scholarly journals Case Report: Minimal Neurological Deficit of Two Adult Patients With Weston–Hurst Syndrome Due to Early Craniectomy: Case Series and Review of Literature on Craniectomy

2021 ◽  
Vol 12 ◽  
Author(s):  
Anna Mira Loesch-Biffar ◽  
Andreas Junker ◽  
Jennifer Linn ◽  
Niklas Thon ◽  
Suzette Heck ◽  
...  
Keyword(s):  
White Matter ◽  
Intracranial Pressure ◽  
White Matter Lesion ◽  
Case Series ◽  
Exact Test ◽  
Progressive Aphasia ◽  
Decompressive Craniotomy ◽  
Typical Morphology ◽  
Cranial Mri ◽  
Immunomodulatory Therapies

Objectives: We describe two new cases of acute hemorrhagic leucoencephalitis (AHLE), who survived with minimal sequelae due to early measures against increased intracranial pressure, particularly craniotomy. The recently published literature review on treatment and outcome of AHLE was further examined for the effect of craniotomy.Methods: We present two cases from our institution. The outcome of 44 cases from the literature was defined either as good (no deficit, minimal deficit/no daily help) or poor outcome (severe deficit/disabled, death). Patients with purely infratentorial lesions (n = 9) were excluded. Fisher's exact test was applied.Results: Two cases are presented: A 43-year-old woman with rapidly progressive aphasia and right hemiparesis due to a huge left frontal white matter lesion with rim contrast enhancement. Pathology was consistent with AHLE. The second case was a 56-year-old woman with rapidly progressive aphasia and right hemiparesis. Cranial MRI showed a huge left temporo-occipital white matter lesion with typical morphology for AHLE. Both patients received craniotomy within the first 24 h and consequent immunosuppressive-immunomodulatory treatment and survived with minimal deficits. Out of 35 supratentorial reported AHLE cases, seven patients received decompressive craniotomy. Comparing all supratentorial cases, patients who received craniotomy were more likely to have a good outcome (71 vs. 29%).Conclusion: Due to early control of the intracranial pressure, particularly due to early craniotomy; diagnosis per biopsy; and immediate start of immunosuppressive-immunomodulatory therapies (cortisone pulse, plasma exchanges), both patients survived with minimal sequelae. Craniotomy plays an important role and should be considered early on in patients with probable AHLE.

White Matter Lesion Burden and Depressive Symptoms in MCI Subtypes

2010 ◽  
Author(s):  
Lisa M. Delano-Wood ◽  
Norman Abeles ◽  
Mark W. Bondi ◽  
David J. Libon ◽  
Melissa Lamar ◽  
...  
Keyword(s):  
Depressive Symptoms ◽  
White Matter ◽  
White Matter Lesion

Early and Mild Phases of Primary Progressive Aphasia: A Case Series

2017 ◽  
Vol 10 (2) ◽  
pp. 136-142
Author(s):  
Jessica Powell ◽  
James Lendrum ◽  
Rosalind Huff ◽  
Christine Belden ◽  
Marwan N. Sabbagh
Keyword(s):  
Case Series ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive

scholarly journals Infratentorial pathology in frontotemporal dementia: cerebellar grey and white matter alterations in FTD phenotypes

2021 ◽  
Author(s):  
Mary Clare McKenna ◽  
Rangariroyashe H. Chipika ◽  
Stacey Li Hi Shing ◽  
Foteini Christidi ◽  
Jasmin Lope ◽  
...  
Keyword(s):  
White Matter ◽  
Fractional Anisotropy ◽  
Cortical Thickness ◽  
Cerebellar Atrophy ◽  
Primary Progressive Aphasia ◽  
Radial Diffusivity ◽  
Axial Diffusivity ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Crus I

AbstractThe contribution of cerebellar pathology to cognitive and behavioural manifestations is increasingly recognised, but the cerebellar profiles of FTD phenotypes are relatively poorly characterised. A prospective, single-centre imaging study has been undertaken with a high-resolution structural and diffusion tensor protocol to systematically evaluate cerebellar grey and white matter alterations in behavioural-variant FTD(bvFTD), non-fluent variant primary progressive aphasia(nfvPPA), semantic-variant primary progressive aphasia(svPPA), C9orf72-positive ALS-FTD(C9 + ALSFTD) and C9orf72-negative ALS-FTD(C9-ALSFTD). Cerebellar cortical thickness and complementary morphometric analyses were carried out to appraise atrophy patterns controlling for demographic variables. White matter integrity was assessed in a study-specific white matter skeleton, evaluating three diffusivity metrics: fractional anisotropy (FA), axial diffusivity (AD) and radial diffusivity (RD). Significant cortical thickness reductions were identified in: lobule VII and crus I in bvFTD; lobule VI VII, crus I and II in nfvPPA; and lobule VII, crus I and II in svPPA; lobule IV, VI, VII and Crus I and II in C9 + ALSFTD. Morphometry revealed volume reductions in lobule V in all groups; in addition to lobule VIII in C9 + ALSFTD; lobule VI, VIII and vermis in C9-ALSFTD; lobule V, VII and vermis in bvFTD; and lobule V, VI, VIII and vermis in nfvPPA. Widespread white matter alterations were demonstrated by significant fractional anisotropy, axial diffusivity and radial diffusivity changes in each FTD phenotype that were more focal in those with C9 + ALSFTD and svPPA. Our findings indicate that FTD subtypes are associated with phenotype-specific cerebellar signatures with the selective involvement of specific lobules instead of global cerebellar atrophy.

scholarly journals Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Harry Knights ◽  
Elizabeth Minas ◽  
Faraan Khan ◽  
Lindsay Shaw ◽  
Muthana Al Obaidi ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Brain Imaging ◽  
Case Series ◽  
Dystrophic Calcification ◽  
Resonance Imaging ◽  
Cutaneous Manifestations ◽  
Street Hospital ◽  
Parry Romberg Syndrome

Abstract Background The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with/without Parry-Romberg syndrome (PRS); and (ii) identify clinical predictors of brain imaging abnormalities. Methods This was a single centre (Great Ormond Street Hospital, London) retrospective case series of patients with ECDS/PRS seen from 2000 to 2018. We identified patients with cutaneous manifestations consistent with the clinical descriptions of ECDS/PRS. Presenting clinical, laboratory, and radiological brain findings are described. Results are expressed as medians and ranges or frequencies and percentages. Fisher’s exact test was used to identify clinical associations with magnetic resonance imaging (MRI) abnormalities. Results Fourteen patients were studied: 6 males and 8 females; median age 14 years (range 3–20). We observed neuroimaging abnormalities in 2/6 ECDS and 5/8 ECDS/PRS patients. White matter signal abnormality, dystrophic calcification, leptomeningeal enhancement, and sulcal crowding were the typical findings on brain imaging. A total of 50% of patients had no MRI abnormality despite some of these patients having neurological symptoms. The presence of seizures was significantly associated with ipsilateral enhanced white matter signalling on MRI (p < 0.05). Conclusions In summary, we observed several distinct radiographic patterns associated with ECDS/PRS. Seizure disorder was strongly associated with the presence of ipsilateral enhanced white matter signalling. Improved neuroimaging techniques that combine morphological with functional imaging may improve the detection rate of brain involvement in children with ECDS/PRS in the future.

scholarly journals Gliosarcoma vs. glioblastoma: a retrospective case series using molecular profiling

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Christopher Dardis ◽  
David Donner ◽  
Nader Sanai ◽  
Joanne Xiu ◽  
Sandeep Mittal ◽  
...  
Keyword(s):  
Copy Number ◽  
Epithelial To Mesenchymal Transition ◽  
Clinical Care ◽  
Case Series ◽  
Molecular Profiling ◽  
Retrospective Case ◽  
Who Grade ◽  
Exact Test ◽  
Mesenchymal Transition ◽  
Dna And Rna

Abstract Background Gliosarcoma (GS) refers to the presence of mesenchymal differentiation (as seen using light microscopy) in the setting of glioblastoma (GB, an astrocytoma, WHO Grade 4). Although the same approach to treatment is typically adopted for GS and GB, there remains some debate as to whether GS should be considered a discrete pathological entity. Differences between these tumors have not been clearly established at the molecular level. Methods Patients with GS (n=48) or GB (n=1229) underwent molecular profiling (MP) with a pan-cancer panel of tests as part of their clinical care. The methods employed included next-generation sequencing (NGS) of DNA and RNA, copy number variation (CNV) of DNA and immunohistochemistry (IHC). The MP comprised 1153 tests in total, although results for each test were not available for every tumor profiled. We analyzed this data retrospectively in order to determine if our results were in keeping with what is known about the pathogenesis of GS by contrast with GB. We also sought novel associations between the MP and GS vs. GB which might improve our understanding of pathogenesis of GS. Results Potentially meaningful associations (p<0.1, Fisher’s exact test (FET)) were found for 14 of these tests in GS vs. GB. A novel finding was higher levels of proteins mediating immuno-evasion (PD-1, PD-L1) in GS. All of the differences we observed have been associated with epithelial-to-mesenchymal transition (EMT) in other tumor types. Many of the changes we saw in GS are novel in the setting of glial tumors, including copy number amplification in LYL1 and mutations in PTPN11. Conclusions GS shows certain characteristics of EMT, by contrast with GB. Treatments targeting immuno-evasion may be of greater therapeutic value in GS relative to GB.

Ginkgolide B promotes oligodendrocyte precursor cell differentiation and survival via Akt/CREB/bcl-2 signaling pathway after white matter lesion

2021 ◽  
pp. 153537022198995
Author(s):  
Jian Huang ◽  
Jun Yang ◽  
Xingju Zou ◽  
Shilun Zuo ◽  
Jing Wang ◽  
...  
Keyword(s):  
White Matter ◽  
White Matter Lesion ◽  
Neuroprotective Effect ◽  
Precursor Cell ◽  
Cerebral Hypoperfusion ◽  
Ginkgolide B ◽  
Oligodendrocyte Precursor Cell ◽  
Learning Abilities ◽  
Phosphorylated Akt ◽  
Oligodendrocyte Precursor

White matter lesion (WML) is caused by chronic cerebral hypoperfusion, which are usually associated with cognitive impairment. Evidence from recent studies has shown that ginkgolide B has a neuroprotective effect that could be beneficial for the treatment of ischemia; however, it is not clear whether ginkgolide B has a protective effect on WML. Our data show that ginkgolide B can promote the differentiation of oligodendrocyte precursor cell (OPC) into oligodendrocytes and promote oligodendrocyte survival following a WML. Ginkgolide B (5, 10, 20 mg/kg) or saline is administered intraperitoneally every day after WML. After 4 weeks, the data of Morris water maze suggested that rats’ memory and learning abilities were impaired, and the administration of ginkgolide B enhanced behavioral achievement. Also, treatment with ginkgolide B significantly attenuated this loss of myelin. Our result suggests that ginkgolide B promotes the differentiation of OPC into oligodendrocytes. We also found that ginkgolide B ameliorates oligodendrocytes apoptosis. Furthermore, ginkgolide B enhanced the expression of phosphorylated Akt and CREB. In conclusion, our data firstly show that ginkgolide B promotes oligodendrocyte genesis and oligodendrocyte myelin following a WML, possibly involving the Akt and CREB pathways.

Sign in / Sign up

Export Citation Format

Share Document