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 ◽  
...  

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.

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Cláudia R. C. Galvão ◽  
Priscilla M. A. Cavalcante ◽  
Ricardo Olinda ◽  
Zodja Graciani ◽  
Mayana Zatz ◽  
...  

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.


2019 ◽  
Author(s):  
Cláudia R. C. Galvão ◽  
Priscilla M. A. Cavalcante ◽  
Ricardo Olinda ◽  
Zodja Graciani ◽  
Mayana Zatz ◽  
...  

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.


2016 ◽  
Vol 21 (03) ◽  
pp. 339-344 ◽  
Author(s):  
Chul-Hyun Cho ◽  
Geon-Myeong Oh

Background: The objective of this study was to determine prognostic factors affecting the clinical outcome of septic arthritis of the shoulder. Methods: We retrospectively reviewed 34 shoulders from 32 patients, two of which had bilateral involvement. Arthroscopic (22 shoulders) or open surgery (12 shoulders) was performed by a single surgeon. The mean follow-up period was 32.4 ± 17.0 months. Clinical outcomes according to the University of California at Los Angeles (UCLA) score, American Shoulder and Elbow Surgeons (ASES) score, and Subjective Shoulder Value (SSV) were assessed at the final follow-up period. Various factors were included for statistical analysis. Results: The mean UCLA, ASES scores, and SSV were 28.9 ± 7.2, 81.3 ± 21.0, 79.7 ± 2.5%, respectively. Positive culture was observed in only 13 shoulders (38.2%) and the most common organism was Staphylococcus aureus (seven shoulders). Five shoulders (14.7%) required two or three operations. Age and comorbidity were negatively correlated with the UCLA, ASES score, and/or SSV (p < 0.05). There was no correlation between clinical outcome and various parameters, including gender, location of lesion, history of previous steroid injection, interval between onset of symptoms and surgical intervention, bacterial organisms, operative method, and presence of rotator cuff tear and reoperation (p > 0.05). Conclusions: Both arthroscopic and open surgery for septic shoulders showed satisfactory clinical outcomes. Old age and comorbidity were poor prognostic factors of clinical outcomes after treatment.


2016 ◽  
Vol 124 (2) ◽  
pp. 288-295 ◽  
Author(s):  
Manasi Ramachandran ◽  
Rohini Retarekar ◽  
Madhavan L. Raghavan ◽  
Benjamin Berkowitz ◽  
Benjamin Dickerhoff ◽  
...  

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.


Author(s):  
Gopal Krishna ◽  
Varun Aggarwal ◽  
Ishwar Singh

Abstract Introduction Traumatic brain injury (TBI) affects the coagulation pathway in a distinct way than does extracranial trauma. The extent of coagulation abnormalities varies from bleeding diathesis to disseminated thrombosis. Design Prospective study. Methods The study included 50 patients of isolated TBI with cohorts of moderate (MHI) and severe head injury (SHI). Coagulopathy was graded according to the values of parameters in single laboratory. The incidence of coagulopathy according to the severity of TBI and correlation with disseminated intravascular coagulation (DIC) score, platelets, prothrombin time (PT), activated partial thromboplastin time (APTT), D-dimer, and fibrinogen was observed. The comparison was also made between expired and discharged patients within each group. It also compared coagulation derailments with clinical presentation (Glasgow Coma Scale [GCS]) and outcome (Glasgow Outcome Scale [GOS]). Results Road traffic accident was the primary (72%) mode of injury. Fifty-two percent had MHI and rest had SHI. Eighty-four percent of cases were managed conservatively. The mean GCS was 12.23 and 5.75 in MHI and SHI, respectively. Sixty-two percent of MHI and 96% of the patients with SHI had coagulation abnormalities. On statistical analysis, DIC score (p < 0.001) strongly correlated with the severity of head injury and GOS. PT and APTT were also significantly associated with the severity of TBI. In patients with moderate TBI, D-dimer and platelet counts showed association with clinical outcome. Fibrinogen levels did not show any statistical significance. The mean platelet counts remained normal in both the groups of TBI. The mean GOS was 1.54 and 4.62 in SHI and MHI, respectively. Conclusion Coagulopathy is common in isolated TBI. The basic laboratory parameters are reliable predictors of coagulation abnormalities in TBI. Coagulopathy is directly associated with the severity of TBI, GCS, and poor outcome.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 807.3-807
Author(s):  
I. Moriyama

Background:No widely accepted view or criteria currently exist concerning whether or not patellar replacement (resurfacing) should accompany total knee arthroplasty for osteoarthritis of the knee.1)2)3)Objectives:We recently devised our own criteria for application of patellar replacement and performed selective patellar replacement in accordance with this set of criteria. The clinical outcome was analyzed.Methods:The study involved 1150 knees on which total knee arthroplasty was performed between 2005 and 2019 because of osteoarthritis of the knee. The mean age at operation was 73, and the mean postoperative follow-up period was 91 months. Our criteria for application of patellar replacement are given below. Criterion A pertains to evaluation of preoperative clinical symptoms related to the patellofemoral joint: (a) interview regarding presence/absence of pain around the patella, (b) cracking or pain heard or felt when standing up from a low chair, (c) pain when going upstairs/downstairs. Because it is difficult for individual patients to identify the origin of pain (patellofemoral joint or femorotibial joint), the examiner advised each patient about the location of the patellofemoral joint when checking for these symptoms. Criterion B pertains to intense narrowing or disappearance of the patellofemoral joint space on preoperative X-ray of the knee. Criterion C pertains to the intraoperatively assessed extent of patellar cartilage degeneration corresponding to class 4 of the Outerbridge classification. Patellar replacement was applied to cases satisfying at least one of these sets of criteria (A-a,-b,-c, B and C). Postoperatively, pain of the patellofemoral joint was evaluated again at the time of the last observation, using Criterion A-a,-b,-c.Results:Patellar replacement was applied to 110 knees in accordance with the criteria mentioned above. There were 82 knees satisfying at least one of the Criterion sets A-a,-b,-c, 39 knees satisfying Criterion B and 70 knees satisfying Criterion C. (Some knees satisfied 2 or 3 of Criteria A, B and C).When the pain originating from patellofemoral joint (Criterion A) was clinically assessed at the time of last observation, pain was not seen in any knee of the replacement group and the non-replacement group.Conclusion:Whether or not patellar replacement is needed should be determined on the basis of the symptoms or findings related to the patellofemoral joint, and we see no necessity of patellar replacement in cases free of such symptoms/findings. When surgery was performed in accordance with the criteria on patellar replacement as devised by us, the clinical outcome of the operated patellofemoral joint was favorable, although the follow-up period was not long. Although further follow-up is needed, the results obtained indicate that selective patellar replacement yields favorable outcome if applied to cases judged indicated with appropriate criteria.References:[1]The Effect of Surgeon Preference for Selective Patellar Resurfacing on Revision Risk in Total Knee Replacement: An Instrumental Variable Analysis of 136,116 Procedures from the Australian Orthopaedic Association National Joint Replacement Registry.Vertullo CJ, Graves SE, Cuthbert AR, Lewis PL J Bone Joint Surg Am. 2019 Jul 17;101(14):1261-1270[2]Resurfaced versus Non-Resurfaced Patella in Total Knee Arthroplasty.Allen W1, Eichinger J, Friedman R. Indian J Orthop. 2018 Jul-Aug;52(4):393-398.[3]Is Selectively Not Resurfacing the Patella an Acceptable Practice in Primary Total Knee Arthroplasty?Maradit-Kremers H, Haque OJ, Kremers WK, Berry DJ, Lewallen DG, Trousdale RT, Sierra RJ. J Arthroplasty. 2017 Apr;32(4):1143-1147.Disclosure of Interests:None declared


1997 ◽  
Vol 171 (1) ◽  
pp. 47-52 ◽  
Author(s):  
Sergio E. Starkstein ◽  
Erán Chemerinski ◽  
Liliana Sabe ◽  
Gabriela Kuzis ◽  
Gustavo Petracca ◽  
...  

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.


2013 ◽  
Vol 19 (1) ◽  
pp. 90-94 ◽  
Author(s):  
Hironobu Sakaura ◽  
Tomoya Yamashita ◽  
Toshitada Miwa ◽  
Kenji Ohzono ◽  
Tetsuo Ohwada

Object A systematic review concerning surgical management of lumbar degenerative spondylolisthesis (DS) showed that a satisfactory clinical outcome was significantly more likely with adjunctive spinal fusion than with decompression alone. However, the role of adjunctive fusion and the optimal type of fusion remain controversial. Therefore, operative management for multilevel DS raises more complicated issues. The purpose of this retrospective study was to elucidate clinical and radiological outcomes after 2-level PLIF for 2-level DS with the least bias in determination of operative procedure. Methods Since 2005, all patients surgically treated for lumbar DS at the authors' hospital have been treated using posterior lumbar interbody fusion (PLIF) with pedicle screws, irrespective of severity of slippage, patient age, or bone quality. The authors conducted a retrospective review of 20 consecutive cases involving patients who underwent 2-level PLIF for 2-level DS and had been followed up for 2 years or longer (2-level PLIF group). They also analyzed data from 92 consecutive cases involving patients who underwent single-level PLIF for single-level DS during the same time period and had been followed for at least 2 years (1-level PLIF group). This second group served as a control. Clinical status was assessed using the Japanese Orthopaedic Association (JOA) score. Fusion status and sagittal alignment of the lumbar spine were assessed by comparing serial plain radiographs. Surgery-related complications and the need for additional surgery were evaluated. Results The mean JOA score improved significantly from 12.8 points before surgery to 20.4 points at the latest follow-up in the 2-level PLIF group (mean recovery rate 51.8%), and from 14.2 points preoperatively to 22.5 points at the latest follow-up in the single-level PLIF group (mean recovery rate 55.3%). At the final follow-up, 95.0% of patients in the 2-level PLIF group and 96.7% of those in the 1-level PLIF group had achieved solid spinal fusion, and the mean sagittal alignment of the lumbar spine was more lordotic than before surgery in both groups. Early surgery-related complications, including transient neurological complications, occurred in 6 patients in the 2-level PLIF group (30.0%) and 11 patients in the 1-level PLIF group (12.0%). Symptomatic adjacent-segment disease was found in 4 patients in the 2-level PLIF group (20.0%) and 10 patients in the 1-level PLIF group (10.9%). Conclusions The clinical outcome of 2-level PLIF for 2-level lumbar DS was satisfactory, although surgery-related complications including symptomatic adjacent-segment disease were not negligible.


2017 ◽  
Vol 35 (5) ◽  
pp. 506-514 ◽  
Author(s):  
Michelle C. Janelsins ◽  
Charles E. Heckler ◽  
Luke J. Peppone ◽  
Charles Kamen ◽  
Karen M. Mustian ◽  
...  

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.


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