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.

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.

The Evolution of Dementia in Idiopathic Parkinson's Disease: Neuropsychological and Clinical Evidence in Support of Subtypes

1992 ◽  
Vol 4 (4) ◽  
pp. 147-160 ◽  
Author(s):  
Wayne G. J. Reid
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Early Onset ◽  
Late Onset ◽  
Age At Onset ◽  
Critical Determinant ◽  
Drug Study ◽  
Baseline Phase ◽  
Onset Group ◽  
Early Onset Group

One hundred and seven newly diagnosed, untreated patients with Parkinson's disease (PD) were divided into two groups according to their age at reported onset of symptoms. Of these, 79 patients were under age 70 (early-onset) and 28 patients were age 70 and over (late-onset). The group of 50 control subjects comprised spouses, friends of the PD patients, and community volunteers. The patients were participants in a multicenter drug study of Parkinson's disease. Each had received a detailed neurological and neuropsychological assessment in the baseline placebo phases of the study. Thirty-4 patients with early-onset and 12 patients with late-onset were reassessed 3 years after treatment with low-dose levodopa, with bromocriptine, or with a combination of the two drugs. The results of the baseline phase of the study revealed that 8% of the early-onset group and 32% of the late-onset group were classified as demented. The 3-year follow-up revealed that the prevalence of dementia had increased to 17% in the early-onset group and to 83% in the late-onset group. This study confirms that at least two distinct subtypes of Parkinson's disease exist. The subtypes differ both clinically and neuropsychologically. The age at onset of symptoms is a critical determinant of the rate and type of cognitive decline in Parkinson's disease.

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.

scholarly journals Differences Ratio Level Soluble FMS-Like Tyrosine Kinase-1 and Placental Growth Factor Early And Late Onset on Preeclampsia and Normal Pregnancy

2018 ◽  
Vol 3 (1) ◽  
pp. 83
Author(s):  
Ria Andina ◽  
Yanwirasti Yanwirasti ◽  
Defrin Defrin
Keyword(s):  
Growth Factor ◽  
Tyrosine Kinase ◽  
Early Onset ◽  
Late Onset ◽  
Normal Pregnancy ◽  
Elisa Test ◽  
Placental Growth Factor ◽  
Cross Sectional ◽  
Onset Group ◽  
Early Onset Group

Preeclampsia is a major maternal morbidity and mortality worldwide including in Indonesia. PIGF concentrations were found to be lower and sFlt-1 to be higher in patients with preeclampsia than normal pregnancy. Futher, this factor has been proposed as a parameter that can help identify women with potentially preeclampsia.This study aims todeterminethe differences ratio level soluble rate fms-like tyrosine kinase-1 and placental growth factor early and late onset on preeclampsia and normal pregnancy. The cross sectional study design was conducted in RSUP M.Djamil, Rasidin Hospital, Reksodiwiryo Hospital and Biomedical Laboratory of Andalas University from July 2017 until January 2018. The sample of this study was pregnant women>20-42 weeks pregnancy totalling 80 people by consecutive sampling.Sample was divided into 3 groups. SFlt-1 and PlGF levels tested using ELISA test. Test the normality of data by Kolmogrov-Smirnov test by using unpaired T test.The results showed median sFLT-1 levels in the early onset group with normal pregnancy with p= 0,88, median sFLT-1 levels in the late onset group with normal pregnancy with p= 0,01 and median levels of sFLT-1 in the early onset group with late onset with p= 0.34. Mean of PlGF in the early onset group with normal pregnancy with p=0,30, mean of PlGF in the late onset group with normal pregnancy with p= 0.63, and mean of PlGF in the early onset group with late onset with p = 0.27. The conclusion of this study was that there was a difference ratio level Soluble Fms-Like Tyrosine Kinase-1 late onset in preeclampsia and normal pregnancy.

Clinical Features of Late-onset Ankylosing Spondylitis: Comparison with Early-onset Disease

2012 ◽  
Vol 39 (5) ◽  
pp. 1008-1012 ◽  
Author(s):  
CARLOS MONTILLA ◽  
JAVIER DEL PINO-MONTES ◽  
EDUARDO COLLANTES-ESTEVEZ ◽  
PILAR FONT ◽  
PEDRO ZARCO ◽  
...  
Keyword(s):  
Ankylosing Spondylitis ◽  
Clinical Features ◽  
Early Onset ◽  
Late Onset ◽  
Age At Onset ◽  
Young Patients ◽  
Lower Limbs ◽  
Joint Disease ◽  
Control Group ◽  
Onset Group

Objective.Ankylosing spondylitis (AS) is generally observed in young patients but can occur later in life or in persons ≥ 50 years of age. Our objective was to characterize the clinical features of late-onset AS in a large multicenter national cohort.Methods.We studied late-onset AS in the National Registry of Spondyloarthritis of the Spanish Society of Rheumatology (REGISPONSER database) cohort (n = 1257), of whom 3.5% had onset at age ≥ 50 years versus a control group with onset at < 50 years.Results.There were no differences between late-onset and early-onset AS according to sex and family history of spondyloarthropathies. Patients in the late-onset group more often showed involvement of the cervical spine (22.7% vs 9.7%; p = 0.03) and arthritis of the upper (13.6% vs 3.0%; p = 0.002) and lower limbs (27.3% vs 15.2%; p = 0.03) as first manifestations than did patients in the early-onset group. A higher percentage of mixed forms (axial and peripheral joint disease) during the course of the disease was also recorded in the late-onset group (50% vs 24%; p = 0.0001).Conclusion.Our study suggests that age at onset of AS affects the patients’ presenting clinical form. Arthritis of the upper limbs requires a differential diagnosis with other conditions frequent in patients over 50 years of age, such as rheumatoid arthritis or crystal-induced arthropathy.

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.

Age at Onset and Clinical Course of Body Dysmorphic Disorder

2017 ◽  
Author(s):  
Andri S. Bjornsson
Keyword(s):  
Early Onset ◽  
Body Dysmorphic Disorder ◽  
Clinical Course ◽  
Late Onset ◽  
Age At Onset ◽  
Treatment Interventions ◽  
Course Of Illness ◽  
Appropriate Treatment ◽  
Onset Group ◽  
Early Onset Group

This chapter reviews studies that have been conducted to determine the age at onset and course of illness of body dysmorphic disorder (BDD). Age at onset has been examined in two large samples, and the mean age at onset was the same in both studies (16.7 years). About two thirds of people had onset of BDD before age 18. There were more similarities than differences between the early-onset group (before age 18) and the later-onset group, although the early-onset group had a higher prevalence of attempted suicide than the late-onset group in both samples. The clinical course of BDD is often chronic unless appropriate treatment is received. These findings point to the need for early education on healthy body image and treatment interventions for BDD, especially among adolescents.

scholarly journals A Cross-sectional Study of Psychiatric Comorbidity and Severity of Addiction in Patients with Early- and Late-Onset Alcohol Dependence

2020 ◽  
Vol 42 (4) ◽  
pp. 334-340
Author(s):  
Anamika Das ◽  
Sujita Kumar Kar ◽  
Pawan Kumar Gupta ◽  
Pronob Kumar Dalal
Keyword(s):  
Alcohol Dependence ◽  
Early Onset ◽  
Late Onset ◽  
Severity Index ◽  
Cross Sectional Study ◽  
Addiction Severity Index ◽  
Cross Sectional ◽  
Addiction Severity ◽  
Onset Group ◽  
Early Onset Group

Background: Alcohol use disorder is a serious health problem with high comorbidities. Early-onset alcohol use has been associated with greater impulsivity, increased severity of dependence, frequent alcohol withdrawal complications, externalizing symptoms, and antisocial behaviors. We aimed to evaluate the psychiatric comorbidities and severity of addiction in early- and late-onset alcohol dependence. Methods: This was a cross-sectional study. All patients fulfilling the diagnostic criteria of alcohol dependence as per International Classification of Diseases (10th edition) were included in it. Semi-Structured Assessment for Genetics of Alcoholism IV was applied to find the age of onset of dependence as per the lifetime frame and also to find comorbidities. Composite International Diagnostic Interview 3.0. was used to find other comorbidities. The severity of addiction was evaluated with Addiction Severity Index, 5th edition. All the sociodemographic and clinical parameters were compared between patients with the early- and late-onset alcohol dependence. Results: Out of the 112 patients screened, 57 met the selection criteria, 26 were with early-onset and 31 with late-onset alcohol dependence. The patients were all males. The patients with early-onset alcohol dependence had a higher family history (P = 0.006) and were nonearning (P = 0.002) in comparison to the group with late-onset dependence. The comorbidity among all patients was 59.6% and 84.2% in current and lifetime frames, respectively. It was significantly higher in the early-onset group, both for current (P = 0.015) and lifetime (P = 0.031) frames. On the domains of Addiction Severity Index 5th edition, the early-onset group had a more severe profile of addiction in all domains except the medical domain. Conclusion: Comorbidity is high among patients with alcohol dependence and is even higher for the early-onset group. The family history was higher in the early-onset group and they have more severe profile of substance use.

scholarly journals Food for Thought: The Ketogenic Diet and Adverse Effects in Children

2005 ◽  
Vol 5 (4) ◽  
pp. 152-154 ◽  
Author(s):  
Michael S. Duchowny
Keyword(s):  
Ketogenic Diet ◽  
Early Onset ◽  
Late Onset ◽  
Intractable Epilepsy ◽  
Density Lipoprotein ◽  
Renal Stones ◽  
Deficiency Anemia ◽  
Lipoid Pneumonia ◽  
Life Threatening

Early- and Late-Onset Complications of the Ketogenic Diet for Intractable Epilepsy Kang HC, Chung da E, Kim DW, Kim HD Epilepsia 2004;45:1116–1123 Purpose This study was undertaken to evaluate the exact limitations of the ketogenic diet (KD) and to collect data on the prevention and management of its risks. Methods Patients (129) who were on the KD from July 1995 to October 2001 at our epilepsy center were assessed in the study. Early-onset (within 4 weeks of the commencement of the KD until stabilization) and late-onset complications (occurring after 4 weeks) were reviewed. Results The most common early-onset complication was dehydration, especially in patients who started the KD with initial fasting. Gastrointestinal disturbances, such as nausea/vomiting, diarrhea, and constipation, also were frequently noted, sometimes associated with gastritis and fat intolerance. Other early-onset complications, in order of frequency, were hypertriglyceridemia, transient hyperuricemia, hypercholesterolemia, various infectious diseases, symptomatic hypoglycemia, hypoproteinemia, hypomagnesemia, repetitive hyponatremia, low concentrations of high-density lipoprotein, lipoid pneumonia due to aspiration, hepatitis, acute pancreatitis, and persistent metabolic acidosis. Late-onset complications also included osteopenia, renal stones, cardiomyopathy, secondary hypocarnitinemia, and iron-deficiency anemia. Most early- and late-onset complications were transient and successfully managed by careful follow-up and conservative strategies. However, 22 (17.1%) patients ceased the KD because of various kinds of serious complications, and 4 (3.1%) patients died during the KD, two of sepsis, one of cardiomyopathy, and one of lipoid pneumonia. Conclusions Most complications of the KD are transient and can be managed easily with various conservative treatments. However, life-threatening complications should be monitored closely during follow-up.

scholarly journals The Constellation of Macrovascular Risk Factors in Early Onset T2DM: A Cross-Sectional Study in Xinjiang Province, China

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Mingchen Zhang ◽  
Jiangfeng Mao ◽  
Ablikm Tuerdi ◽  
Xiaoyun Zeng ◽  
Li Quan ◽  
...  
Keyword(s):  
Risk Factors ◽  
Early Onset ◽  
Late Onset ◽  
Microvascular Complications ◽  
Macrovascular Complications ◽  
Control Group ◽  
Cross Sectional Survey ◽  
Cross Sectional ◽  
Onset Group ◽  
Early Onset Group

Background. Despite a rapid popular of early onset type 2 diabetes (defined as diagnosis at <40 years old) recently, there is a lack of studies on this population in economically undeveloped area. We aimed to investigate the risk factors of macrovascular complications in the early onset T2DM patients in Xinjiang, China. Methods. A cross-sectional survey of 1736 consecutive patients with T2DM was conducted. Macrovascular complications and risk factors were documented. Another nondiabetic population matched with age and sex was as a control group. Logistic regression analysis was performed to obtain odds ratios (OR) for macrovascular complications in early and late onset T2DM, respectively. Results. The final analysis consisted of 1036 late onset and 219 early onset T2DM patients. The mean HbA1c in the early onset group was higher than that in the late onset group (9.1 ± 2.4% versus 8.3 ± 2.2%, P=0.039) despite a higher proportion of patients in the early onset group receiving insulin treatment (73.1% versus 58.7%, P<0.001). Compared to the control, early onset patients had higher blood pressure and worse lipid profiles (all P<0.01). More than half of the early onset T2DM patients already had macro- and microvascular complications, despite of their young age (39.5 ± 10.8) and short DM duration (6.6 ± 8.0). In the early onset group, women had a ~3-fold hazard of atherosclerotic plaques compared with men (OR 3.22, 95% CI 1.53–6.78). Conclusions. Patients with early onset T2DM have worse glycemic control and higher burden of atherogenic risk factors. The prevalence of macro- and microvascular complications is astonishingly high in these young adults with T2DM. Moreover, young women with T2DM are more susceptible to cardiovascular complications than their male counterpart.

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