Ideggyogyaszati Szemle-Clinical Neuroscience最新文献

Background - Leukodystrophies, a hete­ro­­ge­neous group of brain and spinal cord dis­orders, often pose challenges in es­tab­li­shing molecular etiology. Vanishing White Matter Disease (VWMD) is a rare sub­type of leu­ko­dys­trophies presenting with characteristic clinical and MRI features, ne­ver­theless, achieving diag­nostic certainty requires genetic studies.

Case presentation - Our patient is a nine year old girl, who developed progressive gait difficulties at around 3-4 years of age. Her brain MRI showed confluent lesions with in­­creased signal intensity in the cerebral and cerebellar white matter on T2/FLAIR se­quen­ces, within which hypointense regions ap­peared with signal intensity resembling that of the cerebrospinal fluid on T1 sequences. Whole exome sequencing identified a homozygous likely pathogenic variant within the EIF2B5 gene in the proband, which was present in a heterozygous state in both asymptomatic parents. Having the clinical and molecular genetic diagnosis established, we explored therapeutic possibilities for the patient.

Conclusion - VWMD is a severe form of leukodystrophies with little or no disease modifying therapy available until recently. A better understanding of its molecular pathogenesis offers some hope for new inventive therapies. 

Background and purpose:

Chronic subdural hematoma (cSDH) is a challenging pathology with high recurrence rate after surgical treatment and may seriously affect the patient’s quality of life. Membrane formation with angiogenesis plays an important role in the evolution of the disease, providing a promising target for endovascular therapy. Our goal is to categorize angiographic patterns of chronic subdural hematoma for standardized reporting purposes.

Methods:

In our retrospective analysis of prospective data collection, we analyzed angiographic properties of all high recurrence risk patients with cSDH, who were treated by embolization in our hospital between February 2019 and June 2020. Altogether 17 patients were included in the analysis. 

Results:

Based on superselective angiography of the middle meningeal artery (MMA) in the two standard, AP and lateral views, three distinct categories of dural supply were defined: normal vascular pattern (Grade I), cottonwool appearance without enlargement of the MMA branches (Grad II) and strong cottonwool like staining with dilatative remodelling of the MMA branches (Grade III).

Conclusion:

The proposed grading system of the angiographic appearance of cSDH, representing the pathophysiological evolution of the disease should be correlated to therapeutic success rates and could be applied in future clinical studies.

Chronic subdural hematoma is one of the most common diseases requiring a neurosurgical operation that affect elderly and fragile patients. In addition to standard neurosurgical operations (trepanation and craniotomy), embolization of the meningeal artery media is an alternative solution. Several review aerticles have confirmed the very high rate of success and safety of the endovascular treatment. We present the technical details and results of our 10 consecutive selective media meningeal artery embolization procedures for residual chronic subdural hematomas. Our interventions were performed without complications and all resulted in complete recovery. 

Background and purpose:

Long Covid is a complex con­dition characterised by symptoms that per­sist for weeks and months after the Co­vid infection, accompanied by cognitive im­pairment that negatively affects daily life. Understanding this complex condition is im­portant for the development of diagnostic and therapeutic strategies.

This article aims to provide a comprehensive overview of cognitive impairment in long-COVID, including its definition, symptoms, pathophysiology, risk factors, assessment tools, imaging abnormalities, potential biomarkers, management strategies, long-term outcomes, and future directions for research.

Methods:

The search methodology used in this review aimed to include a wide range of research on cognitive impairment related to both COVID-19 and long-COVID. Systematic searches of PubMed and Google Scholar databases were conducted using a mixture of MeSH terms and keywords including ‘cognition’, ‘cognitive impairment’, ‘brain fog’, ‘COVID-19’ and ‘long-COVID’. The search was restricted to studies published in English between 1 January 2019 and 11 February 2024, which presented findings on neurological manifestations in human participants.

Results:

Long-COVID is characterized by persistent symptoms following COVID-19 infection, with cognitive impairment being a prominent feature. Symptoms include brain fog, difficulties with concentration, memory issues, and executive function deficits. Pa­tho­physiological mechanisms involve vi­ral persistence, immune responses, and vas­cular damage. Risk factors include age, pre-existing conditions, and disease seve­rity. Cognitive assessment tools such as the Montreal Cognitive Assessment (MoCA) are essential for diagnosis. Imaging studies, including MRI, PET, and SPECT, reveal structural and functional brain alterations. Potential biomarkers include C-reactive protein, interleukin-6, and neuron-specific enolase. Management strategies encompass cognitive rehabilitation, occupational therapy, medications, and lifestyle modifications.

Conclusion:

Long-COVID poses a multifaceted challenge, and cognitive impairment significantly impacts patients’ lives. A multi­disciplinary approach, including cognitive rehabilitation and medication when appropriate, is essential for effective management. Future research should focus on validating biomarkers and understanding long-term cognitive outcomes.

Conclusion – Long-COVID is a global health concern, and cognitive impairment is a distressing symptom. While pharmacological interventions have potential, they require careful consideration. Continued research is crucial for improving the understanding and treatment of cognitive impairment in long-COVID.

Background and purpose:

Human brain aneurysms may often prove fatal if not re­cognized in time and treated accordingly. The understanding of development and rupture of aneurysms can significantly be improved by the application of numerical modelling, which in turn, requires the knowledge of mechanical properties of vessel wall. This study aims to identify assumed differences with respect to age, sex, spatial orientation, and rupture by utilizing detailed statistical analysis of uniaxial tensile measurements of human brain aneurysm samples, performed by the authors in a previous project.

Methods:

At surgery of 42 patients, aneu­rysm fundi were cut distally to the clip. In each case, depending on size, varying number of stripes (altogether 88) were prepared and uniaxial stress-strain measurements were performed. Quantities related to the capacity, energy absorption or stiffness were determined and statistically analysed.

Results:

The number of specimens in the aneurysm sample was sufficient to establish statistical differences with respect to sex and rupture (p<0.05). No significant differences were detected in orientation, though higher values of stresses and deformations were ob­tained in the circumferential direction com­pared to the meridional direction. 

Conclusion:

Significant differences bet­ween sexes with respect to ultimate deformations were demonstrated according to expectation, and the hypothesis on equality of energy capacity could be supported. Similarity of curves with respect to specimen orientation was also observed and ruptured aneurysm sacs tended to be smaller in size. It seems that differences and trends described in this paper are realistic and need to be applied in numerical modelling.

Peripheral nerve injuries after being struck by lightning have been documented. Here, we report a case of cauda equina syndrome induced by lightning. A 27-year-old man presented with numbness, a burning sensation in the saddle region, and increased urinary urgency after being struck by lightning. He had absent Achilles reflexes and paresthesia in the saddle region upon neurological examination, and magnetic resonance imaging of the spine was normal. Electrophysiological studies indicated involvement of bilateral L5, S1, and S2 myotomes and revealed cauda equina lesions. 
Peripheral nerve injury induced by lightning is rare, and the evaluation of people with neurological complaints using electromyography will help determine the true incidence.

Background and purpose:

Pain intensity is the most frequently assessed health domain in clinical studies among patients with low-back pain. Visual analogue scale (VAS) and Numeric rating scale (NRS) have been the mostly used measurement tools for pain intensity. We proposed to correlate these instruments to a generic health-related quality of life measurement tool in order to show the scale with superior clinical relevance.

Methods:

We used cross-sectional, convenience sampling. 120 patients with chronic low-back pain administered the 29-item Patient Reported Outcomes Measurement Information System Profile with NRS included, and the VAS scale in the National Institute of Mental Health, Neurology and Neurosurgery. We determined the correlation between PROMIS domain T-scores and VAS and NRS scores.

Results:

We performed Spearman rank correlation test to calculate the correlation coefficient. We found VAS scales measuring pain had weak to moderate correlations with all PROMIS health domains (r = 0.24–0.55). Therefore, we compared correlation of PROMIS domain scores with PROMIS pain intensity numeric rating scale and VAS scales. PROMIS domains had moderate to strong correlations with pain intensity scale (r = 0.45–0.71). PROMIS physical function short form [r = –0.65, 95% CI (–0.75) – (–0.55)] and PROMIS pain interference short form (r = 0.71, 95% CI 0.63 – 0.79) had the strongest correlation with pain intensity item.

Conclusion:

NRS has showed greater correlation with PROMIS domain T-scores than VAS scale. This may prove that NRS has greater connection to another health domains, thus it correlated more to health-related quality of life than visual scale. We recommend NRS to use in further clinical studies conducted among patients with low-back pain.

Background and purpose:

Discontinua­tion of medication still remains a key element in the treatment of medication overuse headache (MOH), but there is no consensus on the withdrawal procedure. We aimed to share the promising results of anesthetic blockade of greater occipital nerve (GON), which can be an alternative to existing treatments during the early withdrawal period of MOH treatment.

Methods:

This study was conducted using regular electronic medical records and headache diaries of patients diagnosed with MOH and treated with anesthetic GON blockade with 0.5% bupivacaine solution in a specia­lized headache outpatient clinic. A total of 86 patients who developed MOH while being followed up for chronic migraine were included in the study.

Results:

The treatment schemes for MOH are based on expert consensus and withdrawal strategies are the most challenging part of treatment. In our study, numerical rating scale for headache intensity, overused medication consumption per month, headache frequency (day/month) and the duration of each attack (hour/day) decreased significantly in the first month compared to pre-treatment (p < 0.01). 

Conclusion:

Conclusion – Our study suggests that GON blockade can be used as a good alternative therapy in the treatment of MOH.

Background and purpose:

The management of central retinal artery occlusion (CRAO) has long been conservative therapy with limited efficacy carried out in ophthalmology departments together with etiolo­gi­cal investigations lacking a standardised protocol. However, CRAO is analogous to ischemic central nervous system stroke and is associated with increased stroke risk, thus, systemic thrombolysis treatment and multidisciplinary management can be beneficial. Since May 2022, at Semmelweis University CRAO patients diagnosed within 4.5 hours are given intravenous thrombolysis therapy and undergo etiologic workup based on current stroke protocols. Here we report our experience with the multidisciplinary, protocol-based management of CRAO in comparison with former non-protocol based ophthalmological conservative treatment.

Methods:

We reviewed CRAO patients’ data treated conservatively and with paracentesis within 6 hours at the Department of Ophthalmology between 2013 and 2022 including changes in visual acuity, neurolo­gical and cardiovascular findings compared to those in the thrombolysis project. 

Results:

Of the 78 patients receiving non-protocol care, visual improvement was seen in 37% with natural course, 47% with conservative treatment and 47% with paracentesis. Four patients had significant carotid stenosis (2 underwent endarterectomy), 1 carotid dissection, 6 cardioembolism and 1 giant cell arteritis. Of the 4 patients within 4,5 hours, 3 gave their consent to the clinical trial and were treated with thrombolysis and underwent a full etiological assessment. 
2 pa­tients had improved visual acuity, 2 pa­tients had significant carotid stenosis and underwent endarterectomy, 1 patient was started on anticoagulation for newly diagnosed atrial fibrillation.

Conclusion:

CRAO patients presenting within 4,5 hours are rare and more patients are needed in our study to establish the efficacy of thrombolysis. However uniform protocollized evaluation helps identifying embolic sources thus, avoiding further and potentially more serious thromboembolic events.

The treatment of acute epidural haematoma is surgery as soon as possible, elimination of the source of bleeding and evacuation of the haematoma. In case of small epidural haematoma, strict neurological and radiological follow-up is necessary. In a significant percentage of cases, open surgery must also be performed within a few days. In case of small epidural haematomas, embolization of the middle meningeal artery is considered as an alternative solution. We review the literature on middle meningeal artery embolization and present our first treatment. Our case report is the first European report about an acute epidural haematoma which was treated by embolization of middle meningeal artery. Our case study is the first report in which a patient was treated with both open surgery and endovascular treatment for acute epidural haematoma within a year.