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cerebellar mutism

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...
April 12, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Sean D McEvoy, Amy Lee, Jeffrey G Ojemann, Christine MacDonald
No abstract text is available yet for this article.
August 1, 2016: Neurosurgery
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
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
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
Francesco Nicita, Milena Paiano, Mauro Liberatore, Alberto Spalice, Paola Papoff, Mariacristina Ullo, Manolo Piccirilli, Anna Clerico, Amalia Schiavetti
Post-operative pediatric cerebellar mutism syndrome (PPCMS) is a clinical syndrome arising from cerebellar injury and characterized by absence of speech and other possible symptoms and signs. Rare reports described some benefit after administration of dopamine agonist therapy, but no treatment has proven efficacy. In this paper, we report on the dramatic, sudden resolution of PPCMS induced by midazolam administration in a boy who underwent posterior fossa surgery for choroid plexus papilloma of the fourth ventricle...
March 2017: Acta Neurochirurgica
D Barragan-Martinez, N Nunez-Enamorado, M Berenguer-Potenciano, N Villora-Morcillo, A Martinez de Aragon, A Camacho-Salas
INTRODUCTION: Acute cerebellitis is one of the main causes of cerebellar syndrome in infancy. Among the wide range of manifestations, headache and ataxia being the most predominant, we can find other less frequent, although nonetheless interesting, ones, such as language disorders, which go beyond the well-known cerebellar dysarthria. The different combinations in which the symptoms can appear, especially when not accompanied by ataxia, make the condition a real challenge for the clinician...
January 1, 2017: Revista de Neurologia
Salima S Wahab, Samantha Hettige, Kshtij Mankad, Kristian Aquilina
Posterior fossa syndrome (PFS), or cerebellar mutism syndrome (CMS), is a collection of neurological symptoms that occur following surgical resection of a posterior fossa tumour, and is characterised by either a reduction or an absence of speech. Some authors suggest that CM is only one symptom of the CMS complex that also includes ataxia, hypotonia and irritability as well as cranial nerve deficits, neurobehavioral changes and urinary retention or incontinence. It is seen almost exclusively in children. In 1985 Rekate et al...
October 2016: Quantitative Imaging in Medicine and Surgery
Shivaram Avula, Michaela Spiteri, Ram Kumar, Emma Lewis, Srikrishna Harave, David Windridge, Chan Ong, Barry Pizer
BACKGROUND: The dentato-thalamo-cortical (DTC) pathway is recognized as the anatomical substrate for postoperative pediatric cerebellar mutism (POPCMS), a well-recognized complication affecting up to 31% of children undergoing posterior fossa brain tumour resection. The proximal structures of the DTC pathway also form a segment of the Guillain and Mollaret triangle, a neural network which when disrupted causes hypertrophic olivary degeneration (HOD) of the inferior olivary nucleus (ION)...
October 2016: Quantitative Imaging in Medicine and Surgery
Myung Eun Oh, Pablo Hernáiz Driever, Rajiv K Khajuria, Stefan Mark Rueckriegel, Elisabeth Koustenis, Harald Bruhn, Ulrich-Wilhelm Thomale
Pediatric posterior fossa (PF) tumor survivors experience long-term motor deficits. Specific cerebrocerebellar connections may be involved in incidence and severity of motor dysfunction. We examined the relationship between long-term ataxia as well as fine motor function and alteration of differential cerebellar efferent and afferent pathways using diffusion tensor imaging (DTI) and tractography. DTI-based tractography was performed in 19 patients (10 pilocytic astrocytoma (PA) and 9 medulloblastoma patients (MB)) and 20 healthy peers...
October 26, 2016: Journal of Neuro-oncology
Ethan A Winkler, Harjus Birk, Michael Safaee, John K Yue, John F Burke, Jennifer A Viner, Melike Pekmezci, Arie Perry, Manish K Aghi, Mitchel S Berger, Michael W McDermott
Ependymomas are rare neuroepithelial tumors which may arise anywhere along the ventricular system. Tumors arising in the fourth ventricle present unique challenges. Complete tumor resection favors prolonged survival, but may result in inadvertent injury of surrounding neural structures-such as cranial nerve (CN) nuclei. Here, our institutional experience with surgical resection of fourth ventricular ependymomas is described. A single institution, retrospective analysis of consecutive case series of adult surgically resected fourth ventricular ependymomas with the bilateral telovelar approach...
November 2016: Journal of Neuro-oncology
Iska Moxon-Emre, Michael D Taylor, Eric Bouffet, Kristina Hardy, Cynthia J Campen, David Malkin, Cynthia Hawkins, Normand Laperriere, Vijay Ramaswamy, Ute Bartels, Nadia Scantlebury, Laura Janzen, Nicole Law, Karin S Walsh, Donald J Mabbott
Purpose To evaluate intellectual functioning and the implications of limiting radiation exposure in the four biologically distinct subgroups of medulloblastoma: wingless (WNT), sonic hedgehog (SHH), Group 3, and Group 4. Patients and Methods A total of 121 patients with medulloblastoma (n = 51, Group 4; n = 25, Group 3; n = 28, SHH; and n = 17, WNT), who were treated between 1991 and 2013 at the Hospital for Sick Children (Toronto, Ontario, Canada), Children's National Health System (Washington, DC), or the Lucile Packard Children's Hospital (Palo Alto, CA), had intellectual assessments...
December 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Sean D McEvoy, Amy Lee, Jeffrey G Ojemann, Christine MacDonald
INTRODUCTION: Cerebellar mutism is a common finding after resection of cerebellar tumors, especially in children. It has been reported to occur in up to 39% of posterior fossa tumor resections. Once thought to be a transient finding, a growing body of evidence has associated it with long-term neurological sequelae. The pathophysiology and anatomy of this phenomenon remains controversial. METHODS: All patients with cerebellar tumors undergoing resection at Seattle Children's Hospital from June 2010 to June 2015 were retrospectively reviewed...
August 2016: Neurosurgery
E Nakatani, Y Kanatani, H Kaneda, Y Nagai, S Teramukai, T Nishimura, B Zhou, S Kojima, H Kono, M Fukushima, T Kitamoto, H Mizusawa
BACKGROUND AND PURPOSE: Akinetic mutism is thought to be an appropriate therapeutic end-point in patients with sporadic Creutzfeldt-Jakob disease (sCJD). However, prognostic factors for akinetic mutism are unclear and clinical signs or symptoms that precede this condition have not been defined. The goal of this study was to identify prognostic factors for akinetic mutism and to clarify the order of clinical sign and symptom development prior to its onset. METHODS: The cumulative incidence of akinetic mutism and other clinical signs and symptoms was estimated based on Japanese CJD surveillance data (455 cases) collected from 2003 to 2008...
September 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Thora Gudrunardottir, Angela T Morgan, Andrew L Lux, David A Walker, Karin S Walsh, Elizabeth M Wells, Jeffrey H Wisoff, Marianne Juhler, Jeremy D Schmahmann, Robert F Keating, Coriene Catsman-Berrevoets
INTRODUCTION: Confusion has surrounded the description of post-operative mutism and associated morbidity in pediatric patients with cerebellar tumors for years. The heterogeneity of definitions and diagnostic features has hampered research progress within the field, and to date, no international guidelines exist on diagnosis, prevention, treatment, or follow-up of this debilitating condition. An international group of clinicians and researchers from multiple relevant disciplines recently formed a cohesive panel to formulate a new working definition and agree upon standardized methods for diagnosis and follow-up...
July 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Yun-Ju Choi, Kyung-Wook Kang, Sae-Young Lee, Seung-Ho Kang, Seung-Han Lee, Byeong C Kim
Sporadic Creutzfeldt-Jakob disease (CJD) is clinically characterized by rapidly progressive dementia combined with other cardinal symptoms, such as myoclonus, visual or cerebellar disturbances, extrapyramidal or pyramidal disturbance, and akinetic mutism. However, as an initial manifestation, focal neurologic deficits other than the aforementioned or nonspecific generalized symptoms may lead to a misdiagnosis or a delayed diagnosis. The authors report a case of 66-year-old male patient with sporadic CJD who had dizziness, gaze-evoked nystagmus (GEN), and other central eye signs (impaired smooth pursuit, saccadic dysmetria) as an initial manifestation without dementia...
February 2016: Medicine (Baltimore)
Yasushi Iwasaki, Keiko Mori, Masumi Ito, Shinsui Tatsumi, Maya Mimuro, Ryozo Kuwano, Masato Hasegawa, Mari Yoshida
A 48-year-old Japanese woman experienced slow-onset parkinsonism and speech disturbances. Neurological examinations revealed rigidity in the trunk and extremities, bradykinesia and postural instability, although cognitive impairments and psychiatric symptoms were not apparent in the early disease stage. Neuroimaging revealed progressive bilateral frontotemporal lobe atrophy with cerebral blood flow hypoperfusion. No apparent signs of lower motor neuron involvement were observed, such as fasciculation or electromyogram findings...
June 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Lain Hermes González Quarante, José Hinojosa Mena-Bernal, Beatriz Pascual Martín, Marta Ramírez Carrasco, María Jesús Muñoz Casado, Ana Martínez de Aragón, Rogelio Simón de las Heras
INTRODUCTION: In 1996, Hinchey and colleagues coined the term "Posterior reversible encephalopathy syndrome" (PRES) to describe a condition seen in patients with acute neurological symptoms and reversible subcortical vasogenic edema predominantly involving parieto-occipital areas demonstrated in brain MRI. The occurrence of this phenomenon after surgical resection of CNS tumors is typically linked to pediatric cases. MATERIAL AND METHODS: Two new cases of PRES after posterior fossa surgery are reported...
May 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Shivaram Avula, Conor Mallucci, Ram Kumar, Barry Pizer
INTRODUCTION: Posterior fossa syndrome (PFS), also known as cerebellar mutism syndrome (CMS), is a severe complication affecting children following surgery for posterior fossa brain tumours. Its incidence varies between 8 and 31 %, and its exact pathogenesis remains unclear. In this article, we aim to review the existing theories on its pathogenesis and propose a new hypothesis. DISCUSSION: There is varying level of evidence on existing theories on the pathogenesis of PFS following surgery...
October 2015: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
G Tamburrini, P Frassanito, D Chieffo, L Massimi, M Caldarelli, C Di Rocco
INTRODUCTION: Cerebellar mutism (CM) is defined as a peculiar form of mutism that may complicate the surgical excision of posterior cranial fossa tumor. The incidence is variable in the literature, occurring in up to one third of cases in some series. Commonly occurring peculiar features of CM are delayed onset following surgery, limited duration, and spontaneous recovery usually associated with dysarthria. METHODS: A review has been performed concerning anatomical substrates and circuits actually considered to be involved in the development of cerebellar mutism, as well as risk factors for its development that have been documented in the literature...
October 2015: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
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