scholarly journals Dose Adjustment of Subcutaneous IgG in Chronic Inflammatory Demyelinating Polyneuropathy

2020 ◽  
Vol 12 (1) ◽  
pp. 73-77
Author(s):  
Alanood Alsolaihim ◽  
Steven K. Baker
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Chronic Inflammatory Demyelinating Polyradiculoneuropathy ◽  
Fine Tuning ◽  
Dramatic Improvement ◽  
Hand Grip ◽  
Immune Mediated ◽  
Conduction Velocities ◽  
Ongoing Surveillance ◽  
Scig Therapy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy that is characterized by a slowly progressive sensory and motor involvement lasting at least 2 months. We present a CIDP patient on subcutaneous Ig (SCIg). Upon fine-tuning his dose from 24 to 28 g/week, this showed a dramatic improvement in both hand grip (13–25%) and dorsiflexion (73–278%). Follow-up nerve conduction studies also demonstrated significant improvements in latencies, motor amplitudes, and conduction velocities. Ongoing surveillance of CIDP patients receiving SCIg therapy is therefore necessary to ensure therapeutic optimization.

scholarly journals Pathophysiology of the Different Clinical Phenotypes of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

2021 ◽  
Vol 23 (1) ◽  
pp. 179
Author(s):  
Edyta Dziadkowiak ◽  
Marta Waliszewska-Prosół ◽  
Marta Nowakowska-Kotas ◽  
Sławomir Budrewicz ◽  
Zofia Koszewicz ◽  
...  
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Relevant Literature ◽  
Chronic Inflammatory Demyelinating Polyradiculoneuropathy ◽  
Rapid Progress ◽  
Ongoing Research ◽  
Clinical Phenotypes ◽  
Current State ◽  
Immune Mediated ◽  
Key Points ◽  
Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common form of autoimmune polyneuropathy. It is a chronic disease and may be monophasic, progressive or recurrent with exacerbations and incomplete remissions, causing accumulating disability. In recent years, there has been rapid progress in understanding the background of CIDP, which allowed us to distinguish specific phenotypes of this disease. This in turn allowed us to better understand the mechanism of response or non-response to various forms of therapy. On the basis of a review of the relevant literature, the authors present the current state of knowledge concerning the pathophysiology of the different clinical phenotypes of CIDP as well as ongoing research in this field, with reference to key points of immune-mediated processes involved in the background of CIDP.

Inflammatory Myositis Secondary to Anti-Retroviral Therapy in a Child; Case Report and Review of the Literature

2021 ◽  
pp. 1-7
Author(s):  
Marie Monaghan ◽  
Charlotte Loh ◽  
Stephen Jones ◽  
Agyepong Oware ◽  
Kathryn Urankar ◽  
...  
Keyword(s):  
Drug Regimen ◽  
Dramatic Improvement ◽  
Strand Transfer ◽  
Inflammatory Myositis ◽  
Once Daily ◽  
Immune Mediated ◽  
Show Evidence ◽  
Transfer Inhibitor ◽  
Integrase Strand Transfer Inhibitor

Here, we describe a five year old girl with congenital HIV who had a six-week onset of rapidly deteriorating mobility and progressive proximal muscle weakness, associated with a raised Creatine Kinase (CK) level of 4330 U/L [25–200 U/L], subsequently diagnosed with an inflammatory myositis. Potential causes were investigated by paediatric neurology and immunology teams. Her viral load had been undetectable over the preceding two years, excluding a primary HIV myositis. While MRI scanning did not show evidence of definite myositis, a muscle biopsy showed evidence of an inflammatory process, comprising a moderate endomysial, perimysial and perivascular mononuclear (CD8 + T cell) infiltrate with increased MHC expression. No particular features of dermatomyositis or immune-mediated necrotising myopathy were identified and there were no features of an inclusion body myositis. Given the absence of active HIV infection, the role of anti-retroviral medications was considered. She had had a recent switch in medication, from twice daily Raltegravir (an Integrase Strand Transfer Inhibitor, INSTI) to once daily Dolutegravir (an INSTI) while continuing on an established daily protocol of Abacavir and Lamivudine (Nucleoside Reverse Transcriptase Inhibitors). Changing the Dolutegravir back to Raltegravir, in combination with continuing Lamivudine and Abacavir for two months made no difference to her weakness or CK levels. Moreover, this drug regimen had been well-tolerated over the preceding 19 month period. Changing the anti-retroviral regime completely to a single drug class (Protease Inhibitors) of Ritonavir and Darunavir, resulted in a dramatic improvement in her symptomatology. Within ten days she regained the ability to stand and walk, with a reduction in her CK from 1700 U/L at time of switch to 403 U/L [25–200]. This case highlights the potential risk of developing inflammatory myositis from anti-retrovirals even 19 months into treatment.

Expectations, Prefitting Counseling, and Hearing Aid Outcome

2009 ◽  
Vol 20 (05) ◽  
pp. 320-334 ◽  
Author(s):  
Gabrielle H. Saunders ◽  
M Samantha Lewis ◽  
Anna Forsline
Keyword(s):  
Hearing Aids ◽  
Hearing Aid ◽  
Well Being ◽  
Fine Tuning ◽  
Initial Contact ◽  
High Expectations ◽  
Digital Hearing Aids ◽  
The Impact ◽  
Unrealistic Expectations

Background: Data suggest that having high expectations about hearing aids results in better overall outcome. However, some have postulated that excessively high expectations will result in disappointment and thus poor outcome. It has been suggested that counseling patients with unrealistic expectations about hearing aids prior to fitting may be beneficial. Data, however, are mixed as to the effectiveness of such counseling, in terms of both changes in expectations and final outcome. Purpose: The primary purpose of this study was to determine whether supplementing prefitting counseling with demonstration of real-world listening can (1) alter expectations of new hearing aid users and (2) increase satisfaction over verbal-only counseling. Secondary goals of the study were to examine (1) the relationship between prefitting expectations and postfitting outcome, and (2) the effect of hearing aid fine-tuning on hearing aid outcome. Research Design: Sixty new hearing aid users were fitted binaurally with Beltone Oria behind-the-ear digital hearing aids. Forty participants received prefitting counseling and demonstration of listening situations with the Beltone AVE™ (Audio Verification Environment) system; 20 received prefitting counseling without a demonstration of listening situations. Hearing aid expectations were measured at initial contact and following prefitting counseling. Reported hearing aid outcome was measured after eight to ten weeks of hearing aid use. Study Sample: Sixty new hearing aid users aged between 55 and 81 years with symmetrical sensorineural hearing loss. Intervention: Participants were randomly assigned to one of three experimental groups, between which the prefitting counseling and follow-up differed: Group 1 received prefitting counseling in combination with demonstration of listening situations. Additionally, if the participant had complaints about sound quality at the follow-up visit, the hearing aids were fine-tuned using the Beltone AVE system. Group 2 received prefitting counseling in combination with demonstration of listening situations with the Beltone AVE system, but no fine-tuning was provided at follow-up. Group 3 received prefitting hearing aid counseling that did not include demonstration of listening, and the hearing aids were not fine-tuned at the follow-up appointment. Results: The results showed that prefitting hearing aid counseling had small but significant effects on expectations. The two forms of counseling did not differ in their effectiveness at changing expectations; however, anecdotally, we learned from many participants that that they enjoyed listening to the auditory demonstrations and that they found them to be an interesting listening exercise. The data also show that positive expectations result in more positive outcome and that hearing aid fine-tuning is beneficial to the user. Conclusions: We conclude that prefitting counseling can be advantageous to hearing aid outcome and recommend the addition of prefitting counseling to address expectations associated with quality of life and self-image. The data emphasize the need to address unrealistic expectations prior to fitting hearing aids cautiously, so as not to decrease expectations to the extent of discouraging and demotivating the patient. Data also show that positive expectations regarding the impact hearing aids will have on psychosocial well-being are important for successful hearing aid outcome.

Stability and change in genetic and environmental influences on hand-grip strength in older male twins

2000 ◽  
Vol 89 (5) ◽  
pp. 1879-1883 ◽  
Author(s):  
Dorit Carmelli ◽  
Terry Reed
Keyword(s):  
Grip Strength ◽  
Environmental Influences ◽  
Hand Grip Strength ◽  
Environmental Stability ◽  
Phenotypic Correlation ◽  
Late Adulthood ◽  
Stability And Change ◽  
Hand Grip ◽  
Genetic Modeling

The aim of this study was to investigate aging-related changes in the contribution of genetic and environmental influences to hand-grip strength in late adulthood. Subjects in this study are 152 intact twin pairs (77 monozygotic and 75 dizygotic pairs) from the National Heart, Lung, and Blood Institute Twin Study assessed repeatedly for hand-grip strength at mean ages of 63 and 73 yr. Structural equation genetic modeling was used to investigate stability and change in the genetic and environmental components of variance of hand-grip strength in late adulthood. Average decline in strength over the 10 yr of follow-up was −1.05 ± 6.8 (SD) kg and was highly significant ( P = 0.003). The test-retest correlation between baseline and follow-up grip strength was 0.62 ( P < 0.001). Bivariate genetic modeling found significant genetic and shared environmental stability in hand-grip strength over the 10 yr of follow-up, with genetic and shared environmental influences accounting for 35 and 48%, respectively, of the test-retest phenotypic correlation. We conclude from these results that stability in hand-grip strength in late adulthood is due primarily to continuity of genetic and familial influences.

scholarly journals Significance of Crescentic Glomeruli in Acute Kidney Injury with Rheumatoid Arthritis

2019 ◽  
Vol 9 (1) ◽  
pp. 42-48
Author(s):  
Ali Ayaash ◽  
Dipesh Maan ◽  
Anastasios Kapetanos ◽  
Mark Bunker ◽  
Mary Chester Wasko ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Acute Kidney Injury ◽  
Kidney Injury ◽  
Renal Outcome ◽  
Primary Role ◽  
Tubular Necrosis ◽  
Immune Mediated ◽  
Autoimmune Conditions ◽  
Spontaneous Improvement

Crescentic glomerulonephritis (GN) without immune reactants or deposits (referred to as pauci-immune) is typically characterized by the presence of anti-neutrophilic cytoplasmic antibodies (ANCA). While ANCA-negative patients might be expected to have a more benign course, they often have poor renal outcomes, especially without treatment with steroids and immune-modulating therapy. Pauci-immune crescentic GN can also co-exist with other autoimmune conditions, including rheumatoid arthritis (RA). Here, we describe an ANCA-negative patient with RA who developed dialysis-requiring acute kidney injury (AKI) with findings consistent with focal pauci-immune crescentic GN (i.e., no IgG or immune complex on kidney biopsy). Coexistent conditions included Klebsiella sepsis attributed to pneumonia, rhabdomyolysis, leukocytoclastic immune-mediated skin vasculitis, and positive ANA. He had spontaneous improvement in renal function without immunosuppressive therapy. This crescentic GN was not associated with poor renal outcome as AKI resolved with supportive care and treatment of his infection. The AKI was likely multifactorial with co-existing acute tubular necrosis in the setting of Kebsiella sepsis and rhabdomyolysis, and the crescentic GN was felt more likely to be related to the infection rather than having a primary role. This case highlights the importance of viewing crescentic GN in the context of the clinical picture, as it may not always lead to the need of aggressive immune suppression and is not a universally poor prognostic kidney finding. However, these cases do warrant close follow-up as our patient had recurrent RA disease manifestations over the next 2 years that eventually led to his death from severe pulmonary hypertension.

scholarly journals Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis

2019 ◽  
Vol 32 (11) ◽  
pp. 727
Author(s):  
Raquel Gil-Gouveia ◽  
Natália Marto ◽  
Pedro Vilela ◽  
Ana Catarino
Keyword(s):  
Hearing Loss ◽  
Fungal Infection ◽  
Infectious Agent ◽  
Brain Biopsy ◽  
High Dose ◽  
Radiological Findings ◽  
Susac Syndrome ◽  
Infection Treatment ◽  
Immune Mediated

Susac syndrome is a rare, probably immune-mediated endotheliopathy presenting with encephalopathy, sensorineural hearing loss and retinal arterial occlusions. A 33-year-old female with Susac syndrome was worsening despite high-dose steroids so a brain biopsy was performed which suggested a possible fungal infection. Treatment with amphotericin B resulted in prompt reversal of symptoms and radiological findings, and no further symptoms occurred during 8 years of follow-up. A diagnosis of fungal infection was not confirmed. The etiology of Susac syndrome is unknown and this anecdotal observation suggests that an infectious agent susceptible to amphotericin might have caused or triggered Susac syndrome in this patient.

Clinical Course of Different Types of Immune Reactions following Keratoplasty

2021 ◽  
Author(s):  
Philip Keye ◽  
Thabo Lapp ◽  
Daniel Böhringer ◽  
Sonja Heinzelmann ◽  
Philip Maier ◽  
...  
Keyword(s):  
Clinical Course ◽  
Corneal Graft ◽  
Long Term Outcomes ◽  
Immune Mediated ◽  
Corneal Graft Rejection ◽  
Different Types ◽  
Lost To Follow Up

Abstract Background Immune-mediated corneal graft rejection (IR) is a leading cause of corneal graft failure. The endothelium, stroma, epithelium, or a combination can be affected. Little is known about the long-term outcomes of different types of IR. Methods We reviewed the medical records of all keratoplasties that had been performed at our eye centre between 2003 and 2016 (n = 3934) for any kind of IR that occurred between the surgery and 2019. All patients with a definite diagnosis of IR and sufficient clinical data were included in the analysis. IRs were grouped according to the affected part of the graft (endothelial, stromal, epithelial, and mixed). We analysed the dynamics of recovery and the clinical outcomes. Results We identified a total of 319 patients with IR. Twenty-seven of those were lost to follow-up and were excluded from further analysis. Of the IRs, 89% affected the endothelium. Endothelial IR resulted more frequently in a considerable loss of endothelial cell density than other forms of IR. Stromal IR showed a lower relapse rate and a better visual recovery than other types of IR and resulted less often in a failure of the graft. Conclusions We herein report comprehensive data about the prognosis regarding functional recovery after different types of IR following keratoplasty. Our data underline that timely recognition and correct classification of IR are important because they determine the clinical course and prognosis.

Arthroscopic Debridement and Arthrolysis for the Treatment of Advanced Kienböck's Disease: 18-Month and 5-Year Postoperative Results

2021 ◽  
Author(s):  
Omer Ayik ◽  
Mehmet Demirel ◽  
Necmettin Turgut ◽  
Okyar Altas ◽  
Hayati Durmaz
Keyword(s):  
Grip Strength ◽  
Dash Score ◽  
Arthroscopic Debridement ◽  
Kienböck’S Disease ◽  
Kienböck's Disease ◽  
Hand Grip ◽  
Case Series Study ◽  
The Mean ◽  
Kienbock’S Disease

Abstract Background Salvage procedures, such as proximal row carpectomy, limited or total wrist arthrodesis, and wrist replacement, are generally preferred to treat advanced Kienböck's disease. However, these procedures are particularly aggressive and may have unpredictable results and potentially significant complications. Questions/Purpose This study aimed to present the short- to mid-term clinical and functional results of arthroscopic debridement and arthrolysis in the management of advanced Kienböck's disease. Patients and Methods Fifteen patients in whom Lichtman Stages IIIA to IIIC or IV Kienböck's disease was diagnosed and treated by arthroscopic wrist debridement and arthrolysis were included in this retrospective study. The mean age was 30 years (range: 21–45). The mean follow-up period duration was 36 months (range: 18–60). The Disabilities of the Arm, Shoulder, and Hand (DASH) score, visual analog scale (VAS), wrist range of motion (ROM), and grip strength were measured preoperatively and then again at the final follow-up visit. Results The mean DASH and VAS scores improved from 41 (range: 31–52) and 7.1 (range: 6–8) preoperatively to 13 (range: 8–21) and 2 (range: 0–3; p < 0.001) at the final follow-up visit, respectively. The mean wrist flexion and extension values increased from 32 (range: 20–60 degrees) and 56 degrees (range: 30–70 degrees; p = 0.009) preoperatively to 34 (range: 10–65 degrees; p = 0.218) and 57 degrees (range: 30–70 degrees; p = 0.296) at the final follow-up appointment, respectively, although these findings were statistically insignificant. The mean strength of the hand grip increased from 22.7 (range: 9–33) to 23.3 (range: 10–34; p = 0.372). Conclusion Arthroscopic debridement and arthrolysis may improve wrist function and quality of life due to the preserved ROM and hand grip strength after short- to mid-term follow-up periods despite the radiographic progression of Kienböck's disease. Level of Evidence This is a Level IV, retrospective case series study.

Abstract P319: Can Deep Learning Find the Ischemic Core on CT? Transfer Learning From Pre-Trained MRI-Based Networks

Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Yannan Yu ◽  
Soren Christensen ◽  
Yuan Xie ◽  
Enhao Gong ◽  
Maarten G Lansberg ◽  
...  
Keyword(s):  
Deep Learning ◽  
Ground Truth ◽  
Learning Model ◽  
Fine Tuning ◽  
Learning Models ◽  
Starting Point ◽  
Stroke Lesion ◽  
Ischemic Core ◽  
Deep Learning Model

Objective: Ischemic core prediction from CT perfusion (CTP) remains inaccurate compared with gold standard diffusion-weighted imaging (DWI). We evaluated if a deep learning model to predict the DWI lesion from MR perfusion (MRP) could facilitate ischemic core prediction on CTP. Method: Using the multi-center CRISP cohort of acute ischemic stroke patient with CTP before thrombectomy, we included patients with major reperfusion (TICI score≥2b), adequate image quality, and follow-up MRI at 3-7 days. Perfusion parameters including Tmax, mean transient time, cerebral blood flow (CBF), and cerebral blood volume were reconstructed by RAPID software. Core lab experts outlined the stroke lesion on the follow-up MRI. A previously trained MRI model in a separate group of patients was used as a starting point, which used MRP parameters as input and RAPID ischemic core on DWI as ground truth. We fine-tuned this model, using CTP parameters as input, and follow-up MRI as ground truth. Another model was also trained from scratch with only CTP data. 5-fold cross validation was used. Performance of the models was compared with ischemic core (rCBF≤30%) from RAPID software to identify the presence of a large infarct (volume>70 or >100ml). Results: 94 patients in the CRISP trial met the inclusion criteria (mean age 67±15 years, 52% male, median baseline NIHSS 18, median 90-day mRS 2). Without fine-tuning, the MRI model had an agreement of 73% in infarct >70ml, and 69% in >100ml; the MRI model fine-tuned on CT improved the agreement to 77% and 73%; The CT model trained from scratch had agreements of 73% and 71%; All of the deep learning models outperformed the rCBF segmentation from RAPID, which had agreements of 51% and 64%. See Table and figure. Conclusions: It is feasible to apply MRP-based deep learning model to CT. Fine-tuning with CTP data further improves the predictions. All deep learning models predict the stroke lesion after major recanalization better than thresholding approaches based on rCBF.

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