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Journal of Neurology, Neurosurgery, and... Oct 2017Pseudobulbar affect (PBA) is prevalent in amyotrophic lateral sclerosis (ALS), but there is limited information on its associations and course. (Observational Study)
Observational Study
BACKGROUND
Pseudobulbar affect (PBA) is prevalent in amyotrophic lateral sclerosis (ALS), but there is limited information on its associations and course.
OBJECTIVES
Explore prevalence, associations, course and manifestations of PBA in outpatient cohort of patients with ALS and examine its relationship to depression.
METHODS
Self-reported measures of PBA and depression (Center for Neurologic Study-Lability Scale (CNS-LS) and Patient Health Questionnaire (PHQ-9), respectively) were obtained from consecutive patients with ALS using tablet devices in waiting rooms (Knowledge Program).
RESULTS
PBA (CNS-LS ≥13) was seen in 209/735 patients (28.4%). PBA was associated with bulbar onset and dysfunction, upper motor neuron dysfunction, cognitive impairment, depression and lower quality of life. A multivariable model that included lower bulbar and gross motor subscores, female gender, younger age and shorter duration of disease predicted PBA with 74% accuracy. CNS-LS scores increased only slowly with time. Women with PBA reported more crying than men. Crying (but not laughter) correlated with depression, and crying was associated with poorer quality of life. Exploratory factor analysis of pooled questions of CNS-LS and PHQ-9 identified three underlying factors (laughter, crying and depression) loaded on appropriate questions of the respective instruments.
CONCLUSION
This study identifies associations of PBA and additionally finds PBA (especially crying-predominant PBA) more prevalent in women with ALS. Although the two self-report instruments (CNS-LS and PHQ-9) discriminate well between PBA and depression, there is significant overlap between depression and crying in PBA. Studies of PBA should stratify for gender, examine crying and laughter as separate outcomes and adjust for depression.
Topics: Age Factors; Amyotrophic Lateral Sclerosis; Crying; Depression; Female; Humans; Laughter; Male; Middle Aged; Prevalence; Pseudobulbar Palsy; Quality of Life; Sex Factors; Surveys and Questionnaires
PubMed: 28572273
DOI: 10.1136/jnnp-2017-315622 -
Diagnostics (Basel, Switzerland) Nov 2023The term "Osmotic Demyelination Syndrome" (ODS) is synonymous with central pontine myelinolysis (CPM), denoting a condition characterised by brain damage, particularly...
The term "Osmotic Demyelination Syndrome" (ODS) is synonymous with central pontine myelinolysis (CPM), denoting a condition characterised by brain damage, particularly affecting the white matter tracts of the pontine region. This damage arises due to the rapid correction of metabolic imbalances, primarily cases of hyponatremia. Noteworthy triggers encompass severe burns, liver transplantations, anorexia nervosa, hyperemesis gravidarum, and hyperglycaemia, all linked to the development of CPM. Clinical manifestations encompass a spectrum of signs and symptoms, including dysphagia, dysarthria, spastic quadriparesis, pseudobulbar paralysis, ataxia, lethargy, tremors, disorientation, catatonia, and, in severe instances, locked-in syndrome and coma. A recent case involving a 45-year-old woman illustrates these complexities. Upon admission to the Medicine Intensive Care Unit, she presented with symptoms indicative of diminished responsiveness and bilateral weakness in the upper and lower limbs. Of significance, the patient had a pre-existing medical history of hyperthyroidism. Extensive diagnostic investigations were undertaken, revealing compelling evidence of profound hyponatremia through blood analyses. Furthermore, magnetic resonance imaging (MRI) was performed, unveiling conspicuous areas of abnormal hyperintensity located in the central pons, intriguingly accompanied by spared peripheral regions. These radiological findings align with the characteristic pattern associated with osmotic demyelination syndrome, illuminating the underlying pathology.
PubMed: 37958289
DOI: 10.3390/diagnostics13213393 -
Molecular Medicine Reports Mar 2022Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy described as a syndrome of postural instability, supranuclear vertical gaze palsy, dysarthria,...
Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy described as a syndrome of postural instability, supranuclear vertical gaze palsy, dysarthria, dystonic rigidity of the neck and trunk, dementia, and pseudobulbar palsy. The clinical diagnosis of PSP is often difficult because there are no established biomarkers, and diagnosis is currently based on clinical and imaging findings. Furthermore, the etiology and pathogenesis of PSP remain unknown. Dysregulation of microRNAs (miRNAs/miRs) has been reported to serve an important role in neurodegenerative diseases. However, the miRNA profiles of patients with PSP are rarely reported. The present study aimed to examine cerebrospinal fluid miRNAs, which are considered to be more sensitive indicators of changes in the brain, to elucidate the pathophysiology of PSP and to establish specific biomarkers for diagnosis. The present study used a microarray chip containing 2,632 miRNAs to examine cerebrospinal fluid miRNA expression levels in 11 patients with PSP aged 68‑82 years. A total of 8 age‑ and sex‑matched controls were also included. A total of 38 miRNAs were significantly upregulated and one miRNA was significantly downregulated in the cerebrospinal fluid of patients with PSP. The patients were divided into two groups based on disease stage (early onset and advanced), and changes in miRNA expression were examined. The miRNAs that were most significantly upregulated or downregulated in the early onset group were miR‑204‑3p, miR‑873‑3p and miR‑6840‑5p. The target genes of these miRNAs were associated with molecules related to the ubiquitin‑proteasome system and autophagy pathway. Furthermore, these miRNAs were found to target genes that have been reported to have epigenetic changes following an epigenome‑wide association study of brain tissues of patients with PSP. This suggested that these miRNAs and genes may have some involvement in the pathogenesis of PSP. However, the sample size of the present study was small; therefore, a greater number of patients with PSP should be examined in future studies.
Topics: Aged; Aged, 80 and over; Biomarkers; Female; Gene Expression Profiling; Gene Expression Regulation; Humans; Male; MicroRNAs; Middle Aged; Sensitivity and Specificity; Supranuclear Palsy, Progressive; Syndrome
PubMed: 35039873
DOI: 10.3892/mmr.2022.12604 -
The American Journal of Managed Care Dec 2017Pseudobulbar affect (PBA), despite its prevalence and distinctive symptoms, is widely underrecognized and undertreated. It is characterized by uncontrollable laughing or... (Review)
Review
Pseudobulbar affect (PBA), despite its prevalence and distinctive symptoms, is widely underrecognized and undertreated. It is characterized by uncontrollable laughing or crying that can occur in an exaggerated manner or inappropriately to a given situation or stimuli. PBA is thought to center around preexisting neurological conditions, which include Parkinson disease, multiple sclerosis, amyotrophic lateral sclerosis, Alzheimer disease, traumatic brain injury, and stroke. The PBA Registry Series trial was created to measure the prevalence of PBA among patients with these underlying neurological conditions. Through greater awareness, recognition, and diagnosis, treatment for patients with PBA can be improved.
Topics: Alzheimer Disease; Amyotrophic Lateral Sclerosis; Female; Humans; Male; Middle Aged; Nervous System Diseases; Parkinson Disease; Prevalence; Pseudobulbar Palsy; Quality of Life; Risk Assessment; Severity of Illness Index; United States
PubMed: 29297656
DOI: No ID Found -
Annals of Palliative Medicine Jul 2022The efficacy of acupuncture in the treatment of dysphagia caused by pseudobulbar paralysis after stroke is lack of evidence-based medicine. Our objective was to... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The efficacy of acupuncture in the treatment of dysphagia caused by pseudobulbar paralysis after stroke is lack of evidence-based medicine. Our objective was to synthesize the efficacy of acupuncture in treating dysphagia caused by pseudobulbar paralysis after stroke.
METHODS
A comprehensive literature search was performed in 9 databases [PubMed, Web of Science, Embase, Cochrane, Chinese BioMedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), WanFang Data, Chinese Science and Technology Periodicals database (VIP), and Open Grey online database] to screen eligible randomized controlled studies that evaluated the effect of acupuncture in dysphagia caused by pseudobulbar paralysis after stroke. The search time limit is from establishing the database to October 1, 2020. The random-effects model was used to calculate the significant effect size.
RESULTS
A total of 7 studies comprising 637 participants were included in our meta-analysis. The results showed that compared with rehabilitation, acupuncture had a significant effect on improving dysphagia caused by pseudobulbar paralysis after stroke [the significant effective size: risk ratio (RR)sig =1.51; 95% confidence interval (CI): 1.30-1.75; I2=0%]. In the subgroup analyses, the RRsig of acupuncture + rehabilitation vs. rehabilitation was 1.56 (95% CI: 1.30-1.87; I2=0%), and the RRsig of acupuncture vs. rehabilitation was 1.38 (95% CI: 1.08-1.76; I2=0.8%).
DISCUSSION
Acupuncture can be used as an effective treatment for dysphagia caused by pseudobulbar paralysis after stroke. Acupuncture combined with rehabilitation therapy has better effects.
Topics: Acupuncture Therapy; Deglutition Disorders; Evidence-Based Medicine; Humans; Pseudobulbar Palsy; Stroke
PubMed: 35400158
DOI: 10.21037/apm-21-3551 -
Ugeskrift For Laeger Jul 2022Sudden onset anarthria and dysphagia without lateralised neurologic symptoms should prompt an investigation for pseudobulbar palsy, either due to bilateral vascular...
Sudden onset anarthria and dysphagia without lateralised neurologic symptoms should prompt an investigation for pseudobulbar palsy, either due to bilateral vascular lesions of the corticobulbar tracts or, less frequently, Foix-Chavany-Marie Syndrome (FCMS). Here, bilateral damage to the frontal opercular cortex leads to loss of voluntary control of muscles supplied by cranial nerves V, VII, IX, X, XI, and XII. This case report presents a rare case of FCMS on the background of traumatic cerebral lesions following a bicycle incident.
Topics: Deglutition Disorders; Dysarthria; Facial Paralysis; Humans; Syndrome
PubMed: 35959814
DOI: No ID Found -
Revue Des Maladies Respiratoires Oct 2021Complications following COVID-19 are starting to emerge; neurological disorders are already described in the literature.
INTRODUCTION
Complications following COVID-19 are starting to emerge; neurological disorders are already described in the literature.
CASE REPORT
This case is about a 20-year old male with a severe COVID-19, hospitalized in a Reanimation and Intensive Care Unit with an Acute Respiratory Distress Syndrome, thromboembolic complication and secondary bacterial infection. This patient had a non-specific neurological disorder with a pseudobulbar palsy, (MRI, ENMG and lumbar puncture were normal), associated 4 months later with persistent left shoulder motor deficit and respiratory failure. Respiratory and neurological check-up led to a diagnosis of the Parsonage-Turner syndrome or neuralgic amyotrophy affecting C5-C6 nerve roots, the lateral pectoral and phrenic nerves at the origin of the scapular belt, amyotrophy and left diaphragm paralysis.
CONCLUSIONS
This case shows that persistant dyspnoea after COVID 19 infection should lead to a search for a diaphragmatic cause which is not always the result of Reanimation Neuropathy but may also indicate a neuralgic amyotrophy. It is the fourth case of neuralgic amyotrophy following COVID-19. This brings the medical community to consider the risk of diaphragm paralysis apart from critical illness polyneuropathy. Respiratory muscle evaluation and diaphragmatic ultrasound should be considered in case of persistent dyspnoea.
Topics: Brachial Plexus Neuritis; COVID-19; Humans; Male; Phrenic Nerve; Respiratory Paralysis; SARS-CoV-2; Young Adult
PubMed: 34325956
DOI: 10.1016/j.rmr.2021.06.004 -
Experimental and Therapeutic Medicine Oct 2017The present study aimed to investigate the efficacy of endoscopy in the treatment of post-shunt placement for slit ventricle syndrome (SVS). Endoscopic surgery was...
The present study aimed to investigate the efficacy of endoscopy in the treatment of post-shunt placement for slit ventricle syndrome (SVS). Endoscopic surgery was performed on 18 patients with SVS between October 2004 and December 2012. Sex, age, causes of the hydrocephalus, ventricular size and imaging data were collected and analyzed. All patients were divided into two groups according to ventricular size and underwent endoscopic surgeries, including endoscopic third ventriculostomy (ETV), endoscopic aqueductoplasty and cystocisternostomy. All treated patients were observed postoperatively for a period of 2 to 3 weeks, and outpatient follow-up was subsequently scheduled for >12 months. Clinical results, including catheter adherence, shunt removal and complications, were analyzed during the follow-up period. The success rate of endoscopic surgery was indicated to be 82.7%. Syndromes caused by aqueductal stenosis in 15 patients who underwent ETV were relieved; however, syndromes in the 3 patients with cerebral cysticercosis, suprasellar arachnoid cysts, pinea larea glioma and communicating hydrocephalus, respectively, were not relieved and underwent shunt placement again. Brain parenchyma, choroid plexus and ependymal tissue were the predominant causes for catheter obstruction and the obstruction rate was indicated to be 77.8% (14/18). Complications, such as pseudobulbar paralysis, infection and intraventricular hemorrhage arose in 3 patients. The present study indicates that endoscopic treatments are effective and ETV may be considered as a recommended option in the treatment of post-shunt placement SVS in hydrocephalus patients.
PubMed: 29042922
DOI: 10.3892/etm.2017.4973 -
JAMA Internal Medicine Feb 2019In 2010, the US Food and Drug Administration (FDA) approved a combination of dextromethorphan hydrobromide and quinidine sulfate for the treatment of pseudobulbar affect...
Assessment of Use of Combined Dextromethorphan and Quinidine in Patients With Dementia or Parkinson Disease After US Food and Drug Administration Approval for Pseudobulbar Affect.
IMPORTANCE
In 2010, the US Food and Drug Administration (FDA) approved a combination of dextromethorphan hydrobromide and quinidine sulfate for the treatment of pseudobulbar affect after studies in patients with amyotrophic lateral sclerosis (ALS) or multiple sclerosis (MS). This medication, however, may be commonly prescribed in patients with dementia and/or Parkinson disease (PD).
OBJECTIVE
To investigate the prescribing patterns of dextromethorphan-quinidine, including trends in associated costs.
DESIGN, SETTING, AND PARTICIPANTS
This population-based cohort study of patients prescribed dextromethorphan-quinidine used data from 2 commercial insurance databases, Optum Clinformatics Data Mart and Truven Health MarketScan. The Medicare Part D Prescription Drug Program data set was used to evaluate numbers of prescriptions and total reported spending by the Centers for Medicare & Medicaid Services. Patients were included if they were prescribed dextromethorphan-quinidine from October 29, 2010, when the drug was approved, through March 1, 2017, for Optum and December 31, 2015, for Truven. Data were analyzed from December 1, 2017, through August 1, 2018.
MAIN OUTCOMES AND MEASURES
The proportion of patients prescribed dextromethorphan-quinidine with a diagnosis of MS, ALS, or dementia and/or PD, as well as the number of patients with a history of heart failure (a contraindication for the drug).
RESULTS
In the commercial health care databases, 12 858 patients filled a prescription for dextromethorphan-quinidine during the study period. Mean (SD) age was 66.0 (18.5) years, 66.7% were women, and 13.3% had a history of heart failure. Combining results from both databases, few patients had a diagnosis of MS (8.4%) or ALS (6.8%); most (57.0%) had a diagnosis of dementia and/or PD. In the Medicare Part D database, the number of patients prescribed dextromethorphan-quinidine increased 15.3-fold, from 3296 in 2011 to 50 402 in 2016. Reported spending by Centers for Medicare & Medicaid Services on this medication increased from $3.9 million in 2011 to $200.4 million in 2016.
CONCLUSIONS AND RELEVANCE
Despite approval by the FDA for pseudobulbar affect based on studies of patients with ALS or MS, dextromethorphan-quinidine appears to be primarily prescribed for patients with dementia and/or PD.
Topics: Aged; Amyotrophic Lateral Sclerosis; Dementia; Dextromethorphan; Drug Combinations; Excitatory Amino Acid Antagonists; Female; Humans; Male; Middle Aged; Multiple Sclerosis; Parkinson Disease; Pseudobulbar Palsy; Quinidine; United States; United States Food and Drug Administration
PubMed: 30615021
DOI: 10.1001/jamainternmed.2018.6112