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BMC Neurology Nov 2022Overlap syndromes of anti-NMDA receptor encephalitis and MOG-mediated demyelination have been reported. In this case we provide a long-term longitudinal follow-up of...
BACKGROUND
Overlap syndromes of anti-NMDA receptor encephalitis and MOG-mediated demyelination have been reported. In this case we provide a long-term longitudinal follow-up of clinical and imaging characteristics as well as of antibody dynamics.
CASE PRESENTATION
We report a 32-year-old male patient who presented with psychosis, decreased consciousness and movement disorders and was tested positive for anti-NMDA receptor antibodies. Forty-four months after symptom onset and diagnosis of autoimmune encephalitis, he suffered from relapse. At this time, the patient developed anti-MOG and anti-Caspr2 antibodies. Treatment with plasmapheresis, steroids and rituximab eventually led to substantial clinical and radiological improvement. Anti-Caspr2 antibodies persisted, anti-NMDA receptor antibodies decreased, while anti-MOG antibodies turned negative again.
CONCLUSION
We provide long-term longitudinal follow-up of a patient with anti-NMDA receptor encephalitis who developed triple antibody positivity at the time of relapse. Antibody dynamics were associated with clinical disease course.
Topics: Male; Humans; Adult; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Myelin-Oligodendrocyte Glycoprotein; Follow-Up Studies; Autoantibodies; Neoplasm Recurrence, Local; Receptors, N-Methyl-D-Aspartate; Demyelinating Diseases
PubMed: 36384491
DOI: 10.1186/s12883-022-02974-x -
Annals of Palliative Medicine Nov 2019No systematic reviews of acupuncture as a treatment for myasthenia gravis (MG) have been published in English. The aim of our study is to evaluate the efficacy and... (Meta-Analysis)
Meta-Analysis
BACKGROUND
No systematic reviews of acupuncture as a treatment for myasthenia gravis (MG) have been published in English. The aim of our study is to evaluate the efficacy and safety of acupuncture as a treatment for MG.
METHODS
We searched for randomized controlled trials (RCTs) in seven main electronic databases. Unpublished articles, including conference papers and Chinese doctoral and master's theses, were also included as supplementary sources. The primary outcome was the relative clinical score (RCS) response rate. We performed a meta-analysis using RR and MD with 95% CI.
RESULTS
Thirteen RCTs involving a total of 775 participants were included. Most included trials had a high risk of bias in allocation concealment and blinding. Eleven RCTs used acupuncture as an adjuvant to medication, and this treatment showed a significant improvement in the RCS response rate compared to medication alone (RR: 1.42; 95% CI: 1.06-1.91; P=0.02). The subgroup analysis based on the treatment duration showed a significant effect on the RCS response rate when the treatment duration was longer than 12 weeks (RR: 2.02; 95% CI: 1.31-3.12; P=0.001). In contrast, there was no significant effect of treatment with a duration less than 8 weeks (RR: 1.14; 95% CI: 0.91-1.44; P=0.26). Four RCTs showed a significant difference in the absolute clinical score (ACS) (RR: 3.42; 95% CI: 1.23-5.61; P=0.002). The acupuncture group reported better outcomes. No severe adverse events corresponding to acupuncture were reported.
CONCLUSIONS
This meta-analysis suggests that acupuncture as an integrative therapy has a significant positive effect in treating MG. Acupuncture may enhance the efficacy of medication in MG patients. The safety of acupuncture requires further investigation. The clinical significance of these changes needs to be investigated by further studies using rigorous designs and longer follow-up times.
Topics: Acupuncture Therapy; Humans; Myasthenia Gravis; Patient Safety; Quality of Life
PubMed: 31865721
DOI: 10.21037/apm.2019.10.10 -
Journal of Neuroinflammation Oct 2021Approximately 30% of individuals with severe SARS-CoV-2 infections also develop neurological and psychiatric complaints. In rare cases, the occurrence of autoimmune...
Approximately 30% of individuals with severe SARS-CoV-2 infections also develop neurological and psychiatric complaints. In rare cases, the occurrence of autoimmune encephalitis has been reported after SARS-CoV-2 infection. In this systematic review, we have identified eight SARS-CoV-2-associated cases of anti-NMDA receptor encephalitis. All had cerebrospinal fluid antibodies against the NMDA receptor and a recent onset of working memory deficits, altered mental status, or psychiatric symptoms, such as confusion, agitation, auditory hallucination, catatonia and speech dysfunction. All patients received high-dose steroid and immunoglobulin therapeutics and conditions improved in each case. These findings suggest that clinical attention should be paid to warning signs of autoimmune encephalitis in severe COVID-19 cases. If characteristic features of autoimmune encephalitis are present, autoantibody diagnostics should be performed and confirmed cases should be treated with immunotherapy to minimize neurological impairments.
Topics: Adolescent; Adult; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Autoantibodies; COVID-19; Child; Female; Humans; Infant; Male; Mental Disorders; Middle Aged; Molecular Mimicry; Receptors, N-Methyl-D-Aspartate; SARS-CoV-2; Young Adult
PubMed: 34711233
DOI: 10.1186/s12974-021-02293-x -
The Primary Care Companion For CNS... Jun 2020To present a review of the literature on the clinical presentation and pathophysiology of anti-N-methyl-d-aspartate receptor encephalopathy (ANMDARE) with attention to...
OBJECTIVE
To present a review of the literature on the clinical presentation and pathophysiology of anti-N-methyl-d-aspartate receptor encephalopathy (ANMDARE) with attention to both the more commonly recognized psychotic symptom prodrome and the less well-understood depressive symptom prodrome.
DATA SOURCES
The search for clinical neuropsychiatric phenomena and proposed mechanisms involved in ANMDARE pathophysiology was conducted in PubMed. English-language articles published up to September 2019 were identified using a combination of the following search terms: N-methyl-d-aspartate, anti-NMDA receptor encephalitis, schizophrenia, psychosis, depression, major depressive disorder, bipolar I disorder, bipolar II disorder, anxiety, and posttraumatic stress disorder.
STUDY SELECTION
From 150 articles identified from the initial search, the 73 most relevant clinical studies, reviews, and case reports related to the study objectives were included.
DATA EXTRACTION
Sources were individually analyzed by the 3 authors for the most clinically relevant information.
RESULTS
The pathophysiology and mechanisms involved in anti-NMDA receptor antibody delivery to the brain are incompletely characterized, but antibody binding appears to involve the GluN1 subunit in most cases. Psychotic symptoms are the most commonly recognized components of prodromal psychiatric illness in ANMDARE, which may lead to an initial diagnosis of schizophrenia. In addition to psychotic symptoms, there are reports of depressive symptoms occurring before the emergence of, co-occurring with, or instead of psychotic symptoms in ANMDARE.
CONCLUSIONS
In addition to the better-known psychotic prodrome, depressive symptomatology can occur in ANMDARE patients. ANMDARE should be considered in patients with initial presentation of either psychotic or atypical depressive illnesses. Early recognition of these psychiatric prodromal states as antecedents to ANMDARE could lead to improved diagnosis and better management of this potentially life-threatening autoimmune disorder.
Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Depression; Humans; Prodromal Symptoms; Psychotic Disorders
PubMed: 32569449
DOI: 10.4088/PCC.19r02563 -
BMJ Case Reports Dec 2021Myasthenia gravis (MG) is an autoimmune condition affecting the neuromuscular junction characterised by weakness and fatiguability, carrying a high morbidity if...
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.
Topics: Aged, 80 and over; Female; Humans; Autoantibodies; Myasthenia Gravis; Receptors, Cholinergic; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 34857591
DOI: 10.1136/bcr-2021-246005 -
European Radiology Jul 2024It is uncertain whether modern iodine-based or gadolinium-based contrast media (CM) administration can lead to increased symptoms in patients with myasthenia gravis. (Review)
Review
OBJECTIVES
It is uncertain whether modern iodine-based or gadolinium-based contrast media (CM) administration can lead to increased symptoms in patients with myasthenia gravis.
METHODS
A systematic search in Medline was conducted for studies describing the symptomatology of myasthenia gravis patients before and after receiving intravenous (IV) CM and having a matched control group of myasthenia gravis patients who did not receive IV CM.
RESULTS
Three retrospective studies were selected with a total of 374 myasthenia gravis patients who received iodine-based CM and a total of 313 myasthenia gravis patients who underwent unenhanced CT and served as controls. Pooling of the data from the three retrospective studies showed that in 23 of 374 patients, increased symptoms after iodine-based CM administration were described (6.1%). Increased symptomatology also occurred in 11 of 313 patients after unenhanced CT (3.5%). When looking more deeply into the data of the three studies, conflicting results were found, as two articles did not find any relationship between CM and myasthenia gravis symptoms. The remaining study only found a significant increase in symptomatology within 1 day after CT scanning: seven patients (6.3%) in the contrast-enhanced CT group and one patient (0.6%) in the unenhanced CT group (p = 0.01).
CONCLUSIONS
There is limited evidence on the relationship between CM and myasthenia gravis symptoms. In the vast majority of myasthenia gravis patients, CM are safe. Probably, in less than 5% of the patients, iodine-based CM administration may lead to increased severity of the symptoms within the first 24 h after administration.
CLINICAL RELEVANCE STATEMENT
Be aware that intravenous administration of iodine-based contrast media can lead to an increase of symptoms in patients with myasthenia gravis within the first 24 h. This can probably happen in less than 5% of the patients.
KEY POINTS
• It is unclear whether modern contrast media can lead to increased symptoms in myasthenia gravis patients after intravenous administration. • There seems to be a small risk of increased myasthenia gravis symptoms within 24 h after intravenous administration of iodine-based contrast media, probably in less than 5% of the administrations. • Gadolinium-based contrast media are safe for patients with myasthenia gravis.
Topics: Myasthenia Gravis; Humans; Contrast Media; Tomography, X-Ray Computed; Gadolinium; Practice Guidelines as Topic; Europe; Iodine
PubMed: 38092951
DOI: 10.1007/s00330-023-10463-z -
Neuromuscular Disorders : NMD Aug 2020Myasthenia Gravis (MG) is a chronic autoimmune disease affecting the neuromuscular junction. Although a hallmark of MG is muscle fatigability due to dysfunction of the...
Myasthenia Gravis (MG) is a chronic autoimmune disease affecting the neuromuscular junction. Although a hallmark of MG is muscle fatigability due to dysfunction of the neuromuscular junction (peripheral fatigue), a large number of MG patients also report symptoms of central fatigue, defined as an experienced lack of energy, physically and/or mentally. We systematically reviewed the literature on all aspects of central fatigue in MG. Results were categorized in 5 domains: prevalence, diagnosis, pathophysiology, treatment or impact. The prevalence of patient-reported fatigue varies between 42 and 82%, which is significantly higher than in control subjects. Fatigue severity is usually assessed with standardized questionnaires, but the choice of questionnaire varies widely between studies. The pathophysiology of fatigue is unknown, but it is strongly associated with depressive symptoms, female gender and disease severity. Fatigue is also highly prevalent in ocular MG and patients in remission, suggesting a multifactorial origin. Fatigued MG patients have a lower quality of life. Pharmacological treatment of MG is associated with improvement of fatigue and promising results have been found with physical and psychological training programs. Fatigue is a highly prevalent symptom of MG with a severe negative impact on quality of life. Physicians treating patients with MG should be aware of this symptom, as it may be treatable with physical or psychological training programs.
Topics: Fatigue; Female; Humans; Male; Myasthenia Gravis; Neuromuscular Junction; Prevalence; Quality of Life; Surveys and Questionnaires
PubMed: 32718868
DOI: 10.1016/j.nmd.2020.06.010 -
Journal of Cardiothoracic Surgery Sep 2020Despite the burgeoning literature describing preoperative and postoperative risks of a myasthenic crisis after thymectomy (MCAT) in patients with myasthenia gravis,... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Despite the burgeoning literature describing preoperative and postoperative risks of a myasthenic crisis after thymectomy (MCAT) in patients with myasthenia gravis, substantial differences exist in the risk factors identified by previous studies. We conducted a meta-analysis to assess the reported risk factors and MCAT risk.
METHODS
We collected relevant studies on the risk factors for MCAT by searching the PubMed, Embase, The Cochrane Library, China Biology Medicine (CBM), WanFang Data, VIP and CNKI databases. The search period ranged from the establishment of the database to November 2019.
RESULTS
Twenty-five of the 458 identified studies were eligible for the meta-analysis. Seven retrospective cohort studies and 18 case-control studies were included, and 14 risk factors for MCAT were extracted. Meta-analyses of the association between MCAT and risk factors related to the patient's preoperative condition included a preoperative history of MC, preoperative bulbar symptoms, IIa + IIb + III + VI, IIb + III + VI, VI + V, dosage of pyridostigmine bromide prior to the operation, a preoperative AchR-Ab level > 100 (nm/L), preoperative pulmonary function, preoperative complications, and preoperative disease course. Meta-analyses of the association between MCAT and surgery-related risk factors included intraoperative blood loss > 1000 mL and the mode of operation. Meta-analyses of the association between MCAT and postoperative risk factors included postoperative lung infection, thymoma and the WHO classification. The operation time was not an independent risk factor for MCAT.
CONCLUSIONS
The independent risk factors for MCAT were a preoperative history of MC, preoperative bulbar symptoms, preoperative MG Osserman stage, preoperative dosage of pyridostigmine bromide, preoperative serum AchR-Ab level, lung function, major postoperative complications, disease duration before thymectomy, blood loss, thoracotomy, postoperative lung infection, thymoma, and WHO classification.
Topics: Blood Loss, Surgical; Databases, Factual; Female; Humans; Male; Myasthenia Gravis; Operative Time; Postoperative Complications; Risk Factors; Thymectomy
PubMed: 32993739
DOI: 10.1186/s13019-020-01320-x -
BMC Endocrine Disorders Sep 2020Previous studies have shown inconsistent results about the usefulness of bilateral inferior petrosal sinus sampling (BIPSS) in differential diagnosis of... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Previous studies have shown inconsistent results about the usefulness of bilateral inferior petrosal sinus sampling (BIPSS) in differential diagnosis of adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. This meta-analysis evaluated the diagnostic value of BIPSS via the published literature.
METHODS
This study searched PubMed, Embase, Web of Science, Cochrane library, and Wanfang database for published data on the use of BIPSS in Cushing syndrome differential diagnosis as of October 2019. Sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), diagnostic odds ratio (DOR), and receiver operating characteristic (ROC) curves were calculated based on the relevant data.
RESULTS
This meta-analysis included a total of 23 studies with 1642 patients. The calculated sensitivity, specificity, PLR, and NLR were 0.94 (95% confidence interval, CI: 0.91-0.96), 0.89 (95% CI: 0.79-0.95), 8.8 (95% CI: 4.3-17.9), and 0.07 (95% CI: 0.04-0.11), respectively. The pooled DOR and area under the ROC curve were 129 (95% CI: 48-345) and 0.97 (95% CI: 0.95-0.98), respectively.
CONCLUSION
This meta-analysis indicated that BIPSS had high diagnostic value for detecting ACTH in patients with ACTH-dependent Cushing syndrome, and BIPSS should be used as an effective method to identify ACTH-secretion sources.
Topics: ACTH Syndrome, Ectopic; Adrenocorticotropic Hormone; Cushing Syndrome; Databases, Factual; Diagnosis, Differential; Humans; Petrosal Sinus Sampling
PubMed: 32943040
DOI: 10.1186/s12902-020-00623-3 -
BMC Neurology Nov 2019Myasthenia gravis associated takotsubo syndrome is a rare condition. This study aimed to explore its typical presentation, investigations and treatment through a...
BACKGROUND
Myasthenia gravis associated takotsubo syndrome is a rare condition. This study aimed to explore its typical presentation, investigations and treatment through a systematic review of previously reported cases.
METHODS
Databases and reference lists of the selected articles were searched for case reports on Myasthenia gravis associated takotsubo syndrome. CARE guidelines were used for the quality assessment of the selected articles.
RESULTS
Sixteen cases were selected out of 580 search results. Western Pacific, American and European regions contributed to 88% of the cases. Females were most affected (81%). Features of both myasthenia gravis and takotsubo syndrome were the common clinical presentations. All cases had a myasthenic crisis. Half of the cases had no prior diagnosis of myasthenia gravis. Pyridostigmine and prednisolone were useful for myasthenia gravis while dobutamine was most commonly used for takotsubo syndrome. All cases survived except four (25%).
CONCLUSIONS
Myasthenia gravis associated takotsubo syndrome via a myasthenic crisis is rare but life-threatening. Therefore, predisposition due to emotional and physical triggers needs to be avoided for its prevention. The rare entity should be suspected even in patients without a prior diagnosis of Myasthenia gravis.
Topics: Adult; Female; Humans; Male; Middle Aged; Myasthenia Gravis; Precipitating Factors; Takotsubo Cardiomyopathy
PubMed: 31718587
DOI: 10.1186/s12883-019-1523-z