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Cureus Nov 2022Paraneoplastic syndromes (PNS) are uncommon, distinct clinical complications of a primary tumor. Paraneoplastic cerebellar degeneration (PCD) is a PNS that is described... (Review)
Review
Paraneoplastic syndromes (PNS) are uncommon, distinct clinical complications of a primary tumor. Paraneoplastic cerebellar degeneration (PCD) is a PNS that is described as an autoimmune response targeting Purkinje cells in the cerebellum. Ovarian cancer (OC) is one of the most prevalent causes of cancer-related deaths in women. Anti-Yo is the most common onconeural antibody produced in the PCD immune response and is most typically found in ovarian and breast cancer patients. While the current literature highlights the predisposing genetic factors, diagnostic workflows, and treatment options, the pathophysiology of PCD, among other considerations, remains largely unestablished. This review aimed to systematically observe procedural solutions to facilitate an early diagnosis and improve the prognosis of patients with OC-associated PCD. To that end, we examined literature published from 01/01/2015-11/10/2022 indexed in PubMed by using the keywords "paraneoplastic, cerebellar degeneration" combined with "ovarian cancer." Inclusion criteria were met if PCD and OC diagnoses were made and if studies provided adequate patient information. After screening and assessing records for eligibility using the inclusion and exclusion criteria, 18 articles involving 102 patients were included. The typical patient observed in this sample was diagnosed with International Federation of Gynecology and Obstetrics (FIGO) Stage III, high-grade serous carcinoma. The diagnostic workup typically included a clinical evaluation for dysarthria (50%), ataxia (60%), and gait abnormalities (50%), along with multiple imaging modalities and serological findings (90%). Genetic screening for human leukocyte antigen (HLA) haplotype susceptibility for PCD and immune tolerance modulators regulation may also be recommended prior to starting treatment. Findings support the use of corticosteroids (35%) and intravenous immunoglobulin (IVIg) (40%) as viable treatment options for managing PCD in conjunction with systemic therapy for the primary malignancy. A diagnosis of PCD should be considered if a patient has had a malignancy in the past five years with the presence of explicit cerebellar symptoms. This clinical diagnosis can be further supplemented by serologic and radiologic findings. Recognizing PCD symptoms and scheduling genetic and proteomic testing may help with early diagnosis and better prognosis.
PubMed: 36483902
DOI: 10.7759/cureus.31154 -
Neurological Sciences : Official... Mar 2023Deep brain stimulation (DBS) is a common treatment for Parkinson's disease. However, the clinical efficacy of short pulse width DBS (spDBS) compared with conventional... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Deep brain stimulation (DBS) is a common treatment for Parkinson's disease. However, the clinical efficacy of short pulse width DBS (spDBS) compared with conventional DBS (cDBS) is still unknown.
OBJECTIVE
This meta-analysis investigated the effectiveness of spDBS versus cDBS in patients with PD.
METHODS
Four databases (PubMed, Cochrane, Web of Science, and Embase) were independently searched until October 2021 by two reviewers. We utilized the following scales and items: therapeutic windows (TW), efficacy threshold, side effect threshold, Movement Disorder Society-Sponsored Revision Unified Parkinson's Disease Rating Scale (MDS-UPDRS) part III off-medication score, Speech Intelligence Test (SIT), and Freezing of Gait Questionnaire (FOG-Q).
RESULTS
The analysis included seven studies with a total of 87 patients. The results indicated that spDBS significantly widened the therapeutic windows (0.99, 95% CI = 0.61 to 1.38) while increasing the threshold amplitudes of side effects (2.25, 95% CI = 1.69 to 2.81) and threshold amplitudes of effects (1.60, 95% CI = 0.84 to 2.36). There was no statistically significant difference in UPDRS part III, SIT, and FOG-Q scores between spDBS and cDBS groups, suggesting that treatment with both cDBS and spDBS may result in similar effects of improved dysarthria and gait disorders.
CONCLUSIONS
Compared with cDBS, spDBS is effective in expanding TW. Both types of deep brain stimulation resulted in improved gait disorders and speech intelligibility.
Topics: Humans; Parkinson Disease; Subthalamic Nucleus; Deep Brain Stimulation; Gait Disorders, Neurologic; Treatment Outcome
PubMed: 36383263
DOI: 10.1007/s10072-022-06484-z -
Cancers Nov 2022Awake craniotomy with direct electrical stimulation (DES) is the standard treatment for patients with gliomas in eloquent areas. Even though language is monitored... (Review)
Review
Awake craniotomy with direct electrical stimulation (DES) is the standard treatment for patients with gliomas in eloquent areas. Even though language is monitored carefully during surgery, many patients suffer from postoperative aphasia, with negative effects on their quality of life. Some perioperative factors are reported to influence postoperative language outcome. However, the influence of different intraoperative speech and language errors on language outcome is not clear. Therefore, we investigate this relation. A systematic search was performed in which 81 studies were included, reporting speech and language errors during awake craniotomy with DES and postoperative language outcomes in adult glioma patients up until 6 July 2020. The frequencies of intraoperative errors and language status were calculated. Binary logistic regressions were performed. Preoperative language deficits were a significant predictor for postoperative acute (OR = 3.42, p < 0.001) and short-term (OR = 1.95, p = 0.007) language deficits. Intraoperative anomia (OR = 2.09, p = 0.015) and intraoperative production errors (e.g., dysarthria or stuttering; OR = 2.06, p = 0.016) were significant predictors for postoperative acute language deficits. Postoperatively, the language deficits that occurred most often were production deficits and spontaneous speech deficits. To conclude, during surgery, intraoperative anomia and production errors should carry particular weight during decision-making concerning the optimal onco-functional balance for a given patient, and spontaneous speech should be monitored. Further prognostic research could facilitate intraoperative decision-making, leading to fewer or less severe postoperative language deficits and improvement of quality of life.
PubMed: 36358884
DOI: 10.3390/cancers14215466 -
Health Science Reports Nov 2022Facial palsy is a rare complication of the COVID-19 infection. Herein, we conducted a systematic review of all published cases of facial palsy post-COVID-19 infection in...
BACKGROUND AND AIMS
Facial palsy is a rare complication of the COVID-19 infection. Herein, we conducted a systematic review of all published cases of facial palsy post-COVID-19 infection in an attempt to educate the general population and medical practitioners regarding the likely occurrence of facial palsy in COVID-19 patients, its detection, effective treatment plan, and prognosis of the condition.
METHODS
We searched PubMed, Google Scholar, and Directory of Open Access Journals (DOAJ) from December 1, 2019 to September 21, 2021.
RESULTS
We included 49 studies bearing accounts of 75 cases who had facial palsy. The mean age of patients was 42.9 ± 19.59 years, with a male-to-female ratio of 8:7. The majority of the cases were reported from Brazil ( = 14), USA ( = 9), Turkey ( = 9), and Spain ( = 9). Noticeably, 30.14% of COVID-19 patients were diagnosed with Guillain-Barré syndrome. In total, 22.97% of patients complained of bilateral facial paralysis ( = 17), whereas ipsilateral paralysis was observed in 77.03% ( = 57). These were common complaints of Lagophthalmos, otalgia, facial drooping, dysarthria, and compromised forehead wrinkling. The treatment regimen mainly included the use of corticosteroids ( = 51) (69.86%), antivirals ( = 23) (31.51%), IVIG ( = 18) (24.66%), antibiotics ( = 13) (17.81%), antiretroviral ( = 9) (12.33%), and antimalarial ( = 8) (10.96%) medications. In all, 35.62% of patients ( = 26) adhered to a combination of antiviral and corticosteroid-based therapy. Positive treatment outcomes were observed in 83.58% ( = 56) of cases. In contrast, 10 patients (14.93%) showed nonsignificant recovery, out of which 3 (4.48%) died from the disease.
CONCLUSION
The association of facial palsy with COVID-19 is controversial and therefore requires further investigation and published work to confirm a causal relationship. However, physicians should not overlook the likelihood of facial palsy post-COVID-19 infection and treat it accordingly.
PubMed: 36320650
DOI: 10.1002/hsr2.887 -
Cerebrovascular Diseases (Basel,... 2023Stroke mimics are non-vascular conditions that present with acute focal neurological deficits, simulating an acute ischemic stroke. Susumber berry (SB) toxicity is a...
BACKGROUND
Stroke mimics are non-vascular conditions that present with acute focal neurological deficits, simulating an acute ischemic stroke. Susumber berry (SB) toxicity is a rare cause of stroke mimic with limited case reports available in the literature.
OBJECTIVES
We report four new cases of SB toxicity presenting as stroke mimic, and we performed a systematic review.
METHODS
MEDLINE/EMBASE/WoS were searched for "susumber berries," "susumber," or "solanum torvum."
RESULTS
531 abstracts were screened after removal of duplicates; 5 articles and 2 conference abstracts were selected describing 13 patients. A total of 17 patients who ingested SB and became ill were identified, including our 4 patients. All but one presented with acute neurologic manifestation; 16 (94%) presented with dysarthria, 16 (94%) with unstable gait, 8 (47%) with nystagmus/gaze deviation, 10 (59%) with blurry vision, and 5 (29%) with autonomic symptoms. Six (35%) required ICU admission, and 3 (18%) were intubated. Fourteen (82%) had a rapid complete recovery, and 3 were hospitalized up to 1 month.
CONCLUSIONS
SB toxicity can cause neurological symptoms that mimic an acute stroke typically with a posterior circulation symptom complex. Altered SB toxins (from post-harvest stressors or temperature changes) might stimulate muscarinic/nicotinic cholinergic receptors or inhibit acetylcholinesterase, causing gastrointestinal, neurological, and autonomic symptoms. In cases of multiple patients presenting simultaneously to the ED with stroke-like symptoms or when stroke-like symptoms fail to localize, a toxicological etiology (such as SB toxicity) should be considered.
Topics: Humans; Acetylcholinesterase; Fruit; Ischemic Stroke; Jamaica; Poisoning
PubMed: 36282075
DOI: 10.1159/000525686 -
Neuroanatomical regions associated with non-progressive dysarthria post-stroke: a systematic review.BMC Neurology Sep 2022Dysarthria is a common and persisting sequela to stroke. It can have a negative influence on psychological wellbeing, and quality of life. This systematic review aimed...
BACKGROUND
Dysarthria is a common and persisting sequela to stroke. It can have a negative influence on psychological wellbeing, and quality of life. This systematic review aimed to describe and identify the neuroanatomical regions associated with non-progressive dysarthria following stroke.
METHODS
A systematic search of PubMed, Ovid Medline, CINAHL, Cochrane, Scopus, and ScienceDirect was conducted to identify all relevant articles published in peer-reviewed journals up to December 2021. Following data extraction, the National Institutes of Health (NIH) quality assessment tools were used to evaluate the methodological quality of the included studies.
RESULTS
Out of 2186 papers found in the literature related to dysarthria post-stroke, 24 met the inclusion criteria. Eligible articles assessed 1150 post-stroke subjects. Out of them, 420 subjects had dysarthria from isolated lesions. Regarding dysarthric subjects with ischemic strokes, 153 sustained supratentorial infarctions, while 267 had infratentorial infarctions. The majority had pontine infarctions (n = 142), followed by infarctions in the corona radiata (n = 104), and the cerebellum (n = 64).
CONCLUSION
This systematic review is the first step toward establishing a neuroanatomical model of dysarthria throughout the whole brain. Our findings have many implications for clinical practice and provide a framework for implementing guidelines for early detection and management of dysarthria post-stroke.
Topics: Dysarthria; Humans; Infarction; Ischemic Stroke; Quality of Life; Stroke; United States
PubMed: 36114518
DOI: 10.1186/s12883-022-02877-x -
Therapeutic Advances in Neurological... 2022The safety and efficacy of hyperacute reperfusion therapies in childhood stroke due to focal cerebral arteriopathy (FCA) with an infectious and inflammatory component is...
The safety and efficacy of hyperacute reperfusion therapies in childhood stroke due to focal cerebral arteriopathy (FCA) with an infectious and inflammatory component is unknown. Lyme neuroborreliosis (LNB) is reported as a rare cause of childhood stroke. Intravenous thrombolysis (IVT) and endovascular therapy (EVT) have not been reported in LNB-associated stroke in children. We report two children with acute stroke associated with LNB who underwent hyperacute stroke treatment. A systematic review of the literature was performed to identify case reports of LNB-associated childhood stroke over the last 20 years. Patient 1 received IVT within 73 min after onset of acute hemiparesis and dysarthria; medulla oblongata infarctions were diagnosed on magnetic resonance imaging (MRI). Patient 2 received successful EVT 6.5 hr after onset of progressive tetraparesis, coma, and decerebrate posturing caused by basilar artery occlusion with bilateral pontomesencephalic infarctions. Both patients exhibited a lymphocytic cerebrospinal fluid (CSF) pleocytosis and elevated antibody index (AI) to . Antibiotic treatment, steroids, and platelet inhibitors including tirofiban infusion in patient 2 were administered. No side effects were observed. On follow-up, patient 1 showed good recovery and patient 2 was asymptomatic. In the literature, 12 cases of LNB-associated childhood stroke were reported. LNB-associated infectious and inflammatory FCA is not a medical contraindication for reperfusion therapies in acute childhood stroke. Steroids are discussed controversially in inflammatory FCA due to LNB. Intensified antiplatelet regimes may be considered; secondary prophylaxis with acetyl-salicylic acid (ASA) is recommended because of a high risk of early stroke recurrence.
PubMed: 36061261
DOI: 10.1177/17562864221102842 -
Otolaryngology--head and Neck Surgery :... Mar 2023Deep brain stimulation (DBS) has considerable efficacy for the motor dysfunction of idiopathic Parkinson's disease (PD) on patient quality of life. However, the benefit...
OBJECTIVE
Deep brain stimulation (DBS) has considerable efficacy for the motor dysfunction of idiopathic Parkinson's disease (PD) on patient quality of life. However, the benefit of DBS on voice and speech quality remains controversial. We carried out a systematic review to understand the influence of DBS on parkinsonian dysphonia and dysarthria.
DATA SOURCES
A PubMed/MEDLINE and Cochrane systematic review was carried out following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and Population, Intervention, Comparison, Outcome, Timing, and Setting (PICOTS) statements.
REVIEW METHODS
Three investigators screened studies published in the literature from inception to May 2022. The following data were retrieved: age, demographic, sex, disease duration, DBS duration, DBS location, speech, and voice quality measurements.
RESULTS
From the 180 studies identified, 44 publications met the inclusion criteria, accounting for 866 patients. Twenty-nine studies focused on voice/speech quality in subthalamic DBS patients, and 6 included patients with stimulation of pallidal, thalamic, and zona incerta regions. Most studies (4/6) reported a deterioration of the vocal parameters on subjective voice quality evaluation. For speech, the findings were more contrasted. There was an important heterogeneity between studies regarding the voice and speech quality outcomes used to evaluate the impact of DBS on voice/speech quality.
CONCLUSION
The impact of DBS on voice and speech quality significantly varies between studies. The stimulated anatomical region may have a significant role since the stimulation of the pallidal area was mainly associated with voice quality improvement, in contrast with other regions. Future controlled studies comparing all region stimulation are needed to get reliable findings.
LEVEL OF EVIDENCE
Level III: evidence from evidence summaries developed from systematic reviews.
Topics: Humans; Parkinson Disease; Speech; Deep Brain Stimulation; Quality of Life; Subthalamic Nucleus; Dysphonia
PubMed: 36040825
DOI: 10.1177/01945998221120189 -
Multiple Sclerosis and Related Disorders Dec 2022Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder of the central nervous system,... (Review)
Review
INTRODUCTION
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder of the central nervous system, characterized by symptoms referable to the brainstem and cerebellum such as, diplopia, gait ataxia and cerebellar dysarthria. The features and outcomes of CLIPPERS remains uncertain. we conducted this comprehensive systematic review to summarize all the existing studies that described CLIPPERS in the literature and to provide a quantitative assessment on the clinical characteristics, management, and outcomes of this rare syndrome.
METHODS
A comprehensive search of PubMed and Web of Science databases was conducted from inception until January 15, 2022, was conducted. We only included the cases that clearly reported probable or definite diagnosis of CLIPPERS based on Taieb et al.'s criteria. The quality of the included studies was assessed using the JBI Critical Appraisal Tool. Descriptive statistics were performed to analyze the studies. Data were expressed as mean and standard deviation (SD) for continuous variables and proportions for categorical variables.
RESULTS
We identified 100 case reports and series including a total of 140 patients with CLIPPERS (mean age: 46±18 years and males were 60%). The average follow-up duration was 32.27±57.8 months. Ataxia was the most common presenting symptom. Sixteen percent of the cases were associated with malignancy, mostly hematologic malignancies. The overall relapse rate was 59.2%, and the duration of steroid therapy was considerably shorter in the relapsed cases than in the non-relapsed (mean 6.19±7.9 vs. 10.14±12.1 days, respectively, P = 0.04). The overall mortality rate was 10%, but mortality in patients with malignancy was 30% and it was 12% in patients with relapses. In the case of steroid dosing (less than 20 mg/d versus greater than 20 mg/d) there was no significant modification in the risk of relapse.
CONCLUSION
CLIPPERS is a rare clinical syndrome that affects mainly middle-aged males. Diagnosis of CLIPPERS is often challenging, and delays in diagnosis and treatment can lead to unfavorable outcomes. Therefore, neurologists should maintain a high index of suspicion for CLIPPERS in any patient presenting with symptoms and signs referrable to the brainstem. These patients should be screened for associated malignancies, especially hematological malignancies. The cases associated with malignancy tend to have worse outcomes. The relapse rate is relatively high. The relapse rate may be associated with worse mortality. Based on our findings, we recommend that CLIPPERS be treated with high-dose steroid therapy for at least ten days during the acute phase with a very slow taper. Prospective studies with a larger sample size are needed to validate our findings and guide the clinical care of these patients.
Topics: Middle Aged; Male; Humans; Adult; Magnetic Resonance Imaging; Prospective Studies; Pons; Inflammation; Steroids; Syndrome; Chronic Disease
PubMed: 36029706
DOI: 10.1016/j.msard.2022.104112 -
Frontiers in Human Neuroscience 2022Augmentative and alternative communication brain-computer interface (AAC-BCI) systems are intended to offer communication access to people with severe speech and...
UNLABELLED
Augmentative and alternative communication brain-computer interface (AAC-BCI) systems are intended to offer communication access to people with severe speech and physical impairment (SSPI) without requiring volitional movement. As the field moves toward clinical implementation of AAC-BCI systems, research involving participants with SSPI is essential. Research has demonstrated variability in AAC-BCI system performance across users, and mixed results for comparisons of performance for users with and without disabilities. The aims of this systematic review were to (1) describe study, system, and participant characteristics reported in BCI research, (2) summarize the communication task performance of participants with disabilities using AAC-BCI systems, and (3) explore any differences in performance for participants with and without disabilities. Electronic databases were searched in May, 2018, and March, 2021, identifying 6065 records, of which 73 met inclusion criteria. Non-experimental study designs were common and sample sizes were typically small, with approximately half of studies involving five or fewer participants with disabilities. There was considerable variability in participant characteristics, and in how those characteristics were reported. Over 60% of studies reported an average selection accuracy ≤70% for participants with disabilities in at least one tested condition. However, some studies excluded participants who did not reach a specific system performance criterion, and others did not state whether any participants were excluded based on performance. Twenty-nine studies included participants both with and without disabilities, but few reported statistical analyses comparing performance between the two groups. Results suggest that AAC-BCI systems show promise for supporting communication for people with SSPI, but they remain ineffective for some individuals. The lack of standards in reporting outcome measures makes it difficult to synthesize data across studies. Further research is needed to demonstrate efficacy of AAC-BCI systems for people who experience SSPI of varying etiologies and severity levels, and these individuals should be included in system design and testing. Consensus in terminology and consistent participant, protocol, and performance description will facilitate the exploration of user and system characteristics that positively or negatively affect AAC-BCI use, and support innovations that will make this technology more useful to a broader group of people.
CLINICAL TRIAL REGISTRATION
https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42018095345, PROSPERO: CRD42018095345.
PubMed: 35966988
DOI: 10.3389/fnhum.2022.952380