scholarly journals AS-amyloidosis. Dual pathology or novel disease? A multimodality, multi-centre assessment across health and disease

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
K Patel ◽  
P Scully ◽  
C Nitsche ◽  
S Williams ◽  
T Tillin ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): British Heart Foundation onbehalf AS-Amyloidosis consortium Background The coexistence of severe aortic stenosis (lone AS) and transthyretin cardiac amyloidosis (lone amyloidosis) is common, but the resultant AS-amyloidosis phenotype is unclear. Purpose We characterised AS-amyloidosis, hypothesizing that the dual insult of AS-amyloidosis results is a severe phenotype. Methods We compared four cohorts with deep phenotyping: 81 older age controls, 359 lone AS, 36 AS-amyloidosis (Perugini grade 2 and 3) and 107 lone amyloidosis (Perugini grade 2 and 3). Results AS-amyloidosis was similar to lone AS with respect to left ventricular mass and LVEF (57 (45, 64)%). It was similar to lone amyloidosis with respect to lateral S" (0.04 (0.03, 0.06) m/s), NT-proBNP (4149 (1449, 6459) ng/L) and troponin T (56 (34, 100) ng/L). Whilst, prevalence of carpal tunnel syndrome (CTS) (17%) and diastolic function (E/A ratio 1.1 (0.8, 2.8)) were intermediate. Conclusion AS-amyloidosis is not a double insult from AS and amyloidosis, but a mixed phenotype with features similar to lone amyloidosis (cardiac biomarkers), lone AS (remodelling and LVEF) or intermediate (diastology and CTS). Characteristics across all 4 groups Variable Older age controls (n = 81) Lone AS (n = 359) AS-amyloidosis (n = 36) Lone amyloidosis (n = 107) P value Age (years) 82 (80, 84)*†‡ 85 (80, 88)§∞ 88 (85, 92)# 80 (75, 84) <0.005 Sex (% male) 69 *‡ 49 ∞ 61 # 94 <0.005 Carpal tunnel syndrome (%) 0 2 § 17 # 38 <0.005 Voltage/mass ratio 0.22 (0.14, 0.27)‡ 0.18 (0.13, 0.28)∞ 0.18 (0.09, 0.21)# 0.07 (0.05, 0.10) <0.005 NT-ProBNP (ng/L) 131 (66, 221)*†‡ 1629 (639, 3941)§∞ 4149 (1449, 6459) 2888 (1755, 5483) <0.005 hsTnT (ng/L) 12 (8, 17)*†‡ 24 (15, 40)§∞ 56 (34, 100) 62 (41, 82) <0.005 Inferolateral wall thickness (cm) 0.9 (0.8, 1.0)*†‡ 1.1 (0.9, 1.3)∞ 1.3 (1.1, 1.5)# 1.7 (1.6, 1.9) <0.005 Anteroseptal wall thickness (cm) 1.0 (0.9, 1.2)*†‡ 1.4 (1.2, 1.6)§∞ 1.5 (1.3, 1.8) 1.7 (1.6, 1.9) <0.005 Indexed LV mass (g/m2) 79 (66, 102)*†‡ 128 (99, 152)∞ 126 (116, 140)# 174 (159, 200) <0.005 LVEF (%) 59 (54, 63)‡ 59 (50, 65)∞ 57 (45, 64)# 39 (31, 48) <0.005 Lateral S" (m/s) 0.08 (0.07, 0.09)*†‡ 0.07 (0.05, 0.08)§∞ 0.05 (0.04, 0.07) 0.05 (0.04, 0.06) <0.005 Septal S" (m/s) 0.06 (0.06, 0.08)*†‡ 0.05 (0.04, 0.06)∞ 0.04 (0.03, 0.06) 0.04 (0.03, 0.05) <0.005 E/A 0.7 (0.6, 0.8)*†‡ 0.8 (0.7, 1.3)§∞ 1.1 (0.8, 2.8)# 2.4 (1.8, 3.3) <0.005 RV Wall thickness (cm) 0.4 (0.3, 0.4)*†‡ 0.4 (0.4, 0.6)∞ 0.6 (0.4, 0.7)# 0.8 (0.7, 1.0) <0.005 TAPSE (cm) 2.4 (2.0, 2.7)*†‡ 2.1 (1.6, 2.5)∞ 1.9 (1.5, 2.1)# 1.4 (1.2, 1.9) <0.005 Classical LFLG AS (%) 9 13 0.472 * p < 0.05, Old age control vs Lone AS † p < 0.05, Old age control vs AS-amyloidosis ‡ p < 0.05, Old age control vs Lone amyloidosis § p < 0.05, Lone AS vs AS-amyloidosis ∞ p < 0.05, Lone AS vs Lone amyloidosis # p < 0.05, AS-amyloidosis vs Lone amyloidosis Abstract Figure. AS-amyloidosis compared to other cohorts

Author(s):  
Ahmed Esmael ◽  
Mohamed Gomaa ◽  
Maha Hazem

Background: Compression neuropathies have previously been associated with one another. Migraine has not been considered a type of compression neuropathy but, some recent studies found that some types of migraines may be treated by targeted peripheral nerve decompression. So, the association between migraines and carpal tunnel syndrome (CTS), {the most common compression neuropathy} may exists. Objective: The aims of this study are to found whether there is a relationship between carpal tunnel syndrome and migraine, and if so to determine the factors causing this relationship. Methods: The present study is a cross-sectional case control study of 120 patients with CTS in addition to age and sex matched 120 healthy controls. It was conducted at Mansoura University Hospitals, Egypt in the period from July, 2017 through June, 2018. All subjects underwent neurological examination, nerve conduction study of median nerve bilaterally, and evaluation according to headache by the International Headache Society, 2016 criteria. Also, the clinical severity of CTS was assessed and calculation of body mass index (BMI) was done. Evaluation of patients by Boston questionnaire form (BQF) {composed of two parts, Symptom Severity Scale (SSS) and the Functional Status Scale (FSS)}. Lastly, assessment of patients by Beck Depression Inventory (BDI). Results: Patients had significantly higher rates of migraine headache (28.3 %) either alone (17.5 %) or combined with tension headache (10.8 %) when compared to controls. No statistically significant differences were found between the studied groups regarding the frequency of isolated tension headache. However, the total rate of cases with tension headache (n=43) including those in association with migraine is significantly higher in patients when compared to controls. Patients had significantly higher rates of various grades of symptoms severity. All patients but 17 are functionally affected while none of the controls group is affected. Patients had significantly higher rates of borderline and moderate depression when compared to controls. Univariate logistic regression analysis for predictors of migraine headache in the studied patients shows that, older age (P value = 0.0001), female sex (P value = 0.003), being manual worker (P value = 0.005), obesity class 11 (P value = 0.023), very severe functional severity scale (P value = 0.001), borderline and moderate depression (P value = 0.008 and 0.002 respectively) were significant predictors of migraine. Conclusion: There is a significant association between CTS and migraine headache. This association suggests the possibility of a common risk factors for development of migraine headache include older age, female gender, obesity, low functional and high symptoms scores and depressive symptoms.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A.F Dias De Frias ◽  
P Rodrigues ◽  
M Trepa ◽  
M Fontes-Oliveira ◽  
R Costa ◽  
...  

Abstract Introduction Pacemakers are frequently needed due to a high prevalence of conduction disease in mutated ATTR amyloidosis (mATTR). We aimed to identify the variables associated with the need of pacemaker implantation in this population. Methods We retrospectively studied 255 patients with suspicion of heart involvement of mATTR observed at our cardiology clinic during the last year. Clinical and outcome data were retrieved by chart review. We have defined the need for pacemaker implantation as: 1) the formal guidelines indications or 2) Ventricular pacing >10% in patients who had prophylactic pacemaker implantation prior to liver transplantation (LT). This way, we have defined 3 different groups: group 1: patients with no evidence of conduction disease; group 2: patients with conduction disease, but no formal indication for pacemaker implantation; and group 3: patients with formal indication for pacemaker implantation or ventricular pacing >10% in patients who had prophylactic pacemaker implantation prior to hepatic transplantation. Results We included 255 patients (50±14 years, 53% male, 52.5% treated with tafamidis and 27% had prior LT, and 10% with atrial fibrillation), 43.3% with no evidence of conduction disease, 32.3% with conduction disease, but no formal indication for pacemaker implantation and 24.4% with formal indication for pacemaker implantation. Patients with formal indication for pacemaker implantation were older, with longer duration of neurologic manifestations, with higher concentration of both Troponin T and NT-proBNP and with higher number of organs affected. In multivariate analysis, longer duration of neurologic manifestations (OR 1.090 – 95% IC: 1.036–1.145, p-value 0.001), Left ventricular (LV) maximal wall thickness (OR 1.230 – 95% CI: 1.070–1.414, p-value 0.004), neurologic staging (OR 3.420 – 95% CI: 1.443–8.104, p-value 0.005) and higher number of organs affected (OR 1.719 – 95% CI: 1.218–2.424, p-value 0.002) all showed to be independent predictors of the need for pacemaker implantation, in contrast to LV ejection fraction and serum concentration of Troponin T and NT-proBNP. We've also found a statistical significant association between conduction disease and ophthalmic manifestations. Conclusions Our findings suggest that the need for pacemaker implantation in patients with mATTR is closer linked to the duration, severity and affected number of organs than to cardiac biomarkers or echocardiographic findings. Funding Acknowledgement Type of funding source: None


2015 ◽  
Vol 123 (5) ◽  
pp. 1230-1237 ◽  
Author(s):  
Daniele Vanni ◽  
Francesco Saverio Sirabella ◽  
Renato Galzio ◽  
Vincenzo Salini ◽  
Vincenzo Magliani

OBJECT The purpose of this study was to assess the effectiveness and safety of an alternative minimally invasive technique for the treatment of carpal tunnel syndrome (CTS). METHODS This was designed as a prospective, randomized, open-label, blinded end point evaluation (PROBE) study. The active comparison was double tunnels technique (DTT) (Group A, 110 patients) versus standard open decompression of the median nerve (control [Group B], 110 patients). Patient recruitment started in January 2011. The primary outcomes were the functional Boston Carpal Tunnel Syndrome Questionnaire (BCTSQ) scores and visual analog scale (VAS) scores for pain (pVAS) at Weeks 2 and 4, and at Months 3, 6, and 12. The secondary outcome was the aesthetics (aVAS) score at Weeks 2 and 4, and at Months 3, 6, and 12. RESULTS The Student t-test and ANOVA were used, and the results were considered statistically significant if the p value was ≤ 0.05 for continuous variables. The DTT is a tissue-sparing approach that allows the surgeon to limit the length of the incision (0.6 ± 0.05 cm) and to respect the palmar fascia and the subcutaneous tissue. Recovery from wrist pain, night pain, numbness, stiffness, and weakness was achieved more effectively and quickly compared with the standard approach. Better BCTSQ, pVAS, and aVAS scores were observed in Group A. CONCLUSIONS The DTT is a safe and effective approach for the treatment of CTS. This technique entails faster recovery times, better aesthetic outcomes, and lower risks of complications.


2021 ◽  
Vol 6 (4) ◽  
pp. 89-96
Author(s):  
Jehan Zeb ◽  
Muhammad Ullah ◽  
Muhammad Shoaib ◽  
Syed Shah ◽  
Walayat Shah ◽  
...  

Purpose: To determine the outcome of microscopic carpal tunnel release in patients with carpal tunnel syndrome who failed to respond to conservative treatment. Methodology: This descriptive case series was carried at Department of Neurosurgery, D.H.Q Hosptial Charsadda over 1 year from Jan 2019 to Dec 2020, indicate the sampling method used to select the study participants involving 94 patients; both men and women with ages in the range 30-70 years diagnosed of carpal tunnel syndrome who failed to respond to conservative treatment and were planned for surgical release. Microscopic CTS release was performed and outcomes were assessed in terms of improvement in VAS score for wrist pain, symptom severity score and function status scale 3 and 6 months after the surgery. Recurrence of symptoms was also noted. A written informed consent was obtained from every patient. Indicate the method of data collection and data analysis Findings: The mean age of the patients with carpal tunnel syndrome was 41.6±7.9 years. There was slight female predominance with male to female ratio of 1:2.1. History of diabetes was recorded in 29 (31.0%) patients while 34 (37.0%) patients were obese. Right hand was more frequently involved (53.0%) than the left hand (47.0%). The mean VAS score for wrist pain reduced from 7.9±1.2 at baseline to 1.8±0.7 3 months after the surgery (p- value<0.001). Similar improvements were also noted in symptom severity score (3.8±0.8 to 1.6±0.8; p- value<0.001) and function status scale (2.7±0.8 to 1.5±0.8; p-value<0.001) at the end of 3 months after the surgery. Recurrence was not observed in any patient at the end of 6 months follow-up. Recommendation: Microscopic carpal tunnel release was found to relieve patient’s symptoms and improve wrist function yet with minimal scarring and without recurrence which advocates its preferred use in future practice provided necessary surgical skills and hardware are available.


1970 ◽  
Vol 1 (1) ◽  
pp. 63-69
Author(s):  
Evi Yuliani ◽  
Widjajalaksmi Kusumaningsih ◽  
Vitriana ◽  
Hadyana Sukandar

Objectives : To compare the effect of both wrist splint and wrist-­metacarpophalangeal splint at night using Symptom Severity Scale (SSS) and Functional Status Scale (FSS) and to know the validity andreliability of Indonesian version of SSS dan FSS.Methods : This study enrolled 19 subjects in each of the two groups that were given neutral wrist splint and exercise in one group (group A) and neutral wrist-­metacarpophalangeal splint and exercise in theother group (group B).Results : Indonesian version of SSS and FSS have been proven valid with rs lowest = 0.360 and highest 0.810(>0.3) and relable with α SSS=0.762 dan FSS=0.781 (>0.7). There were significant improvement score of SSS and FSS in each group with p value <0.001. There were greater functional improvement in group treated with wrist-­metacarpophalangeal splint (group B) with p value = 0.036 (p<0.05) Conclusions: There was improvement of SSS and FSS in both groups of study with greater improvement of in wrist-­hand splint group.Keywords: Carpal Tunnel Syndrome, wrist splint, wrist-­hand splint,,nerve and tendon gliding, SSS, FSS.


2018 ◽  
Vol 4 (2) ◽  
pp. 101-107
Author(s):  
Badrunnesa Ahmed ◽  
Md Shafiqul Alam ◽  
Md Abdul Halim ◽  
AKM Salek ◽  
Md Jahidul Islam ◽  
...  

Background: Carpal Tunnel Syndrome (CTS) is the most common upper extremity compression neuropathy which is a common entrapment neuropathy seen in pregnant women.Objective: The purpose of the present study was to assess presentation and severity of various symptoms among women with CTS in pregnancy and to see the effect of conservative management protocols in alleviation of symptoms of the disease and restoration of functional capability of the affected hands.Methodology: This interventional study was included 36 pregnant women attended in the Outpatient Department of Physical Medicine and Rehabilitation Specialist in the Hi-Tech Multicare Hospital, Dhaka during the period from January 2015 to January 2016 for a period of one year and one month. Detail history was taken and examined physically by a specialist in physical medicine to ascertain the clinical diagnosis. Out of 48 referred cases, 42 pregnant women were identified clinically as CTS cases based on symptoms and signs. However, 2 cases were not enrolled because of severe form of pain and referred to other hospital for admission and 6 patient dropped out. Therefore, 36 cases with CTS symptoms included in this study. All the patients were given conservative treatment (Wrist splint, Exercise and activity modification) for 8 weeks. All patients were followed up at 4 weeks interval. Treatment outcome were measured by Boston carpal tunnel questionnaire (BCTQ).Result: The mean age of the patients is 30±3.5 years. 26 (78%) patients mild to moderate type of CTS symptoms showed improvement in conservative management. Before treatment the mean symptoms severity scale (SSS) of BCTQ) was 2.32±0.4 and for functional status scale (FSS) was it was 1.99±0.43. After treatment for SSS 1.86±0.47 and for FSS 1.53±0.42. And in SSS the p value<.05 and in FSS p value is <.05 which was statistically significant.Conclusion: In conclusion conservative management protocols are sufficient enough in alleviation of symptoms of the disease and is effective for the restoration of functional capability of the affected hand or hands.Journal of National Institute of Neurosciences Bangladesh, 2018;4(2): 101-107


2018 ◽  
Vol 3 (2) ◽  
pp. 37-44
Author(s):  
Muthoharoh ◽  
Sarinah Basri K ◽  
Tating Nuraeni

CTS merupakan gangguan umum yang berhubungan dengan pekerjaan, disebabkan oleh gerakan berulang dan posisi yang menetap pada jangka waktu lama. Beberapa faktor diketahui menjadi risiko terjadinya CTS, seperti gerakan berulang dengan kekuatan, tekanan pada otot, postur kerja yang tidak ergonomik dan lain-lain. Penelitian ini menggunakan Cross Sectional dengan jumlah sampel 40 karyawan di dua SPBE bagian Filling Hall area. Fisher Exact Test digunakan untuk analisis data uji statistik. Variabel yang diteliti adalah masa kerja, gerakan repetitive dan postur kerja. Dalam pengumpulan data peneliti menggunakan kuesioner, lembar observasi RULA dan test pemeriksaan fisik. Hasil uji statistik menggunakan Fisher Exact Test untuk masa kerja dan postur kerja didapatkan nilai P-Value = 0,029 dan 0,041. Karena nilai P -Value < 0,05 sehingga Ho ditolak, artinya ada hubungan antara masa kerja dan postur kerja dengan kejadian CTS . sedangkan untuk gerakan repetitive hasil uji statistik menggunakan Fisher Exact Test didapatkan nilai P-Value = 0,464. Karena nilai P-Value >0,05 sehingga Ho diterima, artinya ada hubungan antara gerakan repetitive dengan kejadian CTS.


2003 ◽  
Vol 61 (2A) ◽  
pp. 194-198 ◽  
Author(s):  
Joao Aris Kouyoumdjian ◽  
Maria P.A. Morita ◽  
Amalia F.P. Molina ◽  
Dirce M.T. Zanetta ◽  
Adriana K. Sato ◽  
...  

This study was done to evaluate the long-term patient's satisfaction after carpal tunnel syndrome (CTS) electrodiagnostic done between 1989 and 1994 (5 to 10 years follow-up). Mail contact was made to 528 consecutive cases with a questionnaire to be filled; 165 patients responded after 19 exclusions. CTS severity was graded from 0 (incipient) to 4 (severe) after a combination of median sensory distal latency, sensory median-radial latency difference and amplitude of the median compound muscle action potential. Current symptoms ("cure", improved, unchanged or worsed) and the therapy utilized, either surgical or conservative, were analyzed to the initial CTS severity, age and duration of symptomatology. Surgical release was done in 114 cases (69%). Patient's satisfaction after surgical and non-surgical were respectively, 77.6% and 16% ("cure"), 13.6% and 52% (much improved), 5.4% and 9.3% (little improved), 2.7% and 16% (unchanged), 0.7% and 6.7% (worsed). The frequency of "cure" versus unchanged/worsed or "cure"/much improved versus unchanged/worsed was highly significative (Fisher, P-value < 0.001) and was not influenced by the CTS electrophysiological severity. There was no relationship between the outcome after surgery and duration of symptomatology, age or CTS severity. Conservative benefice was more prevalent in those with shorter symptomatology and older age; the majority of conservative failure cases had mild initial CTS. We concluded the excellent surgical benefice described by patients and the absence of any predictive factors based on CTS severity, age or duration of symptomatology for outcome.


Author(s):  
Raja Kollu ◽  
Sindhu Vasireddy ◽  
Sreekanta Swamy ◽  
Nataraju Boraiah ◽  
H Ramprakash ◽  
...  

Introduction: Carpal Tunnel Syndrome (CTS) is the entrapment neuropathy which is diagnosed based on the clinical history, examinations and the electrophysiological findings. The Cross- sectional Area (CSA) measurement of the median nerve has emerged as an alternative to Nerve Conduction Studies (NCS) for diagnosis of CTS. This study was done to correlate NCS and Ultrasonography (USG) in clinically diagnosed CTS patients. Aim: To evaluate the diagnostic value of Cross-sectional Area (CSA) of median nerve at carpal tunnel inlet in patients with clinically and NCS confirmed Carpal Tunnel Syndrome (CTS) and to assess severity of the syndrome by NCS and its correlation with USG results. Materials and Methods: This was a hospital based, case-control study done on a total of 109 patients of CTS and analysed during the period from June 2017 to June 2019. Total 203 hands of the patients with abnormal NCS formed case group while 101 hands from healthy volunteers constituted the control group. All the patients underwent neurological evaluation by Boston Carpal Tunnel Questionnaire (BCTQ) and were divided into mild, moderate and severe according to the score. An electromyography machine was used to perform electrophysiological studies of both the limbs in all subjects. CTS was diagnosed electro-diagnostically based on the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) guidelines and were classified as mild (Grades 1 and 2), moderate (Grades 3 and 4), and severe (Grades 5 and 6) grades using Bland’s electrophysiological grading scale. USG was performed for all the subjects and all the data of various investigations was analysed using Statistical Package for Social Sciences (SPSS) version 22.0 software. Chi-square test and Mann Whitney U-test were used as test of significance for qualitative data. Results: The mean age of subjects was 44.38±9.561 years. Strongly significant association was observed in BCTQ symptom, functional and total scores with NCS severity grading (p-value <0.001). Moderately significant association was found between BCTQ symptom and total scores with USG severity grading (p-value<0.02). Tunnel grade and NCS grade were found significantly correlated (p-value <0.001). The mean CSA cut-off value of 8.5 mm² at the inlet of carpal tunnel had a good sensitivity 86.21%, specificity 83.17%, Positive Predictive Value (PPV) 91.1% and Negative Predictive Value (NPV) 75%. Conclusion: The diagnostic accuracy of USG assessment and NCS was found to be correlated comparably and complement each other in all grades of CTS. USG, can be considered a preferable screening tool by the patients of CTS due to its painless nature and easy accessibility. It requires minimal time and many a times detects those structural abnormalities which have great therapeutic implications. In mild CTS cases, USG should always be combined with NCS for proper diagnosis as USG might give negative result.


2021 ◽  
Vol 19 (7) ◽  
pp. 149-155
Author(s):  
Shatha Mohammed Abdulmunem ◽  
Hanan L. Al-Omary

Carpal tunnel syndrome is a neurological disease that presented with paresthesias, pain, and numbness in the hand's median nerve compression. Vitamin D was assumed to affect both electrophysiological &clinical gradings, the study aims to assess the correlation between the deficiency of vitamin D and both electrophysiological and clinical gradings. This study was conducted in Ghazi Alhariri Hospital during the period from the first of November/2020 to the twenty-eighth of February/2021, fifty five individuals were referred to as Carpal tunnel syndrome patients, and compared to (55) control individuals, blood samples were withdrawn from the patients (3ml), centrifuged and kept in the freezer (-20°C) until the time of analysis of vitamin D3, Sensory and motor nerve conduction studies of both median and ulnar nerve were done bilaterally. patients were classified electrophysiologically and clinically into two subgroups (mild to moderate) and (severe) groups. The result showed that the differences are not significant in the gender, BMI and vitamin D of the patients versus the control group (p>0.05), the difference of the electrophysiological parameters was not significant between patients with low vitamin D versus those with normal vitamin D (p value>0.05), there was a significant association between the electrophysiological and clinical grading in addition to a significant association between vitamin D level and the clinical grading. Vitamin D deficiency does not affect the electrophysiological parameters while the clinical grading becomes worse with the decrease in its level. The electrophysiological grading is associated with clinical grading.


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