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Acta Medica Portuguesa Feb 1994Central neurocytomas are a well-characterized group of neuronal tumors usually arising in the supratentorial ventricular cavities. We describe the anatomical and... (Review)
Review
Central neurocytomas are a well-characterized group of neuronal tumors usually arising in the supratentorial ventricular cavities. We describe the anatomical and clinical study of seven of these neoplasms. Patients were in the first four decades of life, and tumors were usually located in one of the lateral ventricles. Surgical resection was subtotal in 6 cases. Light microscopic examination revealed predominantly benign lesions with morphological features similar to the so-called ependymoma of the foramen of Monro, of the oligodendrogliomas, and of the neuroblastomas. Immunohistochemistry and electron microscopy confirmed the diagnosis in each case, showing expression of neuron specific enolase and synaptophysin and containing microtubuli, neurosecretory granules, and presynaptic vesicles. In agreement with the literature, the authors stress the benign behaviour of most of these tumors and the need for systematic immunohistochemical and ultrastructural study. The actual roles of the extent of resection and of postoperative radiotherapy are still a matter of discussion.
Topics: Adolescent; Adult; Brain; Cerebral Ventricle Neoplasms; Child; Female; Humans; Immunohistochemistry; Male; Neurocytoma; Radiography
PubMed: 8178691
DOI: No ID Found -
Neuroradiology Oct 2022Grey matter (GM) atrophy due to neuronal loss is a striking feature of patients with CLN3 disease. A precise and quantitative description of disease progression is...
PURPOSE
Grey matter (GM) atrophy due to neuronal loss is a striking feature of patients with CLN3 disease. A precise and quantitative description of disease progression is needed in order to establish an evaluation tool for current and future experimental treatments. In order to develop a quantitative marker to measure brain volume outcome, we analysed the longitudinal volumetric development of GM, white matter (WM) and lateral ventricles and correlated those with the clinical course.
METHODS
One hundred twenty-two MRI scans of 35 patients (21 females; 14 males; age 15.3 ± 4.8 years) with genetically confirmed CLN3 disease were performed. A three-dimensional T1-weighted sequence was acquired with whole brain coverage. Volumetric segmentation of the brain was performed with the FreeSurfer image analysis suite. The clinical severity was assessed by the Hamburg jNCL score, a disease-specific scoring system.
RESULTS
The volumes of supratentorial cortical GM and supratentorial WM, cerebellar GM, basal ganglia/thalamus and hippocampus significantly (r = - 0.86 to - 0.69, p < 0.0001) decreased with age, while the lateral ventricle volume increased (r = 0.68, p < 0.0001). Supratentorial WM volume correlated poorer with age (r = - 0.56, p = 0.0001). Supratentorial cortical GM volume showed the steepest (4.6% (± 0.2%)) and most uniform decrease with strongest correlation with age (r = - 0.86, p < 0.0001). In addition, a strong correlation with disease specific clinical scoring existed for the supratentorial cortical GM volume (r = 0.85, p = < 0.0001).
CONCLUSION
Supratentorial cortical GM volume is a sensitive parameter for assessment of disease progression even in early and late disease stages and represents a potential reliable outcome measure for evaluation of experimental therapies.
Topics: Adolescent; Atrophy; Biomarkers; Brain; Child; Disease Progression; Female; Gray Matter; Humans; Magnetic Resonance Imaging; Male; Membrane Glycoproteins; Molecular Chaperones; Neuronal Ceroid-Lipofuscinoses; Young Adult
PubMed: 35699772
DOI: 10.1007/s00234-022-02988-9 -
PloS One 2017Arterial spin-labeling (ASL) was recently introduced as a noninvasive method to evaluate cerebral hemodynamics. The purposes of this study were to assess the ability of...
BACKGROUND AND PURPOSE
Arterial spin-labeling (ASL) was recently introduced as a noninvasive method to evaluate cerebral hemodynamics. The purposes of this study were to assess the ability of ASL imaging to detect crossed cerebellar diaschisis (CCD) in patients with their first unilateral supratentorial hyperacute stroke and to identify imaging or clinical factors significantly associated with CCD.
MATERIALS AND METHODS
We reviewed 204 consecutive patients who underwent MRI less than 8 hours after the onset of stroke symptoms. The inclusion criteria were supratentorial abnormality in diffusion-weighted images in the absence of a cerebellar or brain stem lesion, bilateral supratentorial infarction, subacute or chronic infarction, and MR angiography showing vertebrobasilar system disease. For qualitative analysis, asymmetric cerebellar hypoperfusion in ASL images was categorized into 3 grades. Quantitative analysis was performed to calculate the asymmetric index (AI). The patients' demographic and clinical features and outcomes were recorded. Univariate and multivariate analyses were also performed.
RESULTS
A total of 32 patients met the inclusion criteria, and 24 (75%) presented CCD. Univariate analyses revealed more frequent arterial occlusions, higher diffusion-weighted imaging (DWI) lesion volumes and higher initial NIHSS and mRS scores in the CCD-positive group compared with the CCD-negative group (all p < .05). The presence of arterial occlusion and the initial mRS scores were related with the AI (all p < .05). Multivariate analyses revealed that arterial occlusion and the initial mRS scores were significantly associated with CCD and AI.
CONCLUSION
ASL imaging could detect CCD in 75% of patients with hyperacute infarction. We found that CCD was more prevalent in patients with arterial occlusion, larger ischemic brain volumes, and higher initial NIHSS and mRS scores. In particular, vessel occlusion and initial mRS score appeared to be significantly related with CCD pathophysiology in the hyperacute stage.
Topics: Acute Disease; Aged; Brain Ischemia; Cerebellum; Cerebrovascular Circulation; Diffusion Magnetic Resonance Imaging; Female; Humans; Magnetic Resonance Angiography; Male; Middle Aged; Observer Variation; Spin Labels; Stroke
PubMed: 28323841
DOI: 10.1371/journal.pone.0173971 -
Developmental Medicine and Child... Mar 2019Neurodevelopmental outcomes in children with congenital cerebellar malformations (CCMs) remain poorly defined. We aimed to assess whether specific neuroimaging features...
AIM
Neurodevelopmental outcomes in children with congenital cerebellar malformations (CCMs) remain poorly defined. We aimed to assess whether specific neuroimaging features in CCM patients correlate with neurodevelopmental outcomes.
METHOD
Hospital records and neuroimaging of 67 children with CCMs were systematically reviewed. Logistic regression analyses were used to assess associations between specific imaging features and neurodevelopmental outcomes.
RESULTS
CCM categories were distributed as follows: 28 percent isolated vermis hypoplasia (n=19), 28 percent global cerebellar hypoplasia (n=19), 15 percent Dandy-Walker malformation (n=10), 13 percent pontocerebellar hypoplasia (PCH, n=9), 9 percent molar tooth malformation (n=6), 3 percent rhombencephalosynapsis (n=2), and 3 percent unilateral cerebellar malformation (n=2). Overall, 85 percent (55/65) of the cohort had global developmental delay (GDD). Intellectual disability was present in 61 percent (27/43) and autism spectrum disorder (ASD) in 12 percent (6/52). Adjusting for supratentorial malformations and presence of genetic findings, severe GDD was associated with cerebellar hypoplasia (p=0.049) and PCH (p=0.030), whereas children with vermis hypoplasia were less likely to have severe GDD (p=0.003). Presence of supratentorial abnormalities was not significantly associated with worse neurodevelopmental outcome but was associated with epilepsy.
INTERPRETATION
Children with CCMs have high prevalence of neurodevelopmental deficits. Specific features on imaging can aid prognostication and establish early intervention strategies.
WHAT THIS PAPER ADDS
Atypical long-term neurodevelopmental outcome is very common in patients with congenital cerebellar malformations (CCMs). Involvement of the brainstem and cerebellar hemispheres predicts more severe neurodevelopmental disability. Most patients with vermis hypoplasia have language delay but are verbal. Supratentorial abnormalities are not significantly associated with worse neurodevelopmental outcome but are associated with epilepsy. Comorbidities are common in CCMs, especially ophthalmological issues in cerebellar hypoplasia and sensorineural hearing loss in pontocerebellar hypoplasia.
Topics: Adolescent; Cerebellum; Child; Child, Preschool; Cohort Studies; Female; Humans; Logistic Models; Magnetic Resonance Imaging; Male; Neurodevelopmental Disorders; Neuroimaging; Prevalence
PubMed: 30320441
DOI: 10.1111/dmcn.14059 -
Developmental Medicine and Child... Oct 2019To improve the genetic, clinical, and neuroradiological characterization of cerebellar involvement in tuberous sclerosis complex (TSC) and determine whether cerebellar...
AIM
To improve the genetic, clinical, and neuroradiological characterization of cerebellar involvement in tuberous sclerosis complex (TSC) and determine whether cerebellar lesions could be a reliable biomarker of neurological impairment.
METHOD
This retrospective cohort study, held at two tertiary paediatric university centres, was conducted on patients with a confirmed diagnosis of TSC who underwent brain magnetic resonance imaging between October 2009 and May 2016. The study population consisted of 112 patients with TSC (median age 10y; range 5mo-38y; 61 females, 51 males).
RESULTS
The results from multivariable statistical analysis indicated that cerebellar involvement (34 out of 112 patients, none carrying a TSC1 mutation) was the most powerful predictor of supratentorial cortical tuber load; however, cerebellar involvement was not the best predictor of clinical phenotype when supratentorial tuber load and TSC2 mutations were taken into consideration. The association between cerebellar lesions and a more severe clinical and neuroradiological phenotype was statistically significant and may be due to its strong association with TSC2 mutations and higher cortical tuber load.
INTERPRETATION
Cerebellar involvement is not the best predictor of neurobehavioural outcome, including TSC-related autism, after adjusting for TSC2 and the number of cortical tubers. Its role in the TSC clinical phenotype needs to be investigated further.
WHAT THIS PAPER ADDS
Cerebellar involvement is a powerful predictor of supratentorial cortical involvement and a potential biomarker of disease severity. Cerebellar lesions significantly correlate with a more severe clinical and neuroradiological phenotype. Cerebellar involvement is not the best predictor of neurobehavioural outcome.
Topics: Adolescent; Cerebellum; Child; Child, Preschool; Female; Humans; Male; Mutation; Phenotype; Retrospective Studies; Tuberous Sclerosis; Tuberous Sclerosis Complex 2 Protein
PubMed: 31313298
DOI: 10.1111/dmcn.14313 -
Human Brain Mapping Nov 2023Brain imaging has significantly contributed to our understanding of the cerebellum being involved in recovery after non-cerebellar stroke. Due to its connections with...
Brain imaging has significantly contributed to our understanding of the cerebellum being involved in recovery after non-cerebellar stroke. Due to its connections with supratentorial brain networks, acute stroke can alter the function and structure of the contralesional cerebellum, known as crossed cerebellar diaschisis (CCD). Data on the spatially precise distribution of structural CCD and their implications for persistent deficits after stroke are notably limited. In this cross-sectional study, structural MRI and clinical data were analyzed from 32 chronic stroke patients, at least 6 months after the event. We quantified lobule-specific contralesional atrophy, as a surrogate of structural CCD, in patients and healthy controls. Volumetric data were integrated with clinical scores of disability and motor deficits. Diaschisis-outcome models were adjusted for the covariables age, lesion volume, and damage to the corticospinal tract. We found that structural CCD was evident for the whole cerebellum, and particularly for lobules V and VI. Lobule VI diaschisis was significantly correlated with clinical scores, that is, volume reductions in contralesional lobule VI were associated with higher levels of disability and motor deficits. Lobule V and the whole cerebellum did not show similar diaschisis-outcome relationships across the spectrum of the clinical scores. These results provide novel insights into stroke-related cerebellar plasticity and might thereby promote lobule VI as a key area prone to structural CCD and potentially involved in recovery and residual motor functioning.
Topics: Humans; Cross-Sectional Studies; Diaschisis; Stroke; Cerebellum; Magnetic Resonance Imaging; Brain Damage, Chronic; Cerebrovascular Circulation
PubMed: 37471691
DOI: 10.1002/hbm.26434 -
NeuroImage. Clinical 2022Patients with infratentorial stroke (IS) exhibit more severe dysphagia and a higher risk of aspiration than patients with supratentorial stroke. Nevertheless, a large...
Patients with infratentorial stroke (IS) exhibit more severe dysphagia and a higher risk of aspiration than patients with supratentorial stroke. Nevertheless, a large proportion of patients with IS regain swallowing function within 6 months; however, the neural mechanism for this recovery remains unclear. We aimed to investigate possible neuroplastic changes involved using functional magnetic resonance imaging (fMRI) and their relation to swallowing function. We assessed 21 patients with IS (mean age: 59.9 ± 11.1 years) exhibiting dysphagia in the subacute phase and 21 healthy controls (mean age: 57.1 ± 7.8 years). Patient evaluations were based on the functional oral intake scale (FOIS), videofluoroscopic swallow study (VFSS), and fMRI. Temporal swallowing measures and the penetration-aspiration scale (PAS) were obtained using VFSS. Whole-brain-medulla resting-state functional connectivity (rsFC) was calculated and compared between patients and healthy controls. The rsFCs were also correlated with functional measures within the patient group. In patients with IS, whole-brain-medulla rsFCs were significantly higher in the precuneus, the left and right precentral gyrus, and the right supplementary motor area compared to those in healthy controls (P < 0.001, family-wise error-corrected cluster-level P < 0.05). The rsFCs to the medulla for the left (r = -0.507, P = 0.027) and right side (r = -0.503, P = 0.028) precentral gyrus were negatively correlated with the PAS. The rsFC between the left (r = 0.470, P = 0.042) and right (r = 0.459, P = 0.048) precentral gyrus to the medulla was positively correlated with upper esophageal sphincter opening durations (UOD). In addition, PAS was also correlated with UOD (r = -0.638, P = 0.003) whereas the laryngeal closure duration was correlated with the hyoid bone movement duration (r = 0.550, P = 0.015). Patients with IS exhibited overall modulation of cortical-medulla connectivity during the subacute phase. Patients with higher connectivities showed better swallowing performance. These findings support that there is cortical involvement in swallowing regulation after IS and can aid in determining potential treatment targets for dysphagia.
Topics: Aged; Brain; Deglutition; Deglutition Disorders; Humans; Hyoid Bone; Middle Aged; Stroke
PubMed: 35792418
DOI: 10.1016/j.nicl.2022.103104 -
Journal of Neuro-oncology Jan 2024To provide a treatment-focused review and develop basic treatment guidelines for patients diagnosed with pineal anlage tumor (PAT).
PURPOSE
To provide a treatment-focused review and develop basic treatment guidelines for patients diagnosed with pineal anlage tumor (PAT).
METHODS
Prospectively collected data of three patients with pineal anlage tumor from Germany was combined with clinical details and treatment information from 17 published cases.
RESULTS
Overall, 20 cases of PAT were identified (3 not previously reported German cases, 17 cases from published reports). Age at diagnosis ranged from 0.3 to 35.0 (median: 3.2 ± 7.8) years. All but three cases were diagnosed before the age of three years. For three cases, metastatic disease at initial staging was described. All patients underwent tumor surgery (gross-total resection: 9, subtotal resection/biopsy: 9, extent of resection unknown: 2). 15/20 patients were alive at last follow-up. Median follow-up for 10/15 surviving patients with available follow-up and treatment data was 2.4 years (0.3-6.5). Relapse was reported for 3 patients within 0.8 years after diagnosis. Five patients died, 3 after relapse and 2 from early postoperative complications. Two-year-progression-free- and -overall survival were 65.2 ± 12.7% and 49.2 ± 18.2%, respectively. All 4 patients who received intensive chemotherapy including high-dose chemotherapy combined with radiotherapy (2 focal, 2 craniospinal [CSI]) had no recurrence. Focal radiotherapy- and CSI-free survival rates in 13 evaluable patients were 46.2% (6/13) and 61.5% (8/13), respectively.
CONCLUSION
PAT is an aggressive disease mostly affecting young children. Therefore, adjuvant therapy using intensive chemotherapy and considering radiotherapy appears to comprise an appropriate treatment strategy. Reporting further cases is crucial to evaluate distinct treatment strategies.
Topics: Adolescent; Adult; Child; Child, Preschool; Humans; Infant; Young Adult; Brain Neoplasms; Neoplasm Recurrence, Local; Pineal Gland; Pinealoma; Recurrence; Supratentorial Neoplasms; Treatment Outcome
PubMed: 38253790
DOI: 10.1007/s11060-023-04547-5 -
Journal of Radiology Case Reports May 2014Delayed leukoencephalopathy is an uncommon complication of hypoxic-ischemic events of different etiologies, including carbon monoxide intoxication. We present a case of... (Review)
Review
Delayed leukoencephalopathy is an uncommon complication of hypoxic-ischemic events of different etiologies, including carbon monoxide intoxication. We present a case of a 40-year-old male patient who was admitted with rapidly progressive neurocognitive and behavioral deficits. There was a history of accidental carbon monoxide intoxication one month before, presenting with loss of consciousness and short hospitalization, followed by a complete clinical recovery. The imaging studies in the delayed phase depicted confluent, symmetric supra-tentorial white matter lesions in keeping with diffuse demyelinization. Restricted diffusion and metabolite abnormalities in magnetic resonance proton spectroscopy were also seen. The diagnosis of CO-mediated delayed post-hypoxic leukoencephalopathy was assumed after exclusion of other mimickers. Hyperbaric oxygen therapy was tentatively performed and the patient had a favorable clinical and radiological evolution.
Topics: Acute Disease; Adult; Brain; Carbon Monoxide Poisoning; Cognition Disorders; Demyelinating Diseases; Humans; Hyperbaric Oxygenation; Leukoencephalopathies; Male; Mental Disorders; Neuroimaging; Proton Magnetic Resonance Spectroscopy; Time Factors; Treatment Outcome
PubMed: 25426224
DOI: 10.3941/jrcr.v8i5.1721 -
Scientific Reports Oct 2017Aiming to investigate whether handedness-related language lateralization is related to the intrinsic resting-state functional connectivity (RSFC) pattern within the...
Aiming to investigate whether handedness-related language lateralization is related to the intrinsic resting-state functional connectivity (RSFC) pattern within the language network, the present study integrated the information of functional activations during a semantic task of Chinese characters and FC in resting-state based on functional magnetic resonance imaging (fMRI) data of healthy left handers (LH) and right handers (RH). RSFC was calculated on a voxel-based level between the seed regions chosen from functional activations during the task and the rest of the brain. The results demonstrated that LH had significantly stronger RSFC than RH between the cerebellum and supratentorial areas of the frontal, parietal and temporal lobe, and between the occipital lobe and frontal/parietal lobe. Correlation analysis showed that RSFC values between right MFG and left cerebellum_crus2, between SMA and right cerebellum_crus2, and between the right cerebellum_crus1 and left MFG were negatively correlated with cerebral laterality index in LH and RH groups. Our results highlight key nodes of Chinese language brain network processing in the cerebellum, and suggest that atypical language dominance relates to stronger crossed reciprocal RSFC in the frontal-cerebellar system. The findings provide new insights into the intrinsic FC substrates underlying the atypical language lateralization of LH.
Topics: Adult; Brain Mapping; Cerebellum; China; Female; Functional Laterality; Humans; Language; Magnetic Resonance Imaging; Male; Rest; Semantics; Young Adult
PubMed: 29070895
DOI: 10.1038/s41598-017-14600-9