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Acta Neurologica Scandinavica Apr 2021Dravet syndrome (DS) is a severe, drug-resistant, developmental epileptic encephalopathy. Despite multiple anti-epileptic drug regimens, the syndrome remains poorly... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Dravet syndrome (DS) is a severe, drug-resistant, developmental epileptic encephalopathy. Despite multiple anti-epileptic drug regimens, the syndrome remains poorly controlled and nearly half of patients still experience at least four tonic-clonic seizure per month. Recently, several clinical trials demonstrated that fenfluramine may provide a significant reduction in convulsive seizure frequency in the treatment of Dravet syndrome.
METHODS
A computerized literature search of Web of Science, MEDLINE (Ovid and PubMed), Cochrane Library, EMBASE, and Google Scholar was performed from inception until December 31, 2019. We included randomized placebo-controlled trials for the treatment of Dravet syndrome. We calculated the risk ratio (RR) of ≥50% and 100% reduction seizure frequency from baseline, along with the treatment-related withdrawals and serious adverse events, using the fixed-effect model. Quality assessment of included studies was performed with the Cochrane Collaboration's tool.
KEY RESULTS
Two trials with a total of 206 patients were included. The pooled RR of 5.49 (95% CI 3.13-9.65) showed that a significantly greater proportion in the fenfluramine group achieved ≥50% reduction in monthly convulsive seizure frequency (MCSF). As for the complete seizure free rate, the pooled RR of 5.75 (95% CI 1.03-32.07) also demonstrated the favorable efficacy of fenfluramine, even though the difference was not statistically significant (p = 0.046). However, a significantly greater proportion of patients in the fenfluramine group experienced no more than one seizure during the treatment period (RR 13.82, 95% CI 2.68-71.27, p = 0.002). There were no significant differences in withdrawals and serious adverse events between the two treatment groups. No valvular heart disease or pulmonary arterial hypertension was observed in participants. The most common adverse events reported by included trials were diarrhea, fatigue, lethargy, nasopharyngitis, pyrexia, seizure, decreased appetite, and weight loss.
CONCLUSIONS
Fenfluramine is an effective antiepileptic drug for pediatric patients with Dravet syndrome, demonstrating clinically meaningful reduction in convulsive frequency, and generally could be well tolerated.
Topics: Anticonvulsants; Child; Child, Preschool; Epilepsies, Myoclonic; Fatigue; Fenfluramine; Fever; Humans; Multicenter Studies as Topic; Randomized Controlled Trials as Topic; Seizures
PubMed: 33336426
DOI: 10.1111/ane.13387 -
Journal of Pediatric Urology Dec 2013Dilutional hyponatremia is a serious adverse effect of desmopressin, a vasopressin analog that is widely prescribed to manage monosymptomatic enuresis. The presentation... (Review)
Review
OBJECTIVE
Dilutional hyponatremia is a serious adverse effect of desmopressin, a vasopressin analog that is widely prescribed to manage monosymptomatic enuresis. The presentation of hyponatremia, largely related to cerebral dysfunction, can include severe signs like altered mental status and seizures.
METHODS
We reviewed the literature dealing with altered mental status or seizures in enuretic subjects on desmopressin. The retained publications included patients who were described individually, revealing data on mode of administration, further identifiable factors predisposing to hyponatremia, presentation and clinical course.
RESULTS
We found 54 cases of hyponatremia secondary to desmopressin treatment presenting with altered mental status or seizures. In most cases the complication developed 14 days or less after starting desmopressin. An intranasal formulation had been used in 47 patients. Excess fluid intake was documented as a contributing factor in at least 22 cases. In 6 cases severe signs of hyponatremia developed in the context of intercurrent illnesses.
CONCLUSION
Altered mental status or seizures are very rare but recognized complications of desmopressin in enuresis. This complication mostly develops in subjects managed with the intranasal formulation 14 days or less after starting the medication, following excess fluid intake and during intercurrent illnesses.
Topics: Antidiuretic Agents; Deamino Arginine Vasopressin; Enuresis; Humans; Hyponatremia; Lethargy; Seizures; Severity of Illness Index
PubMed: 23619353
DOI: 10.1016/j.jpurol.2013.02.012 -
PloS One 2024Malaria is a vector-borne disease that initially manifests as fever, headache, and chills. The illness could progress to more severe conditions, including lethargy,...
INTRODUCTION
Malaria is a vector-borne disease that initially manifests as fever, headache, and chills. The illness could progress to more severe conditions, including lethargy, impaired consciousness, convulsions, shortness of breath, blood in urine, jaundice, and haemorrhage if left untreated. The risk of contracting malaria is considerably heightened in specific occupational settings, particularly among forest rangers, following frequent exposure to natural habitats. Consequently, advancing the understanding of malaria and emphasising how specific occupational environments (including those of forest rangers) contribute to disease risk and management is imperative.
OBJECTIVE
The present study aims to determine the factors associated with malaria infection among forest rangers by systematically reviewing electronic articles from three databases (EBSCOhost, ScienceDirect, and ResearchGate).
METHODS
The current review was prepared based on the updated preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. First, three independent reviewers screened the titles and abstracts of the data collected. The information was then stored in Endnote20 based on the inclusion and exclusion criteria. The articles were critically appraised with the mixed methods appraisal tool (MMAT) to assess their quality.
RESULT
A total of 103, 31, and 51 articles from EBSCOhost, ScienceDirect, and ResearchGate, respectively, were selected, resulting in 185 unique hits. Nevertheless, only 63 full-text publications were assessed following a rigorous selection screening, from which only five were included in the final review. The studies revealed that several factors contribute to malaria infection among forest rangers. The parameters were classified into sociodemographic, individual, and living condition-related.
CONCLUSION
A better understanding of malaria progresses and identifying its potential risk factors is essential to impact worker well-being. The findings might be utilised to improve malaria infection prevention programme implementations, hence maximising their success. Pre-employment and regular health screenings could also aid in evaluating and identifying potential risks for malaria infection among forest rangers.
Topics: Humans; Malaria; Forests; Risk Factors; Animals
PubMed: 38748721
DOI: 10.1371/journal.pone.0303406 -
Expert Opinion on Drug Metabolism &... Jun 2020Recent trials using cannabidiol (CBD) have shown that most acute and prolonged adverse effects of CBD are mild to moderate, with rare serious adverse effects (SAEs)....
INTRODUCTION
Recent trials using cannabidiol (CBD) have shown that most acute and prolonged adverse effects of CBD are mild to moderate, with rare serious adverse effects (SAEs). This review focused on analyzing SAEs of CBD and their possible relation to drug-drug interactions.
AREAS COVERED
We systematically analyzed the SAEs reported in randomized controlled trials (RCTs) involving the administration of oral CBD for at least 1 week in both healthy volunteers and clinical samples.
EXPERT OPINION
SAEs related to CBD in RCT are rare and include mainly elevated transaminases, convulsion, sedation, lethargy, and upper respiratory tract infections. Elevated transaminases are related to concomitant valproate use, while sedation, lethargy, and upper respiratory tract infections are related to concomitant clobazam use. Epileptic patients should be monitored when using CBD concomitantly with these and other antiepileptic drugs for other possible drug-drug interactions.
Topics: Administration, Oral; Anticonvulsants; Cannabidiol; Drug Interactions; Drug Therapy, Combination; Epilepsy; Humans; Randomized Controlled Trials as Topic
PubMed: 32271618
DOI: 10.1080/17425255.2020.1754793 -
Frontiers in Neurology 2023Relapsing polychondritis (RP) is a rare rheumatologic disorder that may affect the neurological system with various presentations. In this study, we present a case and...
BACKGROUND AND PURPOSE
Relapsing polychondritis (RP) is a rare rheumatologic disorder that may affect the neurological system with various presentations. In this study, we present a case and summarize the clinical characteristics of RP-associated meningoencephalitis.
CASE PRESENTATION
A 48-year-old man presented with first-ever seizures that were well controlled by valproate. Physical examination results were unremarkable, except for binaural deformation. The initial brain magnetic resonance imaging (MRI) without contrast and electroencephalogram (EEG) findings were normal. However, the patient subsequently developed recurrent fever, scleritis, headache, lethargy, and left arm paresis. Repeated brain MRI with contrast demonstrated increased enhancement of the pia mater and abnormal diffusion-weighted imaging (DWI) signals in the bilateral auricles. The cerebrospinal fluid (CSF) analysis showed 2 leukocytes/μL, 736.5 mg/L of protein, and no evidence of infectious disease or autoimmune encephalitis. Meningoencephalitis secondary to RP was considered. The patient's condition improved significantly and quickly with the administration of dexamethasone (10 mg per day). Oral methylprednisolone was continued, and the patient remained well without relapse during the 9-month follow-up period.
CONCLUSION
RP-associated meningoencephalitis is rare but fatal. Although symptoms vary, red or deformed ears remain the most common and suggestive features. Non-specific parenchymal changes and/or meningeal enhancement can be observed on brain MRI scans. CSF lymphocytic pleocytosis with mild protein elevation was observed in most patients.
PubMed: 38033767
DOI: 10.3389/fneur.2023.1265345 -
Research in Veterinary Science Jul 2024Leptospirosis is a neglected bacterial zoonosis that affects a wide range of mammals, with important implications from a One Health perspective. Over the past years... (Meta-Analysis)
Meta-Analysis
Leptospirosis is a neglected bacterial zoonosis that affects a wide range of mammals, with important implications from a One Health perspective. Over the past years feline leptospirosis has gained increased attention in the scientific community. Here we describe a systematic review with meta-analysis that followed the PRISMA guidelines, with an additional PROSPERO registration. The study provides global seropositivity, urinary shedding rates, global serogroup distribution, descriptive data of leptospires that had been isolated from cats and clinical and laboratory features presented by symptomatic cats with acute disease. The search was carried out in six different databases, with the identification of 79 reports describing leptospiral infection in cats. The pooled frequency of seroreactive cats was 11% (95% CI: 9%-13%), with Javanica and Pomona as the most frequent serogroups found. Frequency for urinary shedding was 8% (95% CI: 5%-10%), with L. interrogans identified in most samples. A total of 16 isolates were isolated from cats, with Bataviae as the most frequent serogroup. Twenty symptomatic cats with confirmed leptospiral infection were identified. Anorexia, lethargy, polydipsia, and bleeding disorders were the clinical signs most frequently reported. The results suggest that cats from some locations are exposed to leptospires and may act as urinary shedders of this pathogen, thus indicating a possible role of this species in disease transmission. Clinical data indicates that acute infection is mostly atypical when compared to dogs, and due to difficulties to define an archetypal clinical presentation in cats, feline leptospirosis is likely to be underdiagnosed disease in this species.
Topics: Animals; Cats; Cat Diseases; Leptospira; Leptospirosis
PubMed: 38759347
DOI: 10.1016/j.rvsc.2024.105292 -
General Hospital Psychiatry 2020To ascertain the phenomenological characterisation of catatonia in N-methyl-d-aspartate receptor antibody encephalitis (NMDAr-AbE).
OBJECTIVE
To ascertain the phenomenological characterisation of catatonia in N-methyl-d-aspartate receptor antibody encephalitis (NMDAr-AbE).
METHODS
A systematic review of case reports was undertaken in accordance with PRISMA guidelines. Case reports of NMDAr-AbE containing sufficient information on the cases' clinical presentation and meeting the study's inclusion criteria were selected. Cases were searched for clinical data in keeping with core catatonic signs by applying the screening instrument of the Bush-Francis Catatonia Rating Scale. When two or more core signs were ascertained catatonia was considered to be present.
RESULTS
2645 records were identified through the database search. Following screening and application of eligibility/inclusion criteria 139 articles were selected reporting on 189 individual subjects. Catatonia was present in 60% of these cases. The most prevalent signs were immobility/stupor (70%), mutism (67%), excitement (50%), posturing/catalepsy (34%), stereotypies (31%), and rigidity (30%). Immobility/stupor and excitement co-occurred in the same patient in 33% of cases.
CONCLUSION
The phenomenological profile of catatonia in this sample of cases of NMDAr-AbE was characterised by a preponderance of signs in the hypokinetic spectrum. However, excitement often co-occurred in these patients suggesting that fluctuations in catatonic semiology may be frequent.
Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Catatonia; Humans
PubMed: 32070914
DOI: 10.1016/j.genhosppsych.2020.01.002 -
L' Orthodontie Francaise Apr 2023Several cross-sectional studies have shown the association of a dysfunctional orofacial environment with a greater prevalence of malocclusions. Orofacial myofunctional...
INTRODUCTION
Several cross-sectional studies have shown the association of a dysfunctional orofacial environment with a greater prevalence of malocclusions. Orofacial myofunctional reeducation (OFMR) is the rehabilitation of the muscles, functions and resting postures of the orofacial complex. It is used in the therapeutic management of orofacial dysfunction in patients of all ages and with a wide range of disorders and comorbidities. RMOF mainly uses isotonic and isometric exercises targeting the oral and oropharyngeal structures, combined with specific exercises for ventilation, swallowing and mastication. It may involve the use of prefabricated reeducation appliances (PRAs), which may also be prescribed to modify the shape and relationship of the dental arches.
OBJECTIVES
The primary objective of this systematic review of the literature was to describe and evaluate the efficacy of prefabricated reeducation appliance-assisted OFMR in orthodontics, occlusodontics and dental sleep medicine. Its secondary objective was to assess whether the use of currently available PRAs is associated with adverse effects.
MATERIALS AND METHODS
The systematic literature review was undertaken using five electronic databases: Medline (via PubMed), Web of Science, Cochrane Library, Embase, and Google Scholar, to identify studies evaluating the efficacy of PRA-assisted OFMR in the treatment of orofacial dysfunctions and parafunctions, temporo-mandibular dysfunction (TMD) or obstructive sleep apnea (OSA) in children, adolescents and adults, published until 20 March 2023. The primary outcome of interest was the therapeutic efficacy of PRA-assisted OFMR. In patients with obstructive sleep apnoea (OSA), efficacy was assessed primarily by a decrease in the apnoea/hypopnoea index (AHI) of at least five episodes per hour from baseline, improvement in subjective sleep quality, sleep quality measured by nocturnal polysomnography and subjectively measured quality of life. In patients with orofacial dysfunctions, parafunctions or TMD, efficacy was assessed mainly by electromyography (EMG), history and clinical examination. Secondary outcomes were dentoalveolar or skeletal improvements, and possible adverse effects of the PRAs used, including adverse effects on occlusion.
RESULTS
Only fourteen studies met all inclusion criteria: two randomised controlled trials, one non-randomised controlled trial, five prospective case-control studies, two retrospective case-control studies, two prospective case series and two retrospective case series. The two randomised controlled trials were assessed as "low risk of bias" according to the Cochrane Back Review Group's 12 risk of bias criteria. The methodological quality of the remaining 12 included studies was assessed using the ROBINS-I tool, according to the recommendations of the Cochrane Handbook. One was judged to have a measured risk of bias, eight a significant risk of bias and three a critical risk of bias. Based on the available evidence, PRA-assisted OFMR results in a statistically significant (p=0.0425) reduction in AHI in children with mild to moderate obstructive sleep apnea. In children with obstructive sleep apnoea undergoing adenoid and/or tonsil surgery, postoperative OFMR combined with a flexible PRA leads to a greater reduction in AHI compared to a control group and an improvement in SaO2 at 6 months and 12 months after surgery (p<0.01). It also contributes to greater improvement in sleep disturbance, physical fitness, daytime lethargy in the treated group than in the control group 6 months and 12 months after surgery (p<0.05). PRA-assisted OFMR provides correction of atypical swallowing and improvement in orofacial muscle balance. GRPs are generally less effective than activators for the treatment of Class II Division 1 malocclusions and appear to cause more adverse effects, mainly vestibuloversion of the mandibular incisors. The use of PRA-assisted OFMR for the management of TMD is not validated by current evidence.
CONCLUSIONS
Published data, albeit of uneven methodological quality, appear to show the superiority of OFMR combined with the use of a PRA, compared with the implementation of OFMR without PRA. Prospective studies with large sample sizes would be useful to better evaluate the new therapeutic possibilities brought by the combination of OFMR with a PRA. Continued attention should be paid to the monitoring of possible adverse effects of PRA-assisted OFMR on the dental arches, especially the vestibuloversion of the mandibular incisors. It might be useful to reflect on the relevance of the arguments put forward by manufacturers about the particularities of their devices and their supposed effects. PRA-assisted OFMR appears to be a necessary paradigm shift , which it seems useful to bring to our patients.
PROTOCOL REGISTRATION
This protocol was registered on March 02, 2023 in the International Prospective Register of Systematic Review (PROSPERO) and received the CRD number: CRD42023400421.
Topics: Adolescent; Adult; Child; Humans; Cross-Sectional Studies; Prospective Studies; Quality of Life; Retrospective Studies; Sleep Apnea, Obstructive; Patient Education as Topic; Malocclusion; Controlled Clinical Trials as Topic
PubMed: 37114821
DOI: 10.1684/orthodfr.2023.126 -
World Neurosurgery Nov 2020Preoperative embolization of spinal metastases may improve outcomes of resection by reducing surgical blood loss and operative time. Neurologic complications are rarely...
BACKGROUND
Preoperative embolization of spinal metastases may improve outcomes of resection by reducing surgical blood loss and operative time. Neurologic complications are rarely reported and the mechanisms leading to injury are poorly described.
METHODS
We present 2 illustrative cases of embolization-related neurologic injury from distinct mechanisms and the findings of a systemic literature review of similar complications according to the PRISMA guidelines.
RESULTS
A 77-year-old man with a history of renal cell carcinoma presented with gait dyscoordination and arm pain/weakness. Magnetic resonance imaging showed a C7/T1 mass causing severe compression of the C7/T1 roots and spinal cord. After embolization and tumor resection/fusion, lethargy prompted imaging showing multiple posterior circulation infarcts believed to be secondary to reflux of embolic particles. A 75-year-old man with renal cell carcinoma presented with L1 level metastasis causing conus compression and experienced paraplegia after superselective particle embolization presumed to be secondary to flow disruption of the artery of Adamkiewicz. Analysis of the literature yielded 6 articles reporting instances of cranial infarction/ischemia occurring in 10 patients, 12 articles reporting spinal cord ischemia/infarction occurring in 17 patients, and 5 articles reporting symptomatic postembolization tumoral swelling in 5 patients.
CONCLUSIONS
Neurologic injury is a risk of preoperative embolization of spinal metastasis from either compromise of spinal cord vascular supply or cranial stroke from reflux of embolic particles. Postprocedural tumor swelling rarely leads to clinical deficit. Awareness of these complications and the presumed mechanisms of injury may aid clinicians in implementing interventions and in counseling patients before treatment.
Topics: Carcinoma, Renal Cell; Embolization, Therapeutic; Humans; Kidney Neoplasms; Magnetic Resonance Imaging; Male; Middle Aged; Nervous System Diseases; Paraplegia; Preoperative Care; Spinal Neoplasms
PubMed: 32805465
DOI: 10.1016/j.wneu.2020.08.006