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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 -
Revista Da Sociedade Brasileira de... 2023Complications of leprosy neuritis are considered serious and apparent, with the potential to disable and/or limit individuals. These complications affect not only a...
BACKGROUND
Complications of leprosy neuritis are considered serious and apparent, with the potential to disable and/or limit individuals. These complications affect not only a patient's physical functioning, but also their family and social lives, while directly impacting the ability to work and/or maintain financial independence, subsequently interfering with their overall quality of life. The present review, therefore, aimed to analyze the effectiveness of neurolysis as an alternative treatment for the complications associated with leprosy neuritis.
METHODS
The present review was performed based on the Joanna Briggs Institute methodology, in an effort to answer the following research question: what is the effectiveness of neurolysis as a treatment for leprosy neuritis complications? This research question was defined using the patient-intervention-outcome (PIO) framework, where leprosy represents 'P', neurolysis for 'I', and neuropathic pain/motor function/sensorial function/physical disability/quality of life for 'O'. Randomized and non-randomized clinical trials and prospective observational cohort studies were included in the present review, with no time or date restrictions.
RESULTS
The present review included 1 randomized clinical trial and 10 prospective studies, published between 1976 and 2020. All of the outcomes showed improvement, with relief from neuropathic pain being the primary finding.
CONCLUSIONS
The evidence obtained in the present review suggested that neurolysis is an effective alternative for the treatment of physical disabilities, the recovery of sensory and motor function, the restoration of quality of life, and neuropathic pain relief.
Topics: Humans; Prospective Studies; Quality of Life; Leprosy; Neuritis; Neuralgia; Observational Studies as Topic
PubMed: 37531518
DOI: 10.1590/0037-8682-0123-2023 -
The Journal of International Medical... Jul 2023The exact etiology of Parsonage-Turner syndrome is unknown, but it is known to be preceded by infection, vaccination, or surgical intervention. In this review, we... (Review)
Review
OBJECTIVES
The exact etiology of Parsonage-Turner syndrome is unknown, but it is known to be preceded by infection, vaccination, or surgical intervention. In this review, we describe associations of Parsonage-Turner syndrome with COVID-19 infection and vaccination.
METHODS
A systematic literature search was conducted using PubMed/MEDLINE, ScienceDirect, and Google Scholar. Microsoft Excel was used for data extraction and statistical analysis. The quality of case reports and case series was assessed using the Joanna Briggs Institute Critical Appraisal Tool.
RESULTS
We selected 44 case reports and 10 case series, including 68 patients (32 post-vaccination and 36 with post-COVID-19 infection Parsonage-Turner syndrome). Middle-aged males were predominantly affected in both groups. The most frequently administered vaccine was Comirnaty (Pfizer) (53%). The mean latency was 11.7 days in the post-vaccination group and 20.3 days in the post-infection group. The most affected nerves in both groups were the axillary, suprascapular, and musculocutaneous nerves; and 78.1% and 38.9% of patients showed partial amelioration of their symptoms in the post-vaccination and post-infection groups, respectively.
CONCLUSION
Post-vaccination Parsonage-Turner syndrome presents earlier than post-infection disease. Pain and sensorimotor deficits of the upper limb are common in both situations. Complete or partial recovery occurs in most cases.
Topics: Male; Middle Aged; Humans; Brachial Plexus Neuritis; COVID-19; Pain; Upper Extremity; Vaccination
PubMed: 37523491
DOI: 10.1177/03000605231187939 -
Rheumatology International Jul 2023Central nervous system (CNS) involvement can occur in primary Sjögren's syndrome (pSS) due to co-existing neuromyelitis optica spectrum disorder (NMOSD) which has a...
Central nervous system (CNS) involvement can occur in primary Sjögren's syndrome (pSS) due to co-existing neuromyelitis optica spectrum disorder (NMOSD) which has a highly relapsing course requiring indefinite immunosuppression, and if not diagnosed early, damage accrual occurs over time leading to permanent disability and morbidity. In this review, we describe and outline the clinical course and outcomes of anti-aquaporin 4 (AQP4) antibody seropositive NMOSD with pSS overlap cases. To investigate the co-existence of AQP4 + NMOSD with pSS, we conducted a review of individual patient data from case reports and case series found in major databases. The study extracted clinico-demographic features, imaging and laboratory profiles, treatment approaches, and outcomes of these patients. Inclusion criteria for the review required patients to have positivity for anti-AQP4 or NMO-IgG autoantibodies in the blood and/or cerebrospinal fluid (CSF) and exhibit at least one manifestation of both pSS and NMOSD. In this overlap between AQP4 + NMOSD and pSS, 44 patients were included of whom 41 (93.2%) were females. The mean age of pSS onset was 44.8 ± 18.4 years and NMOSD onset was 43.2 ± 19.8 years. In 20 (45.5%) patients, NMOSD preceded pSS onset, 13 (29.5%) NMOSD occurred after pSS onset, and 11 (25%) patients had a simultaneous presentation. 31 (70.5%) patients experienced acute transverse myelitis, 21 (47.7%) optic neuritis, 14 (31.8%) cerebral syndrome, 10 (22.7%) acute brainstem syndrome, 5 (11.4%) area postrema syndrome, and 2 (4.5%) diencephalic clinical syndromes. For the treatment of acute phase, 40 (90.9%) patients received intravenous methylprednisolone, 15 (34.1%) received plasma exchange, and 10 (22.7%) received intravenous immunoglobulin; and for the induction/maintenance therapy, 16 (36.4%) patients received cyclophosphamide, 6 (13.6%) received rituximab, 16 (36.4%) received azathioprine, and 10 (22.7%) received mycophenolate mofetil. Disease course was monophasic in 2 (4.5%) and relapsing in 27 (61.4%) patients. At median (IQR) follow-up duration of 2.4 (6) years, 39 (88.6%) patients showed improvement, 3 (6.8%) showed stabilization and 2 (4.5%) showed worsening of their NMOSD manifestations. In this overlap syndrome of AQP4 + NMOSD and pSS, patients have a neurologically disabling disorder that can mimic neurological manifestations of pSS, frequently occurs prior to the onset of pSS, has a relapsing course, responds well to immunosuppressants, and necessitates indefinite treatment. Collaborative multicentre studies are needed to clarify the natural history and outcomes of this rare overlap syndrome.
PubMed: 37500817
DOI: 10.1007/s00296-023-05397-0 -
Annals of Medicine Dec 2023To appraise whether plasma exchange (PLEX) effectively improves visual function for acute optic neuritis (ON) in neuromyelitis optica (NMO) or neuromyelitis optica... (Review)
Review
OBJECTIVES
To appraise whether plasma exchange (PLEX) effectively improves visual function for acute optic neuritis (ON) in neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD).
METHODS AND ANALYSIS
We searched Medline, Embase, Cochrane Library, ProQuest Central, and Web of Science to identify relevant articles published between 2006 and 2020.Eligible studies were in English and evaluated visual outcomes for people with acute ON in NMO or NMOSD treated with PLEX. They also had adequate pre- and posttreatment data. Excluded were studies with 1 or 2 case reports, or incomplete data.
RESULTS
Twelve studies were qualitatively synthesized (1 RCT; 1 controlled NRSI; 10 observational studies). Five before-and-after observational studies were used for quantitative synthesis. The PLEX in the 5 studies (3 to 7 cycles over 2 to 3 weeks) was performed as second-line or adjunctive therapy for acute ON in NMO/NMOSD.The qualitative synthesis revealed that visual-acuity recovery occurred between one day and 6 months after the first PLEX cycle completion. Thirty-two of 48 participants in the 5 quantitative-synthesis studies received PLEX. Relative to pre-PLEX values, visual-acuity improvements were nonsignificant at these post-PLEX time points: 1 day (SMD 0.611; 95% CI -0.620 to 1.842); 2 weeks (SMD 0.0214; 95% CI -1.250 to 1.293); 3 months (SMD 1.014; 95% CI -0.954 to 2.982); and 6 months (SMD 0.450; 95% CI -2.643 to 3.543).
CONCLUSIONS
There were inadequate data to determine whether PLEX effectively treats acute ON in NMO/NMOSD.
Topics: Humans; Plasma Exchange; Neuromyelitis Optica; Optic Neuritis; Outcome Assessment, Health Care
PubMed: 37387119
DOI: 10.1080/07853890.2023.2227422 -
Efficacy of Vestibular Rehabilitation in Vestibular Neuritis: A Systematic Review and Meta-analysis.American Journal of Physical Medicine &... Jan 2024This study aimed to evaluate the efficacy of vestibular rehabilitation in vestibular neuritis. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
This study aimed to evaluate the efficacy of vestibular rehabilitation in vestibular neuritis.
DESIGN
A randomized controlled trial was collected from MEDLINE, Embase, Cochrane Library, PEDro, LILACS, and Google Scholar before May 2023.
RESULTS
This study included 12 randomized controlled trials involving 536 patients with vestibular neuritis. Vestibular rehabilitation was comparable with steroids in dizziness handicap inventory score at the first, sixth, and 12th months (pooled mean differences: -4.00, -0.21, and -0.31, respectively); caloric lateralization at the third, sixth, and 12th months (pooled mean difference: 1.10, 4.76, and -0.31, respectively); and abnormal numbers of vestibular-evoked myogenic potentials at the first, sixth, and 12th months. Patients receiving a combination of rehabilitation and steroid exhibited significant improvement in dizziness handicap inventory score at the first, third, and 12th months (mean difference: -14.86, pooled mean difference: -4.63, mean difference: -9.50, respectively); caloric lateralization at the first and third months (pooled mean difference: -10.28, pooled mean difference: -8.12, respectively); and numbers of vestibular-evoked myogenic potentials at the first and third months (risk ratios: 0.66 and 0.60, respectively) than did those receiving steroids alone.
CONCLUSIONS
Vestibular rehabilitation is recommended for patients with vestibular neuritis. A combination of vestibular rehabilitation and steroids is more effective than steroids alone in the treatment of patients with vestibular neuritis.
Topics: Humans; Vestibular Neuronitis; Dizziness; Steroids; Randomized Controlled Trials as Topic
PubMed: 37339059
DOI: 10.1097/PHM.0000000000002301 -
Journal of Neuro-ophthalmology : the... Dec 2023Papilledema must be managed distinctly from other causes of optic disc edema (ODE) due to its basis in raised intracranial pressure (ICP). However, evidence indicates...
BACKGROUND
Papilledema must be managed distinctly from other causes of optic disc edema (ODE) due to its basis in raised intracranial pressure (ICP). However, evidence indicates that the term "papilledema" is widely misused across specialties to describe ODE without raised ICP. Sources of this misconception remain undiscerned. Because all physicians consult medical databases, our objective was to evaluate whether nonspecific "papilledema" subject heading definitions misleadingly associate articles on other conditions with papilledema proper.
METHODS
Systematic review of case reports, prospectively registered on PROSPERO (CRD42022363651). MEDLINE and Embase were searched to July 2022 for any full-length case report indexed to the "papilledema" subject heading. Studies were graded for incorrect indexing, defined as cases lacking evidence for raised ICP. Nonpapilledema diagnoses were assigned to a predefined set of diseases and pathophysiological mechanisms for subsequent comparison.
RESULTS
Incorrect indexing occurred in 40.67% of 949 included reports. Embase-derived studies were misindexed significantly less than MEDLINE-derived studies ( P < 0.01). There was also significant heterogeneity in incorrect indexing among specific diseases ( P = 0.0015) and mechanisms ( P = 0.0003). The most commonly misindexed diseases were uveitis (21.24% of errors), optic neuritis (13.47%), and instances with no mention of ODE (13.99%). The most commonly misindexed mechanisms were inflammation (34.97%), other mechanism (e.g., genetic; 25.91%), and ischemia (20.47%).
CONCLUSIONS
Database subject headings, especially from MEDLINE, do not adequately distinguish between true papilledema and other causes of ODE. Inflammatory diseases were most often incorrectly indexed among other diseases and mechanisms. Current "papilledema" subject headings should be revised to reduce the probability of misinformation.
Topics: Humans; Inflammation; Intracranial Hypertension; Optic Neuritis; Papilledema; Case Reports as Topic
PubMed: 37307081
DOI: 10.1097/WNO.0000000000001895 -
Multiple Sclerosis and Related Disorders Jul 2023Vaccination in patients with neuromyelitis optica spectrum disorders (NMOSD) is challenging because there is a concern that vaccines can lead to clinical attacks.... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Vaccination in patients with neuromyelitis optica spectrum disorders (NMOSD) is challenging because there is a concern that vaccines can lead to clinical attacks. However, little is known about the risk and the characteristics of attacks occurring after vaccination.
METHODS
We performed a systematic review and meta-analysis using PubMed and Embase databases to estimate a summary frequency of attacks occurring after vaccination and describe the clinical features of theses attacks. We defined attacks occurring after vaccination as typical NMOSD attacks that occurred up to 30 days after vaccine administration. For the frequency of attacks occurring after vaccination, we selected observational studies that reported the number of attacks and total number of patients that received vaccines; for the clinical description of the attacks, case reports and case series were also included.
RESULTS
We included 377 participants from 5 studies to estimate the frequency of NMOSD attacks occurring after vaccination. We found a summary frequency of of 2% (95% CI 1-4%, I = 0%). We evaluated 17 studies to identify that 13 different vaccines were associated with NMOSD attacks. A higher-than-expected proportion of males, simultaneous optic neuritis and transverse myelitis attacks, and anti-aquaporin 4 antibody negative cases were identified in vaccine-associated attacks from 24 participants from 17 studies. Nearly two-thirds of attacks occurring after vaccination were an initial event of NMOSD.
CONCLUSION
The frequency of NMOSD attacks occurring after vaccination is low and non-specific to different vaccine technologies. Our work reinforces the safety of vaccine recommendations in patients with NMOSD.
Topics: Male; Humans; Neuromyelitis Optica; Myelitis, Transverse; Optic Neuritis; Vaccination; Vaccines; Autoantibodies
PubMed: 37182477
DOI: 10.1016/j.msard.2023.104741 -
Cureus Mar 2023Coronavirus disease 2019 (COVID-19), which is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has killed millions of people around the world... (Review)
Review
Coronavirus disease 2019 (COVID-19), which is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has killed millions of people around the world so far and has turned into a disaster for people and healthcare systems. Neurological problems are often seen in people with COVID-19 in the general population, but it is unclear how common they are in pregnant women. This study provides a summary of studies on pregnant women with proven SARS-CoV-2 infection and a particular neurologic diagnosis from different parts of the world. After applying the inclusion and exclusion criteria, a total of 15 papers were assessed to create this review article. Based on our findings, the peripheral and central nervous systems were both equally impacted: Guillain-Barré syndrome (GBS, n=1), bifacial weakness, paresthesia, and vestibulocochlear neuritis (n=1), eclampsia types (n=2), and neurological disease (n=2); case reports, retrospective studies, editorials, and prospective observational studies were included. The median gestational age was 34 (30-36.5) weeks, and the median maternal age was 32.5 (25-35) years. Given the number of reports of neurologic problems associated with COVID-19 in the general community, our findings might be overstated, and we chose the ones that fit our criteria. We hope that this review helps in the early detection and management of neurological diseases during pregnancy.
PubMed: 37090385
DOI: 10.7759/cureus.36388 -
Multiple Sclerosis and Related Disorders May 2023Neuromyelitis optica spectrum disorders (NMOSD) is considered a complex multifactorial disorder. Most cases are sporadic, and familial NMOSD is assumed as a rare...
BACKGROUND
Neuromyelitis optica spectrum disorders (NMOSD) is considered a complex multifactorial disorder. Most cases are sporadic, and familial NMOSD is assumed as a rare occurrence. However, few studies reported familial aggregation of the disorder.
OBJECTIVES
To report familial NMOSD cases in Thailand and conduct a systematic review of familial NMOSD.
METHODS
A retrospective chart review of familial NMOSD patients at the university hospital was performed. Articles related to "genetic" and "NMOSD" were systematically searched and reviewed. We included NMOSD patients whose one or more relatives were diagnosed with the same disease or multiple sclerosis (MS). Data regarding demographics, clinical features, disease outcomes, and genetic testing were collected and analyzed using descriptive statistics.
RESULTS
We identified 6 familial cases from 165 NMOSD cases (3.6%) at our hospital and gathered 77 cases from a systematic review, totaling 83 cases from 40 families. The mean (SD) age at onset was 37.2 (18.0) years. Familial NMOSD involved 1-2 generations with mainly 2 affected individuals. The most common kinship pattern was siblingship in 21 families (52.5%). Initial syndromes were mostly optic neuritis and transverse myelitis. Serum aquaporin-4 IgG was positive in 79.7% of cases. Median number of relapses was 3 (range 1-26). Median expanded disability status scale in the last visit was 2 (range 0-8). Reported human leukocyte antigens (HLA) alleles shared between familial cases were HLA-A*01 and HLA-DRB1*03.
CONCLUSION
Familial clustering of NMOSD is more common than would be expected in the general population. The demographic, clinical, and outcome profiles of familial cases were not different from sporadic cases. Certain specific HLA haplotypes were shared among familial cases. Our systematic review highlighted complex genetic predisposition to NMOSD.
Topics: Humans; Adult; Neuromyelitis Optica; Retrospective Studies; Autoantibodies; Neoplasm Recurrence, Local; Aquaporin 4
PubMed: 37015139
DOI: 10.1016/j.msard.2023.104627