Case report: Intrathecal baclofen therapy improved gait pattern in a stroke patient with spastic dystonia

BackgroundIntrathecal baclofen (ITB) therapy, a viable alternative for unsuitable candidates of conventional spasticity medications, is a preferred method of administration over the oral route. Owing to its enhanced bioavailability, ITB ensures a more effective delivery at the target site.ObjectiveThere is a lack of conclusive evidence regarding the use of ITB treatment in managing ambulatory patients with spastic dystonia. Before ITB pump implantation, patients commonly undergo an ITB bolus injection trial to rule out potential adverse reactions and verify the therapeutic effects on hypertonic issues. In this report, we highlight a case of spastic dystonia, particularly focusing on an ambulatory patient who demonstrated significant improvement in both the modified Ashworth scale (MAS) score and gait pattern following the ITB injection trial.Case reportThis case report outlines the medical history of a 67-year-old male diagnosed with left-side hemiplegia and spastic dystonia, resulting from his second episode of intracranial hemorrhage in the right thalamus. An ITB injection trial was initiated because the patient was not suitable for continued botulinum toxin injections and oral medications. This was due to the persistent occurrence of spastic dystonia in both the upper and lower extremities. The patient underwent a four-day ITB injection trial with progressively increasing doses, resulting in improved MAS scores and gait parameters, including cadence, step length, step time, stride length, and stride time were increased. Particularly, kinematic gait analysis demonstrates a substantial improvement of increased knee flexion in the swing phase in stiff knee gait pattern. These findings indicated a gradual reduction in spasticity-related symptoms, signifying the positive effect of the ITB injection trial. The patient eventually received an ITB pump implantation.ConclusionIn this post-stroke patient with spastic dystonia, ITB therapy has demonstrated effective and substantial management of spasticity, along with improvement in gait patterns

Factors associated with stroke in patients with paroxysmal atrial fibrillation beyond CHADS2 score

Background: This study was conducted to investigate factors associated with stroke in pa­tients with paroxysmal atrial fibrillation (PAF) beyond CHADS2 score in terms of left ventricular (LV) diastolic function or left atrial (LA) function. Methods: One hundred and sixty-one patients with PAF and age less than 75 (mean age 61 ± 10; 69 male) who underwent transthoracic echocardiography were investigated. Patients were divided into two groups according to the stroke status (group 1 — no stroke vs. group 2 — presence of stroke). Baseline echocardiographic parameters and LA segmental (4 segments: basal septal, lateral, inferior, and anterior) strain rate (SR) during normal sinus rhythm were analyzed. Results: CHAD score (except S2) was similar between the two groups (0.6 ± 0.7 vs. 0.9 ± 0.7, p = 0.125). Patients with stroke had slightly lower body mass index (24.5 ± 2.7 vs. 23.4 ± ± 2.4, p = 0.052). Echocardiographic parameters did not show any differences in both systolic and diastolic functions between the two groups, however elevated E/E’ ratio was noted (9.5 ± ± 3.8 vs. 11.6 ± 3.9, p = 0.010) due to higher E velocity (63.5 ± 15.9 vs. 70.9 ± 16.0 cm/s, p = 0.046). In the analysis of LA SR, there are no differences of SR among the 4 segments. However, standard deviations (SD) of time to peak SR (SD of tA-SR) of the 4 segments were higher in patients with stroke (10.9 ± 9.9 vs. 22.1 ± 18.1 ms, p = 0.009) which indicates dyssynchronous contraction of LA. In multivariate analysis, SD of tA-SR (OR 1.074, CI 1.024–1.128, p = 0.004) and elevated E/E’ (OR 1.189, CI 1.006–1.406, p = 0.048) were independently associated with stroke in patients with PAF. Conclusions: Elevated E velocity, E/E’ and SD of tA-SR were associated with occurrence of stroke in patients with PAF even with similar CHAD scores. Increased SD of tA-SR and E/E’ were independently associated with stroke in patients with PAF.

The Effectiveness of Budesonide Nasal Irrigation After Endoscopic Sinus Surgery in Chronic Rhinosinusitis With Asthma

Objectives Budesonide nasal irrigation was introduced recently for postoperative management of patients with chronic rhinosinusitis. The safety and therapeutic effectiveness of this procedure is becoming accepted by many physicians. The objective of this study was to evaluate the efficacy of postoperative steroid irrigation in patients with chronic rhinosinusitis and asthma. Methods This prospective study involved 12 chronic rhinosinusitis patients with nasal polyps and asthma who received oral steroid treatment for recurring or worsening disease. The 22-item Sinonasal Outcomes Test (SNOT-22) and Lund-Kennedy endoscopy scores were checked before nasal budesonide irrigation, and 1, 2, 4, and 6 months after irrigation. We also calculated the total amount of oral steroids and inhaled steroids in the 6 months before irrigation and the 6 months after it. Results The mean SNOT-22 score improved from 30.8±14.4 before irrigation to 14.2±8.7 after 6 months of irrigation (P=0.030). The endoscopy score also improved from 7.4±4.7 before irrigation to 2.2±2.7 after 6 months (P<0.001). The total amount of oral steroid was decreased from 397.8±97.6 mg over the 6 months before irrigation to 72.7±99.7 mg over the 6 months after irrigation (P<0.001). Conclusion Nasal irrigation with budesonide is an effective postoperative treatment for chronic rhinosinusitis with asthma, which recurs frequently, reducing the oral steroid intake

Meteorological characteristics and assessment of the effect of local emissions during high PM10 concentration in the Seoul Metropolitan Area

In this study, we investigate the meteorological characteristics and the effect of local emissions during high PM10 concentrations in the Seoul Metropolitan Area (SMA) by utilizing data from a high-resolution urban meteorological observation system network (UMS-Seoul) and The Air Pollution Model (TAPM). For a detailed analysis, days with PM10 concentrations higher than 80 ??g m-3 for daily average PM10 concentration (classified as unhealthy by the Korean Ministry of Environment) in the Seoul Metropolitan Area (SMA) were classified into 3 Cases. Case I was defined as when the prevailing effect was from outside the SMA. Case II was defined as when the prevailing effect was a local effect with outside. Case III was defined as when the prevailing effect was local. Overall, high PM10 concentrations in the SMA mostly occurred under weak migratory anticyclone systems over the Korean Peninsula during warm temperatures. Prior to the PM10 concentration reaching the peak concentration, the pattern in each case was distinctive. After peak concentrations, however, the pattern for the 3 cases became less distinct. This study showed that nearly 50% of the high PM10 concentrations in the SMA occurred in spring and were governed by the conditions for Case II more than these for Cases I and III. In spring, the main sources of the high PM10 concentrations in the SMA were local emissions due to the predominance of weak winds and local circulation. The simulation showed that the non-SMA emissions were about 63 to 73% contribution to the spring high PM10 concentrations in the SMA. Specifically, local point sources including industrial combustion, electric utility, incineration and cement production facilities scattered around the SMA and could account for PM10 concentrations more than 10 ??g m-3 in the SMA

Differentially Expressed Potassium Channels Are Associated with Function of Human Effector Memory CD8+T cells

The voltage-gated potassium channel, Kv1.3, and the Ca2+-activated potassium channel, KCa3.1, regulate membrane potentials in T cells, thereby controlling T cell activation and cytokine production. However, little is known about the expression and function of potassium channels in human effector memory ( EM) CD8+ T cells that can be further divided into functionally distinct subsets based on the expression of the interleukin ( IL)-7 receptor alpha ( IL-7R alpha) chain. Herein, we investigated the functional expression and roles of Kv1.3 and KCa3.1 in EM CD8+ T cells that express high or low levels of the IL-7 receptor alpha chain ( IL-7R alpha(high) and IL-7R alpha(low), respectively). In contrast to the significant activity of Kv1.3 and KCa3.1 in IL-7Rahigh EM CD8+ T cells, IL-7Ralow EM CD8+ T cells showed lower expression of Kv1.3 and insignificant expression of KCa3.1. Kv1.3 was involved in the modulation of cell proliferation and IL-2 production, whereas KCa3.1 affected the motility of EM CD8+ T cells. The lower motility of IL-7Ralow EM CD8+ T cells was demonstrated using transendothelial migration and motility assays with intercellular adhesion molecule 1-and/or chemokine stromal cell-derived factor-1 alpha-coated surfaces. Consistent with the lower migration property, IL-7Ralow EM CD8+ T cells were found less frequently in human skin. Stimulating IL-7Ralow EM CD8+ T cells with IL-2 or IL-15 increased their motility and recovery of KCa3.1 activity. Our findings demonstrate that Kv1.3 and KCa3.1 are differentially involved in the functions of EM CD8+ T cells. The weak expression of potassium channels in IL-7Ralow EM CD8+ T cells can be revived by stimulation with IL-2 or IL-15, which restores the associated functions. This study suggests that IL-7Rahigh EM CD8+ T cells with functional potassium channels may serve as a reservoir for effector CD8+ T cells during peripheral inflammation.112Ysciescopu

Subthalamic deep brain stimulation in Parkinson's disease with SNCA mutations: Based on the follow-up to 10 years

Backgrounds: Although the short-term efficacy of bilateral subthalamic deep brain stimulation (DBS) has been reported in a limited number of Parkinson's disease (PD) patients with SNCA mutations, there are no data for long-term outcome. Methods: This multicenter retrospective study investigated previously reported PD patients with SNCA mutations, implanted with bilateral subthalamic DBS. We compared demographic and clinical data at baseline and last follow-up. Clinical data of motor and nonmotor symptoms and motor fluctuation were collected up to 10 years from DBS surgery. Results: Among four subjects, three had SNCA duplication and one had c.158C.A (p.A53E) mutation. The mean post-implantation follow-up duration was 5.4 +/- 3.7 years. All patients with SNCA duplication showed favorable outcome, although one died from breast cancer 1.5 years after DBS. The patient with the missense mutation became wheelchair-bound due to progressed axial, cognitive and psychiatric symptoms after 3.5 years from DBS despite the benefit on motor fluctuation. Conclusion: Based on findings in our small cohort, subthalamic DBS could be beneficial for motor fluctuation in PD patients with SNCA mutations, especially those with SNCA duplication, and cognitive and psychiatric symptoms are important for the long-term outcome of subthalamic DBS.</p

Genetic Analysis of 10 Unrelated Korean Families with p22-phox-deficient Chronic Granulomatous Disease: An Unusually Identical Mutation of the CYBA Gene on Jeju Island, Korea

Chronic granulomatous disease (CGD) is a rare hereditary disorder characterized by recurrent life-threatening bacterial and fungal infections. The underlying defect in CGD is an inability of phagocytes to produce reactive oxygen species as a result of defects in NADPH oxidase. Considering that CGD generally affects about 3-4 in 1,000,000 individuals, it is surprising that the prevalence of CGD on Jeju Island is 20.7 in 1,000,000 individuals. We performed genetic analysis on 12 patients from 10 unrelated families and found that all patients had an identical homozygous single-base substitution of C to T in exon 1 (c.7C>T) of the CYBA gene, which was expected to result in a nonsense mutation (p.Q3X). Because Jeju Island has long been a geologically isolated region, the high prevalence of CGD on Jeju Island is presumably associated with an identical mutation inherited from a common ancestor or proband

TAP1 and TAP2 Gene Polymorphisms in Korean Patients with Allergic Rhinitis

Antigen peptides are actively transported across the endoplasmic reticulum by the transporters associated with antigen presentation (TAP). TAP genes polymorphism could influence the selection process that determines which antigen peptides play a role in the pathogenesis of allergic rhinitis. The aim of this study was to investigate the association of TAP genes polymorphism with allergic rhinitis. TAP1 and TAP2 genotyping were performed on 110 allergic rhinitis patients and 107 healthy controls. TAP1 polymorphic residues at codons 333 and 637, and TAP2 polymorphic residues at codons 379, 565, 651, and 665 were analyzed by the amplification refractory mutation system-polymerase chain reaction (ARMS-PCR). Analysis of TAP1 gene polymorphism demonstrated decreased frequencies of Ile/Val genotype at codon 333, Asp/Gly genotype at codon 637, and haplotype A and B in allergic rhinitis patients when compared to controls (p<0.05). However, there was no significant difference in the genotype, phenotype, or allele frequencies at four TAP2 codons between controls and allergic rhinitis patients. In conclusion, TAP1 gene polymorphism may be an important factor contributing to the genetic susceptibility in the development of allergic rhinitis in the Korean population

A Case of Pulmonary Vein Tumor Presenting as a Left Atrial Mass

Primary cardiac tumors are extremely rare and can originate within the heart or be the result of tumor spread from other sites. We report a female patient with a pulmonary vein tumor extending into the left atrium that had a suspicious primary malignant origin with a sacral metastatic carcinoma. The patient was admitted complaining of pain in her buttock area as a result of a sacral tumor. It was believed that the sacral tumor was a metastasis from the imaging study and clinical manifestation. The primary malignant origin was evaluated. The chest CT showed a left atrium thrombus-like lesion without a pulmonary abnormality. After a transesophageal echocardiogram, the patient was diagnosed with a pulmonary vein tumor extending to the left atrium. The patient was given palliative radiotherapy for the sacral pain. Initially, the clinical impression was a metastatic sacral tumor with a thromboembolism of the left atrium. However, this patient was finally diagnosed with a pulmonary vein tumor with a left atrium extension by a transesophageal echocardiogram