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Neurological Sciences : Official... Mar 2024
Review
Topics: Humans; Spastic Paraplegia, Hereditary; Mutation
PubMed: 37968432
DOI: 10.1007/s10072-023-07200-1 -
Annals of Medicine and Surgery (2012) Nov 2023Guillain-Barré syndrome (GBS) is an acute inflammatory disease of the peripheral nervous system, rarely following Varicella-zoster virus (VZV) infection. The authors...
BACKGROUND
Guillain-Barré syndrome (GBS) is an acute inflammatory disease of the peripheral nervous system, rarely following Varicella-zoster virus (VZV) infection. The authors aimed to review all cases in the English literature of GBS that occurred after primary VZV infection to investigate the clinical features, diagnostic workup, treatment, and outcome of patients with GBS following VZV.
METHODS
PubMed, Scopus, and Embase are systematically searched from their inception to 9 May 2022 to collect all cases of GBS following varicella-zoster infection. Patients with GBS following VZV reactivation were excluded.
RESULTS
Among the 29 patients, the age of presentation ranged from 1.5 to 70 years with a median of 37, with a yield for males (81.5%). Most of the patients presented with sensory-motor symptoms (65.4%) and suffered from tetraparesis (81.5%). Cranial nerve palsy was present in (84%) of patients, and the seventh cranial nerve was the most commonly affected nerve (75%). Lumbar puncture showed albuminocytological dissociation in (80%) of patients. The dominant nerve conduction study subtype was acute inflammatory demyelinating polyneuropathy (65.3%). in addition, the magnetic resonance imaging showed pathological findings in only (47.5%) of the patients. Intravenous immunoglobulin is now the drug of choice for all cases of GBS following VZV infection.
CONCLUSION
GBS is a rare neurological complication of primary infection with VZV. However, the authors should suspect this syndrome when a patient develops ascending weakness, regardless of the absence of areflexia and albuminocytological dissociation. Drug therapy with IIVIg ensures a gradual improvement for the patient over a period of weeks to several months.
PubMed: 37915710
DOI: 10.1097/MS9.0000000000001370 -
Human Vaccines & Immunotherapeutics Aug 2023A 20-month-old girl was diagnosed with Guillain - Barré syndrome (GBS) based on progressive muscle weakness, areflexia, and albuminocytologic dissociation of the...
A 20-month-old girl was diagnosed with Guillain - Barré syndrome (GBS) based on progressive muscle weakness, areflexia, and albuminocytologic dissociation of the cerebrospinal fluid. Despite timely and systematic treatment, she eventually became paralyzed. There is a temporal correlation between the girl's GBS and the DTaP vaccination, but the exact causal relationship between the two is still debatable. Furthermore, we summarized clinical features of other 45 published GBS cases after DTP vaccines (or vaccine substances containing tetanus) through a systematic review. The mean onset age, sex distribution, onset time after vaccination, detection of antiganglioside antibodies, and other basic clinical features of GBS after DTP vaccination (or vaccine substances containing tetanus) were analyzed. The temporal pattern of GBS after vaccination was similar to that of GBS after infection. Herein, we report this rare case of presumptive pediatric GBS after DTaP vaccination and review similar cases to draw the attention of medical personnel to similar events after vaccination. An association between DTP vaccines and GBS has been proposed, and the causal relationship between these two incidents are worthy further exploration. Moreover, surveillance and vigilance for GBS after vaccination are highly recommended.
Topics: Female; Humans; Infant; Diphtheria-Tetanus-Pertussis Vaccine; Guillain-Barre Syndrome
PubMed: 37753771
DOI: 10.1080/21645515.2023.2261199 -
BMC Neurology Sep 2023Miller Fisher syndrome (MFS) is a subtype of Guillain-Barré syndrome (GBS) which is characterized by the three components of ophthalmoplegia, ataxia, and areflexia....
BACKGROUND
Miller Fisher syndrome (MFS) is a subtype of Guillain-Barré syndrome (GBS) which is characterized by the three components of ophthalmoplegia, ataxia, and areflexia. Some studies reported MFS as an adverse effect of the COVID-19 vaccination. We aimed to have a detailed evaluation on demographic, clinical, and para-clinical characteristics of subjects with MFS after receiving COVID-19 vaccines.
MATERIALS AND METHODS
A thorough search strategy was designed, and PubMed, Web of Science, and Embase were searched to find relevant articles. Each screening step was done by twice, and in case of disagreement, another author was consulted. Data on different characteristics of the patients and types of the vaccines were extracted. The risk of bias of the studies was assessed using Joanna Briggs Institute (JBI) tools.
RESULTS
In this study, 15 patients were identified from 15 case studies. The median age of the patients was 64, ranging from 24 to 84 years. Ten patients (66.6%) were men and Pfizer made up 46.7% of the injected vaccines. The median time from vaccination to symptoms onset was 14 days and varied from 7 to 35 days. Furthermore,14 patients had ocular signs, and 78.3% (11/14) of ocular manifestations were bilateral. Among neurological conditions, other than MFS triad, facial weakness or facial nerve palsy was the most frequently reported side effect that was in seven (46.7%) subjects. Intravenous immunoglobulin (IVIg) was the most frequently used treatment (13/15, 86.7%). Six patients received 0.4 g/kg and the four had 2 g/kg. Patients stayed at the hospital from five to 51 days. No fatal outcomes were reported. Finally, 40.0% (4/15) of patients completely recovered, and the rest experienced improvement.
CONCLUSION
MFS after COVID-19 immunization has favorable outcomes and good prognosis. However, long interval from disease presentation to treatment in some studies indicates that more attention should be paid to MFS as the adverse effect of the vaccination. Due to the challenging diagnosis, MFS must be considered in list of the differential diagnosis in patients with a history of recent COVID-19 vaccination and any of the ocular complaints, ataxia, or loss of reflexes, specially for male patients in their 60s and 70s.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Young Adult; Ataxia; COVID-19; COVID-19 Vaccines; Drug-Related Side Effects and Adverse Reactions; Facial Paralysis; Miller Fisher Syndrome; Prognosis; Vaccination
PubMed: 37735648
DOI: 10.1186/s12883-023-03375-4 -
Nutrients Aug 2023Alpha-lipoic acid (ALA) was found to improve the symptoms in patients with diabetic sensorimotor peripheral neuropathy (DSPN) by reducing oxidative stress and... (Meta-Analysis)
Meta-Analysis Review
Alpha-lipoic acid (ALA) was found to improve the symptoms in patients with diabetic sensorimotor peripheral neuropathy (DSPN) by reducing oxidative stress and ameliorating microcirculation. Our meta-analysis is aimed at evaluating the effects of oral-administered ALA versus a placebo in patients with DSPN and determining the optimal dosage for this treatment. We systematically reviewed randomized controlled trials (RCTs) in the PubMed, Embase, and Cochrane databases to determine the efficacy of oral ALA for patients with DSPN. The primary outcome was total symptoms' score (TSS), and secondary outcomes were the neurological disability score (NDS), neuropathy impaired score (NIS), NIS-lower limb (NIS-LL), vibration perception threshold (VPT), nerve conduction study (NCS) results, and global satisfaction. A subgroup analysis of the ALA dosage (600, 1200, and 1800 mg/day) was also conducted. Ten RCTs (1242 patients) were included. ALA treatment produced favorable results for TSS (a dose-related trend was observed), NDS, and the global satisfaction score. For VAS, VPT, NIS-LL, and NCS results, ALA did not produce favorable results. ALA treatment had favorable effects on DSPN by reducing sensory symptoms, and it resulted in a dose-dependent response relative to the placebo for TSS and the global satisfaction score. The use of ALA to prevent neurological symptoms should be further researched.
Topics: Humans; Diabetic Neuropathies; Thioctic Acid; Administration, Oral; Databases, Factual; Lower Extremity; Diabetes Mellitus
PubMed: 37630823
DOI: 10.3390/nu15163634 -
Brain Structure & Function Sep 2023Pain-related evoked potentials with concentric surface electrodes (PREP with CE) have been increasingly used in the diagnostics of polyneuropathies as well as in pain...
Pain-related evoked potentials with concentric surface electrodes (PREP with CE) have been increasingly used in the diagnostics of polyneuropathies as well as in pain research. However, the study results are partly inconsistent regarding their utility to distinguish between normal and abnormal findings. The present systematic review aimed to summarise and compare study results, where PREP with CE were used in healthy subjects or patients and to identify possible influencing factors. We found 36 research articles, of which 21 investigated disorders in patients compared to healthy controls, while the other 15 focussed on basic research in healthy subjects. Patients with polyneuropathies showed the most consistent PREP results with similar prolonged latencies and reduced amplitude values. Findings in other patient groups or in healthy subjects were more heterogeneous. There was evidence for an influence by age and height as well as by central effects like emotions, which should be considered in further studies. Further systematic research analysing PREP results depending on individual and disease-specific factors is needed to develop optimal normative values.
Topics: Humans; Healthy Volunteers; Evoked Potentials; Pain; Electrodes; Polyneuropathies
PubMed: 37555924
DOI: 10.1007/s00429-023-02690-3 -
Acta Neurologica Belgica Oct 2023COVID-19 (CoranaVirus disease 2019) is an ongoing infectious disease caused by the RNA SARS-CoV-2 virus (Severe Acute Respiratory Syndrome CoronaVirus-2). The virus...
BACKGROUND
COVID-19 (CoranaVirus disease 2019) is an ongoing infectious disease caused by the RNA SARS-CoV-2 virus (Severe Acute Respiratory Syndrome CoronaVirus-2). The virus mainly causes respiratory symptoms, but neurological symptoms have also been reported to be part of the clinical manifestations of the disease. The aim of this study was to systematically review Miller fisher syndrome (MFS) published cases, in the context of COVID-19 infection or vaccination.
METHODS
A systematic literature review on Medline was performed. A total of 21 papers were included in the present review.
RESULTS
Twenty-two MFS cases (77% males) were identified, 14 related to COVID-19 infection and 8 to vaccination against COVID-19. The median age of the adult patients was 50 years (interquartile range 36-63 years). Sixteen patients (73%) had the classic triad of MFS (ophthalmoplegia, ataxia, areflexia), four (18%) had acute ophthalmoplegia and one other characteristic symptom and two patients (9%) had only one other characteristic symptom, but they tested positive for GQ1b antibodies. Nine (41%) patients had positive GQ1b antibodies and were classified as "definite" MFS. Albuminocytologic dissociation was found in half of the cases. The outcome was favourable in the majority of cases (86%) whereas one patient, despite the initial improvement, died because of a cardiac arrest, after cardiac arrythmia.
CONCLUSIONS
MFS after COVID-19 infection/vaccination was found to have the typical epidemiological characteristics of classic MFS; being rare, occurring more often after infection than vaccination, affecting mainly middle-aged males usually within 3 weeks after the event and having an excellent prognosis after treatment with IVIG or even with no treatment at all. We found no evidence that MFS after COVID-19 infection was different from MFS after COVID-19 vaccination, although the former tended to occur earlier.
Topics: Adult; Female; Humans; Male; Middle Aged; COVID-19; COVID-19 Vaccines; Miller Fisher Syndrome; Ophthalmoplegia; SARS-CoV-2; Vaccines
PubMed: 37468803
DOI: 10.1007/s13760-023-02336-5 -
Journal of Clinical Medicine Jun 2023Our aim was to evaluate osteomyelitis and other major lower limb safety outcomes (i.e., peripheral artery disease or PAD, ulcers, atraumatic fractures, amputations,... (Review)
Review
Association of Sodium-Glucose Cotransporter 2 Inhibitors with Osteomyelitis and Other Lower Limb Safety Outcomes in Type 2 Diabetes Mellitus: A Systematic Review and Meta-Analysis of Randomised Controlled Trials.
Our aim was to evaluate osteomyelitis and other major lower limb safety outcomes (i.e., peripheral artery disease or PAD, ulcers, atraumatic fractures, amputations, symmetric polyneuropathy, and infections) in patients affected by type 2 diabetes mellitus (T2DM) and treated with sodium-glucose cotransporter 2 inhibitors (SGLT2-is). We thus performed a systematic review and meta-analysis of randomised controlled trials (RCTs) comparing SGLT2-is at approved doses for T2DM with a placebo or standard of care. MEDLINE, Embase, and Cochrane CENTRAL were searched through August 2022. Separate intention-to-treat analyses were implemented for each molecule to calculate Mantel-Haenszel risk ratios (RR) with 95% confidence intervals (CIs) through a random-effects model. We processed data from 42 RCTs for a total of 29,491 and 23,052 patients, respectively assigned to SGLT2-i and comparator groups. SGLT2-is showed a pooled neutral effect on osteomyelitis, PAD, fractures, and symmetric polyneuropathy, whereas slightly deleterious sway on ulcers (RR 1.39 [1.01-1.91]), amputations (RR 1.27 [1.04-1.55]), and infections (RR 1.20 [1.02-1.40]). In conclusion, SGLT2-is appear to not significantly interfere with the onset of osteomyelitis, PAD, lower limb fractures, or symmetric polyneuropathy, even though the number of these events proved consistently higher in the investigational groups; otherwise, local ulcers, amputations, and overall infections may be favoured by their employment. This study is registered with the Open Science Framework (OSF).
PubMed: 37373652
DOI: 10.3390/jcm12123958 -
Journal of Medical Genetics Dec 2023Alström syndrome (ALMS; #203800) is an ultrarare monogenic recessive disease. This syndrome is associated with variants in the gene, which encodes a... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Alström syndrome (ALMS; #203800) is an ultrarare monogenic recessive disease. This syndrome is associated with variants in the gene, which encodes a centrosome-associated protein involved in the regulation of several ciliary and extraciliary processes, such as centrosome cohesion, apoptosis, cell cycle control and receptor trafficking. The type of variant associated with ALMS is mostly complete loss-of-function variants (97%) and they are mainly located in exons 8, 10 and 16 of the gene. Other studies in the literature have tried to establish a genotype-phenotype correlation in this syndrome with limited success. The difficulty in recruiting a large cohort in rare diseases is the main barrier to conducting this type of study.
METHODS
In this study we collected all cases of ALMS published to date. We created a database of patients who had a genetic diagnosis and an individualised clinical history. Lastly, we attempted to establish a genotype-phenotype correlation using the truncation site of the patient's longest allele as a grouping criteria.
RESULTS
We collected a total of 357 patients, of whom 227 had complete clinical information, complete genetic diagnosis and meta-information on sex and age. We have seen that there are five variants with high frequency, with p.(Arg2722Ter) being the most common variant, with 28 alleles. No gender differences in disease progression were detected. Finally, truncating variants in exon 10 seem to be correlated with a higher prevalence of liver disorders in patients with ALMS.
CONCLUSION
Pathogenic variants in exon 10 of the gene were associated with a higher prevalence of liver disease. However, the location of the variant in the gene does not have a major impact on the phenotype developed by the patient.
Topics: Humans; Alstrom Syndrome; Cell Cycle Proteins; Phenotype; Exons; Genetic Association Studies
PubMed: 37321834
DOI: 10.1136/jmg-2023-109175 -
Clinical Case Reports Jun 2023Guillain-Barré syndrome (GBS) is a rare but possible complication that may occur after COVID-19 vaccination. In this systematic review, we found that GBS presented in...
KEY CLINICAL MESSAGE
Guillain-Barré syndrome (GBS) is a rare but possible complication that may occur after COVID-19 vaccination. In this systematic review, we found that GBS presented in patients with an average age of 58. The average time for symptoms to appear was 14.4 days. Health care providers should be aware of this potential complication.
ABSTRACT
Most instances of Guillain-Barré syndrome (GBS) are caused by immunological stimulation and are discovered after vaccinations for tetanus toxoid, oral polio, and swine influenza. In this systematic study, we investigated at GBS cases that were reported after receiving the COVID-19 vaccination. Based on PRISMA guidelines, we searched five databases (PubMed, Google Scholar, Ovid, Web of Science, and Scopus databases) for studies on COVID-19 vaccination and GBS on August 7, 2021. To conduct our analysis, we divided the GBS variants into two groups, acute inflammatory demyelinating polyneuropathy and non-acute inflammatory demyelinating polyneuropathy (AIDP and non-AIDP), and compared the two groups with mEGOS and other clinical presentation In this systematic review, 29 cases were included in 14 studies. Ten cases belonged to the AIDP variant, 17 were non-AIDP (one case had the MFS variant, one AMAN variant, and 15 cases had the BFP variant), and the two remaining cases were not mentioned. Following COVID-19 vaccination, GBS cases were, on average, 58 years of age. The average time it took for GBS symptoms to appear was 14.4 days. About 56 percent of the cases (56%) were classified as Brighton Level 1 or 2, which defines the highest level of diagnostic certainty for patients with GBS. This systematic review reports 29 cases of GBS following COVID-19 vaccination, particularly those following the AstraZeneca/Oxford vaccine. Further research is needed to assess all COVID-19 vaccines' side effects, including GBS.
PubMed: 37305891
DOI: 10.1002/ccr3.7456