scholarly journals Outcome measures and treatment effectiveness in late onset myasthenia gravis

2020 ◽  
Vol 2 (1) ◽  
Author(s):  
Francesca Pasqualin ◽  
Silvia V. Guidoni ◽  
Mario Ermani ◽  
Elena Pegoraro ◽  
Domenico M. Bonifati
Keyword(s):  
Side Effects ◽  
Myasthenia Gravis ◽  
Outcome Measures ◽  
Clinical Features ◽  
Early Onset ◽  
Treatment Effectiveness ◽  
Late Onset ◽  
Positive Outcome ◽  
Response To Treatment

Abstract Background Recently different subtypes of myasthenia gravis (MG) have been described. They differ for clinical features and pathogenesis but the prognosis and response to treatment is less clear. The aim of the study was to evaluate outcome and treatment effectiveness including side effects in late onset MG (LOMG) compared with early onset MG (EOMG). Methods We analysed retrospectively 208 MG patients. Clinical features were recorded as well as treatment and side effects. Outcome at the last follow-up was evaluated with MGSTI and MGPIS scales. Results The 208 patients included were classified as follow: 36 ocular MG, 40 EOMG, 72 LOMG, 25 thymoma-associated, 14 anti-MuSK and 21 double seronegative. Similar positive outcome was achieved in either early and late onset subgroup. We found pharmacological remission and minimal manifestations at the MGFA-PIS in the 95% and 94,4% of EOMG and LOMG respectively but in LOMG a lower dose of immunosuppressors (MGSTI< 2) was required compared to EOMG (p = 0,048). Severe side effects were present in a small percentage of patients in both group but diabetes was more frequent in LOMG vs EOMG (2,2% vs 5%, p = 0.017). Conclusions Despite LOMG has more comorbidities that might interfere with treatment and outcome, therapeutic management does not seem to differ between EOMG and LOMG. A similar positive outcome was seen in both subgroups but LOMG group seems to require lower doses of medication to control symptoms.

A PROSPECTIVE CLINICAL AND IMMUNOLOGICAL STUDY OF LATE ONSET MYASTHENIA GRAVIS

2015 ◽  
Vol 86 (11) ◽  
pp. e4.49-e4
Author(s):  
Girija Sadalage ◽  
Saiju Jacob ◽  
Camilla Buckley ◽  
David Hilton-Jones ◽  
Angela Vincent ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Clinical Features ◽  
Late Onset ◽  
Single Fibre ◽  
Response To Treatment ◽  
Immunological Study ◽  
Serum Samples ◽  
Usual Practice ◽  
Composite Scores

There is an increasing incidence and prevalence of MG in older patients (>50 years) – defined as Late Onset Myasthenia Gravis (LOMG). This study aims to define the demographics, clinical features, response to treatment and immunological features which may distinguish LOMG from the early onset patients (who will also be recruited as controls).Who to refer?▸ All patients should be over the age of 18 years, able to provide informed consent.▸ The study aims to recruit patients who are willing to travel to one of the myasthenia clinics in Birmingham, Nottingham or Oxford▸ All patients with a new diagnosis of MG (or diagnosed in the last 12 months) prior to immunosuppression.▸ Diagnosis of MG based on typical clinical features and the presence of AChR or MuSK Abs, or evidence of neuromuscular transmission defect on single fibre EMG.What it involves for patients▸ Management will be as per usual practice▸ Annual serum samples at follow up with QoL and MG composite scores▸ Follow up for 5 years (3 years by the clinical research fellow and 2 years optionally by the local participating neurologists).

scholarly journals Clinical and therapeutic features of myasthenia gravis in adults based on age at onset

Neurology ◽  
2020 ◽  
Vol 94 (11) ◽  
pp. e1171-e1180 ◽  
Author(s):  
Elena Cortés-Vicente ◽  
Rodrigo Álvarez-Velasco ◽  
Sonia Segovia ◽  
Carmen Paradas ◽  
Carlos Casasnovas ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Early Onset ◽  
Late Onset ◽  
Age At Onset ◽  
Neuromuscular Diseases ◽  
Cross Sectional ◽  
Life Threatening ◽  
Onset Group ◽  
Anti Acetylcholine

ObjectiveTo describe the characteristics of patients with very-late-onset myasthenia gravis (MG).MethodsThis observational cross-sectional multicenter study was based on information in the neurologist-driven Spanish Registry of Neuromuscular Diseases (NMD-ES). All patients were >18 years of age at onset of MG and onset occurred between 2000 and 2016 in all cases. Patients were classified into 3 age subgroups: early-onset MG (age at onset <50 years), late-onset MG (onset ≥50 and <65 years), and very-late-onset MG (onset ≥65 years). Demographic, immunologic, clinical, and therapeutic data were reviewed.ResultsA total of 939 patients from 15 hospitals were included: 288 (30.7%) had early-onset MG, 227 (24.2%) late-onset MG, and 424 (45.2%) very-late-onset MG. The mean follow-up was 9.1 years (SD 4.3). Patients with late onset and very late onset were more frequently men (p < 0.0001). Compared to the early-onset and late-onset groups, in the very-late-onset group, the presence of anti–acetylcholine receptor (anti-AChR) antibodies (p < 0.0001) was higher and fewer patients had thymoma (p < 0.0001). Late-onset MG and very-late-onset MG groups more frequently had ocular MG, both at onset (<0.0001) and at maximal worsening (p = 0.001). Although the very-late-onset group presented more life-threatening events (Myasthenia Gravis Foundation of America IVB and V) at onset (p = 0.002), they required fewer drugs (p < 0.0001) and were less frequently drug-refractory (p < 0.0001).ConclusionsPatients with MG are primarily ≥65 years of age with anti-AChR antibodies and no thymoma. Although patients with very-late-onset MG may present life-threatening events at onset, they achieve a good outcome with fewer immunosuppressants when diagnosed and treated properly.

299 Immune-dysregulation in late onset myasthenia gravis

2018 ◽  
Vol 89 (10) ◽  
pp. A48.1-A48
Author(s):  
Sadalage Girija ◽  
Jacob Saiju ◽  
Maddison Paul
Keyword(s):  
Myasthenia Gravis ◽  
Early Onset ◽  
Late Onset ◽  
Treg Cells ◽  
Double Positive ◽  
Serial Measurement ◽  
Significant Difference ◽  
Antibody Titres ◽  
Composite Scores

IntroductionThe aim of our study was to establish if there were any clinical and immunological differences between early (EOMG) and late onset myasthenia gravis (LOMG).Methods and resultsWe recruited 150 patients with myasthenia gravis making this the largest prospective cohort study in MG to date. Most (74%) patients have LOMG. Of these, 8 (5.33%) are seronegative. Most (88%) are positive for AChR Abs, 4% have MuSK, 2% LRP4 and 10% are double positive to AChR and MuSK Ab on CBA.The incidence of LOMG is higher than EOMG. There is a statistically significant fall in AChR titre levels on serial measurement (p <0.0001). LOMG pts have higher AChR titres at recruitment compared to EOMG (p=0.0498) with higher MG composite scores (p=0.0287). Significantly higher numbers of LOMG pts are positive for AChR Ab on RIA in generalised MG compared to EOMG. A third of the pts with Early Onset Generalised MG are positive for MuSK Abs on CBA. There is no significant difference in clinical presentation between single seropositive pts Vs double seropositive pts. AChR Ab titres are lower in ocular EOMG versus ocular LOMG and titres in ocular patients are lower than in generalised patients.Flow cytometric analysis of PBMCs showed a significantly reduced percentage of Treg cells in our patient cohort compared to healthy controls (p<0.0001) and a significant difference in the levels of TNFa, IL10 and IL17 produced.ConclusionOur study has shown that there are clinical and immunological differences between early onset and late onset myasthenia gravis, both respond well to treatment with an improvement in composite scores and a fall in antibody titres. We are continuing to study this cohort of MG patients with extended follow-up.

P.309 Late-life depression: How do patients with early-onset vs late-onset differ in clinical features and treatment response?

2020 ◽  
Vol 31 ◽  
pp. S52-S53
Author(s):  
W.R. Chae ◽  
M. Fuentes Casan ◽  
F. Gutknecht ◽  
A. Ljubez ◽  
S.M. Gold ◽  
...  
Keyword(s):  
Treatment Response ◽  
Clinical Features ◽  
Early Onset ◽  
Late Onset ◽  
Late Life ◽  
Late Life Depression

scholarly journals Response to treatment with intra-articular triamcinolone hexacetonide and triamcinolone acetonide in oligo articular juvenile idiopathic arthritis

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Rana Harhay ◽  
Wajiha Jeelani ◽  
Barbine Tchamba Agbor Agbor ◽  
Teresa Hennon ◽  
Brian H. Wrotniak ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Side Effects ◽  
Triamcinolone Acetonide ◽  
The United States ◽  
Response To Treatment ◽  
Joint Injection ◽  
Triamcinolone Hexacetonide ◽  
Active Arthritis ◽  
General Response

Abstract Background Oligo-articular juvenile idiopathic arthritis (Oligo JIA) is the most common subtype of juvenile idiopathic arthritis. Intra-articular corticosteroid (IAC) injection is a mainstay treatment of oligo JIA providing pain relief, improving mobility and preventing further joint destruction in the majority of patients. In 2015, production of triamcinolone hexacetonide (TH) an intra-articular corticosteroid was discontinued in the United States leading to use of triamcinolone acetonide (TA) as an alternative. In this study, we compared response to treatment in children with oligo JIA who underwent therapy with intra-articular TA and TH injection. Methods Our study is a retrospective chart review of children with oligo JIA who were treated with IAC injections with TH between January 2012 and June 2015 and TA between J uly 2015 and December 2018. The two groups were followed at John R. Oishei Children’s Hospital of Buffalo and were evaluated for response to treatment, side effects and predictors of response including duration of disease before treatment, erythrocyte sedimentation rate (ESR), and c-reactive protein (CRP). Response to treatment was defined as at least 6 months follow up without evidence of active arthritis in injected joints. Patients were considered to be non-responders if they continued to show active arthritis during their first follow up after joint injection. The primary objective was to evaluate whether there was a significant difference in rate of response between TH and TA. Results Forty-nine patients, 38 female and 11 male with oligo JIA were included in the study. The average age was 6.7 years. A total of 111 joints were injected includin g 78 knees, 13 ankles, 9 wrists, 4 hips, 4 elbows, 2 TMJ and one subtalar joint. In the TA group, 49% (29/59) did not show response to injection compared to 27% (14/52) in the TH group. After 6 months, response rates were better for individuals injected with TH compared to TA (73% vs. 51%). In general, response to intra-articular TH was superior to TA with P = .016 using chi-square test of independence. This difference in outcome was not influenced by other variables such as duration of illness before treatment (P value 0.784) or elevated ESR and CRP. No difference in side effects between the two groups were noted. Conclusion Our results in conjunction with prior published data suggests that TH intra-articular joint injection in oligo JIA is superior to TA, although future controlled trials are necessary for confirmation. An effective, long lasting treatment can have a great impact on the outcome of these children.

scholarly journals Treatment satisfaction in 5q-spinal muscular atrophy under nusinersen therapy

2021 ◽  
Vol 14 ◽  
pp. 175628642199890 ◽  
Author(s):  
Alma Osmanovic ◽  
Gresa Ranxha ◽  
Mareike Kumpe ◽  
Claudia D. Wurster ◽  
Benjamin Stolte ◽  
...  
Keyword(s):  
Side Effects ◽  
Spinal Muscular Atrophy ◽  
Outcome Measures ◽  
Treatment Satisfaction ◽  
Treatment Duration ◽  
Treatment Effectiveness ◽  
Muscular Atrophy ◽  
Patients Satisfaction ◽  
Motor Outcome ◽  
Ambulatory Patients

Background: Nusinersen was the first approved disease-modifying therapy for all 5q-spinal muscular atrophy (SMA) patients regardless of age or disease severity. Its efficacy in adults has recently been demonstrated in a large cohort by motor outcome measures, which were only partially suitable to detect changes in very mildly or severely affected patients. Patient-reported outcome measures (PROs) have been suggested as a valuable addition. Here, we aimed to assess treatment satisfaction and investigate whether it may be a useful PRO to monitor SMA patients. Methods: We enrolled 91 mainly adult 5q-SMA patients treated with nusinersen in a national, multicenter, cross-sectional observational study. 21 patients underwent longitudinal follow up. Patients’ satisfaction with treatment in four dimensions (global, effectiveness, convenience, side effects) was assessed by the Treatment Satisfaction Questionnaire for Medication German version 1.4 (TSQM-1.4©) and related to clinical parameters, motor scores, and treatment duration. Results: More than 90% of SMA patients were consistently satisfied over a median treatment duration of 10 months. Highest mean scores were observed in the dimensions ‘side effects,’ ‘global satisfaction,’ and ‘effectiveness’ (93.5 ± 14.8 versus 73.1 ± 21.0 and 64.8 ± 20.6, respectively). Patients’ satisfaction with the convenience of treatment was considerably lower (43.6 ± 20.2). Interestingly, satisfaction with the effectiveness was higher in ambulatory ( p = 0.014) compared with non-ambulatory patients and directly correlated to motor outcome measures. Five non-ambulatory patients withdrew from therapy. All of them presented with a deterioration of motor outcome measures and reported dissatisfaction with treatment effectiveness and convenience. Conclusion: Most patients were satisfied with nusinersen treatment effectiveness. Less severely affected patients indicated higher satisfaction. The TSQM-1.4© helped to identify therapy non-responders, who mainly addressed dissatisfaction with effectiveness and convenience. We suggest introducing the TSQM-1.4© as an additional PRO in SMA into clinical practice.

Post-thymectomy myasthenia gravis: a case report and systematic review of literature

2021 ◽  
Vol 14 (12) ◽  
pp. e246005
Author(s):  
Louise Gurowich ◽  
Gabriel Yiin ◽  
Adam Maxwell ◽  
Alexandra Rice
Keyword(s):  
Systematic Review ◽  
Case Report ◽  
Myasthenia Gravis ◽  
Early Onset ◽  
Late Onset ◽  
High Morbidity ◽  
Review Of Literature ◽  
Prompt Treatment ◽  
Autoimmune Condition ◽  
Anti Acetylcholine

Myasthenia gravis (MG) is an autoimmune condition affecting the neuromuscular junction characterised by weakness and fatiguability, carrying a high morbidity if treatment is delayed. A clear association with thymoma has led to management with thymectomy as a common practice, but MG presenting post-thymectomy has rarely been reported. We present a case of an 82- year-old woman developing fatigue, ptosis and dysarthria 3 months after thymectomy. After a clinical diagnosis of MG was made, she responded well to prompt treatment with prednisolone and pyridostigmine. Her anti-acetylcholine receptor antibody (anti-AChR) subsequently came back positive. Our systematic review reveals that post-thymectomy MG can be categorised as early-onset or late-onset form with differing aetiology, and demonstrated correlation between preoperative anti-AChR titres and post-thymectomy MG. The postulated mechanisms for post-thymectomy MG centre around long-lasting peripheral autoantibodies. Clinicians should actively look for MG symptoms in thymoma patients and measure anti-AChR preoperatively to aid prognostication.

Age, Age of Onset and Course of Primary Depressive Illness in the Elderly*

1983 ◽  
Vol 28 (2) ◽  
pp. 102-104 ◽  
Author(s):  
Martin G. Cole
Keyword(s):  
Elderly Patients ◽  
Depressive Episode ◽  
Early Onset ◽  
Age Of Onset ◽  
Late Onset ◽  
The Elderly ◽  
Depressive Illness ◽  
Physical Illness ◽  
Course Of Illness

Thirty-eight elderly patients with primary depressive illness (Feighner criteria) were followed up for 7–31 months. In the absence of persistent organic signs and severe physical illness, age of onset (first depressive episode after 60) but not age was significantly related to course of illness. Compared to early onset depressives, late onset depressives were more likely to remain completely well during the follow-up period and less likely to have frequent or disabling relapses.

scholarly journals Angiotensin-converting enzyme inhibition in the prevention and treatment of chronic renal damage in the hypertensive fawn-hooded rat.

1997 ◽  
Vol 8 (2) ◽  
pp. 249-259 ◽  
Author(s):  
G H Verseput ◽  
A P Provoost ◽  
B B Braam ◽  
J J Weening ◽  
H A Koomans
Keyword(s):  
Capillary Pressure ◽  
Enzyme Inhibition ◽  
Early Onset ◽  
Renal Damage ◽  
Late Onset ◽  
Converting Enzyme ◽  
Glomerular Hypertension ◽  
Converting Enzyme Inhibition

The spontaneously hypertensive fawn-hooded rat (FHH) develops accelerated albuminuria and focal glomerular sclerosis (FGS), leading to ESRD and shortening of lifespan. The FHH is characterized by moderate systemic hypertension, a relatively low afferent to efferent arteriolar resistance ratio, and glomerular hypertension. The FHH study presented here was designed to examine the efficacy of early-onset, late-onset, or early-temporary angiotensin I-converting enzyme inhibition (ACE-i) in ameliorating long-term hypertension and FGS, and improving survival, as well as to relate its protective efficacy to preexistent FGS and to reduction of glomerular pressure (PGC) Untreated rats developed hypertension and high PGC, and all (N = 22) except one died of ESRD within the 72-wk follow-up period. Early-onset (at 7 wk of age) ACE-i prevented development of systemic and glomerular hypertension, and it largely prevented proteinuria and FGS; all rats survived throughout the follow-up period. Rats treated with late-onset (22 wk) ACE-i were hypertensive and proteinuric at the start of ACE-i, and they showed beginning FGS. ACE-i corrected the hypertension, albuminuria, and PGC but could not fully prevent some hypertension, albuminuria, and FGS at the later stage. Early-temporary (7 to 22 wk) ACE-i adequately controlled blood pressure and development of FGS during therapy, but after withdrawal of ACE-i, systemic and glomerular hypertension developed as in untreated animals. This regimen postponed but did not control FGS development and early mortality. The results of this study indicate that: (1) early-onset ACE-i very effectively protects against development of renal damage in the FHH; (2) this protection is associated with normalization of the elevated glomerular capillary pressure; (3) ACE-i cannot completely prevent further development of previously established FGS, despite lowering glomerular capillary pressure; (4) early-temporary ACE-i has no long-term controlling effect on arterial and glomerular pressure, and it cannot control development of FGS.

Choice of Antenatal Steroids for Prevention of Complications of Prematurity: Betamethasone Versus Dexamethasone

2021 ◽  
pp. 097321792110483
Author(s):  
Tanushree Sahoo ◽  
Abhishek Somasekhara Aradhya ◽  
Kanya Mukhopadhyay
Keyword(s):  
Side Effects ◽  
Early Onset ◽  
Lower Cost ◽  
Antenatal Steroids ◽  
Term Adverse Effect ◽  
Early Onset Sepsis ◽  
Poor Neurological Outcome ◽  
Long Term Follow Up

Antenatal steroids (ANS) are proven strategies to maximize outcomes of premature neonates without any major maternal side effects. Their use results in decreased incidence of neonatal mortality and major morbidities (respiratory distress syndrome, early onset sepsis, necrotizing enterocolitis, and intraventricular hemorrhage). However, due to concerns of long-term adverse effect (early onset hypertension and poor neurological outcome), a close follow-up is required. Similarly, due to lack of long-term follow-up data and potential risk of hypoglycemia, a cautious use is recommended in late preterms and elective cesareans. There is currently no consensus regarding preferential use of one ANS over the other. The current review therefore tried to address these issues for use of ANS in Indian prospective in light of recent emerging evidence. Due to better safety profile, lesser side effects, lower cost, and easy storage, we recommend dexamethasone as a steroid of choice for antenatal prophylaxis.

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