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Brain and Behavior Feb 2021We reviewed the psychotic symptoms of anti-NMDA receptor encephalitis (NMDARE) to differentiate its presentation from those found in a primary psychiatric disorder. We...
OBJECTIVE
We reviewed the psychotic symptoms of anti-NMDA receptor encephalitis (NMDARE) to differentiate its presentation from those found in a primary psychiatric disorder. We hypothesized that the cycloid psychosis (CP) phenotype would be a frequent clinical presentation in the psychiatric phase of NMDARE.
METHOD
A systematic literature review in PubMed of all case reports published on NMDARE was performed from database inception to March 2020. We included all cases where psychotic symptoms were reported and whose diagnoses were confirmed by the presence of anti-NMDAR antibodies in the cerebrospinal fluid (CSF). An email including a short test (CP phenotype, Perris and Brockington's criteria) was sent to all case report authors asking them to describe the psychotic symptoms.
RESULTS
We identified 335 case reports fulfilling our criteria, and the authors of 200 replied. Our analyses were based exclusively on those answers and data extracted from the articles. Median patient age was 25 years (+-11.4), 81% were female, and 39% had an ovarian teratoma. A complete CP phenotype was identified in 175 patients (87%). These were acute psychotic episodes with a sudden onset and a fluctuating clinical pattern mostly characterized by confusion (97%), delusions (75%), hallucinations (69%), motility disturbances (87%), and mood oscillations (80%).
CONCLUSION
The complete CP phenotype was frequently the expression of psychotic symptoms in NMDARE. We suggest that patients with a first psychotic episode who initially exhibit the CP phenotype should undergo CSF analysis to determine whether antibodies against neuronal cell surface or synaptic receptors are present to rule out a possible diagnosis of autoimmune encephalitis.
Topics: Adult; Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Female; Hashimoto Disease; Humans; Psychotic Disorders; Receptors, N-Methyl-D-Aspartate; Teratoma
PubMed: 33270360
DOI: 10.1002/brb3.1980 -
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 -
Tremor and Other Hyperkinetic Movements... Jul 2020Chorea consists of involuntary movements affecting the limbs, trunk, neck or face, that can move from one body part to another. Chorea is conceptualized as being...
BACKGROUND
Chorea consists of involuntary movements affecting the limbs, trunk, neck or face, that can move from one body part to another. Chorea is conceptualized as being "primary" when it is attributed to Huntington's disease (HD) or other genetic etiologies, or "secondary" when it is related to infectious, pharmacologic, metabolic, autoimmune disorders, or paraneoplastic syndromes. The mainstay of the secondary chorea management is treating the underlying causative disorder; here we review the literature regarding secondary chorea. We also discuss the management of several non-HD genetic diseases in which chorea can be a feature, where metabolic targets may be amenable to intervention and chorea reduction.
METHODS
A PubMed literature search was performed for articles relating to chorea and its medical and surgical management. We reviewed the articles and cross-references of pertinent articles to assess the current clinical practice, expert opinion, and evidence-based medicine to synthesize recommendations for the management of secondary chorea.
RESULTS
There are very few double-blind randomized controlled trials assessing chorea treatments regardless of etiology. Most recommendations are based on small open-label studies, case reports, and expert opinion.
DISCUSSION
Treatment of secondary chorea is currently based on expert opinion, clinical experience, and small case studies, with limited evidence-based medical data. When chorea is secondary to an underlying infection, medication, metabolic abnormality, autoimmune process, or paraneoplastic illness, the movements typically resolve following treatment of the underlying disease. Tardive dyskinesia is most rigorously studied secondary chorea with the best evidence-based medicine treatment guidelines recommending the use of pre-synaptic dopamine-depleting agents. Even though there is an insufficient pool of EBM, small clinical trials, case reports, and expert opinion are valuable for guiding treatment and improving the quality of life for patients with chorea.
HIGHLIGHTS
There is a dearth of well-controlled studies regarding the treatment of chorea. Expert opinion and clinical experiences are fundamental in guiding chorea management and determining successful treatment. In general, secondary chorea improves with treating the underlying medical abnormality; treatments include antibiotics, antivirals, immunosuppression, dopamine depleting agents, chelation, and supportive care.
Topics: Chorea; Humans; Tardive Dyskinesia
PubMed: 32775036
DOI: 10.5334/tohm.351 -
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 -
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 -
Medicine Jun 2020The pathogenesis of myasthenia gravis (MG) has strong connection with thymic abnormalities. Thymic hyperplasia or thymoma can be found with most patients. Thymectomy is... (Comparative Study)
Comparative Study
Effectiveness and safety of thymectomy plus prednisone compares with prednisone monotherapy for the treatment of non-thymomatous Myasthenia Gravis: Protocol for a systematic review.
BACKGROUND
The pathogenesis of myasthenia gravis (MG) has strong connection with thymic abnormalities. Thymic hyperplasia or thymoma can be found with most patients. Thymectomy is currently one of the regular treatment in clinic, which is, however, still controversial for non-thymomatous MG. This research will assess the effectiveness and safety of thymectomy plus prednisone compared to prednisone monotherapy for the treatment of non-thymomatous MG systematically.
METHODS
According to eligibility and ineligibility criteria, 8 databases, including PubMed, EMBASE, the Web of Science, the Cochrane Library, China National Knowledge Infrastructure (CNKI), Wan-fang Database, Chinese Biomedical Literature Database (CBM), China Science and Technology Journal Database (CSTJ), will be searched to gather the up-to-standard articles from September 2000 to September 2025. Inclusion criteria are as follows: randomized controlled trials of thymectomy plus prednisone for the treatment of non-thymomatous MG. The quantitative myasthenia gravis score (QMG) and the dose of prednisone required will be accepted as the main outcomes. Data synthesis, subgroup analysis, sensitivity analysis, and meta-regression analysis will be conducted using RevMan 5.3 software. We will use Egger or Begg test to evaluate symmetry on a funnel plot which is made to assess reporting bias, and use trial sequential analysis (TSA) to exclude the probability of false positives.
RESULTS
This systematic review will measure the QMG and the dose of prednisone required, the myasthenia gravis activities of daily living scale scores (MG-ADL), treatment-associated complications, incidence of myasthenic crisis and other aspects to comprehensively assess the clinical benefits of thymectomy plus prednisone for MG patients without thymoma.
CONCLUSION
The conclusion of this study will achieve convincing evidence to evaluate the effectiveness and safety of thymectomy plus prednisone for the treatment of non-thymomatous MG.
PROSPERO REGISTRATION NUMBER
CRD 42020167735.
Topics: Anti-Inflammatory Agents; Combined Modality Therapy; Humans; Myasthenia Gravis; Prednisone; Thymectomy; Treatment Outcome
PubMed: 32569233
DOI: 10.1097/MD.0000000000020832 -
Hematology/oncology and Stem Cell... Jun 2021Neurological complications occur at a high frequency after hematopoietic cell transplantation (HCT). However, an absence is noted in the published literature as regards... (Meta-Analysis)
Meta-Analysis
OBJECTIVE/BACKGROUND
Neurological complications occur at a high frequency after hematopoietic cell transplantation (HCT). However, an absence is noted in the published literature as regards the quantification of the exact burden and the outcomes thereof. In this systematic review, we endeavored to detail if the recipients of HCT developed any noninfectious neurological events/complications.
METHODS
According to the PICO criteria, medical literature was searched. Complications that were evaluated included: stroke, peripheral neuropathy, myasthenia gravis, seizures, and posterior reversible encephalopathy syndrome. After strictly defining relevant variables and parameters, data from 173 eligible articles were then extracted accordingly, from the full text for each, for quantitative analysis; additionally, two American Society of Hematology conference abstracts were also subject to data extraction.
RESULTS
As is evident from the results of the data analysis, an increased frequency of these complications was seen in the HCT recipient population in comparison to the general population. The relative risk ranged from 1.33× to 142× depending on the complication studied.
CONCLUSION
These findings demonstrate that the recipients of HCT had a significantly higher risk of neurological complications and that their early recognition can enhance the monitoring of HCT survivors for the early developmental signs of neurological toxicity. This would facilitate timely interventions, thus ensuring a better quality of life.
Topics: Animals; Hematopoietic Stem Cell Transplantation; Humans; Myasthenia Gravis; Peripheral Nervous System Diseases; Posterior Leukoencephalopathy Syndrome; Seizures; Stroke
PubMed: 32516577
DOI: 10.1016/j.hemonc.2020.05.006 -
Neurology(R) Neuroimmunology &... May 2020To describe the main syndrome and clinical course in a large cohort of patients with anti-Ri-associated paraneoplastic neurologic syndrome (Ri-PNS).
OBJECTIVE
To describe the main syndrome and clinical course in a large cohort of patients with anti-Ri-associated paraneoplastic neurologic syndrome (Ri-PNS).
METHODS
Twenty-year retrospective nationwide study and systematic review of the literature.
RESULTS
Thirty-six patients with complete clinical information were identified (median age 66 years, range: 47-87 years). In this French cohort, the majority were women (78%). At onset, 4 main patterns were observed: cerebellar syndrome (39%), isolated tremor (24%), oculomotor disturbances (17%), and other symptoms (19%). Course was multistep for 78% of cases. At the time the disease reached the plateau phase (median 12 weeks, range: 1-64 weeks; 28% >3 months), 24 (67%) showed an overt cerebellar syndrome, which was isolated in 3 patients, and was most frequently (21/24 cases) part of a multisystem neurologic disease. Patients manifested a variety of movement disorders, including myoclonus (33%), dystonia (17%), either cervical or oromandibular, and parkinsonism (17%). Most patients had cancer (92%), mainly breast cancer (n = 22). Misdiagnoses concerned 22% of patients (n = 8) and included atypical parkinsonism (n = 2), MS (n = 2), Bickerstaff encephalitis (n = 1), hyperekplexia (n = 1), vestibular neuritis (n = 1), and functional neurologic disorder (n = 1). Survival at 12 months was 73% (95% CI [0.54-0.85]), at 24 months 62% (95% CI [0.41-0.78]), and at 36 months 47% (95% CI [0.25-0.65]). There was no major clinical difference between cases retrieved from the systematic review of the literature (n = 55) and the French cohort.
CONCLUSIONS
Ri-PNS is a multisystem neurologic syndrome with prominent cerebellum/brainstem involvement. Opsoclonus-myoclonus is less common than expected, and the disorder can mimic neurodegenerative diseases.
Topics: Aged; Aged, 80 and over; Female; France; Humans; Male; Middle Aged; Movement Disorders; Nerve Tissue Proteins; Neuro-Oncological Ventral Antigen; Paraneoplastic Cerebellar Degeneration; Paraneoplastic Syndromes, Nervous System; RNA-Binding Proteins; Retrospective Studies
PubMed: 32170042
DOI: 10.1212/NXI.0000000000000699 -
Neuroepidemiology 2020Myasthenia gravis (MG) is an autoimmune disease whose period of typical onset is around 20-40 years (i.e., early onset), thus in the peak of working age, or around 60-80... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Myasthenia gravis (MG) is an autoimmune disease whose period of typical onset is around 20-40 years (i.e., early onset), thus in the peak of working age, or around 60-80 years (i.e., late onset). However, the information on work-related issues and employment status are sparse and not systematically reported. Therefore, we performed a systematic literature review with meta-analysis to address the employment status of MG patients.
METHODS
We searched for papers reporting employment status on participants with MG published between January 2000 and May 2019. Information on employment was extracted. Random-effects models were used to produce meta-analytic estimates for the proportion of employed patients.
RESULTS
In total, 1,045 records were retrieved, of which 19 fitted the inclusion criteria. In total, 3,600 participants (average age 47.5, range 35-60) were included in the studies and 1,579 of them were employed. The proportion of employed patients varied from 28 to 82%, with an extreme heterogeneity between studies. Overall, the pooled proportion of workers was 50% (95% CI 41-60%). Subgroup analyses suggested a possible, although not significant, higher proportion of workers among women, younger participants, those with a higher level of education, shorter MG duration, and less frequently thymectomized, whereas a lower proportion was observed among those with generalized, bulbar, and respiratory symptoms.
CONCLUSIONS
The results of our meta-analysis show that the percentage of employment is considerably low if we take into account that the mean age of MG patients involved in the included studies was around 48 years, thus in peak of working life. Therefore, it is important to understand what kind of influence MG exerts on work dynamics.
Topics: Adult; Employment; Female; Humans; Male; Middle Aged; Myasthenia Gravis
PubMed: 32097937
DOI: 10.1159/000506310