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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 -
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 -
BioMed Research International 2021To investigate the etiology, clinical as well as neuroimaging characteristics, and outcomes after proper treatment in a series of 18 patients with osmotic demyelination...
OBJECTIVE
To investigate the etiology, clinical as well as neuroimaging characteristics, and outcomes after proper treatment in a series of 18 patients with osmotic demyelination syndrome.
METHODS
Medical records, including video records, of 18 patients with osmotic demyelination syndrome were retrospectively examined. Demographic and clinical information, imaging results, plans of management, and outcomes during the follow-up period were collected and analyzed.
RESULTS
Eighteen patients, including 10 males and 8 females, were included in the present study. The mean age at diagnosis of CNS insult was 47.4 ± 13.3 years (ranged from 30 to 78 years). Etiologies included rapidly corrected hyponatremia (50%), alcoholism (27.8%), and others. Neurological manifestations included encephalopathy (61.1%), dysphonia (50%), extrapyramidal symptoms (38.9%), and seizures (22.2%). Neuroimaging results showed that 6 patients (33.3%) had central pontine myelinolysis, 5 (27.8%) had extrapontine myelinolysis, and 7 (38.9%) had both. After treatment, 12 patients showed improvement and the other 6 did not. Among these patients, those who showed symptoms of encephalopathy had a favorable outcome. The majority of those who presented with mental retardation, seizures, and no other symptoms recovered better than their counterparts who had other symptoms. Nine out of 11 patients with pseudobulbar paralysis and/or extrapyramidal symptoms showed improvement, but the other 2 did not show improvement. Five patients who did not improve after treatment during admission were followed up for 1-3 months with rehabilitation training recommended, and it was found that 3 showed significant improvement after training, and the other 2 did not respond to this training.
CONCLUSIONS
Osmotic demyelination syndrome is a complex disease entity due to a variety of etiologies, manifesting with symptoms involving diverse systems of the brain. Early identification and removal/correction of conditions leading to osmotic demyelination syndrome are the key to prevent and/or manage this disease.
Topics: Adult; Aged; Brain; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Myelinolysis, Central Pontine; Neuroimaging; Osmosis; Prognosis; Retrospective Studies; Treatment Outcome
PubMed: 34136577
DOI: 10.1155/2021/9944632 -
Frontiers in Neurology 2022Up-to-date, accurate information on the disease burden of motor neuron disease (MND) is the cornerstone for evidence-based resource allocation and healthcare planning....
Up-to-date, accurate information on the disease burden of motor neuron disease (MND) is the cornerstone for evidence-based resource allocation and healthcare planning. We aimed to estimate the burden of MND globally from 1990 to 2019, as part of the Global Burden of Disease, Injuries and Risk Factor (GBD) study. Amyotrophic lateral sclerosis, progressive muscular atrophy, primary lateral sclerosis, pseudobulbar palsy, spinal muscular atrophy and hereditary spastic paraplegia- were included for analysis as MNDs. We measured age-standardized incidence, prevalence, death, and disability-adjusted life-years (DALYs) in 204 countries and territories worldwide from 1990 to 2019 using spatial Bayesian analyses. The effects of age, sex, and the sociodemographic index (measures of income per capita, education, and fertility) on incidence, prevalence, death, and disability-adjusted life-years due to MNDs were explored. According to 2019 GBD estimates, there were ~268,673 [95% uncertainty interval (UI), 213,893-310,663] prevalent cases and 63,700 (95% UI, 57,295-71,343) incident cases of MND worldwide. In 2019, MND caused 1,034,606 (95% UI, 979,910-1,085,401) DALYs and 39,081 (95% UI, 36,566-41,129) deaths worldwide. The age-standardized rates of prevalence, incidence, death, and DALYs for MNDs in 2019 were 3.37 (95% UI, 2.9-3.87) per 100,000 people, 0.79 (95% UI, 0.72-0.88) per 100,000 people, 0.48 (95% UI, 0.45-0.51) per 100,000 people, and 12.66 (95% UI, 11.98-13.29) per 100,000 people, respectively. The global prevalence and deaths due to MND in 2019 were increased (1.91% [95% UI, 0.61-3.42] and 12.39% [95% UI, 5.81-19.27], respectively) compared to 1990, without significant change in incidence. More than half of the prevalence and deaths due to MND occurred in three high-income regions (North America, Western Europe, and Australasia). In most cases, the prevalence, incidence, and DALYs of MNDs were high in regions with high sociodemographic index; however, in high-income East Asia, these were relatively low compared to similar sociodemographic index groups elsewhere. The burden of MND increased between 1990 and 2019. Its expected increase in the future highlights the importance of global and national healthcare planning using more objective evidence. Geographical heterogeneity in the MND burden might suggest the influences of sociodemographic status and genetic background in various regions.
PubMed: 35528743
DOI: 10.3389/fneur.2022.864339 -
Child's Nervous System : ChNS :... Jun 2020Cerebellar mutism (CM), pseudobulbar palsy, posterior fossa syndrome (PFS), and cerebellar cognitive affective syndrome (CCAS) are terms that have been used, sometimes... (Review)
Review
Pediatric post-operative cerebellar mutism syndrome, cerebellar cognitive affective syndrome, and posterior fossa syndrome: historical review and proposed resolution to guide future study.
BACKGROUND
Cerebellar mutism (CM), pseudobulbar palsy, posterior fossa syndrome (PFS), and cerebellar cognitive affective syndrome (CCAS) are terms that have been used, sometimes interchangeably, to refer to the complex neurological constellation that occurs following surgical removal of cerebellar and fourth ventricular tumors, mostly in children, but also sometimes in adults.
METHODS
This paper reviews the origins of what is now regarded as pediatric post-operative cerebellar mutism, the cerebellar cognitive affective syndrome, and the neurological manifestations of injury to or disruption of brainstem and cerebellar structures. It examines the specific components of each of these phenomena in the context of the evolving understanding of the role of the cerebellum in nervous system function.
RESULTS
Children undergoing surgical management of tumors in the posterior cranial fossa are at risk of experiencing cranial neuropathies, corticospinal damage, cerebellar ataxia and related motor disorders, neuropsychiatric and cognitive changes, and in some patients, mutism. These clinical presentations are differentiated from each other and examined in the context of the relevant anatomical structures and distributed neural circuits. The term posterior fossa syndrome is not sufficiently helpful in distinguishing the different elements of the clinical phenomena from each other, and because of this lack of precision and specificity, there is consensus among investigators in the international Posterior Fossa Society that the designation be retired.
CONCLUSIONS
Using contemporary brain imaging methods and guided by careful clinical observation and meticulous definition of clinical phenomenology, it is now feasible to perform detailed structure function correlation analyses to achieve two critical goals in the care of children with tumors in the posterior cranial fossa. The first goal is to identify and understand the neural circuits responsible for the different manifestations-arousal, cranial neuropathies, long tract signs, cerebellar motor syndrome, cerebellar vestibular syndrome, cerebellar cognitive affective syndrome including emotional dyscontrol, and mutism. The second goal is to transform this knowledge into practical clinical intervention, preventing the complications inherent in the necessary surgery whenever possible, and develop new approaches to treatment with methods including brain modulation targeting interconnected nodes of the damaged neural circuits.
Topics: Adult; Cerebellar Diseases; Cerebellar Neoplasms; Cerebellum; Child; Cognition; Cranial Fossa, Posterior; Humans; Mutism; Postoperative Complications
PubMed: 31240391
DOI: 10.1007/s00381-019-04253-6 -
European Journal of Case Reports in... 2021Foix-Chavany-Marie syndrome (FCMS) is a type of pseudobulbar palsy that affects facio-pharyngo-glosso-masticatory muscles.
INTRODUCTION
Foix-Chavany-Marie syndrome (FCMS) is a type of pseudobulbar palsy that affects facio-pharyngo-glosso-masticatory muscles.
MATERIALS AND METHODS
A 62-year-old man was admitted to the emergency department after 9 hours of acute dysarthria and dysphagia. MRI showed restricted diffusion in the right operculum on diffusion-weighted imaging (DWI). No thrombolytic therapy was given. The patient had a history of mechanical aortic valve replacement under anticoagulation with a vitamin K antagonist. Work-up demonstrated suboptimal levels of INR. Due to severe dysphagia during hospitalization, a percutaneous endoscopic gastrostomy (PEG) was performed.
RESULTS
The patient was discharged 5 days later, with a modified Rankin scale (mRs) score of 3, and secondary stroke prevention. He had achieved an excellent functional outcome (mRs 1) at 6-month follow-up.
CONCLUSION
Our patient had a satisfactory recovery due to prompt diagnosis, secondary stroke prevention, and compliance with treatment.
LEARNING POINTS
In the presence of acute dysarthria and dysphagia, Foix-Chavany-Marie syndrome (FCMS) should be considered.FCMS may occur in the presence of unilateral opercular stroke.Swallowing and speech therapy play an essential role in rehabilitation after the acute setting.
PubMed: 33987126
DOI: 10.12890/2021_002462 -
BMJ Case Reports Oct 2021We present a case of a 73-year-old man who developed sudden onset dysarthria, dysphagia and bilateral facial weakness with automato-voluntary dissociation, which...
We present a case of a 73-year-old man who developed sudden onset dysarthria, dysphagia and bilateral facial weakness with automato-voluntary dissociation, which deteriorated rapidly to anarthria and aphonia within a few days. MRI scan of the head showed acute infarct in right internal capsule and an old infarct in the left corona radiata while the rest of the investigations were normal. Based on these findings, diagnosis was thought to be subopercular syndrome. He recovered significantly in a few weeks' time.
Topics: Aged; Dysarthria; Humans; Internal Capsule; Magnetic Resonance Imaging; Male; Pseudobulbar Palsy; Syndrome
PubMed: 34711624
DOI: 10.1136/bcr-2021-245613 -
Frontiers in Neurology 2019To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction. A retrospective analysis was...
To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction. A retrospective analysis was performed on 11 patients diagnosed with bilateral cerebral peduncular infarction in the Affiliated Hospital of Xuzhou Medical University from December 2014 to December 2018. Their clinical and imaging features were analyzed and summarized in combination with the relevant national and international literature. Among all the patients, there were eight cases with a history of hypertension, four cases with a history of diabetes mellitus, and four cases with a history of smoking. Conscious disturbance was observed in nine cases, quadriplegia in seven cases, pseudobulbar paralysis in three cases, and ataxia in one case. Brain magnetic resonance (MR) scans of bilateral cerebral peduncles showed patchy abnormal shadows with a hypointense signal on T1-weighted imaging (T1WI) and apparent diffusion coefficient (ADC) and hyperintense signal on T2-weighted imaging (T2WI), fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI). Computed tomography angiography (CTA) scans of head and neck showed severe stenosis or occlusion of vertebral artery, basilar artery, or posterior cerebral artery. All the patients received standardized treatment for cerebral infarction. Six patients died while five were left disabled. Bilateral cerebral peduncle infarction may be related to cerebral perfusion insufficiency caused by the stenosis or occlusion of vertebrobasilar artery and its branches. The main clinical manifestations are locked-in syndrome and persistent vegetative state. The specific imaging feature of "Mickey Mouse ear"-like infarction is associated with a poor prognosis.
PubMed: 31708855
DOI: 10.3389/fneur.2019.01107