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Minerva Anestesiologica 2008The awake craniotomy technique was originally introduced for the surgical treatment of epilepsy and has subsequently been used in patients undergoing surgical management... (Review)
Review
UNLABELLED
The awake craniotomy technique was originally introduced for the surgical treatment of epilepsy and has subsequently been used in patients undergoing surgical management of supratentorial tumors, arteriovenous malformation, deep brain stimulation, and mycotic aneurysms near critical brain regions. This surgical approach aims to maximize lesion resection while sparing important areas of the brain (motor, somatosensory, and language areas). Awake craniotomy offers great advantages with respect to patient outcome. In this type of procedure, the anesthetist's goal is to make the operation safe and effective and reduce the psychophysical distress of the patient. Many authors have described different anesthetic care protocols for awake craniotomy based on monitored or general anesthesia; however, there is still no consensus as to the best anesthetic technique. The most commonly used drugs for awake craniotomies are propofol and remifentanil, but dexmedetomidine is beginning to be used more commonly outside of Europe. Personal experience, careful planning, and attention to detail are the basis for obtaining good awake craniotomy
RESULTS
Additional studies are necessary in order to optimize the procedure, reduce complications, and improve patient tolerance. The aim of this review is to present a thorough report of the literature, with particular attention to neuro-oncology surgery.
Topics: Anesthesia; Awareness; Brain; Craniotomy; Forecasting; Humans
PubMed: 18612268
DOI: No ID Found -
Brain Pathology (Zurich, Switzerland) Sep 2020Advances in our understanding of the biological basis and molecular characteristics of ependymal tumors since the latest iteration of the World Health Organization (WHO)... (Review)
Review
Advances in our understanding of the biological basis and molecular characteristics of ependymal tumors since the latest iteration of the World Health Organization (WHO) classification of CNS tumors (2016) have prompted the cIMPACT-NOW group to recommend a new classification. Separation of ependymal tumors by anatomic site is an important principle of the new classification and was prompted by methylome profiling data to indicate that molecular groups of ependymal tumors in the posterior fossa and supratentorial and spinal compartments are distinct. Common recurrent genetic or epigenetic alterations found in tumors belonging to the main molecular groups have been used to define tumor types at intracranial sites; C11orf95 and YAP1 fusion genes for supratentorial tumors and two types of posterior fossa ependymoma defined by methylation group, PFA and PFB. A recently described type of aggressive spinal ependymoma with MYCN amplification has also been included. Myxopapillary ependymoma and subependymoma have been retained as histopathologically defined tumor types, but the classification has dropped the distinction between classic and anaplastic ependymoma. While the cIMPACT-NOW group considered that data to inform assignment of grade to molecularly defined ependymomas are insufficiently mature, it recommends assigning WHO grade 2 to myxopapillary ependymoma and allows grade 2 or grade 3 to be assigned to ependymomas not defined by molecular status.
Topics: Brain Neoplasms; Central Nervous System Neoplasms; Ependyma; Ependymoma; Glioma; Humans; Supratentorial Neoplasms
PubMed: 32502305
DOI: 10.1111/bpa.12866 -
Journal of Veterinary Internal Medicine Sep 2016Quantification of brain herniation on MRI and its immediate clinical implications are poorly described.
BACKGROUND
Quantification of brain herniation on MRI and its immediate clinical implications are poorly described.
OBJECTIVES
Define the normal position of caudal fossa structures on brain MRIs in dogs and cats utilizing morphometry, compare this to dogs and cats with caudal transtentorial herniation (CTH), foramen magnum herniation (FMH) or both identified on MRI, and investigate associations between herniation severity, clinical signs, and 24-hour outcome.
ANIMALS
Ninety-two controls (66 dogs, 26 cats), 119 cases with herniation (88 dogs, 31 cats).
METHODS
Retrospective case series. The MRI database was searched for controls with normal brain anatomy and cases with brain herniation. Morphometry in controls established TTX (transtentorial to rostroventral cerebellum) to quantify CTH and FMX (caudoventral cerebellum to foramen magnum) to quantify FMH. Measurements were compared between cases and controls. Correlations with specific clinical variables and outcome were investigated.
RESULTS
Measurements in medium/large control dogs versus small dog and cat controls were significantly different (P < .001, TTX: -0.46, -0.305, -0.3, FMX: 0.695, 0.27, 0.25, respectively). 119/1564 (7.6%) cases that underwent brain imaging had brain herniation. TTX and FMX were significantly different between controls and cases with CTH or FMH (P < .001). 67/89 (75%) cases with supratentorial lesions had no signs directly attributable to herniation. 71/119 (60%) had a normal anesthetic recovery. TTX was significantly associated with 24-hour survival (P < .001).
CONCLUSIONS AND CLINICAL IMPORTANCE
Brain herniation can be quantified on MRI. Clinical signs directly attributable to brain herniation commonly are absent, and more severe CTH based on TTX is associated with a worse short-term outcome.
Topics: Animals; Body Size; Brain; Cat Diseases; Cats; Dog Diseases; Dogs; Magnetic Resonance Imaging; Retrospective Studies
PubMed: 27616749
DOI: 10.1111/jvim.14526 -
AJNR. American Journal of Neuroradiology May 2019Moebius sequence comprises a spectrum of brain congenital malformations predominantly affecting the function of multiple cranial nerves. Reported neuroimaging findings... (Observational Study)
Observational Study
BACKGROUND AND PURPOSE
Moebius sequence comprises a spectrum of brain congenital malformations predominantly affecting the function of multiple cranial nerves. Reported neuroimaging findings are heterogeneous and based on case reports or small case series. Our goal was to describe the neuroimaging findings of Moebius sequence in a large population of patients scanned with MR imaging.
MATERIALS AND METHODS
An observational cross-sectional study was performed to assess brain MR imaging findings in 38 patients with Moebius syndrome studied between 2013 and 2016.
RESULTS
Retrospective analysis of MR imaging studies showed flattening of the floor of the fourth ventricle floor secondary to a bilateral absent facial colliculus in 38 patients (100%) and unilateral absence in 1. A hypoplastic pons was found in 23 patients (60.5%). Mesencephalic malformations consisted of tectal beaking in 15 patients (39.5%) and increased anteroposterior midbrain diameter with a shallow interpeduncular cistern in 12 (31.6%). Infratentorial arachnoid cysts were found in 5 patients (13.2%), and cerebellar vermis hypoplasia, in 2 (5.3%). Supratentorial findings included the following: thalamic fusion (26.3%), periventricular nodular heterotopias (26.3%), ventriculomegaly (26.3%), callosal abnormalities (23.7%), and hippocampal malrotations (23.7%).
CONCLUSIONS
Findings seen in this large patient cohort agreed with previously published reports. Flattening of the fourth ventricle floor secondary to a bilaterally absent facial colliculus was the most frequent MR imaging finding. The presence of other brain stem and cerebellar malformations as well as supratentorial abnormalities may help explain clinical symptoms and achieve a correct diagnosis.
Topics: Adolescent; Adult; Brain; Child; Child, Preschool; Cohort Studies; Cross-Sectional Studies; Female; Humans; Infant; Magnetic Resonance Imaging; Male; Mobius Syndrome; Neuroimaging; Retrospective Studies; Young Adult
PubMed: 30948378
DOI: 10.3174/ajnr.A6028 -
AJNR. American Journal of Neuroradiology Nov 2022According to the medical literature, it is known that intrauterine growth restriction is associated with abnormal fetal brain findings. The aim of this study was to...
BACKGROUND AND PURPOSE
According to the medical literature, it is known that intrauterine growth restriction is associated with abnormal fetal brain findings. The aim of this study was to assess the volume of fetal brain structures in fetuses with intrauterine growth restriction compared with the control group and to examine the effect of intrauterine growth restriction on birth weight in relation to the effect on the volumes of these structures.
MATERIALS AND METHODS
This historical cohort study included 26 fetuses diagnosed with intrauterine growth restriction due to placental insufficiency. The control group included 66 fetuses with MR imaging scans demonstrating normal brain structures. The volumes of the supratentorial brain, left and right hemispheres, and the cerebellum were measured using a semiautomatic method. In addition, the cerebellum and supratentorial brain ratio was calculated. The measurements of each brain structure were then converted to percentiles according to growth curves.
RESULTS
The absolute volumes and percentiles of all brain structures examined were smaller in the intrauterine growth restriction group. All examined brain structures showed results that were statistically significant ( < .015). There was no statistically significant difference in the cerebellum/supratentorial brain ratio ( > .39). The difference in brain volume percentiles was statistically smaller than the difference in birth weight and birth weight percentiles (Dolberg growth curves) between the groups.
CONCLUSIONS
Intrauterine growth restriction affects the volume of brain structures, as measured by quantitative MR imaging. Compared with healthy controls, the effect on birth weight was more prominent than the effect on brain structures, possibly due to the "brain-preserving" capability.
Topics: Humans; Female; Pregnancy; Fetal Growth Retardation; Birth Weight; Cohort Studies; Placenta; Fetus; Magnetic Resonance Imaging; Brain; Gestational Age; Ultrasonography, Prenatal
PubMed: 36202548
DOI: 10.3174/ajnr.A7665 -
AJNR. American Journal of Neuroradiology Oct 2020Neuronal ceroid lipofuscinoses are a group of neurodegenerative disorders characterized by the accumulation of autofluorescent lipopigments in neuronal cells. As a...
BACKGROUND AND PURPOSE
Neuronal ceroid lipofuscinoses are a group of neurodegenerative disorders characterized by the accumulation of autofluorescent lipopigments in neuronal cells. As a result of storage material in the brain and retina, clinical manifestations include speech delay, cognitive dysfunction, motor regression, epilepsy, vision loss, and early death. At present, 14 different ceroid lipofuscinosis (CLN) genes are known. Recently, the FDA approved the use of recombinant human proenzyme of tripeptidyl-peptidase 1 for CLN2 disease, while phase I/IIa clinical trials for gene therapy in CLN3 and CLN6 are ongoing. Early diagnosis is, therefore, key to initiating treatment and arresting disease progression. Neuroimaging features of CLN1, CLN2, CLN3, and CLN5 diseases are well-described, with sparse literature on other subtypes. We aimed to investigate and expand the MR imaging features of genetically proved neuronal ceroid lipofuscinoses subtypes at our institution and also to report the time interval between the age of disease onset and the diagnosis of neuronal ceroid lipofuscinoses.
MATERIALS AND METHODS
We investigated and analyzed the age of disease onset and neuroimaging findings (signal intensity in periventricular, deep, and subcortical white matter, thalami, basal ganglia, posterior limb of the internal capsule, insular/subinsular regions, and ventral pons; and the presence or absence of supratentorial and/or infratentorial atrophy) of patients with genetically proved neuronal ceroid lipofuscinoses at our institution. This group consisted of 24 patients who underwent 40 brain MR imaging investigations between 1993 and 2019, with a male preponderance (male/female ratio = 15:9).
RESULTS
The mean ages of disease onset, first brain MR imaging, and diagnosis of neuronal ceroid lipofuscinoses were 4.70 ± 3.48 years, 6.76 ± 4.49 years, and 7.27 ± 4.78 years, respectively. Findings on initial brain MR imaging included T2/FLAIR hypointensity in the thalami (= 22); T2/FLAIR hyperintensity in the periventricular and deep white matter ( = 22), posterior limb of the internal capsule ( = 22), ventral pons ( = 19), and insular/subinsular region ( = 18); supratentorial ( = 21) and infratentorial atrophy ( = 20). Eight of 9 patients who had follow-up neuroimaging showed progressive changes.
CONCLUSIONS
We identified reported classic neuroimaging features in all except 1 patient with neuronal ceroid lipofuscinoses in our study. CLN2, CLN5, and CLN7 diseases showed predominant cerebellar-over-cerebral atrophy. We demonstrate that abnormal signal intensity in the deep white matter, posterior limb of the internal capsule, and ventral pons is more common than previously reported in the literature. We report abnormal signal intensity in the insular/subinsular region for the first time. The difference in the median time from disease onset and diagnosis was 1.5 years.
Topics: Brain; Child; Child, Preschool; Female; Humans; Infant; Magnetic Resonance Imaging; Male; Neuroimaging; Neuronal Ceroid-Lipofuscinoses; Phenotype; Tripeptidyl-Peptidase 1
PubMed: 32855186
DOI: 10.3174/ajnr.A6726 -
Child's Nervous System : ChNS :... Apr 2017The purpose of this paper is to study the presentation and analyse the results of multimodality treatment of brain arterio-venous malformations (AVMs) in children at our... (Review)
Review
Multimodality management and outcomes of brain arterio-venous malformations (AVMs) in children: personal experience and review of the literature, with specific emphasis on age at first AVM bleed.
PURPOSE
The purpose of this paper is to study the presentation and analyse the results of multimodality treatment of brain arterio-venous malformations (AVMs) in children at our centre and review age at first AVM rupture in the literature.
METHODS
Of 52 patients aged <18 years, 47 with brain AVMs (27 males and 20 females) aged 4-17 years (mean 12.2) were retrospectively reviewed. PubMed search revealed five additional studies including 267 patients where the prevalence of age-related AVMs rupture was analysed.
RESULTS
In our study, 37 patients had bled, 9 were symptomatic without haemorrhage and 1 was incidental. Spetzler-Martin score distribution was 5 cases grade I, 18 grade II, 21 grade III and 3 grade IV. Appropriate imaging was performed, either CT/MRI angiogram only (in emergency cases) or catheter angiogram, prior to definitive treatment. There were 40 supratentorial and 7 infratentorial AVMs. Twenty-nine patients had microsurgery alone and 9 patients were treated by radiosurgery only. Three patients were embolised, all followed by radiosurgery, with one requiring surgery too, while 4 patients had combined surgery and radiosurgery. One patient is awaiting radiosurgery while another was not treated. Good outcomes, classified as modified Rankin score (mRS) 0-2 improved significantly after intervention to 89.4% from 38.3% pre-treatment (p value <0.0001). Angiography confirmed 96.6% obliteration after first planned operation. Repeat cerebral angiogram around age 18 was negative in all previously cured patients. Reviewing the literature, 82.0% (95% CI = [77-87]; N = 267) of children diagnosed with brain AVMs (mean age 11.4 ± 0.4) presented with a bleed in the last 22 years. Males significantly outnumbered females (136 vs 84) (p < 0.001). Ninety-five patients underwent surgical intervention alone when compared to other treatment modalities (p < 0.001).
CONCLUSIONS
Microsurgical excision of surgically accessible intracranial AVMs remains the primary treatment option with very good outcomes. A significant number of patients' AVMs ruptured around puberty; therefore, understanding the pathophysiology of AVM instability at this age may aid future therapy.
Topics: Adolescent; Arteriovenous Fistula; Brain; Child; Child, Preschool; Disease Management; Female; Humans; Intracranial Arteriovenous Malformations; Male; Microsurgery; Neuroimaging; PubMed; Treatment Outcome
PubMed: 28324183
DOI: 10.1007/s00381-017-3383-4 -
AJNR. American Journal of Neuroradiology Oct 2010CS is an autosomal recessive multisystem disorder, which is mainly characterized by neurologic and sensory impairment, cachectic dwarfism, and photosensitivity. We...
CS is an autosomal recessive multisystem disorder, which is mainly characterized by neurologic and sensory impairment, cachectic dwarfism, and photosensitivity. We describe the neuroimaging features (MR imaging, ¹H-MR spectroscopy, and CT) in the various clinical subtypes of CS from a cohort of genetically and biochemically proved cases. Hypomyelination, calcifications, and brain atrophy were the main imaging features. Calcifications were typically found in the putamen and less often in the cortex and dentate nuclei. Severe progressive atrophy was seen in the supratentorial white matter, the cerebellum, the corpus callosum, and the brain stem. Patients with early-onset disease displayed more severe hypomyelination and prominent calcifications in the sulcal depth of the cerebral cortex, but atrophy was less severe in late-onset patients. On proton MR spectroscopy, lactate was detected and Cho and NAA values were decreased. These combined neuroradiologic findings can help in the differential diagnosis of CS, distinguishing it from other leukoencephalopathies and/or cerebral calcifications in childhood.
Topics: Adolescent; Biomarkers; Brain; Child; Child, Preschool; Cockayne Syndrome; Female; Humans; Infant; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Male; Middle Aged; Protons; Radionuclide Imaging; Tomography, X-Ray Computed; Young Adult
PubMed: 20522568
DOI: 10.3174/ajnr.A2135 -
Acta Neuropathologica Communications Feb 2020Glioblastoma is the most frequent and aggressive primary brain tumor, characterized by extensive brain invasion and rarely, systemic metastases. The pathogenesis of...
Glioblastoma is the most frequent and aggressive primary brain tumor, characterized by extensive brain invasion and rarely, systemic metastases. The pathogenesis of metastatic glioblastoma is largely unknown. We present the first integrated clinical/histologic/genetic analysis of 5 distinct brain and lung foci from a unique case of recurrent, multifocal, multicentric and metastatic glioblastoma. The initial right frontotemporal gliosarcoma received standard surgical/chemoradiation therapy and recurred 1.5 years later, co-occurring with three additional masses localized to the ipsilateral temporal lobe, cerebellum and lung. Synchronous metastatic lung carcinoma was suspected in this long-term smoker patient with family history of cancer. However, glioblastoma was confirmed in all tumors, although with different morphologic patterns, including ependymomatous and epithelioid. Genomic profiling revealed a germline FANCD2 variant of unknown significance, and a 4-gene somatic mutation signature shared by all tumors, consisting of TERT promoter and PTEN, RB1 and TP53 tumor suppressor mutations. Additional GRIN2A and ATM heterozygous mutations were selected in the cerebellar and lung foci, but were variably present in the supratentorial foci, indicating reduced post-therapeutic genetic evolution in brain foci despite morphologic variability. Significant genetic drift characterized the lung metastasis, likely explaining the known resistance of circulating glioblastoma cells to systemic seeding. MET overexpression was detected in the initial gliosarcoma and lung metastasis, possibly contributing to invasiveness. This comprehensive analysis sheds light on the temporospatial evolution of glioblastoma and underscores the importance of genetic testing for diagnosis and personalized therapy.
Topics: Brain; Brain Neoplasms; Female; Glioblastoma; Humans; Lung; Lung Neoplasms; Middle Aged; Mutation; Neoplasm Recurrence, Local
PubMed: 32014051
DOI: 10.1186/s40478-020-0889-x -
Cerebellum (London, England) Sep 2009Traumatic brain injury (TBI) is a major cause of morbidity and mortality worldwide. Studies of human TBI demonstrate that the cerebellum is sometimes affected even when... (Review)
Review
Traumatic brain injury (TBI) is a major cause of morbidity and mortality worldwide. Studies of human TBI demonstrate that the cerebellum is sometimes affected even when the initial mechanical insult is directed to the cerebral cortex. Some of the components of TBI, including ataxia, postural instability, tremor, impairments in balance and fine motor skills, and even cognitive deficits, may be attributed in part to cerebellar damage. Animal models of TBI have begun to explore the vulnerability of the cerebellum. In this paper, we review the clinical presentation, pathogenesis, and putative mechanisms underlying cerebellar damage with an emphasis on experimental models that have been used to further elucidate this poorly understood but important aspect of TBI. Animal models of indirect (supratentorial) trauma to the cerebellum, including fluid percussion, controlled cortical impact, weight drop impact acceleration, and rotational acceleration injuries, are considered. In addition, we describe models that produce direct trauma to the cerebellum as well as those that reproduce specific components of TBI including axotomy, stab injury, in vitro stretch injury, and excitotoxicity. Overall, these models reveal robust characteristics of cerebellar damage including regionally specific Purkinje cell injury or loss, activation of glia in a distinct spatial pattern, and traumatic axonal injury. Further research is needed to better understand the mechanisms underlying the pathogenesis of cerebellar trauma, and the experimental models discussed here offer an important first step toward achieving that objective.
Topics: Animals; Brain Injuries; Cerebellum; Disease Models, Animal; Humans; Purkinje Cells
PubMed: 19495901
DOI: 10.1007/s12311-009-0114-8