No patient had a history of exposure to COVID-19 patients from Wuhan or had travelled to Wuhan. The mean age of Tibetan patients was 39.3 years and 59% of the patients were male. Seven patients presented with fever on admission and lymphocytopenia was present in 20 patients. 47 patients had abnormal chest CTs at admission instead of stating that 20 were unchanged. Lactate dehydrogenase levels were increased in 31 patients. Seven patients progressed to severe COVID-19; however, after treatment, their condition was stable. No patients died. Of the 36 asymptomatic patients, the mean age was younger than the symptomatic group (34.4 ± 17.3vs 44.9 ± 18.1 years, P = 0.02). Lymphocyte count and prealbumin levels were higher in the asymptomatic group than the group with clinical symptoms (1.6 ± 0.5 vs 1.3 ± 0.6 and 241.8 ± 68.2 vs 191.9 ± 60.3, respectively; P < 0.05). Conclusions: Imported cases of COVID-19 in Tibetan patients were generally mild in this high-altitude area. Absence of fever or radiologic abnormalities on initial presentation were common.


1991 ◽  
Vol 10 (4) ◽  
pp. 275-278 ◽  
Author(s):  
Jacob Peedicayil ◽  
Kalpana Ernest ◽  
Molly Thomas ◽  
A.S. Kanagasabapathy ◽  
P.M. Stephen

This paper presents the findings of a study of serum pseudocholinesterase activity in a group of 36 industrial workers chronically exposed to organophosphorus (OP) compounds. The mean pseudocholinesterase level of the workers was significantly lower than that of 36 other workers without a history of similar exposure. Although there was a high incidence of clinical features suggestive of OP compound toxicity in the exposed workers, no significant correlation between serum pseudocholinesterase levels and clinical symptoms and signs was found. Six exposed workers, found to have low serum pseudocholinesterase levels, were transferred for 6 months to work areas which did not involve OP exposure, whereupon their levels rose significantly back to the normal range.


2020 ◽  
Vol 32 (2) ◽  
pp. 191-199 ◽  
Author(s):  
Zach Pennington ◽  
Daniel Lubelski ◽  
Erick M. Westbroek ◽  
A. Karim Ahmed ◽  
Jeff Ehresman ◽  
...  

OBJECTIVEPostoperative C5 palsy affects 7%–12% of patients who undergo posterior cervical decompression for degenerative cervical spine pathologies. Minimal evidence exists regarding the natural history of expected recovery and variables that affect palsy recovery. The authors investigated pre- and postoperative variables that predict recovery and recovery time among patients with postoperative C5 palsy.METHODSThe authors included patients who underwent posterior cervical decompression at a tertiary referral center between 2004 and 2018 and who experienced postoperative C5 palsy. All patients had preoperative MR images and full records, including operative note, postoperative course, and clinical presentation. Kaplan-Meier survival analysis was used to evaluate both times to complete recovery and to new neurological baseline—defined by deltoid strength on manual motor testing of the affected side—as a function of clinical symptoms, surgical maneuvers, and the severity of postoperative deficits.RESULTSSeventy-seven patients were included, with an average age of 64 years. The mean follow-up period was 17.7 months. The mean postoperative C5 strength was grade 2.7/5, and the mean time to first motor examination with documented C5 palsy was 3.5 days. Sixteen patients (21%) had bilateral deficits, and 9 (12%) had new-onset biceps weakness; 36% of patients had undergone C4–5 foraminotomy of the affected root, and 17% had presented with radicular pain in the dermatome of the affected root. On univariable analysis, patients’ reporting of numbness or tingling (p = 0.02) and a baseline deficit (p < 0.001) were the only predictors of time to recovery. Patients with grade 4+/5 weakness had significantly shorter times to recovery than patients with grade 4/5 weakness (p = 0.001) or ≤ grade 3/5 weakness (p < 0.001). There was no difference between those with grade 4/5 weakness and those with ≤ grade 3/5 weakness. Patients with postoperative strength < grade 3/5 had a < 50% chance of achieving complete recovery.CONCLUSIONSThe timing and odds of recovery following C5 palsy were best predicted by the magnitude of the postoperative deficit. The use of C4–5 foraminotomy did not predict the time to or likelihood of recovery.


2021 ◽  
Vol 2 (11) ◽  
Author(s):  
Jorn Van Der Veken ◽  
Marguerite Harding ◽  
Saba Hatami ◽  
Marc Agzarian ◽  
Nick Vrodos

BACKGROUND Chiari Type I malformation (CM1) is a disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Syringomyelia is frequently found in patients with CM1, but the pathophysiology of syringomyelia remains an enigma. As a general consensus, symptomatic patients should be treated and asymptomatic patients without a syrinx should not be treated. Mildly symptomatic patients or asymptomatic patients with a syrinx, on the other hand, pose a more challenging dilemma, as the natural evolution is uncertain. For many surgeons, the presence of a syrinx is an indication to offer surgery even if the patient is asymptomatic or mildly symptomatic. OBSERVATIONS The authors describe an illustrative case of a 31-year-old female with an incidental finding of a CM1 malformation and cervical syrinx in 2013. Conservative management was advocated as the patient was asymptomatic. Monitoring of the syrinx over a course of 8 years showed resolution, followed by reappearance and finally a complete resolution in 2021. A review of the literature and the possible pathophysiology is discussed. LESSONS The unusual course of this patient highlights the importance of guiding treatment by clinical symptoms, not radiological findings. Furthermore it reflects the complexity of the pathophysiology and the uncertain natural history of syringomyelia.


2020 ◽  
Vol 32 (1) ◽  
Author(s):  
Ayodele S. Babalola ◽  
J. Jonathan ◽  
Boluwatife E. Michael

Abstract Background Asymptomatic malaria is a threat to malaria eradication program. Host-related factors (e.g., immunity, oxidative stress, anti-oxidants activities) associated with asymptomatic malaria remain a gray area in research. This study seeks to determine the serum level of oxidative stress and anti-oxidants in 130 symptomatic and asymptomatic patients with different intensities of malaria parasite infection from a hospital in Ibadan, Nigeria. Results The prevalence of infection was 48.5% among the respondents. Most of the patients with parasitemia were asymptomatic (64.7%). The mean titer value of malondialdehyde (MDA) was significantly higher (p < 0.05) among those with malaria infection (6.05 ± 0.60) compared with those who tested negative for malaria parasites (2.38 ± 0.28). Furthermore, the mean titer value of MDA was significantly higher (p < 0.05) among patients who showed symptoms of malaria (5.49 ± 0.77) compared with those without symptoms (2.93 ± 0.47). A strong positive relationship existed between MDA (r = 0.717, p < 0.05), glutathione peroxidase (GPx) (r = 0.695, p < 0.05), and density of infection. On the other hand, a weak negative correlation existed between intensity and superoxide dismutase (SOD) (r = − 0.115, p > 0.05) and glutathione (GSH) (r = − 0.278, p > 0.05). The level of SOD and GSH also decreased significantly (p < 0.05) with an increase in MDA level. Conclusions This study showed that lipid peroxidation did not only increase in positive patients, it also rises in patients with clinical symptoms of malaria. Furthermore, a similar level of anti-oxidant responses was observed in both symptomatic and asymptomatic malaria patients. There is a need to inform health policies that encourage routine diagnosis and treatment of malaria in apparently healthy people if the malaria elimination goal is to be achieved in Africa.


2021 ◽  
Vol 12 ◽  
Author(s):  
Klára Fekete ◽  
Judit Tóth ◽  
László Horváth ◽  
Sándor Márton ◽  
Máté Héja ◽  
...  

Introduction: Intracerebral hemorrhage (ICH) is a devastating disease, which may lead to severe disability or even death. Although many factors may influence the outcome, neurophysiological examinations might also play a role in its course. Our aim was to examine whether the findings of electroencephalography (EEG) and transcranial magnetic stimulation (TMS) can predict the prognosis of these patients.Methods: Between June 1 2017 and June 15 2021, 116 consecutive patients with ICH were enrolled prospectively in our observational study. Clinical examinations and non-Contrast computed tomography (NCCT) scan were done on admission for ICH; follow-up NCCT scans were taken at 14 ± 2 days and at 3 months ± 7 days after stroke onset. EEG and TMS examinations were also carried out.Results: Of the patients in the study, 65.5% were male, and the mean age of the study population was 70 years. Most patients had a history of hypertension, 50.8% of whom had been untreated. In almost 20% of the cases, excessive hypertension was measured on admission, accompanied with &gt;10 mmol/L blood glucose level, whereas their Glasgow Coma Scale was 12 on average. Presence of blood in the ventricles or subarachnoid space and high blood and perihematomal volumes meant poor prognosis. Pathological EEG was prognostic of a worse outcome. With TMS examination at 14 days, it might be possible to estimate outcome in a univariate model and the absence, or reduction of the amplitude of the motor evoked potentials was associated with poor prognosis.Conclusion: Together with the clinical symptoms, the volume of bleeding, perihematomal edema (or their combined volume), and neurophysiological examinations like EEG and TMS play an important role in the neurological outcome of patients with ICH. This might affect the patients' rehabilitation plans in the future, since with the help of the examinations the subset of patients with potential for recovery could be identified.


2014 ◽  
Vol 121 (Suppl_2) ◽  
pp. 143-149 ◽  
Author(s):  
Shibin Sun ◽  
Ali Liu

ObjectThe aim of this study was to evaluate long-term clinical outcomes after Gamma Knife surgery (GKS) for patients with neurofibromatosis Type 2 (NF2) and the role of GKS in the management of NF2.MethodsFrom December 1994 through December 2008, a total of 46 patients (21 male, 25 female) with NF2 underwent GKS and follow-up evaluation for at least 5 years at the Gamma Knife Center of the Beijing Neurosurgical Institute. GKS was performed using the Leksell Gamma Knife Models B and C. The mean age of the patients was 30 years (range 13–59 years). A family history of NF2 was found for 9 (20%) patients. The NF2 phenotype was thought to be Wishart for 20 (44%) and Feiling-Gardner for 26 (56%) patients. Among these 46 patients, GKS was performed to treat 195 tumors (73 vestibular schwannomas and 122 other tumors including other schwannomas and meningiomas). For vestibular schwannomas, the mean volume was 5.1 cm3 (median 3.6 cm3, range 0.3–27.3 cm3), the mean margin dose was 12.9 Gy (range 10–14 Gy), and the mean maximum dose was 27.3 Gy (range 16.2–40 Gy). For other tumors, the mean volume was 1.7 cm3 (range 0.3–5.5 cm3), the mean margin dose was 13.3 Gy (range 11–14 Gy), and the mean maximum dose was 26.0 Gy (range 18.0–30.4 Gy). The median duration of follow-up was 109 months (range 8–195 months).ResultsFor the 73 vestibular schwannomas that underwent GKS, the latest follow-up MR images demonstrated regression of 30 (41%) tumors, stable size for 31 (43%) tumors, and enlargement of 12 (16%) tumors. The total rate of tumor control for bilateral vestibular schwannomas in patients with NF2 was 84%. Of the 122 other types of tumors that underwent GKS, 103 (85%) showed no tumor enlargement. The rate of serviceable hearing preservation after GKS was 31.9% (15/47). The actuarial rates for hearing preservation at 3 years, 5 years, 10 years, and 15 years were 98%, 93%, 44%, and 17%, respectively. Of the 46 patients, 22 (48%) became completely bilaterally deaf, 17 (37%) retained unilateral serviceable hearing, and 7 (15%) retained bilateral serviceable hearing. The mean history of the disease course was 12 years (range 5–38 years).ConclusionsGKS was confirmed to provide long-term local tumor control for small- to medium-sized vestibular schwannomas and other types of tumors, although vestibular schwannomas in patients with NF2 responded less well than did unilateral sporadic vestibular schwannomas. Phenotype is the most strongly predictive factor of final outcome after GKS for patients with NF2. The risk for loss of hearing is high, whereas the risk for other cranial nerve complications is low.


Author(s):  
Majid Rezaei Tavirani ◽  
Hazhir Heidari Beigvand

Introduction: Drug abuse and its complications is a socio-health problem in Middle Eastern countries such as Iran. Smugglers may add lead to drug during drug production, which is in order to increase its weight for greater benefit. Considering the frequency of the patients with various complaints among Iranians, this study was designed and conducted to evaluate the clinical symptoms and serum levels of lead in patients being admitted to Hazrat Rasoul Akram, Firoozgar, Firouzabadi and Haft Tir hospitals with any complaints. Materials and Methods: In this case-control study, serum levels of lead were measured in 128 samples in case and control groups. The case group consisted of 64 patients using oral opium who being admitted to Rasoul Akram and Firoozgar, Haft Tir and Firouzabadi hospitals with different complaints in May 2017. The control group consisted of 64 patients with no history of addiction that were homogenized with the case group in terms of age and sex. They were evaluated for serum levels of lead and other variables. Data were analyzed by SPSS software. Results: The mean serum lead level was 76.34±17.82 in the group using opium and was 7.68±3.72 in the control group that the difference was statistically significant (P <0.001). The most common complaints of patients were abdominal pain and symptoms of bowel obstruction. The mean rate of oral opium consumption was 1.73±0.23 in subjects under 50 years old and with a mean of 2.89±0.27 in subjects over 50 years old. Serum lead level was significantly (P = 0.032) increased compared to the amount consumed. Duration of oral opium consumption was 5 months to 30 years with a mean of 15.24 years, which was not significantly correlated to serum lead level (P = 0.213). Also, the hemoglobin range was significantly correlated to different levels of lead in patients consuming oral opium (P = 0.027). Conclusion: The findings of the present study showed a high mean serum lead level in oral opium addicts in the study population. The results also confirm numerous reports suggesting the definitive diagnosis of lead poisoning as a justifying factor in addicted patients with nonspecific symptoms, which may indicate the need for serum lead level screening in opiate addicts to prevent more serious complications.


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