BMC Neurology最新文献

Introduction: Cerebral ischemic strokes cause brain damage, primarily through inflammatory factors. One of the regions most affected by middle cerebral artery occlusion (MCAO) is the hippocampus, specifically the CA1 area, which is highly susceptible to ischemia. Previous studies have demonstrated the anti-inflammatory properties of quercetin. Therefore, this study aimed to investigate the neuroprotective effects of quercetin on hippocampal CA1 neurons following MCAO.

Materials and methods: Fifty-six male Albino Wistar rats were divided into seven groups (intact, sham, stroke, vehicle, and three quercetin-treated groups receiving 5, 10, and 20 mg/kg, respectively), each containing 8 rats. Various assessments, including brain water content, the rotarod test, the Bederson neurological score, the Morris water maze (MWM) test, the shuttle box test, histopathological evaluations, and measurements of interleukin-10 (IL-10) and interleukin-1β (IL-1β) levels, were conducted across the groups.

Results: Compared with control rats, 5 and 10 mg/kg quercetin-treated rats presented significant improvements in brain water content, neurological function, and motor function and improved performance in the MWM and shuttle box tests. Histopathological analyses revealed better preservation of CA1 neurons in these groups. Additionally, IL-10 levels significantly increased, whereas IL-1β levels significantly decreased. However, the group receiving 20 mg/kg quercetin showed no statistically significant changes in the parameters assessed (P > 0.05).

Conclusion: Quercetin may help prevent or ameliorate brain injuries caused by acute stroke, suggesting its neuroprotective effects. The reduction in IL-1β and increase in IL-10 may play key roles in quercetin's protective mechanism.

Background: Vanishing white matter disease (VWMD) is a rare autosomal recessive leukoencephalopathy. It is typified by a gradual loss of white matter in the brain and spinal cord, which results in impairments in vision and hearing, cerebellar ataxia, muscular weakness, stiffness, seizures, and dysarthria cogitative decline. Many reports involve minors. Very few instances worldwide have been reported, with adult onset of vanishing white matter considered to account for 15% of cases. Clinical evaluation, MRI results, and confirmatory genetic testing are used to diagnose VWMD.

Case presentation: A 39-year-old male from Hebron, Palestine, presented with a 7-month history of postural instability, imbalanced gait, and progressive deterioration of his lower extremities. Additionally, the patient suffered from ocular abnormalities and sphincteric issues. The patient's sibling showed comparable symptoms but was never diagnosed, as he passed away because of colon cancer. Reduced cognitive function, spastic quadriparesis, hyperreflexia, bradykinesia, and shuffling gait were found during a neurological examination. Normal results were obtained from routine laboratory tests, including cerebrospinal fluid (CSF), blood, and urine. Periventricular white matter hyperintensities, which are indicative of vanishing white matter leukoencephalopathy (VWML), were identified during an MRI. The diagnosis of adult-onset VWML with movement disability was substantiated by genetic testing, which named a homozygous pathogenic missense variant, EIF2B3, and a deletion in PRKN/PARK2. The patient's motor symptoms were temporarily alleviated following the administration of Levodopa/Carbidopa. Nevertheless, the long-term consequences are uncertain due to the illness's ongoing progression and the absence of a cure currently.

Conclusion: This instance of vanishing white matter leukoencephalopathy (VWML) is particularly remarkable in adults because of its rarity and complexity. The diagnosis is further complicated by the coexistence of Parkinsonism and VWML. Although a cure is not currently known. Early discovery is crucial to effectively manage symptoms. This example underscores the importance of more VWML research, particularly in Palestine, where studies on neurological disorders are limited. These findings underscore the importance of enhancing the region's diagnostic and therapeutic capabilities.

Background: Due to improved treatment options, more SMA patients reach childbearing age. Currently, limited data on pregnant SMA patients is available, especially in relation to disease-modifying therapies (DMT). This case report helps to elucidate new approaches for future guidelines in the management of pregnancy and SMA.

Case report: A 33-year-old wheelchair-bound patient with SMA type 3 (sitter) became pregnant following 36 months of Nusinersen treatment. The last dose was administered in the third gestational week. After pregnancy was confirmed, therapy was stopped immediately. A healthy child was born in the 34th gestational week by caesarean section. After a short nursing period, Nusinersen was restarted 6 weeks after the expected gestational date. At this time, the patient reported deteriorated motor functions, which stabilized at a lower level compared to pre-pregnancy in the 2-year follow-up, despite restarting Nusinersen treatment.

Discussion: So far, only few cases of successful pregnancies of SMA patients on DMT have been reported. In natural history, the majority of patients experienced an increased deterioration of motor function while fetal outcome was not impaired. Our case shows that although Nusinersen treatment was applied in the third gestational week prior to proof of pregnancy, outcome was positive for mother and child. Future studies will have to determine whether ongoing treatment with Nusinersen during pregnancy should be recommended.

Background: Parkinson's disease (PD) exerts a considerable burden on the elderly. Studies on long-term costs for Parkinson's disease patients in Taiwan are not available.

Objectives: This study aims to examine the medical resource utilization and medical costs including drug costs for PD patients in Taiwan over up to 15 years of follow-up.

Methods: Incident PD patients and matched non-PD subjects were identified between 2003 and 2016 from the National Health Insurance (NHI) research database. Differences in annual healthcare utilization and costs between PD and non-PD subjects from 2003 to 2018 were predicted by generalized linear models. We performed analyses stratified by PD severity and also by age, gender, and duration of follow-up.

Results: We identified 50,290 PD cases and 201,153 non-PD subjects. From the payer's perspective, the average total medical costs (drug costs) associated with PD and non-PD subjects were NT$631,080 (NT$222,810) and NT$480,880 (NT$140,120), respectively. Total medical and drug costs of PD after diagnosis remained high, from NT$138,487 per patient in the first year following diagnosis up to NT$154,676 per patient at year 15. The largest components of costs were for outpatient care (55% of total medical costs), and total drugs cost (35% of total medical costs). Patients with severe PD incurred higher total medical costs compared to those with moderate or mild PD, with outpatient and inpatient costs as well as drug costs rising with disease severity.

Conclusions: This is the first study of its kind in Taiwan that comprehensively analyzes long-term healthcare utilization and costs among PD patients. PD imposes a significant economic burden in Taiwan, with medical (drug) costs being 1.31 (1.59) times that of non-PD individuals and costs increasing substantially with PD severity. Our findings can aid health policymakers in understanding the healthcare needs and medical costs of PD patients, supporting effective policy formulation.

Background and purpose: White matter hyperintensities in brain MRI are key indicators of various neurological conditions, and their accurate segmentation is essential for assessing disease progression. This study aims to evaluate the performance of a 3D convolutional neural network and a 3D Transformer-based model for white matter hyperintensities segmentation, focusing on their efficacy with limited datasets and similar computational resources.

Materials and methods: We implemented a convolution-based model (3D ResNet-50 U-Net with spatial and channel squeeze & excitation) and a Transformer-based model (3D Swin Transformer with a convolutional stem). The models were evaluated on two clinical datasets from Kaohsiung Chang Gung Memorial Hospital and National Center for High-Performance Computing. Four metrics were used for evaluation: Dice similarity coefficient, lesion segmentation, lesion F1-Score, and lesion sensitivity.

Results: The Transformer-based model, with appropriate adjustments, outperformed the well-established convolution-based model in foreground Dice similarity coefficient, lesion F1-Score, and sensitivity, demonstrating robust segmentation accuracy. DRLoc enhanced the Transformer's performance, achieving comparable results on internal and benchmark datasets despite limited data availability.

Conclusion: With comparable computational overhead, a Transformer-based model can surpass a well-established convolution-based model in white matter hyperintensities segmentation on small datasets by capturing global context effectively, making them suitable for clinical applications where computational resources are constrained.

Background: Tyrosine kinase inhibitors (TKIs) improve prognosis in chronic myeloid leukemia (CML). Nilotinib and ponatinib, second- and third-generation TKIs, respectively, have been reported to cause adverse vascular occlusive events such as myocardial infarction and peripheral arterial disease. However, little is known about the risk of cerebral infarction associated with severe cerebrovascular stenosis, which is a late complication of TKIs. Herein, we report two cases of cerebrovascular stenosis associated with TKIs for CML.

Case presentation: A 53-year-old man with CML experienced transient right-sided hemiparesis and dysarthria. The patient had been treated with ponatinib for 5 years. Digital subtraction angiography revealed diffuse stenosis with luminal narrowing from the terminal portion of the internal carotid artery (ICA) to the entire M1 length of the middle cerebral artery (MCA). He was diagnosed with hemodynamic cerebral ischemia due to severe intracranial ICA stenosis and underwent superficial temporal artery (STA)-MCA bypass surgery. He had no atherosclerotic factors or immunological serum markers such as vasculitis. As a side effect of TKI therapy was suspected, ponatinib therapy was discontinued. A 74-year-old man treated with nilotinib for CML presented with gait disturbances. Diffusion-weighted magnetic resonance imaging revealed multiple infarctions in the right cerebral hemisphere, and magnetic resonance angiography revealed severe bilateral intracranial ICA and MCA stenosis. The patient underwent a STA-MCA bypass surgery. We discontinued nilotinib treatment. The postoperative course was uneventful.

Conclusions: CML prognosis has steadily improved with the advent of new TKIs. In the future, reports of cerebrovascular stenosis caused by TKIs for CML may increase and systemic complications may become a problem. We should be aware that some TKIs may cause cerebrovascular stenosis.

Background: Awareness of the characteristics of glial fibrillary acidic protein autoantibody (GFAP-IgG) associated myelitis facilitates early diagnosis and treatment. We explored features in GFAP-IgG myelitis and compared them with those in myelitis associated with aquaporin-4 IgG (AQP4-IgG) and myelin oligodendrocyte glycoprotein IgG (MOG-IgG).

Methods: We retrospectively reviewed data from patients with GFAP-IgG myelitis at the First Affiliated Hospital of Zhengzhou University and Henan Children's Hospital from May 2018 to May 2023. AQP4-IgG and MOG-IgG myelitis patients served as controls.

Results: Thirty-four patients with GFAP-IgG myelitis were included (15 women, 12 children; median age at onset, 28.5 years). Over half experienced prodromal symptoms and required intensive care support. The median Expanded Disability Status Scale (EDSS) score was 4 at admission and 0 at final follow-up (median, 20 months). Cerebrospinal fluid (CSF) analysis showed markedly elevated leukocyte counts in 23 patients, elevated total protein in 28 patients, and decreased glucose levels in 9 patients. Longitudinally sagittal T2 and gadolinium-enhancing spinal cord lesions were detected. Features favoring GFAP-IgG over the other types included presence of fever and neck stiffness, requirement of intensive care and mechanical ventilation, higher monocyte-to-lymphocyte ratio (MLR), presence of hyponatremia, markedly elevated CSF leukocyte counts, increased CSF total protein levels, and decreased CSF glucose levels. Imaging findings more common in GFAP-IgG than in AQP4-IgG myelitis were longer diseased segments, central canal enhancement, and gadolinium-enhancing brain lesions. Higher EDSS scores at discharge distinguished GFAP-IgG from MOG-IgG.

Conclusion: Clinical, laboratory, imaging, and outcome variables facilitate differential diagnosis of myelitis subtypes.

Background: Migraine is associated with cervical artery dissection (CeAD). Calcitonin gene-related peptide (CGRP) is a multifunctional neuropeptide with vasodilatory effects. The use of anti-CGRP monoclonal antibodies (CGRP mAb) may affect cerebrovascular disease risk; however, no reports have associated CGRP mAb with CeAD.

Case presentation and faers database analysis: We report a case of vertebral artery dissection in a 39-year-old woman with migraine treated with galcanezumab. We searched the number of cases where cerebral and cervical artery dissection were reported as adverse effects of CGRP mAb using the FDA Adverse Event Reporting System (FAERS) database. Six and ten such cases were reported regarding galcanezumab and CGRP mAbs use, respectively. The reporting odds ratios for galcanezumab and CGRP mAbs were elevated.

Conclusion: Although migraine is reported to be associated with CeAD, the use of CGRP mAb might be related to CeAD and warrant further investigation.

This is an unusual case of voltage gated calcium channel (VGCC) antibodies leading to two distinct and chronologically separated neurologic syndromes without the presence of an underlying neoplasm. Lambert Eaton Myasthenic Syndrome (LEMS) presented five years prior to cerebellar ataxia. Both LEMS and cerebellar ataxia were responsive to treatment, but not the same therapy. He was diagnosed with LEMS through history, exam, electromyography/nerve conduction studies (EMG/NCS) with repetitive nerve stimulation (RNS) and antibody testing. He was treated with 3,4 diaminopyridine (3,4 DAP) with an excellent response. Five years later, he developed acute ataxia. The patient required months of intensive and continued immunomodulating therapy.