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Child's Nervous System : ChNS :... Jun 2024Acalvaria, or acrania, is a rare congenital cranial vault defect with neurocranium absences, including complete or part of calvaria flat bones, dura mater, and...
BACKGROUND
Acalvaria, or acrania, is a rare congenital cranial vault defect with neurocranium absences, including complete or part of calvaria flat bones, dura mater, and associated muscles, but with a still present in the central nervous system, skull base, facial bones, and skin-covered the defect. It is a sporadic incidence without apparent genetic factors confirmed. Acalvaria is often misdiagnosed as anencephaly; the distinguishable difference is that anencephaly has an absence (partial or complete) of the brain tissue, including the skull and scalp. Acalvaria is considered a fatal anomaly with a low survival rate, and only a few cases of extended survival have been reported until now. To the best of the author's knowledge, no acalvaria case has been published in Papua, and only one reported case of the coexistence of acalvaria with schizencephaly in Brazil (2018).
CASE REPORT
Herein, we present a case of an indigenous South Papuan living newborn with primary acalvaria and open-lip schizencephaly in a frontoparietal region. A male newborn baby was born from a 39-year-old female Marind-Anim tribe patient with a 38th week of gestation, with no previous history of miscarriage, is not a consanguineous marriage, and had an unremarkable medical history during this pregnancy. Post-natal physical examinations showed an irregularly shaped head with 11.5 cm diameter concave of the right side, with a soft brain-like consistency palpable and the absence of half right frontoparietal calvarium covered with a presence of scalp and hair. Cranial 2-dimension ultrasonography shows an absence of half right frontoparietal calvaria bone with a complete presence of scalp and periosteum covering the defect with a fluid accumulation (anechoic) below the periosteum. A transverse axis view shows a complete structure but hypoplasia of brain cortex with visible slightly dysgenesis of gyrus and sulcus in both hemispheres convincing the acalvaria condition not anencephaly. A fluid accumulation above brain parenchyma of the frontoparietal region happened to be a cerebrospinal fluid coming from a wide-open cleft extending from the left lateral and fourth ventricles to the cerebral cortex, suggesting a typical condition of open-lip schizencephaly. Further health follow-ups until 6 months old showed still normal physical and behavioral development with no sign of complications.
CONCLUSIONS
No standard acalvaria treatment is being established; conservative and supportive therapy is mostly taken considering their low survival rate. With the advancement of medical technology nowadays, surgical approaches, including scalp defect closure, bone graft, and 3D-printed defect filling, are being performed and have succeeded in a few cases. Long-term follow-up is required to monitor their neuro-psychological development and complication incidences that need further intervention.
PubMed: 38822831
DOI: 10.1007/s00381-024-06473-x -
Neuro-oncology Jun 2024The incidence of leptomeningeal metastases (LM) has been reported diversely. This study aimed to investigate the incidence, risk factors, and prognosis of LM in patients...
BACKGROUND
The incidence of leptomeningeal metastases (LM) has been reported diversely. This study aimed to investigate the incidence, risk factors, and prognosis of LM in patients with IDH-wildtype glioblastoma.
METHODS
A total of 828 patients with IDH-wildtype glioblastoma were enrolled between 2005 and 2022. Baseline preoperative MRI including post-contrast fluid-attenuated inversion recovery (FLAIR) was used for LM diagnosis. Qualitative and quantitative features, including distance between tumor and subventricular zone (SVZ) and tumor volume by automatic segmentation of the lateral ventricles and tumor, were assessed. Logistic analysis of LM development was performed using clinical, molecular, and imaging data. Survival analysis was performed.
RESULTS
The incidence of LM was 11.4%. MGMTp unmethylation (odds ratio [OR] = 1.92, P = 0.014), shorter distance between tumor and SVZ (OR = 0.94, P = 0.010), and larger contrast-enhancing tumor volume (OR = 1.02, P < 0.001) were significantly associated with LM. The overall survival (OS) was significantly shorter in patients with LM than in those without (log-rank test; P < 0.001), with median OS of 12.2 and 18.5 months, respectively. Presence of LM remained an independent prognostic factor for OS in IDH-wildtype glioblastoma (hazard ratio = 1.42, P = 0.011), along with other clinical, molecular, imaging, and surgical prognostic factors.
CONCLUSION
The incidence of LM is high in patients with IDH-wildtype glioblastoma, and aggressive molecular and imaging factors are correlated with LM development. The prognostic significance of LM based on post-contrast FLAIR imaging suggests acknowledgement of post-contrast FLAIR as a reliable diagnostic tool for clinicians.
PubMed: 38822538
DOI: 10.1093/neuonc/noae091 -
World Neurosurgery May 2024To summarize the preliminary application experience of intraoperative ultrasound with burr hole probe in minimally invasive neurosurgery and to explore its application...
OBJECTIVE
To summarize the preliminary application experience of intraoperative ultrasound with burr hole probe in minimally invasive neurosurgery and to explore its application value.
METHODS
29 patients who underwent intraoperative ultrasound guided puncture with burr hole probe in our center from August 2018 to November 2023 were collected, including 15 cases of ventriculoperitoneal shunt operation, 5 cases of assisted stereotactic needle biopsy, 3 cases of intracranial pressure probe implantation in lateral ventricle, 3 cases of brain abscess puncture for external drainage, and 3 cases of intracranial cyst puncture and peritoneal drainage. During the procedures, the burr hole probe was used to locate the intracranial targets and guide the puncture. The postoperative CT scans or combined postoperative pathological results could verify the accuracy of puncture. In addition, the intervention effect and recovery status of patients were also recorded.
RESULTS
The intraoperative ultrasound with burr hole probe could clearly display all the purposed targets and accurately guide the puncture procedures in all cases. All patients achieved satisfactory diagnostic and therapeutic results without new neurological dysfunction and serious complications.
CONCLUSIONS
The intraoperative ultrasound with burr hole probe is an effective device for demonstrating intracranial structures. It not only enables minimally invasive and precise diagnosis or treatment of many neurosurgical diseases, but also is simple and safe to operate, which has important promotional value in the neurosurgery.
PubMed: 38821403
DOI: 10.1016/j.wneu.2024.05.146 -
Japanese Journal of Radiology May 2024To investigate glymphatic system function in patients with brain tumors, including both primary and secondary tumors, using diffusion tensor imaging along perivascular...
PURPOSE
To investigate glymphatic system function in patients with brain tumors, including both primary and secondary tumors, using diffusion tensor imaging along perivascular spaces (DTI-ALPS).
METHODS
We retrospectively analyzed the MR DTI of 24 patients with unilateral brain tumors and compared them with age and sex-matched controls. We compared the DTI-ALPS index of the ipsi- and contralateral brain hemispheres. The region of interest was placed in the periventricular vessels adjacent to the lateral ventricles. Differences between sex, age, and kind of tumor (primary or brain metastasis) were evaluated. Correlations between DTI-ALPS index and age and the tumor's apparent diffusion coefficient (ADC) were also investigated.
RESULTS
The DTI-ALPS index was significantly lower (p < 0.05) in the tumor-affected hemisphere (mean = 1.26 ± 0.24) than contralateral (mean = 1.43 ± 0.28). A comparison with healthy controls revealed no significant difference on the matched ipsilateral side. However, the DTI-ALPS index of the contralateral side of the patients was larger than the HC. Additionally, no statistically significant differences were found when analyzing the DTI-ALPS index vs. age, sex, and tumor entity. Additionally, we did not find a correlation between the DTI-ALPS index and patient age or tumor ADC.
CONCLUSION
The decreased DTI-ALPS index in the tumor-affected hemisphere may be related to impaired glymphatic system function. However, cancer is often a systemic disease; thus, the DTI-ALPS index from the contralateral brain hemisphere may not generally be considered as a normal control. Nonetheless, the DTI-ALPS index does not only reflect diffusion in the perivascular spaces but it can also be influenced by factors such as axonal degeneration. Therefore, it does not directly reflect brain waste clearance and changes in the index should be interpreted carefully.
PubMed: 38819694
DOI: 10.1007/s11604-024-01602-7 -
Molecular Neurobiology May 2024The neuroscience community has largely accepted the notion that functional neurons can be generated from neural stem cells in the adult brain, especially in two brain... (Review)
Review
The neuroscience community has largely accepted the notion that functional neurons can be generated from neural stem cells in the adult brain, especially in two brain regions: the subventricular zone of the lateral ventricles and the subgranular zone in the dentate gyrus of the hippocampus. However, impaired neurogenesis has been observed in some neurodegenerative diseases, particularly in Alzheimer's, Parkinson's, and Huntington's diseases, and also in Lewy Body dementia. Therefore, restoration of neurogenic function in neurodegenerative diseases emerges as a potential therapeutic strategy to counteract, or at least delay, disease progression. Considering this, the present study summarizes the different neuronal niches, provides a collection of the therapeutic potential of different pro-neurogenic strategies in pre-clinical and clinical research, providing details about their possible modes of action, to guide future research and clinical practice.
PubMed: 38816676
DOI: 10.1007/s12035-024-04246-w -
Rinsho Shinkeigaku = Clinical Neurology May 2024A 59-year-old man had developed visual abnormality, nausea, headache, and weight loss since three months before. The ophthalmologist found severe optic disc edema in...
A 59-year-old man had developed visual abnormality, nausea, headache, and weight loss since three months before. The ophthalmologist found severe optic disc edema in both eyes, and referred him to our hospital. The patient had mild cerebellar ataxia. Increased cerebrospinal fluid pressure, increased protein and cell counts, positive oligoclonal band, and contrast-enhanced head MRI showed multiple linear perivascular radial gadolinium enhancement around bilateral lateral ventricles. His subjective and objective findings significantly improved with steroid treatment. The cerebrospinal fluid was found to be positive for glial fibrillary acidic protein (GFAP) antibodies, and a diagnosis of GFAP astrocytopathy was obtained. When optic edema or radial contrast effects was observed on contrast-enhanced MRI, GFAP astrocytopathy should be considerd. Prompt immunotherapy is required to circumvent the development of permanent visual impairment.
PubMed: 38811204
DOI: 10.5692/clinicalneurol.cn-001949 -
Scientific Reports May 2024Previous investigations have highlighted notable variations in cardiovascular risk indicators associated with various professional categories. However, only a few...
Previous investigations have highlighted notable variations in cardiovascular risk indicators associated with various professional categories. However, only a few studies have examined structural and functional cardiac parameters using echocardiography within distinct occupational groups. Hence, this study endeavored to assess cardiac structural and functional parameters in three additional occupations: firefighters (FFs), police officers (POs), and office workers (OWs). This prospective study encompassed 197 male participants (97 FFs, 54 POs, and 46 OWs) from Germany. All participants underwent 2D and Doppler echocardiography in resting conditions; standard parasternal and apical axis views were employed to evaluate structural (diastolic and systolic) and functional (systolic and diastolic function, and strain) cardiac parameters. All three occupational groups exhibited a tendency towards septal hypertrophy. Notably, OWs exhibited the largest diastolic interventricular septum diameter (IVSd), at 1.33 ± 0.25 cm. IVSd significantly varied between POs and OWs (p = 0.000) and between POs and FFs (p = 0.025). Additionally, during diastole a substantially larger left ventricular posterior wall diameter (LVPWd) was observed in OWs compared to FFs (p = 0.001) and POs (p = 0.013). The left ventricular diastolic cavity diameter (LVIDd) and the left ventricular systolic cavity diameter (LVIDs) were significantly higher in POs than they were in FFs (LVIDd: p = 0.001; LVIDs: p = 0.009), and the LVIDd was notably higher in FFs (p = 0.015) and POs compared to OWs (p = 0.000). FFs exhibited significantly better diastolic function, indicated by higher diastolic peak velocity ratios (MV E/A ratio) and E/E' ratios, compared to POs (E/A ratio: p = 0.025; E/E' ratio: p = 0.014). No significant difference in diastolic performance was found between OWs and FFs. Significantly higher E'(lateral) values were noted in POs compared to FFs (p = 0.003) and OWs (p = 0.004). Ejection fraction did not significantly differ among FFs, POs, and OWs (p > 0.6). The left ventricular mass (LV Mass) was notably higher in POs than it was in FFs (p = 0.039) and OWs (p = 0.033). Strain parameter differences were notably improved in two- (p = 0.006) and four-chamber (p = 0.018) views for FFs compared to POs. Concentric remodeling was the predominant change observed in all three occupational groups. Significant differences in the presence of various forms of hypertrophy were observed in FFs, POs, and OWs (exact Fisher test p-values: FFs vs. OWs = 0.021, POs vs. OWs = 0.002). OWs demonstrated notably higher rates of concentric remodeling than FFs did (71.77% vs. 47.9%). This study underscores disparities in both functional and structural parameters in diverse occupational groups. Larger prospective studies are warranted to investigate and delineate differences in structural and functional cardiac parameters across occupational groups, and to discern their associated effects and risks on the cardiovascular health of these distinct professional cohorts.
Topics: Humans; Male; Cross-Sectional Studies; Adult; Middle Aged; Prospective Studies; Echocardiography; Echocardiography, Doppler; Firefighters; Heart Ventricles; Germany; Occupations; Diastole; Police; Heart; Workplace; Ventricular Function, Left; Working Conditions
PubMed: 38802474
DOI: 10.1038/s41598-024-62190-0 -
Experimental Neurology May 2024Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis results in chronic epilepsy and permanent cognitive impairment. One of the possible causes of cognitive...
OBJECTIVE
Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis results in chronic epilepsy and permanent cognitive impairment. One of the possible causes of cognitive impairment in anti-NMDAR could be aberrant neurogenesis, an established contributor to memory loss in idiopathic drug-resistant epilepsy. We developed a mouse model of anti-NMDAR encephalitis and showed that mice exposed to patient anti-NMDAR antibodies for 2 weeks developed seizures and memory loss. In the present study, we assessed the delayed effects of patient-derived antibodies on cognitive phenotype and examined the corresponding changes in hippocampal neurogenesis.
METHODS
Monoclonal anti-NMDAR antibodies or control antibodies were continuously infused into the lateral ventricle of male C56BL/6 J mice (8-12 weeks) via osmotic minipumps for 2 weeks. The motor and anxiety phenotypes were assessed using the open field paradigm, and hippocampal memory and learning were assessed using the object location, Y maze, and Barnes maze paradigms during weeks 1 and 3-4 of antibody washout. The numbers of newly matured granule neurons (Prox-1+) and immature progenitor cells (DCX+) as well as their spatial distribution within the hippocampus were assessed at these time points. Bromodeoxyuridine (BrdU: 50 mg/kg, i.p., daily) was injected on days 2-12 of the infusion, and proliferating cell immunoreactivity was compared in antibody-treated mice and control mice at week 3 of the washout.
RESULTS
Mice infused with anti-NMDAR antibodies demonstrated spatial memory impairment during week 1 of antibody washout (p = 0.02, t-test; n = 9-11). Histological analysis of hippocampal sections from these mice revealed an increased ectopic displacement of Prox-1+ cells in the dentate hilus compared to the control-antibody-treated mice (p = 0.01; t-test). Mice exposed to anti-NMDAR antibodies also had an impairment of spatial memory and learning during weeks 3-4 of antibody washout (object location: p = 0.009; t-test; Y maze: p = 0.006, t-test; Barnes maze: p = 0.008, ANOVA; n = 8-10). These mice showed increased ratios of the low proliferating (bright) to fast proliferating (faint) BrdU+ cell counts and decreased number of DCX+ cells in the hippocampal dentate gyrus (p = 0.006 and p = 0.04, respectively; t-tests) suggesting ectopic migration and delayed cell proliferation.
SIGNIFICANCE
These findings suggest that memory and learning impairments induced by patient anti-NMDAR antibodies are sustained upon removal of antibodies and are accompanied by aberrant hippocampal neurogenesis. Interventions directed at the manipulation of neuronal plasticity in patients with encephalitis and cognitive loss may be protective and therapeutically relevant.
PubMed: 38801989
DOI: 10.1016/j.expneurol.2024.114838 -
International Medical Case Reports... 2024Giant Clinically Non-Functioning Pituitary Adenomas (GCNFPA) are pituitary neuroendocrine tumours spanning beyond 4 cm in diameter without clinically apparent secretory...
BACKGROUND
Giant Clinically Non-Functioning Pituitary Adenomas (GCNFPA) are pituitary neuroendocrine tumours spanning beyond 4 cm in diameter without clinically apparent secretory function. They elicit insidious growth pertaining to its asymptomatic nature and present at large sizes from mass effect. Certain clinical features such as headache and visual disturbances are common presentations of GCNFPAs owing to their size, while others such as seizures are extremely rare.
CASE REPORT
A 63-year-old woman presented with back-to-back generalized tonic clonic seizures to the A&E and was treated with Levetiracetam. Following initial normal blood investigations, a visual field analysis revealed a bitemporal upper quadrantanopia and further evaluation using MRI found a giant pituitary lesion with suprasellar extension through the third ventricle into the lateral ventricles with significant mass effect. She underwent neuro-navigation guided endonasal transsphenoidal subtotal resection 2 weeks later. The histological diagnosis of null cell adenoma with a Ki67 of 3% was made. At 3 months of follow-up, she is symptom free and monitored with serial MRIs.
CONCLUSION
Seizures are an uncommon presentation of GCNFPAs limited to a few case reports. This illustrates the importance of careful evaluation of patient presentations to correctly diagnose pituitary neoplasms and prioritizing symptom relief in choosing surgical approaches.
PubMed: 38799383
DOI: 10.2147/IMCRJ.S465564 -
MedRxiv : the Preprint Server For... May 2024This study sought to identify magnetoencephalography (MEG) power spectra patterns associated with cerebrovascular damage (white matter hyperintensities - WMH) and their...
OBJECTIVE
This study sought to identify magnetoencephalography (MEG) power spectra patterns associated with cerebrovascular damage (white matter hyperintensities - WMH) and their relationship with cognitive performance and brain structure integrity in aging individuals without cognitive impairment.
METHODS
We hypothesized a "slowness" pattern characterized by increased power in δ and θ bands and decreased power in the β band associated with the severity of vascular damage. MEG signals were analyzed in cognitively healthy older adults to investigate these associations.
RESULTS
Contrary to expectations, we did not observe an increase in δ and θ power. However, we found a significant negative correlation between β band power and WMH volume. This β power reduction was linked to structural brain changes, such as larger lateral ventricles, reduced white matter volume, and decreased fractional anisotropy in critical white matter tracts, but not to cognitive performance. This suggests that β band power reduction may serve as an early marker of vascular damage before the onset of cognitive symptoms.
CONCLUSION
Our findings partially confirm our initial hypothesis by demonstrating a decrease in β band power with increased vascular damage but not the anticipated increase in slow band power. The lack of correlation between the βpow marker and cognitive performance suggests its potential utility in early identification of at-risk individuals for future cognitive impairment due to vascular origins. These results contribute to understanding the electrophysiological signatures of preclinical vascular damage and highlight the importance of MEG in detecting subtle brain changes associated with aging.
PubMed: 38798609
DOI: 10.1101/2024.05.15.24307438