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Frontiers in Neurology 2023Hereditary spastic paraplegias (HSPs) are a group of inheritance diseases resulting in gait abnormalities, which may be detected using instrumented gait analysis. The...
BACKGROUND
Hereditary spastic paraplegias (HSPs) are a group of inheritance diseases resulting in gait abnormalities, which may be detected using instrumented gait analysis. The aim of this systematic review was 2-fold: to identify specific gait analysis patterns and interventions improving gait in HSP subjects.
METHODS
A systematic review was conducted in PubMed, Cochrane Library, REHABDATA, and PEDro databases, in accordance with reporting guidelines of PRISMA statement and Cochrane's recommendation. The review protocol was recorded on the PROSPERO register. Patients with pure and complicated HSP of any age were included. All types of studies were included. Risk of bias, quality assessment, and meta-analysis were performed.
RESULTS
Forty-two studies were included: 19 were related to gait analysis patterns, and 24 were intervention studies. The latter ones were limited to adults. HSP gait patterns were similar to cerebral palsy in younger subjects and stroke in adults. Knee hyperextension, reduced range of motion at knee, ankle, and hip, reduced foot lift, and increased rapid trunk and arm movements were reported. Botulinum injections reduced spasticity but uncovered weakness and improved gait velocity at follow-up. Weak evidence supported intrathecal baclofen, active intensive physical therapy (i.e., robot-assisted gait training, functional exercises, and hydrotherapy), and functional electrical stimulation. Some improvements but adverse events were reported after transcranial magnetic stimulation, transcutaneous spinal direct current stimulation, and spinal cord stimulation implant.
CONCLUSION
Knee hyperextension, non-sagittal pelvic movements, and reduced ROM at the knee, ankle, and hip represent the most peculiar patterns in HSP, compared to diplegic cerebral palsy and stroke. Botulinum improved comfortable gait velocity after 2 months. Nonetheless, interventions reducing spasticity might result in ineffective functional outcomes unveiling weakness. Intensive active physical therapy and FES might improve gait velocity in the very short term.
PubMed: 37799279
DOI: 10.3389/fneur.2023.1256392 -
Expert Review of Anti-infective Therapy May 2024Human T-cell leukemia virus type 1 (HTLV-1) carriers may develop adult T-cell leukemia (ATL), or HTLV-1-associated myelopathy (HAM)/tropical spastic paraparesis (TSP)....
INTRODUCTION
Human T-cell leukemia virus type 1 (HTLV-1) carriers may develop adult T-cell leukemia (ATL), or HTLV-1-associated myelopathy (HAM)/tropical spastic paraparesis (TSP). The evidence is limited regarding other diseases potentially associated with HTLV-1, such as HTLV-1-associated autoimmune diseases.
AREA COVERED
We summarized the available information on complications associated with HTLV-1 infection.
EXPERT OPINION
Previous studies showed that HTLV-1 carriers have an increased incidence of collagen diseases including Sjögren's syndrome, as well as dysthyroidism, diabetes mellitus, and atherosclerosis. Furthermore, cognitive deficits are observed in asymptomatic carriers and in symptomatic carriers who develop HAM/TSP. It is hypothesized that altered immunoregulation occurs as a result of persistent HTLV-1 infection. A systematic review and meta-analysis demonstrated that HTLV-1 infection itself has an adverse impact on overall survival. ATL alone cannot entirely explain the adverse impact of HTLV-1 infection on overall mortality, because the incidence is low, and therefore HTLV-1-associated diseases as a whole may contribute to the inferior clinical outcome. However, there are insufficient data to determine the causal relationship between HTLV-1 infection and each complication. While non-cancerous events linked to HTLV-1 infection are not fatal, they are likely to reduce quality of life. Large prospective studies should be conducted by international collaborators.
Topics: Humans; Autoimmune Diseases; Carrier State; HTLV-I Infections; Human T-lymphotropic virus 1; Leukemia-Lymphoma, Adult T-Cell; Paraparesis, Tropical Spastic
PubMed: 38536666
DOI: 10.1080/14787210.2024.2336547 -
World Neurosurgery Mar 2024Intramedullary meningiomas are an exceptionally rare subtype of spinal tumors, accounting for only 5% of primary spinal neoplasms. Given their scarcity and unique... (Review)
Review
BACKGROUND
Intramedullary meningiomas are an exceptionally rare subtype of spinal tumors, accounting for only 5% of primary spinal neoplasms. Given their scarcity and unique characteristics, understanding optimal management approaches is crucial for improved clinical decision-making. This systematic review aims to consolidate existing literature and present a detailed case illustration to enhance understanding of this uncommon spinal tumor entity.
METHODS
A systematic search adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted. Eligible studies included case reports, case series, cohort studies, reviews, and meta-analyses. Data extraction and synthesis focused on demographic characteristics, tumor location, clinical presentation, imaging findings, surgical interventions, histopathological features, and outcomes.
RESULTS
A total of 15 high-quality scientific articles were included in the systematic review, providing insights into various aspects of intramedullary meningiomas. Demographic analysis revealed a broad age distribution with an equal gender distribution among affected patients. Common clinical presentations included difficulty walking, sensory disturbances, spastic paraparesis, and urinary incontinence. Neuroimaging findings demonstrated heterogeneous signal intensity variations on T1- and T2-weighted images, with variable enhancement patterns on gadolinium-enhanced images. Surgical interventions, predominantly total resection, resulted in favorable postoperative outcomes in most cases.
CONCLUSIONS
Intramedullary meningiomas pose diagnostic and therapeutic challenges due to their rarity and unique characteristics. Tailored surgical approaches, incorporating techniques such as intraoperative neurophysiological monitoring and fluorescence-aided resection, are crucial for minimizing neurological deficits and optimizing patient outcomes. Despite their infrequency, recognizing intramedullary meningiomas in the differential diagnosis of spinal tumors is essential for prompt diagnosis and timely intervention, ultimately improving patient prognosis.
PubMed: 38548054
DOI: 10.1016/j.wneu.2024.03.124 -
Journal of Research in Medical Sciences... 2023Human T-cell lymph tropic virus type 1 (HTLV-I)-related myelopathy/tropical spastic paraparesis (TSP) is a progressive inflammatory process affecting the spinal cord... (Review)
Review
Nonpharmacological interventions and outcomes in the management of complications of human T-cell lymphotropic virus type 1-related myelopathy/tropical spastic paraparesis: A systematic review.
BACKGROUND
Human T-cell lymph tropic virus type 1 (HTLV-I)-related myelopathy/tropical spastic paraparesis (TSP) is a progressive inflammatory process affecting the spinal cord that occurs as a result of HTLV 1. The use of nonpharmacological approaches has always been one of the treatment strategies in these patients, but disagreement about these interventions and their results has led to their limited use. Therefore, this study aimed to identify nonpharmacological interventions and their consequences in these patients.
MATERIALS AND METHODS
We followed the Cochrane Handbook for systematic reviews of interventions. The present report is organized according to the preferred reporting items for systematic reviews and meta-analyses. This study was conducted at PubMed, Cochrane Library, Web of Science, and Scopus, among all published studies by December 30, 2021. Keywords were: HTLV-1, Human T-lymph tropic virus 1, HTLV-I-associated myelopathy, HAM/TSP, tropical spastic paraparesis, nonpharmacological intervention, nonpharmacological treatment, massage, physiotherapy, acupuncture, acupressure, and exercise. The quality of the studies was assessed using JADAD.
RESULTS
Of 288 articles, 11 were eligible for data extraction published between 2014 and 2021. 90/9% of studies were randomized clinical trials. 81/8% of articles were of high quality. The total sample size was 253 people, of which 137 (54/15%) were women. Approaches such as exercise and motion therapy, electrotherapy, behavioral therapy, and virtual reality can be used for these patients. With these interventions, results such as improved mobility and balance, physical condition, pain, quality of life, muscle spasticity, maximum inspiratory pressure, and urinary symptoms can be achieved.
CONCLUSION
The most common physical therapy method used in studies was active and passive body movements, which are associated with positive results for patients. Due to the small sample size in this group of studies, it is necessary to conduct more clinical trials for more accurate conclusions. Furthermore, due to the limited number of studies that have used electrical stimulation or combined intervention packages, it is not possible to say with certainty what effect these methods have on patients. It is necessary to conduct more clinical trials.
PubMed: 38510783
DOI: 10.4103/jrms.jrms_300_22 -
Annals of Cardiothoracic Surgery Sep 2023Spinal cord ischemia (SCI) is one of the most devastating complications of thoracic endovascular aortic repair (TEVAR). Prophylactic cerebrospinal fluid drainage (CSFD)...
BACKGROUND
Spinal cord ischemia (SCI) is one of the most devastating complications of thoracic endovascular aortic repair (TEVAR). Prophylactic cerebrospinal fluid drainage (CSFD) has been shown to decrease the risk of SCI in open thoracic aortic procedures; however, its utility in TEVAR remains uncertain. This systematic review and meta-analysis aim to determine the role of prophylactic CSFD in preventing SCI in TEVAR.
METHODS
A literature search of five databases was performed and all studies published before September 2022 that reported SCI rates in TEVAR patients undergoing prophylactic CSFD were included. A random effects meta-analysis of means or proportions was performed for single-arm data. Odds ratios (ORs) with 95% confidence intervals (CIs) were reported for comparisons between groups.
RESULTS
A total of 4,793 patients undergoing TEVAR from 40 studies were included. The mean age was 68.8 years and 70.9% of patients were male. The overall SCI rate was 3.5%, with a 1.3% rate of immediate SCI and a 1.9% rate of delayed SCI. There were no significant differences in SCI rates between prophylactic CSFD patients and non-drained patients. Routine CSFD did not have a significant impact on SCI rates compared to non-drained patients. There was an increased rate of transient SCI with selective CSFD compared to non-drained patients (OR 2.08; 95% CI: 1.06-4.08; P=0.03). The most common drain-related complication was spinal headache (4.3%). The major complication rate was 1.6%, of which epidural or spinal hematoma (0.9%) was the most common, followed by intracranial or subdural hemorrhage (0.8%) and paraparesis or paraplegia (0.8%).
CONCLUSIONS
This study found no significant difference in SCI rates between prophylactic CSFD patients and their non-drained counterparts. CSFD is associated with a small but non-negligible risk of serious complications. Multi-center randomized controlled trials (RCTs) are warranted to help stratify the risk of both SCI and CSFD-related complications in patients undergoing endovascular aortic procedures.
PubMed: 37817857
DOI: 10.21037/acs-2023-scp-17 -
Cancers May 2024Intramedullary melanocytomas are exceedingly rare, with only twenty-four cases reported up to now. They present as local invasive tumors despite their benign biological... (Review)
Review
BACKGROUND
Intramedullary melanocytomas are exceedingly rare, with only twenty-four cases reported up to now. They present as local invasive tumors despite their benign biological behavior. Attempting a complete safe resection often results in severe post-operative neurological deficits, as in our case presented here.
METHODS
A systematic review was conducted across the PubMed and Scopus databases including studies published till February 2024.
RESULTS
A total of 19 studies were included, encompassing 24 cases. A similar distribution between sexes was noted (M:F 13:11), with ages ranging from 19 to 79 years. The thoracic segment was most affected, and intermediate-grade melanocytoma (19 cases) was the most common histotype. Radiographically, intramedullary melanocytomas usually appear as hyperintense hemorrhagic lesions peripheral to the central canal with focal nodular enhancement. Intraoperatively, they are black-reddish to tan and are tenaciously adherent lesions. In the sampled studies, IONM employment was uncommon, and post-operative new-onset neurological deficits were described in 16 cases. Adjuvant RT was used in four cases and its value is debatable. Recurrence is common (10 cases), and adjuvant therapies (RT or repeated surgery) seem to play a palliative role.
CASE PRESENTATION
A 68-year-old woman presented with a three-year history of worsening spastic paraparesis and loss of independence in daily activities (McCormick grade 4). An MRI revealed an intramedullary tumor from Th5 to Th7, characterized by T1-weighted hyperintensity and signs of recent intralesional hemorrhage. Multimodal neuromonitoring, comprising the D-Wave, guided the resection of a black-tan-colored tumor with hyper-vascularization and strong adherence to the white matter. During final dissection of the lesion to obtain gross total resection (GTR), a steep decline in MEPs and D-Wave signals was recorded. Post-operatively, the patient had severe hypoesthesia with Th9 level and segmental motor deficits, with some improvement during neurorehabilitation. Histopathology revealed an intermediate-grade melanocytoma (CNS WHO 2021 classification). A four-month follow-up documented the absence of relapse.
CONCLUSIONS
This literature review highlights that intramedullary T1 hyperintense hemorrhagic thoracic lesions in an adult patient should raise the suspicion of intramedullary melanocytoma. They present as locally aggressive tumors, due to local invasiveness, which often lead to post-operative neurological deficits, and frequent relapses, which overwhelm therapeutic strategies leading to palliative care after several years.
PubMed: 38791946
DOI: 10.3390/cancers16101867 -
Spontaneous Spinal Hematoma in Patients Using Antiplatelets and Anticoagulants: A Systematic Review.World Neurosurgery Apr 2024Spontaneous spinal hematoma (SSH) is a debilitating complication in patients taking either antiplatelet (AP) or anticoagulation (AC) medications. SSH is rare and,...
BACKGROUND
Spontaneous spinal hematoma (SSH) is a debilitating complication in patients taking either antiplatelet (AP) or anticoagulation (AC) medications. SSH is rare and, therefore, a systematic review is warranted to re-examine and outline trends, clinical characteristics, and outcomes associated with SSH formation.
METHODS
PubMed, EMBASE, Scopus, and Web-of-Science were searched. Studies reporting clinical data of patients with SSH using AC medications were included. In addition, clinical studies meeting our a priori inclusion criteria limited to SSH were further defined in quality through risk bias assessment.
RESULTS
We included 10 studies with 259 patients' pooled data post-screening 3083 abstracts. Within the cohort (n = 259), the prevalence of idiopathic, nontraumatic SSH with concomitant treatment with AC medications was greater 191 (73.75%) compared with AP treatment (27%). The lumbar spine was the most common site of hematoma (41.70%), followed by the cervical (22.01%) and thoracic (8.49%) spine. Most patients had surgical intervention (70.27%), and 29.73% had conservative management. The pooled data suggest that immediate diagnosis and intervention are the best prognostic factors in clinical outcomes. American Spinal Injury Association grading at initial symptom onset and post-treatment showed the greatest efficacy in symptomatic relief (87.64%) and return of motor and sensory symptoms (39.19%).
CONCLUSIONS
Our review suggested that AC medications were related to SSH in most patients (74%), followed by APs (27%) and combined ACs + APs (1.9%). We recommend prompt intervention, a high suspicion for patients with neurologic deficits and diagnostic imaging before intervention to determine a case-specific treatment plan.
Topics: Humans; Anticoagulants; Hematoma, Epidural, Spinal; Spinal Cord Diseases; Lumbar Vertebrae; Risk Assessment; Magnetic Resonance Imaging
PubMed: 38278210
DOI: 10.1016/j.wneu.2024.01.082 -
Movement Disorders Clinical Practice Jun 2024As the diagnosis of Parkinson's disease (PD) is fundamentally clinical, the usefulness of ioflupane (I) single-photon emission computed tomography (SPECT) or DaTSCAN as... (Review)
Review
BACKGROUND
As the diagnosis of Parkinson's disease (PD) is fundamentally clinical, the usefulness of ioflupane (I) single-photon emission computed tomography (SPECT) or DaTSCAN as a diagnostic tool has been a matter of debate for years. The performance of DaTSCAN is generally recommended in the follow-up of patients with a clinically uncertain diagnosis, especially in those with a suspected essential tremor, drug-induced parkinsonism, or vascular parkinsonism. However, there is a dearth of DaTSCAN findings regarding neurodegenerative parkinsonisms besides PD and atypical parkinsonisms. To date, a specific nigrostriatal dopamine uptake pattern that would help differentiate PD from the most frequent atypical parkinsonisms is yet to be described. This fact is further complicated by the possible visualization of abnormalities in the uptake pattern in patients with rarer neurodegenerative parkinsonisms.
OBJECTIVES
We aimed to summarize the current literature regarding DaTSCAN findings in patients with rare neurodegenerative parkinsonisms.
METHODS
The PubMed database was systematically screened for studies in English or Spanish up to October 15, 2023, using search terms "DaTSCAN", "ioflupane", "DaT-SPECT", "123I-FP-CIT SPECT", "dopamine transporter imaging", and "[123I] FP-CIT SPECT". Duplicated publications and studies regarding PD, atypical parkinsonisms, dystonia-parkinsonism, essential tremor, and parkinsonism due to non-degenerative causes were excluded.
RESULTS
The obtained results were reviewed and summarized, including DaTSCAN findings in fragile X-associated tremor/ataxia syndrome, prion diseases, Huntington's disease, spinocerebellar ataxia, hereditary spastic paraparesis, metabolic disorders, and other diseases (anti-IgLON5 disease, ring chromosome 20 syndrome, chorea-acanthocytosis, and neuronal ceroid lipofuscinosis).
CONCLUSIONS
This review highlights the need to determine in the future the utility and cost-effectiveness of DaTSCAN, both as a diagnostic and a prognostic tool, in patients with parkinsonian symptoms in rare neurodegenerative diseases.
Topics: Humans; Tomography, Emission-Computed, Single-Photon; Parkinsonian Disorders; Tropanes; Parkinson Disease
PubMed: 38693679
DOI: 10.1002/mdc3.14055 -
Neurosurgical Review May 2024Surgery and endovascular therapy are the primary treatment options for spinal dural arteriovenous fistula (SDAVF). Due to the absence of a consensus regarding which... (Comparative Study)
Comparative Study
Surgery and endovascular therapy are the primary treatment options for spinal dural arteriovenous fistula (SDAVF). Due to the absence of a consensus regarding which therapy yields a superior outcome, we conducted a comparative analysis of the surgical and endovascular treatment of SDAVF through a multicenter case series and a systematic literature review. Patients with SDAVF, surgically or endovascularly treated at four neurosurgical centers from January 2001 to December 2021, were included in this study. Level of SDAVF, primary treatment modality, baseline and post-procedural neurological status were collected. The primary outcomes were failure, complication rates, and a newly introduced parameter named as therapeutic delay. A systematic review of the literature was performed according to PRISMA-P guidelines. The systematic review identified 511 papers, of which 18 were eligible for analysis, for a total of 814 patients, predominantly male (72%) with a median age of 61 and mainly thoracic SDAVFs (65%). The failure rate was significantly higher for endovascular therapy (20%) compared to surgery (4%) (p < 0.01). Neurological complications were generally rare, with similar rates among the two groups (endovascular 2.9%; surgery 2.6%). Endovascular treatment showed a statistically significantly higher rate of persistent neurological complications than surgical treatment (2.9% versus 0.2%; p < 0.01). Both treatments showed similar rates of clinical improvement based on Aminoff Logue scale score. The multicenter, retrospective study involved 131 patients. The thoracic region was the most frequent location (58%), followed by lumbar (37%). Paraparesis (45%) and back pain (41%) were the most common presenting symptoms, followed by bladder dysfunction (34%) and sensory disturbances (21%). The mean clinical follow-up was 21 months, with all patients followed for at least 12 months. No statistically significant differences were found in demographic and clinical data, lesion characteristics, or outcomes between the two treatment groups. Median pre-treatment Aminoff-Logue score was 2.6, decreasing to 1.4 post-treatment with both treatments. The mean therapeutic delay for surgery and endovascular treatment showed no statistically significant difference. Surgical treatment demonstrated significantly lower failure rates (5% vs. 46%, p < 0.01). In the surgical group, 2 transient neurological (1 epidural hematoma, 1 CSF leak) and 3 non-neurological (3 wound infections) complications were recorded; while 2 permanent neurological (spinal infarcts), and 5 non-neurological (inguinal hematomas) were reported in the endovascular group. According to the literature review and this multicenter clinical series, surgical treatment has a significantly lower failure rate than endovascular treatment. Although the two treatments have similar complication rates, endovascular treatment seems to have a higher rate of persistent neurological complications.
Topics: Humans; Central Nervous System Vascular Malformations; Endovascular Procedures; Neurosurgical Procedures; Male; Female; Middle Aged; Treatment Outcome; Aged; Postoperative Complications; Embolization, Therapeutic
PubMed: 38713376
DOI: 10.1007/s10143-024-02443-8 -
Clinical and Experimental Pediatrics Jul 2023The peripartum period is both a highly vulnerable stage and a significant indicator of a population's health status. Interest is increasing in human T-cell lymphotropic...
BACKGROUND
The peripartum period is both a highly vulnerable stage and a significant indicator of a population's health status. Interest is increasing in human T-cell lymphotropic virus type-1 (HTLV-1) transmission due to its adverse health impacts. However, nationally representative data on HTLV-1 that are important for health planning are unavailable for this subpopulation.
PURPOSE
This study aimed to conduct a pooled estimate of HTLV-1 prevalence among pregnant women in Nigeria to quantify its clinical burden and public health implications.
METHODS
This systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis 2020 statement.
RESULTS
After a systematic review of the Nigerian literature, 12 studies (2,821 pregnant or postnatal women) were included in the final evidence synthesis. The estimated HTLV-1 prevalence in Nigerian peripartum women following a positive screening test by enzyme-linked immunosorbent assay was 5.44% (95% confidence interval [CI], 3.16%-9.20%). A subgroup analysis of the 2 major regions showed a slightly higher prevalence in the Western versus Southern region (5.55% [95% CI, 2.49%-11.87%]; and 4.91% [95% CI, 2.11%-11.02%]; P=0.84). However, a subgroup analysis by geopolitical zone revealed that Southwestern and Northwestern Nigeria had the highest prevalence (9.23% [95% CI, 4.35%-18.55%; I2=93%] and 7.15% [95% CI, 1.54%-27.54%]; I2=92%). Our decade-old subgroup analysis found inconsistencies in the HTLV-1 prevalence. Furthermore, our literature review revealed a prevalence of HTLV infection among patients with various clinical types of lymphomas/leukemias and myelopathy of 2%-22%.
CONCLUSION
These findings have important implications in defining the epidemiological patterns of HTLV-1 infection in Nigeria. They also suggest the presence of HTLV-endemic clusters near low-endemic areas, even within the same geopolitical zones.
PubMed: 36550773
DOI: 10.3345/cep.2022.00710