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Pediatric Neurosurgery 2022Novel targeted and tailored therapies can substantially improve the prognosis for optic pathway glioma (OPG), especially when implemented in a timely manner. However,...
INTRODUCTION
Novel targeted and tailored therapies can substantially improve the prognosis for optic pathway glioma (OPG), especially when implemented in a timely manner. However, their tremendous potential remains underestimated. Therefore, in this study, we provide an updated overview of the clinical trials, current trends, and future perspectives for OPG's novel therapeutic strategies.
METHODS
We completed an extensive literature review using the PubMed, MEDLINE, and ClinicalTrials.gov databases. We analyzed and reported the data following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
RESULTS
Thioguanine, procarbazine, lomustine, and vincristine/vinblastine, as well as cisplatin-etoposide, provided excellent results in advanced-phase trials. Selumetinib and trametinib, two oral MEK inhibitors, have been approved for recurrent or refractory OPGs in association with the angiogenetic inhibitor bevacizumab. Among the mTOR inhibitors, everolimus and sirolimus showed the best results. Stereotactic radiosurgery and proton beam radiation therapy have advantages over conventional radiotherapy regimens. Timely treatment is imperative for acute visual symptoms with evidence of tumor progression. This latest evidence can help define a novel "T-Dimension" for pediatric OPG therapies.
CONCLUSION
The novel "T-Dimension" for pediatric OPGs is based on recent evidence-based treatments, including combination chemotherapy regimens, molecular targeted therapies, stereotactic radiosurgery, and proton beam radiation therapy. Additional clinical trials are essential for validating each of these new therapies.
Topics: Child; Combined Modality Therapy; Glioma; Humans; Lomustine; Radiosurgery; Vincristine
PubMed: 35588700
DOI: 10.1159/000524873 -
World Neurosurgery Aug 2022Optic pathway gliomas (OPGs) typically occur in the first decade of life and 40%-50% are not associated with neurofibromatosis 1 (NF1) (sporadic). Management strategies... (Review)
Review
OBJECTIVE
Optic pathway gliomas (OPGs) typically occur in the first decade of life and 40%-50% are not associated with neurofibromatosis 1 (NF1) (sporadic). Management strategies are often patient specific because of the variable and unpredictable course. No study has summarized the effect of treatment strategies on visual outcomes in the subset of pediatric patients with sporadic OPG.
METHODS
We conducted a systematic review to determine the nature of visual outcomes in pediatric patients with sporadic, non-NF1-associated OPG using the PubMed, Embase, Scopus, Cochrane, and CINAHL Plus databases. Visual outcomes were categorized as improved, unchanged, or deteriorated.
RESULTS
Of 1316 results, 31 articles were included. Treatment indications are unknown with full clinical detail. A total of 45.2% (14/31) reported deteriorated outcomes after treatment, 35.5% (11/31) no change, and 19.4% (6/31) improvement. Of radiotherapy studies, 50.0% (4/8) found no change, 37.5% (3/8) deterioration, and 12.5% (1/8) improvement. Of chemotherapy studies, 35.7% (5/14) each showed improvement and deterioration, whereas 28.6% (4/14) showed no change. Of surgical studies, 62.5% (5/8) indicated deterioration, and 37/5% (3/8) indicated no change. The singular study examining observation reported deterioration in visual outcomes. Factors associated with poor visual outcomes included signs and symptoms of visual decline at presentation, involvement of the intraorbital optic nerve, and intracranial hypertension requiring surgery. Causality cannot be determined from systematic review.
CONCLUSIONS
Most studies showed that vision in pediatric patients with sporadic OPG is stable to poor after observation, chemotherapy, radiotherapy, or surgery. Chemotherapy may be associated with most favorable visual outcomes.
Topics: Child; Follow-Up Studies; Humans; Neurofibromatosis 1; Optic Nerve Glioma; Optic Nerve Neoplasms; Retrospective Studies
PubMed: 35430402
DOI: 10.1016/j.wneu.2022.04.033 -
Frontiers in Neuroscience 2022Neuroplasticity following deafness has been widely demonstrated in both humans and animals, but the anatomical substrate of these changes is not yet clear in human...
Neuroplasticity following deafness has been widely demonstrated in both humans and animals, but the anatomical substrate of these changes is not yet clear in human brain. However, it is of high importance since hearing loss is a growing problem due to aging population. Moreover, knowing these brain changes could help to understand some disappointing results with cochlear implant, and therefore could improve hearing rehabilitation. A systematic review and a coordinate-based meta-analysis were realized about the morphological brain changes highlighted by MRI in severe to profound hearing loss, congenital and acquired before or after language onset. 25 papers were included in our review, concerning more than 400 deaf subjects, most of them presenting prelingual deafness. The most consistent finding is a volumetric decrease in gray matter around bilateral auditory cortex. This change was confirmed by the coordinate-based meta-analysis which shows three converging clusters in this region. The visual areas of deaf children is also significantly impacted, with a decrease of the volume of both gray and white matters. Finally, deafness is responsible of a gray matter increase within the cerebellum, especially at the right side. These results are largely discussed and compared with those from deaf animal models and blind humans, which demonstrate for example a much more consistent gray matter decrease along their respective primary sensory pathway. In human deafness, a lot of other factors than deafness could interact on the brain plasticity. One of the most important is the use of sign language and its age of acquisition, which induce among others changes within the hand motor region and the visual cortex. But other confounding factors exist which have been too little considered in the current literature, such as the etiology of the hearing impairment, the speech-reading ability, the hearing aid use, the frequent associated vestibular dysfunction or neurocognitive impairment. Another important weakness highlighted by this review concern the lack of papers about postlingual deafness, whereas it represents most of the deaf population. Further studies are needed to better understand these issues, and finally try to improve deafness rehabilitation.
PubMed: 35418829
DOI: 10.3389/fnins.2022.850245 -
Diagnostics (Basel, Switzerland) Feb 2022Radiofrequency (RF) is a minimally invasive procedure used to interrupt or alter nociceptive pathways for treating musculoskeletal pain. It seems a useful tool to... (Review)
Review
Radiofrequency (RF) is a minimally invasive procedure used to interrupt or alter nociceptive pathways for treating musculoskeletal pain. It seems a useful tool to relieve chronic pain syndromes, even if, to date, solid evidence is still needed about the effectiveness of this therapy. By this systematic review and meta-analysis, we aimed to evaluate the efficacy of RF in treating musculoskeletal pain. PubMed, Medline, Cochrane, and PEDro databases were searched to identify randomized controlled trials (RCTs) presenting the following: patients with chronic musculoskeletal pain as participants; RF as intervention; placebo, anesthetic injection, corticosteroid injection, prolotherapy, conservative treatment, physiotherapy, and transcutaneous electrical nerve stimulation as comparisons; and pain and functioning as outcomes. Continuous random-effect models with standardized mean difference (SMD) were used to compare the clinical outcomes. Overall, 26 RCTs were eligible and included in the systematic review. All of them analyzed the efficacy of RF in four different regions: cervical and lumbar spine, knee, sacroiliac (SI) joint, shoulder. The outcomes measures were pain, disability, and quality of life. A medium and large effect in favor of the RF treatment group (SMD < 0) was found for the shoulder according to the Visual Analogical Scale and for the SI joint according to the Oswestry Disability Index. A small effect in favor of the RF treatment group (SMD > 0) was found for the spine according to the 36-item Short Form Survey. Non-significant SMD was found for the other outcomes. RF represents a promising therapy for the treatment of chronic musculoskeletal pain, especially when other approaches are ineffective or not practicable. Further studies are warranted to better deepen the effectiveness of RF for pain and joint function for each anatomical region of common application.
PubMed: 35328153
DOI: 10.3390/diagnostics12030600 -
Ophthalmic & Physiological Optics : the... Jul 2022As the prevalence of eye diseases increases, demand for effective, accessible and equitable eye care grows worldwide. This is especially true in lower and middle-income... (Review)
Review
PURPOSE
As the prevalence of eye diseases increases, demand for effective, accessible and equitable eye care grows worldwide. This is especially true in lower and middle-income countries, which have variable levels of infrastructure and economic resources to meet this increased demand. In the present study we aimed to review the literature on eye care in Pakistan comprehensively, with a particular focus on eye care pathways, patient priorities and economics.
METHODS
A systematic scoping review was performed to identify literature relating to eye care in Pakistan. Searches of relevant electronic databases and grey literature were carried out. The results were analysed through a mixed methods approach encompassing descriptive numerical summary and thematic analysis. To consolidate results and define priority areas for future study, expert consultation exercises with key stakeholders were conducted using qualitative semi-structured interviews.
RESULTS
One hundred and thirty-two papers (published and unpublished) were included in the final review. The majority (n = 93) of studies utilised a quantitative design. Seven interlinked themes were identified: eye care pathways, burden of eye disease, public views on eye-related issues, workforce, barriers to uptake of eye care services, quality of eye care services and economic impact of blindness. Research priorities included investigating the eye care workforce, the quality and efficiency of current eye care services, eye care services available in rural Pakistan and the costs and benefits related to eye care provision and sustaining eye care programmes.
CONCLUSIONS
To the best of our knowledge, this is the first review to synthesise evidence from papers across the field relating to eye care in Pakistan. As such, this work provides new insights into the achievements of the national eye health programme, challenges in eye care in Pakistan and priority areas for future research.
Topics: Critical Pathways; Humans; Pakistan; Referral and Consultation
PubMed: 35318687
DOI: 10.1111/opo.12977 -
Neuropsychology Review Mar 2023Pediatric survivors of brain tumors and acute lymphoblastic leukemia (ALL) are at risk for long-term deficits in their neuropsychological functioning. Researchers have... (Review)
Review
Pediatric survivors of brain tumors and acute lymphoblastic leukemia (ALL) are at risk for long-term deficits in their neuropsychological functioning. Researchers have begun examining associations between germline single nucleotide polymorphisms (SNPs), which interact with cancer treatment, and neuropsychological outcomes. This review synthesizes the impact of treatment-related toxicity from germline SNPs by neuropsychological domain (i.e., working memory, processing speed, psychological functioning) in pediatric survivors. By focusing on specific neuropsychological domains, this review will examine outcome measurement and critique methodology. Fourteen studies were identified and included in this review using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). All studies were published in peer-reviewed journals in English by November 24, 2021. Reviewed studies were not of sufficient quality for a meta-analysis due to varying measurement strategies, gaps in reported descriptive variables, and low power. All neuropsychological domains evaluated in this review had associations with SNPs, except fine motor and visual integration abilities. Only five SNPs had consistent neuropsychological findings in more than one study or cohort. Future research and replication studies should use validated measures of discrete skills that are central to empirically validated models of survivors' long-term outcomes (i.e., attention, working memory, processing speed). Researchers should examine SNPs across pathophysiological pathways to investigate additive genetic risk in pediatric cancer survivors. Two SNPs were identified that confer resiliency in neuropsychological functioning, and future work should investigate resiliency genotypes and their underlying biological mechanisms.
Topics: Child; Humans; Brain Neoplasms; Cancer Survivors; Genomics; Memory, Short-Term; Neuropsychological Tests; Survivors
PubMed: 35305234
DOI: 10.1007/s11065-022-09539-2 -
Neurocirugia (English Edition) 2023The literature that explains the neurological mechanisms underlying the development or compensation of idiopathic scoliosis is limited. The objective of the article is... (Review)
Review
The literature that explains the neurological mechanisms underlying the development or compensation of idiopathic scoliosis is limited. The objective of the article is to describe and integrate the mechanisms and nerve pathways through which idiopathic scoliosis is compensated and/or developed. A narrative systematic review in different databases of the studies published between January 1, 1967 and April 1, 2021 was performed, using the following terms: "scoliosis", "vision", "eye", "vestibule", "labyrinth" "posture", "balance", "eye movements", "cerebellum", "proprioception", and "physiological adaptation". In the search, 1112 references were identified, of which 50 were finally included: 46 observational analytical clinical studies-descriptive (between cohorts, report and series of cases) and 4 experimental studies. In the neurological response to idiopathic scoliosis, the sensory-cortical integration of the afferences in the visual-oculomotor-vestibular-proprioceptive systems, allows modifications at the postural level in order to achieve an initial compensation on the sagittal balance and the centre of body mass; however, over time these compensation mechanisms may be exhausted causing progression of the initial deformity.
Topics: Humans; Scoliosis; Proprioception; Posture; Vestibule, Labyrinth; Neural Pathways
PubMed: 35256329
DOI: 10.1016/j.neucie.2022.02.009 -
Orphanet Journal of Rare Diseases Mar 2022Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder with heterogeneous clinical manifestations and rare neurologic involvement. The existing clinical...
BACKGROUND
Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder with heterogeneous clinical manifestations and rare neurologic involvement. The existing clinical literature about neurologic RDD has yet to be critically examined.
METHODS
We performed a four-database English-language systematic literature search for cases of RDD neurohistiocytosis, excluding secondary literature. Individual patient data for neurologic symptoms, disease sites, treatments, and responses were captured. Responses to first-line and second-line surgical interventions, post-surgical radiotherapy, and systemic therapies were analyzed.
RESULTS
Among 4769 articles yielded by literature search, 154 articles were fully reviewed, containing data on 224 patients with neurologic RDD. 128 (83.1%) articles were single case reports. 149 (66.5%) patients were male, 74 (33.5%) female, with a median age of 37.6 years (range 2-79). Presenting neurologic symptoms included headache (45.1%), focal neurological deficits (32.6%), visual symptoms (32.1%), and seizures (24.6%). RDD involvement was multifocal in 32 (14.3%) cases. First-line treatment involved resection in 200 (89.6%) patients, with subsequent progression in 52 (26%), including 41 (78.8%) with unifocal disease. No difference was observed in progression-free survival comparing post-operative radiotherapy to no radiotherapy following partial resection. Chemotherapy given alone as first-line treatment led to complete or partial response in 3/7(43%) patients. Second-line treatments led to complete or partial response in 18/37(37.5%) patients. Mutational data were reported on 10 patients (4.46%).
CONCLUSIONS
This review highlights the limited published data about neurologic RDD, which presents with varied symptomatology and outcome. Further study is needed about its mutational landscape, and more effective therapies are needed for recurrent and refractory disease.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Female; Histiocytosis, Sinus; Humans; Male; Middle Aged; Nervous System; Young Adult
PubMed: 35236371
DOI: 10.1186/s13023-022-02220-0 -
Neurological Sciences : Official... Apr 2022Hemianopia is a complete or partial blindness in the visual fields of both eyes, commonly caused by cerebral infarction. It has been hypothesized that systematic... (Review)
Review
BACKGROUND
Hemianopia is a complete or partial blindness in the visual fields of both eyes, commonly caused by cerebral infarction. It has been hypothesized that systematic audio-visual (AV) stimulation of the blind hemifield can improve accuracy and search times, probably due to the stimulation of bimodal representations in the superior colliculus (SC), an important multisensory structure involved in both the initiation and execution of saccades.
METHODS
A narrative synthesis of the findings is presented to highlight how AV rehabilitation impacts on patients with hemianopia including visual oculomotor function, functional ability in activities of daily living, hemianopic dyslexia, visual scanning and searching tasks, maintaining of functional ability post training and the effect on brain multisensory integration by using neuroimaging.
RESULTS
Sixteen studies were included (fourteen articles (188 participants) and two literature reviews). Results were grouped into AV training of hemianopia in adults and in children and then further grouped according to the AV task type: tasks measuring the training effects by comparing visual stimulation training to audio-visual training, localization abilities in homonymous hemianopia (HH) and AV integration in patients with HH.
CONCLUSION
Systematic AV training may improve the processing of visual information by recruiting subcortical pathways, and because most of the patients with visual cortex damage have an intact SC, it might be useful to use the bimodal AV training to activate retinotectal functions. Nevertheless, the underlying mechanisms supporting the reported positive effects are not currently understood. Systematic functional and/or structural imaging studies may help in understanding the underlying mechanism and inform the design of optimal training paradigms.
Topics: Acoustic Stimulation; Activities of Daily Living; Adult; Child; Hemianopsia; Humans; Photic Stimulation; Stroke; Survivors; Visual Fields
PubMed: 35149925
DOI: 10.1007/s10072-022-05926-y -
Acta Dermato-venereologica Feb 2022Cholestatic itch is a disabling symptom that may be secondary to liver or biliary diseases. Management of cholestatic pruritus is complex. A systematic review and... (Meta-Analysis)
Meta-Analysis
Cholestatic itch is a disabling symptom that may be secondary to liver or biliary diseases. Management of cholestatic pruritus is complex. A systematic review and meta-analysis on the efficacy of treatments for cholestatic pruritus were performed. PubMed and Cochrane Library were searched using the algorithm "(hepatitis OR cholestatic OR liver) AND (pruritus OR itch) AND (management OR treatment OR treatments)" for 1975-2019. Of the 2,264 articles identified, 93 were included in a systematic review and 15 in a meta-analysis (studies evaluating pruritus with a visual analogue scale). Some treatments act by reducing levels of pruritogens in the enterohepatic cycle, others modify the metabolism or secretion of these pruritogens, or act on pruritus pathways. A further possible treatment is albumin dialysis. However, due to many heterogeneities in the reviewed studies it is difficult to identify and recommend an optimum treatment. Only 15 studies were included in the meta-analysis, due to the small number of randomized studies using a visual analogue scale.
Topics: Cholestasis; Humans; Pruritus; Renal Dialysis
PubMed: 35088869
DOI: 10.2340/actadv.v102.310