Article
Radiology, Nuclear Medicine & Medical Imaging
Wei Yang, Ping Yang, Yiming Li, Jiahui Chen, Jiashu Chen, Yingjie Cai, Kaiyi Zhu, Hong Zhang, Yanhua Li, Yun Peng, Ming Ge
Summary: This study aimed to develop a CMS discrimination model based on multiparametric MRI radiomics. The results showed that multiparametric MRI radiomics may be more effective in predicting CMS than single-parameter MRI models and clinical models.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2023)
Article
Neuroimaging
Wei Yang, Yiming Li, Zesheng Ying, Yingjie Cai, Xiaojiao Peng, HaiLang Sun, Jiashu Chen, Kaiyi Zhu, Geli Hu, Yun Peng, Ming Ge
Summary: This study aimed to investigate the cerebellar mutism syndrome (CMS)-related brain regions and build a voxel-wise predictive model for CMS. The study found that the brain regions associated with CMS were mainly located in bilateral superior and inferior cerebellar peduncles and the superior part of the cerebellum. The areas under the curves for the predictive model in the training, validation, and prospective validation sets were 0.889, 0.784, and 0.791, respectively.
NEUROIMAGE-CLINICAL
(2023)
Article
Oncology
Martin A. Schaller-Paule, Peter Baumgarten, Volker Seifert, Marlies Wagner, Eike Steidl, Elke Hattingen, Felix Wicke, Joachim P. Steinbach, Christian Foerch, Juergen Konczalla
Summary: This study compared the risk of causing HOD and CMS in posterior fossa tumor surgery using different surgical approaches. It was found that a paravermal trans-cerebellar approach was more likely to cause HOD, while there was no clear correlation between the presence of HOD and development of CMS. Therefore, in planning posterior fossa surgery, it is important for neurosurgeons to consider trajectories and approaches that spare the dentate nucleus to avoid HOD.
Article
Clinical Neurology
Stephanie Schmidt, Edina Kovacs, Diren Usta, Rouven Behnisch, Felix Sahm, Daniel Haux, Olaf Witt, Till Milde, Andreas Unterberg, Ahmed El Damaty
Summary: This study evaluated the incidence of cerebellar mutism syndrome (CMS) after posterior fossa tumor surgery in pediatric patients and analyzed its association with multiple risk factors such as tumor entity, surgical approach, and hydrocephalus. The significant risk factors for CMS included a transvermian approach, vermian splitting when added to another approach, an initial presentation with acute hydrocephalus, and hydrocephalus present after tumor surgery.
WORLD NEUROSURGERY
(2023)
Article
Oncology
Molly E. Wickenhauser, Raja B. Khan, Darcy Raches, Jason M. Ashford, Kathryn M. W. Russell, Kristin Lyons, Giles W. Robinson, Amar Gajjar, Paul Klimo, Heather M. Conklin
Summary: This study describes the psychometric properties of a new questionnaire called the Posterior Fossa Syndrome Questionnaire (PFSQ), which aims to improve diagnostic consistency for posterior fossa syndrome (PFS) across clinical and research groups. The study found that different items of the PFSQ had varying sensitivity and specificity, and identified four main factors. The results of this study are important for accelerating the understanding of PFS etiology, predicting long-term impairments, and developing targeted interventions.
JOURNAL OF NEURO-ONCOLOGY
(2022)
Article
Neurosciences
Louisa Dahmani, Yan Bai, Meiling Li, Jianxun Ren, Lunhao Shen, Jianjun Ma, Haiyang Li, Wei Wei, Pengyu Li, Danhong Wang, Lei Du, Weigang Cui, Hesheng Liu, Meiyun Wang
Summary: High-intensity Magnetic Resonance-guided Focused Ultrasound (MRgFUS) is a non-invasive treatment for medication-resistant tremor. Lesioning the thalamic ventral intermediate nucleus (VIM) using MRgFUS resulted in significant tremor alleviation and functional reorganization of the hand region with the cerebellum. Control regions in other networks showed no association with tremor alleviation or normalization.
NPJ PARKINSONS DISEASE
(2023)
Article
Oncology
Raja B. Khan, Zoltan Patay, Paul Jr Jr Klimo, Jie Huang, Rahul Kumar, Frederick A. Boop, Darcy Raches, Heather M. Conklin, Richa Sharma, Andrea Simmons, Zsila S. Sadighi, Arzu Onar-Thomas, Amar Gajjar, Giles W. Robinson
Summary: Posterior fossa syndrome (PFS) is a known consequence of medulloblastoma resection, with younger age and surgery in a low-volume surgery center increasing the risk of PFS development. Sonic Hedgehog tumors reduce the risk of PFS. Children with PFS1 and PFS2 regained speech and gait at median times of 2.3/0.7 and 2.1/1.5 months, respectively.
Article
Clinical Neurology
S. M. Toescu, P. W. Hales, J. Cooper, E. W. Dyson, K. Mankad, J. D. Clayden, K. Aquilina, C. A. Clark
Summary: This study investigated how changes in perfusion metrics in children with posterior fossa tumors are influenced by cerebellar mutism syndrome and hydrocephalus requiring pre-resection CSF diversion. The results showed that cerebral blood flow increased after tumor resection and at follow-up scanning, while bolus arrival time decreased. Patients who underwent midline approach had longer bolus arrival time. Children with symptomatic obstructive hydrocephalus had more pronounced changes in regional perfusion. Children with cerebellar mutism syndrome had significantly increased cerebral blood flow at follow-up imaging.
AMERICAN JOURNAL OF NEURORADIOLOGY
(2022)
Review
Clinical Neurology
Maria Angeles de Miquel
Summary: This paper simplifies the evaluation of the main veins related to the brainstem and cerebellum. The posterior fossa venous drainage is best understood in relation to its three main collectors: superior, posterior, and anterior. A fourth possible drainage path is directed towards the inferior petrosal sinus.
FRONTIERS IN NEUROLOGY
(2021)
Review
Cell Biology
Francesco Fabozzi, Stella Margoni, Bianca Andreozzi, Maria Simona Musci, Giada Del Baldo, Luigi Boccuto, Angela Mastronuzzi, Andrea Carai
Summary: Cerebellar mutism syndrome is a common complication following surgical resection of childhood tumors in the posterior fossa. It is characterized by impaired linguistic production, long recovery time, and often results in long-term disabilities. The pathogenic mechanism is likely related to damage to the cerebellar pathway, and the treatment mainly focuses on rehabilitation.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Review
Clinical Neurology
Narjes Ahmadian, K. M. van Baarsen, P. A. J. T. Robe, E. W. Hoving
Summary: Pediatric postoperative cerebellar mutism syndrome (ppCMS) is a common complication following the resection of a cerebellar tumor in children. This systematic review found that reduced cerebral perfusion is observed in ppCMS patients compared to those without ppCMS, especially in the frontal lobe. Well-powered prospective studies with preoperative imaging are needed to further investigate the role of hypoperfusion in the pathophysiology of the syndrome.
CHILDS NERVOUS SYSTEM
(2021)
Review
Pediatrics
Cleiton Formentin, Andrei Fernandes Joaquim, Enrico Ghizoni
Summary: Pediatric posterior fossa tumors have a higher incidence rate compared to adult tumors. Molecular biomarkers play a crucial role in the diagnosis, prognosis, and treatment of these tumors. The current treatment strategy is mainly based on surgical resection, but molecular targeted therapies are also being explored.
EUROPEAN JOURNAL OF PEDIATRICS
(2023)
Article
Pediatrics
Jonathan Kjaer Gronbaek, Morten Wibroe, Sebastian Toescu, Radek Fric, Birthe Lykke Thomsen, Lisbeth Norgaard Moller, Pernilla Grillner, Bengt Gustavsson, Conor Mallucci, Kristian Aquilina, Greg Adam Fellows, Emanuela Molinari, Magnus Aasved Hjort, Mia Westerholm-Ormio, Rosita Kiudeliene, Katalin Mudra, Peter Hauser, Kirsten van Baarsen, Eelco Hoving, Julian Zipfel, Karsten Nysom, Kjeld Schmiegelow, Astrid Sehested, Marianne Juhler, Rene Mathiasen
Summary: This study found that midline tumour location, younger age, and high-grade tumour histology all increase the risk of speech impairment after posterior fossa tumour surgery in children. There was no evidence to recommend a preference for telovelar over transvermian surgical approach in the management of posterior fossa tumours in relation to the risk of developing POSI.
LANCET CHILD & ADOLESCENT HEALTH
(2021)
Article
Neurosciences
Christian Thaler, Qiyuan Tian, Max Wintermark, Pejman Ghanouni, Casey H. H. Halpern, Jaimie M. M. Henderson, Raag D. D. Airan, Michael Zeineh, Maged Goubran, Christoph Leuze, Jens Fiehler, Kim Butts Pauly, Jennifer A. McNab
Summary: This study detected microstructural changes within the CTCT after tcMRgFUS treatment and found a correlation between these changes and lesion-tract overlap, which helps to elucidate the treatment mechanism and improve targeting strategies.
BRAIN CONNECTIVITY
(2023)
Article
Pediatrics
Federica S. Ricci, Rossella D'Alessandro, Alessandra Soma, Anna Salvalaggio, Francesca Rossi, Sara Rampone, Giorgia Gamberini, Chiara Davico, Paola Peretta, Mario Cacciacarne, Pierpaolo Gaglini, Paolo Pacca, Giulia Pilloni, Paola Ragazzi, Daniele Bertin, Stefano G. Vallero, Franca Fagioli, Benedetto Vitiello
Summary: A diagnostic scale was developed to grade the duration and severity of post-operative pediatric cerebellar mutism syndrome (CMS). The study found that greater severity at onset predicted longer symptom persistence and severity at follow-up. Additionally, children with a midline tumor location and those aged under 5 years at diagnosis were at higher risk of CMS.
EUROPEAN JOURNAL OF PEDIATRICS
(2022)
Article
Oncology
George Michaiel, Douglas Strother, Nicholas Gottardo, Ute Bartels, Hallie Coltin, Juliette Hukin, Beverly Wilson, Shayna Zelcer, Jordan R. Hansford, Timothy Hassall, Mohamed S. AbdelBaki, Kristina A. Cole, Lindsey Hoffman, Natasha P. Smiley, Amy Smith, Anna Vinitsky, Nicholas A. Vitanza, Avery Wright, Kee K. Yeo, Lionel M. L. Chow, Magimairajan Vanan, Girish Dhall, Eric Bouffet, Lucie Lafay-Cousin
JOURNAL OF NEURO-ONCOLOGY
(2020)
Article
Biochemistry & Molecular Biology
Laura K. Donovan, Alberto Delaidelli, Sujith K. Joseph, Kevin Bielamowicz, Kristen Fousek, Borja L. Holgado, Alex Manno, Dilakshan Srikanthan, Ahmed Z. Gad, Randy Van Ommeren, David Przelicki, Cory Richman, Vijay Ramaswamy, Craig Daniels, Jonelle G. Pallota, Tajana Douglas, Alyssa C. M. Joynt, Joonas Haapasalo, Carolina Nor, Maria C. Vladoiu, Claudia M. Kuzan-Fischer, Livia Garzia, Stephen C. Mack, Srinidhi Varadharajan, Matthew L. Baker, Liam Hendrikse, Michelle Ly, Kaitlin Kharas, Polina Balin, Xiaochong Wu, Lei Qin, Ning Huang, Ana Guerreiro Stucklin, A. Sorana Morrissy, Florence M. G. Cavalli, Betty Luu, Raul Suarez, Pasqualino De Antonellis, Antony Michealraj, Avesta Rastan, Meenakshi Hegde, Martin Komosa, Olga Sirbu, Sachin A. Kumar, Zied Abdullaev, Claudia C. Faria, Stephen Yip, Juliette Hukin, Uri Tabori, Cynthia Hawkins, Ken Aldape, Mads Daugaard, John M. Maris, Poul H. Sorensen, Nabil Ahmed, Michael D. Taylor
Article
Oncology
Girish Dhall, Sharon H. O'Neil, Lingyun Ji, Kelley Haley, Ashley M. Whitaker, Marvin D. Nelson, Floyd Gilles, Sharon L. Gardner, Jeffrey C. Allen, Albert S. Cornelius, Kamnesh Pradhan, James H. Garvin, Randal S. Olshefski, Juliette Hukin, Melanie Comito, Stewart Goldman, Mark P. Atlas, Andrew W. Walter, Stephen Sands, Richard Sposto, Jonathan L. Finlay
Article
Pathology
Adrian Levine, Juliette Hukin, Christopher Dunham
PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
(2020)
Article
Oncology
Adriana Fonseca, Samina Afzal, Lynette Bowes, Bruce Crooks, Valerie Larouche, Nada Jabado, Sebastien Perreault, Donna L. Johnston, Shayna Zelcer, Adam Fleming, Katrin Scheinemann, Mariana Silva, Magimairajan Issai Vanan, Chris Mpofu, Beverly Wilson, David D. Eisenstat, Lucie Lafay-Cousin, Juliette Hukin, Cynthia Hawkins, Ute Bartels, Eric Bouffet
JOURNAL OF NEURO-ONCOLOGY
(2020)
Editorial Material
Clinical Neurology
Juliette Hukin
DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY
(2020)
Article
Oncology
Rahul Kumar, Kyle S. Smith, Maximilian Deng, Colt Terhune, Giles W. Robinson, Brent A. Orr, Anthony P. Y. Liu, Tong Lin, Catherine A. Billups, Murali Chintagumpala, Daniel C. Bowers, Timothy E. Hassall, Jordan R. Hansford, Dong Anh Khuong-Quang, John R. Crawford, Anne E. Bendel, Sridharan Gururangan, Kristin Schroeder, Eric Bouffet, Ute Bartels, Michael J. Fisher, Richard Cohn, Sonia Partap, Stewart J. Kellie, Geoffrey McCowage, Arnold C. Paulino, Stefan Rutkowski, Gudrun Fleischhack, Girish Dhall, Laura J. Klesse, Sarah Leary, Javad Nazarian, Marcel Kool, Pieter Wesseling, Marina Ryzhova, Olga Zheludkova, Andrey Golanov, Roger E. McLendon, Roger J. Packer, Christopher Dunham, Juliette Hukin, Maryam Fouladi, Claudia C. Faria, Jose Pimentel, Andrew W. Walter, Nada Jabado, Yoon-Jae Cho, Sebastien Perreault, Sidney E. Croul, Michal Zapotocky, Cynthia Hawkins, Uri Tabori, Michael D. Taylor, Stefan M. Pfister, Paul Klimo, Frederick A. Boop, David W. Ellison, Thomas E. Merchant, Arzu Onar-Thomas, Andrey Korshunov, David T. W. Jones, Amar Gajjar, Vijay Ramaswamy, Paul A. Northcott
Summary: The study investigated clinical outcomes of relapsed medulloblastoma and compared molecular features between patient-matched diagnostic and relapsed tumors. Results showed variations in molecular subgroup performance and survival status at relapse.
JOURNAL OF CLINICAL ONCOLOGY
(2021)
Review
Pathology
Joanna Triscott, Stephen Yip, Donna Johnston, Jean Michaud, Shahrad R. Rassekh, Juliette Hukin, Sandra Dunn, Christopher Dunham
Summary: The study reviewed 120 cases of pediatric medulloblastoma and found that nodularity without desmoplasia and rhythmic palisades were more common in non-WNT/SHH molecular groups, while focal anaplasia was predictive of unfavorable outcomes.
PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
(2021)
Article
Oncology
Rebecca Ronsley, Celine D. Hounjet, Sylvia Cheng, Shahrad Rod Rassekh, Walter J. Duncan, Christopher Dunham, Jane Gardiner, Arvindera Ghag, Jeffrey P. Ludemann, David Wensley, Wingfield Rehmus, Michael A. Sargent, Juliette Hukin
Summary: Six NF-1 patients were treated with trametinib, showing either partial response or stability in all cases, with generally well-tolerated adverse effects of grade 1-2 paronychia or dermatitis. Further data and clinical trials are needed to assess the efficacy and durability of this treatment method.
Correction
Biochemistry & Molecular Biology
Laura K. Donovan, Alberto Delaidelli, Sujith K. Joseph, Kevin Bielamowicz, Kristen Fousek, Borja L. Holgado, Alex Manno, Dilakshan Srikanthan, Ahmed Z. Gad, Randy Van Ommeren, David Przelicki, Cory Richman, Vijay Ramaswamy, Craig Daniels, Jonelle G. Pallota, Tajana Douglas, Alyssa C. M. Joynt, Joonas Haapasalo, Carolina Nor, Maria C. Vladoiu, Claudia M. Kuzan-Fischer, Livia Garzia, Stephen C. Mack, Srinidhi Varadharajan, Matthew L. Baker, Liam Hendrikse, Michelle Ly, Kaitlin Kharas, Polina Balin, Xiaochong Wu, Lei Qin, Ning Huang, Ana Guerreiro Stucklin, A. Sorana Morrissy, Florence M. G. Cavalli, Betty Luu, Raul Suarez, Pasqualino De Antonellis, Antony Michealraj, Avesta Rastan, Meenakshi Hegde, Martin Komosa, Olga Sirbu, Sachin A. Kumar, Zied Abdullaev, Claudia C. Faria, Stephen Yip, Juliette Hukin, Uri Tabori, Cynthia Hawkins, Ken Aldape, Mads Daugaard, John M. Maris, Poul H. Sorensen, Nabil Ahmed, Michael D. Taylor
Article
Oncology
Richard T. Graham, Mohammad H. Abu-Arja, Joseph R. Stanek, Andrea Cappellano, Christina Coleman, Susan Chi, Tabitha Cooney, Girish Dhall, Jacob G. Ellen, Jonathan L. Finlay, Michael J. Fisher, Gregory K. Friedman, Amar Gajjar, Karen Gauvain, Lindsey M. Hoffman, Juliette Hukin, John T. Lucas, Sabine Mueller, Pournima Navalkele, Rebecca Ronsley, Christopher Tinkle, Stephanie Villeneuve, Kee Kiat Yeo, Jack M. Su, Ashley Margol, Nicholas G. Gottardo, Jeffrey Allen, Roger Packer, Ute Bartels, Mohamed S. Abdelbaki
Summary: Patients with basal ganglia/thalamus germinomas have excellent survival outcomes, with combined chemotherapy and radiotherapy showing superior efficacy compared to chemotherapy alone. Radiotherapy is proven to be an integral component in the treatment plan for these patients.
PEDIATRIC BLOOD & CANCER
(2021)
Review
Clinical Neurology
Stephano J. Chang, Ruth Mitchell, Juliette Hukin, Ash Singhal
Summary: Holmes tremor (HT) is a rare and difficult-to-treat movement disorder associated with hydrocephalus. Treatment options include CSF diversion, antiparkinsonian agents, antiepileptic agents, deep brain stimulation, and MR-guided focused ultrasound.
JOURNAL OF NEUROSURGERY-PEDIATRICS
(2022)
Article
Clinical Neurology
Martin Hasselblatt, Christian Thomas, Aniello Federico, Susanne Bens, Mats Hellstrom, Olivera Casar-Borota, Uwe Kordes, Julia E. Neumann, Matthias Dottermusch, Fausto J. Rodriguez, Andrea C. Lo, Sylvia Cheng, Glenda Hendson, Juliette Hukin, Christian Hartmann, Arend Koch, David Capper, Reiner Siebert, Werner Paulus, Karolina Nemes, Pascal D. Johann, Michael C. Fruehwald, Marcel Kool
Summary: Low-grade diffusely infiltrative tumour (LGDIT), SMARCB1-mutant, is a distinctive low-grade lesion found in older children and young adults, showing epigenetic similarity with ATRT-MYC and having the potential for malignant progression.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2022)
Article
Cell Biology
Pooja Panwalkar, Benita Tamrazi, Derek Dang, Chan Chung, Stefan Sweha, Siva Kumar Natarajan, Matthew Pun, Jill Bayliss, Martin P. Ogrodzinski, Drew Pratt, Brendan Mullan, Debra Hawes, Fusheng Yang, Chao Lu, Benjamin R. Sabari, Abhinav Achreja, Jin Heon, Olamide Animasahun, Marcin Cieslik, Christopher Dunham, Stephen Yip, Juliette Hukin, Joanna J. Phillips, Miriam Bornhorst, Andrea M. Griesinger, Andrew M. Donson, Nicholas K. Foreman, Hugh J. L. Garton, Jason Heth, Karin Muraszko, Javad Nazarian, Carl Koschmann, Li Jiang, Mariella G. Filbin, Deepak Nagrath, Marcel Kool, Andrey Korshunov, Stefan M. Pfister, Richard J. Gilbertson, C. David Allis, Arul M. Chinnaiyan, Sophia Y. Lunt, Stefan Bluml, Alexander R. Judkins, Sriram Venneti
Summary: Childhood posterior fossa group A ependymomas (PFAs) have limited treatment options and poor prognoses compared to group B ependymomas (PFBs). PFAs exhibit enhanced glycolysis and tricarboxylic acid (TCA) cycle metabolism, with high expression of glycolytic genes associated with a poor outcome. These tumors also show high EZHIP expression linked to a dismal prognosis and elevated activating mark histone H3 lysine 27 acetylation (H3K27ac). Repurposing the antidiabetic drug metformin has shown therapeutic efficacy in vitro and in vivo in patient-derived PFA xenografts by targeting integrated metabolic/epigenetic pathways.
SCIENCE TRANSLATIONAL MEDICINE
(2021)
Article
Oncology
Craig Erker, Martin Mynarek, Simon Bailey, Claire M. Mazewski, Lorena Baroni, Maura Massimino, Juliette Hukin, Dolly Aguilera, Andrea M. Cappellano, Vijay Ramaswamy, Alvaro Lassaletta, Sebastien Perreault, Cassie N. Kline, Revathi Rajagopal, George Michaiel, Michal Zapotocky, Vicente Santa-Maria Lopez, Andres Morales La Madrid, Chantel Cacciotti, Eric S. Sandler, Lindsey M. Hoffman, Darren Klawinski, Sara Khan, Ralph Salloum, Anna L. Hoppmann, Valerie Larouche, Kathleen Dorris, Helen Toledano, Stephen W. Gilheeney, Mohamed S. Abdelbaki, Beverly Wilson, Derek S. Tsang, Jeffrey Knipstein, Michal Yalon Oren, Shafqat Shah, Jeffrey C. Murray, Kevin F. Ginn, Zhihong J. Wang, Gudrun Fleischhack, Denise Obrecht, Svenja Tonn, Virginia L. Harrod, Kara Matheson, Bruce Crooks, Douglas R. Strother, Kenneth J. Cohen, Jordan R. Hansford, Sabine Mueller, Ashley Margol, Amar Gajjar, Girish Dhall, Jonathan L. Finlay, Paul A. Northcott, Stefan Rutkowski, Steven C. Clifford, Giles Robinson, Eric Bouffet, Lucie Lafay-Cousin
Summary: The purpose of this study was to assess salvage strategies and prognostic features of patients with infant and young childhood medulloblastoma (iMB) who relapse after craniospinal irradiation (CSI)-sparing therapy. The results showed that the 3-year postrelapse survival rate for patients treated with curative intent was 52.4%. Salvage radiotherapy and chemotherapy might be beneficial for a subset of patients. Therefore, CSI-sparing therapy can be effective in a substantial proportion of relapsed iMB patients.
JOURNAL OF CLINICAL ONCOLOGY
(2023)