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cerebellar mutism

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https://www.readbyqxmd.com/read/29675068/cerebellar-mutism-syndrome-following-midline-posterior-fossa-tumor-resection-in-children-an-institutional-experience
#1
Nand Kishore Gora, Ashok Gupta, Virendra Deo Sinha
Aim: Cerebellar mutism (CM) syndrome is a well-known and annoying complication of posterior fossa surgery in the pediatric age group. Risk factors such as the type of tumor, size, involvement of posterior fossa structures and hydrocephalus, and postoperative cerebellar swelling for CM were investigated in this study. Materials and Methods: A consecutive series of 33 children with midline posterior fossa tumors were operated at the SMS Medical College and Hospital, Department of Neurosurgery, Jaipur India, between September 2015 and December 2016...
October 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29509064/a-chinese-patient-of-p102l-gerstmann-str%C3%A3-ussler-scheinker-disease-contains-three-other-disease-associated-mutations-in-syne1
#2
Jing Wang, Kang Xiao, Wei Zhou, Chen Gao, Cao Chen, Qi Shi, Xiao-Ping Dong
Gerstmann-Sträussler-Scheinker disease (GSS) with the P102L mutation in PRNP gene is characterized with progressive cerebellar dysfunction clinically and PrPSc plaques neurologically. Due to the cerebellar ataxia in the early stage, GSS P102L is often misdiagnosed as other neurodegenerative disorders. We presented here a 49-year-old female patient with proven P102L PRNP mutation, and three heterologous mutations in hereditary ataxias associated gene SYNE1, including p.V3643L, p.M3376V and p.T2860A. The patient appeared progressive unsteady gait in early stage and developed the Creutzfeldt-Jacob disease (CJD) - associated clinical manifestations, including progressive dementia, myoclonus, pyramidal and extrapyramidal signs...
March 6, 2018: Prion
https://www.readbyqxmd.com/read/29433337/development-of-a-pre-operative-scoring-system-for-predicting-risk-of-post-operative-paediatric-cerebellar-mutism-syndrome
#3
Jo-Fen Liu, Robert A Dineen, Shivaram Avula, Tom Chambers, Manali Dutta, Tim Jaspan, Donald C MacArthur, Simon Howarth, Daniele Soria, Philip Quinlan, Srikrishna Harave, Chan Chang Ong, Conor L Mallucci, Ram Kumar, Barry Pizer, David A Walker
BACKGROUND: Despite previous identification of pre-operative clinical and radiological predictors of post-operative paediatric cerebellar mutism syndrome (CMS), a unifying pre-operative risk stratification model for use during surgical consent is currently lacking. The aim of the project is to develop a simple imaging-based pre-operative risk scoring scheme to stratify patients in terms of post-operative CMS risk. METHODS: Pre-operative radiological features were recorded for a retrospectively assembled cohort of 89 posterior fossa tumour patients from two major UK treatment centers (age 2-23yrs; gender 28 M, 61 F; diagnosis: 38 pilocytic astrocytoma, 32 medulloblastoma, 12 ependymoma, 1 high grade glioma, 1 pilomyxoid astrocytoma, 1 atypical teratoid rhabdoid tumour, 1 hemangioma, 1 neurilemmoma, 2 oligodendroglioma)...
February 12, 2018: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29354585/neuroanatomical-mechanism-of-cerebellar-mutism-after-stroke
#4
Sekwang Lee, Yoon Hye Na, Hyun Im Moon, Woo Suk Tae, Sung-Bom Pyun
Cerebellar mutism (CM) is a rare neurological condition characterized by lack of speech due to cerebellar lesions. CM is often reported in children. We describe a rare case of CM after spontaneous cerebellar hemorrhage. The patient showed mutism, irritability, decreased spontaneous movements and oropharyngeal apraxia. Diffusion tensor imaging revealed significant volume reduction of medial frontal projection fibers from the corpus callosum. In Tracts Constrained by UnderLying Anatomy (TRACULA) analysis, forceps major and minor and bilateral cingulum-angular bundles were not visualized...
December 2017: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/29336639/intellectual-educational-and-situation-based-social-outcome-in-adult-survivors-of-childhood-medulloblastoma
#5
Virginie Kieffer, Mathilde P Chevignard, Georges Dellatolas, Stephanie Puget, Frederic Dhermain, Jacques Grill, Dominique Valteau-Couanet, Christelle Dufour
PURPOSE: To investigate intellectual and situation-based social outcome and educational achievement in adult survivors of childhood medulloblastoma and analyse factors influencing outcome Methods: We collected demographic, medical and cognitive data, and social and educational outcome at a mean time since the end of treatments of 14.9 years in 58 adults, aged 19-35 years, consecutively treated in a single cancer center between 1989 and 2005. RESULTS: Ten survivors had severe intellectual disability, 12 were still studying, 23 had a regular employment and 13 were unemployed...
January 16, 2018: Developmental Neurorehabilitation
https://www.readbyqxmd.com/read/29310343/cerebrospinal-fluid-real-time-quaking-induced-conversion-test-for-sporadic-creutzfeldt-jakob-disease-in-an-18-year-old-woman-a-case-report
#6
Yuan Yao, Xiaoping Dong, Hongzhi Guan, Qiang Lu
RATIONALE: Sporadic Creutzfeldt-Jakob disease (sCJD) mainly occurs in the elderly, with the peak age of onset ranging from 55 to 75 years. The symptoms of sCJD are not unique, and laboratory tests such as magnetic resonance imaging (MRI), electroencephalogram (EEG) and cerebrospinal fluid (CSF)14-3-3 protein have low sensitivity or specificity. Therefore, excluding treatable diseases and establishing a diagnosis could be difficult in young patients with suspected sCJD. Recently, real-time quaking-induced conversion (RT-QuIC) has been used in the diagnosis of sCJD, with more than 95% sensitivity and 100% specificity...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29279964/hypertrophic-olivary-degeneration-in-children-after-posterior-fossa-surgery-an-underdiagnosed-condition
#7
Matheus Fernando Manzolli Ballestero, Dinark Conceição Viana, Thiago Lyrio Teixeira, Marcelo Volpon Santos, Ricardo Santos de Oliveira
BACKGROUND: Hypertrophic olivary degeneration (HOD) is a rare transsynaptic form of degeneration occurring after injury to the dentato-rubro-olivary pathway ("Guillain-Mollaret triangle"). The majority of studies have described HOD resulting from posterior fossa (PF) hemorrhage or infarction. HOD in patients undergoing PF surgery has not been well characterized. These lesions are rare and symptomatic children with HOD are even more uncommon. The purpose of this study was to evaluate HOD that develops after PF operations in children...
March 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29180084/cerebellar-mutism-syndrome-and-other-complications-after-surgery-in-the-posterior-fossa-in-adults-a-prospective-study
#8
Morten Wibroe, Per Rochat, Marianne Juhler
BACKGROUND: Cerebellar mutism syndrome (CMS) is rarely described in adults; however, data on self-assessed linguistic complications after posterior fossa surgery do not exist. METHODS: Through a prospective single-center study, data on 59 tumor operations in the posterior fossa were collected preoperatively as well as 1 week and 1 month postoperatively. Data on self-assessed problems in 5 CMS-related domains, CMS scores, and neurology as well as surgical procedure and complications were obtained...
February 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29067507/non-surgical-transient-cerebellar-mutism-case-report-and-systematic-review
#9
Serge Makarenko, Navneet Singh, Patrick J McDonald
INTRODUCTION: Transient cerebellar mutism has been well recognized in literature as a complication of posterior fossa tumor resection. It is marked by profound impairment of fluency, articulation, and modulation of speech, irritability and autistic features and typically resolves within days to months. Underlying pathophysiology is debated, but currently unknown. METHODS: We present a case of a child with similar clinical findings after cerebellitis, demonstration of diffuse cerebellar signal changes, swelling, and protruding tonsils at the level of foramen magnum...
October 24, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28987056/the-role-of-the-cerebellum-in-the-regulation-of-language-functions
#10
REVIEW
Anna Starowicz-Filip, Adrian Andrzej Chrobak, Marek Moskała, Roger M Krzyżewski, Borys Kwinta, Stanisław Kwiatkowski, Olga Milczarek, Anna Rajtar-Zembaty, Dorota Przewoźnik
The present paper is a review of studies on the role of the cerebellum in the regulation of language functions. This brain structure until recently associated chiefly with motor skills, visual-motor coordination and balance, proves to be significant also for cognitive functioning. With regard to language functions, studies show that the cerebellum determines verbal fluency (both semantic and formal) expressive and receptive grammar processing, the ability to identify and correct language mistakes, and writing skills...
August 29, 2017: Psychiatria Polska
https://www.readbyqxmd.com/read/28637445/cerebellar-mutism-syndrome-in-children-with-brain-tumours-of-the-posterior-fossa
#11
MULTICENTER STUDY
Morten Wibroe, Johan Cappelen, Charlotte Castor, Niels Clausen, Pernilla Grillner, Thora Gudrunardottir, Ramneek Gupta, Bengt Gustavsson, Mats Heyman, Stefan Holm, Atte Karppinen, Camilla Klausen, Tuula Lönnqvist, René Mathiasen, Pelle Nilsson, Karsten Nysom, Karin Persson, Olof Rask, Kjeld Schmiegelow, Astrid Sehested, Harald Thomassen, Ingrid Tonning-Olsson, Barbara Zetterqvist, Marianne Juhler
BACKGROUND: Central nervous system tumours constitute 25% of all childhood cancers; more than half are located in the posterior fossa and surgery is usually part of therapy. One of the most disabling late effects of posterior fossa tumour surgery is the cerebellar mutism syndrome (CMS) which has been reported in up to 39% of the patients but the exact incidence is uncertain since milder cases may be unrecognized. Recovery is usually incomplete. Reported risk factors are tumour type, midline location and brainstem involvement, but the exact aetiology, surgical and other risk factors, the clinical course and strategies for prevention and treatment are yet to be determined...
June 21, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28527006/analysis-of-surgical-and-mri-factors-associated-with-cerebellar-mutism
#12
Anjali Sergeant, Michelle Masayo Kameda-Smith, Branavan Manoranjan, Brij Karmur, JoAnn Duckworth, Tina Petrelli, Katey Savage, Olufemi Ajani, Blake Yarascavitch, M Constantine Samaan, Katrin Scheinemann, Cheryl Alyman, Saleh Almenawer, Forough Farrokhyar, Adam J Fleming, Sheila Kumari Singh, Nina Stein
The surgical risk factors and neuro-imaging characteristics associated with cerebellar mutism (CM) remain unclear and require further investigation. Therefore, we aimed to examine surgical and MRI findings associated with CM in children following posterior fossa tumor resection. Using our data registry, we retrospectively collected data from pediatric patients who acquired CM and were matched based on age and pathology type with individuals who did not acquire CM after posterior fossa surgery. The strength of association between surgical and MRI variables and CM were examined using odds ratios (ORs) and corresponding 95% confidence intervals (CIs)...
July 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28498095/risk-factors-for-development-of-postoperative-cerebellar-mutism-syndrome-in-children-after-medulloblastoma-surgery
#13
San Y C V Pols, Marie Lise C van Veelen, Femke K Aarsen, Antonia Gonzalez Candel, Coriene E Catsman-Berrevoets
OBJECTIVE Postoperative cerebellar mutism syndrome (pCMS) occurs in 7%-50% of children after cerebellar tumor surgery. Typical features include a latent onset of 1-2 days after surgery, transient mutism, emotional lability, and a wide variety of motor and neurobehavioral abnormalities. Sequelae of this syndrome usually persist long term. The principal causal factor is bilateral surgical damage (regardless of tumor location) to any component of the proximal efferent cerebellar pathway, which leads to temporary dysfunction of cerebral cortical regions as a result of diaschisis...
July 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28497710/the-complex-diagnostic-challenge-in-children-with-non-central-nervous-system-cancer-and-cerebellar-mutism
#14
Kathleen Helton, Amy L Patterson, Raja B Khan, Zsila Sadighi
Multiple etiologies should be considered in the differential diagnosis of immunocompromised patients with non-central nervous system cancer and viral infections who develop mutism. Acute cerebellitis, caused by infections or by neurotoxicity resulting from chemotherapy; paraneoplastic cerebellar degeneration; atypical posterior reversible encephalopathy syndrome; and acute disseminated encephalomyelitis may all cause mutism in such patients. This condition warrants prompt recognition and may require treatment with immunotherapy, as it may be an immune-mediated process...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28490165/remote-cerebellar-hemorrhage-presenting-with-cerebellar-mutism-after-spinal-surgery-an-unusual-case-report
#15
Halil Murat Sen, Mustafa Guven, Adem Bozkurt Aras, Murat Cosar
Dural injury during spinal surgery can subsequently give rise to a remote cerebellar hemorrhage (RCH). Although the incidence of such injury is low, the resulting hemorrhage can be life threatening. The mechanism underlying the formation of the hemorrhage is not known, but it is mostly thought to develop after venous infarction. Cerebellar mutism (CM) is a frequent complication of posterior fossa operations in children, but it is rarely seen in adults. The development of CM after an RCH has not been described...
May 2017: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/28402042/mm1-type-sporadic-creutzfeldt-jakob-disease-with-1-month-total-disease-duration-and-early-pathologic-indicators
#16
Yasushi Iwasaki, Hiroko Kato, Tetsuo Ando, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida
A 62-year-old man presented with abnormal behavior and cognitive impairment. Diffusion-weighted images (DWI) obtained on MRI showed extensive hyperintense regions in the cerebral cortex and striatum. Myoclonus was recognized, and the patient died 1 month after the onset; his condition did not reach the akinetic mutism state. The brain weighed 1300 g and showed no apparent atrophy. Extensive spongiform changes were observed in the cerebral neocortex, striatum, thalamus and cerebellar cortex, but gliosis was mild or absent...
October 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28175427/126%C3%A2-anatomy-of-cerebellar-mutism-reduced-fractional-anisotropy-in-the-superior-cerebellar-peduncle
#17
Sean D McEvoy, Amy Lee, Jeffrey G Ojemann, Christine MacDonald
No abstract text is available yet for this article.
August 1, 2016: Neurosurgery
https://www.readbyqxmd.com/read/28118304/cerebellar-mutism-syndrome-cause-and-rehabilitation
#18
Coriene E Catsman-Berrevoets
PURPOSE OF REVIEW: Mutism of cerebellar origin may occur in the context of various causes but is most frequent in children after resection of a large midline cerebellar tumour. In this review, the endeavour to reach a consensus on name and definition of postoperative mutism of cerebellar origin and associated symptoms is highlighted. In addition, progress in understanding of cause and risk factors for the syndrome is discussed as well as the rehabilitation issues. RECENT FINDINGS: Consensus on the term cerebellar mutism syndrome (CMS) has been reached...
April 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28118217/postoperative-pediatric-cerebellar-mutism-after-posterior-fossa-surgery-a-case-report
#19
Jerry Y Chao, Che Liu, Naveen Shetty, Ushma Shah
Cerebellar mutism syndrome (CMS) is a common complication of posterior fossa surgery that can confound the postanesthetic examination and have long-lasting impacts. There is confusion surrounding its precise description, diagnostic features, and associated morbidity. Here, we discuss the most up-to-date knowledge of CMS drawing from a clinical case in the context of 3 new reports: (1) an international consensus paper presenting a new proposed working definition by the Iceland Delphi Group, (2) a knowledge update by Gadgil et al, (3) and a review of neuroimaging-based data elucidating the etiology of CMS by Patay...
April 15, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28074148/apraxia-of-speech-and-cerebellar-mutism-syndrome-a-case-report
#20
E De Witte, I Wilssens, D De Surgeloose, G Dua, M Moens, J Verhoeven, M Manto, P Mariën
BACKGROUND: Cerebellar mutism syndrome (CMS) or posterior fossa syndrome (PFS) consists of a constellation of neuropsychiatric, neuropsychological and neurogenic speech and language deficits. It is most commonly observed in children after posterior fossa tumor surgery. The most prominent feature of CMS is mutism, which generally starts after a few days after the operation, has a limited duration and is typically followed by motor speech deficits. However, the core speech disorder subserving CMS is still unclear...
2017: Cerebellum & Ataxias
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