Neurology International最新文献

Neurodegenerative dementia, a collective term for a range of disorders characterized by progressive cognitive and functional decline, is an urgent challenge in global healthcare [...].

Introduction: Serum metabolome changes after acute ischemic stroke (AIS), but the significance of this is poorly understood. We evaluated whether this change is associated with AIS outcomes in patients with large vessel occlusion (LVO). To improve validity, we combined cross-sectional and longitudinal designs and analyzed serum using Nuclear Magnetic Resonance (NMR) and Liquid Chromatography-Mass Spectrometry (LC-MS). Methodology: In the cross-sectional part, we compared serum metabolome from 48 LVO strokes, collected at 48-72 h, and analyzed with NMR, while in the longitudinal part, we compared metabolome from 15 LVO strokes, collected at <24 h, 48-72 h, 5-7 days, and 80-120 days, and analyzed with LC-MS between patients with modified Rankin Scores (mRS) of 0-3 and 4-6 at 90 days. We hypothesized that compounds elevated in patients with mRS 0-3 in the cross-sectional part would also be elevated in the longitudinal part, and vice versa. We used regression for the analysis and TSBH for multiple testing. Results: In the cross-sectional part, cholesterol, choline, phosphoglycerides, sphingomyelins, and phosphatidylethanolamines had lower levels in patients with an mRS of 0-3 compared to an mRS of 4-6. In the longitudinal part, lower levels of sphingomyelin (d18:1/19:0, d19:1/18:0)* significantly correlated with an mRS of 0-3 in patients with small infarction volume, while lower levels of sphingolipid N-palmitoyl-sphingosine (d18:1/16:0), 1-palmitoyl-2-docosahexaenoyl-GPC (16:0/22:6), 1-palmitoyl-2-docosahexaenoyl-GPE, palmitoyl-docosahexaenoyl-glycerol (16:0/22:6), campesterol, and 3beta-hydroxy-5-cholestenoate correlated with an mRS of 0-3 in patients with large infarction volume. Conclusions: This pilot study showed that lower levels of lipidomic components nerve cell membrane correlate with good AIS outcomes. If proven on large-scale studies, these compounds may become important AIS outcome markers.

Objectives: Nonconvulsive status epilepticus (NCSE) is a disease with a high mortality rate and a very diverse etiology. The disease prognosis is related to the etiology. We aimed to investigate the etiology, mortality rates, and factors affecting mortality in patients diagnosed with NCSE in a tertiary epilepsy center in Turkey.

Methods: All electroencephalograms (EEGs) were taken in the EEG laboratory of the Department of Neurology, Antalya Training and Research Hospital, between June 2021 and February 2024. Patients who met the Salzburg Consensus Criteria (SCC) for NCSE were included. Demographic data, etiologic factors, comorbidities, neuroradiological imaging, laboratory data, treatments administered for NCSE and responses to treatment, short- and long-term outcomes, and EEG findings at follow-up, if any, were noted from the medical records of all patients who met the criteria.

Results: A total of 200 patients were included in the study. Mortality was observed in 76 (38.4%) patients with NCSE. There was a statistically significant correlation between NCSE etiology and mortality (p < 0.001). Mortality was most common in patients with cerebral tumors as the etiology, with a rate of 63.6%. The lowest mortality rate was observed in patients with autoimmune encephalitis and epilepsy (14.3% and 17.2%, respectively). After appropriate antiseizure medication (ASM) treatment, 117 (58.5%) patients with NCSE improved. When post-treatment improvement and etiologic factors were analyzed, the highest rate of improvement was observed in the autoimmune encephalitis and CVD groups.

Conclusions: Our study showed that advanced age and the presence of stroke are associated with mortality and that patients with NCSE due to autoimmune encephalitis respond well to treatment.

Background: Metachromatic leukodystrophy (MLD) is a rare autosomal recessive disorder that causes demyelination of both the central (CNS) and peripheral nervous systems (PNS). Objective: This study aims to report a unique MLD case presenting with cranial neuropathies and ataxia, initially without white matter changes on MRI, leading to diagnostic uncertainty. Results: A 20-month-old presented with bilateral abduction deficits, facial diplegia, and ataxia, raising the possibility of an acquired demyelinating condition. An MRI scan showed the enhancement of multiple cranial nerves, but normal white matter. A follow-up MRI showed new white matter changes that spared the U-fibers, suggesting a leukodystrophy. Biochemical assays were suggestive of metachromatic leukodystrophy, which was confirmed with genetic testing demonstrating a homozygous c.848+3A > G variant in ARSA. Conclusions: Our patient suggests that the initial presentation of MLD may mimic an acquired demyelinating condition and manifest with multiple cranial nerve palsies before more typical white matter changes evolve.

Background/Objectives: Intracerebral hemorrhage (ICH) and subarachnoid hemorrhage (SAH) are severe forms of stroke with high morbidity and mortality rates. HMG-CoA reductase inhibitors, commonly referred to as statins, known for their lipid-lowering abilities, also possess pleiotropic properties, including anti-inflammatory and neuroprotective effects. We aimed to evaluate the impact of statin therapy on the functional outcomes and survival in patients with ICH and SAH. Methods: This retrospective cohort study analyzed data from the Get With The Guidelines (GWTG) stroke registry at a tertiary care center, including patients diagnosed with ICH or SAH between January 2008 and June 2022. Patients were categorized based on prior initiation of statin therapy: no statin, low-intensity statin, or high-intensity statin. The primary outcome was the Modified Rankin Scale (mRS) score at discharge, dichotomized to good (0-2) and poor (3-6) outcomes. A multivariate logistic regression model controlled for age, gender, and National Institutes of Health Stroke Scale (NIHSS) score at admission. Results: A total of 663 patients with ICH and 159 patients with SAH were included in the analysis. In the ICH patients, low-intensity statin therapy was associated with significantly higher odds of a good functional outcome (aOR 2.56, 95% CI 1.247-5.246, p = 0.0104), as was high-intensity statin therapy (aOR 2.445, 95% CI 1.313-4.552, p = 0.0048). Among the SAH patients, all 39 deaths occurred in the no statin therapy group. Conclusions: Both low- and high-intensity statin therapy are associated with improved functional outcomes in ICH and may offer a survival benefit in SAH. These findings highlight the potential neuroprotective role of statins in hemorrhagic stroke. Further prospective studies and randomized controlled trials are needed to confirm these observations and to clarify the optimal use of statins in this patient population.

Background: The purpose of this study is the evaluation of imaging findings of acute-phase cardiac CT (cCT) in stroke patients with large vessel occlusion (LVO) to identify potential cardioembolic sources (CES) in patients without intracardiac thrombi and atrial fibrillation (AF). Material and Methods: This retrospective study included 315 patients with LVO who underwent cCT imaging in the acute stroke setting. The images were analysed for 15 imaging findings following the established minor and major cardioembolic risk factors. The final stroke aetiology was determined using the TOAST classification through interdisciplinary consensus following a thorough clinical evaluation. Multivariate regression analysis was performed to identify imaging findings associated with CES. Results: A cardioembolic aetiology was identified on cardiac CT in 211 cases (70%). After adjustment for AF and intracardiac thrombi, the multivariate regression analysis revealed significant associations with left ventricular dilation (adjusted odds-ratio (AOR) 32.4; 95% CI 3.0-349; p = 0.004), visible interatrial right-to-left shunt (AOR 30.8; 95% CI 2.7-341.3; p = 0.006), valve implants (AOR 24.5; 95% CI 2.2-270.9; p = 0.009), aortic arch atheroma grade > II (AOR 6.9; 95% CI 1.5-32.8; p = 0.015) and post-ischaemic myocardial scars (AOR 6.3, 95% CI 1.2-34.1; p = 0.032) as independent risk factors for a cardioembolic aetiology. The combined model achieved an area under the ROC curve of 0.83. Conclusions: In patients with LVO without AF and intracardiac thrombi as a cause, the presence of left ventricular dilatation, interatrial right-to-left shunts, valve implants, post-ischaemic myocardial scarring and advanced aortic arch atheroma (grade > 2) in particular is significantly associated with a cardioembolic cause of stroke and should be add-on evaluated in acute-phase cCT. Further investigations are warranted to confirm these associations.

Alzheimer's and Parkinson's are the most common neurodegenerative diseases (NDDs). The development of aberrant protein aggregates and the progressive and permanent loss of neurons are the major characteristic features of these disorders. Although the precise mechanisms causing Alzheimer's disease (AD) and Parkinson's disease (PD) are still unknown, there is a wealth of evidence suggesting that misfolded proteins, accumulation of misfolded proteins, dysfunction of neuroreceptors and mitochondria, dysregulation of enzymes, and the release of neurotransmitters significantly influence the pathophysiology of these diseases. There is no effective protective medicine or therapy available even with the availability of numerous medications. There is an urgent need to create new and powerful bioactive compounds since the number of people with NDDs is rising globally. Heterocyclic compounds have consistently played a pivotal role in drug discovery due to their exceptional pharmaceutical properties. Many clinically approved drugs, such as galantamine hydrobromide, donepezil hydrochloride, memantine hydrochloride, and opicapone, feature heterocyclic cores. As these heterocyclic compounds have exceptional therapeutic potential, heterocycles are an intriguing research topic for the development of new effective therapeutic drugs for PD and AD. This review aims to provide current insights into the development and potential use of heterocyclic compounds targeting diverse therapeutic targets to manage and potentially treat patients with AD and PD.

Background: Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune disease and approximately 74 cases have been reported in the literature, mostly in childhood. Methods: We reported this case report according to the CARE guidelines. Results: A 13-year-old female presented with a 4-day history of persistent fever and hallucinations. She rapidly developed nystagmus associated with blurred vision with ataxic gait. She also developed altered mental status, blepharoptosis, diplopia and extrinsic ocular motility. An EEG showed asymmetric brain electrical activity with slow and spiky abnormalities in the left cerebral hemisphere. Lumbar puncture showed mild pleocytosis with lymphocytic predominance, elevated protein, with normal glucose. Anti-GM1 and anti-GM2 antibodies were positive. She was administered intravenous immunoglobulin therapy due to a suspicion of BBE, showing rapid improvement in mental status. Conclusions: BBE is a diagnosis of exclusion and should be considered especially in pediatric age.

Peripheral nerve injuries are common complications in surgical and dental practices, often resulting in functional deficiencies and reduced quality of life. Current treatment choices, such as autografts, have limitations, including donor site morbidity and suboptimal outcomes. Adipose-derived stem cells (ADSCs) have shown assuring regenerative potential due to their accessibility, ease of harvesting and propagation, and multipotent properties. This review investigates the therapeutic potential of ADSCs in peripheral nerve regeneration, focusing on their use in bioengineered nerve conduits and supportive microenvironments. The analysis is constructed on published case reports, organized reviews, and clinical trials from Phase I to Phase III that investigate ADSCs in managing nerve injuries, emphasizing both peripheral and orofacial applications. The findings highlight the advantages of ADSCs in promoting nerve regeneration, including their secretion of angiogenic and neurotrophic factors, support for cellular persistence, and supplementing scaffold-based tissue repair. The regenerative capabilities of ADSCs in peripheral nerve injuries offer a novel approach to augmenting nerve repair and functional recovery. The accessibility of adipose tissue and the minimally invasive nature of ADSC harvesting further encourage its prospective application as an autologous cell source in regenerative medicine. Future research is needed to ascertain standardized protocols and optimize clinical outcomes, paving the way for ADSCs to become a mainstay in nerve regeneration.

Background/objectives: Brown tumors are rare bone lesions associated with hyperparathyroidism, particularly secondary hyperparathyroidism (SHPT), in chronic renal failure. While brown tumors commonly affect bones rich in marrow, the involvement of the sphenoid sinus is extremely rare and can present with neurological symptoms. This study reports a case of a sphenoid sinus brown tumor in a patient on hemodialysis, highlighting its clinical presentation and diagnostic challenges.

Methods: A 31-year-old woman undergoing chronic hemodialysis presented with a severe headache, diplopia, and progressive vision loss in her left eye. Laboratory tests revealed hypercalcemia, hypophosphatemia, and elevated parathyroid hormone (PTH) levels, consistent with SHPT. The diagnosis was confirmed through a clinical examination and magnetic resonance imaging (MRI).

Results: The clinical examination confirmed decreased visual acuity in the left eye. The laboratory results revealed serum calcium of 15.5 mg/dL, phosphate of 1.0 mg/dL, and PTH of 2000 pg/mL, consistent with SHPT. The imaging studies identified a brown tumor in the sphenoid sinus exerting a mass effect on adjacent structures. This case underscores the rarity of brown tumors in this location, with very few similar reports in the literature.

Conclusions: Although rare, brown tumors should be considered in patients with SHPT who present with neurological symptoms or cranial lesions. An early diagnosis through biochemical and imaging studies is crucial to prevent severe complications. The management involves treating the underlying hyperparathyroidism, with surgical intervention indicated in cases of neural compression.