scholarly journals Motor impairment in a rare form of spastic paraplegia (Spoan syndrome): a 10-year follow-up

2019 ◽  
Author(s):  
Cláudia R. C. Galvão ◽  
Priscilla M. A. Cavalcante ◽  
Ricardo Olinda ◽  
Zodja Graciani ◽  
Mayana Zatz ◽  
...  
Keyword(s):  
Statistical Significance ◽  
Hand Function ◽  
Age At Onset ◽  
Motor Impairment ◽  
Spastic Paraplegia ◽  
Prospective Longitudinal Study ◽  
Left Hand ◽  
The Mean ◽  
Prospective Longitudinal

Abstract Background: Spastic paraplegia, optic atrophy and neuropathy (Spoan syndrome) is an autosomal recessive disease with approximately 70 cases recorded in Brazil and Egypt. Methods: This is a prospective longitudinal study performed with 47 patients affected with Spoan syndrome of seven communities of Rio Grande do Norte (Brazil) to investigate changes in motor function based on comparative data obtained from a 10-year follow-up. Results: The mean age of the participants was 47.21±12.42 years old, and the mean age at loss of ambulation and hand function were 10.78±5.55 and 33.58±17.47 years old, respectively. Spearman’s correlation analysis between the score on the Modified Barthel Index and the investigated variables evidenced statistical significance for age (p<0.001) and right- and left-hand grip strength (p=0.042 and p=0.021, respectively). Statistical significance was not evidenced for the remainder of the variables, including age at onset of symptoms (p=0.634), age at loss of ambulation (p=0.664) and age at loss of hand function (p=0.118). Conclusions: Our analysis allows asserting that the participants exhibited slight dependence until age 35. The greatest losses occurred from ages 35 to 41, and starting at 50, practically all patients become completely dependent. These findings are relevant for determining the prognosis as well as suitable treatment, rehabilitation and assistive technology for these individuals.

scholarly journals Motor impairment in a rare form of spastic paraplegia (Spoan syndrome): a 10-year follow-up

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Cláudia R. C. Galvão ◽  
Priscilla M. A. Cavalcante ◽  
Ricardo Olinda ◽  
Zodja Graciani ◽  
Mayana Zatz ◽  
...  
Keyword(s):  
Statistical Significance ◽  
Hand Function ◽  
Age At Onset ◽  
Motor Impairment ◽  
Spastic Paraplegia ◽  
Prospective Longitudinal Study ◽  
Left Hand ◽  
The Mean ◽  
Prospective Longitudinal

Abstract Background Spastic paraplegia, optic atrophy and neuropathy (Spoan syndrome) is an autosomal recessive disease with approximately 70 cases recorded in Brazil and Egypt. Methods This is a prospective longitudinal study performed with 47 patients affected with Spoan syndrome of seven communities of Rio Grande do Norte (Brazil) to investigate changes in motor function based on comparative data obtained from a 10-year follow-up. Results The mean age of the participants was 47.21 ± 12.42 years old, and the mean age at loss of ambulation and hand function were 10.78 ± 5.55 and 33.58 ± 17.47 years old, respectively. Spearman’s correlation analysis between the score on the Modified Barthel Index and the investigated variables evidenced statistical significance for age (p < 0.001) and right- and left-hand grip strength (p = 0.042 and p = 0.021, respectively). Statistical significance was not evidenced for the remainder of the variables, including age at onset of symptoms (p = 0.634), age at loss of ambulation (p = 0.664) and age at loss of hand function (p = 0.118). Conclusions Our analysis allows asserting that the participants exhibited slight dependence until age 35. The greatest losses occurred from ages 35 to 41, and starting at 50, practically all patients become completely dependent. These findings are relevant for determining the prognosis as well as suitable treatment, rehabilitation and assistive technology for these individuals.

scholarly journals Prognostic factors and clinical outcome in a rare form of spastic paraplegia (Spoan syndrome): a 10-year follow-up

2019 ◽  
Author(s):  
Cláudia R. C. Galvão ◽  
Priscilla M. A. Cavalcante ◽  
Ricardo Olinda ◽  
Zodja Graciani ◽  
Mayana Zatz ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Clinical Outcome ◽  
Statistical Significance ◽  
Hand Function ◽  
Age At Onset ◽  
Spastic Paraplegia ◽  
Prospective Longitudinal Study ◽  
The Mean ◽  
Prospective Longitudinal

Abstract Background: Spastic paraplegia, optic atrophy and neuropathy (Spoan syndrome) is an autosomal recessive disease with approximately 70 cases recorded in Brazil and Egypt. Methods: This is a prospective longitudinal study performed with 47 patients affected with Spoan syndrome of seven communities of Rio Grande do Norte (Brazil) to investigate prognostic factors and clinical outcome based on comparative data obtained from a 10-year follow-up. Results: The mean age of the participants was 47.21±12.42 years old, and the mean ages at loss of ambulation and hand function were 10.78±5.55 and 33.58±17.47 years old, respectively. Spearman’s correlation analysis between the score on the Modified Barthel Index and the investigated variables evidenced statistical significance for age (p<0.001) and right- and left-hand grip strength (p=0.042 and p=0.021, respectively). Statistical significance was not evidenced for the remainder of the variables, including age at onset of symptoms (p=0.634), age at loss of ambulation (p=0.664) and age at loss of hand function (p=0.118). Conclusions: Our analysis allows asserting that the participants exhibited slight dependence until age 35. The greatest losses occurred from ages 35 to 41, and starting at 50, practically all patients become completely dependent. In fact, age is the main prognostic factor of impaired motor function among Spoan syndrome patients.

scholarly journals Assessment of image-derived risk factors for natural course of unruptured cerebral aneurysms

2016 ◽  
Vol 124 (2) ◽  
pp. 288-295 ◽  
Author(s):  
Manasi Ramachandran ◽  
Rohini Retarekar ◽  
Madhavan L. Raghavan ◽  
Benjamin Berkowitz ◽  
Benjamin Dickerhoff ◽  
...  
Keyword(s):  
Statistical Significance ◽  
Cerebral Aneurysms ◽  
Expectant Management ◽  
Wall Tension ◽  
Prospective Longitudinal Study ◽  
Longitudinal Outcome ◽  
Imaging Surveillance ◽  
Unruptured Cerebral Aneurysms ◽  
Prospective Longitudinal

OBJECT The goal of this prospective longitudinal study was to test whether image-derived metrics can differentiate unruptured aneurysms that will become unstable (grow and/or rupture) from those that will remain stable. METHODS One hundred seventy-eight patients harboring 198 unruptured cerebral aneurysms for whom clinical observation and follow-up with imaging surveillance was recommended at 4 clinical centers were prospectively recruited into this study. Imaging data (predominantly CT angiography) at initial presentation was recorded. Computational geometry was used to estimate numerous metrics of aneurysm morphology that described the size and shape of the aneurysm. The nonlinear, finite element method was used to estimate uniform pressure-induced peak wall tension. Computational fluid dynamics was used to estimate blood flow metrics. The median follow-up period was 645 days. Longitudinal outcome data on these aneurysm patients—whether their aneurysms grew or ruptured (the unstable group) or remained unchanged (the stable group)—was documented based on follow-up at 4 years after the beginning of recruitment. RESULTS Twenty aneurysms (10.1%) grew, but none ruptured. One hundred forty-nine aneurysms (75.3%) remained stable and 29 (14.6%) were lost to follow-up. None of the metrics—including aneurysm size, nonsphericity index, peak wall tension, and low shear stress area—differentiated the stable from unstable groups with statistical significance. CONCLUSIONS The findings in this highly selected group do not support the hypothesis that image-derived metrics can predict aneurysm growth in patients who have been selected for observation and imaging surveillance. If aneurysm shape is a significant determinant of invasive versus expectant management, selection bias is a key limitation of this study.

Prospective longitudinal study of depression and anosognosia in Alzheimer's disease

1997 ◽  
Vol 171 (1) ◽  
pp. 47-52 ◽  
Author(s):  
Sergio E. Starkstein ◽  
Erán Chemerinski ◽  
Liliana Sabe ◽  
Gabriela Kuzis ◽  
Gustavo Petracca ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Major Depression ◽  
Cognitive Status ◽  
Initial Evaluation ◽  
Consecutive Series ◽  
Psychiatric Interview ◽  
Prospective Longitudinal Study ◽  
Prospective Longitudinal

BackgroundThe aim was to examine the longitudinal evolution of depression and anosognosia in patients with probable Alzheimer's disease (AD).MethodSixty-two of a consecutive series of 116 AD patients that were examined with a structured psychiatric interview had a follow-up evaluation between one and two years after the initial evaluation.ResultsAt the initial evaluation 19% of the 62 patients had major depression, 34% had dysthymia, and 47% were not depressed. After a mean follow-up of 16 months, 58% of patients with major depression at the initial evaluation were still depressed, whereas only 28% of patients with initial dysthymia and 21% of the non-depressed patients were depressed at follow-up. During the follow-up period, all three groups showed similar declines in cognitive status and activities of daily living. At the initial evaluation, 39% of the patients had anosognosia, and there was a significant increment of anosognosia during the follow-up period.ConclusionsWhile dysthymia in AD is a brief emotional disorder, major depression is a longer-lasting mood change. Anosognosia is another prevalent disorder among AD patients, and increases with the progression of the illness.

scholarly journals Cognitive Complaints in Survivors of Breast Cancer After Chemotherapy Compared With Age-Matched Controls: An Analysis From a Nationwide, Multicenter, Prospective Longitudinal Study

2017 ◽  
Vol 35 (5) ◽  
pp. 506-514 ◽  
Author(s):  
Michelle C. Janelsins ◽  
Charles E. Heckler ◽  
Luke J. Peppone ◽  
Charles Kamen ◽  
Karen M. Mustian ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Cognitive Impairment ◽  
Cognitive Function ◽  
Reading Ability ◽  
Menopausal Status ◽  
Prospective Longitudinal Study ◽  
Cognitive Difficulties ◽  
Community Oncology ◽  
Prospective Longitudinal

Purpose Cancer-related cognitive impairment is an important problem for patients with breast cancer, yet its trajectory is not fully understood. Some previous cancer-related cognitive impairment research is limited by heterogeneous populations, small samples, lack of prechemotherapy and longitudinal assessments, use of normative data, and lack of generalizability. We addressed these limitations in a large prospective, longitudinal, nationwide study. Patients and Methods Patients with breast cancer from community oncology clinics and age-matched noncancer controls completed the Functional Assessment of Cancer Therapy-Cognitive Function (FACT-Cog) at prechemotherapy and postchemotherapy and at a 6-month follow-up as an a priori exploratory aim. Longitudinal models compared FACT-Cog scores between patients and controls at the three assessments and adjusted for age, education, race, menopausal status, and baseline reading ability, anxiety, and depressive symptoms. A minimal clinically important difference cutoff determined percentages of impairment over time. Results Of patients, 581 patients with breast cancer (mean age, 53 years; 48% anthracycline-based regimens) and 364 controls (mean age, 53 years) were assessed. Patients reported significantly greater cognitive difficulties on the FACT-Cog total score and four subscales from prechemotherapy to postchemotherapy compared with controls as well as from prechemotherapy to 6-month follow-up (all P < .001). Increased baseline anxiety, depression, and decreased cognitive reserve were significantly associated with lower FACT-Cog total scores. Treatment regimen, hormone, or radiation therapy was not significantly associated with FACT-Cog total scores in patients from postchemotherapy to 6-month follow-up. Patients were more likely to report a clinically significant decline in self-reported cognitive function than were controls from prechemotherapy to postchemotherapy (45.2% v 10.4%) and from prechemotherapy to 6-month follow-up (36.5% v 13.6%). Conclusion Patients with breast cancer who were treated in community oncology clinics report substantially more cognitive difficulties up to 6 months after treatment with chemotherapy than do age-matched noncancer controls.

scholarly journals Renal functional improvement after pediatric pyeloplasty in kidneys with split renal function less than 20%: a single institute experience

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Elsayed Salih ◽  
Ibrahim Abdelmaksoud ◽  
Mohamed Elfeky ◽  
Gamal Selmy ◽  
Hussein Galal ◽  
...  
Keyword(s):  
Renal Function ◽  
Statistical Significance ◽  
Functional Improvement ◽  
Renal Unit ◽  
Poor Renal Function ◽  
Split Renal Function ◽  
Renal Function Deterioration ◽  
Number Of Patients ◽  
The Mean

Abstract Background Pediatric pyeloplasty in ureteropelvic junction obstruction (UPJO) is indicated in renal impaired drainage or renal function deterioration. The improvement of renal function after pediatric pyeloplasty is still controversial in poorly functioning kidneys. Past studies on poorly functioning kidneys had a variable SRF specification, and these studies often had a limited number of patients so that they did not achieve statistical significance. The study aims to detect the renal functional improvement after pediatric Anderson-Hynes pyeloplasty (AHP) with split renal function (SRF) less than 20% (poor renal function). Results A retrospective study included 46 pediatric patients with unilateral UPJO who underwent open AHP with SRF < 20% on a renal isotope scan from August 2012 to October 2018. Success was defined based on either improvement in symptoms, improvement in drainage on postoperative renography, and/or improvement or stability in SRF on the renal scan done 6 months postoperatively and yearly thereafter. Deterioration of SRF by more than 5% was deemed to be deterioration. An increase in SRF of more than 5% was deemed to be an improvement. A total of 46 patients with a mean age of 32 months with poor renal function on isotope renogram (SRF < 20%) were included. All patients had an obstructive pattern on the preoperative radionuclide scans. The median preoperative SRF was 9.26%. The mean (range) follow-up was 30 months. The success rate was 91.3%. Three patients underwent redo pyeloplasty, whereas a secondary nephrectomy was necessary for one. The remaining (42) patients showed stability or improvement of SRF with no further symptoms. Renal scintigraphy at 6 and 12 months after surgery revealed significantly increased SRF compared to preoperative one. Conclusion Poorly functioning renal unit with SRF < 20% can show functional improvement and recoverability after pediatric pyeloplasty.

The effect of sickle cell anemia on the linear growth of Nigerian children

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Ugo Nnenna Chikani ◽  
Adaobi Bisi-Onyemaechi ◽  
Ijeoma Ohuche ◽  
Justus Onu ◽  
Shalewa Ugege ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Linear Growth ◽  
Clinical Care ◽  
Growth Failure ◽  
Age At Diagnosis ◽  
Height Velocity ◽  
Prospective Longitudinal Study ◽  
High Prevalence ◽  
The Mean ◽  
Prospective Longitudinal

Abstract Objectives Despite the high prevalence of children with sickle cell anaemia (SCA) in West Africa, there is paucity of data on the height velocity and prevalence of growth failure in SCA patients. With advances in clinical care of SCA patients, could there be a spatial and secular trend in the growth pattern of these children? Hence, the compelling needs to embark on this study. The objectives of the study were to determine the prevalence of growth failure among patients with SCA and its correlation with age, gender and age at diagnosis. Methods A Prospective longitudinal study of a cohort of sickle cell anaemic paediatric patients from Pediatrics SCA Clinic, University of Nigeria Teaching Hospital, Ituku Ozalla. Patients were enrolled over a period of two years using a non-parametric convenient sampling method. Their heights were measured at baseline, three months, six months and at 12 months intervals and subsequently plotted on a standard WHO growth chart. The height velocities at different monthly intervals were calculated and compared with the WHO standard normal linear growth rates) for children (used as control) to identify those with GF. (i.e. <10th percentile). The main outcome measures were the mean height velocities at different months' intervals calculated and compared using the repeated measurement analysis of variance (ANOVA) and the Wilcoxon signed test. Results A cohort of 316 children aged 1–18 years with SCA was evaluated with a male preponderance of 161 (57.4%). The mean age and age at diagnosis were 11.04 ± 5.56 and 4.2 ± 1.7 years, respectively. The prevalence of growth failure and short stature was 84.7%. The burden of GF was highest among post-pubertal participants (94.1%). The most important predictor of growth velocity deficit was age (R2=0.045, standard β coefficient = −0.22, t=−03.51, p=0.001). Conclusions The study demonstrated high prevalence of growth failure in children and adolescents with SCA which intensified with advancement in age and older age at diagnosis.

scholarly journals A Five Year Longitudinal Study of the Educational Environment in a Newly Integrated Medical Curriculum

2019 ◽  
Vol 19 (4) ◽  
pp. 335
Author(s):  
Syed I. Shehnaz
Keyword(s):  
Longitudinal Study ◽  
United Arab Emirates ◽  
Medical Curriculum ◽  
Educational Environment ◽  
Traditional Curriculum ◽  
Prospective Longitudinal Study ◽  
Significant Difference ◽  
The Mean ◽  
Prospective Longitudinal ◽  
Course Of Study

Objectives: The College of Medicine at Gulf Medical University (GMU), Ajman, United Arab Emirates, was subjected to a curricular reform, which shifted the institution from a traditional curriculum to a hybrid, studentcentred, integrated curriculum. There are no previous studies analysing the consequences of such a curricular modification on the educational environment (EE). Therefore, this study aimed to analyse the EE of a pioneer cohort of the innovative curriculum over their five-year course of study. Methods: This prospective longitudinal study was conducted between September 2009 and September 2013 at the College of Medicine. The Dundee Ready Education Environment Measure questionnaire was completed by a pioneer cohort of students at the start of each academic session for five consecutive years. The mean overall, subscale and individual statement scores were evaluated using the Kruskal-Wallis and Wilcoxon-rank sum tests. Results: A total of 178 responses were collected (response rate: 90.4%). The mean overall score throughout the five years of study was 130/200. A significant difference in the scores (P <0.05) was observed as the students proceeded through the course. The overall and subscale scores were significantly higher in the first and final years of study. Analysis of the statements recognised the medical knowledge of the teachers’ and students’ awareness of empathy and social interactions as persistent strengths of the college over the entire course of study. A curricular overload, a want for support systems for stressed students, students’ waning interest levels and assessment strategies emerged as areas that warranted further attention. Conclusion: The pioneer cohort of the new curriculum rated their EE as positive throughout their five years of study at GMU. An examination of individual statements revealed the programme’s strengths and areas for improvement for the institution.Keywords: Curriculum; Environment; Longitudinal Study; Medical Students; Perception; Undergraduate Medical Education; United Arab Emirates.

scholarly journals Botulinum Toxin Injection with Conjunctival Microincision for the Treatment of Acute Acquired Comitant Esotropia and Its Effectiveness

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Hongjia Xu ◽  
Weifeng Sun ◽  
Shuying Dai ◽  
Yanyan Cheng ◽  
Jing Zhao ◽  
...  
Keyword(s):  
Botulinum Toxin ◽  
Binocular Vision ◽  
Botulinum Toxin A ◽  
Botulinum Toxin Injection ◽  
Age At Onset ◽  
Brain Magnetic Resonance Imaging ◽  
Significant Difference ◽  
Angle Of Deviation ◽  
The Mean

Purpose. To report on an improved botulinum toxin injection with conjunctival microincision for beginners, and to determine the effectiveness of botulinum toxin A (BTXA) in the treatment of patients with acute acquired comitant esotropia (AACE). Methods. Medical records of 29 AACE patients were retrospectively analyzed. BTXA was injected into the unilateral or bilateral medial rectus muscle with conjunctival microincision without electromyographic guidance. Success was defined as total horizontal deviation ≤10 prism diopters (PD) and evidence of binocular vision. Results. Twenty-nine patients were included, of whom 22 were male and 7 were female. The mean age at onset was 14.2 ± 7.4 (range, 4–34) years. The mean time from onset of AACE to injection was 18.4 ± 20.3 (range, 1–96) weeks. All patients completed at least 6 months of follow-up, and the mean follow-up after BTXA injection was 12.3 ± 4.8 months (range, 7–24 months). Neurological evaluation and brain magnetic resonance imaging (MRI) were unremarkable in all patients. The mean spherical equivalent refraction was −1.22 ± 2.85D and −0.97 ± 2.80D in the right and left eyes, respectively. Mean preinjective esotropia was 38.4 ± 18.9 PD (range, +10–+80 PD) at near and 40.2 ± 17.7 PD (range, +20–+80 PD) at far distance. The mean angle of deviation at 6 months after injection was 0.6 ± 4.1 PD (range, −3–+15 PD) at near and 3.0 ± 5.9 PD (range, 0–+20 PD) at far distance. There was significant difference in the angle of deviation at near and far fixation between pre-BTXA and post-BTXA 6 months ( p < 0.001 , p < 0.001 , resp.). There was no significant difference in the angle of deviation at near and far fixation between post-BTXA 6 months and post-BTXA at final follow-up ( p  = 0.259 and 0.326, resp.). Mean stereoacuity improved from 338 to 88 arc seconds. During the follow-up period, 5 of 29 patients had recurrent esotropia. Two patients refused all further treatment, and the other 3 patients required incisional strabismus surgery. The success rates were 86.2% (25/29) at 6 months and 82.8% (24/29) at final follow-up. Conclusion. Conjunctival microincision injection of botulinum toxin is a practical and safe method for beginners to locate an extraocular muscle, which is as effective as the traditional methods. Botulinum toxin injection can be preferred as the first-line treatment for AACE patients with potential binocular vision.

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