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Lupus Sep 2023Neurological involvement can occur in systemic lupus erythematosus (SLE) due to co-existing neuromyelitis optica spectrum disorder (NMOSD). The symptoms can mimic those...
BACKGROUND
Neurological involvement can occur in systemic lupus erythematosus (SLE) due to co-existing neuromyelitis optica spectrum disorder (NMOSD). The symptoms can mimic those of neuropsychiatric manifestations of SLE. Pathogenic anti-aquaporin-4 (AQP4) antibodies, commonly found in NMOSD, are responsible for the neuroinflammatory response and secondary demyelinating lesions. These anti-AQP4 antibodies can be the drivers of neuroinflammatory process in SLE patients, which is distinct from the immunopathogenesis seen in traditional neuropsychiatric SLE. The clinical course is often a relapsing one and is managed differently. In this review, we describe and outline the clinical course and outcomes of AQP4+ NMOSD/SLE overlap cases.
METHODS
To investigate the co-existence of SLE with AQP4+NMOSD, we conducted a systematic review of individual patient data from case reports and case series reported in major databases. The study extracted clinic-demographic features, imaging and laboratory profiles, treatment approaches, and outcomes of these patients. Inclusion criteria for the review required patients to have positivity for AQP4 or NMO in the blood and/or cerebrospinal fluid (CSF) and exhibit at least one manifestation of both NMOSD and SLE.
RESULTS
In this overlap between SLE and AQP4+NMOSD, a high female preponderance was observed, with 42 out of 46 patients (91.3%) being female. Nearly half of the NMOSD cases (47.8%) had onset after lupus, with a median of 5 years between the two diagnoses. Hematological manifestations were seen in the majority of patients (63%), as well as longitudinally extensive transverse myelitis (87%), and brainstem involvement on imaging (29.6%). Cerebrospinal fluid analysis showed a dominantly lymphocytic pleocytosis, with oligoclonal bands being reported scarcely. Although cyclophosphamide was the most common steroid sparing agent used for maintenance, robust evidence for both efficacy and safety in AQP4+NMOSD is available for mycophenolate mofetil, azathioprine, and rituximab. The majority of reported cases showed a relapsing course, while one patient had a monophasic course.
CONCLUSION
AQP4+NMOSD in SLE patients is a relapsing and neurologically disabling disorder that can mimic neuropsychiatric manifestations, frequently occurs after the onset of lupus or may predate, responds to immunosuppressants, and necessitates indefinite treatment.
Topics: Humans; Female; Male; Neuromyelitis Optica; Lupus Erythematosus, Systemic; Neoplasm Recurrence, Local; Aquaporin 4; Syndrome; Disease Progression; Autoantibodies
PubMed: 37487596
DOI: 10.1177/09612033231191180 -
Pediatrics Dec 2021Peripheral nerve injuries in children can result in devastating lifelong deficits. Because of the time-sensitive nature of muscle viability and the limited speed of...
Peripheral nerve injuries in children can result in devastating lifelong deficits. Because of the time-sensitive nature of muscle viability and the limited speed of nerve regeneration, early recognition and treatment of nerve injuries are essential to restore function. Innovative surgical techniques have been developed to combat the regenerative length and speed; these include nerve transfers. Nerve transfers involve transferring a healthy, expendable donor nerve to an injured nerve to restore movement and sensation. Nerve transfers are frequently used to treat children affected by conditions, including UE trauma, brachial plexus birth injury, and acute flaccid myelitis. Pediatricians play an important role in the outcomes of children with these conditions through early diagnosis and timely referrals. With this review, we aim to provide awareness of state-of-the-art surgical treatment options that significantly improve the function of children with traumatic nerve injuries, brachial plexus birth injury, and acute flaccid myelitis.
Topics: Child; Compartment Syndromes; Forearm Injuries; Humans; Humeral Fractures; Median Nerve; Myelitis; Neonatal Brachial Plexus Palsy; Nerve Regeneration; Nerve Transfer; Neurosurgical Procedures; Peripheral Nerve Injuries; Peripheral Nerves; Radial Nerve; Plastic Surgery Procedures; Recovery of Function; Time Factors; Ulnar Nerve
PubMed: 34851416
DOI: 10.1542/peds.2021-052556 -
Brain Injury Oct 2020Following the outbreak of coronavirus 2019 (COVID-19), there is strong evidence of neurological involvement in these patients. We aimed to determine the clinical... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Following the outbreak of coronavirus 2019 (COVID-19), there is strong evidence of neurological involvement in these patients. We aimed to determine the clinical characteristics of neurological manifestations in COVID-19.
METHOD
A systematic review of studies reporting neurological manifestations published between 1 December, 2019 and 11 May, 2020 was performed. Studies were grouped based on neurological manifestation. Pooled analyses of individual patient's clinical characteristics and olfactory and gustatory dysfunction prevalence were performed.
RESULTS
Of 486 studies identified, 48 were included. 70 patients with 73 neurological manifestations were reported. 39 (53.4%) patients had stroke, 18 (24.7%) had Guillain-Barré syndrome and variants, 11 (15.1%) had meningitis, encephalitis, encephalopathy, or myelitis, and five (6.8%) had seizures. They had a mean age of 61.9 ± 17.7 years (60.6% male). Neurological disease occurred 8.1 ± 6.8 days from initial symptoms. Average mortality rate was 17.8%. Stroke has a mortality rate of 25.6%. Olfactory and gustatory dysfunction occurred in 59.9% and 57.5%, respectively.
CONCLUSIONS
Stroke is the most frequently reported neurological manifestation in COVID-19 and has the highest mortality rate. Neurological manifestations tend to develop one to two weeks after the onset of respiratory disease. There is significant morbidity and mortality associated with COVID-19 neurological manifestations.
Topics: COVID-19; Cerebrovascular Disorders; Encephalitis; Guillain-Barre Syndrome; Humans; Nervous System Diseases
PubMed: 33074036
DOI: 10.1080/02699052.2020.1831606 -
Expert Review of Vaccines Jun 2021: Established in 1994, Pakistan's polio program demonstrated early success. However, despite over 120 supplementary immunization activities in the last decade, polio...
: Established in 1994, Pakistan's polio program demonstrated early success. However, despite over 120 supplementary immunization activities in the last decade, polio eradication efforts in Pakistan have been unable to achieve their objective of halting polio transmission. Variable governance, and inconsistent leadership and accountability have hindered the success of the polio program and the quality of the campaigns. Insecurity and terrorism has interrupted polio activities, and community fears and misbeliefs about polio vaccinations continue to persist.: The article consists of a systematic review of the barriers and facilitators associated with the delivery of polio eradication activities in Pakistan. We also provide a comprehensive review of the policy and programmatic decisions made by the Pakistan Polio Programme since 1994. Searches were conducted on Embase and Medline databases and 25 gray literature sources.: Polio eradication efforts must be integrated with other preventive health services, particularly immunization services. Addressing the underlying causes of polio refusals including underdevelopment and social exclusion will help counteract resistance to polio vaccination. Achieving polio eradication will require building health systems that provide comprehensive community-centered care, and improving governance and systems of accountability.
Topics: Disease Eradication; Humans; Immunization Programs; Pakistan; Policy; Poliomyelitis
PubMed: 33896306
DOI: 10.1080/14760584.2021.1915139 -
Multiple Sclerosis and Related Disorders Aug 2019Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory and autoimmune disorder of the central nervous system that typically presents with optic neuritis and... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory and autoimmune disorder of the central nervous system that typically presents with optic neuritis and myelitis. Azathioprine (AZA) is one of the available immunotherapies with purported beneficial effects for patients with NMOSD. At present, there are no systematic reviews that extensively pooled the effects of AZA compared to other interventions for this condition. The objective of this study, therefore, is to determine the efficacy and safety of AZA in patients with NMOSD using systematic review of relevant studies.
METHODS
Major health electronic databases, which included CENTRAL, MEDLINE, EMBASE, Scopus, LILACS, ClinicalTrials.gov, and HERDIN, were searched from May 2017 to November 2018 for relevant studies involving adult and pediatric patients with NMOSD. Randomized controlled trials, and either prospective or retrospective cohort designs that assessed the reduction or prevention of relapse or disability and the occurrence of adverse events related to AZA use compared to placebo or to other active drugs were considered. Assessment of risk of bias was performed using the Cochrane Collaboration tool and Newcastle-Ottawa Scale.
RESULTS
From a total of 273 records, 9 relevant studies (1 randomized controlled trial (RCT), 3 prospective cohort studies, 5 retrospective studies) which involved a total of 977 patients, were included. One RCT and several observational studies revealed that AZA regimen may be inferior to rituximab in terms of annualized relapse rate, reduction of disability as measured by the expanded disability status scale (EDSS), risk for relapse and relapse-free rate. Efficacy data were very limited in the comparison of AZA to mycophenolate mofetil (MMF), to cyclophosphamide, and to interferon-β for patients with NMOSD. Occurrence of any adverse event, elevated liver enzymes/hepatoxicity, leukopenia and hair loss associated with AZA use were significantly greater compared to MMF, which may lead to medication noncompliance.
CONCLUSION
AZA improves relapses and disability in patients with NMOSD but this regimen is associated with relatively frequent adverse events based on limited published evidences. More well-conducted clinical trials are necessary to establish with certainty the beneficial and harmful effects of AZA in patients with NMOSD.
Topics: Azathioprine; Humans; Immunosuppressive Agents; Neuromyelitis Optica
PubMed: 31136907
DOI: 10.1016/j.msard.2019.05.011 -
Neurological Sciences : Official... Nov 2023Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy with evidence of neuroinflammation and demyelination that affects the central nervous... (Review)
Review
The temporal relationship of paraneoplastic aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD) and breast cancer: a systematic review and meta-analysis.
OBJECTIVE
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy with evidence of neuroinflammation and demyelination that affects the central nervous system and is mediated by aquaporin-4 (AQP4) immunoglobulin (IgG). AQP4-IgG may also be present in paraneoplastic syndromes secondary to malignancy such as breast cancer.
METHODS
A systematic review and meta-analysis of the literature were completed using PubMed, Scopus, and ScienceDirect databases (CRD42022352109).
RESULTS
A total of 12 publications, which included 19 cases, met the inclusion criteria and were assessed in both the qualitative and quantitative synthesis. The mean age was 51.26 years (SD: 13.12, SEM: 3.01), and 100% of the cases were reported in women. Speech abnormalities and symptoms of myelopathy were the most observed neurological manifestations. MRI often revealed longitudinally extensive transverse myelitis (LETM) involving the cervical spine. Three of 19 (15.9%) cases were diagnosed with NMOSD and breast cancer within the same month. Five of 19 (26.1%) cases had a diagnosis of breast cancer preceding that of NMOSD. Eight of 19 (42.1%) cases were diagnosed with breast cancer after NMOSD. The median time of breast cancer diagnosis was 1.0 months (range 216 months) after NMOSD.
CONCLUSIONS
The diagnosis of breast cancer most often occurs after the onset of the paraneoplastic NMOSD symptoms. However, a wide time range for the diagnosis of breast cancer was observed both before and after the onset of neurological symptoms. Older women with a new diagnosis of NMOSD should be considered for frequent breast cancer screening.
PubMed: 37453952
DOI: 10.1007/s10072-023-06952-0 -
Journal of Rehabilitation Medicine Apr 2021To evaluate and assess the effectiveness of muscle strengthening and cardiovascular interventions in improving outcomes in poliomyelitis (polio) survivors. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To evaluate and assess the effectiveness of muscle strengthening and cardiovascular interventions in improving outcomes in poliomyelitis (polio) survivors.
DATA SOURCES
A systematic literature search was conducted in Medline, PubMed, CINAHL, PsychINFO, Web of Science, and Google Scholar for experimental and observational studies. Study selection and extraction: Screening, data-extraction, risk of bias and quality assessment were carried out independently by the authors. The quality appraisal and risk of bias were assessed using the Downs and Black Checklist. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement was followed to increase clarity of reporting.
DATA SYNTHESIS
A total of 21 studies that met all the inclusion criteria were subjected to statistical analyses according to intervention (muscle strengthening or cardiovascular fitness). A random-effects meta-analysis showed a statistically significant effect for the exercise interventions favouring improvement in outcomes according to the International Classification of Functioning, Disability and Health (ICF).
CONCLUSION
This review provides further insight into the effects associated with muscle strengthening and cardiovascular interventions among polio survivors, and helps to further identify the current state of research in this area. Future research is needed, focusing on individualized approaches to exercise with polio survivors and specific exercise prescription recommendations, based on established frameworks, such as the ICF.
Topics: Cardiovascular Diseases; Exercise Therapy; Female; Humans; Male; Muscle Strength; Poliomyelitis; Survivors
PubMed: 33876251
DOI: 10.2340/16501977-2832 -
Neuropsychiatric Disease and Treatment 2020To evaluate the use of immunosuppressive treatment, clinical outcome and diagnostic strategy in patients with systemic lupus erythematosus (SLE) presenting with clinical...
PURPOSE
To evaluate the use of immunosuppressive treatment, clinical outcome and diagnostic strategy in patients with systemic lupus erythematosus (SLE) presenting with clinical features of transverse myelitis (TM), but normal MRI of the spinal cord (sMRI) and normal cerebrospinal fluid (CSF) assessment, and to suggest a clinical guideline.
PATIENTS AND METHODS
All patients with SLE and clinical features compatible with (sub)acute TM visiting the NPSLE clinic of the LUMC between 2007 and 2020 were included. Information on baseline characteristics, investigations, treatment and outcomes was collected from electronic medical records. In addition, a systematic review of individual participant data was performed up to April 2020 in PubMed, Embase and Web of Science, identifying all patients with TM, SLE and sMRI assessment. Data regarding sMRI, CSF analysis, treatment and outcome were extracted, and outcome was compared between patients with normal sMRI and CSF (sMRI-/CSF-) and patients with abnormalities.
RESULTS
Twelve SLE patients with a clinical diagnosis of TM were identified: four sMRI-/CSF- and one sMRI- with CSF not available. All patients received immunosuppressive treatment, but outcome in sMRI-/CSF- patients was worse: no recovery (n=1) or partial recovery (n=3) compared to partial recovery (n=4) and (nearly) complete recovery (n=3) in MRI+ patients. The systematic literature review yielded 146 articles eligible for inclusion, 90% case reports. A total of 427 SLE patients with TM were identified, of which only four cases were sMRI-/CSF- (1%), showing no improvement (n=1), partial improvement (n=2) and complete recovery (n=1) after immunosuppressive treatment.
CONCLUSION
Outcome in SLE patients presenting with clinically suspected TM with normal sMRI and CSF is less favorable, despite treatment with immunosuppressive therapy. Taking a functional neurological disorder into consideration may be helpful in order to start other therapeutic strategies. We suggest prescribing immunosuppressive treatment for a restricted period of time to evaluate its effect in cases where a functional disorder initially is considered unlikely.
PubMed: 33376333
DOI: 10.2147/NDT.S267000 -
Neurology(R) Neuroimmunology &... Sep 2023Glial fibrillary acidic protein (GFAP) antibodies can associate with an astrocytopathy often presenting as a meningoencephalitis. Visual involvement has been reported...
BACKGROUND AND OBJECTIVES
Glial fibrillary acidic protein (GFAP) antibodies can associate with an astrocytopathy often presenting as a meningoencephalitis. Visual involvement has been reported but scarcely defined. We describe 2 cases of GFAP astrocytopathy with predominant visual symptoms and present a systematic review of the literature.
METHODS
We describe 2 patients with GFAP astrocytopathy from our neurology department. We performed a systematic review of the literature according to PRISMA guidelines, including all patients with this disease and available clinical data, focusing on visual involvement.
RESULTS
Patient 1 presented with bilateral optic disc edema and severe sudden bilateral loss of vision poorly responsive to therapy. Patient 2 showed bilateral optic disc edema, headache, and mild visual loss with complete recovery after steroids. We screened 275 records and included 84 articles (62 case reports and 22 case series) for a total of 592 patients. Visual involvement was reported in 149/592 (25%), with either clinical symptoms or paraclinical test-restricted abnormalities. Bilateral optic disc edema was found in 80/159 (50%) of patients investigated with fundoscopy, among which 49/80 (61%) were asymptomatic. One hundred (100/592, 17%) reported visual symptoms, often described as blurred vision or transient visual obscurations. Optic neuritis was rare and diagnosed in only 6% of all patients with GFAP astrocytopathy, often without consistent clinical and paraclinical evidence to support the diagnosis. Four patients (including patient 1) manifested a severe, bilateral optic neuritis with poor treatment response. In patients with follow-up information, a relapsing disease course was more frequently observed in those with vs without visual involvement (35% vs 11%, = 0.0035, OR 3.6 [CI 1.44-8.88]).
DISCUSSION
Visual system involvement in GFAP astrocytopathy is common and heterogeneous, ranging from asymptomatic bilateral optic disc edema to severe bilateral loss of vision, but optic neuritis is rare. GFAP CSF antibody testing should be considered in patients with encephalitis/meningoencephalitis or myelitis and bilateral optic disc edema, even without visual symptoms, and in patients with severe bilateral optic neuritis, especially when AQP4 antibodies are negative. Visual symptoms might associate with a higher relapse risk and help to identify patients who may require chronic immunosuppression.
Topics: Humans; Papilledema; Glial Fibrillary Acidic Protein; Meningoencephalitis; Optic Neuritis; Antibodies
PubMed: 37582612
DOI: 10.1212/NXI.0000000000200146 -
The Journal of Infectious Diseases Feb 2022Pertussis, diphtheria, and tetanus (DTP)-containing vaccines combined with polio vaccines are recommended by the World Health Organization as part of routine... (Meta-Analysis)
Meta-Analysis
Pertussis, diphtheria, and tetanus (DTP)-containing vaccines combined with polio vaccines are recommended by the World Health Organization as part of routine immunization programs. The decline of immunity after vaccination has been considered as a possible reason for the reemergence of vaccine-preventable diseases worldwide. In this study, we evaluated the potential duration of protective immunity of pertussis, diphtheria, tetanus, and polio through a systematic review and meta-analysis. We examined data on immunological and clinical outcomes. We observed evidence of waning postvaccination immunity for pertussis and diphtheria, whereas tetanus and polio vaccines provided sustained protection. Further research on the risk factors of waning immunity after vaccination and the optimal timing of booster doses for pertussis and diphtheria is needed.
Topics: Antibodies, Bacterial; Diphtheria; Diphtheria-Tetanus-Pertussis Vaccine; Diphtheria-Tetanus-acellular Pertussis Vaccines; Humans; Immunization, Secondary; Poliomyelitis; Tetanus; Vaccination; Vaccines, Combined; Whooping Cough
PubMed: 34543411
DOI: 10.1093/infdis/jiab480