Search Our Scientific Publications & Authors

Clin Neuropathol 2020 Jul 7. Epub 2020 Jul 7.

Although increasing numbers of central nervous system (CNS) tumors with stereotypic morphological, molecular, and/or site-specific features have been recently reported, morphological diversity is often recognized within a tumor category as more cases are encountered. Such was the case with diffuse midline gliomas, H3K27M-mutant. Therefore, it is not surprising that two cases of H3G34-mutant. Read More

Indian J Pathol Microbiol 2020 Apr-Jun;63(2):262-266

Department of Pathology, Huashan Hospital, Fudan University, Shanghai, China.

Recurrent mutations in H3F3A that encodes the histone 3 variant H3.3, lead to amino acid substitutions including K27M and G34R/V-which are observed in high-grade gliomas (HGGs) of children and young adults. Previous studies have focused on gliomas with K27M mutation, whereas gliomas with G34R/V mutation have received little attention. Read More

Mol Biol (Mosk) 2020 Jan-Feb;54(1):128-136

Institute of Cell Biophysics, Russian Academy of Sciences, Pushchino, Moscow region, 142290 Russia.

Neuroinflammation plays a key role in the pathogenesis of neurodegenerative diseases. Microglial cells are the main immune cells of the central nervous system. On exposure to lipopolysaccharides (LPS, components of the cell wall of Gram-negative enterobacteria), microglia is activated to produce reactive oxygen species (ROS), cytokines, and inflammatory mediators, which may cause neuron death. Read More

Rom J Morphol Embryol 2019 ;60(3):761-767

1st Medical Department, Department of Morphofunctional Sciences I - Pathology, Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, Iaşi, Romania;

In childhood and in adolescence, primary tumors of the central nervous system (CNS) are the second most common malignancy after leukemia. The most common entities are gliomas, craniopharyngiomas and embryonal tumors, including primitive neuroectodermal tumors of the CNS, such as medulloblastoma. Proper management of malignancies requires a histological diagnosis, especially in childhood, since diagnostic errors have a significant negative influence on the treatment and subsequently on the patient's care. Read More

Int J Mol Sci 2019 Dec 29;21(1). Epub 2019 Dec 29.

IRCCS San Raffaele Scientific Institute, via Olgettina 58, 20132 Milan, Italy.

RE-1 silencing transcription factor (REST) (known also as NRSF) is a well-known transcription repressor whose strong decrease induces the distinction of neurons with respect to the other cells. Such distinction depends on the marked increased/decreased expression of specific genes, accompanied by parallel changes of the corresponding proteins. Many properties of REST had been identified in the past. Read More

Anticancer Res 2020 Jan;40(1):53-66

Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden

Background/aim: Medulloblastoma (MB) accounts for ~20% of pediatric malignant central nervous system tumors. Treatment strategies, including surgery, radiation therapy and/or chemotherapy, are effective, but recurrence and metastasis frequently occur. Therefore, novel therapies are required. Read More

BMJ Case Rep 2019 Dec 29;12(12). Epub 2019 Dec 29.

Department of Urology, MIOT International, Chennai, India.

A 20-year-old man presented to the department of neurology with diplopia, occipital headache and right flank pain for 1-week duration. CT of the brain revealed skull metastasis with heterogeneously enhancing dural-based mass lesion at the occipital region. Positron emission tomography revealed tracer avid soft tissue mass involving the upper pole of the right kidney with loss of fat planes with the inferior surface of the liver. Read More

Nat Commun 2019 12 20;10(1):5829. Epub 2019 Dec 20.

Department of Neurology, University of North Carolina School of Medicine, Chapel Hill, NC, 27599, USA.

Targeting oncogenic pathways holds promise for brain tumor treatment, but inhibition of Sonic Hedgehog (SHH) signaling has failed in SHH-driven medulloblastoma. Cellular diversity within tumors and reduced lineage commitment can undermine targeted therapy by increasing the probability of treatment-resistant populations. Using single-cell RNA-seq and lineage tracing, we analyzed cellular diversity in medulloblastomas in transgenic, medulloblastoma-prone mice, and responses to the SHH-pathway inhibitor vismodegib. Read More

J Toxicol Environ Health A 2019 9;82(21):1129-1136. Epub 2019 Dec 9.

Public Health Research Institute of Jiangsu Province, Jiangsu Center for Disease Control and Prevention, Jiangsu, China.

Microcystin-LR (MC-LR) variant exposure poses a potential health hazard to ecosystem, animals, and humans. Previously investigators showed that autophagy plays a key role in MC-LR induced cytotoxicity immortalized murine ovarian granular KK-1 cells and rat Sertoli cells. Recently exposure to MC-LR via drinking water was reported to accumulate in mouse brain with associated adverse oxidant and inflammatory responses. Read More

Front Vet Sci 2019 14;6:401. Epub 2019 Nov 14.

Department of Veterinary Clinical Medicine, University of Illinois at Urbana-Champaign, Urbana, IL, United States.

A medulloblastoma was surgically debulked from a 6 year old American Staffordshire Terrier, who then received a modified lomustine (CCNU), vincristine, procarbazine, and prednisolone (LOPP) protocol. The dog improved significantly and continued to do well until deterioration and euthanasia 5 months following surgery. This is the first known published case report of surgical cytoreductive surgery of a medulloblastoma in a dog with documented response to surgery and chemotherapy. Read More

World Neurosurg 2020 Feb 27;134:e1115-e1120. Epub 2019 Nov 27.

Department of Neurosurgery, Chinese PLA General Hospital, Beijing, China. Electronic address:

Background: The suboccipital midline approach is common dealing with posterior fossa tumors but has a high risk of postoperative complications, such as pseudomeningocele, cerebrospinal fluid (CSF) leak, and meningitis. Neurosurgeons used various kinds of method to lower its rate.

Methods: A retrospective, single-center review of patients diagnosed with posterior fossa tumor underwent a suboccipital midline approach. Read More

Nat Genet 2019 12 25;51(12):1702-1713. Epub 2019 Nov 25.

Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada.

Childhood brain tumors have suspected prenatal origins. To identify vulnerable developmental states, we generated a single-cell transcriptome atlas of >65,000 cells from embryonal pons and forebrain, two major tumor locations. We derived signatures for 191 distinct cell populations and defined the regional cellular diversity and differentiation dynamics. Read More

Pan Afr Med J 2019 11;34:23. Epub 2019 Sep 11.

Neurosurgery Department, Ibn-Tofail Hospital, Marrakech, Morocco.

Central nervous system tuberculosis is a major cause of morbidity and mortality in developing countries. Intracranial tuberculoma is rare and is one of the most severe cases of tuberculosis. We present two cases. Read More

Adv Anat Pathol 2020 Jan;27(1):44-50

Department of Pathology.

Molecular studies have identified distinct genomic drivers providing insights in biology of brain tumors. Advances in genetic and epigenetic analysis, as well as development of mutation-specific antibodies enable more accurate classification of histologically indistinguishable tumors. Compared with histopathologic grading, molecular biomarkers are also superior in predicting natural behavior of tumors and therapeutic response. Read More

F1000Res 2019 29;8. Epub 2019 Oct 29.

Montreal Clinical Research Institute (IRCM), Montreal, Quebec, Canada.

Medulloblastoma, the most common of the malignant pediatric brain tumors, is a group of four molecularly and clinically distinct cancers with different cells of origin. One of these medulloblastoma groups displays activation of Sonic hedgehog (SHH) signaling and originates from granule cell precursors of the developing cerebellum. Ongoing basic and clinical research efforts are tailored to discover targeted and safer therapies, which rely on the identification of the basic mechanisms regulating tumor initiation, progression, and metastasis. Read More

Rev Gastroenterol Peru 2019 Jul-Sep;39(3):280-283

Servicio de Gastroenterología, Clínica Ricardo Palma. Lima, Peru.

Turcot syndrome is an association of primary neuroepithelial tumors of the central nervous system with adenomatous polyposis coli. It is a genetic disorder, with two forms; In type I, glioblastomas are usually associated with hereditary nonpolyposis colorectal cancer (HNPC or Lynch). In Type II, medulloblastomas are often associated with familial adenomatous polyposis coli (classical or attenuated). Read More

Eur J Pharm Sci 2020 Jan 25;142:105106. Epub 2019 Oct 25.

Department of Pharmaceutical Sciences, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 28105, USA. Electronic address:

Prexasertib (LY2606368) is a potent and selective small molecule inhibitor of cell-cycle checkpoint CHK1 and CHK2 protein kinases and is currently under clinical evaluation for treatment of pediatric malignancies. As a candidate therapy for pediatric Group 3 medulloblastoma (G3MB), prexasertib CNS penetration was evaluated in mice using cerebral microdialysis and pharmacokinetic modeling. A plasma pharmacokinetic study with a population-based design was performed in CD1 nude mice bearing G3MB orthotopically implanted in the brain and receiving a single dose of prexasertib (10 mg/kg, subcutaneously) to characterize prexasertib disposition and to establish a limited plasma sampling model for the microdialysis studies. Read More

AJNR Am J Neuroradiol 2019 11 10;40(11):1796-1803. Epub 2019 Oct 10.

Department of Radiology (L.A.H.), New York Presbyterian Hospital, New York, New York.

Background And Purpose: Supratentorial primitive neuroectodermal tumors and pineoblastomas have traditionally been grouped together for treatment purposes. Molecular profiling of these tumors has revealed a number of distinct entities and has led to the term "CNS-primitive neuroectodermal tumors" being removed from the 2016 World Health Organization classification. The purpose of this study was to describe the MR imaging findings of histologically diagnosed primitive neuroectodermal tumors and pineoblastomas and correlate them with molecular diagnoses and outcomes. Read More

Br J Radiol 2020 Mar 1;93(1107):20190673. Epub 2019 Nov 1.

Department of Radiation Oncology, Harvard Medical School, Massachusetts General Hospital, Boston, United States.

Objective: The Pediatric Proton/Photon Consortium Registry (PPCR) is a comprehensive data registry composed of pediatric patients treated with radiation. It was established to expedite outcomes-based research. The attributes which allow the PPCR to be a successful collaboration are reviewed. Read More

Pediatr Blood Cancer 2020 01 8;67(1):e28032. Epub 2019 Oct 8.

Gustave Roussy Cancer Center, Department of Pediatric and Adolescent Oncology, Villejuif, France.

Aim: To assess objective response after two cycles of temozolomide and topotecan (TOTEM) in children with refractory or relapsed miscellaneous extracranial solid and central nervous system (CNS) tumors, including medulloblastoma and primitive neuroectodermal tumors (PNET).

Procedure: Multicenter, nonrandomized, phase 2 basket trial including children with solid tumors, completed by a one-stage design confirmatory cohort for medulloblastoma, and an exploratory cohort for PNET. Main eligibility criteria were refractory/relapsed measurable disease and no more than two prior treatment lines. Read More

Mutat Res 2019 09 30;845:402990. Epub 2018 Nov 30.

Department of Molecular Biology and Translational Research, Institute of Rural Health, Jaczewskiego 2, 20-090, Lublin, Poland; Department of Medical Biology and Translational Research, Faculty of Medicine, University of Information Technology and Management, Sucharskiego 2, 35-225, Rzeszów, Poland.

Biomonitoring of the effects of environmental and occupational exposure relevant chemical or physical factors on central nervous system is difficult due to the problems with sampling of biological material. Thus, surrogate systems allowing for the estimation of effect intensity are necessary to evaluate a potential risk of exposure. Cancerous neural cells in culture seem to be a reliable trustworthy alternative to ex vivo primary cells culture, where brain tissue is hardly available. Read More

Acta Oncol 2019 Dec 12;58(12):1752-1756. Epub 2019 Sep 12.

Radiation Oncology, The Hospital of the University of Pennsylvania, Philadelphia, PA, USA.

Radiation therapy (RT) plays an important role in management of pediatric central nervous system (CNS) malignancies. Centers are increasingly utilizing pencil beam scanning proton therapy (PBS-PT). However, the risk of brainstem necrosis has not yet been reported. Read More

Pediatr Blood Cancer 2019 12 12;66(12):e27972. Epub 2019 Sep 12.

Department of Radiation Oncology, University of Pennsylvania, Philadelphia, Pennsylvania.

Background: Radiotherapy is often deferred in very young children with medulloblastoma, in favor of more intense chemotherapy and stem cell rescue; however, posterior fossa radiation has been shown to improve overall survival (OS) and event-free survival compared with adjuvant chemotherapy alone. This study was performed to assess the OS, recurrence-free survival (RFS), patterns of failure, and clinical toxicity for children aged five and under who received focal proton radiation to the tumor bed alone.

Procedure: From 2010 to 2017, 14 patients with newly diagnosed medulloblastoma at one institution received tumor bed irradiation following surgery and chemotherapy. Read More

Zhonghua Bing Li Xue Za Zhi 2019 Sep;48(9):694-699

Department of Pathology, Institute of Basic Medical Sciences Chinese Academy of Medical Science, School of Basic Medicine, Peking Union Medical College; Molecular Pathology Center, Chinese Academy of Medical Sciences, Beijing 100005, China.

To investigate the expression and potential role of heterogeneous nuclear ribonucleo-protein A2B1 (HNRNPA2B1) in mouse cerebellar development and the significance of HNRNPA2B1 in human medulloblastoma. The data of HNRNPA2B1 RNA expression in mouse and human cerebella were obtained from databases. Western blot and immunohistochemical staining were performed to detect the protein level of HNRNPA2B1 in mouse cerebella at different ages. Read More

Int J Radiat Oncol Biol Phys 2019 12 28;105(5):1034-1042. Epub 2019 Aug 28.

Department of Radiation Oncology, Brigham and Women's Hospital, Dana-Farber Cancer Institute, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address:

Purpose: Brainstem necrosis is a rare, but dreaded complication of radiation therapy; however, data on the incidence of brainstem injury for tumors involving the posterior fossa in photon-treated patient cohorts are still needed.

Methods And Materials: Clinical characteristics and dosimetric parameters were recorded for 107 pediatric patients who received photon radiation for posterior fossa tumors without brainstem involvement from 2000 to 2016. Patients were excluded if they received a prescription dose <50. Read More

Nat Commun 2019 08 29;10(1):3885. Epub 2019 Aug 29.

Center for Translational Medicine, Department of Pharmacology, Lewis Katz School of Medicine at Temple University, Philadelphia, PA, 19140, USA.

Impairments in neuronal intracellular calcium (Ca) handling may contribute to Alzheimer's disease (AD) development. Metabolic dysfunction and progressive neuronal loss are associated with AD progression, and mitochondrial calcium (Ca) signaling is a key regulator of both of these processes. Here, we report remodeling of the Ca exchange machinery in the prefrontal cortex of individuals with AD. Read More

Gene Expr Patterns 2019 12 20;34:119068. Epub 2019 Aug 20.

Department of Biochemistry and Cellular Biology, National Institute of Neuroscience, NCNP, Tokyo, 187-8502, Japan. Electronic address:

Cerebellar granule cell precursors (GCPs) and granule cells (GCs) constitute a good model system to investigate proliferation of neural precursors and differentiation of neurons. During development, GCPs proliferate in the outer external granule cell layer (outer EGL) and then exit the cell cycle in the inner EGL to become GCs, which inwardly migrate to the inner granule cell layer (IGL). Misregulation of GCP proliferation or GC differentiation leads to maldevelopment of the cerebellum and the formation of a cerebellar tumor, medulloblastoma. Read More

World Neurosurg 2019 Nov 26;131:90-94. Epub 2019 Jul 26.

Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota, USA.

Background: Glioblastoma multiforme (GBM) is a rapid-growing central nervous system neoplasm. We report a case of GBM with extensive intramedullary lumbar drop metastasis and highly unusual osseous spine metastasis from a primary infratentorial GBM occurring 10 years after the initial diagnosis, which to our knowledge has not been described previously.

Case Description: This 37-year-old man presented with new-onset headaches of increasing severity. Read More

Brain Tumor Pathol 2019 Oct 26;36(4):169-173. Epub 2019 Jul 26.

Department of Pathology, Nara Medical University Hospital, Nara, Japan.

Here, we report a rare case of anaplastic pleomorphic xanthoastrocytoma (PXA) associated with an H3G34 mutation. A 12-year-old male presented with loss of appetite, vomiting, headache, and a generalized seizure, and CT revealed a 9.0 cm left frontal lobe mass with some septal walls and a localized high-density area suggestive of hemorrhage or calcification, causing severe midline shift. Read More

Nature 2019 08 24;572(7767):74-79. Epub 2019 Jul 24.

Department of Developmental Neurobiology, St Jude Children's Research Hospital, Memphis, TN, USA.

Medulloblastoma is a malignant childhood cerebellar tumour type that comprises distinct molecular subgroups. Whereas genomic characteristics of these subgroups are well defined, the extent to which cellular diversity underlies their divergent biology and clinical behaviour remains largely unexplored. Here we used single-cell transcriptomics to investigate intra- and intertumoral heterogeneity in 25 medulloblastomas spanning all molecular subgroups. Read More

Cancer Radiother 2019 Sep 19;23(5):370-377. Epub 2019 Jul 19.

Radiation Therapy Department, National Institute of Oncology, Mohamed V University, Rabat, Morocco. Electronic address:

Pupose: Medulloblastoma is the most common primary malignant central nervous system tumor in childhood, accounting for 16-25% of cases (1). New treatment approaches have led to improved survival rates; however toxicities are still a major concern.

Patients And Methods: Participants were selected from the records of patients who were treated with craniospinal irradiation for medulloblastoma. Read More

Pathol Res Pract 2019 Oct 13;215(10):152536. Epub 2019 Jul 13.

Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, CA, 90033, United States.

Primitive neuroectodermal tumor (PNET) traditionally encompasses two different classes of tumors with similar morphology - PNET of the peripheral nervous system (pPNET) and PNET of the central nervous system (cPNET). The latter also includes germ cell tumor-derived PNET (gPNET). There are currently no specific markers for gPNET. Read More

Neurochem Int 2019 10 17;129:104507. Epub 2019 Jul 17.

Undergraduate of Qingdao University. NO.308 Ningxia Road. Qingdao, Shandong, 266071, China.

Heightened activity of glycogen synthase kinase-3β (GSK-3β) is linked to the degeneration of dopaminergic neurons in Parkinson's disease (PD). Phytic acid (PA), a naturally occurring compound with potent antioxidant property, has been shown to confer neuroprotection on dopaminergic neurons in PD. However, the underlying mechanism remains unclear. Read More

J Neurooncol 2019 Aug 16;144(1):227-229. Epub 2019 Jul 16.

Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, NY, 10065, USA.

Magn Reson Imaging 2019 10 11;62:181-190. Epub 2019 Jul 11.

Laboratory of Biofunctional Imaging, Graduate School of Frontier Biosciences, Osaka University, Suita, Osaka 560-0871, Japan; Institute for Open and Transdisciplinary Research Initiatives, Osaka University, Suita, Osaka 560-0871, Japan.

Purpose: The purpose of this study was (i) to determine the optimal magnetization transfer (MT) pulse parameter for amide proton transfer (APT) chemical exchange saturation transfer (CEST) imaging on an ultra-high-field magnetic resonance imaging (MRI) system and (ii) to use APT CEST imaging to noninvasively assess brain orthotopic and ectopic tumor cells transplanted into the mouse brain.

Methods: To evaluate APT without the influence of other metabolites, we prepared egg white phantoms. Next, we used 7-11-week-old nude female mice and the following cell lines to establish tumors after injection into the left striatum of mice: C6 (rat glioma, n = 8) as primary tumors and Neuro-2A (mouse neuroblastoma, n = 11) and MDA-MB231 (human breast cancer, n = 8) as metastatic tumors. Read More

Food Chem Toxicol 2019 Oct 5;132:110665. Epub 2019 Jul 5.

Laboratorio de Farmacognosia, Dpto. de Farmacia, Facultad de Farmacia, P.O. Box 237, Universidad de Concepción, Concepción, Chile. Electronic address:

In this study we evaluate the chemical composition and neuroprotective effects of alkaloid fractions of the Amaryllidaceae species Rhodophiala pratensis, Rhodolirium speciosum, Phycella australis and Phaedranassa lehmannii. Gas chromatography-mass spectrometry (GC/MS) enable the identification of 41 known alkaloids. Rhodolirium speciosum and Rhodophiala pratensis were the most active extracts against acetylcholinesterase (AChE), with IC values of 35. Read More

Mol Pharmacol 2019 08 26;96(2):247-258. Epub 2019 Jun 26.

Departments of Anesthesiology and Critical Care Medicine (T.I., R.W., G.W.F.) and Neurology (G.W.P., Y.-X.P.), and Molecular Pharmacology Program (R.S., I.D., A.H., V.L.R., J.X., G.W.P.), Memorial Sloan-Kettering Cancer Center, New York, New York

Circular RNAs (circRNAs) are a distinct category of single-stranded, covalently closed RNAs formed by backsplicing. The functions of circRNAs are incompletely known and are under active investigation. Here, we report that in addition to traditional linear mRNAs (linRNA), mouse, rat, and human opioid receptor genes generate exonic circRNA isoforms. Read More

J Clin Neurosci 2019 Sep 22;67:93-98. Epub 2019 Jun 22.

Department of Neurosurgery, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400016, China.

Cerebellopontine angle (CPA) medulloblastoma is rare and short of system description. We attempted to clarify its epidemiology, clinical manifestations, imaging features, pathological and molecular types, and surgical outcomes. 8 patients from 7 to 52 years old were enrolled in this retrospective study, with mean age 21. Read More

Eur J Cancer Care (Engl) 2019 Sep 11;28(5):e13102. Epub 2019 Jun 11.

Department of Pediatrics, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.

Introduction: This study aims to assess the impact of paediatric benign and malignant solid tumours and its treatment on the health-related quality of life of children and adolescents who were followed up in a Reference Center in Pediatric Oncology in Rio de Janeiro.

Methods: It is a prospective cohort study. Quality of life assessment was performed using the PedsQL™ 4. Read More

Neurosci Lett 2019 08 23;707:134287. Epub 2019 May 23.

School of Forensic Medicine, Kunming Medical University, Kunming, Yunnan Province, China. Electronic address:

Methamphetamine (METH) has been shown to induce neuropathological dysfunction and irreversible brain cell damage. Prior studies indicated the involvement of autophagy in METH-induced neurotoxicity. However, the underlying mechanism by which autophagy contributes to METH-induced neurotoxicity remains elusive. Read More

J Biol Chem 2019 06 20;294(26):10336-10348. Epub 2019 May 20.

From the Department of Biomedical Sciences, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104,

Parkinson's disease (PD) is a major human disease associated with degeneration of the central nervous system. Evidence suggests that several endogenously formed 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-mimicking chemicals that are metabolic conversion products, especially β-carbolines and isoquinolines, act as neurotoxins that induce PD or enhance progression of the disease. We have demonstrated previously that mitochondrially targeted human cytochrome P450 2D6 (CYP2D6), supported by mitochondrial adrenodoxin and adrenodoxin reductase, can efficiently catalyze the conversion of MPTP to the toxic 1-methyl-4-phenylpyridinium ion. Read More

J Radiat Res 2019 Jul;60(4):527-537

Department of Radiation Medicine, Faculty of Medicine, Hokkaido University.

The outcomes of intensity-modulated proton craniospinal irradiation (ipCSI) are unclear. We evaluated the clinical benefit of our newly developed ipCSI system that incorporates two gantry-mounted orthogonal online X-ray imagers with a robotic six-degrees-of-freedom patient table. Nine patients (7-19 years old) were treated with ipCSI. Read More

Cancer Radiother 2019 Jun 18;23(3):179-187. Epub 2019 May 18.

Xhinhua general hospital, 1665 Kongjiang Rd, Yangpu, Shanghai, China. Electronic address:

Purpose: Medulloblastoma is the most common primary malignant central nervous system tumour in children. These last decades, treatment modalities have largely evolved resulting in better survival rates. Nevertheless, long-term toxicity is a major concern in this setting. Read More

Acta Neuropathol Commun 2019 05 20;7(1):78. Epub 2019 May 20.

Department of Neuropathology, DGNN Brain Tumor Reference Center, University of Bonn Medical Center, Bonn, Germany.

The recently described malignant neuro-epithelial tumors with histone H3F3A point mutations at G34 (NET-H3-G34) occur most often in cerebral hemispheres of teenagers and young adults, and have a generally adverse prognosis. These tumors have been histologically classified as glioblastoma or primitive neuroectodermal tumor (PNET) in the past, and have not been defined as a separate entity in the revised WHO classification of tumors of the CNS 2016. Here, we report two cases of NET-H3-G34 with glial and dysplastic ganglion cell components affecting teenagers. Read More

Klin Onkol 2019 ;32(Supplementum1):171-173

Background: Medulloblastoma (MB) is the most common malignant tumour of the central nervous system in children. MB is considered to be high risk tumour propensity to metastasize. In the Czech Republic, approximately 10-12 children are affected annually by this tumour. Read More

Toxicol Lett 2019 Sep 3;312:11-21. Epub 2019 May 3.

School of Forensic Medicine, Southern Medical University, Guangzhou 510515, People's Republic of China. Electronic address:

Methamphetamine (METH) is a widely abused illicit psychoactive drug. Our previous study has shown that CCAAT-enhancer binding protein β (C/EBPβ) is an important regulator in METH-induced neuronal autophagy and apoptosis. However, the detailed molecular mechanisms underlying this process remain poorly understood. Read More

Nature 2019 08 1;572(7767):67-73. Epub 2019 May 1.

Developmental & Stem Cell Biology Program, The Hospital for Sick Children, Toronto, Ontario, Canada.

Study of the origin and development of cerebellar tumours has been hampered by the complexity and heterogeneity of cerebellar cells that change over the course of development. Here we use single-cell transcriptomics to study more than 60,000 cells from the developing mouse cerebellum and show that different molecular subgroups of childhood cerebellar tumours mirror the transcription of cells from distinct, temporally restricted cerebellar lineages. The Sonic Hedgehog medulloblastoma subgroup transcriptionally mirrors the granule cell hierarchy as expected, while group 3 medulloblastoma resembles Nestin stem cells, group 4 medulloblastoma resembles unipolar brush cells, and PFA/PFB ependymoma and cerebellar pilocytic astrocytoma resemble the prenatal gliogenic progenitor cells. Read More

Pediatr Neurol 2019 08 28;97:50-55. Epub 2019 Mar 28.

Division of Child Neurology, Department of Pediatrics, Montreal, Québec, Canada. Electronic address:

Background: Seizures are one of the most common symptoms of pediatric brain tumors. The purpose of this study was to define seizures related to primary central nervous system tumors and to identify risk factors predictive of seizure occurrence and recurrence.

Methods: We reviewed the records of children treated from January 1, 2004, to January 1, 2018 and collected data including age, gender, tumor location, histology, extent of initial resection, seizure characteristics, treatment modalities, recurrence, and seizure control. Read More

J Mol Biol 2019 06 26;431(14):2581-2598. Epub 2019 Apr 26.

Institute of Pharmaceutical Sciences, University of Graz, Schubertstr. 1, 8010 Graz, Austria. Electronic address:

The recent discovery of biologically active fully disordered, so called random fuzzy protein-protein interactions leads to the question of how the high flexibility of these protein complexes correlates to aggregation and pathologic misfolding. We identify the structural mechanism by which a random fuzzy protein complex composed of the intrinsically disordered proteins alpha-Synuclein and SERF1a is able to potentiate cytotoxic aggregation. A structural model derived from an integrated NMR/SAXS analysis of the reconstituted aSyn:SERF1a complex enabled us to observe the partial deprotection of one precise aSyn amyloid nucleation element in the fully unstructured ensemble. Read More

J Neurosurg Pediatr 2019 04;24(1):35-40

Neurogenic stunned myocardium (NSM) is a potentially fatal cause of sudden cardiogenic dysfunction due to an acute neurological event, most commonly aneurysmal subarachnoid hemorrhage in adults. Only two pediatric cases of hydrocephalus-induced NSM have been reported. Here the authors report a third case in a 14-year-old boy who presented with severe headache, decreased level of consciousness, and shock in the context of acute hydrocephalus secondary to fourth ventricular outlet obstruction 3 years after standard-risk medulloblastoma treatment. Read More