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European Journal of Neurology Jul 2024This study was undertaken to provide a comprehensive review of neuroimaging characteristics and corresponding clinical phenotypes of autoimmune glial fibrillary acidic... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
This study was undertaken to provide a comprehensive review of neuroimaging characteristics and corresponding clinical phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A), a rare but severe neuroinflammatory disorder, to facilitate early diagnosis and appropriate treatment.
METHODS
A PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis)-conforming systematic review and meta-analysis was performed on all available data from January 2016 to June 2023. Clinical and neuroimaging phenotypes were extracted for both adult and paediatric forms.
RESULTS
A total of 93 studies with 681 cases (55% males; median age = 46, range = 1-103 years) were included. Of these, 13 studies with a total of 535 cases were eligible for the meta-analysis. Clinically, GFAP-A was often preceded by a viral prodromal state (45% of cases) and manifested as meningitis, encephalitis, and/or myelitis. The most common symptoms were headache, fever, and movement disturbances. Coexisting autoantibodies (45%) and neoplasms (18%) were relatively frequent. Corticosteroid treatment resulted in partial/complete remission in a majority of cases (83%). Neuroimaging often revealed T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities (74%) as well as perivascular (45%) and/or leptomeningeal (30%) enhancement. Spinal cord abnormalities were also frequent (49%), most commonly manifesting as longitudinally extensive myelitis. There were 88 paediatric cases; they had less prominent neuroimaging findings with lower frequencies of both T2/FLAIR hyperintensities (38%) and contrast enhancement (19%).
CONCLUSIONS
This systematic review and meta-analysis provide high-level evidence for clinical and imaging phenotypes of GFAP-A, which will benefit the identification and clinical workup of suspected cases. Differential diagnostic cues to distinguish GFAP-A from common clinical and imaging mimics are provided as well as suitable magnetic resonance imaging protocol recommendations.
Topics: Humans; Astrocytes; Autoantibodies; Autoimmune Diseases of the Nervous System; Glial Fibrillary Acidic Protein; Neuroimaging; Neuroinflammatory Diseases; Phenotype
PubMed: 38506182
DOI: 10.1111/ene.16284 -
European Journal of Neurology Jun 2022Neurosarcoidosis can affect all parts of the nervous system of which myelitis is relatively frequent. The aim of this study was to describe clinical characteristics,... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND PURPOSE
Neurosarcoidosis can affect all parts of the nervous system of which myelitis is relatively frequent. The aim of this study was to describe clinical characteristics, treatment and prognosis of patients with myelitis attributable to neurosarcoidosis.
METHODS
We performed a retrospective cohort study and a systematic review and meta-analysis of neurosarcoidosis-associated myelitis.
RESULTS
Myelitis was identified in 41 of 153 (27%) neurosarcoidosis patients seen at our clinic from 2015 to 2020. Classification of neurosarcoidosis was definite in three (7%), probable in 29 (71%) and possible in nine patients (22%). The median (interquartile range) age at onset was 49 (41-53) years and 20 of the patients were female (49%). The presenting symptoms included muscle weakness in 31 of 41 patients (78%), sensory loss in 35 (88%) and micturition abnormalities in 30 (75%). Spinal magnetic resonance imaging showed longitudinally extensive myelitis in 27 of 36 patients (75%) and cerebrospinal fluid examination showed an elevated leukocyte count in 21 patients (81%). Initial treatment consisted of glucocorticoids in 38 of 41 patients (93%), with additional methotrexate or azathioprine in 21 of 41 patients (51%) and infliximab in 10 of 41 patients (24%). Treatment led to remission, improvement or stabilization of disease in 37 of 39 patients (95%). Despite treatment, 18 of 30 patients (60%) could not walk independently at the end of follow-up (median 36 months). A review of the literature published between 2000 and 2020 identified 215 patients with comparable clinical characteristics and results of ancillary investigations.
CONCLUSION
Sarcoidosis-associated myelitis is observed in 27% of neurosarcoidosis patients. Although treatment often led to a decrease in disease activity, residual neurological deficits leading to loss of ambulation occurred frequently.
Topics: Central Nervous System Diseases; Female; Humans; Magnetic Resonance Imaging; Male; Myelitis; Retrospective Studies; Sarcoidosis
PubMed: 35189010
DOI: 10.1111/ene.15295 -
Autoimmunity Reviews Feb 2022Transverse myelitis (TM) is a rare but severe systemic lupus erythematosus (SLE) manifestation. To date, the prognostic factors for SLE-associated TM have been far less... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Transverse myelitis (TM) is a rare but severe systemic lupus erythematosus (SLE) manifestation. To date, the prognostic factors for SLE-associated TM have been far less well-studied. There are also controversial data on the association of antiphospholipid antibodies (aPLs), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score, longitudinal extensive transverse myelitis (LETM), and decreased complement levels with the outcome of TM. We aimed to review the potential prognostic factors and integrate relapse rates of observational studies for SLE-associated TM.
METHOD
To review the prognosis for SLE-associated TM, relevant articles published up to July 30, 2021, were comprehensively and systematically identified from PubMed, EMBASE and Web of Science databases. Five studies encompassing 283 patients with SLE-related TM were included in this meta-analysis; raw data were obtained from three studies.
RESULTS
The risk factors for unfavorable neurological outcome included demographic features, clinical characteristics, laboratory data, among which a grade of A, B or C on the American Spinal Injury Association Impairment Scale (AIS) at the onset of TM was associated with poor prognosis (OR: 56.05, 95% CI: 6.29-499.25, P < 0.001). The presence of hypoglycorrhachia was also correlated with a worse prognosis (OR: 10.78, 95% CI: 3.74-31.07, P < 0.001). No noticeable correlation was revealed between a poor outcome and positive aPLs and different aPLs profiles (anticardiolipin antibody [aCL], anti-β2-glycoprotein I (anti-β2GPI], lupus anticoagulant [LA]). The pooled 1-, 3- and 5-year relapse rates were 22% (95% CI: 0.13-0.31), 34% (95% CI: 0.22-0.47) and 36% (95% CI: 0.14-0.58), respectively. No significant publication bias was found.
CONCLUSION
A grade of A, B, or C on the AIS at initial TM and the presence of hypoglycorrhachia were found to be related to a worse prognosis in patients with SLE-associated TM. Notably, aPLs and different aPLs profiles may not suggest poor neurological outcome. The long-term relapse rate of patients with SLE-associated TM was relatively high. We recommend that treatment be stratified based on the initial severity of myelitis. For patients with severe myelitis, early intensive therapy may be initiated as soon as possible.
Topics: Humans; Lupus Erythematosus, Systemic; Myelitis, Transverse; Neoplasm Recurrence, Local; Prognosis; Risk Factors
PubMed: 34798313
DOI: 10.1016/j.autrev.2021.102996 -
Acute Flaccid Myelitis: Review of Clinical Features, Diagnosis, and Management with Nerve Transfers.Plastic and Reconstructive Surgery Jan 2023Acute flaccid myelitis (AFM) is a devastating neurologic condition in children, manifesting as acute limb weakness and/or paralysis. Despite increased awareness of AFM...
BACKGROUND
Acute flaccid myelitis (AFM) is a devastating neurologic condition in children, manifesting as acute limb weakness and/or paralysis. Despite increased awareness of AFM following initiation of U.S. surveillance in 2014, no treatment consensus exists. The purpose of this systematic review was to summarize the most current knowledge regarding AFM epidemiology, cause, clinical features, diagnosis, and supportive and operative management, including nerve transfer.
METHODS
The authors systematically reviewed the literature based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using multiple databases to search the keywords ("acute flaccid myelitis"), ('acute flaccid myelitis'/exp OR 'acute flaccid myelitis'), and (Acute AND flaccid AND myelitis). Included articles reported on (1) AFM diagnosis and (2) patient-specific data regarding epidemiology, cause, clinical features, diagnostic features, or management of AFM.
RESULTS
Ninety-nine articles were included in this review. The precise cause and pathophysiologic mechanism of AFM remain undetermined, but AFM is strongly associated with nonpolio enterovirus infections. Clinical presentation typically comprises preceding viral prodrome, pleocytosis, spinal cord lesions on T2-weighted magnetic resonance imaging, and acute onset of flaccid weakness/paralysis with hyporeflexia in at least one extremity. Supportive care includes medical therapy and rehabilitation. Early studies of nerve transfer for AFM have shown favorable outcomes for patients with persistent weakness.
CONCLUSIONS
Supportive care and physical therapy are the foundation of a multidisciplinary approach to managing AFM. For patients with persistent limb weakness, nerve transfer has shown promise for improving function in distal muscle groups. Surgeons must consider potential spontaneous recovery, patient selection, donor nerve availability, recipient nerve appropriateness, and procedure timing.
Topics: Child; Humans; Nerve Transfer; Neuromuscular Diseases; Myelitis; Paralysis; Muscle Hypotonia
PubMed: 36219869
DOI: 10.1097/PRS.0000000000009788 -
Child's Nervous System : ChNS :... Mar 2022Acute flaccid myelitis (AFM) is a rare disease that commonly affects young children. AFM's pathophysiology involves loss of lower motor neurons following a viral... (Review)
Review
BACKGROUND
Acute flaccid myelitis (AFM) is a rare disease that commonly affects young children. AFM's pathophysiology involves loss of lower motor neurons following a viral infection and induces acute asymmetric flaccid paralysis most commonly in the upper extremities. Nerve transfers have emerged as a treatment option for these patients with permanent motor deficits.
OBJECTIVE
To summarize the literature and report safety and efficacy outcomes following nerve transfers for recovery of shoulder abduction and external rotation, and elbow flexion and extension in pediatric patients with AFM. Recovery of at least antigravity function was defined as a successful outcome. This systematic review was performed according to the PRISMA guidelines. The PubMed, Embase and Cochrane databases were utilized.
RESULTS
Five studies comprising 44 patients (median age 2.95 years; 71% male), and 93 upper extremity nerve transfers were included. Thirty-eight patients received 65 nerve transfer procedures aiming for recovery of shoulder abduction and/or external rotation with a transfer to the axillary and/or suprascapular nerve. The recovery of shoulder abduction and external rotation was achieved in 40.7% (n = 11/27) and 60% (n = 6/10) of patients, respectively. Time from injury to surgery showed an inverse relationship with the odds for successful recovery (OR: 0.81; 95% CI: 0.64-1.02; p = 0.07); however, statistical significance was not reached. Successful recovery of elbow flexion with a transfer to the musculocutaneous was reported at a rate of 92.3% (n = 12/13). Successful re-innervation of the radial nerve with recovery of elbow extension was found in 75% (n = 6/8) of patients. No complications were reported.
CONCLUSIONS
Upper extremity nerve transfers appear to be promising and safe for AFM patients. Shoulder abduction is the most challenging upper extremity function to recover. Further studies are warranted to identify whether nerve transfers are associated with superior outcomes when performed earlier.
Topics: Brachial Plexus Neuropathies; Central Nervous System Viral Diseases; Child; Child, Preschool; Female; Humans; Male; Myelitis; Nerve Transfer; Neuromuscular Diseases; Range of Motion, Articular; Recovery of Function; Upper Extremity
PubMed: 34982205
DOI: 10.1007/s00381-021-05419-x -
PLoS Neglected Tropical Diseases Feb 2022A substantial amount of epidemiological data has been reported on Enterovirus D68 (EV-D68) infections after the 2014 outbreak. Our goal was to map the case fatality rate... (Meta-Analysis)
Meta-Analysis
A substantial amount of epidemiological data has been reported on Enterovirus D68 (EV-D68) infections after the 2014 outbreak. Our goal was to map the case fatality rate (CFR) and prevalence of current and past EV-D68 infections. We conducted a systematic review (PROSPERO, CRD42021229255) with published articles on EV-68 infections in PubMed, Embase, Web of Science and Global Index Medicus up to January 2021. We determined prevalences using a model random effect. Of the 4,329 articles retrieved from the databases, 89 studies that met the inclusion criteria were from 39 different countries with apparently healthy individuals and patients with acute respiratory infections, acute flaccid myelitis and asthma-related diseases. The CFR estimate revealed occasional deaths (7/1353) related to EV-D68 infections in patients with severe acute respiratory infections. Analyses showed that the combined prevalence of current and past EV-D68 infections was 4% (95% CI = 3.1-5.0) and 66.3% (95% CI = 40.0-88.2), respectively. The highest prevalences were in hospital outbreaks, developed countries, children under 5, after 2014, and in patients with acute flaccid myelitis and asthma-related diseases. The present study shows sporadic deaths linked to severe respiratory EV-D68 infections. The study also highlights a low prevalence of current EV-D68 infections as opposed to the existence of EV-D68 antibodies in almost all participants of the included studies. These findings therefore highlight the need to implement and/or strengthen continuous surveillance of EV-D68 infections in hospitals and in the community for the anticipation of the response to future epidemics.
Topics: Antibodies, Viral; Asthma; Central Nervous System Viral Diseases; Enterovirus D, Human; Enterovirus Infections; Humans; Myelitis; Neuromuscular Diseases; Prevalence; Respiratory Tract Infections
PubMed: 35134062
DOI: 10.1371/journal.pntd.0010073 -
BMC Neurology Mar 2015Postpolio syndrome (PPS) is characterized by progressive disabilities that develop decades after prior paralytic poliomyelitis. Because chronic inflammation may be the... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Postpolio syndrome (PPS) is characterized by progressive disabilities that develop decades after prior paralytic poliomyelitis. Because chronic inflammation may be the process underlying the development of PPS, immunomodulatory management, such as intravenous immunoglobulin (IVIg) administration, may be beneficial.
METHODS
We performed a systematic review and meta-analysis of published randomized controlled trials (RCTs) and prospective studies that evaluated the efficacy of IVIg in managing PPS. Electronic databases, including PubMed, EMBASE, CINAHL, and the Cochrane Central Register of Controlled Trials, were searched for articles on PPS published before December 2014. The primary outcomes were pain severity, fatigue scores, and muscle strength. The secondary outcomes were physical performance, quality of life (QoL), and cytokine expression levels.
RESULTS
We identified 3 RCTs involving 241 patients and 5 prospective studies involving 267 patients. The meta-analysis of pain severity (weighted mean difference [WMD] = -1.02, 95% confidence interval [CI] = -2.51 to 0.47), fatigue scores (WMD = 0.28, 95% CI -0.56 to 1.12), and muscle strength revealed no significant differences between the IVIg and the placebo group. Regarding QoL, the RCTs yielded controversial outcomes, with improvement in only certain domains of the Short Form 36 (SF-36). Moreover, one prospective study reported significant improvement on SF-36, particularly in patients aged younger than 65 years, those with paresis of the lower limbs, and high pain intensity.
CONCLUSION
The present review indicated that IVIg is unlikely to produce significant improvements in pain, fatigue, or muscle strength. Thus, routinely administering IVIg to patients with PPS is not recommended based on RCTs. However, a potential effect in younger patients with lower limbs weakness and intense pain requires confirmation from further well-structured trials.
Topics: Fatigue; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Muscle Strength; Muscle Weakness; Pain; Postpoliomyelitis Syndrome; Prospective Studies; Quality of Life; Treatment Outcome
PubMed: 25886512
DOI: 10.1186/s12883-015-0301-9 -
Journal of Central Nervous System... 2023Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an uncommon neurological disease affecting the central nervous system (CNS). Numerous... (Review)
Review
BACKGROUND
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an uncommon neurological disease affecting the central nervous system (CNS). Numerous neurological disorders, including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), acute transverse myelitis (ATM), and MOGAD, have been reported following the COVID-19 infection during the current COVID-19 pandemic. On the other hand, it has been suggested that patients with MOGAD may be at greater risk for infection (particularly in the current pandemic).
OBJECTIVE
In this systematic review, we gathered separately 1) MOGAD cases following COVID-19 infection as well as 2) clinical course of patients with MOGAD infected with COVID-19 based on case reports/series.
METHODS
329 articles were collected from 4 databases. These articles were conducted from inception to March 1, 2022.
RESULTS
Following the screening, exclusion criteria were followed and eventually, 22 studies were included. In 18 studies, a mean ± SD time interval of 18.6 ± 14.9 days was observed between infection with COVID-19 and the onset of MOGAD symptoms. Symptoms were partially or completely recovered in a mean of 67 days of follow-up.Among 4 studies on MOGAD patients, the hospitalization rate was 25%, and 15% of patients were hospitalized in the intensive care unit (ICU).
CONCLUSION
Our systematic review demonstrated that following COVID-19 infection, there is a rare possibility of contracting MOGAD. Moreover, there is no clear consensus on the susceptibility of MOGAD patients to severe COVID-19. However, obtaining deterministic results requires studies with a larger sample size.
PubMed: 37008248
DOI: 10.1177/11795735231167869 -
Journal of Neuroimmunology Oct 2022Neurosarcoidosis is a rare disorder in children. We identified 30 pediatric NS cases through a systematic review. Twenty-one (70%) had systemic sarcoidosis with 30%...
Neurosarcoidosis is a rare disorder in children. We identified 30 pediatric NS cases through a systematic review. Twenty-one (70%) had systemic sarcoidosis with 30% having primary neurosarcoidosis. Eyes (37%), lymph nodes (37%) and lungs (30%) were most commonly involved. Isolated neurosarcoidosis were more likely in children (30%) than in adults (6%, p = 0.0005). Seizures and optic neuritis were also more common in children than adults (33% vs 14%, p = 0.002; and 30% versus 6%, p = 0.008, respectively). Evaluation, imaging, laboratory findings, and treatments are discussed. Additional research, including multi-center studies, is needed.
Topics: Adult; Central Nervous System Diseases; Child; Humans; Lung; Optic Neuritis; Sarcoidosis
PubMed: 35944453
DOI: 10.1016/j.jneuroim.2022.577938 -
Frontiers in Neurology 2023Neuromyelitis optica spectrum disorder (NMOSD) is a rare chronic neuroinflammatory autoimmune condition. Since the onset of the COVID-19 pandemic, there have been... (Review)
Review
BACKGROUND
Neuromyelitis optica spectrum disorder (NMOSD) is a rare chronic neuroinflammatory autoimmune condition. Since the onset of the COVID-19 pandemic, there have been reports of NMOSD clinical manifestations following both SARS-CoV-2 infections and COVID-19 vaccinations.
OBJECTIVE
This study aims to systematically review the published literature of NMOSD clinical manifestations associated with SARS-CoV-2 infections and COVID-19 vaccinations.
METHODS
A Boolean search of the medical literature was conducted between December 1, 2019 to September 1, 2022, utilizing Medline, Cochrane Library, Embase, Trip Database, Clinicaltrials.gov, Scopus, and Web of Science databases. Articles were collated and managed on Covidence software. The authors independently appraised the articles for meeting study criteria and followed PRISMA guidelines. The literature search included all case reports and case series that met study criteria and involved NMOSD following either the SARS-CoV-2 infection or the COVID-19 vaccination.
RESULTS
A total of 702 articles were imported for screening. After removing 352 duplicates and 313 articles based on exclusion criteria, 34 articles were analyzed. A total of 41 cases were selected, including 15 patients that developed new onset NMOSD following a SARS-CoV-2 infection, 21 patients that developed NMOSD following COVID-19 vaccination, 3 patients with known NMOSD that experienced a relapse following vaccination, and 2 patients with presumed Multiple Sclerosis (MS) that was unmasked as NMOSD post-vaccination. There was a female preponderance of 76% among all NMOSD cases. The median time interval between the initial SARS-CoV-2 infection symptoms and NMOSD symptom onset was 14 days (range 3-120 days) and the median interval between COVID-19 vaccination and onset of NMO symptoms was 10 days (range 1 to 97 days). Transverse myelitis was the most common neurological manifestation in all patient groups (27/41). Management encompassed acute treatments such as high dose intravenous methylprednisolone, plasmapheresis, and intravenous immunoglobulin (IVIG) and maintenance immunotherapies. The majority of patients experienced a favorable outcome with complete or partial recovery, but 3 patients died.
CONCLUSION
This systematic review suggests that there is an association between NMOSD and SARS-CoV-2 infections and COVID-19 vaccinations. This association requires further study using quantitative epidemiological assessments in a large population to better quantify the risk.
PubMed: 37426444
DOI: 10.3389/fneur.2023.1099758