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cerebellar mutism

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...
August 9, 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...
November 27, 2015: 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
Dillon Y Chen, Paula Aristizabal, John R Crawford
No abstract text is available yet for this article.
September 2015: Pediatric Neurology
Zoltan Patay
BACKGROUND: Posterior fossa syndrome (PFS) is a severe, postoperative complication occurring in about 25 % of pediatric patients undergoing surgery for midline tumors in the posterior fossa. In recent years, MRI-based research elucidated the cause of PFS and shed new light on its putative pathophysiology. This review highlights the major advances arising from research of this topic. RESULTS: Postsurgical damage patterns in patients after posterior fossa surgery show that PFS results from bilateral surgical damage to the proximal efferent cerebellar pathways (pECPs)...
October 2015: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
C Vigneron, N Entz-Werlé, P Lutz, A Spiegel, S Jannier, S Helfre, C Alapetite, A Coca, P Kehrli, G Noël
Medulloblastoma are cerebellar tumours belonging to the group of primitive neuroectodermal tumours (PNET) and are the most common malignant brain tumours of childhood. These tumours are rare and heterogeneous, requiring some multicentric prospective studies and multidisciplinary care. The classical therapeutic approaches are based on clinical, radiological and surgical data. They involve surgery, radiation therapy and chemotherapy. Some histological features were added to characterize risk. More recently, molecular knowledge has allowed to devise risk-adapted strategies and helped to define groups with good outcome and reduce long-term sequelae, improve the prognostic of high-risk medulloblastoma and develop new therapeutic tools...
August 2015: Cancer Radiothérapie: Journal de la Société Française de Radiothérapie Oncologique
Francesco Tomasello, Alfredo Conti, Salvatore Cardali, Domenico La Torre, Filippo Flavio Angileri
OBJECTIVE: Fourth ventricle tumors have been traditionally approached by opening the cerebellar vermis. The "telovelar" approach is an alternative approach performed through the cerebellomedullary fissure to gain access to the fourth ventricle, avoiding neural tissue damage. We describe our experience with this approach and predictive factors for the extent of resection (EOR) and for outcomes. METHODS: We retrospectively analyzed the data of patients who underwent resection of fourth ventricle lesions using a bilateral telovelar approach between June 1998 and June 2013...
June 2015: World Neurosurgery
Rajeev Kariyattil, Mohamed I A Rahim, Unnikrishnan Muthukuttiparambil
Cerebellar mutism is a rare occurrence following paediatric trauma. Although it is quite common after posterior fossa surgery in children, this phenomenon has rarely been reported following other insults, such as trauma, and its pathophysiology remains poorly understood. We report a seven-year-old child who presented to the casualty department of Sultan Qaboos University Hospital in Muscat, Oman, in May 2013 with a traumatic right cerebellar contusion. The child presented with clinical features of cerebellar mutism but underwent a rapid and spontaneous recovery...
February 2015: Sultan Qaboos University Medical Journal
Francesco Tomasello, Alfredo Conti, Filippo F Angileri, Salvatore Cardali
BACKGROUND: The "telo-velar" approach is an alternative to cerebellar splitting to gain access to the fourth ventricle through the so-called cerebello-medullary fissure (CMF). METHOD: In this approach, the CMF is exposed and access to the ventricle is obtained by incising the tela chorioidea and inferior medullary velum. This approach enables the exploration of the entire ventricle cavity from the obex to the aqueduct. CONCLUSIONS: The exposure of the fourth ventricle is satisfactory and the floor of the fourth ventricle can be visualised early and protected...
April 2015: Acta Neurochirurgica
David Walker, S A Thomas, E J Talbot, E J Bennett, A Starza-Smith, Stephanie L Da Silva
Cerebellar Mutism Syndrome (CMS), also known as posterior fossa syndrome (CMS/PFS), refers to a transient loss of speech followed by dysarthria and is a common complication in the pediatric population following resection of a cerebellar tumor. Recognition and rehabilitation of CMS is critical in the post-operative phases of recovery. Two case studies, including neuropsychological assessments, along with the family and patient's perspectives are reviewed.
2014: Journal of Pediatric Rehabilitation Medicine
N Ait Khelifa-Gallois, F Laroussinie, S Puget, C Sainte-Rose, G Dellatolas
UNLABELLED: Abstract Purpose: A number of studies report neurological and cognitive deficits and behavioural disorders in children after surgical treatment for a benign cerebellar tumour. The present study explores functional outcome in adolescents and adults treated for a low-grade cerebellar astrocytoma in childhood. MATERIALS AND METHODS: Participants were 18 adolescents and 46 adults treated for low-grade astrocytoma in childhood. Academic achievement, professional status and neurological, cognitive and behavioural disturbances were collected using self-completed and parental questionnaires for adolescents and phone interview for adults...
2015: Brain Injury: [BI]
Yasushi Iwasaki, Keiko Mori, Masumi Ito, Kazuya Nokura, Shinsui Tatsumi, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida
We describe an autopsied case of a Japanese woman with Gerstmann-Straeussler-Scheinker disease (GSS) presenting with a rapidly progressive clinical course. Disease onset occurred at the age of 54 with dementia and gait disturbance. Her clinical course progressively deteriorated until she reached a bedridden state with myoclonus 9 months after onset. Two months later, she reached the akinetic mutism state. Nasal tube feeding was introduced at this point and continued for several years. Electroencephalograms showed diffuse slowing without periodic sharp-wave complexes...
September 2014: Clinical Neuropathology
Kirsten Margaretha van Baarsen, Joachim André Grotenhuis
Up to 39% of children operated for a posterior fossa tumor develop the cerebellar mutism syndrome. Although they are alert and cooperative, with normal language comprehension, they are unable to speak. In addition, patients may demonstrate apathy, bladder and bowel incontinence and long-term language and cognitive disturbances. This devastating syndrome is at the same time intriguing, because it confirms a role for the cerebellum in language and cognitive functions. Recent investigations have led to new insights regarding the cerebellar mutism syndrome...
June 2014: Medical Hypotheses
Sarah McAndrew, Robert Listernick, Nancy Kuntz
BACKGROUND: Cerebellar mutism in children occurs after posterior fossa tumor resection and can have lasting effects on cognition, language, and behavior. Cerebellar mutism in acute disseminated encephalomyelitis is rare. PATIENT: A 7-year-old boy with a 3-day history of fever, vomiting, and diarrhea presented with altered mental status and expressive aphasia. Magnetic resonance imaging showed new diffusion restriction in the bilateral dentate nuclei and right cerebellum...
May 2014: Pediatric Neurology
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