Reversible cerebral vasoconstriction syndrome in Guillain-Barre syndrome: a case report and literature review.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by transient constriction of cerebral arteries, leading to severe headache and potential complications. The association between RCVS and Guillain-Barre syndrome (GBS) is rare and poorly understood and warrants further investigation. METHODS: A detailed case of RCVS in a patient with GBS was presented, followed by a comprehensive literature review. PubMed, Embase, and Google Scholar were searched for relevant cases and studies. RESULTS: The case involved a 62-year-old woman with GBS who developed RCVS. The literature review identified three additional reported cases. RCVS in GBS primarily affected middle-aged women and presented with a variety of neurological symptoms. Neuroimaging showed reversible vasoconstriction in the cerebral arteries, along with other complications such as posterior reversible encephalopathy syndrome, subarachnoid hemorrhage, and infarcts. While the treatment for GBS consisted mainly of intravenous immunoglobulin, specific treatments for RCVS remain unclear. CONCLUSIONS: The coexistence of RCVS and GBS is a rare occurrence. RCVS in GBS may result from the disruption of cerebral vascular tone regulation, possibly influenced by GBS-related dysautonomia and consequent high blood pressure. Recognizing RCVS in GBS patients is critical for appropriate management.

Whole-Body Muscle Magnetic Resonance Imaging in 81 Patients with Spinal and Bulbar Muscular Atrophy: A Prospective Study.

OBJECTIVE: Spinal and bulbar muscular atrophy (SBMA) is characterized by slow, progressive bulbar and limb muscle weakness; however, the pattern of progression of muscle fat infiltration remains unclear. We assessed the progression of muscle involvement in 81 patients with SBMA using whole-body muscle magnetic resonance imaging (MRI), alongside clinical and laboratory findings. METHODS: This prospective study included patients with genetically confirmed SBMA who underwent whole-body muscle MRI. We analyzed muscle fat infiltration and the pattern of involved muscles using cluster analysis, visualizing the sequential progression of fat infiltration. Muscle clusters demonstrated correlation with clinical scales and laboratory findings. Additionally, linear regression analysis was performed to identify the MRI section most strongly associated with 6-minute walk test (6MWT). RESULTS: We included 81 patients with SBMA (age = 54.3 years). After categorizing the patients into 6 clusters based on the pattern of muscle fat infiltration, we observed that muscle involvement began in the posterior calf and progressed to the posterior thigh, pelvis, trunk, anterior thigh, medial thigh, anterior calf, and upper extremity muscles. These muscle clusters correlated significantly with disease duration (τ = 0.47, p < 0.001), 6MWT (τ = -0.49, p < 0.001), and serum creatinine level (τ = -0.46, p < 0.001). The whole-body MRI indicated the thigh as the section most significantly correlated with 6MWT. INTERPRETATION: We used whole-body muscle MRI to determine the sequential progression of the fat infiltration in SBMA. Our findings may enable the identification of objective and reliable imaging outcome measures in the study of the natural history or future clinical trials of SBMA. ANN NEUROL 2024;95:596-606.

Neurofilament light chain as a biomarker in neuromyelitis optica spectrum disorder: a comprehensive review and integrated analysis with glial fibrillary acidic protein.

BACKGROUND: In the context of neuromyelitis optica spectrum disorder (NMOSD), there are several measures that serve as a biomarker. However, each of the methods has the intrinsic limitations. While neurofilament light chain (NfL) and glial fibrillary acidic protein (GFAP) have emerged as an additional biomarker for NMOSD, a thorough investigation of their role remains incomplete. Our aim is to provide a comprehensive review of the current literature regarding NfL and GFAP as a biomarker and explore their potential utility in NMOSD. METHODS: We performed a comprehensive search using PubMed and Google Scholar to identify peer-reviewed articles investigating NfL and GFAP as a biomarker in NMOSD. RESULTS: Our search identified 13 relevant studies. NfL consistently showed promise in distinguishing NMOSD patients from healthy individuals, although it had limited specificity in distinguishing NMOSD from other demyelinating diseases. NfL offered certain advantages over GFAP, notably its ability to predict disability worsening during attacks. In contrast, GFAP provided valuable insight, particularly in distinguishing NMOSD from multiple sclerosis and identifying clinical relapses. In addition, GFAP showed predictive potential for future attacks. Some studies even suggested that NfL may serve as an indicator of treatment response in NMOSD. CONCLUSIONS: NfL and GFAP hold promise as biomarkers for NMOSD, demonstrating their usefulness in distinguishing patients from healthy individuals, assessing disease severity, and possibly reflecting treatment response. However, it is important to recognize that NfL and GFAP may, at some point, have different roles.

Convergence Gas Sensors with One-Dimensional Nanotubes and Pt Nanoparticles Based on Ultraviolet Photonic Energy for Room-Temperature NO2 Gas Sensing.

Zinc oxide (ZnO) is a promising material for nitrogen dioxide (NO2) gas sensors because of its nontoxicity, low cost, and small size. We fabricated one-dimensional (1D) and zero-dimensional (0D) convergence gas sensors activated via ultraviolet (UV) photonic energy to sense NO2 gas at room temperature. One-dimensional ZnO nanorod (ZNR)-based and ZnO nanotube (ZNT)-based gas sensors were synthesized using a simple hydrothermal method. All the sensors were tested under UV irradiation (365 nm) so that they could be operated at room temperature rather than a high temperature. In addition, we decorated 0D Pt nanoparticles (NPs) on the gas sensors to further improve their sensing responsivity. The NO2-sensing response of the ZNT/Pt NP convergence gas sensor was 2.93 times higher than that of the ZNR gas sensor. We demonstrated the complex effects of UV radiation on 1D ZnO nanostructures and 0D metal nanostructures in NO2 gas sensing.

Acute Hemorrhagic Encephalomyelitis in the Context of MOG Antibody-Associated Disease. Comment on Chen et al. Rapid Progressive Fatal Acute Hemorrhagic Encephalomyelitis. Diagnostics 2023, 13, 2481.

The study by Chen et al. of a 56-year-old man diagnosed with acute hemorrhagic encephalomyelitis (AHEM) had a significant impact on us. The authors provided a comprehensive account of their diagnostic journey and emphasized the need to differentiate myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) from AHEM. However, recent research suggests that AHEM may not be an isolated entity, but rather a phenotype within MOGAD. The patient's clinical presentation included MRI brain lesions characteristic of MOGAD in addition to hemorrhagic abnormalities. These findings raise the possibility that AHEM in this case represents a MOGAD phenotype. In conclusion, it is important to recognize the potential association between AHEM and MOGAD, especially when distinct MOGAD brain MRI patterns are present, as in this case.

Immunogenicity and Protective Efficacy of Recombinant Protective Antigen Anthrax Vaccine (GC1109) in A/J Mice Model.

A recombinant protective antigen anthrax vaccine (GC1109) is being developed as a new-generation vaccine by the Korea Disease Control and Prevention Agency. In accordance with the ongoing step 2 of phase II clinical trials, the immunogenicity and protective efficacy of the booster dose of GC1109 were evaluated in A/J mice after 3 serial vaccinations at 4-week intervals. The results indicated that the booster dose significantly increased the production of anti-protective antigen (PA) IgG and toxin-neutralizing antibody (TNA) compared with those of the group without booster. An enhanced protective effect of the booster dose was not observed because the TNA titers of the group without booster were high enough to confer protection against spore challenge. Additionally, the correlation between TNA titers and probability of survival was determined for calculating the threshold TNA titer levels associated with protection. The threshold 50 % neutralization factor (NF50) of TNA showing 70 % probability of protection was 0.21 in A/J mice with 1,200 LD50 Sterne spores challenge. These results indicate that GC1109 is a promising candidate as a new-generation anthrax vaccine and that a booster dose might provide enhanced protection by producing toxin-neutralizing antibodies.

The frequency and characteristics of saccadic dysmetria in isolated cerebellar infarction.

OBJECTIVES: To investigate the frequency and pattern of horizontal saccadic dysmetria in unilateral cerebellar infarction and identify the responsible region for horizontal saccadic dysmetria. METHODS: From the acute stroke registry of Keimyung University Dongsan Medical Center between July 2016 and October 2020, 43 patients with acute unilateral cerebellar infarction were enrolled. Eye movements were recorded during the acute period and the lesion was mapped using MRIcron software for subtraction analysis. Saccadic dysmetria was marked as hypometric when the gain is < 0.85 and hypermetric when > 1.0. RESULTS: Among the 43 participants, 30 patients (69.8%) demonstrated saccadic dysmetria. The age was significantly higher in patients with dysmetria (66.87 ± 12.82 vs. 53.54 ± 14.09, p = 0.004). Type of dysmetria showed a significant difference according to the vascular territory of the lesion. The posterior inferior cerebellar artery (PICA) infarction group presented ipsiversive saccadic dysmetria, while the superior cerebellar artery (SCA) group showed contraversive dysmetria (p < 0.001). In the SCA group, the culmen, fastigium, and dentate were the most frequently damaged regions, while the tonsil and inferior semilunar lobule were in the PICA group. CONCLUSION: Saccadic dysmetria was observed in a large proportion of cerebellar stroke patients, and the types of saccades were distinctive according to the vascular territory of the lesion.