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Sample records for cerebellar neoplasms

  1. Cerebellar Mutism

    OpenAIRE

    1994-01-01

    Of a series of 15 children operated for cerebellar tumor at University Hospital Rotterdam-Dijkzigt, The Netherlands, 5 developed “cerebellar mutism” and subsequent dysarthria after surgery, and 2 had mild speech problems.

  2. Cerebellar pleomorphic xanthoastrocytoma in a patient with neurofibromatosis type 1

    Energy Technology Data Exchange (ETDEWEB)

    Naidich, M.J.; Walker, M.T.; Han, G. [Northwestern University Feinberg School of Medicine, Department of Radiology, Chicago, Illinois (United States); Northwestern Memorial Hospital, Chicago, IL (United States); Gottardi-Littell, N.R. [Northwestern Memorial Hospital, Chicago, IL (United States); Northwestern University Feinberg School of Medicine, Department of Pathology, Chicago, Illinois (United States); Chandler, J.P. [Northwestern Memorial Hospital, Chicago, IL (United States); Northwestern University Feinberg School of Medicine, Department of Neurological Surgery, Chicago, Illinois (United States)

    2004-10-01

    We describe a case of cerebellar pleomorphic xanthoastrocytoma (PXA) occurring in a patient with neurofibromatosis type 1 (NF1). The histomorphology of this uncommon glial (astrocytic) neoplasm is discussed. The occurrence of this tumor within the posterior fossa is extremely rare. To our knowledge, this is the first reported case of a cerebellar PXA in a patient with NF1. (orig.)

  3. Acute cerebellar ataxia

    Science.gov (United States)

    Cerebellar ataxia; Ataxia - acute cerebellar; Cerebellitis; Post-varicella acute cerebellar ataxia; PVACA ... Acute cerebellar ataxia in children, especially younger than age 3, may occur several weeks after an illness caused by a virus. ...

  4. Cerebellar liponeurocytoma: a case-report

    Directory of Open Access Journals (Sweden)

    K.V. Sreedhar Babu

    Full Text Available Cerebellar liponeurocytoma is a rare cerebellar neoplasm of adults with advanced neuronal / neurocytic and focal lipomatous differentiation, a low proliferative potential and a favorable clinical prognosis corresponding to World Health Organization grade I or II. Only a few cases have been described in the literature (approximately 20 cases by different names. A 48-years old female, presented with history of headache and dizziness associated with neck pain; restricted neck movements, drop attacks and occasional regurgitation of food since one year. Magnetic resonance imaging disclosed a right cerebellar mass lesion. Gross total resec- tion of the tumour was accomplished through a suboccipital craniotomy. The excised tissue was diagnosed as cerebellar liponeurocytoma, a rare entity, based on histopathological examination and immunohistochemistry. The morphological appearance of this neoplasm can be confused with that of oligodendroglioma, neurocytoma, ependymoma, medulloblastoma, solid hemangioblastoma and metastatic carcinomas etc., with unpredictable prognosis, which require postoperative radiotherapy, hence the importance of accurately diagnosing this rare neoplasm. This tumour should be added to the differential diagnosis of mass lesions of the posterior fossa.

  5. Síndrome de gerstmann de desenvolvimento associada a neoplasia cerebelar: relato de um caso e revisão da literatura Developmental Gerstmann syndrome associated with cerebellar neoplasm: a case report with review of litterature

    Directory of Open Access Journals (Sweden)

    Lineu César Werneck

    1975-03-01

    Full Text Available É relatado um caso de síndrome de Gerstmann de Desenvolvimento, que apresentava acalculia, agrafía, confusão direita-esquerda, agnosia de dedos e apraxia construcional, em um menino de 7 anos. O tratamento inicial com metilfenidato determinou bons resultados no que concerne à hiperatividade, à atenção e à apraxia construcional. Durante o curso clínico, o paciente apresentou sintomatologia cerebelar, hipertensão intracraniana, tendo sido feita intervenção cirúrgica na fossa posterior, sendo excisado um meduloblastoma de cerebelo. O autor acredita que é esta a primeira descrição na literatura da concomitância da síndrome de Gerstmann de Desenvolvimento e neoplasia de fossa posterior. São feitos comentários focalizando cada um dos aspectos clínicos, fatores etiológicos e topográficos.A Developmental Gerstmann syndrome in a 7 years-old-boy with hyperactivity, short attention span, acalculia, agraphia, right-left confusion, finger agnosia and constructional apraxia is reported. An initial trial with methylphenidate was done with good improvement regarding hyperactivity, attention span and constructional apraxia. In the clinical course developed a cerebellar syndrome and intracranial hypertension. Surgical exploration of the cerebellum discovered a medulloblastoma. The author believes that this is the first described association of Developmental Gerstmann syndrome and cerebellar neoplasia. The clinical findings, the etiology and the topography of the Gerstmann syndrome are discussed.

  6. Congenital Cerebellar Mixed Germ Cell Tumor Presenting with Hemorrhage in a Newborn

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Sung Mok; Kim, Ji Hye; Yoo, So Young; Park, Won Soon; Jang, Yun Sil; Shin, Hyung Jin; Suh, Yeon Lim [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2008-07-15

    We report here on a neonate with congenital cerebellar mixed germ cell tumor, and this initially presented as cerebellar hemorrhage. Postnatal cranial ultrasonography revealed an echogenic cerebellar mass that exhibited the signal characteristics of hemorrhage rather than tumor on MR images. The short-term follow-up images also suggested a resolving cerebellar hemorrhage. One month later, the neonate developed vomiting. A second set of MR images demonstrated an enlarged mass that exhibited changed signal intensity at the same site, which suggested a neoplasm. Histological examination after the surgical resection revealed a mixed germ cell tumor.

  7. Bilateral otogenic cerebellar abscesses.

    Directory of Open Access Journals (Sweden)

    Nadkarni T

    1993-01-01

    Full Text Available An unusual presentation of bilateral otogenic cerebellar abscesses observed in two of our patients is reported. Both gave a history of otorrhoea, fever, headache, vomiting and had bilateral cerebellar signs and conductive hearing loss. The abscesses were detected on computerised tomography. X-rays revealed bilateral mastoiditis. The therapy followed was excision of abscesses, mastoidectomy and antibiotic therapy.

  8. Iatrogenic postoperative cerebellar cyst.

    Science.gov (United States)

    Sharif, Robin; Moscovici, Samuel; Wygoda, Marc; Eliahou, Ruth; Spektor, Sergey

    2016-12-01

    Cerebellar cyst is a known but uncommon entity. It is congenital in most cases, or may develop after brain parenchyma injuries or interventions. To our knowledge, de novo cerebellar cyst after extra-axial tumor excision, has not been described in the literature. We present the first reported case of a de novo cerebellar cyst developing in a 70-year-old woman following retrosigmoid craniotomy for vestibular schwannoma excision, and discuss the possible causes. Following cyst fenestration, there was no clinical or radiological evidence of a residual cyst.

  9. Chronic Myeloproliferative Neoplasms Treatment

    Science.gov (United States)

    ... way to treat some chronic myeloproliferative neoplasms. Platelet apheresis Platelet apheresis is a treatment that uses a special machine ... using interferon alfa or pegylated interferon alpha . Platelet apheresis . A clinical trial of a new treatment. Check ...

  10. Peroxiredoxins in colorectal neoplasms

    OpenAIRE

    Wu, X.Y.; Fu, X.Z.; Wang, X. H.

    2010-01-01

    Peroxiredoxins (Prxs) are novel group proteins with efficient antioxidant capacity, and some of them also have effects on cell proliferation, differentiation, apoptosis, and chemotherapy and radiotherapy resistance. Altogether six distinct Prxs expressions were investigated in histological samples of colorectal neoplasm and the distant normal tissues and investigated associatedly with parameters such as clinical stage and lymphnodes metastasis. Normal colorectal tis...

  11. Treatment Options for Myelodysplastic/Myeloproliferative Neoplasms

    Science.gov (United States)

    ... Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Myelodysplastic/ Myeloproliferative ...

  12. Cerebellar anatomy as applied to cerebellar microsurgical resections

    Directory of Open Access Journals (Sweden)

    Alejandro Ramos

    2012-06-01

    Full Text Available OBJECTIVE: To define the anatomy of dentate nucleus and cerebellar peduncles, demonstrating the surgical application of anatomic landmarks in cerebellar resections. METHODS: Twenty cerebellar hemispheres were studied. RESULTS: The majority of dentate nucleus and cerebellar peduncles had demonstrated constant relationship to other cerebellar structures, which provided landmarks for surgical approaching. The lateral border is separated from the midline by 19.5 mm in both hemispheres. The posterior border of the cortex is separated 23.3 mm from the posterior segment of the dentate nucleus; the lateral one is separated 26 mm from the lateral border of the nucleus; and the posterior segment of the dentate nucleus is separated 25.4 mm from the posterolateral angle formed by the junction of lateral and posterior borders of cerebellar hemisphere. CONCLUSIONS: Microsurgical anatomy has provided important landmarks that could be applied to cerebellar surgical resections.

  13. Cerebellar Hypoplasia and Autism

    OpenAIRE

    1989-01-01

    The size of the cerebellar hemisphere and vermal lobules was measured in ten autistic and eight normal control subjects at the Neuropsychology Research Laboratory, Children’s Hospital Research Center, and the Departments of Neurosciences and Radiology, School of Medicine, University of California at San Diego, LaJolla.

  14. Genetics Home Reference: lissencephaly with cerebellar hypoplasia

    Science.gov (United States)

    ... Conditions lissencephaly with cerebellar hypoplasia lissencephaly with cerebellar hypoplasia Enable Javascript to view the expand/collapse boxes. ... Open All Close All Description Lissencephaly with cerebellar hypoplasia (LCH) affects brain development, resulting in the brain ...

  15. Falls in degenerative cerebellar ataxias

    NARCIS (Netherlands)

    van de Warrenburg, Bart P C; Steijns, Janneke A G; Munneke, Marten; Kremer, Berry P H; Bloem, Bastiaan R

    2005-01-01

    We retrospectively and prospectively assessed the frequency and characteristics of falls in patients with degenerative cerebellar ataxias. The results show that falls occur very frequently in patients with degenerative cerebellar ataxias and that these falls are serious and often lead to injuries or

  16. Obesity and gastrointestinal neoplasms

    Directory of Open Access Journals (Sweden)

    Izabela Binkowska-Borgosz

    2014-10-01

    Full Text Available Being overweight or obese is a significant public health problem in the 21st century due to its scale, common existence and its cause-effect association with multiple diseases. Excessive accumulation of adipose tissue in humans is regarded as a major risk factor for development of cardiovascular and skeletal diseases. However, data from recent years have revealed that obesity is also strongly associated with increased risk of the majority of cancers in humans, including those originating from the gastrointestinal tract. During the last few year this association has been thoroughly proven and supported by several epidemiological analyses. The authors present i the current state of knowledge regarding key (pathomechanisms that link metabolism of human adipose tissue to development/progression of neoplasms (especially in the gastrointestinal tract, as well as ii the results of selected clinical studies in which the influence of obesity on risk of gastrointestinal cancer development has been addressed.

  17. Sleep disorders in cerebellar ataxias

    Directory of Open Access Journals (Sweden)

    José L. Pedroso

    2011-04-01

    Full Text Available Cerebellar ataxias comprise a wide range of etiologies leading to central nervous system-related motor and non-motor symptoms. Recently, a large body of evidence has demonstrated a high frequency of non-motor manifestations in cerebellar ataxias, specially in autosomal dominant spinocerebellar ataxias (SCA. Among these non-motor dysfunctions, sleep disorders have been recognized, although still under or even misdiagnosed. In this review, we highlight the main sleep disorders related to cerebellar ataxias focusing on REM sleep behavior disorder (RBD, restless legs syndrome (RLS, periodic limb movement in sleep (PLMS, excessive daytime sleepiness (EDS, insomnia and sleep apnea.

  18. Complex partial seizures: cerebellar metabolism

    Energy Technology Data Exchange (ETDEWEB)

    Theodore, W.H.; Fishbein, D.; Deitz, M.; Baldwin, P.

    1987-07-01

    We used positron emission tomography (PET) with (/sup 18/F)2-deoxyglucose to study cerebellar glucose metabolism (LCMRglu) and the effect of phenytoin (PHT) in 42 patients with complex partial seizures (CPS), and 12 normal controls. Mean +/- SD patient LCMRglu was 6.9 +/- 1.8 mg glucose/100 g/min (left = right), significantly lower than control values of 8.5 +/- 1.8 (left, p less than 0.006), and 8.3 +/- 1.6 (right, p less than 0.02). Only four patients had cerebellar atrophy on CT/MRI; cerebellar LCMRglu in these was 5.5 +/- 1.5 (p = 0.054 vs. total patient sample). Patients with unilateral temporal hypometabolism or EEG foci did not have lateralized cerebellar hypometabolism. Patients receiving phenytoin (PHT) at the time of scan and patients with less than 5 years total PHT exposure had lower LCMRglu, but the differences were not significant. There were weak inverse correlations between PHT level and cerebellar LCMRglu in patients receiving PHT (r = -0.36; 0.05 less than p less than 0.1), as well as between length of illness and LCMRglu (r = -0.22; 0.05 less than p less than 0.1). Patients with complex partial seizures have cerebellar hypometabolism that is bilateral and due only in part to the effect of PHT.

  19. Acute post-infectious cerebellar ataxia due to co-infection of human herpesvirus-6 and adenovirus mimicking myositis.

    Science.gov (United States)

    Naselli, Aldo; Pala, Giovanna; Cresta, Federico; Finetti, Martina; Biancheri, Roberta; Renna, Salvatore

    2014-11-26

    Acute cerebellar ataxia (ACA) is a relatively common neurological disease in children. Most common types of ACA are acute post-infectious (APCA) and acute disseminated encephalomyelitis (ADEM). Less common but important causes include opsoclonus-myoclonus syndrome (OMS) and acute cerebellitis. Cerebellar neoplasms and acute hydrocephalus are additional causes of paediatric ataxia. APCA is the most common cause of ACA in children, comprising about 30-50% of total cases. This is a report about an immunocompetent 4-yrs-old male affected by APCA, due to co-infection by human herpesvirus-6 (HHV-6) and adenovirus, with symptoms mimicking myositis.

  20. Cognition and Emotion in Cerebellar Disorders

    Science.gov (United States)

    ... cerebral cortical “association” areas important for thought, reasoning, motivation, memory and feelings. Damage to the non-motor ... tolerance. Psychosocial interaction may be impaired, particularly in children with cerebellar damage. Dementia is uncommon in cerebellar ...

  1. Adult cerebellar medulloblastoma: CT and MRI findings in eight cases

    Energy Technology Data Exchange (ETDEWEB)

    Carvalho Neto, Arnolfo de; Bertoldi, Guilherme A. [Parana Univ., Curitiba, PR (Brazil). Radiologia Diagnostica]. E-mail: arnolfo.carvalho@avalon.sul.com.br; Gasparetto, Emerson L. [Parana Univ., Curitiba, PR (Brazil). Hospital das Clinicas. Secao de Radiologia Diagnostica; Ono, Sergio E. [Parana Univ., Curitiba, PR (Brazil). Faculdade de Medicina; Gomes, Andre F. [Diagnostico Avancado Por Imagem (DAPI), Curitiba, PR (Brazil)

    2003-06-01

    Medulloblastoma is a brain tumor of neuro epithelial origin, which represents 15 to 30% of all pediatric brain tumors, and less than 1% of CNS adult neoplasms. We report the imaging findings of 8 adult patients with medulloblastoma. The mean age was 35 years, ranging from 20 to 65 years, and the male:female rate was 3:5. The tumors were predominantly lateral (63%), hyperdense on CT scans (83%), and on the MRI, hypointense on T1 (100%) and hyperintense on T2 (80%) weighted images. It was seen intratumoral necrosis and cysts in six cases and calcifications in three. Hydrocephalus was observed in 5 cases and brain stem invasion in four. The imaging findings of medulloblastomas in adults are different of those in child, and also nonspecific. Although these tumors are uncommon in adults, they must be considered in the differential diagnosis of cerebellar masses in the posterior fossa of this age group. (author)

  2. Drugs Approved for Myeloproliferative Neoplasms

    Science.gov (United States)

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for myeloproliferative neoplasms. The list includes generic names, brand names, and common drug combinations, which are shown in capital letters. The drug names link to NCI's Cancer Drug Information summaries.

  3. Language Impairment in Cerebellar Ataxia

    NARCIS (Netherlands)

    van Gaalen, Judith; de Swart, Bert J. M.; Oostveen, Judith; Knuijt, Simone; van de Warrenburg, Bart P. C.; Kremer, Berry (H. ) P. H.

    2014-01-01

    Background: Several studies have suggested that language impairment can be observed in patients with cerebellar pathology. The aim of this study was to investigate language performance in patients with spinocerebellar ataxia type 6 (SCA6). Methods: We assessed speech and language in 29 SCA6 patients

  4. Cerebellar Zones: A Personal History

    NARCIS (Netherlands)

    J. Voogd (Jan)

    2011-01-01

    textabstractCerebellar zones were there, of course, before anyone noticed them. Their history is that of young people, unhindered by preconceived ideas, who followed up their observations with available or new techniques. In the 1960s of the last century, the circumstances were fortunate because thr

  5. Cerebellar arteriovenous malformations in children

    Energy Technology Data Exchange (ETDEWEB)

    Griffiths, P.D. [Sheffield Univ. (United Kingdom). Acad. Dept. of Radiol.; Blaser, S.; Armstrong, D.; Chuang, S.; Harwood-Nash, D. [Division of Neuroradiology, The Hospital for Sick Children and University of Toronto, Toronto (Canada); Humphreys, R.P. [Division of Neurosurgery, The Hospital for Sick Children and University of Toronto, Toronto (Canada)

    1998-05-01

    We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. (orig.) With 4 figs., 4 tabs., 23 refs.

  6. MR angiography in abdominal neoplasms

    Energy Technology Data Exchange (ETDEWEB)

    Squillaci, E. [Dept. of Radiology, Rome-2 Univ., Hospital S. Eugenio, Rome (Italy); Crecco, M. [Dept. of Radiology, Cancer Research Inst. (Regina Elena), Rome (Italy); Grandinetti, M.L. [Dept. of Radiology, Cancer Research Inst. (Regina Elena), Rome (Italy); Maspes, F. [Dept. of Radiology, Rome-2 Univ., Hospital S. Eugenio, Rome (Italy); Lo Presti, G. [Dept. of Radiology, Rome-2 Univ., Hospital S. Eugenio, Rome (Italy); Squillaci, S. [Dept. of Radiology, Cancer Research Inst. (Regina Elena), Rome (Italy); Simonetti, G. [Dept. of Radiology, Rome-2 Univ., Hospital S. Eugenio, Rome (Italy)

    1994-10-01

    The role of magnetic resonance angiography (MRA) in the evaluation of vascular involvement was studied in 55 patients with abdominal neoplasms. A 2-D time-of-flight (TOF) technique was used in all patients. All patients underwent CT and MR examinations before MRA. Also, MR angiograms were compared with digital subtraction angiography in 22 cases, with Doppler US in 13 cases, and with surgical findings in 20 cases. In all patients with liver neoplasms (n=29) MRA demonstrated the absence of flow in the infiltrated segments. Pericapsular neovascularization was observed in 12 patients. Portal vein involvement was correctly detected in 27 patients. In all cases MRA demonstrated in relationship between the tumor and venous structures. Portosystemic shunts were visualized in 20 of 21 patients with portal hypertension. Vena cava thrombosis (3 cases), compression (5 cases), and displacement (2 cases) were correctly demonstrated. In renal (n=6) and adrenal gland (n=3) tumors renal vein compression was correctly detected in 2 cases, displacement in 1 case, and thrombosis in 3 cases, with only 1 false-positive finding. In 7 patients with pancreatic tumors MRA demonstrated splenic vein thrombosis in 2 cases and compression in 2 cases, with one false-positive finding. Our results indicate that MRA provides precise information regarding venous vascular involvement in abdominal neoplasms, but preoperative arterial mapping is still problematic. (orig.)

  7. Cerebellar ataxia and functional genomics : Identifying the routes to cerebellar neurodegeneration

    NARCIS (Netherlands)

    Smeets, C J L M; Verbeek, D S

    2014-01-01

    Cerebellar ataxias are progressive neurodegenerative disorders characterized by atrophy of the cerebellum leading to motor dysfunction, balance problems, and limb and gait ataxia. These include among others, the dominantly inherited spinocerebellar ataxias, recessive cerebellar ataxias such as Fried

  8. Nonsurgical cerebellar mutism (anarthria) in two children.

    Science.gov (United States)

    Mewasingh, Leena D; Kadhim, Hazim; Christophe, Catherine; Christiaens, Florence J; Dan, Bernard

    2003-01-01

    Cerebellar mutism (anarthria) is a well-described complication of posterior fossa tumor resection. It is accompanied by a characteristic behavior including irritability and autistic features. This syndrome is typically reversible within days to months. Underlying pathophysiology is unknown. We describe two children who presented with a similar clinical finding after nonsurgical cerebellar involvement, hemolytic-uremic syndrome in one and cerebellitis in the other. Postmortem pathologic findings in the first patient indicated cerebellar ischemic necrosis. Single-photon emission computed tomography in the second patient revealed diffuse cerebellar hypoperfusion with no supratentorial abnormalities, refuting a phenomenon of diaschisis between cerebellar and frontal connections. These findings confirm that this clinical syndrome may occur in a nonsurgical, nontraumatic context. They are consistent with recent integrative hypotheses explaining cerebellar anarthria.

  9. Crossed cerebral - cerebellar diaschisis : MRI evaluation.

    Directory of Open Access Journals (Sweden)

    Chakravarty A

    2002-07-01

    Full Text Available MRI, done later in life, in two patients with infantile hemiplegia syndrome showed significant volume loss in the cerebellar hemisphere contralateral to the side of the affected cerebrum. The cerebellar volume loss seemed to correlate with the degree of volume loss in the contralateral cerebral hemisphere. These observations provide morphological evidence of the phenomenon of crossed cerebral-cerebellar diaschisis (CCD. Functional neuroimaging studies in support of the concept of CCD has been critically reviewed.

  10. Autosomal recessive cerebellar ataxias : the current state of affairs

    NARCIS (Netherlands)

    Vermeer, S.; van de Warrenburg, B. P. C.; Willemsen, M. A. A. P.; Cluitmans, M.; Scheffer, H.; Kremer, B. P.; Knoers, N. V. A. M.

    2011-01-01

    Among the hereditary ataxias, autosomal recessive cerebellar ataxias (ARCAs) encompass a diverse group of rare neurodegenerative disorders in which a cerebellar syndrome is the key clinical feature. The clinical overlap between the different cerebellar ataxias, the occasional atypical phenotypes, an

  11. Genetics Home Reference: VLDLR-associated cerebellar hypoplasia

    Science.gov (United States)

    ... Conditions VLDLR-associated cerebellar hypoplasia VLDLR-associated cerebellar hypoplasia Enable Javascript to view the expand/collapse boxes. ... Open All Close All Description VLDLR -associated cerebellar hypoplasia is an inherited condition that affects the development ...

  12. [Peripheral neuropathies associated with hereditary cerebellar ataxias].

    Science.gov (United States)

    Anheim, M; Tranchant, C

    2011-01-01

    Inherited cerebellar ataxias constitute a complicated and heterogeneous group of neurodegenerative disorders affecting the cerebellum and/or spinocerebellar tract, spinal cord and peripheral nerves. A peripheral neuropathy is frequently seen in inherited cerebellar ataxias although it rarely reveals the disease. Moreover, the peripheral neuropathy is helpful for the diagnostic procedure and contributes to the functional prognosis of the disease. Thus, electroneuromyography is essential in the algorithm for the diagnosis of inherited cerebellar ataxias, as well as brain MRI (looking especially for cerebellar atrophy) and the assessment of several biomarkers (alpha-foetoprotein, vitamin E, albumin, LDL cholesterol, lactic acid, phytanic acid).

  13. NEOPLASMS

    Institute of Scientific and Technical Information of China (English)

    1997-01-01

    970246 Detection of point mutations of p53 gene bynon-isotopic PCR-SSCP in paraffin-embedded malig-nant mesothelioma tissue. LUO Suqiong(罗素琼), etal. Pneumoconiosis Res Unit, Public Health Sch,West-China Med Univ, Chengdu, 610041. Chin J Ind

  14. NEOPLASMS

    Institute of Scientific and Technical Information of China (English)

    1995-01-01

    950253 The characteristics of bone metastatic tumorsin the elderly-a report of 163 cases.LI Xiaoying(李小鹰),et al.General Hosp,PLA,Beijing,100853.ChinJ Geriatr 1994;13(6):333-334.A study of bone metastatic tumors(BMT) was car-ried out in 163 cases with age of 60 years and over.The characteristics of BMT in the elderly were as fol-lows:1.the elderly patients with BMT made up 7.9percent of all the patients with primary malignant tu-

  15. NEOPLASMS

    Institute of Scientific and Technical Information of China (English)

    2004-01-01

    2004193 Quantitation and detection of deletion in tumor mitochondrial DNA by microarray technique.HAN Chengbo (韩琤波), et al. Tumor Instit, 1st Affili Hosp, China Med Univ, Shenyang 110001. Chin J Oncol 2004;26(1):10-13.Objective: To develop a method to rapidly quanti-tate and detect deletion of mitochondrial DNA (mtD-

  16. NEOPLASMS

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    2003172 Impact of cyclin-dependent kinase inhibitor p27 on resistance of ovarian cancer multicellular spheroids to taxol. XING Hui(刑辉), et al. Dept Ob-stetr Gynecol.Tongji Hosp.Tongiji Med Coll, Huazhong Univ Sci & Technol, Wuhan 430030. Nad Med J China 2003;83(1):37-43.

  17. NEOPLASMS

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    2003034 NOEY2 gene mRNA expression in breast cancer tissue and its relation to clinicopathological parameters. SHI Zonggao ( 施宗高 ), et al. Molec Pathol Lab, Fudan Univ Cancer Hosp, Shanghai 200032. Chin J Oncol 2002;24(5) :475 - 478.Objective: To investigate the expression of NOEY2 gene in breast cancer tissue and its relation to clinico-

  18. NEOPLASMS

    Institute of Scientific and Technical Information of China (English)

    1992-01-01

    920632 Phenotypic analysis of T lympho-cytes from the patient with thymoma com-plicated with pure red cell aplasia. LIUBai(刘白), et al. Beijing Med Univ. Chin J Hema-tol 1992; 13(5): 244-246. The thymocytes in thymoma tissue and mono-nuclear cells in peripheral blood and bone marrowwere obtained from a patient with thymomacomplicated with pure red cell aplasia. The

  19. Gastrointestinal Surgery of Neuroendocrine Neoplasms

    DEFF Research Database (Denmark)

    Hansen, Carsten Palnæs; Olsen, Ingrid Marie Holst; Knigge, Ulrich

    2015-01-01

    Surgery is the only treatment that may cure the patient with gastroenteropancreatic (GEP) neuroendocrine neoplasms (NENs) and should always be considered as the first-line treatment if radical resection can be achieved. Even in cases where radical surgery is not possible, palliative resection may...... be performed to reduce local or hormone-induced symptoms and to improve quality of life. The surgical procedures for GEP-NENs are accordingly described below. In most patients life-long follow-up is required, even following radical surgery, as recurrence may occur several years later....

  20. Less common neoplasms of the pancreas

    Institute of Scientific and Technical Information of China (English)

    Abby L Mulkeen; Peter S Yoo; Charles Cha

    2006-01-01

    Recently, there has been an increased recognition of neoplasms of the pancreas other than ductal adenocarcinoma. Although not as well studied or characterized as pancreatic adenocarcinoma there are many distinct lesions which exhibit diverse biological behaviors and varying degrees of malignancy. These lesions include: endocrine neoplasms, cystic tumors, solid pseudopapillary tumors, acinar cell carcinoma, squamous cell carcinoma, primary lymphoma of the pancreas, and metastatic lesions to the pancreas. These less common neoplasms are being diagnosed more frequently as the number and sensitivity of diagnostic imaging studies increase. This review article discusses the clinical course,diagnosis, and treatment of these less common, but quite relevant, neoplasms of the pancreas.

  1. The clinical presentation of preterm cerebellar haemorrhage

    NARCIS (Netherlands)

    G.M. Ecury-Goossen (Ginette); J. Dudink (Jeroen); M. Leguin (Maarten); M. Feijen-Roon (Monique); S. Horsch (Sandra); P. Govaert (Paul)

    2010-01-01

    textabstractThe objective of this study was to evaluate clinical symptoms and findings on cranial ultrasound (CUS) in preterm infants with cerebellar haemorrhage through retrospective analysis of all preterm infants with a postnatal CUS or MRI diagnosis of cerebellar haemorrhage admitted in a tertia

  2. Stages of Plasma Cell Neoplasms (Including Multiple Myeloma)

    Science.gov (United States)

    ... Treatment Health Professional Plasma Cell Neoplasms Treatment Research Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®)–Patient Version General Information About Plasma Cell Neoplasms Go to Health Professional Version Key ...

  3. Cellular and Molecular Basis of Cerebellar Development

    Directory of Open Access Journals (Sweden)

    Salvador eMartinez

    2013-06-01

    Full Text Available Historically, the molecular and cellular mechanisms of cerebellar development were investigated through structural descriptions and studying spontaneous mutations in animal models and humans. Advances in experimental embryology, genetic engineering and neuroimaging techniques render today the possibility to approach the analysis of molecular mechanisms underlying histogenesis and morphogenesis of the cerebellum by experimental designs. Several genes and molecules were identified to be involved in the cerebellar plate regionalization, specification and differentiation of cerebellar neurons, as well as the establishment of cellular migratory routes and the subsequent neuronal connectivity. Indeed, pattern formation of the cerebellum requires the adequate orchestration of both key morphogenetic signals, arising from distinct brain regions, and local expression of specific transcription factors. Thus, the present review wants to revisit and discuss these morphogenetic and molecular mechanisms taking place during cerebellar development in order to understand causal processes regulating cerebellar cytoarchitecture, its highly topographically ordered circuitry and its role in brain function.

  4. Cerebellar stroke-manifesting as mania

    Directory of Open Access Journals (Sweden)

    Venkatesan Jagadesan

    2014-01-01

    Full Text Available Secondary mania resulting from cerebral Cortex are described commonly. But secondary mania produced by cerebellar lesions are relatively uncommon. This case report describes a patient who developed cerebellar stoke and manic features simultaneously. 28 years old male developed giddiness and projectile vomiting. Then he would lie down for about an hour only to find that he could not walk. He became quarrelsome. His Psycho motor activities and speech were increased. He was euphoric and was expressing grandiose ideas. Bender Gestalt Test showed signs of organicity. Score in Young mania relating scale was 32; productivity was low in Rorschach. Neurological examination revealed left cerebellar signs like ataxia and slurring of speech. Computed tomography of brain showed left cerebellar infarct. Relationship between Psychiatric manifestations and cerebellar lesion are discussed.

  5. The bihemispheric posterior inferior cerebellar artery

    Energy Technology Data Exchange (ETDEWEB)

    Cullen, Sean P. [Brigham and Women' s Hospital and Children' s Hospital, Department of Radiology and Neurosurgery, Boston, MA (United States); Ozanne, Augustin; Alvarez, Hortensia; Lasjaunias, Pierre [Service de Neuroradiologie Diagnostic et Therapeutique, Hopital de Bicetre-Universite Paris-sud Orsay (France)

    2005-11-01

    Rarely, a solitary posterior inferior cerebellar artery (PICA) will supply both cerebellar hemispheres. We report four cases of this variant. We present a retrospective review of clinical information and imaging of patients undergoing angiography at our institution to identify patients with a bihemispheric PICA. There were four patients: three males and one female. One patient presented with a ruptured arteriovenous malformation, and one with a ruptured aneurysm. Two patients had normal angiograms. The bihemispheric PICA was an incidental finding in all cases. The bihemispheric vessel arose from the dominant left vertebral artery, and the contralateral posterior inferior cerebellar artery was absent or hypoplastic. In all cases, contralateral cerebellar supply arose from a continuation of the ipsilateral PICA distal to the choroidal point and which crossed the midline dorsal to the vermis. We conclude that the PICA may supply both cerebellar hemispheres. This rare anatomic variant should be considered when evaluating patients with posterior fossa neurovascular disease. (orig.)

  6. Altered cerebellar feedback projections in Asperger syndrome.

    Science.gov (United States)

    Catani, Marco; Jones, Derek K; Daly, Eileen; Embiricos, Nitzia; Deeley, Quinton; Pugliese, Luca; Curran, Sarah; Robertson, Dene; Murphy, Declan G M

    2008-07-15

    It has been proposed that the biological basis of autism spectrum disorder includes cerebellar 'disconnection'. However, direct in vivo evidence in support of this is lacking. Here, the microstructural integrity of cerebellar white matter in adults with Asperger syndrome was studied using diffusion tensor magnetic resonance tractography. Fifteen adults with Asperger syndrome and 16 age-IQ-gender-matched healthy controls underwent diffusion tensor magnetic resonance imaging. For each subject, tract-specific measurements of mean diffusivity and fractional anisotropy were made within the inferior, middle, superior cerebellar peduncles and short intracerebellar fibres. No group differences were observed in mean diffusivity. However, people with Asperger syndrome had significantly lower fractional anisotropy in the short intracerebellar fibres (pAsperger syndrome. The localised abnormalities in the main cerebellar outflow pathway may prevent the cerebral cortex from receiving those cerebellar feedback inputs necessary for a successful adaptive social behaviour.

  7. Neurological Findings in Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Semra Paydas

    2013-04-01

    Full Text Available Myeloproliferative neoplasms (MPN arise from genetic deficiencies at the level of pluripotent stem cells. Each of these neoplasms is a clonal stem cell disorder with specific phenotypic, genetic and clinical properties. Age is one of the most important factors in the development of symptoms and complications associated with MPNs.High white blood cell counts in chronic myelocytic leukemia also known as leukocytosis may lead to central nervous system findings. Tumors developing outside the bone marrow named as extramedullary myeloid tumors (EMMT could be detected at the initial diagnosis or during the prognosis of the disease, which may cause neurological symptoms due to pressure of leukemic cell mass on various tissues along with spinal cord. Central nervous system involvement and thrombocytopenic hemorrhage may lead to diverse neurological symptoms and findings.Transient ischemic attack and thrombotic stroke are the most common symptoms in polycythemia vera. Besides thrombosis and hemorrage, transformation to acute leukemia can cause neurological symptoms and findings. Transient ischemic attack, thrombotic stroke and specifically hemorrage can give rise to neurological symptoms similar to MPN in essential thrombocytosis.Extramedullary hematopoiesis refers to hematopoietic centers arise in organ/tissues other than bone marrow in myelofibrosis. Extramedullar hematopoietic centers may cause intracranial involvement, spinal cord compression, seizures and hydrocephalia. Though rare, extramedullary hematopoiesis can be detected in cranial/spinal meninges, paraspinal tissue and intracerebral regions. Extramedullary hematopoiesis has been reported in peripheral neurons, choroid plexus, pituitary, orbits, orbital and lacrimal fossa and in sphenoidal sinuses. [Cukurova Med J 2013; 38(2.000: 157-169

  8. Ataxia and tremor due to lesions involving cerebellar projection pathways: a DTI tractographic study in six patients.

    Science.gov (United States)

    Marek, M; Paus, S; Allert, N; Mädler, B; Klockgether, T; Urbach, H; Coenen, V A

    2015-01-01

    Focal lesions of brainstem, thalamus, and subcortical white matter may cause movement disorders that are clinically indistinguishable from cerebellar symptoms. It is suspected that ataxia in these cases is due to damage of efferent or afferent pathways of the cerebellum. However, the precise anatomical correlate often remains undefined. We used deterministic diffusion tensor magnetic resonance imaging (DTI) tractography to study the anatomical relationship between lesions causing ataxia and efferent cerebellar pathways. Study subjects were six male patients with focal lesions of different etiology (demyelination, hemorrhage, ischemia, neoplasm) outside the cerebellum. Five patients had cerebellar-like ataxia with prominent contralateral upper limb involvement. One patient with an almost midline mesencephalic lesion had a symmetrical ataxic syndrome. We used 3T MRI (Intera, Philips Medical Systems, Best, Netherlands) and DTI tractography (32 directions, StealthViz DTI, Medtronic Navigation, Louisville, USA) to delineate the dentato-rubro-thalamo-cortical tract (DRT). In all patients, tractography demonstrated focal lesions affecting the DRT in different locations. We conclude that in vivo mapping of cerebral pathways using DTI tractography in patients with focal extracerebellar brain lesions may provide direct evidence of circumscribed damage to the DRT, causing unilateral cerebellar-like ataxia. Also, a unilateral mesencephalic lesion at the level of the crossing of the DRT may cause bilateral ataxia.

  9. Mucinous Cystic Neoplasms of Pancreas

    Science.gov (United States)

    Naveed, Shah; Qari, Hasina; Banday, Tanveer; Altaf, Asma; Para, Mah

    2014-01-01

    The purpose of this study was to investigate the actual management of mucinous cystic neoplasm (MCN) of the pancreas. A systematic review was performed in December 2009 by consulting PubMed MEDLINE for publications and matching the key words “pancreatic mucinous cystic neoplasm”, “pancreatic mucinous cystic tumor”, “pancreatic mucinous cystic mass”, “pancreatic cyst” and “pancreatic cystic neoplasm” to identify English language articles describing the diagnosis and treatment of the MCN of the pancreas. In total, 16,322 references ranging from January 1969 to December 2009 were analyzed and 77 articles were identified. No articles published before 1996 were selected because MCNs were not previously considered to be a completely autonomous disease. Definition, epidemiology, anatomopathological findings, clinical presentation, preoperative evaluation, treatment and prognosis were reviewed. MCNs are pancreatic mucin-producing cysts with a distinctive ovarian-type stroma localized in the body-tail of the gland and occurring in middle-aged females. The majority of MCNs are slow growing and asymptomatic. The prevalence of invasive carcinoma varies between 6% and 55%. Preoperative diagnosis depends on a combination of clinical features, tumor markers, computed tomography (CT), magnetic resonance imaging, endoscopic ultrasound with cyst fluid analysis and positron emission tomography-CT. Surgery is indicated for all MCNs.

  10. Treatment Option Overview (Chronic Myeloproliferative Neoplasms)

    Science.gov (United States)

    ... way to treat some chronic myeloproliferative neoplasms. Platelet apheresis Platelet apheresis is a treatment that uses a special machine ... using interferon alfa or pegylated interferon alpha . Platelet apheresis . A clinical trial of a new treatment. Check ...

  11. Treatment Options for Chronic Myeloproliferative Neoplasms

    Science.gov (United States)

    ... way to treat some chronic myeloproliferative neoplasms. Platelet apheresis Platelet apheresis is a treatment that uses a special machine ... using interferon alfa or pegylated interferon alpha . Platelet apheresis . A clinical trial of a new treatment. Check ...

  12. General Information about Chronic Myeloproliferative Neoplasms

    Science.gov (United States)

    ... way to treat some chronic myeloproliferative neoplasms. Platelet apheresis Platelet apheresis is a treatment that uses a special machine ... using interferon alfa or pegylated interferon alpha . Platelet apheresis . A clinical trial of a new treatment. Check ...

  13. Premalignant cystic neoplasms of the pancreas.

    Science.gov (United States)

    Dudeja, Vikas; Allen, Peter J

    2015-02-01

    Due to increasing utilization of cross-sectional imaging, asymptomatic pancreatic cysts are frequently being diagnosed. Many of these cysts have premalignant potential and offer a unique opportunity for cancer prevention. Mucinous cystic neoplasm and intraductal papillary mucinous neoplasm are the major premalignant cystic neoplasms of pancreas. The prediction of the risk of malignancy (incidental and future risk of malignant transformation) and balancing the risks of watchful waiting with that of operative management with associated mortality and morbidity is the key to the management of these lesions. We review the literature that has contributed to the development of our approach to the management of these cystic neoplasms. We provide an overview of the key features used in diagnosis and in predicting malignancy. Particular attention is given to the natural history and management decision making.

  14. Cerebellar Involvement in Ataxia and Generalized Epilepsy

    NARCIS (Netherlands)

    L. Kros (Lieke)

    2015-01-01

    markdownabstract__Abstract__ The work described in this thesis was performed in order to elucidate the role of different cerebellar modules in ataxia and generalized epilepsy using various techniques including in vivo electrophysiology, optogenetics, pharmacological interventions, immunohistology a

  15. Computerized tomography in evaluation of hepatic neoplasms

    Energy Technology Data Exchange (ETDEWEB)

    Luna, R.F.; Resende, C.; Tishler, J.M.A.; Aldrete, J.S.; Shin, M.S.; Rubin, E.; Rahn, N.H.

    1984-08-01

    The authors reviewed their experience with computerized tomography (CT) of the abdomen in 212 patients with histologically documented liver neoplasms seen during a 30-month period. The CT findings in cavernous hemangioma and focal nodular hyperplasia were specific, and permitted accurate diagnosis of this lesion before biopsy. The CT appearance of all other lesions was variable. CT is useful in providing an accurate evaluation of the intrahepatic and extrahepatic extent of the neoplasm.

  16. Synchrony and neural coding in cerebellar circuits

    Directory of Open Access Journals (Sweden)

    Abigail L Person

    2012-12-01

    Full Text Available The cerebellum regulates complex movements and is also implicated in cognitive tasks, and cerebellar dysfunction is consequently associated not only with movement disorders, but also with conditions like autism and dyslexia. How information is encoded by specific cerebellar firing patterns remains debated, however. A central question is how the cerebellar cortex transmits its integrated output to the cerebellar nuclei via GABAergic synapses from Purkinje neurons. Possible answers come from accumulating evidence that subsets of Purkinje cells synchronize their firing during behaviors that require the cerebellum. Consistent with models predicting that coherent activity of inhibitory networks has the capacity to dictate firing patterns of target neurons, recent experimental work supports the idea that inhibitory synchrony may regulate the response of cerebellar nuclear cells to Purkinje inputs, owing to the interplay between unusually fast inhibitory synaptic responses and high rates of intrinsic activity. Data from multiple laboratories lead to a working hypothesis that synchronous inhibitory input from Purkinje cells can set the timing and rate of action potentials produced by cerebellar nuclear cells, thereby relaying information out of the cerebellum. If so, then changing spatiotemporal patterns of Purkinje activity would allow different subsets of inhibitory neurons to control cerebellar output at different times. Here we explore the evidence for and against the idea that a synchrony code defines, at least in part, the input-output function between the cerebellar cortex and nuclei. We consider the literature on the existence of simple spike synchrony, convergence of Purkinje neurons onto nuclear neurons, and intrinsic properties of nuclear neurons that contribute to responses to inhibition. Finally, we discuss factors that may disrupt or modulate a synchrony code and describe the potential contributions of inhibitory synchrony to other motor

  17. Cerebellar mutism: review of the literature

    DEFF Research Database (Denmark)

    Gudrunardottir, Thora; Sehested, Astrid; Juhler, Marianne;

    2011-01-01

    Cerebellar mutism is a common complication of posterior fossa surgery in children. This article reviews current status with respect to incidence, anatomical substrate, pathophysiology, risk factors, surgical considerations, treatment options, prognosis and prevention.......Cerebellar mutism is a common complication of posterior fossa surgery in children. This article reviews current status with respect to incidence, anatomical substrate, pathophysiology, risk factors, surgical considerations, treatment options, prognosis and prevention....

  18. Neoplasms derived from plasmacytoid dendritic cells.

    Science.gov (United States)

    Facchetti, Fabio; Cigognetti, Marta; Fisogni, Simona; Rossi, Giuseppe; Lonardi, Silvia; Vermi, William

    2016-02-01

    Plasmacytoid dendritic cell neoplasms manifest in two clinically and pathologically distinct forms. The first variant is represented by nodular aggregates of clonally expanded plasmacytoid dendritic cells found in lymph nodes, skin, and bone marrow ('Mature plasmacytoid dendritic cells proliferation associated with myeloid neoplasms'). This entity is rare, although likely underestimated in incidence, and affects predominantly males. Almost invariably, it is associated with a myeloid neoplasm such as chronic myelomonocytic leukemia or other myeloid proliferations with monocytic differentiation. The concurrent myeloid neoplasm dominates the clinical pictures and guides treatment. The prognosis is usually dismal, but reflects the evolution of the associated myeloid leukemia rather than progressive expansion of plasmacytoid dendritic cells. A second form of plasmacytoid dendritic cells tumor has been recently reported and described as 'blastic plasmacytoid dendritic cell neoplasm'. In this tumor, which is characterized by a distinctive cutaneous and bone marrow tropism, proliferating cells derive from immediate CD4(+)CD56(+) precursors of plasmacytoid dendritic cells. The diagnosis of this form can be easily accomplished by immunohistochemistry, using a panel of plasmacytoid dendritic cells markers. The clinical course of blastic plasmacytoid dendritic cell neoplasm is characterized by a rapid progression to systemic disease via hematogenous dissemination. The genomic landscape of this entity is currently under intense investigation. Recurrent somatic mutations have been uncovered in different genes, a finding that may open important perspectives for precision medicine also for this rare, but highly aggressive leukemia.

  19. [Primary nontransitional neoplasms of the bladder].

    Science.gov (United States)

    Varo Solís, C; Soto Delgado, M; Hens Pérez, A; Baez Perea, J M; Estudillo González, F; Juárez Soto, A; Bachiller Burgos, J; Beltrán Aguilar, V

    1999-01-01

    Revision of all primitive tumours of the bladder diagnosed in our Service between July 1990 and July 1998. Among a total of 703 neoplasms of the bladder only 14 were non-transitional primitive tumours, accounting for just 1.98%. Eleven were malignant neoplasms with a diagnosis of epidermoid carcinoma in nine cases, one adenocarcinoma and one bladder adenocarcinoma. The other three were benign tumours: one haemangioma and two leiomyomas. From a clinical perspective, the predominant symptom was haematuria, followed by irritative symptoms. The two leiomyomas were accidental findings during a gynaecological examination (ultrasound) and a diagnostic examination for a nephritic colic (urography). The diagnostic means used and the extension studies were the same as used for transitional neoplasms. In general, treatment of benign neoplasms was partial cystectomy or transurethral resection while it was radical surgery for the malignant tumours when the existing criteria were an indication for that type of surgery (cystoprostatectomy with bypass), since there are no definite criteria with regards to therapy due to the low incidence of these tumours. Only three of the 11 patients with malignant neoplasms are still alive. All the others died within one year of diagnosis, an evidence of the aggressiveness of these tumours. These cases were considered primitive bladder tumours once it was concluded that there was no relation with any previous or simultaneous transitional neoplasms and that there had been no primitive tumour in a different organ.

  20. Calreticulin Mutations in Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Noa Lavi

    2014-10-01

    Full Text Available With the discovery of the JAK2V617F mutation in patients with Philadelphia chromosome-negative (Ph− myeloproliferative neoplasms (MPNs in 2005, major advances have been made in the diagnosis of MPNs, in understanding of their pathogenesis involving the JAK/STAT pathway, and finally in the development of novel therapies targeting this pathway. Nevertheless, it remains unknown which mutations exist in approximately one-third of patients with non-mutated JAK2 or MPL essential thrombocythemia (ET and primary myelofibrosis (PMF. At the end of 2013, two studies identified recurrent mutations in the gene encoding calreticulin (CALR using whole-exome sequencing. These mutations were revealed in the majority of ET and PMF patients with non-mutated JAK2 or MPL but not in polycythemia vera patients. Somatic 52-bp deletions (type 1 mutations and recurrent 5-bp insertions (type 2 mutations in exon 9 of the CALR gene (the last exon encoding the C-terminal amino acids of the protein calreticulin were detected and found always to generate frameshift mutations. All detected mutant calreticulin proteins shared a novel amino acid sequence at the C-terminal. Mutations in CALR are acquired early in the clonal history of the disease, and they cause activation of JAK/STAT signaling. The CALR mutations are the second most frequent mutations in Ph− MPN patients after the JAK2V617F mutation, and their detection has significantly improved the diagnostic approach for ET and PMF. The characteristics of the CALR mutations as well as their diagnostic, clinical, and pathogenesis implications are discussed in this review.

  1. Bilateral Cerebellar Cortical Dysplasia without Other Malformations: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Jung Seok; Ahn Kook Jin; Kim, Jee Young; Lee, Sun Jin; Park, Jeong Mi [Catholic University Yeouido St. Mary' s Hospital, College of Medicine, Seoul (Korea, Republic of)

    2010-06-15

    Recent advances in MRI have revealed congenital brain malformations and subtle developmental abnormalities of the cerebral and cerebellar cortical architecture. Typical cerebellar cortical dysplasia as a newly categorized cerebellar malformation, has been seen in patients with Fukuyama congenital muscular dystrophy. Cerebellar cortical dysplasia occurs at the embryonic stage and is often observed in healthy newborns. It is also incidentally and initially detected in adults without symptoms. To the best of our knowledge, cerebellar dysplasia without any related disorders is very rare. We describe the MRI findings in one patient with disorganized foliation of both cerebellar hemispheres without a related disorder or syndrome

  2. Changes in the cerebellar and cerebro-cerebellar circuit in type 2 diabetes.

    Science.gov (United States)

    Fang, Peng; An, Jie; Tan, Xin; Zeng, Ling-Li; Shen, Hui; Qiu, Shijun; Hu, Dewen

    2017-01-11

    Currently, 422 million adults suffer from diabetes worldwide, leading to tremendous disabilities and a great burden to families and society. Functional and structural MRIs have demonstrated that patients with type 2 diabetes mellitus (T2DM) exhibit abnormalities in brain regions in the cerebral cortex. However, the changes of cerebellar anatomical connections in diabetic patients remains unclear. In the current study, diffusion tensor imaging deterministic tractography and statistical analysis were employed to investigate abnormal cerebellar anatomical connections in diabetic patients. This is the first study to investigate the altered cerebellar anatomical connectivity in T2DM patients. Decreased anatomical connections were found in the cerebellar and cerebro-cerebellar circuits of T2DM patients, providing valuable new insights into the potential neuro-pathophysiology of diabetes-related motor and cognitive deficits.

  3. INTRAOPERATIVE ULTRASOUND FOR HEPATIC NEOPLASM DURING SURGERY

    Institute of Scientific and Technical Information of China (English)

    1999-01-01

    Objective.Th purpose of this study was to determine the impact of intraoperative ultrasound(IOUS)on the management of patients with neoplasms of the liver.Methods.Forty-nine patients operated on for liver or other pathologic processes were examined intraopertively with 5.0 MHz special ultrasound transducers during surgical exploration of the abdomen.Subjects were evaluated because of known or suspected disease of the liver.Preoperative imaging studies included percutaneous ultrasound(n=49),magnetic resonance imaging(n=11),and computed tomography(n=34).Intraoperative evaluation on all patients included inspection,bimanual palpation,and ultrasnography.Comparison between preoperative imagings and IOUS were analysed.Results.Sensitivity for detection of hepatic neoplasms showed in intraoperative ultrasound,percutaneous ultrasound,magnetic resonance imaging andcomputed tomography as 100%(23/23),74%(17/23),74%(14/19) and 75%(6/8).Specificity showed 100%(26/26),100%(26/26),93%(14/15) and 67(2/3).In seven patients(14%),the neoplasms were not found by inspection,bimanual palpation,and identified only by IOUS.Conclusions.Intraoperative ultrasound is the most sensitive and specific method for detection and surgery of liver neoplasms,especially the occult neoplasms and small size lesion(<2cm).

  4. Metabolic anatomy of paraneoplastic cerebellar degeneration

    Energy Technology Data Exchange (ETDEWEB)

    Anderson, N.E.; Posner, J.B.; Sidtis, J.J.; Moeller, J.R.; Strother, S.C.; Dhawan, V.; Rottenberg, D.A.

    1988-06-01

    Eleven patients with acquired cerebellar degeneration (10 of whom had paraneoplastic cerebellar degeneration (PCD)) were evaluated using neuropsychological tests and /sup 18/F-fluorodeoxyglucose/positron emission tomography to (1) quantify motor, cognitive, and metabolic abnormalities; (2) determine if characteristic alterations in the regional cerebral metabolic rate for glucose (rCMRGlc) are associated with PCD; and (3) correlate behavioral and metabolic measures of disease severity. Eighteen volunteer subjects served as normal controls. Although some PCD neuropsychological test scores were abnormal, these results could not, in general, be dissociated from the effects of dysarthria and ataxia. rCMRGlc was reduced in patients with PCD (versus normal control subjects) in all regions except the brainstem. Analysis of patient and control rCMRGlc data using a mathematical model of regional metabolic interactions revealed two metabolic pattern descriptors, SSF1 and SSF2, which distinguished patients with PCD from normal control subjects; SSF2, which described a metabolic coupling between cerebellum, cuneus, and posterior temporal, lateral frontal, and paracentral cortex, correlated with quantitative indices of cerebellar dysfunction. Our inability to document substantial intellectual impairment in 7 of 10 patients with PCD contrasts with the 50% incidence of dementia in PCD reported by previous investigators. Widespread reductions in PCD rCMRGlc may result from the loss of cerebellar efferents to thalamus and forebrain structures, a reverse cerebellar diaschisis.

  5. Genetics Home Reference: autosomal recessive cerebellar ataxia type 1

    Science.gov (United States)

    ... Genetics Home Health Conditions ARCA1 autosomal recessive cerebellar ataxia type 1 Enable Javascript to view the expand/ ... Open All Close All Description Autosomal recessive cerebellar ataxia type 1 ( ARCA1 ) is a condition characterized by ...

  6. Conventional radiological strategy of common gastrointestinal neoplasms

    Institute of Scientific and Technical Information of China (English)

    Yi-Zhuo; Li; Pei-Hong; Wu

    2015-01-01

    This article summarizes the clinical characteristics and imaging features of common gastrointestinal(GI) neoplasms in terms of conventional radiological imaging methods. Barium studies are readily available for displaying primary malignancies and are minimallyor not at all invasive. A neoplasm may be manifested as various imaging findings, including mucosal disruption, soft mass, ulcer, submucosal invasion and lumen stenosis on barium studies. Benign tumors typically appear as smoothly marginated intramural masses. Malignant neoplasms most often appear as irregular infiltrative lesions on barium examination. Tumor extension to adjacent GI segments may be indistinct on barium images. Cross-sectional images such as computed tomography and magnetic resonance imaging may provide more accurate details of the adjacent organ invasion, omental or peritoneal spread.

  7. Intraductal Oncocytic Papillary Neoplasm of the Pancreas: A Case of a Second Neoplasm in a Pancreas Cancer Survivor

    Directory of Open Access Journals (Sweden)

    Mrinal S Garg

    2015-01-01

    Full Text Available Context Cystic neoplasms, which are less common forms of exocrine pancreatic neoplasms, consist of mainly intraductal papillary mucinous neoplasms (IPMN and mucinous cystic neoplasms. Mucinous cystic neoplasms, unlike IPMN, are not associated with ductal growth, are usually multilocular in nature, and have ovarian type stroma. Mucinous cystadenocarcinoma is a type of mucinous cystic neoplasm more commonly found in women. Intraductal oncocytic papillary neoplasms of the pancreas are the least common variant of IPMN. Despite this classification, intraductal oncocytic papillary neoplasms have been compared to mucinous cystic neoplasms in previous studies and the classification is still questioned. Case report We report a rare case of an intraductal oncocytic papillary neoplasm of the pancreas occurring in a 52-year-old male with a prior history of surgically excised mucinous cystadenocarcinoma. This is the first known case of an intraductal oncocytic papillary neoplasm occurring after a prior pancreatic neoplasm. Conclusion As the diagnosis of intraductal oncocytic papillary neoplasms are rare, having only a few case reports and small series on which to understand its disease process, it is imperative to discuss each case and detail possible correlations with other pancreatic cystic neoplasms as well as distinctions from its current association within IPMN.

  8. Landmark based shape analysis for cerebellar ataxia classification and cerebellar atrophy pattern visualization

    Science.gov (United States)

    Yang, Zhen; Abulnaga, S. Mazdak; Carass, Aaron; Kansal, Kalyani; Jedynak, Bruno M.; Onyike, Chiadi; Ying, Sarah H.; Prince, Jerry L.

    2016-03-01

    Cerebellar dysfunction can lead to a wide range of movement disorders. Studying the cerebellar atrophy pattern associated with different cerebellar disease types can potentially help in diagnosis, prognosis, and treatment planning. In this paper, we present a landmark based shape analysis pipeline to classify healthy control and different ataxia types and to visualize the characteristic cerebellar atrophy patterns associated with different types. A highly informative feature representation of the cerebellar structure is constructed by extracting dense homologous landmarks on the boundary surfaces of cerebellar sub-structures. A diagnosis group classifier based on this representation is built using partial least square dimension reduction and regularized linear discriminant analysis. The characteristic atrophy pattern for an ataxia type is visualized by sampling along the discriminant direction between healthy controls and the ataxia type. Experimental results show that the proposed method can successfully classify healthy controls and different ataxia types. The visualized cerebellar atrophy patterns were consistent with the regional volume decreases observed in previous studies, but the proposed method provides intuitive and detailed understanding about changes of overall size and shape of the cerebellum, as well as that of individual lobules.

  9. Recently described neoplasms of the sinonasal tract.

    Science.gov (United States)

    Bishop, Justin A

    2016-03-01

    Surgical pathology of the sinonasal region (i.e., nasal cavity and the paranasal sinuses) is notoriously difficult, due in part to the remarkable diversity of neoplasms that may be encountered in this area. In addition, a number of neoplasms have been only recently described in the sinonasal tract, further compounding the difficulty for pathologists who are not yet familiar with them. This manuscript will review the clinicopathologic features of some of the recently described sinonasal tumor types: NUT midline carcinoma, HPV-related carcinoma with adenoid cystic-like features, SMARCB1 (INI-1) deficient sinonasal carcinoma, biphenotypic sinonasal sarcoma, and adamantinoma-like Ewing family tumor.

  10. A probabilistic atlas of the cerebellar white matter

    NARCIS (Netherlands)

    Baarsen, K.M. van; Kleinnijenhuis, M.; Jbabdi, S.; Sotiropoulos, S.N.; Grotenhuis, J.A.; Cappellen van Walsum, A.M. van

    2016-01-01

    Imaging of the cerebellar cortex, deep cerebellar nuclei and their connectivity are gaining attraction, due to the important role the cerebellum plays in cognition and motor control. Atlases of the cerebellar cortex and nuclei are used to locate regions of interest in clinical and neuroscience studi

  11. Rhythmic finger tapping reveals cerebellar dysfunction in essential tremor

    NARCIS (Netherlands)

    Buijink, A. W. G.; Broersma, M.; van der Stouwe, A. M. M.; van Wingen, G. A.; Groot, P. F. C.; Speelman, J. D.; Maurits, N. M.; van Rootselaar, A. F.

    2015-01-01

    Introduction: Cerebellar circuits are hypothesized to play a central role in the pathogenesis of essential tremor. Rhythmic finger tapping is known to strongly engage the cerebellar motor circuitry. We characterize cerebellar and, more specifically, dentate nucleus function, and neural correlates of

  12. 21 CFR 882.5820 - Implanted cerebellar stimulator.

    Science.gov (United States)

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Implanted cerebellar stimulator. 882.5820 Section... (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Therapeutic Devices § 882.5820 Implanted cerebellar stimulator. (a) Identification. An implanted cerebellar stimulator is a device used to...

  13. Paraneoplastic cerebellar dysfunction in Hodgkin's lymphoma

    Directory of Open Access Journals (Sweden)

    Kazi Sazzad Manir

    2015-01-01

    Full Text Available Paraneoplastic cerebellar degeneration (PCD is a rare presentation of Hodgkin's Lymphoma (HL manifests as acute/sub-acute nature. We report a case of 21 yr old male presented with acute cerebellar signs along with underlying HL.MRI brain was normal. CSF study was unremarkable. Patient was treated with six cycles of chemotherapy followed by radiotherapy. Neurological manifestations remarkably improved along with complete resolution of underlying HL. Anti-cancer therapy of underlying HL is the main strategy of treating associated PCD.

  14. Philadelphia-negative chronic myeloproliferative neoplasms

    Directory of Open Access Journals (Sweden)

    Rosane Isabel Bittencourt

    2012-01-01

    Full Text Available Chronic myeloproliferative diseases without the Philadelphia chromosome marker (Ph-, although first described 60 years ago, only became the subject of interest after the turn of the millennium. In 2001, the World Health Organization (WHO defined the classification of this group of diseases and in 2008 they were renamed myeloproliferative neoplasms based on morphological, cytogenetic and molecular features. In 2005, the identification of a recurrent molecular abnormality characterized by a gain of function with a mutation in the gene encoding Janus kinase 2 (JAK2 paved the way for greater knowledge of the pathophysiology of myeloproliferative neoplasms. The JAK2 mutation is found in 90-98% of polycythemia vera and in about 50% essential thrombocytosis and primary myelofibrosis. In addition to the JAK2 mutation, other mutations involving TET2 (ten-eleven translocation, LNK (a membrane-bound adaptor protein; IDH1/2 (isocitrate dehydrogenase 1/2 enzyme; ASXL1 (additional sex combs-like 1 genes were found in myeloproliferative neoplasms thus showing the importance of identifying molecular genetic alterations to confirm diagnosis, guide treatment and improve our understanding of the biology of these diseases. Currently, polycythemia vera, essential thrombocytosis, myelofibrosis, chronic neutrophilic leukemia, chronic eosinophilic leukemia and mastocytosis are included in this group of myeloproliferative neoplasms, but are considered different situations with individualized diagnostic methods and treatment. This review updates pathogenic aspects, molecular genetic alterations, the fundamental criteria for diagnosis and the best approach for each of these entities.

  15. Myeloproliferative neoplasms in five multiple sclerosis patients

    DEFF Research Database (Denmark)

    Thorsteinsdottir, Sigrun; Bjerrum, Ole Weis

    2013-01-01

    The concurrence of myeloproliferative neoplasms (MPNs) and multiple sclerosis (MS) is unusual. We report five patients from a localized geographic area in Denmark with both MS and MPN; all the patients were diagnosed with MPNs in the years 2007-2012. We describe the patients' history and treatment...

  16. The new WHO nomenclature: lymphoid neoplasms.

    Science.gov (United States)

    Leclair, Susan J; Rodak, Bernadette F

    2002-01-01

    The development of the WHO classification of lymphoid neoplasms is a remarkable example of cooperation and communication between pathologists and oncologists from around the world. Joint classification committees of the major hematopathology societies will periodically review and update this classification, facilitating further progress in the understanding and treatment of hematologic malignancies.

  17. SNP Array in Hematopoietic Neoplasms: A Review

    Directory of Open Access Journals (Sweden)

    Jinming Song

    2015-12-01

    Full Text Available Cytogenetic analysis is essential for the diagnosis and prognosis of hematopoietic neoplasms in current clinical practice. Many hematopoietic malignancies are characterized by structural chromosomal abnormalities such as specific translocations, inversions, deletions and/or numerical abnormalities that can be identified by karyotype analysis or fluorescence in situ hybridization (FISH studies. Single nucleotide polymorphism (SNP arrays offer high-resolution identification of copy number variants (CNVs and acquired copy-neutral loss of heterozygosity (LOH/uniparental disomy (UPD that are usually not identifiable by conventional cytogenetic analysis and FISH studies. As a result, SNP arrays have been increasingly applied to hematopoietic neoplasms to search for clinically-significant genetic abnormalities. A large numbers of CNVs and UPDs have been identified in a variety of hematopoietic neoplasms. CNVs detected by SNP array in some hematopoietic neoplasms are of prognostic significance. A few specific genes in the affected regions have been implicated in the pathogenesis and may be the targets for specific therapeutic agents in the future. In this review, we summarize the current findings of application of SNP arrays in a variety of hematopoietic malignancies with an emphasis on the clinically significant genetic variants.

  18. Clinical experience in appendiceal neuroendocrine neoplasms

    Science.gov (United States)

    Ozcelik, Caglar K.; Bozdogan, Nazan; Dibekoglu, Cengiz

    2015-01-01

    Aim of the study To analyse the incidence of appendiceal neuroendocrine neoplasms in appendectomy specimens and establish the epidemiological and histopathological features, treatment, and clinical course. Material and methods Between 2004 and 2013, 975 patients who underwent appendectomy in Ankara Oncology Education and Research Hospital were retrospectively analysed. Results Neuroendocrine neoplasm was detected in the nine of 975 (0.9%) patients. Neuroendocrine neoplasms were diagnosed in eight patients by appendectomy, which was performed because of the prediagnosis of acute appendicitis, and in one patient by the suspicious mass detection during surgical procedures that were done in the appendix for a different reason. Eight of the patients’ tumours were in the tip of the appendix, and one of the patients’ tumours was at the base of appendix. Tumour size in 77.8% of patients was equal or less than 1 cm, in 22.2% patients it was 1–2 cm. There was tumour invasion in the muscularis propria layer in four patients, in the serosa layer in three patients, and in the deep mesoappendix in two patients. Patients were followed for a median of 78 months. In the follow-up of patients who were operated because of colon cancer, metachronous colon tumour evolved. This patient died due to progressive disease. Other patients are still disease-free. Conclusions The diagnosis of neuroendocrine neoplasm is often incidentally done after appendectomy. Tumour size is important in determining the extent of disease and in the selection of the surgical method during operation. PMID:26793027

  19. Non-progressive cerebellar ataxia and previous undetermined acute cerebellar injury: a mysterious clinical condition

    Directory of Open Access Journals (Sweden)

    Wladimir Bocca Vieira de Rezende Pinto

    2015-01-01

    Full Text Available Cerebellar ataxias represent a wide group of neurological diseases secondary to dysfunctions of cerebellum or its associated pathways, rarely coursing with acute-onset acquired etiologies and chronic non-progressive presentation. We evaluated patients with acquired non-progressive cerebellar ataxia that presented previous acute or subacute onset. Clinical and neuroimaging characterization of adult patients with acquired non-progressive ataxia were performed. Five patients were identified with the phenotype of acquired non-progressive ataxia. Most patients presented with a juvenile to adult-onset acute to subacute appendicular and truncal cerebellar ataxia with mild to moderate cerebellar or olivopontocerebellar atrophy. Establishing the etiology of the acute triggering events of such ataxias is complex. Non-progressive ataxia in adults must be distinguished from hereditary ataxias.

  20. Non-progressive cerebellar ataxia and previous undetermined acute cerebellar injury: a mysterious clinical condition

    Directory of Open Access Journals (Sweden)

    Wladimir Bocca Vieira de Rezende Pinto

    2015-10-01

    Full Text Available Cerebellar ataxias represent a wide group of neurological diseases secondary to dysfunctions of cerebellum or its associated pathways, rarely coursing with acute-onset acquired etiologies and chronic non-progressive presentation. We evaluated patients with acquired non-progressive cerebellar ataxia that presented previous acute or subacute onset. Clinical and neuroimaging characterization of adult patients with acquired non-progressive ataxia were performed. Five patients were identified with the phenotype of acquired non-progressive ataxia. Most patients presented with a juvenile to adult-onset acute to subacute appendicular and truncal cerebellar ataxia with mild to moderate cerebellar or olivopontocerebellar atrophy. Establishing the etiology of the acute triggering events of such ataxias is complex. Non-progressive ataxia in adults must be distinguished from hereditary ataxias.

  1. Case of subacute cerebellar degeneration associated with pleocytosis and cerebellar swelling shown in computed tomography scanning

    Energy Technology Data Exchange (ETDEWEB)

    Yoshino, Hiide; Anezaki, Toshiharu; Takashima, Noriko; Inuzuka, Takashi; Miyatake, Tadashi

    1988-02-01

    A 44 year old woman was healthy until January 3, 1986, when she had headache. On January 9, she developed gait ataxia and dysarthria. Cerebellar ataxia worsened rapidly. Aftar a week she could not sit without support and her consciousness was disturbed. Corticosteroid was administrated and consciousness proved alert, but cerebellar ataxia and dysarthria remained unchanged. The patient was found carcinoma of the lung in August 1986. Characteristic features of clinical and laboratory findings of this patient are acute progression, cerebrospinal fluid pleocytosis of 1,064/3 cells (860 mononuclear cell, 204 polymorphonuclear cell), and cerebellar swelling shown in computed tomography scanning. Though the mechanism of acute cerebellar degeneration is still uncertained, inflammatory process was supported to exist in cerebellum of this case.

  2. Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2

    NARCIS (Netherlands)

    Nangalia, J.; Massie, C.E.; Baxter, E.J.; Nice, F.L.; Gundem, G.; Wedge, D.C.; Avezov, E.; Li, J.; Kollmann, K.; Kent, D.G.; Aziz, A.; Godfrey, A.L.; Hinton, J.; Martincorena, I.; Loo, P. Van; Jones, A.V.; Guglielmelli, P.; Tarpey, P.; Harding, H.P.; Fitzpatrick, J.D.; Goudie, C.T.; Ortmann, C.A.; Loughran, S.J.; Raine, K.; Jones, D.R.; Butler, A.P.; Teague, J.W.; O'Meara, S.; McLaren, S.; Bianchi, M.; Silber, Y.; Dimitropoulou, D.; Bloxham, D.; Mudie, L.; Maddison, M.; Robinson, B.; Keohane, C.; Maclean, C.; Hill, K.; Orchard, K.; Tauro, S.; Du, M.Q.; Greaves, M.; Bowen, D.; Huntly, B.J.; Harrison, C.N.; Cross, N.C.; Ron, D.; Vannucchi, A.M.; Papaemmanuil, E.; Campbell, P.J.; Green, A.R.

    2013-01-01

    BACKGROUND: Somatic mutations in the Janus kinase 2 gene (JAK2) occur in many myeloproliferative neoplasms, but the molecular pathogenesis of myeloproliferative neoplasms with nonmutated JAK2 is obscure, and the diagnosis of these neoplasms remains a challenge. METHODS: We performed exome sequencing

  3. Cerebellar Hypoplasia and Dysmorphia in Neurofibromatosis Type 1.

    Science.gov (United States)

    Toelle, Sandra P; Poretti, Andrea; Weber, Peter; Seute, Tatjana; Bromberg, Jacoline E C; Scheer, Ianina; Boltshauser, Eugen

    2015-12-01

    Unidentified bright objects (UBO) and tumors are well-known cerebellar abnormalities in neurofibromatosis type 1 (NF1). Literature reports on malformative cerebellar anomalies in neurofibromatosis type 1 (NF1), however, are scant. We retrospectively studied the clinical and neuroimaging findings of 5 patients with NF1 (4 females, age 6 to 29 years at last follow-up) and cerebellar anomalies. Cerebellar symptoms on neurological examination were mild or even not evident whereas learning disabilities were more or less pronounced in four patients. Two patients had cerebellar hypoplasia (diffusely enlarged cerebellar interfoliar spaces) and three cerebellar dysmorphias involving mainly one cerebellar hemisphere. In NF1, malformative cerebellar anomalies are rare (estimated prevalence of about 1%), but most likely underestimated and easily overlooked, because physicians tend to focus on more prevalent, obvious, and well-known findings such as optic pathway gliomas, other tumors, and UBO. This kind of cerebellar anomaly in NF1 has most likely a malformative origin, but the exact pathogenesis is unknown. The individual clinical significance is difficult to determine. We suggest that cerebellar anomalies should be systematically evaluated in neuroimaging studies of NF1 patients.

  4. Climbing fiber signaling and cerebellar gain control

    NARCIS (Netherlands)

    G. Ohtsuki (Gen); C. Piochon (Claire); C.R.W. Hansel (Christian)

    2009-01-01

    textabstractThe physiology of climbing fiber signals in cerebellar Purkinje cells has been studied since the early days of electrophysiology. Both the climbing fiber-evoked complex spike and the role of climbing fiber activity in the induction of long-term depression (LTD) at parallel fiber-Purkinje

  5. Crossed cerebellar diaschisis in ischemic stroke

    DEFF Research Database (Denmark)

    Meneghetti, G; Vorstrup, S; Mickey, B

    1984-01-01

    depression was evident in five patients with severe hemispheric low flow areas, which correlated with large, hypodense lesions on the computerized tomographic scan. In a sixth patient with a small, deep infarct, a transient crossed cerebellar low flow was observed, while the clinical symptoms persisted...... in the infarcted hemisphere, in which a period of relative hyperemia is frequently seen....

  6. Improving cerebellar segmentation with statistical fusion

    Science.gov (United States)

    Plassard, Andrew J.; Yang, Zhen; Prince, Jerry L.; Claassen, Daniel O.; Landman, Bennett A.

    2016-03-01

    The cerebellum is a somatotopically organized central component of the central nervous system well known to be involved with motor coordination and increasingly recognized roles in cognition and planning. Recent work in multiatlas labeling has created methods that offer the potential for fully automated 3-D parcellation of the cerebellar lobules and vermis (which are organizationally equivalent to cortical gray matter areas). This work explores the trade offs of using different statistical fusion techniques and post hoc optimizations in two datasets with distinct imaging protocols. We offer a novel fusion technique by extending the ideas of the Selective and Iterative Method for Performance Level Estimation (SIMPLE) to a patch-based performance model. We demonstrate the effectiveness of our algorithm, Non- Local SIMPLE, for segmentation of a mixed population of healthy subjects and patients with severe cerebellar anatomy. Under the first imaging protocol, we show that Non-Local SIMPLE outperforms previous gold-standard segmentation techniques. In the second imaging protocol, we show that Non-Local SIMPLE outperforms previous gold standard techniques but is outperformed by a non-locally weighted vote with the deeper population of atlases available. This work advances the state of the art in open source cerebellar segmentation algorithms and offers the opportunity for routinely including cerebellar segmentation in magnetic resonance imaging studies that acquire whole brain T1-weighted volumes with approximately 1 mm isotropic resolution.

  7. Ultrasonically detectable cerebellar haemorrhage in preterm infants.

    LENUS (Irish Health Repository)

    McCarthy, Lisa Kenyon

    2011-07-01

    To determine the frequency and pattern of cerebellar haemorrhage (CBH) on routine cranial ultrasound (cUS) imaging in infants of ≤32 weeks gestation, and to investigate how extremely preterm infants with CBH differ from those with severe intraventricular haemorrhage (IVH).

  8. Cerebellar endocannabinoids: retrograde signaling from purkinje cells.

    Science.gov (United States)

    Marcaggi, Païkan

    2015-06-01

    The cerebellar cortex exhibits a strikingly high expression of type 1 cannabinoid receptor (CB1), the cannabinoid binding protein responsible for the psychoactive effects of marijuana. CB1 is primarily found in presynaptic elements in the molecular layer. While the functional importance of cerebellar CB1 is supported by the effect of gene deletion or exogenous cannabinoids on animal behavior, evidence for a role of endocannabinoids in synaptic signaling is provided by in vitro experiments on superfused acute rodent cerebellar slices. These studies have demonstrated that endocannabinoids can be transiently released by Purkinje cells and signal at synapses in a direction opposite to information transfer (retrograde). Here, following a description of the reported expression pattern of the endocannabinoid system in the cerebellum, I review the accumulated in vitro data, which have addressed the mechanism of retrograde endocannabinoid signaling and identified 2-arachidonoylglycerol as the mediator of this signaling. The mechanisms leading to endocannabinoid release, the effects of CB1 activation, and the associated synaptic plasticity mechanisms are discussed and the remaining unknowns are pointed. Notably, it is argued that the spatial specificity of this signaling and the physiological conditions required for its induction need to be determined in order to understand endocannabinoid function in the cerebellar cortex.

  9. Cerebellar cortical inhibition and classical eyeblink conditioning.

    Science.gov (United States)

    Bao, Shaowen; Chen, Lu; Kim, Jeansok J; Thompson, Richard F

    2002-02-01

    The cerebellum is considered a brain structure in which memories for learned motor responses (e.g., conditioned eyeblink responses) are stored. Within the cerebellum, however, the relative importance of the cortex and the deep nuclei in motor learning/memory is not entirely clear. In this study, we show that the cerebellar cortex exerts both basal and stimulus-activated inhibition to the deep nuclei. Sequential application of a gamma-aminobutyric acid type A receptor (GABA(A)R) agonist and a noncompetitive GABA(A)R antagonist allows selective blockade of stimulus-activated inhibition. By using the same sequential agonist and antagonist methods in behaving animals, we demonstrate that the conditioned response (CR) expression and timing are completely dissociable and involve different inhibitory inputs; although the basal inhibition modulates CR expression, the conditioned stimulus-activated inhibition is required for the proper timing of the CR. In addition, complete blockade of cerebellar deep nuclear GABA(A)Rs prevents CR acquisition. Together, these results suggest that different aspects of the memories for eyeblink CRs are encoded in the cerebellar cortex and the cerebellar deep nuclei.

  10. Intraductal Oncocytic Papillary Neoplasm Having Clinical Characteristics of Mucinous Cystic Neoplasm and a Benign Histology

    Directory of Open Access Journals (Sweden)

    Takatomi Oku

    2007-03-01

    Full Text Available Context An intraductal oncocytic papillary neoplasm is a rare pancreatic tumor which was first described by Adsay et al. in 1996. It has been defined as a new subgroup of IPMN. Case report We report the case of a 76-year-old woman who presented with nausea. Imaging studies revealed a cystic mass in the body of the pancreas. She underwent a successful distal pancreatectomy and splenectomy, and has subsequently remained well. Microscopically, the cyst was lined by columnar epithelium similar to pancreatic duct epithelium, and the nodular projection consisted of arborizing papillary structures, lined by plump cells with abundant eosinophilic cytoplasm. These eosinophilic cells were immunohistochemically positively stained with anti-mitochondrial antibody. The cellular atypism was mild and the proliferating index was low, compatible with adenoma of an intraductal oncocytic papillary neoplasm. Although no ovarian type stroma was identified, in our case, no communication to main pancreatic duct (located in the pancreatic body and rapid growth by intracystic hemorrhage were clinical characteristics of a mucinous cystic neoplasm, but not IPMN. Conclusion With only 17 cases reported to date, the clinical and pathological details of an intraductal oncocytic papillary neoplasm are still unclear. We herein add one case with different characteristics from those of the past reports. To our knowledge, this is the first case report of an intraductal oncocytic papillary neoplasm with the clinical characteristics of a mucinous cystic neoplasm.

  11. Perinatal Cerebellar Injury in Human and Animal Models

    Directory of Open Access Journals (Sweden)

    Valerie Biran

    2012-01-01

    Full Text Available Cerebellar injury is increasingly recognized through advanced neonatal brain imaging as a complication of premature birth. Survivors of preterm birth demonstrate a constellation of long-term neurodevelopmental deficits, many of which are potentially referable to cerebellar injury, including impaired motor functions such as fine motor incoordination, impaired motor sequencing and also cognitive, behavioral dysfunction among older patients. This paper reviews the morphogenesis and histogenesis of the human and rodent developing cerebellum, and its more frequent injuries in preterm. Most cerebellar lesions are cerebellar hemorrhage and infarction usually leading to cerebellar abnormalities and/or atrophy, but the exact pathogenesis of lesions of the cerebellum is unknown. The different mechanisms involved have been investigated with animal models and are primarily hypoxia, ischemia, infection, and inflammation Exposure to drugs and undernutrition can also induce cerebellar abnormalities. Different models are detailed to analyze these various disturbances of cerebellar development around birth.

  12. Endoscopic submucosal dissection for gastrointestinal neoplasms

    Institute of Scientific and Technical Information of China (English)

    Naomi Kakushima; Mitsuhiro Fujishiro

    2008-01-01

    Endoscopic submucosal dissection (ESD) is an advanced technique of therapeutic endoscopy for superficial gastrointestinal neoplasms. Three steps characterize it:injecting fluid into the submucosa to elevate the lesion,cutting the surrounding mucosa of the lesion, and dissecting the submucosa beneath the lesion. The ESD technique has rapidly permeated in Japan for treatment of early gastric cancer, due to its excellent results of enbloc resection compared to endoscopic mucosal resection (EMR). Although there is still room for improvement to lessen its technical difficulty, ESD has recently been applied to esophageal and colorectal neoplasms.Favorable short-term results have been reported, but the application of ESD should be well considered by three aspects: (1) the possibility of nodal metastases of the lesion, (2) technical difficulty such as location, ulceration and operator's skill, and (3) organ characteristics.

  13. Solid and papillary neoplasm of the pancreas

    DEFF Research Database (Denmark)

    Jørgensen, L J; Hansen, A B; Burcharth, F;

    1992-01-01

    In two cases of solid and papillary neoplasm of the pancreas (SPN), positive staining for argyrophil granules, chromogranin-A, neuron-specific enolase, chymotrypsin, alpha 1-antitrypsin, vimentin, cytokeratin, and estrogen receptors was present. Ultrastructurally, neurosecretory as well as zymoge......In two cases of solid and papillary neoplasm of the pancreas (SPN), positive staining for argyrophil granules, chromogranin-A, neuron-specific enolase, chymotrypsin, alpha 1-antitrypsin, vimentin, cytokeratin, and estrogen receptors was present. Ultrastructurally, neurosecretory as well...... as zymogenlike granules were demonstrated. Measurements of mean nuclear volume and volume-corrected mitotic index discriminated between SPN and well-differentiated ductal adenocarcinoma of the pancreas, with notably lower values being seen in SPN. Silver-stained nucleolar organizer region counts showed wide...

  14. Primary bone neoplasms in dogs: 90 cases

    Directory of Open Access Journals (Sweden)

    Maria E. Trost

    2012-12-01

    Full Text Available A retrospective study of necropsy and biopsy cases of 90 primary bone tumors (89 malignant and one benign in dogs received over a period of 22 years at the Laboratório de Patologia Veterinária, Universidade Federal de Santa Maria, was performed. Osteosarcoma was the most prevalent bone tumor, accounting for 86.7% of all malignant primary bone neoplasms diagnosed. Most cases occurred in dogs of large and giant breeds with ages between 6 and 10-years-old. The neoplasms involved mainly the appendicular skeleton, and were 3.5 times more prevalent in the forelimbs than in the hindlimbs. Osteoblastic osteosarcoma was the predominant histological subtype. Epidemiological and pathological findings of osteosarcomas are reported and discussed.

  15. Intrathoracic neoplasms in the dog and cat

    Energy Technology Data Exchange (ETDEWEB)

    Weller, R.E.

    1994-03-01

    Very little is known regarding the epidemiology, etiology, and mechanisms of spontaneous intrathoracic neoplasia in companion animals. Much of what we know or suspect about thoracic neoplasia in animals has been extrapolated from experimentally-induced neoplasms. Most studies of thoracic neoplasia have focused on the pathology of primary and metastatic neoplasms of the lung with little attention given to diagnostic and therapeutic considerations. Although the cited incidence rate for primary respiratory tract neoplasia is low, 8.5 cases per 100,000 dogs and 5.5 cases per 100,000 cats, intrathoracic masses often attract attention out of proportion to their actual importance since they are often readily visualized on routine thoracic radiographs.

  16. A probabilistic atlas of the cerebellar white matter.

    Science.gov (United States)

    van Baarsen, K M; Kleinnijenhuis, M; Jbabdi, S; Sotiropoulos, S N; Grotenhuis, J A; van Cappellen van Walsum, A M

    2016-01-01

    Imaging of the cerebellar cortex, deep cerebellar nuclei and their connectivity are gaining attraction, due to the important role the cerebellum plays in cognition and motor control. Atlases of the cerebellar cortex and nuclei are used to locate regions of interest in clinical and neuroscience studies. However, the white matter that connects these relay stations is of at least similar functional importance. Damage to these cerebellar white matter tracts may lead to serious language, cognitive and emotional disturbances, although the pathophysiological mechanism behind it is still debated. Differences in white matter integrity between patients and controls might shed light on structure-function correlations. A probabilistic parcellation atlas of the cerebellar white matter would help these studies by facilitating automatic segmentation of the cerebellar peduncles, the localization of lesions and the comparison of white matter integrity between patients and controls. In this work a digital three-dimensional probabilistic atlas of the cerebellar white matter is presented, based on high quality 3T, 1.25mm resolution diffusion MRI data from 90 subjects participating in the Human Connectome Project. The white matter tracts were estimated using probabilistic tractography. Results over 90 subjects were symmetrical and trajectories of superior, middle and inferior cerebellar peduncles resembled the anatomy as known from anatomical studies. This atlas will contribute to a better understanding of cerebellar white matter architecture. It may eventually aid in defining structure-function correlations in patients with cerebellar disorders.

  17. Acquired uniparental disomy in myeloproliferative neoplasms.

    Science.gov (United States)

    Score, Joannah; Cross, Nicholas C P

    2012-10-01

    The finding of somatically acquired uniparental disomy, where both copies of a chromosome pair or parts of chromosomes have originated from one parent, has led to the discovery of several novel mutated genes in myeloproliferative neoplasms and related disorders. This article examines how the development of single nucleotide polymorphism array technology has facilitated the identification of regions of acquired uniparental disomy and has led to a much greater understanding of the molecular pathology of these heterogeneous diseases.

  18. MR appearance of skeletal neoplasms following cryotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Richardson, M.L. [Dept. of Radiology SB-05, Washington Univ., Seattle, WA (United States); Lough, L.R. [Pitts Radiological Associates, Columbia, SC (United States); Shuman, W.P. [Dept. of Radiology, Medical Center Hospital of Vermont, Burlington, VT (United States); Lazerte, G.D. [Dept. of Pathology RC-72, Washington Univ., Medical Center Hospital of Vermont, Burlington, VT (United States); Conrad, E.U. [Dept. of Orthopedic Surgery RK-10, Washington Univ., Medical Center of Vermont, Burlington, VT (United States)

    1994-02-01

    Cryotherapy is an increasingly popular mode of therapy adjunctive to surgical curettage in the treatment of certain skeletal neoplasms, such as giant cell tumors or chondrosarcomas. The magnetic resonance (MR) findings following cryotherapy have not been previously reported. We reviewed the MR findings in seven patients with skeletal neoplasms following curettage and cryotherapy. In six cases we found a zone of varying thickness extending beyond the surgical margins, corresponding to an area of cryoinjury to medullary bone. This zone displayed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, consistent with the presence of marrow edema. This zone of edema almost certainly reflects underlying thermal osteonecrosis. This zone may vary in size and intensity over time as the area of cryoinjury evolves or resolves. MR is currently the imaging procedure of choice for follow-up of most musculoskeletal neoplasms. Knowledge of the MR findings following cryotherapy should help prevent confusion during the interpretation of follow-up MR examinations. (orig.)

  19. A toolbox to visually explore cerebellar shape changes in cerebellar disease and dysfunction

    Science.gov (United States)

    Abulnaga, S. Mazdak; Yang, Zhen; Carass, Aaron; Kansal, Kalyani; Jedynak, Bruno M.; Onyike, Chiadi U.; Ying, Sarah H.; Prince, Jerry L.

    2016-03-01

    The cerebellum plays an important role in motor control and is also involved in cognitive processes. Cerebellar function is specialized by location, although the exact topographic functional relationship is not fully understood. The spinocerebellar ataxias are a group of neurodegenerative diseases that cause regional atrophy in the cerebellum, yielding distinct motor and cognitive problems. The ability to study the region-specific atrophy patterns can provide insight into the problem of relating cerebellar function to location. In an effort to study these structural change patterns, we developed a toolbox in MATLAB to provide researchers a unique way to visually explore the correlation between cerebellar lobule shape changes and function loss, with a rich set of visualization and analysis modules. In this paper, we outline the functions and highlight the utility of the toolbox. The toolbox takes as input landmark shape representations of subjects' cerebellar substructures. A principal component analysis is used for dimension reduction. Following this, a linear discriminant analysis and a regression analysis can be performed to find the discriminant direction associated with a specific disease type, or the regression line of a specific functional measure can be generated. The characteristic structural change pattern of a disease type or of a functional score is visualized by sampling points on the discriminant or regression line. The sampled points are used to reconstruct synthetic cerebellar lobule shapes. We showed a few case studies highlighting the utility of the toolbox and we compare the analysis results with the literature.

  20. Hereditary spastic paraplegia with cerebellar ataxia

    DEFF Research Database (Denmark)

    Nielsen, J E; Johnsen, B; Koefoed, P

    2004-01-01

    Complex forms of hereditary spastic paraplegia (HSP) are rare and usually transmitted in an autosomal recessive pattern. A family of four generations with autosomal dominant hereditary spastic paraplegia (AD-HSP) and a complex phenotype with variably expressed co-existing ataxia, dysarthria...... in those individuals who were clinically affected by a complex phenotype consisting of HSP and cerebellar ataxia. Other features noted in this kindred including epilepsy, cognitive impairment, depression, and migraine did not segregate with the HSP phenotype or mutation, and therefore the significance...... relatively decreased regional cerebral blood flow in most of the cerebellum. We conclude that this kindred demonstrates a considerable overlap between cerebellar ataxia and spastic paraplegia, emphasizing the marked clinical heterogeneity of HSP associated with spastin mutations....

  1. An update on Spino-cerebellar ataxias

    Directory of Open Access Journals (Sweden)

    Banashree Mondal

    2013-01-01

    Full Text Available The dominantly inherited ataxias, also known as Spino-cerebellar ataxias (SCAs, are rapidly expanding entities. New mutations are being identified at remarkable regularity. Recent awareness of molecular abnormalities in SCAs has addressed some of the long sought questions, but gaps in knowledge still exist. Three major categories of SCAs, according to molecular mechanisms, have evolved over recent few years: Polyglutamate expansion ataxia, non-coding zone repeat ataxia, and ataxia due to conventional mutation. Using the fulcrum of these mechanisms, the article provides an update of SCAs. Shared and specific clinical features, genetic abnormalities, and possible links between molecular abnormalities and cerebellar degeneration have been discussed. Emphasis has been placed on the mechanisms of polyglutamate toxicity.

  2. Cerebellar Dysfunction in a Patient with HIV.

    Science.gov (United States)

    Gonzalez-Ibarra, Fernando; Abdul, Waheed; Eivaz-Mohammadi, Sahar; Foscue, Christopher; Gongireddy, Srinivas; Syed, Amer

    2014-01-01

    A 50-year-old AIDS patient with a CD4 T-cell count of 114/mm(3) was admitted with cerebellar symptoms of left CN XI weakness, wide-based gait with left-sided dysmetria, abnormal heel-knee-shin test, and dysdiadochokinesia. MRI showed region of hyperintensity in the left inferior cerebellar hemisphere involving the cortex and underlying white matter. Serological tests for HSV1, HSV2, and syphilis were negative. Her CSF contained high protein content and a WBC of 71/mm(3), predominantly lymphocytes. The CSF was also negative for cryptococcal antigen and VDRL. CSF culture did not grow microbes. CSF PCR assay was negative for HSV1 and HSV2 but was positive for JC virus (1,276 copies). The most likely diagnosis is granule cell neuronopathy (GCN), which can only be definitively confirmed with biopsy and immunohistochemistry.

  3. Hereditary spastic paraplegia with cerebellar ataxia

    DEFF Research Database (Denmark)

    Nielsen, J E; Johnsen, B; Koefoed, P

    2004-01-01

    Complex forms of hereditary spastic paraplegia (HSP) are rare and usually transmitted in an autosomal recessive pattern. A family of four generations with autosomal dominant hereditary spastic paraplegia (AD-HSP) and a complex phenotype with variably expressed co-existing ataxia, dysarthria...... relatively decreased regional cerebral blood flow in most of the cerebellum. We conclude that this kindred demonstrates a considerable overlap between cerebellar ataxia and spastic paraplegia, emphasizing the marked clinical heterogeneity of HSP associated with spastin mutations....

  4. Memory consolidation in the cerebellar cortex.

    Directory of Open Access Journals (Sweden)

    Daniel O Kellett

    Full Text Available Several forms of learning, including classical conditioning of the eyeblink, depend upon the cerebellum. In examining mechanisms of eyeblink conditioning in rabbits, reversible inactivations of the control circuitry have begun to dissociate aspects of cerebellar cortical and nuclear function in memory consolidation. It was previously shown that post-training cerebellar cortical, but not nuclear, inactivations with the GABAA agonist muscimol prevented consolidation but these findings left open the question as to how final memory storage was partitioned across cortical and nuclear levels. Memory consolidation might be essentially cortical and directly disturbed by actions of the muscimol, or it might be nuclear, and sensitive to the raised excitability of the nuclear neurons following the loss of cortical inhibition. To resolve this question, we simultaneously inactivated cerebellar cortical lobule HVI and the anterior interpositus nucleus of rabbits during the post-training period, so protecting the nuclei from disinhibitory effects of cortical inactivation. Consolidation was impaired by these simultaneous inactivations. Because direct application of muscimol to the nuclei alone has no impact upon consolidation, we can conclude that post-training, consolidation processes and memory storage for eyeblink conditioning have critical cerebellar cortical components. The findings are consistent with a recent model that suggests the distribution of learning-related plasticity across cortical and nuclear levels is task-dependent. There can be transfer to nuclear or brainstem levels for control of high-frequency responses but learning with lower frequency response components, such as in eyeblink conditioning, remains mainly dependent upon cortical memory storage.

  5. Computed tomography in hypertensive cerebellar hemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Nose, T.; Maki, Y.; Ono, Y.; Yoshizawa, T.; Tsuboi, K. (Tsukuba Univ., Sakura, Ibaraki (Japan))

    1981-11-01

    Fourteen cases of cerebellar hemorrhage were analysed from the point of CT-scan, and the following results were obtained. 1. The number of cases of cerebellar hemorrhage forms 4.4% of that of total intracranial hemorrhage. 2. Most of the cerebellar hematomas extend upward. Downward extension is rare. 3. In acute dead cases hematomas are 5 cm or more in diameter and lie over bilateral hemispheres with the extension to third or fourth ventricles in CT-scans. 4. Slowly progressive cases are detriorated by the secondary hydrocephalus. 5. In mild cases hematomas are 3cm or less in diameter on CT-scans and the hematoma evacuation is not indicated for these cases. 6. The shunt operation alone is sufficient for the life saving of the slowly progressive cases, but the hematoma evacuation is indicated in these cases if the functional prognosis is taken into consideration. 7. Immediate hematoma evacuation together with the ventricular drainage is considered to be effective for the life saving of the acute fulminant cases.

  6. An integrator circuit in cerebellar cortex.

    Science.gov (United States)

    Maex, Reinoud; Steuber, Volker

    2013-09-01

    The brain builds dynamic models of the body and the outside world to predict the consequences of actions and stimuli. A well-known example is the oculomotor integrator, which anticipates the position-dependent elasticity forces acting on the eye ball by mathematically integrating over time oculomotor velocity commands. Many models of neural integration have been proposed, based on feedback excitation, lateral inhibition or intrinsic neuronal nonlinearities. We report here that a computational model of the cerebellar cortex, a structure thought to implement dynamic models, reveals a hitherto unrecognized integrator circuit. In this model, comprising Purkinje cells, molecular layer interneurons and parallel fibres, Purkinje cells were able to generate responses lasting more than 10 s, to which both neuronal and network mechanisms contributed. Activation of the somatic fast sodium current by subthreshold voltage fluctuations was able to maintain pulse-evoked graded persistent activity, whereas lateral inhibition among Purkinje cells via recurrent axon collaterals further prolonged the responses to step and sine wave stimulation. The responses of Purkinje cells decayed with a time-constant whose value depended on their baseline spike rate, with integration vanishing at low ( 30 per s). The model predicts that the apparently fast circuit of the cerebellar cortex may control the timing of slow processes without having to rely on sensory feedback. Thus, the cerebellar cortex may contain an adaptive temporal integrator, with the sensitivity of integration to the baseline spike rate offering a potential mechanism of plasticity of the response time-constant.

  7. The microvasculature of the human cerebellar meninges.

    Science.gov (United States)

    Nonaka, Hiroko; Akima, Michiko; Hatori, Tsutomu; Nagayama, Tadashi; Zhang, Zean; Ihara, Fumie

    2002-12-01

    The vascular architecture of the human cerebellar meninges was investigated. The surface meninges were poor in vasculature. In the sulci, the meninges were highly vascular but had few capillaries. The venous blood vessels gave long side branches at right angles to the parent vessels in a cruciform pattern, running horizontally along the cerebellar sulci. They were situated at the origin of the secondary or tertiary sulci. Anastomoses between these horizontal branches gave a crosshatched appearance. Short branches often extended to the bases of the sulci, terminating in T-shaped bifurcations with numerous tiny branches, like the roots of a tree. The arteries ran perpendicular to venous branches which were parallel to each other exclusively along the sagittal plane. These arteries bifurcated to straddle the horizontally running veins at the origin of the secondary or tertiary sulci. They gave off many small branches like teeth of a fork from each artery in the secondary or tertiary sulci after they bifurcated to straddle the venous branches and penetrated the cerebellar cortex at the bases of sulci. These fork-like ramifications in the bases of the sulci were most likely responsible for the ready development of pronounced ischemic state. They might also play an important role in the occurrence of ischemic damage at the bases of sulci in cases of severe generalized ischemia.

  8. Emotional disorders in patients with cerebellar damage – case studies

    OpenAIRE

    Siuda, Katarzyna; Chrobak, Adrian Andrzej; Starowicz-Filip, Anna; Tereszko,Anna; Dudek, Dominika

    2014-01-01

    Aim: Growing number of research shows the role of the cerebellum in the regulation of affect. Lesions of the cerebellum can lead to emotional disregulation, a significant part of the Cerebellar Cognitive Affective Syndrome. The aim of this article is to analyze the most recent studies concerning the cerebellar participation in emotional reactions and to present three cases: two female and one male who suffered from cerebellar damage and presented post-traumatic affective and personality chang...

  9. Cerebro-cerebellar circuits in autism spectrum disorder.

    Science.gov (United States)

    D'Mello, Anila M; Stoodley, Catherine J

    2015-01-01

    The cerebellum is one of the most consistent sites of abnormality in autism spectrum disorder (ASD) and cerebellar damage is associated with an increased risk of ASD symptoms, suggesting that cerebellar dysfunction may play a crucial role in the etiology of ASD. The cerebellum forms multiple closed-loop circuits with cerebral cortical regions that underpin movement, language, and social processing. Through these circuits, cerebellar dysfunction could impact the core ASD symptoms of social and communication deficits and repetitive and stereotyped behaviors. The emerging topography of sensorimotor, cognitive, and affective subregions in the cerebellum provides a new framework for interpreting the significance of regional cerebellar findings in ASD and their relationship to broader cerebro-cerebellar circuits. Further, recent research supports the idea that the integrity of cerebro-cerebellar loops might be important for early cortical development; disruptions in specific cerebro-cerebellar loops in ASD might impede the specialization of cortical regions involved in motor control, language, and social interaction, leading to impairments in these domains. Consistent with this concept, structural, and functional differences in sensorimotor regions of the cerebellum and sensorimotor cerebro-cerebellar circuits are associated with deficits in motor control and increased repetitive and stereotyped behaviors in ASD. Further, communication and social impairments are associated with atypical activation and structure in cerebro-cerebellar loops underpinning language and social cognition. Finally, there is converging evidence from structural, functional, and connectivity neuroimaging studies that cerebellar right Crus I/II abnormalities are related to more severe ASD impairments in all domains. We propose that cerebellar abnormalities may disrupt optimization of both structure and function in specific cerebro-cerebellar circuits in ASD.

  10. Acute bilateral cerebellar infarction in the territory of the medial branches of posterior inferior cerebellar arteries.

    Science.gov (United States)

    Gurer, G; Sahin, G; Cekirge, S; Tan, E; Saribas, O

    2001-10-01

    The most frequent type of cerebellar infarcts involved the posterior inferior cerebellar artery (PICA) and superior cerebellar artery territories but bilateral involvement of lateral or medial branches of PICA is extremely rare. In this report, we present a 55-year-old male who admitted to hospital with vomiting, nausea and dizziness. On examination left-sided hemiparesia and ataxic gait were detected. Infarct on bilateral medial branch of PICA artery territories was found out with cranial magnetic resonance imaging (MRI) technique and 99% stenosis of the left vertebral artery was found out with digital subtraction arteriography. The patient was put on heparin treatment. After 3 weeks, his complaints and symptoms had disappeared except for mild gait ataxia.

  11. Cerebellar ataxia as the presenting manifestation of Lyme disease.

    Science.gov (United States)

    Arav-Boger, Ravit; Crawford, Thomas; Steere, Allen C; Halsey, Neal A

    2002-04-01

    A 7-year-old boy from suburban Baltimore who presented with cerebellar ataxia and headaches was found by magnetic resonance imaging to have multiple cerebellar enhancing lesions. He had no history of tick exposure. He was initially treated with steroids for presumptive postinfectious encephalitis. Lyme disease was diagnosed 10 weeks later after arthritis developed. Testing of the cerebrospinal fluid obtained at the time cerebellar ataxia was diagnosed revealed intrathecal antibody production to Borrelia burgdorferi. Treatment with intravenous antibiotics led to rapid resolution of persistent cerebellar findings.

  12. Pitch discrimination in cerebellar patients: evidence for a sensory deficit.

    Science.gov (United States)

    Parsons, Lawrence M; Petacchi, Augusto; Schmahmann, Jeremy D; Bower, James M

    2009-12-15

    In the last two decades, a growing body of research showing cerebellar involvement in an increasing number of nonmotor tasks and systems has prompted an expansion of speculations concerning the function of the cerebellum. Here, we tested the predictions of a hypothesis positing cerebellar involvement in sensory data acquisition. Specifically, we examined the effect of global cerebellar degeneration on primary auditory sensory function by means of a pitch discrimination task. The just noticeable difference in pitch between two tones was measured in 15 healthy controls and in 15 high functioning patients afflicted with varying degrees of global cerebellar degeneration caused by hereditary, idiopathic, paraneoplastic, or postinfectious pancerebellitis. Participants also performed an auditory detection task assessing sustained attention, a test of verbal auditory working memory, and an audiometric test. Patient pitch discrimination thresholds were on average five and a half times those of controls and were proportional to the degree of cerebellar ataxia assessed independently. Patients and controls showed normal hearing thresholds and similar performance in control tasks in sustained attention and verbal auditory working memory. These results suggest there is an effect of cerebellar degeneration on primary auditory function. The findings are consistent with other recent demonstrations of cerebellar-related sensory impairments, and with robust cerebellar auditorily evoked activity, confirmed by quantitative meta-analysis, across a range of functional neuroimaging studies dissociated from attention, motor, affective, and cognitive variables. The data are interpreted in the context of a sensory hypothesis of cerebellar function.

  13. Four types of neoplasms in Asian sea bass (Lates calcarifer)

    Institute of Scientific and Technical Information of China (English)

    Ramalingam Vijayakumar; Kuzhanthaivel Raja; Vijayapoopathi Singaravel; Ayyaru Gopalakrishnan

    2015-01-01

    Objective:To describe and observe four types of neoplasms on different parts (external and internal organs) of an Asian sea bass (Lates calcarifer). Methods:The sample was collected from local fish landing center (south east coast of India). Histopathology of normal and tumour tissues were analyzed. Results:A total of 83 tumour masses (neoplasm) were recorded on the fish skin, also the neoplasms were recorded in internal organs of fish such as liver, stomach and ovary. Conclusions:Aetiology of such neoplasm’s are unknown, further more researches need to confirm the causative agent for this type of neoplasm.

  14. Cerebellar Hemangioblastoma: Four Case Reports and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Sevgi Bakaris

    2015-03-01

    Full Text Available Hemangioblastoma (HB is a benign, slow-growing, highly vascular tumour of not well defined histological origin. These tumors make up about 1 to 2 percent of all intracranial neoplasms and occur primarily in the posterior fossa. Hemangioblastomas can occur sporadically but in about 20% to 30% cases, it is associated with von Hippel-Lindau (VHL disease. Four cases of cerebellar haemangioblastoma, not associated with von Hippel-Lindau disease (sporadic haemangioblastomas, were presented and reviewed the relevant literature.Four hemangioblastomas of the central nervous system were examined with haematoxylin and eosin (H and E, reticulin stain and with a panel of antibodies including CD34, vimentin, NSE, S-100, CD99, CD56, GFAP, cytoceratin, epithelial membrane antigen (EMA, CD10. Of the 4 patients in this study 1 was male and 3 were female. Their ages ranged from 46 years to 60 years with a mean age of 54.75 years. All of them were as cystic nodules about 2-3 cm in diameter. In the histopathological examination, the tumors sections showed large and vacuolated stromal cells and numerous arborizing capillary-size blood vessels. Some tumors showed atypical nuclei. Vimentin was strongly positive both stromal cells and blood veessels in all tumors. In 4 cases of HB, some stromal cells were positive for NSE and CD99. Three tumors were positive for S-100 and CD56, two tumors were focally positive for glial fibrillary acidic protein (GFAP. CD34 immunostaining highlighted the arborizing and complex vascular network, whereas the tumor stromal cells were negative. The stromal cells were negative for epithelial markers such as cytokeratin, EMA and CD10. Ki-67 index was less than 1% of the tumor cells. Hemangioblastoma, a rare, benign tumors of uncertain histogenesis, is characterized histologically by the presence of vacuolated, lipid containing cells and a well developed, fine capillary network. The main histological differential diagnosis of HB is metastatic

  15. Malignant neoplasms of the head and neck.

    Science.gov (United States)

    Dickson, Paxton V; Davidoff, Andrew M

    2006-05-01

    Head and neck masses represent a common clinical entity in children. In general, these masses are classified as developmental, inflammatory, or neoplastic. Having a working knowledge of lesions within this region and conducting a thorough history and physical examination generally enables the clinician to facilitate an appropriate workup and establish a diagnosis. The differential diagnosis is broad, and expeditiously distinguishing benign from malignant masses is critical for instituting a timely multidisciplinary approach to the management of malignant lesions. Neoplasms of the head and neck account for approximately 5% of all childhood malignancies. A diagnosis of malignancy may represent a primary tumor or metastatic foci to cervical nodes. In this review, we discuss the general approach to evaluating suspicious masses and adenopathy in the head and neck region and summarize the most common malignant neoplasms of the head and neck with regard to their incidence, clinical presentation, diagnostic evaluation, staging, and management. Thyroid, parathyroid, and salivary gland tumors are discussed elsewhere in this issue of Seminars in Pediatric Surgery.

  16. Topical treatment options for conjunctival neoplasms

    Directory of Open Access Journals (Sweden)

    Jonathan W Kim

    2008-10-01

    Full Text Available Jonathan W Kim, David H AbramsonOphthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, NY, USAAbstract: Topical therapies offer a nonsurgical method for treating conjunctival tumors by delivering high drug concentrations to the ocular surface. Over the past ten years, topical agents have been used by investigators to treat various premalignant and malignant lesions of the conjunctiva, such as primary acquired melanosis with atypia, conjunctival melanoma, squamous intraepithelial neoplasia and squamous cell carcinoma of the conjunctiva, and pagetoid spread of the conjunctiva arising from sebaceous cell carcinoma. Despite the enthusiasm generated by the success of these agents, there are unanswered questions regarding the clinical efficacy of this new nonsurgical approach, and whether a single topical agent can achieve cure rates comparable with traditional therapies. Furthermore, the long-term consequences of prolonged courses of topical chemotherapeutic drugs on the ocular surface are unknown, and the ideal regimen for each of these agents is still being refined. In this review, we present specific guidelines for treating both melanocytic and squamous neoplasms of the conjunctiva, utilizing the available data in the literature as well as our own clinical experience at the Memorial Sloan-Kettering Cancer Center.Keywords: topical therapies, conjunctival neoplasms melanosis, Mitomycin-C, 5-Fluorouracil

  17. Pancreatic neuroendocrine neoplasms; Neuroendokrine Neoplasien des Pankreas

    Energy Technology Data Exchange (ETDEWEB)

    Beiderwellen, K.; Lauenstein, T.C. [Universitaetsklinikum Essen, Institut fuer Diagnostische und Interventionelle Radiologie und Neuroradiologie, Essen (Germany); Sabet, A.; Poeppel, T.D. [Universitaetsklinikum Essen, Klinik fuer Nuklearmedizin, Essen (Germany); Lahner, H. [Universitaetsklinikum Essen, Klinik fuer Endokrinologie und Stoffwechselerkrankungen, Essen (Germany)

    2016-04-15

    Pancreatic neuroendocrine neoplasms (NEN) account for 1-2 % of all pancreatic neoplasms and represent a rare differential diagnosis. While some pancreatic NEN are hormonally active and exhibit endocrine activity associated with characteristic symptoms, the majority are hormonally inactive. Imaging techniques such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) or as combined PET/CT play a crucial role in the initial diagnosis, therapy planning and control. Endoscopic ultrasound (EUS) and multiphase CT represent the reference methods for localization of the primary pancreatic tumor. Particularly in the evaluation of small liver lesions MRI is the method of choice. Somatostatin receptor scintigraphy and somatostatin receptor PET/CT are of particular value for whole body staging and special aspects of further therapy planning. (orig.) [German] Neuroendokrine Neoplasien (NEN) des Pankreas stellen mit einem Anteil von 1-2 % aller pankreatischen Tumoren eine seltene Differenzialdiagnose dar. Ein Teil der Tumoren ist hormonell aktiv und faellt klinisch durch charakteristische Symptome auf, wohingegen der ueberwiegende Anteil hormonell inaktiv ist. Bildgebende Verfahren wie Sonographie, Computertomographie (CT), Magnetresonanztomographie (MRT) und nicht zuletzt Positronenemissionstomographie (PET oder kombiniert als PET/CT) spielen eine zentrale Rolle fuer Erstdiagnose, Therapieplanung und -kontrolle. Die Endosonographie und die multiphasische CT stellen die Referenzmethoden zur Lokalisation des Primaertumors dar. Fuer die Differenzierung insbesondere kleiner Leberlaesionen bietet die MRT die hoechste Aussagekraft. Fuer das Ganzkoerperstaging und bestimmte Aspekte der Therapieplanung lassen sich die Somatostatinrezeptorszintigraphie und v. a. die Somatostatinrezeptor-PET/CT heranziehen. (orig.)

  18. Cerebellar transcranial direct current stimulation effects on saccade adaptation

    NARCIS (Netherlands)

    E. Avila (Eric); J.N. van der Geest (Jos); S. Kengne Kamga (Sandra); M.C. Verhage (M. Claire); O. Donchin (Opher); M.A. Frens (Maarten)

    2015-01-01

    textabstractSaccade adaptation is a cerebellar-mediated type of motor learning in which the oculomotor system is exposed to repetitive errors. Different types of saccade adaptations are thought to involve distinct underlying cerebellar mechanisms. Transcranial direct current stimulation (tDCS) induc

  19. Drug-induced cerebellar ataxia: a systematic review

    NARCIS (Netherlands)

    Gaalen, J. van; Kerstens, F.G.; Maas, R.P.P.W.M.; Harmark, L.; Warrenburg, B.P.C. van de

    2014-01-01

    BACKGROUND AND OBJECTIVES: Cerebellar ataxia can be induced by a large number of drugs. We here conducted a systemic review of the drugs that can lead to cerebellar ataxia as an adverse drug reaction (ADR). METHODS: We performed a systematic literature search in Pubmed (1966 to January 2014) and EMB

  20. Excitatory Cerebellar Nucleocortical Circuit Provides Internal Amplification during Associative Conditioning

    NARCIS (Netherlands)

    Gao, Zhenyu; Proietti-Onori, Martina; Lin, Zhanmin; Ten Brinke, Michiel M; Boele, Henk-Jan; Potters, Jan-Willem; Ruigrok, Tom J H; Hoebeek, Freek E; De Zeeuw, Chris I

    2016-01-01

    Closed-loop circuitries between cortical and subcortical regions can facilitate precision of output patterns, but the role of such networks in the cerebellum remains to be elucidated. Here, we characterize the role of internal feedback from the cerebellar nuclei to the cerebellar cortex in classical

  1. Cerebellar cortical infarct cavities and vertebral artery disease

    Energy Technology Data Exchange (ETDEWEB)

    Cocker, Laurens J.L. de [University Medical Center Utrecht, Department of Radiology, Utrecht (Netherlands); Kliniek Sint-Jan Radiologie, Brussels (Belgium); Compter, A.; Kappelle, L.J.; Worp, H.B. van der [University Medical Center Utrecht, Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus, Utrecht (Netherlands); Luijten, P.R.; Hendrikse, J. [University Medical Center Utrecht, Department of Radiology, Utrecht (Netherlands)

    2016-09-15

    Cerebellar cortical infarct cavities are a newly recognised entity associated with atherothromboembolic cerebrovascular disease and worse physical functioning. We aimed to investigate the relationship of cerebellar cortical infarct cavities with symptomatic vertebrobasilar ischaemia and with vascular risk factors. We evaluated the MR images of 46 patients with a recent vertebrobasilar TIA or stroke and a symptomatic vertebral artery stenosis ≥50 % from the Vertebral Artery Stenting Trial (VAST) for the presence of cerebellar cortical infarct cavities ≤1.5 cm. At inclusion in VAST, data were obtained on age, sex, history of vertebrobasilar TIA or stroke, and vascular risk factors. Adjusted risk ratios were calculated with Poisson regression analyses for the relation between cerebellar cortical infarct cavities and vascular risk factors. Sixteen out of 46 (35 %) patients showed cerebellar cortical infarct cavities on the initial MRI, and only one of these 16 patients was known with a previous vertebrobasilar TIA or stroke. In patients with symptomatic vertebrobasilar ischaemia, risk factor profiles of patients with cerebellar cortical infarct cavities were not different from patients without these cavities. Cerebellar cortical infarct cavities are seen on MRI in as much as one third of patients with recently symptomatic vertebral artery stenosis. Since patients usually have no prior history of vertebrobasilar TIA or stroke, cerebellar cortical infarct cavities should be added to the spectrum of common incidental brain infarcts visible on routine MRI. (orig.)

  2. Molecular markers of neuronal progenitors in the embryonic cerebellar anlage.

    Science.gov (United States)

    Morales, Daniver; Hatten, Mary E

    2006-11-22

    The cerebellum, like the cerebrum, includes a nuclear structure and an overlying cortical structure. Experiments in the past decade have expanded knowledge beyond the traditional function of the cerebellum to include critical roles in motor learning and memory and sensory discrimination. The initial steps in cerebellar development depend on inductive signaling involving FGF and Wnt proteins produced at the mesencephalic/metencephalic boundary. To address the issue of how individual cerebellar cell fates within the cerebellar territory are specified, we examined the expression of transcription factors, including mammalian homologues of LIM homeodomain-containing proteins, basic helix-loop-helix proteins, and three amino acid loop-containing proteins. The results of these studies show that combinatorial codes of transcription factors define precursors of the cerebellar nuclei, and both Purkinje cells and granule neurons of the cerebellar cortex. Examination of gene expression patterns in several hundred lines of Egfp-BAC (bacterial artificial chromosome) transgenic mice in the GENSAT Project revealed numerous genes with restricted expression in cerebellar progenitor populations, including genes specific for cerebellar nuclear precursors and Purkinje cell precursors. In addition, we identified patterns of gene expression that link granule and Purkinje cells to their precerebellar nuclei. These results identify molecular pathways that offer new insights on the development of the nuclear and cortical structures of the cerebellum, as well as components of the cerebellar circuitry.

  3. Cerebellar vermis plays a causal role in visual motion discrimination.

    Science.gov (United States)

    Cattaneo, Zaira; Renzi, Chiara; Casali, Stefano; Silvanto, Juha; Vecchi, Tomaso; Papagno, Costanza; D'Angelo, Egidio

    2014-09-01

    Cerebellar patients have been found to show deficits in visual motion discrimination, suggesting that the cerebellum may play a role in visual sensory processing beyond mediating motor control. Here we show that triple-pulse online transcranial magnetic stimulation (TMS) over cerebellar vermis but not over the cerebellar hemispheres significantly impaired motion discrimination. Critically, the interference caused by vermis TMS on motion discrimination did not depend on an indirect effect of TMS over nearby visual areas, as demonstrated by a control experiment in which TMS over V1 but not over cerebellar vermis significantly impaired orientation discrimination. These findings demonstrate the causal role of the cerebellar vermis in visual motion processing in neurologically normal participants.

  4. New evidence for the cerebellar involvement in personality traits.

    Science.gov (United States)

    Picerni, Eleonora; Petrosini, Laura; Piras, Fabrizio; Laricchiuta, Daniela; Cutuli, Debora; Chiapponi, Chiara; Fagioli, Sabrina; Girardi, Paolo; Caltagirone, Carlo; Spalletta, Gianfranco

    2013-01-01

    Following the recognition of its role in sensory-motor coordination and learning, the cerebellum has been involved in cognitive, emotional, and even personality domains. This study investigated the relationships between cerebellar macro- and micro-structural variations and temperamental traits measured by Temperament and Character Inventory (TCI). High resolution T1-weighted, and Diffusion Tensor Images of 100 healthy subjects aged 18-59 years were acquired by 3 Tesla Magnetic Resonance scanner. In multiple regression analyses, cerebellar Gray Matter (GM) or White Matter (WM) volumes, GM Mean Diffusivity (MD), and WM Fractional Anisotropy (FA) were used as dependent variables, TCI scores as regressors, gender, age, and education years as covariates. Novelty Seeking scores were associated positively with the cerebellar GM volumes and FA, and negatively with MD. No significant association between Harm Avoidance, Reward Dependence or Persistence scores and cerebellar structural measures was found. The present data put toward a cerebellar involvement in the management of novelty.

  5. Primary cardiac neoplasms:a clinicopathologic analysis of 81 cases

    Institute of Scientific and Technical Information of China (English)

    王继纲

    2013-01-01

    Objective To study the disease spectrum,clinical and pathologic features of primary cardiac neoplasms at asingle medical in stitution during a period of eight years.Methods The clinical and pathologic features of 81 cases of primary cardiac neoplasms encountered at the Affiliated

  6. Disorganized foliation of unilateral cerebellar hemisphere as cerebellar cortical dysplasia in patients with recurrent seizures: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Baek, Hye Jin [Dept. of Radiology, Haeundae Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2013-09-15

    We present a rare case of abnormal foliation for one cerebellar hemisphere on MR imaging, showing vertically-oriented folia. Foliation of contralateral cerebellar hemisphere and other structures in the posterior fossa were normal, and the patient has no neurologic deficits. This rare and unique abnormality is considered a kind of developmental error of the cerebellum.

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  15. Solid pseudopapillary neoplasm of pancreas: a rare presentation

    Directory of Open Access Journals (Sweden)

    Mohd Jafar Memon

    2016-07-01

    Full Text Available Pancreatic neoplasms are rare in children and have a different histo-logic spectrum and prognosis than those in adults. Pancreatoblastoma is the most common pancreatic neoplasm in young children. Solid pseudopapillary neoplasm occurs in adolescent girls. It is heterogeneous in internal architecture, with a mixture of solid and cystic hemorrhagic and necrotic elements. All pancreatic neoplasms in children are capable of producing metastases, usually to the liver and lymph nodes; however, on the whole, these tumors have a better clinical outcome than most pancreatic tumors in adults. We present a case of solid pseudopapillary neoplasm with a liver metastasis in a 13 year old male patient. [Int J Res Med Sci 2016; 4(7.000: 3090-3093

  16. Cerebellar ependymal cyst in a dog.

    Science.gov (United States)

    Wyss-Fluehmann, G; Konar, M; Jaggy, A; Vandevelde, M; Oevermann, A

    2008-11-01

    An 11-week-old, male, Staffordshire Bull Terrier had a history of generalized ataxia and falling since birth. The neurologic findings suggested a localization in the cerebellum. Magnetic resonance imaging of the brain was performed. In all sequences the area of the cerebellum was almost replaced by fluid isointense to cerebrospinal fluid. A complete necropsy was performed after euthanasia. Histologically, the lesion was characterized by extensive loss of cerebellar tissue in both hemispheres and vermis. Toward the surface of the cerebellar defect, the cavity was confined by ruptured and folded membranes consisting of a layer of glial fibrillary acidic (GFAP)-positive glial cells covered multifocally by epithelial cells. Some of these cells bore apical cilia and were cytokeratin and GFAP negative, supporting their ependymal origin. The histopathologic features of our case are consistent with the diagnosis of an ependymal cyst. Its glial and ependymal nature as demonstrated by histopathologic and immunohistochemical examination differs from arachnoid cysts, which have also been reported in dogs. The origin of these cysts remains controversial, but it has been suggested that they develop during embryogenesis subsequent to sequestration of developing neuroectoderm. We speculate that the cyst could have been the result of a pre- or perinatal, possibly traumatic, insult because hemorrhage, and tissue destruction had occurred. To our knowledge, this is the first description of an ependymal cyst in the veterinary literature.

  17. Remote cerebellar hemorrhage after lumbar spinal surgery

    Energy Technology Data Exchange (ETDEWEB)

    Cevik, Belma [Baskent University Faculty of Medicine, Department of Radiology, Fevzi Cakmak Cad. 10. sok. No: 45, Bahcelievler, Ankara 06490 (Turkey)], E-mail: belmac@baskent-ank.edu.tr; Kirbas, Ismail; Cakir, Banu; Akin, Kayihan; Teksam, Mehmet [Baskent University Faculty of Medicine, Department of Radiology, Fevzi Cakmak Cad. 10. sok. No: 45, Bahcelievler, Ankara 06490 (Turkey)

    2009-04-15

    Background: Postoperative remote cerebellar hemorrhage (RCH) as a complication of lumbar spinal surgery is an increasingly recognized clinical entity. The aim of this study was to determine the incidence of RCH after lumbar spinal surgery and to describe diagnostic imaging findings of RCH. Methods: Between October 1996 and March 2007, 2444 patients who had undergone lumbar spinal surgery were included in the study. Thirty-seven of 2444 patients were scanned by CT or MRI due to neurologic symptoms within the first 7 days of postoperative period. The data of all the patients were studied with regard to the following variables: incidence of RCH after lumbar spinal surgery, gender and age, coagulation parameters, history of previous arterial hypertension, and position of lumbar spinal surgery. Results: The retrospective study led to the identification of two patients who had RCH after lumbar spinal surgery. Of 37 patients who had neurologic symptoms, 29 patients were women and 8 patients were men. CT and MRI showed subarachnoid hemorrhage in the folia of bilateral cerebellar hemispheres in both patients with RCH. The incidence of RCH was 0.08% among patients who underwent lumbar spinal surgery. Conclusion: RCH is a rare complication of lumbar spinal surgery, self-limiting phenomenon that should not be mistaken for more ominous pathologic findings such as hemorrhagic infarction. This type of bleeding is thought to occur secondary to venous infarction, but the exact pathogenetic mechanism is unknown. CT or MRI allowed immediate diagnosis of this complication and guided conservative management.

  18. Vestibular and cerebellar contribution to gaze optimality.

    Science.gov (United States)

    Sağlam, Murat; Glasauer, Stefan; Lehnen, Nadine

    2014-04-01

    Patients with chronic bilateral vestibular loss have large gaze variability and experience disturbing oscillopsia, which impacts physical and social functioning, and quality of life. Gaze variability and oscillopsia in these patients are attributed to a deficient vestibulo-ocular reflex, i.e. impaired online feedback motor control. Here, we assessed whether the lack of vestibular input also affects feed-forward motor learning, i.e. the ability to choose optimal movement parameters that minimize variability during active movements such as combined eye-head gaze shifts. A failure to learn from practice and reshape feed-forward motor commands in response to sensory error signals to achieve appropriate movements has been proposed to explain dysmetric gaze shifts in patients with cerebellar ataxia. We, therefore, assessed the differential roles of both sensory vestibular information and the cerebellum in choosing optimal movement kinematics. We have previously shown that, in the course of several gaze shifts, healthy subjects adjust the motor command to minimize endpoint variability also when movements are experimentally altered by an increase in the head moment of inertia. Here, we increased the head inertia in five patients with chronic complete bilateral vestibular loss (aged 45.4±7.1 years, mean±standard deviation), nine patients with cerebellar ataxia (aged 56.7±12.6 years), and 10 healthy control subjects (aged 39.7±6.3 years) while they performed large (75° and 80°) horizontal gaze shifts towards briefly flashed targets in darkness and, using our previous optimal control model, compared their gaze shift parameters to the expected optimal movements with increased head inertia. Patients with chronic bilateral vestibular loss failed to update any of the gaze shift parameters to the new optimum with increased head inertia. Consequently, they displayed highly variable, suboptimal gaze shifts. Patients with cerebellar ataxia updated some movement parameters to

  19. Endoscopic submucosal dissection for stomach neoplasms

    Institute of Scientific and Technical Information of China (English)

    Mitsuhiro Fujishiro

    2006-01-01

    Recent advances in techniques of therapeutic endoscopy for stomach neoplasms are rapidly achieved. One of the major topics in this field is endoscopic submucosal dissection (ESD). ESD is a new endoscopic technique using cutting devices to remove the tumor by thefollowing three steps: injecting fluid into the submucosa to elevate the tumor from the muscle layer, pre-cutting the surrounding mucosa of the tumor, and dissecting the connective tissue of the submucosa beneath the tumor. So the tumors are resectable in an en bloc fashion, regardless of the size, shape, coexisting ulcer,and location. Indication for ESD is strictly confined by two aspects: the possibility of nodal metastases and technical difficulty, which depends on the operators. Although long-term outcome data are still lacking, short-term outcomes of ESD are extremely favourable and laparotomy with gastrectomy is replaced with ESD in some parts of therapeutic strategy for early gastric cancer.

  20. Cholesteryl esters in human malignant neoplasms.

    Science.gov (United States)

    Tosi, M R; Bottura, G; Lucchi, P; Reggiani, A; Trinchero, A; Tugnoli, V

    2003-01-01

    Cholesteryl esters (CholE) were detected in human malignant neoplasms by means of in vitro nuclear magnetic resonance spectroscopy. Spectroscopic analysis of the total lipid extracts obtained from cerebral tumors revealed appreciable amount of esterified cholesterol in high grade gliomas such as glioblastomas and anaplastic oligodendrogliomas, characterized by prominent neovascularity. The finding that no CholE were detected in the healthy brain and in low grade and benign tumors supports a possible correlation between this class of lipids and histological vascular proliferation. Compared with high grade gliomas, renal cell carcinomas show higher levels of CholE, absent in the healthy renal parenchyma and in benign oncocytomas. In nefro-carcinomas, cytoplasmic lipid inclusions and prominent vascularization contribute to the increased levels of CholE present mainly as oleate. CholE are discussed as potential biochemical markers of cancer and as a target for new therapeutic strategies.

  1. Contribution of cerebellar sensorimotor adaptation to hippocampal spatial memory.

    Directory of Open Access Journals (Sweden)

    Jean-Baptiste Passot

    Full Text Available Complementing its primary role in motor control, cerebellar learning has also a bottom-up influence on cognitive functions, where high-level representations build up from elementary sensorimotor memories. In this paper we examine the cerebellar contribution to both procedural and declarative components of spatial cognition. To do so, we model a functional interplay between the cerebellum and the hippocampal formation during goal-oriented navigation. We reinterpret and complete existing genetic behavioural observations by means of quantitative accounts that cross-link synaptic plasticity mechanisms, single cell and population coding properties, and behavioural responses. In contrast to earlier hypotheses positing only a purely procedural impact of cerebellar adaptation deficits, our results suggest a cerebellar involvement in high-level aspects of behaviour. In particular, we propose that cerebellar learning mechanisms may influence hippocampal place fields, by contributing to the path integration process. Our simulations predict differences in place-cell discharge properties between normal mice and L7-PKCI mutant mice lacking long-term depression at cerebellar parallel fibre-Purkinje cell synapses. On the behavioural level, these results suggest that, by influencing the accuracy of hippocampal spatial codes, cerebellar deficits may impact the exploration-exploitation balance during spatial navigation.

  2. Fatal remote cerebellar hemorrhage after supratentorial unruptured aneurysm surgery in patient with previous cerebellar infarction

    Science.gov (United States)

    Koh, Eun-Jeong; Park, Jung-Soo

    2017-01-01

    Abstract Rationale: Remote cerebellar hemorrhage (RCH) is a rare complication of supratentorial and spinal surgeries, seldom requiring intervention but occasionally causing significant morbidity or even mortality. Although a number of theories have been proposed, the exact pathophysiology of RCH remains incompletely understood. Patient concerns: We present a 62-year-old patient with RCH encountered following surgical clipping of an unruptured middle cerebral artery bifurcation aneurysm in a patient with previous cerebellar infarction. Lessons: It is extremely rare, but sometimes, RCH can be life-threatening. It is necessary to check the patient's general condition, underlying diseases and medical history. And controlled drainage of the CSF seems to be most important. Arachnoidplasty may be a consideration and the position of the drain string might have to be carefully determined. PMID:28121936

  3. CT findings of intrathoricic neoplasm associated with hypertrophic osteoarthropathy

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, Hee Sung; Choe, Kyu Ok; Chung, Jin Il; Oh, Sei Chung [College of Medicine Yonsei University, Seoul (Korea, Republic of)

    1994-02-15

    Hypertrophic osteoarthropathy(HOA) is a clinical syndrome consisting of clubbing, periostitis and synovitis. Most frequent causes of hypertrophic osteoarthropathy are intrathoracic neoplasms, among which the bronchogenic carcinoma ranks the highest. But computed tomographic evaluation of intrathoracic neoplasm associated with HOA has been seldom reported. The purpose of this study is to evaluate CT findings of intrathoracic neoplasm associated with HOA, and to infer possible mechanism. Seven cases of intrathoracic neoplasm associated with HOA were included in our study. Diagnoses of HOA were made by Tc99m bone scintigraphy or plain radiography. The findings of chest CT scans were reviewed retrospectively, with main interests on their size, location and internal characteristics, ect. Seven cases of intrathoracic neoplasm consisted of five bronchogenic carcinomas and two thymic tumors. The size of intrathoracic tumors were relatively large ranging from 6cm to 13cm(average 8.0cm). All thoracic neoplasms showed wide pleural contact, and one of them invaded thoracic wall. The range of length of pleural contact was 5-18cm(average 9.9cm). All of seven patients had internal necrosis, and one of them showed cavitation in thoracic mass. Intrathoracic neoplasms associated with HOA had a tendency to be large, to contain internal necrosis, and to widely abut the thoracic pleura.

  4. Lissencephaly with brainstem and cerebellar hypoplasia and congenital cataracts.

    Science.gov (United States)

    Abumansour, Iman S; Wrogemann, Jens; Chudley, Albert E; Chodirker, Bernard N; Salman, Michael S

    2014-06-01

    Classical lissencephaly may be associated with cerebellar hypoplasia and when significant cerebellar abnormalities occur, defects in proteins encoded by TUBA1A, RELN, and very-low-density lipoprotein receptor (VLDLR) genes have been reported. We present a neonate with a severe neurologic phenotype associated with hypotonia, oropharyngeal incoordination that required a gastric tube for feeding, intractable epilepsy, and congenital cataracts. Her brain magnetic resonance imaging (MRI) showed classical lissencephaly, ventriculomegaly, absent corpus callosum, globular and vertical hippocampi, and severe cerebellar and brainstem hypoplasia. She died at 6 weeks of age. No specific molecular diagnosis was made. This likely represents a previously undescribed genetic lissencephaly syndrome.

  5. Occurrence of crossed cerebellar diaschisis in cerebrovascular disease

    Energy Technology Data Exchange (ETDEWEB)

    Biersack, H.J.; Hartmann, A.; Friedrich, G.; Froescher, M.; Reichmann, K.; Reske, S.N.; Knopp, R.

    1984-10-01

    In 31 patients with completed stroke (n = 30) or PRIND (n = 1) a brain SPECT with /sup 123/I-labeled amphetamines was performed. In 14 (= 45%) of the respective patients - suffering from long-lasting hemiplegia - crossed cerebellar diaschisis was present. The interval between onset of the disease and time of examination varied between 1 week and 7 years. On the other hand, patients without crossed cerebellar diaschisis did not, with one exception, suffer from hemiplegia. It is likely that this phenomenon is caused by the reduction of spino-cerebellar stimuli due to the paresis of the respective extremities.

  6. Cerebral venous thrombosis presenting with cerebellar ataxia and cortical blindness.

    Science.gov (United States)

    Ben Sassi, Samia; Mizouni, Habiba; Nabli, Fatma; Kallel, Lamia; Kefi, Mounir; Hentati, Fayçal

    2010-01-01

    Venous infarction in the cerebellum has been reported only rarely, probably because of the abundant venous collateral drainage in this region. Bilateral occipital infarction is a rare cause of visual loss in cerebral venous thrombosis. We describe a 50-year-old woman with a history of ulcerative colitis who developed acute cerebellar ataxia and cortical blindness. She had bilateral cerebellar and occipital lesions related to sigmoid venous thrombosis and achieved complete recovery with anticoagulation therapy. Cerebral venous thrombosis should be considered in cases of simultaneous cerebellar and occipital vascular lesions.

  7. Hereditary Cerebellar Ataxias: A Korean Perspective

    Directory of Open Access Journals (Sweden)

    Ji Sun Kim

    2015-05-01

    Full Text Available Hereditary ataxia is a heterogeneous disorder characterized by progressive ataxia combined with/without peripheral neuropathy, extrapyramidal symptoms, pyramidal symptoms, seizure, and multiple systematic involvements. More than 35 autosomal dominant cerebellar ataxias have been designated as spinocerebellar ataxia, and there are 55 recessive ataxias that have not been named systematically. Conducting genetic sequencing to confirm a diagnosis is difficult due to the large amount of subtypes with phenotypic overlap. The prevalence of hereditary ataxia can vary among countries, and estimations of prevalence and subtype frequencies are necessary for planning a diagnostic strategy in a specific population. This review covers the various hereditary ataxias reported in the Korean population with a focus on the prevalence and subtype frequencies as the clinical characteristics of the various subtypes.

  8. Classically conditioned postural reflex in cerebellar patients.

    Science.gov (United States)

    Kolb, F P; Lachauer, S; Maschke, M; Timmann, D

    2004-09-01

    The aim of the current study was to compare postural responses to repetitive platform-evoked perturbations in cerebellar patients with those of healthy subjects using a classical conditioning paradigm. The perturbations consisted of tilting of the platform (unconditioned stimulus: US) at random time intervals, preceded by an auditory signal that represented the conditioning stimulus (CS). Physiological reactions were recorded biomechanically by measuring the vertical ground forces, yielding the center of vertical pressure (CVP), and electrophysiologically by EMG measurements of the main muscle groups of both legs. The recording session consisted of a control section with US-alone trials, a testing section with paired stimuli and a brief final section with US-alone trials. Healthy control subjects were divided into those establishing conditioned responses (CR) in all muscles tested (strategy I) and those with CR in the gastrocnemius muscles only (strategy II), suggesting an associative motor-related process is involved. Patients with a diffuse, non-localized disease were almost unable to establish CR. This was also true for a patient with a focal surgical lesion with no CR on the affected side but who, simultaneously, showed an essentially normal CR incidence on the intact side. During US-alone trials healthy controls exhibited a remarkable decay of the UR amplitude due to a non-associative motor-related process such as habituation. The decay was most prominent in the paired trials section. In contrast, patients showed no significant differences in the UR amplitude throughout the entire recording session. Analysis of the CVP supported the electrophysiological findings, showing CR in the controls only. The differences between the responses of control subjects and those of the cerebellar patients imply strongly that the cerebellum is involved critically in controlling associative and non-associative motor-related processes.

  9. Intraductal papillary mucinous neoplasm of pancreas

    Directory of Open Access Journals (Sweden)

    Norman Oneil Machado

    2015-01-01

    Full Text Available Intraductal papillary mucinous neoplasms (IPMNs of the pancreas are neoplasms that are characterized by ductal dilation, intraductal papillary growth, and thick mucus secretion. This relatively recently defined pathology is evolving in terms of its etiopathogenesis, clinical features, diagnosis, management, and treatment guidelines. A PubMed database search was performed. All the relevant abstracts in English language were reviewed and the articles in which cases of IPMN could be identified were further scrutinized. Information of IPMN was derived, and duplication of information in several articles and those with areas of persisting uncertainties were excluded. The recent consensus guidelines were examined. The reported incidence of malignancy varies from 57% to 92% in the main duct-IPMN (MD-IPMN and from 6% to 46% in the branch duct-IPMN (BD-IPMN. The features of high-risk malignant lesions that raise concern include obstructive jaundice in a patient with a cystic lesion in the pancreatic head, the findings on radiological imaging of a mass lesion of >30 mm, enhanced solid component, and the main pancreatic duct (MPD of size ≥10 mm; while duct size 5-9 mm and cyst size <3 mm are considered as "worrisome features." Magnetic resonance imaging (MRI and endoscopic ultrasound (EUS are primary investigations in diagnosing and following up on these patients. The role of pancreatoscopy and the analysis of aspirated cystic fluid for cytology and DNA analysis is still to be established. In general, resection is recommended for most MD-IPMN, mixed variant, and symptomatic BD-IPMN. The 5-year survival of patients after surgical resection for noninvasive IPMN is reported to be at 77-100%, while for those with invasive carcinoma, it is significantly lower at 27-60%. The follow-up of these patients could vary from 6 months to 1 year and would depend on the risk stratification for invasive malignancy and the pathology of the resected specimen. The

  10. Cerebellar giant cell glioblastoma multiforme in an adult

    Directory of Open Access Journals (Sweden)

    Sudhansu Sekhar Mishra

    2014-01-01

    Full Text Available Cerebellar glioblastoma multiforme (GBM is a rare tumor that accounts for only 1% of all cases of GBM and its giant cell variant is even much rarely encountered in adults. A case of cerebellar giant cell GBM managed at our institution reporting its clinical presentation, radiological and histological findings, and treatment instituted is described. In conjunction, a literature review, including particular issues, clinical data, advances in imaging studies, pathological characteristics, treatment options, and the behavior of such malignant tumor is presented. It is very important for the neurosurgeon to make the differential diagnosis between the cerebellar GBM, and other diseases such as metastasis, anaplastic astrocytomas, and cerebellar infarct because their treatment modalities, prognosis, and outcome are different.

  11. Bilateral cerebellar activation in unilaterally challenged essential tremor

    Directory of Open Access Journals (Sweden)

    Marja Broersma

    2016-01-01

    Conclusions: Our results expand on previous findings of bilateral cerebellar involvement in ET. We have identified specific areas in the bilateral somatomotor regions of the cerebellum: lobules V, VI and VIII.

  12. Cerebellar infarct patterns: The SMART-Medea study

    Directory of Open Access Journals (Sweden)

    Laurens J.L. De Cocker, MD

    2015-01-01

    Conclusions: Small cerebellar infarcts proved to be much more common than larger infarcts, and preferentially involved the cortex. Small cortical infarcts predominantly involved the posterior lobes, showed sparing of subcortical white matter and occurred in characteristic topographic patterns.

  13. Anomalous cerebellar anatomy in Chinese children with dyslexia

    Directory of Open Access Journals (Sweden)

    Ying-Hui eYang

    2016-03-01

    Full Text Available The cerebellar deficit hypothesis for developmental dyslexia (DD claims that cerebellar dysfunction causes the failures in the acquisition of visuomotor skills and automatic reading and writing skills. In people with dyslexia in the alphabetic languages, the abnormal activation and structure of the right or bilateral cerebellar lobes have been identified. Using a typical implicit motor learning task, however, one neuroimaging study demonstrated the left cerebellar dysfunction in Chinese children with dyslexia. In the present study, using voxel-based morphometry, we found decreased gray matter volume in the left cerebellum in Chinese children with dyslexia relative to age-matched controls. The positive correlation between reading performance and regional gray matter volume suggests that the abnormal structure in the left cerebellum is responsible for reading disability in Chinese children with dyslexia.

  14. Cerebellar blood flow in methylmercury poisoning (Minamata disease)

    Energy Technology Data Exchange (ETDEWEB)

    Itoh, K.; Korogi, Y.; Tomiguchi, S.; Takahashi, M. [Dept. of Radiology, Kumamoto University School of Medicine (Japan); Okajima, T. [Dept. of Neurology, Johnan Hospital, Maihara, Johnan-mochi (Japan); Sato, H. [Dept. of Neurology, Minamata City General Hospital and Medical Centre (Japan)

    2001-04-01

    We looked at regional cerebellar blood flow in patients with Minamata disease (MD) using technetium-99 m ethyl cysteinate dimer (99m-Tc-ECD). We carried out single-photon emission computed tomography (SPECT) on 15 patients with MD (eight men, seven women, aged 51-78 years, mean 70.5 years) and 11 control subjects (eight men, three women, aged 62-80 years, mean 72.5 years). Regional blood flow was measured in the superior, middle, and inferior portions of the cerebellar hemispheres, and the frontal, temporal and occipital cerebral lobes. The degree of cerebellar atrophy was assessed on MRI. There were significant differences in regional blood flow in all parts of the cerebellum between patients and control, but no significant decrease was observed in the cerebrum. Blood flow was lower in the inferior cerebellum than in the other parts. Even in patients without cerebellar atrophy, flow was significantly decreased regional blood flow in the inferior part. (orig.)

  15. Cerebellar blood flow in methylmercury poisoning (Minamata disease).

    Science.gov (United States)

    Itoh, K; Korogi, Y; Tomiguchi, S; Takahashi, M; Okajima, T; Sato, H

    2001-04-01

    We looked at regional cerebellar blood flow in patients with Minamata disease (MD) using technetium-99m ethyl cysteinate dimer (99m-Tc-ECD). We carried out single-photon emission computed tomography (SPECT) on 15 patients with MD (eight men, seven women, aged 51-78 years, mean 70.5 years) and 11 control subjects (eight men, three women, aged 62-80 years, mean 72.5 years). Regional blood flow was measured in the superior, middle, and inferior portions of the cerebellar hemispheres, and the frontal, temporal and occipital cerebral lobes. The degree of cerebellar atrophy was assessed on MRI. There were significant differences in regional blood flow in all parts of the cerebellum between patients and control, but no significant decrease was observed in the cerebrum. Blood flow was lower in the inferior cerebellum than in the other parts. Even in patients without cerebellar atrophy, flow was significantly decreased regional blood flow in the inferior part.

  16. Cerebellar transcranial direct current stimulation in neurological disease

    OpenAIRE

    Ferrucci, Roberta; Bocci, Tommaso; Cortese, Francesca; Ruggiero, Fabiana; Priori, Alberto

    2016-01-01

    Several studies have highlighted the therapeutic potential of transcranial direct current stimulation (tDCS) in patients with neurological diseases, including dementia, epilepsy, post-stroke dysfunctions, movement disorders, and other pathological conditions. Because of this technique’s ability to modify cerebellar excitability without significant side effects, cerebellar tDCS is a new, interesting, and powerful tool to induce plastic modifications in the cerebellum. In this report, we review...

  17. Oxidative Stress in Autism: Elevated Cerebellar 3-nitrotyrosine Levels

    Directory of Open Access Journals (Sweden)

    Elizabeth M. Sajdel-Sulkowska

    2008-01-01

    Full Text Available It has been suggested that oxidative stress and/or mercury compounds play an important role in the pathophysiology of autism. This study compared for the first time the cerebellar levels of the oxidative stress marker 3-nitrotyrosine (3-NT, mercury (Hg and the antioxidant selenium (Se levels between control and autistic subjects. Tissue homogenates were prepared in the presence of protease inhibitors from the frozen cerebellar tissue of control (n=10; mean age, 15.5 years; mean PMI, 15.5 hours and autistic (n=9; mean age 12.1 years; mean PMI, 19.3 hours subjects. The concentration of cerebellar 3-NT, determined by ELISA, in controls ranged from 13.69 to 49.04 pmol g-1 of tissue; the concentration of 3-NT in autistic cases ranged from 3.91 to 333.03 pmol g-1 of tissue. Mean cerebellar 3-NT was elevated in autism by 68.9% and the increase was statistically significant (p=0.045. Cerebellar Hg, measured by atomic absorption spectrometry ranged from 0.9 to 35 pmol g-1 tissue in controls (n=10 and from 3.2 to 80.7 pmol g-1 tissue in autistic cases (n=9; the 68.2% increase in cerebellar Hg was not statistically significant. However, there was a positive correlation between cerebellar 3-NT and Hg levels (r=0.7961, p=0.0001. A small decrease in cerebellar Se levels in autism, measured by atomic absorption spectroscopy, was not statistically significant but was accompanied by a 42.9% reduction in the molar ratio of Se to Hg in the autistic cerebellum. While preliminary, the results of the present study add elevated oxidative stress markers in brain to the growing body of data reflecting greater oxidative stress in autism.

  18. Eponyms in cardiothoracic radiology: Part I. Neoplasms.

    Science.gov (United States)

    Mohammed, Tan-Lucien H; Saettele, Megan R; Saettele, Timothy; Patel, Vikas; Kanne, Jeffrey P

    2014-01-01

    Eponyms serve the purpose of honoring individuals who have made important observations and discoveries. As with other fields of medicine, eponyms are frequently encountered in radiology, particularly in chest radiology. However, inappropriate use of an eponym may lead to potentially dangerous miscommunication. Moreover, an eponym may honor the incorrect person or a person who falls into disrepute. Despite their limitations, eponyms are still widespread in medical literature. Furthermore, in some circumstances, more than one individual may have contributed to the description or discovery of a particular anatomical structure or disease, whereas in others, an eponym may have been incorrectly applied initially and propagated for years in medical literature. Nevertheless, radiologic eponyms are a means of honoring those who have made lasting contributions to the field of radiology, and familiarity with these eponyms is critical for proper reporting and accurate communication. In addition, the acquisition of some historical knowledge about those whose names are associated with various structures or pathologic conditions conveys a sense of humanity in the field of medicine. In this article, the first of a multipart series, the authors discuss a number of chest radiology eponyms as they relate to neoplasms, including relevant clinical and imaging features, as well biographic information of the respective eponym׳s namesake.

  19. Future therapies for the myeloproliferative neoplasms.

    Science.gov (United States)

    Scherber, Robyn; Mesa, Ruben A

    2011-03-01

    Ever since their description as "myeloproliferative syndromes" by William Dameshek in 1951, the myeloproliferative neoplasms (MPNs) have been managed by the selective use of rather mundane, nonspecific therapies that rely on either antiplatelet effects or myelosuppression. The year 2005 ushered in a new era of drug development and discovery for the MPNs after the description of the JAK2 V617F mutation and the role this constitutively active tyrosine kinase has in MPN pathogenesis. Subsequently, multiple pharmacologic agents have begun (or are about to begin) testing for the inhibition of JAK2 in an attempt to improve the treatment of MPNs. Both primary myelofibrosis and myelofibrosis following essential thrombocythemia or polycythemia vera have been the targets of the most extensive testing of these agents to date. Responses to these oral JAK2 inhibitors have been primarily intended to reduce splenomegaly and meaningfully improve symptoms; effects on the JAK2 V617F allele burden or marrow histology are limited. Toxicities have ranged from myelosuppression to significant diarrhea. Additional agents with other mechanisms of action are also targeting JAK2, including histone deacetylase inhibitors and mTOR inhibitors. The results of preliminary trials of JAK2 inhibitors in polycythemia vera and essential thrombocythemia have been mixed but are premature. Many questions remain as to the optimal JAK2 inhibitory strategy and the full extent of the benefit of single-agent JAK2 inhibition.

  20. Origin and Molecular Pathology of Adrenocortical Neoplasms

    Science.gov (United States)

    Bielinska, M.; Parviainen, H.; Kiiveri, S.; Heikinheimo, M.; Wilson, D.B.

    2008-01-01

    Neoplastic adrenocortical lesions are common in humans and several species of domestic animals. Although there are unanswered questions about the origin and evolution of adrenocortical neoplasms, analysis of human tumor specimens and animal models indicates that adrenocortical tumorigenesis involves both genetic and epigenetic alterations. Chromosomal changes accumulate during tumor progression, and aberrant telomere function is one of the key mechanisms underlying chromosome instability during this process. Epigenetic changes serve to expand the size of the uncommitted adrenal progenitor population, modulate their phenotypic plasticity (i.e., responsiveness to extracellular signals), and increase the likelihood of subsequent genetic alterations. Analyses of heritable and spontaneous types of human adrenocortical tumors have documented alterations in either cell surface receptors or their downstream effectors that impact neoplastic transformation. Many of the mutations associated with benign human adrenocortical tumors result in dysregulated cyclic AMP signaling, whereas key factors/signaling pathways associated with adrenocortical carcinomas include dysregulated expression of the IGF2 gene cluster, activation of the Wnt/β-catenin pathway, and inactivation of the p53 tumor suppressor. A better understanding of the factors and signaling pathways involved in adrenal tumorigenesis is necessary to develop targeted pharmacologic and genetic therapies. PMID:19261630

  1. Adrenocortical oncocytic neoplasm presenting with Cushing's syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Kabayegit Ozlem

    2008-07-01

    Full Text Available Abstract Introduction Oncocytic neoplasms occur in several organs and are most commonly found in the thyroid, kidneys and salivary glands. Oncocytic neoplasms of the adrenal cortex are extremely rare and are usually non-functioning. Case presentation We report the case of an adrenocortical oncocytic neoplasm with uncertain malignant potential in a 31-year-old man with Cushing's syndrome. The patient had been operated on following diagnosis of a 7 cm adrenal mass. Following surgery, the Cushing's syndrome resolved. The patient is still alive with no metastases one year after the surgery. Conclusion Adrenocortical oncocytic neoplasms must be considered in the differential diagnosis of both functioning and non-functioning adrenal masses.

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  13. File list: NoD.Prs.20.AllAg.Prostatic_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available NoD.Prs.20.AllAg.Prostatic_Neoplasms mm9 No description Prostate Prostatic Neoplasm...s http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/NoD.Prs.20.AllAg.Prostatic_Neoplasms.bed ...

  14. Proprioceptive Localization Deficits in People With Cerebellar Damage.

    Science.gov (United States)

    Weeks, Heidi M; Therrien, Amanda S; Bastian, Amy J

    2017-04-01

    It has been hypothesized that an important function of the cerebellum is predicting the state of the body during movement. Yet, the extent of cerebellar involvement in perception of limb state (i.e., proprioception, specifically limb position sense) has yet to be determined. Here, we investigated whether patients with cerebellar damage have deficits when trying to locate their hand in space (i.e., proprioceptive localization), which is highly important for everyday movements. By comparing performance during passive robot-controlled and active self-made multi-joint movements, we were able to determine that some cerebellar patients show improved precision during active movement (i.e., active benefit), comparable to controls, whereas other patients have reduced active benefit. Importantly, the differences in patient performance are not explained by patient diagnosis or clinical ratings of impairment. Furthermore, a subsequent experiment confirmed that active deficits in proprioceptive localization occur during both single-joint and multi-joint movements. As such, it is unlikely that localization deficits can be explained by the multi-joint coordination deficits occurring after cerebellar damage. Our results suggest that cerebellar damage may cause varied impairments to different elements of proprioceptive sense. It follows that proprioceptive localization should be adequately accounted for in clinical testing and rehabilitation of people with cerebellar damage.

  15. Plurihormonal Cosecretion by a Case of Adrenocortical Oncocytic Neoplasm

    Directory of Open Access Journals (Sweden)

    J. J. Corrales

    2016-01-01

    Full Text Available Adrenocortical oncocytic neoplasms (oncocytomas are extremely rare; only approximately 159 cases have been described so far. The majority are nonfunctional and benign. We describe an unusual case of a functional oncocytoma secreting an excess of glucocorticoids (cortisol and androgens (androstenedione and DHEAS, a pattern of plurihormonal cosecretion previously not reported in men, presenting with endocrine manifestations of Cushing’s syndrome. The neoplasm was considered to be of uncertain malignant potential (borderline according to the Lin-Weiss-Bisceglia criteria.

  16. Morbidity and mortality of malignant neoplasms in Macedonia

    OpenAIRE

    Vukovikj, Viktorija; Markovski, Velo

    2015-01-01

    Introductions: Malignant neoplasms are the second cause of death among the population in Republic of Macedonia with representation of and represent 19.0% in the structure of total deaths. Objective: To analyze the morbidity and mortality of the most common malignant neoplasms in Republic of Macedonia. Material and methods: Were used a data from the Institute of Public Health of the Republic of Macedonia, National institute for statistic of Republic Macedonia. Results and discussions:...

  17. Relationship between the cerebellar function and cerebellar atrophy in Minamata disease. Investigations using body balance analyzer and MR imaging method

    Energy Technology Data Exchange (ETDEWEB)

    Okajima, Toru [Johnan Hospital, Minami, Kumamoto (Japan); Ikeda, Osamu; Sannomiya, Kunihiro; Korogi, Yukinori; Uchino, Makoto

    1995-11-01

    Interrelations between the cerebellar function and cerebellar atrophy were studied in the cases with Minamata disease and spinocerebellar degeneration and in the healthy subjects. For evaluation of the cerebellar function, the statokinesigraph (SKG) was recorded and the shifting length (L-SKG) and moving area (A-SKG) of postural sway were obtained using body balance analyzer. Cerebellar atrophy was evaluated by the rostrocaudal and ventrodorsal diameters of whole vermis and the total area of upper and lower parts (area-UL) of vermis on the midsagittal plane of MR imaging. It was disclosed that there was significant correlation between the L-SKG and the measurement of rostrocaudal diameter as well as the area-UL of vermis through the patients with Minamata disease and the healthy subjects. When added the patients with spinocerebellar degeneration, the significant correlation was not obtainable probably because of the progressive processes of the disease. (author).

  18. Origin of B-Cell Neoplasms in Autoimmune Disease.

    Directory of Open Access Journals (Sweden)

    Kari Hemminki

    Full Text Available Autoimmune diseases (ADs are associated with a number of B-cell neoplasms but the associations are selective in regard to the type of neoplasm and the conferred risks are variable. So far no mechanistic bases for these differential associations have been demonstrated. We speculate that developmental origin of B-cells might propose a mechanistic rationale for their carcinogenic response to autoimmune stimuli and tested the hypothesis on our previous studies on the risks of B-cell neoplasms after any of 33 ADs. We found that predominantly germinal center (GC-derived B-cells showed multiple associations with ADs: diffuse large B cell lymphoma associated with 15 ADs, follicular lymphoma with 7 ADs and Hodgkin lymphoma with 11 ADs. Notably, these neoplasms shared significant associations with 5 ADs (immune thrombocytopenic purpura, polymyositis/dermatomyositis, rheumatoid arthritis, Sjogren syndrome and systemic lupus erythematosis. By contrast, primarily non-GC neoplasms, acute lymphocytic leukemia, chronic lymphocytic leukemia and myeloma associated with 2 ADs only and mantle cell lymphoma with 1 AD. None of the neoplasms shared associated ADs. These data may suggest that autoimmune stimulation critically interferes with the rapid cell division, somatic hypermutation, class switch recombination and immunological selection of maturing B-cell in the GC and delivers damage contributing to transformation.

  19. CT characteristics of primary retroperitoneal neoplasms in children

    Energy Technology Data Exchange (ETDEWEB)

    Xu Yufeng; Wang Jichen [Department of Radiology, Peking University First Hospital, No. 8, Xishike Street, Xicheng District, Beijing 100034 (China); Peng Yun [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China); Zeng Jinjin, E-mail: jzeng5567@yahoo.co [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China)

    2010-09-15

    Primary retroperitoneal neoplasms are uncommon in children. Retroperitoneal neoplasms are either mesodermal, neurogenic, germ cell ectodermal or lymphatic in origin. In general, primary retroperitoneal neoplasms in children have different spectrum and prevalence compared to those in adults. Neuroblastoma, rhabdomyosarcoma, benign teratoma and lymphoma are the common retroperitoneal neoplasms. In this review, the clinical and CT futures of common retroperitoneal neoplasms in children are described. Coarse, amorphous, and mottled calcification are very common in neuroblastoma. Paraganglioma tends to show marked and early enhancement and may present with clinical symptoms associated with the excess catecholamine. Sarcomas are often very large and have heterogeneous appearance. Imaging cannot be reliably used to identify the type of retroperitoneal sarcomas due to overlapped radiographic features. In children, lipoblastoma is the most common lipomatous tumor in the retroperitoneum. The percentage of visible fat in tumor varies depending on the cellular composition of the lesion. The CT characteristics of teratoma are quite variable, which may be cystic, solid, on a combination of both. Typically teratoma appears as a large complex mass containing fluid, fat, fat-fluid level, and calcifications. Lymphoma is often homogeneous on both enhanced and unenhanced CT scans. Necrosis and calcification are rare on CT. In conclusion, making a final histological diagnosis of retroperitoneal tumor base on CT features is not often possible; however, CT can help to develop a differential diagnosis and determine the size and extent of the retroperitoneal neoplasms.

  20. Mucins in the diagnosis and differential diagnosis of pancreatic cystic neoplasms: report of 40 cases

    Institute of Scientific and Technical Information of China (English)

    JI Yuan; TAN Yun-shan; XU Jian-fang; QI Wei-dong; LI Xiao-ping; SU-JIE Ake-su; ZHU Xiong-zeng

    2006-01-01

    @@ Cystic neoplasms of the pancreas account for 10% to 15% of all cystic pancreatic lesions.The majority (85% to 90%) of cystic lesions of the pancreas are pseudocysts. Although cystic neoplasms of the pancreas are rare, they range from benign to malignant neoplasms. The clinical challenge is the differential diagnosis and management of the cystic neoplasms, which represent 10% to 25% of primary pancreatic neoplasms. Pancreatic neoplasms and tumour like lesions with cystic features have been recently reviewed. The incidence of pancreatic cystic neoplasms reported is variable. Because there is no large, systematic study on tne cases from China comparing the incidence and biology of cystic neoplasms of pancreas to that of Western series, we reviewed all the cases of cystic neoplasms from Zhongshan Hospital over 6 years. Most of the neoplasms in our series were classified according to the recent World Health Organization (WHO)classification.1,2

  1. Cerebellar Ataxia and Glutamic Acid Decarboxylase Antibodies

    Science.gov (United States)

    Ariño, Helena; Gresa-Arribas, Nuria; Blanco, Yolanda; Martínez-Hernández, Eugenia; Sabater, Lidia; Petit-Pedrol, Mar; Rouco, Idoia; Bataller, Luis; Dalmau, Josep O.; Saiz, Albert; Graus, Francesc

    2016-01-01

    IMPORTANCE Current clinical and immunologic knowledge on cerebellar ataxia (CA) with glutamic acid decarboxylase 65 antibodies (GAD65-Abs) is based on case reports and small series with short-term follow-up data. OBJECTIVE To report the symptoms, additional antibodies, prognostic factors, and long-term outcomes in a cohort of patients with CA and GAD65-Abs. DESIGN, SETTING, AND PARTICIPANTS Retrospective cohort study and laboratory investigations at a center for autoimmune neurologic disorders among 34 patients with CA and GAD65-Abs, including 25 with long-term follow-up data (median, 5.4 years; interquartile range, 3.1-10.3 years). MAIN OUTCOMES AND MEASURES Analysis of clinicoimmunologic features and predictors of response to immunotherapy. Immunochemistry on rat brain, cultured neurons, and human embryonic kidney cells expressing GAD65, GAD67, α1-subunit of the glycine receptor, and a repertoire of known cell surface autoantigens were used to identify additional antibodies. Twenty-eight patients with stiff person syndrome and GAD65-Abs served as controls. RESULTS The median age of patients was 58 years (range, 33-80 years); 28 of 34 patients (82%) were women. Nine patients (26%) reported episodes of brainstem and cerebellar dysfunction or persistent vertigo several months before developing CA. The clinical presentation was subacute during a period of weeks in 13 patients (38%). Nine patients (26%) had coexisting stiff person syndrome symptoms. Systemic organ-specific autoimmunities (type 1 diabetes mellitus and others) were present in 29 patients (85%). Twenty of 25 patients with long-term follow-up data received immunotherapy (intravenous immunoglobulin in 10 and corticosteroids and intravenous immunoglobulin or other immunosuppressors in 10), and 7 of them (35%) improved. Predictors of clinical response included subacute onset of CA (odds ratio [OR], 0.50; 95% CI, 0.25-0.99; P = .047) and prompt immunotherapy (OR, 0.98; 95% CI, 0.96-0.99; P = .01). Similar

  2. Imaging calcium waves in cerebellar Bergmann glia.

    Science.gov (United States)

    Beierlein, Michael

    2013-01-01

    This protocol describes methods for recording synaptically evoked Ca(2+) waves from individual Bergmann glia (BG) in slices of cerebellar cortex. Unlike protoplasmic, star-shaped astrocytes, whose thin processes pose a serious challenge to stable Ca(2+) measurements, BG are large radial cells, with several main processes that run over distances of several hundred micrometers toward the pia and ensheathe thousands of parallel fiber (PF) synapses. Stimulation of PF synapses with brief bursts can trigger long-lasting Ca(2+) responses in BG processes, which can be reliably recorded using a cooled charge-coupled device (CCD) camera. This protocol was developed to enable measurements of Ca(2+) waves in individual BG loaded with a high-affinity Ca(2+) indicator such as Fura-2 for up to 2 h. Because BG recorded in slices rarely display spontaneous (i.e., tetrodotoxin [TTX]-sensitive) or intrinsic Ca(2+) transients, Ca(2+) waves can be evoked repeatedly and reliably, which permits quantitative studies using pharmacological tools. Fluorescence measurements obtained using CCD technology offer a straightforward means of characterizing the mechanisms and potential functional consequences of widespread and long-lasting, store-mediated Ca(2+) increases in astrocytes.

  3. Electrophysiological mapping of novel prefrontal - cerebellar pathways

    Directory of Open Access Journals (Sweden)

    Thomas C Watson

    2009-08-01

    Full Text Available Whilst the cerebellum is predominantly considered a sensorimotor control structure, accumulating evidence suggests that it may also subserve non motor functions during cognition. However, this possibility is not universally accepted, not least because the nature and pattern of links between higher cortical structures and the cerebellum are poorly characterized. We have therefore used in vivo electrophysiological methods in anaesthetized rats to directly investigate connectivity between the medial prefrontal cortex (prelimbic subdivision, PrL and the cerebellum. Stimulation of deep layers of PrL evoked distinct field potentials in the cerebellar cortex with a mean latency to peak of approximately 35ms. These responses showed a well-defined topography, and were maximal in lobule VII of the contralateral vermis (a known oculomotor centre; they were not attenuated by local anesthesia of the overlying M2 motor cortex, though M2 stimulation did evoke field potentials in lobule VII with a shorter latency. Single-unit recordings showed that prelimbic cortical stimulation elicits complex spikes in lobule VII Purkinje cells, indicating transmission via a previously undescribed cerebro-olivocerebellar pathway. Our results therefore establish a physiological basis for communication between PrL and the cerebellum. The role(s of this pathway remain to be resolved, but presumably relate to control of eye movements and/or distributed networks associated with integrated prefrontal cortical functions.

  4. Sudden stopping in patients with cerebellar ataxia.

    Science.gov (United States)

    Serrao, Mariano; Conte, Carmela; Casali, Carlo; Ranavolo, Alberto; Mari, Silvia; Di Fabio, Roberto; Perrotta, Armando; Coppola, Gianluca; Padua, Luca; Monamì, Stefano; Sandrini, Giorgio; Pierelli, Francesco

    2013-10-01

    Stopping during walking, a dynamic motor task frequent in everyday life, is very challenging for ataxic patients, as it reduces their gait stability and increases the incidence of falls. This study was conducted to analyse the biomechanical characteristics of upper and lower body segments during abrupt stopping in ataxic patients in order to identify possible strategies used to counteract the instability in the sagittal and frontal plane. Twelve patients with primary degenerative cerebellar ataxia and 12 age- and sex-matched healthy subjects were studied. Time-distance parameters, dynamic stability of the centre of mass, upper body measures and lower joint kinematic and kinetic parameters were analysed. The results indicate that ataxic patients have a great difficulty in stopping abruptly during walking and adopt a multi-step stopping strategy, occasionally with feet parallel, to compensate for their inability to coordinate the upper body and to generate a well-coordinated lower limb joint flexor-extensor pattern and appropriate braking forces for progressively decelerating the progression of the body in the sagittal plane. A specific rehabilitation treatment designed to improve the ability of ataxic patients to transform unplanned stopping into planned stopping, to coordinate upper body and to execute an effective flexion-extension pattern of the hip and knee joints may be useful in these patients in order to improve their stopping performance and prevent falls.

  5. Ethanol-Induced Cerebellar Ataxia: Cellular and Molecular Mechanisms.

    Science.gov (United States)

    Dar, M Saeed

    2015-08-01

    The cerebellum is an important target of ethanol toxicity given that cerebellar ataxia is the most consistent physical manifestation of acute ethanol consumption. Despite the significance of the cerebellum in ethanol-induced cerebellar ataxia (EICA), the cellular and molecular mechanisms underlying EICA are incompletely understood. However, two important findings have shed greater light on this phenomenon. First, ethanol-induced blockade of cerebellar adenosine uptake in rodent models points to a role for adenosinergic A1 modulation of EICA. Second, the consistent observation that intracerebellar administration of nicotine in mice leads to antagonism of EICA provides evidence for a critical role of cerebellar nitric oxide (NO) in EICA reversal. Based on these two important findings, this review discusses the potential molecular events at two key synaptic sites (mossy fiber-granule cell-Golgi cell (MGG synaptic site) and granule cell parallel fiber-Purkinje cell (GPP synaptic site) that lead to EICA. Specifically, ethanol-induced neuronal NOS inhibition at the MGG synaptic site acts as a critical trigger for Golgi cell activation which leads to granule cell deafferentation. Concurrently, ethanol-induced inhibition of adenosine uptake at the GPP synaptic site produces adenosine accumulation which decreases glutamate release and leads to the profound activation of Purkinje cells (PCs). These molecular events at the MGG and GPP synaptic sites are mutually reinforcing and lead to cerebellar dysfunction, decreased excitatory output of deep cerebellar nuclei, and EICA. The critical importance of PCs as the sole output of the cerebellar cortex suggests normalization of PC function could have important therapeutic implications.

  6. Abnormal cerebellar volume in acute and remitted major depression.

    Science.gov (United States)

    Depping, Malte S; Wolf, Nadine D; Vasic, Nenad; Sambataro, Fabio; Hirjak, Dusan; Thomann, Philipp A; Wolf, Robert C

    2016-11-01

    Abnormal cortical volume is well-documented in patients with major depressive disorder (MDD), but cerebellar findings have been heterogeneous. It is unclear whether abnormal cerebellar structure relates to disease state or medication. In this study, using structural MRI, we investigated cerebellar volume in clinically acute (with and without psychotropic treatment) and remitted MDD patients. High-resolution structural MRI data at 3T were obtained from acute medicated (n=29), acute unmedicated (n=14) and remitted patients (n=16). Data from 29 healthy controls were used for comparison purposes. Cerebellar volume was investigated using cerebellum-optimized voxel-based analysis methods. Patients with an acute MDD episode showed increased volume of left cerebellar area IX, and this was true for both medicated and unmedicated individuals (pvolume. In remitted, but not in acutely ill patients, area IX volume was significantly associated with measures of depression severity, as assessed by the Hamilton Depression Rating Scale (HAMD). In addition, area IX volume in remitted patients was significantly related to the duration of antidepressant treatment. In acutely ill patients, no significant relationships were established using clinical variables, such as HAMD, illness or treatment duration and number of depressive episodes. The data suggest that cerebellar area IX, a non-motor region that belongs to a large-scale brain functional network with known relevance to core depressive symptom expression, exhibits abnormal volume in patients independent of clinical severity or medication. Thus, the data imply a possible trait marker of the disorder. However, given bilaterality and an association with clinical scores at least in remitted patients, the current findings raise the possibility that cerebellar volume may be reflective of successful treatment as well.

  7. Cerebellar cognitive affective syndrome CCAS – a case report

    Directory of Open Access Journals (Sweden)

    Starowicz-Filip, Anna

    2013-09-01

    Full Text Available Aim. The aim of the study was to describe a case of the patient with cerebellar cognitive affective syndrome CCAS, characterize the role of cerebellum in the regulation of cognitive functions and present theprocedure of neuropsychological diagnosis useful in indicating the specific cognitive and emotional problems in patients with cerebellar damage.Case report. A 41- year old man with an ischemic cerebellar stroke of its right hemisphere manifested the neuropsychological symptoms typical for the frontal damage: euphoric mood, disorganized behavior,lack of criticism and mental plasticity, tendency to shorten the personal distance, problems with mistake correction. In neuropsychological diagnosis we used following methods: Raven Progressive Matrices Test, Mini Mental Stage Examination (MMSE, Trail Making Test, Wisconsin Card Sorting Test, Stroop Interference Test, Word Fluency Test, Auditory Verbal Learning Test by Łuria, Benton Visual Retention Test, Digit Span.Results. Analyzing the obtained results we observed the significant decrease of all executive functions: planning, abstract thinking, cognitive flexibility, adaptation to new situations as well as memory impairments and changes in emotional and behavioral state similar to frontal syndrome. The whole of impairments including the typical cerebellar symptoms (ataxia, dysarthria, dysmetria,hypotonia create the cerebellar cognitive affective syndrome CCAS with leading role of dysexecutive syndrome.Conclusions. The cerebellum takes part in the regulation of cognitive functions. The cerebellar damages can imitate the emotional- cognitive problems of patients after frontal damages what additionally stress the functional link between these two brain structures. Patient’s with cerebellar damages should have neuropsychological and neuropsychiatric diagnosis and care.

  8. A cerebellar neuroprosthetic system: computational architecture and in vivo experiments

    Directory of Open Access Journals (Sweden)

    Ivan eHerreros Alonso

    2014-05-01

    Full Text Available Emulating the input-output functions performed by a brain structure opens the possibility for developing neuro-prosthetic systems that replace damaged neuronal circuits. Here, we demonstrate the feasibility of this approach by replacing the cerebellar circuit responsible for the acquisition and extinction of motor memories. Specifically, we show that a rat can undergo acquisition, retention and extinction of the eye-blink reflex even though the biological circuit responsible for this task has been chemically inactivated via anesthesia. This is achieved by first developing a computational model of the cerebellar microcircuit involved in the acquisition of conditioned reflexes and training it with synthetic data generated based on physiological recordings. Secondly, the cerebellar model is interfaced with the brain of an anesthetized rat, connecting the model's inputs and outputs to afferent and efferent cerebellar structures. As a result, we show that the anesthetized rat, equipped with our neuro-prosthetic system, can be classically conditioned to the acquisition of an eye-blink response. However, non-stationarities in the recorded biological signals limit the performance of the cerebellar model. Thus, we introduce an updated cerebellar model and validate it with physiological recordings showing that learning becomes stable and reliable. The resulting system represents an important step towards replacing lost functions of the central nervous system via neuro-prosthetics, obtained by integrating a synthetic circuit with the afferent and efferent pathways of a damaged brain region. These results also embody an early example of science-based medicine, where on the one hand the neuro-prosthetic system directly validates a theory of cerebellar learning that informed the design of the system, and on the other one it takes a step towards the development of neuro-prostheses that could recover lost learning functions in animals and, in the longer term

  9. Cerebellar development in the absence of Gbx function in zebrafish.

    Science.gov (United States)

    Su, Chen-Ying; Kemp, Hilary A; Moens, Cecilia B

    2014-02-01

    The midbrain-hindbrain boundary (MHB) is a well-known organizing center during vertebrate brain development. The MHB forms at the expression boundary of Otx2 and Gbx2, mutually repressive homeodomain transcription factors expressed in the midbrain/forebrain and anterior hindbrain, respectively. The genetic hierarchy of gene expression at the MHB is complex, involving multiple positive and negative feedback loops that result in the establishment of non-overlapping domains of Wnt1 and Fgf8 on either side of the boundary and the consequent specification of the cerebellum. The cerebellum derives from the dorsal part of the anterior-most hindbrain segment, rhombomere 1 (r1), which undergoes a distinctive morphogenesis to give rise to the cerebellar primordium within which the various cerebellar neuron types are specified. Previous studies in the mouse have shown that Gbx2 is essential for cerebellar development. Using zebrafish mutants we show here that in the zebrafish gbx1 and gbx2 are required redundantly for morphogenesis of the cerebellar primordium and subsequent cerebellar differentiation, but that this requirement is alleviated by knocking down Otx. Expression of fgf8, wnt1 and the entire MHB genetic program is progressively lost in gbx1-;gbx2- double mutants but is rescued by Otx knock-down. This rescue of the MHB genetic program depends on rescued Fgf signaling, however the rescue of cerebellar primordium morphogenesis is independent of both Gbx and Fgf. Based on our findings we propose a revised model for the role of Gbx in cerebellar development.

  10. Somatic CALR Mutations in Myeloproliferative Neoplasms with Nonmutated JAK2

    Science.gov (United States)

    Baxter, E.J.; Nice, F.L.; Gundem, G.; Wedge, D.C.; Avezov, E.; Li, J.; Kollmann, K.; Kent, D.G.; Aziz, A.; Godfrey, A.L.; Hinton, J.; Martincorena, I.; Van Loo, P.; Jones, A.V.; Guglielmelli, P.; Tarpey, P.; Harding, H.P.; Fitzpatrick, J.D.; Goudie, C.T.; Ortmann, C.A.; Loughran, S.J.; Raine, K.; Jones, D.R.; Butler, A.P.; Teague, J.W.; O’Meara, S.; McLaren, S.; Bianchi, M.; Silber, Y.; Dimitropoulou, D.; Bloxham, D.; Mudie, L.; Maddison, M.; Robinson, B.; Keohane, C.; Maclean, C.; Hill, K.; Orchard, K.; Tauro, S.; Du, M.-Q.; Greaves, M.; Bowen, D.; Huntly, B.J.P.; Harrison, C.N.; Cross, N.C.P.; Ron, D.; Vannucchi, A.M.; Papaemmanuil, E.; Campbell, P.J.; Green, A.R.

    2014-01-01

    BACKGROUND Somatic mutations in the Janus kinase 2 gene (JAK2) occur in many myeloproliferative neoplasms, but the molecular pathogenesis of myeloproliferative neoplasms with nonmutated JAK2 is obscure, and the diagnosis of these neoplasms remains a challenge. METHODS We performed exome sequencing of samples obtained from 151 patients with myeloproliferative neoplasms. The mutation status of the gene encoding calreticulin (CALR) was assessed in an additional 1345 hematologic cancers, 1517 other cancers, and 550 controls. We established phylogenetic trees using hematopoietic colonies. We assessed calreticulin subcellular localization using immunofluorescence and flow cytometry. RESULTS Exome sequencing identified 1498 mutations in 151 patients, with medians of 6.5, 6.5, and 13.0 mutations per patient in samples of polycythemia vera, essential thrombocythemia, and myelofibrosis, respectively. Somatic CALR mutations were found in 70 to 84% of samples of myeloproliferative neoplasms with nonmutated JAK2, in 8% of myelodysplasia samples, in occasional samples of other myeloid cancers, and in none of the other cancers. A total of 148 CALR mutations were identified with 19 distinct variants. Mutations were located in exon 9 and generated a +1 base-pair frameshift, which would result in a mutant protein with a novel C-terminal. Mutant calreticulin was observed in the endoplasmic reticulum without increased cell-surface or Golgi accumulation. Patients with myeloproliferative neoplasms carrying CALR mutations presented with higher platelet counts and lower hemoglobin levels than patients with mutated JAK2. Mutation of CALR was detected in hematopoietic stem and progenitor cells. Clonal analyses showed CALR mutations in the earliest phylogenetic node, a finding consistent with its role as an initiating mutation in some patients. CONCLUSIONS Somatic mutations in the endoplasmic reticulum chaperone CALR were found in a majority of patients with myeloproliferative neoplasms with

  11. Upregulation of cortico-cerebellar functional connectivity after motor learning.

    Science.gov (United States)

    Mehrkanoon, Saeid; Boonstra, Tjeerd W; Breakspear, Michael; Hinder, Mark; Summers, Jeffery J

    2016-03-01

    Interactions between the cerebellum and primary motor cortex are crucial for the acquisition of new motor skills. Recent neuroimaging studies indicate that learning motor skills is associated with subsequent modulation of resting-state functional connectivity in the cerebellar and cerebral cortices. The neuronal processes underlying the motor-learning-induced plasticity are not well understood. Here, we investigate changes in functional connectivity in source-reconstructed electroencephalography (EEG) following the performance of a single session of a dynamic force task in twenty young adults. Source activity was reconstructed in 112 regions of interest (ROIs) and the functional connectivity between all ROIs was estimated using the imaginary part of coherence. Significant changes in resting-state connectivity were assessed using partial least squares (PLS). We found that subjects adapted their motor performance during the training session and showed improved accuracy but with slower movement times. A number of connections were significantly upregulated after motor training, principally involving connections within the cerebellum and between the cerebellum and motor cortex. Increased connectivity was confined to specific frequency ranges in the mu- and beta-bands. Post hoc analysis of the phase spectra of these cerebellar and cortico-cerebellar connections revealed an increased phase lag between motor cortical and cerebellar activity following motor practice. These findings show a reorganization of intrinsic cortico-cerebellar connectivity related to motor adaptation and demonstrate the potential of EEG connectivity analysis in source space to reveal the neuronal processes that underpin neural plasticity.

  12. Development of the cerebellar cortex in the mouse

    Institute of Scientific and Technical Information of China (English)

    Xiangshu Cheng; Jin Du; Dongming Yu; Qiying Jiang; Yanqiu Hu; Lei Wang; Mingshan Li; Jinbo Deng

    2011-01-01

    The cerebellum is a highly conserved structure in the central nervous system of vertebrates, and is involved in the coordination of voluntary motor behavior. Supporting this function, the cerebellar cortex presents a layered structure which requires precise spatial and temporal coordination of proliferation, migration, differentiation, and apoptosis events. The formation of the layered structure in the developing cerebellum remains unclear. The present study investigated the development of the cerebellar cortex. The results demonstrate that the primordium of the cerebellum comprises the ependymal, mantle, and marginal layers at embryonic day 12 (E12). Subsequently, the laminated cerebellar cortex undergoes cell proliferation, differentiation, and migration, and at about postnatal day 0 (P0), the cerebellar cortex presents an external granular layer, a molecular layer, a Purkinje layer, and an internal granular layer. The external granular layer is thickest at P6/7 and disappears at P20. From P0 to P30, the internal granular cells and the Purkinje cells gradually differentiate and develop until maturity. Apoptotic neurons are evident in the layered structure in the developing cerebellar cortex. The external granular layer disappears gradually because of cell migration and apoptosis. The cells of the other layers primarily undergo differentiation, development, and apoptosis.

  13. Thalamic, brainstem, and cerebellar glucose metabolism in the hemiplegic monkey

    Energy Technology Data Exchange (ETDEWEB)

    Shimoyama, I.; Dauth, G.W.; Gilman, S.; Frey, K.A.; Penney, J.B. Jr.

    1988-12-01

    Unilateral ablation of cerebral cortical areas 4 and 6 of Brodmann in the macaque monkey results in a contralateral hemiplegia that resolves partially with time. During the phase of dense hemiplegia, local cerebral metabolic rate for glucose (1CMRG1c) is decreased significantly in most of the thalamic nuclei ipsilateral to the ablation, and there are slight contralateral decreases. The lCMRGlc is reduced bilaterally in most of the brainstem nuclei and bilaterally in the deep cerebellar nuclei, but only in the contralateral cerebellar cortex. During the phase of partial motor recovery, lCMRGlc is incompletely restored in many of the thalamic nuclei ipsilateral to the ablation and completely restored in the contralateral nuclei. In the brainstem and deep cerebellar nuclei, poor to moderate recovery occurs bilaterally. Moderate recovery occurs in the contralateral cerebellar cortex. The findings demonstrate that a unilateral cerebral cortical lesion strongly affects lCMRGlc in the thalamus ipsilaterally and in the cerebellar cortex contralaterally, but in the brainstem bilaterally. Partial recovery of lCMRGlc accompanies the progressive motor recovery. The structures affected include those with direct, and also those with indirect, connections to the areas ablated.

  14. Cerebellar network plasticity: from genes to fast oscillation.

    Science.gov (United States)

    Cheron, G; Servais, L; Dan, B

    2008-04-22

    The role of the cerebellum has been increasingly recognized not only in motor control but in sensory, cognitive and emotional learning and regulation. Purkinje cells, being the sole output from the cerebellar cortex, occupy an integrative position in this network. Plasticity at this level is known to critically involve calcium signaling. In the last few years, electrophysiological study of genetically engineered mice has demonstrated the topical role of several genes encoding calcium-binding proteins (calretinin, calbindin, parvalbumin). Specific inactivation of these genes results in the emergence of a fast network oscillation (ca. 160 Hz) throughout the cerebellar cortex in alert animals, associated with ataxia. This oscillation is produced by synchronization of Purkinje cells along the parallel fiber beam. It behaves as an electrophysiological arrest rhythm, being blocked by sensorimotor stimulation. Pharmacological manipulations showed that the oscillation is blocked by GABA(A) and NMDA antagonists as well as gap junction blockers. This cerebellar network oscillation has also been documented in mouse models of human conditions with complex developmental cerebellar dysfunction, such as Angelman syndrome and fetal alcohol syndrome. Recent evidence suggests a relationship between fast oscillation and cerebellar long term depression (LTD). This may have major implications for future therapeutic targeting.

  15. Molecular biology of Philadelphia-negative myeloproliferative neoplasms

    Directory of Open Access Journals (Sweden)

    Paulo Vidal Campregher

    2012-01-01

    Full Text Available Myeloproliferative neoplasms are clonal diseases of hematopoietic stem cells characterized by myeloid hyperplasia and increased risk of developing acute myeloid leukemia. Myeloproliferative neoplasms are caused, as any other malignancy, by genetic defects that culminate in the neoplastic phenotype. In the past six years, since the identification of JAK2V617F, we have experienced a substantial increase in our knowledge about the genetic mechanisms involved in the genesis of myeloproliferative neoplasms. Mutations described in several genes have revealed a considerable degree of molecular homogeneity between different subtypes of myeloproliferative neoplasms. At the same time, the molecular differences between each subtype have become clearer. While mutations in several genes, such as JAK2, myeloproliferative leukemia (MPL and LNK have been validated in functional assays or animal models as causative mutations, the roles of other recurring mutations in the development of disease, such as TET2 and ASXL1 remain to be elucidated. In this review we will examine the most prevalent recurring gene mutations found in myeloproliferative neoplasms and their molecular consequences.

  16. Increased risk of lymphoid neoplasms in patients with Philadelphia chromosome-negative myeloproliferative neoplasms.

    Science.gov (United States)

    Vannucchi, Alessandro M; Masala, Giovanna; Antonioli, Elisabetta; Chiara Susini, Maria; Guglielmelli, Paola; Pieri, Lisa; Maggi, Laura; Caini, Saverio; Palli, Domenico; Bogani, Costanza; Ponziani, Vanessa; Pancrazzi, Alessandro; Annunziato, Francesco; Bosi, Alberto

    2009-07-01

    Association of myeloproliferative neoplasm (MPN) with lymphoproliferative neoplasm (LPN) has been occasionally reported. The aim of this study, which included 353 patients with polycythemia vera and 467 with essential thrombocythemia, was to assess whether the risk of developing LPN is increased in MPN patients. Expected numbers of LPN incident cases were calculated based on 5-year age group, gender, and calendar time-specific cancer incidence rates in the general population of the same area. Standardized incidence ratios were computed to estimate the relative risk of developing LPN. Analyses were carried out for the whole series and then separately for essential thrombocythemia and polycythemia vera, gender, and JAK2V617F genotype. With 4,421 person-years, we found 11 patients developing LPN, including four chronic lymphocytic leukemias, five non-Hodgkin's lymphomas, and two plasma cell disorders, after a median interval time of 68 months from MPN diagnosis. Cumulative risk to develop LPN at 5 and 10 years was 0.93% (95% confidence interval, 0.39-2.22) and 2.96% (95% confidence interval, 1.52-5.72), respectively. There was a 3.44-fold increased risk of LPN compared with the general population, ranging from 2.86 for plasma cell disorder to 12.42 for chronic lymphocytic leukemia; the risk was significantly increased in JAK2V617F mutated patients (5.46-fold) and in males (4.52-fold). The JAK2V617F mutation was found in lymphoid tumor cells in two of three cases evaluated, indicating that, in some patients, LPN originated in a JAK2V617F mutated common lymphoid-myeloid hematopoietic progenitor cell. We conclude that the risk of developing LPN is significantly increased in MPN patients compared with the general population.

  17. Cerebellar hemorrhage after embolization of ruptured vertebral dissecting aneurysm proximal to PICA including parent artery

    Directory of Open Access Journals (Sweden)

    Akira Tamase

    2014-01-01

    Full Text Available Background: Some complications related to vertebral artery occlusion by endovascular technique have been reported. However, cerebellar hemorrhage after vertebral artery occlusion in subacute phase is rare. In this report, we describe a patient who showed cerebellar hemorrhage during hypertensive therapy for vasospasm after embolization of a vertebral dissecting aneurysm. Case Description: A 56-year-old female with a ruptured vertebral dissecting aneurysm proximal to the posterior inferior cerebellar artery developed cerebellar hemorrhage 15 days after embolization of the vertebral artery, including the dissected site. In this patient, the preserved posterior inferior cerebellar artery fed by retrograde blood flow might have been hemodynamically stressed during hypertensive and antiplatelet therapies for subarachnoid hemorrhage, resulting in cerebellar hemorrhage. Conclusion: Although cerebellar hemorrhage is not prone to occur in the nonacute stage of embolization of the vertebral artery, it should be taken into consideration that cerebellar hemorrhage may occur during hypertensive treatment.

  18. Mitotic Events in Cerebellar Granule Progenitor Cells that Expand Cerebellar Surface Area Are Critical for Normal Cerebellar Cortical Lamination in Mice

    Science.gov (United States)

    Chang, Joshua C.; Leung, Mark; Gokozan, Hamza Numan; Gygli, Patrick Edwin; Catacutan, Fay Patsy; Czeisler, Catherine; Otero, José Javier

    2015-01-01

    Late embryonic and postnatal cerebellar folial surface area expansion promotes cerebellar cortical cytoarchitectural lamination. We developed a streamlined sampling scheme to generate unbiased estimates of murine cerebellar surface area and volume using stereological principles. We demonstrate that during the proliferative phase of the external granule layer (EGL) and folial surface area expansion, EGL thickness does not change and thus is a topological proxy for progenitor self-renewal. The topological constraints indicate that during proliferative phases, migration out of the EGL is balanced by self-renewal. Progenitor self-renewal must, therefore, include mitotic events yielding either 2 cells in the same layer to increase surface area (β-events) and mitotic events yielding 2 cells, with 1 cell in a superficial layer and 1 cell in a deeper layer (α-events). As the cerebellum grows, therefore, β-events lie upstream of α-events. Using a mathematical model constrained by the measurements of volume and surface area, we could quantify inter-mitotic times for β-events on a per-cell basis in post-natal mouse cerebellum. Furthermore, we found that loss of CCNA2, which decreases EGL proliferation and secondarily induces cerebellar cortical dyslamination, shows preserved α-type events. Thus, CCNA2-null cerebellar granule progenitor cells are capable of self-renewal of the EGL stem cell niche; this is concordant with prior findings of extensive apoptosis in CCNA2-null mice. Similar methodologies may provide another layer of depth to the interpretation of results from stereological studies. PMID:25668568

  19. Cerebellar Development and Autism Spectrum Disorder in Tuberous Sclerosis Complex.

    Science.gov (United States)

    Sundberg, Maria; Sahin, Mustafa

    2015-12-01

    Approximately 50% of patients with the genetic disease tuberous sclerosis complex present with autism spectrum disorder. Although a number of studies have investigated the link between autism and tuberous sclerosis complex, the etiology of autism spectrum disorder in these patients remains unclear. Abnormal cerebellar function during critical phases of development could disrupt functional processes in the brain, leading to development of autistic features. Accordingly, the authors review the potential role of cerebellar dysfunction in the pathogenesis of autism spectrum disorder in tuberous sclerosis complex. The authors also introduce conditional knockout mouse models of Tsc1 and Tsc2 that link cerebellar circuitry to the development of autistic-like features. Taken together, these preclinical and clinical investigations indicate the cerebellum has a profound regulatory role during development of social communication and repetitive behaviors.

  20. An unusual cause of adult onset cerebellar ataxia with hypogonadism

    Directory of Open Access Journals (Sweden)

    Menon Ramshekhar

    2009-01-01

    Full Text Available We report an unusual case of sporadic adult onset cerebellar ataxia with hypogonadism. A 40-year-old unmarried man presented with progressive ataxia and dysarthria along with complaints of non-development of secondary sexual characteristics and erectile dysfunction. There were complaints of intermittent diarrhea. Clinical examination revealed a pan-cerebellar syndrome with features of hypoandrogenism. No eye movement abnormalities were evident. There were signs of malabsorption. Investigations confirmed the presence of auto-antibodies found in celiac disease, and a duodenal biopsy confirmed the same. Hypoandrogenism was postulated to be due to hypergonadotropic hypogonadism which has been mentioned in a few patients of celiac disease. However, the pattern seen in our patient was of a hypogonadotropic hypogonadism. This is probably secondary to an autoimmune hypophysitis seen in some patients in the absence of other clinical manifestations. Autoantibody testing should be a diagnostic necessity in any adult with a sporadic cerebellar ataxia.

  1. Intraductal papillary mucinous neoplasms and other pancreatic cystic lesions

    Institute of Scientific and Technical Information of China (English)

    Hugh James Freeman

    2008-01-01

    Pancreatic cystic neoplasms are being increasingly recognized, even in the absence of symptoms, in large part, due to markedly improved imaging modalities such as magnetic resonance imaging (MRI)/magnetic resonance cholangio pancreatography (MRCP) and computer tomography (CT) scanning. During the past 2 decades, better imaging of these cystic lesions has resulted in definition of different types, including pancreatic intraductal papillary mucinous neoplasms (IPMN). While IPMN represent only a distinct minority of all pancreatic cancers, they appear to be a relatively frequent neoplastic form of pancreatic cystic neoplasm. Moreover, IPMN have a much better outcome and prognosis compared to pancreatic ductal adenocarcinomas. Therefore, recognition of this entity is exceedingly important for the clinician involved in diagnosis and further evaluation of a potentially curable form of pancreatic cancer.

  2. Interdisciplinary Management of Cystic Neoplasms of the Pancreas

    Directory of Open Access Journals (Sweden)

    Linda S. Lee

    2012-01-01

    Full Text Available Cystic neoplasms of the pancreas are increasingly recognized due to the frequent use of abdominal imaging. It is reported that up to 20% of abdominal cross-sectional scans identify incidental asymptomatic pancreatic cysts. Proper characterization of pancreatic cystic neoplasms is important not only to recognize premalignant lesions that will require surgical resection, but also to allow nonoperative management of many cystic lesions that will not require resection with its inherent morbidity. Though reliable biomarkers are lacking, a wide spectrum of diagnostic modalities are available to evaluate pancreatic cystic neoplasms, including radiologic, endoscopic, laboratory, and pathologic analysis. An interdisciplinary approach to management of these lesions which incorporates recent, specialty-specific advances in the medical literature is herein suggested.

  3. Synchronous laparoscopic resection of colorectal and renal/adrenal neoplasms.

    Science.gov (United States)

    Ng, Simon S M; Lee, Janet F Y; Yiu, Raymond Y C; Li, Jimmy C M; Leung, Ka Lau

    2007-08-01

    Synchronous laparoscopic resections of coexisting abdominal diseases are shown to be feasible without additional postoperative morbidity. We report our experience with synchronous laparoscopic resection of colorectal carcinoma and renal/adrenal neoplasms with an emphasis on surgical and oncologic outcomes. Five patients diagnosed to have synchronous colorectal carcinoma and renal/adrenal neoplasms (renal cell carcinoma in 2 patients, adrenal cortical adenoma in 2 patients, and adrenal metastasis in 1 patient) underwent synchronous laparoscopic resection. The median operative time was 420 minutes and the median operative blood loss was 1000 mL. Three patients developed minor complications, including wound infection in 2 patients and retention of urine in 1 patient. There was no operative mortality. The median duration of hospital stay was 11 days. At a median follow-up of 17.6 months, no patient developed recurrence of disease. Synchronous laparoscopic resection of colorectal and renal/adrenal neoplasms is technically feasible and safe.

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  9. Cerebellar diaschisis in pontine infractions: a report of five cases

    Energy Technology Data Exchange (ETDEWEB)

    Tsuda, Yoshiyasu [Kagawa Medical School (Japan). Second Dept. of Internal Medicine; Ayada, Yoshihide [Kagawa Medical School (Japan). Second Dept. of Internal Medicine; Izumi, Yoshinari [Kagawa Medical School (Japan). Second Dept. of Internal Medicine; Ichihara, Sin-Ichiro [Kagawa Medical School (Japan). Second Dept. of Internal Medicine; Hosomi, Naohisa [Kagawa Medical School (Japan). Second Dept. of Internal Medicine; Ohkawa, Motoomi [Kagawa Medical School (Japan). Dept. of Radiology; Matsuo, Hirohide [Kagawa Medical School (Japan). Second Dept. of Internal Medicine

    1995-05-01

    We evaluate regional cerebral and cerebellar perfusion to prove the occurrence and follow the persistence of crossed cerebellar diaschisis in infratentorial pontine infarction. Six consecutive patients exhibiting mild hemiparetic symptoms or a heavy feeling in the head (mean age 65 years; four women, two men) and diagnosed as having pontine infarction by magnetic resonance imaging were sugjected to evaluation. Lesions due to infarction were located at the upper basis pontis in five partients and the upper tegmentum pontis in one, and medially at the paramedian portion in four and laterally in two. Regional cerebral and cerebellar perfusion was evaluated semiquantitatively by iodine-123 N-isopropyl-p-iodoamphetamine (IMP) single-photon emission tomography (SPET); this was done during the acute stage in five cases (mean time after onset: 0.7 months) and during the chronic stage in three (mean time after onset: 14.8 months) Significant asymmetry in cerebellar perfusion, which was reduced in the contralateral or ipsilateral cerebellar hemisphere, was demonstrated semiquantitatively in four cases during the acute stage and in one during the chronic stage, as compared with normal controls. This asymmetry continued to the chronic stage (6.5 and 33.0 months) in two cases, while no patient showed any significant asymmetries in cerebral perdusion in any region of interest in either SPET study. The pontine lesion may damage the pyramidal tract and corticocerebellar pathway, and interruption of the cerebrocerebellar pontine circuits may be regarded as the cause of the crossed cerebellar diaschisis observed in five of the six reported patients with pontine infarction. (orig.)

  10. Cerebellar substrates for error correction in motor conditioning.

    Science.gov (United States)

    Gluck, M A; Allen, M T; Myers, C E; Thompson, R F

    2001-11-01

    The authors evaluate a mapping of Rescorla and Wagner's (1972) behavioral model of classical conditioning onto the cerebellar substrates for motor reflex learning and illustrate how the limitations of the Rescorla-Wagner model are just as useful as its successes for guiding the development of new psychobiological theories of learning. They postulate that the inhibitory pathway that returns conditioned response information from the cerebellar interpositus nucleus back to the inferior olive is the neural basis for the error correction learning proposed by Rescorla and Wagner (Gluck, Myers, & Thompson, 1994; Thompson, 1986). The authors' cerebellar model expects that behavioral processes described by the Rescorla-Wagner model will be localized within the cerebellum and related brain stem structures, whereas behavioral processes beyond the scope of the Rescorla-Wagner model will depend on extracerebellar structures such as the hippocampus and related cortical regions. Simulations presented here support both implications. Several novel implications of the authors' cerebellar error-correcting model are described including a recent empirical study by Kim, Krupa, and Thompson (1998), who verified that suppressing the putative error correction pathway should interfere with the Kamin (1969) blocking effect, a behavioral manifestation of error correction learning. The authors also discuss the model's implications for understanding the limits of cerebellar contributions to associative learning and how this informs our understanding of hippocampal function in conditioning. This leads to a more integrative view of the neural substrates of conditioning in which the authors' real-time circuit-level model of the cerebellum can be viewed as a generalization of the long-term memory module of Gluck and Myers' (1993) trial-level theory of cerebellar-hippocampal interaction in motor conditioning.

  11. Emotional disorders in patients with cerebellar damage – case studies

    Directory of Open Access Journals (Sweden)

    Siuda, Katarzyna

    2014-04-01

    Full Text Available Aim: Growing number of research shows the role of the cerebellum in the regulation of affect. Lesions of the cerebellum can lead to emotional disregulation, a significant part of the Cerebellar Cognitive Affective Syndrome. The aim of this article is to analyze the most recent studies concerning the cerebellar participation in emotional reactions and to present three cases: two female and one male who suffered from cerebellar damage and presented post-traumatic affective and personality change. Method: The patients’ neuropsychological examination was performed with Raven’s Progressive Matrices Test – standard version, Trial Making Test, Wisconsin Card Sorting Test, Auditory Verbal Learning Test by Łuria, Benton Visual Retention Test, Verbal Fluency Test, Stroop Interference Test, Attention and Perceptivity Test (Test Uwagi i Spostrzegawczości TUS, Frontal Behavioral Inventory (FBI. Results: The review of the literature suggest cerebellar participation, especially teh vermis and paravermial regions, in the detection, integration and filtration of emotional information and in regulation of autonomic emotional responses. In the described patients we observed: oversensitivity, irritability, impulsivity and self-neglect. The man and the woman with right-sided lesions presented similar symptoms: rigidity of thought, stubbornness, lack of criticism, jocular and inappropriate behavior. The woman with left-sided cerebellar lesion was adynamic, apathic and passive, she presented emotional blunting, social isolation, lack of interests and motivation, general cognitive slowdown. Conclusions: Both the analyzed research and the described cases indicate the connection between the cerebellum and emotion regulation. The symptoms presented by the described patients were most probably a consequence of damaged cerebellar projections to subcortical structures (the limbic system and frontal areas. The diversification of symptoms depending on the localization

  12. Disseminated encephalomyelitis-like central nervous system neoplasm in childhood.

    Science.gov (United States)

    Zhao, Jianhui; Bao, Xinhua; Fu, Na; Ye, Jintang; Li, Ting; Yuan, Yun; Zhang, Chunyu; Zhang, Yao; Zhang, Yuehua; Qin, Jiong; Wu, Xiru

    2014-08-01

    A malignant neoplasm in the central nervous system with diffuse white matter changes on magnetic resonance imaging (MRI) is rare in children. It could be misdiagnosed as acute disseminated encephalomyelitis. This report presents our experience based on 4 patients (3 male, 1 female; aged 7-13 years) whose MRI showed diffuse lesions in white matter and who were initially diagnosed with acute disseminated encephalomyelitis. All of the patients received corticosteroid therapy. After brain biopsy, the patients were diagnosed with gliomatosis cerebri, primitive neuroectodermal tumor and central nervous system lymphoma. We also provide literature reviews and discuss the differentiation of central nervous system neoplasm from acute disseminated encephalomyelitis.

  13. CD4~+CD56~+ hematodermic neoplasm in a child

    Institute of Scientific and Technical Information of China (English)

    GUO Xia; LI Qiang; ZHOU Chen-yan

    2010-01-01

    @@ CD4~+CD56~+ hematodermic neoplasm (HN) is a rare, highly aggressive systemic neoplasm, which had been described under various names including lymphoblastic lymphoma of natural killer (NK) phenotype, blastic NK cell lymphoma (BNK), leukemic lymphoma of immature NK lineage and CD4~+CD56~+ HN. This malignancy is mainly involved in elderly people and usually a rapidly fatal disease, since consistently effective treatments have not yet been developed. It is relatively rare in children.~(1-6) Herein we report a boy with CD4~+CD56~+ HN.

  14. PATIENTS WITH METASTATIC GESTATIONAL TROPHOBLASTIC NEOPLASMS AND NO GYNECOLOGICAL SYMPTOMS

    Directory of Open Access Journals (Sweden)

    F. Ghaemmaghami T. Ashraf Ganjoie

    2008-04-01

    Full Text Available Early recognition of Gestational Trophoblastic Neoplasm (GTN will maximize the chances of cure with chemotherapy but some patients present with many different symptoms months or even years after the causative pregnancy making diagnosis difficult. Clinicians should be aware of the possibility of GTN in any reproductive age woman with bizarre central nervous system, gastrointestinal, pulmonary symptoms or radiographic evidence of metastatic tumor of unknown primary origin. We reported five cases of metastatic gestational trophoblastic neoplasms with bizarre pulmonary symptoms, acute abdomen, neurologic symptoms presenting without gynecological symptoms.

  15. Unicentric Castleman’s Disease Masquerading Pancreatic Neoplasm

    Directory of Open Access Journals (Sweden)

    Saurabh Jain

    2012-01-01

    Full Text Available Castleman’s disease is a rare nonclonal proliferative disorder of the lymph nodes with an unknown etiology. Common locations of Castleman’s disease are mediastinum, neck, axilla, and abdomen. Castleman’s disease of a peripancreatic location masquerading as pancreatic neoplasm is an even rarer entity. On search of published data, we came across about 17 cases published on peripancreatic Castleman’s disease until now. Here we are reporting a case of retropancreatic Castleman's disease masquerading as retroperitoneal neoplasm in a 46-year-old male patient.

  16. Choline Uptake by Glomerular Synapses Isolated from Bovine Cerebellar Vermis.

    Science.gov (United States)

    1986-01-01

    28 034 UNCLASSIFIED -7t. holing uptake by glomerular aynapaea isolated from bovine cerebellar venni - . 1) N1 IrRRIAN.E L NfISINndwr EtIIOMAS86 .t...w. -%FAt~Jr~a~etn 0,oAAM TX78215-5301 IL’SAJ) A-xpid ( kaolin 22nd. 19W5) hh.lhoac-anln uplake -ainalnnn 177 DIOMIDICAL DmIVIIN,~ F-5’. . Brain...Research. 366 (1986) 401-404 401 Elsevier BRE 21387 Choline uptake by glomerular synapses isolated from bovine cerebellar vermis D.M. TERRIAN, E.L

  17. Inside the Thompson laboratory during the "cerebellar years" and the continuing cerebellar story.

    Science.gov (United States)

    Lavond, D G; Wikgren, J; Nokia, M S

    2011-02-01

    This paper is based on the talk by one of the authors (DL) given at the symposium for the retirement of RF Thompson (RF Thompson: A bridge between 20th and 21st century neuroscience). We first make some informal observations of the historical times and research conditions in the Thompson laboratory when the cerebellum was found to play a critical role in eye lid classical conditioning, the "cerebellar years". These conditions influenced our collaborative international program on the phenomenon known as "transfer of training" or "savings". Our research shows that the appearance of "savings" is an artifact of the order of testing, and depends upon the functioning of the contralateral interpositus nucleus (IPN) in a way that is complementary to the role of the IPN in normal eyelid classical conditioning.

  18. Postural Ataxia in Cerebellar Downbeat Nystagmus: Its Relation to Visual, Proprioceptive and Vestibular Signals and Cerebellar Atrophy

    Science.gov (United States)

    Helmchen, Christoph; Kirchhoff, Jan-Birger; Göttlich, Martin; Sprenger, Andreas

    2017-01-01

    Background The cerebellum integrates proprioceptive, vestibular and visual signals for postural control. Cerebellar patients with downbeat nystagmus (DBN) complain of unsteadiness of stance and gait as well as blurred vision and oscillopsia. Objectives The aim of this study was to elucidate the differential role of visual input, gaze eccentricity, vestibular and proprioceptive input on the postural stability in a large cohort of cerebellar patients with DBN, in comparison to healthy age-matched control subjects. Methods Oculomotor (nystagmus, smooth pursuit eye movements) and postural (postural sway speed) parameters were recorded and related to each other and volumetric changes of the cerebellum (voxel-based morphometry, SPM). Results Twenty-seven patients showed larger postural instability in all experimental conditions. Postural sway increased with nystagmus in the eyes closed condition but not with the eyes open. Romberg’s ratio remained stable and was not different from healthy controls. Postural sway did not change with gaze position or graviceptive input. It increased with attenuated proprioceptive input and on tandem stance in both groups but Romberg’s ratio also did not differ. Cerebellar atrophy (vermal lobule VI, VIII) correlated with the severity of impaired smooth pursuit eye movements of DBN patients. Conclusions Postural ataxia of cerebellar patients with DBN cannot be explained by impaired visual feedback. Despite oscillopsia visual feedback control on cerebellar postural control seems to be preserved as postural sway was strongest on visual deprivation. The increase in postural ataxia is neither related to modulations of single components characterizing nystagmus nor to deprivation of single sensory (visual, proprioceptive) inputs usually stabilizing stance. Re-weighting of multisensory signals and/or inappropriate cerebellar motor commands might account for this postural ataxia. PMID:28056109

  19. Dyslexic Children Show Atypical Cerebellar Activation and Cerebro-Cerebellar Functional Connectivity in Orthographic and Phonological Processing.

    Science.gov (United States)

    Feng, Xiaoxia; Li, Le; Zhang, Manli; Yang, Xiujie; Tian, Mengyu; Xie, Weiyi; Lu, Yao; Liu, Li; Bélanger, Nathalie N; Meng, Xiangzhi; Ding, Guosheng

    2017-04-01

    Previous neuroimaging studies have found atypical cerebellar activation in individuals with dyslexia in either motor-related tasks or language tasks. However, studies investigating atypical cerebellar activation in individuals with dyslexia have mostly used tasks tapping phonological processing. A question that is yet unanswered is whether the cerebellum in individuals with dyslexia functions properly during orthographic processing of words, as growing evidence shows that the cerebellum is also involved in visual and spatial processing. Here, we investigated cerebellar activation and cerebro-cerebellar functional connectivity during word processing in dyslexic readers and typically developing readers using tasks that tap orthographic and phonological codes. In children with dyslexia, we observed an abnormally higher engagement of the bilateral cerebellum for the orthographic task, which was negatively correlated with literacy measures. The greater the reading impairment was for young dyslexic readers, the stronger the cerebellar activation was. This suggests a compensatory role of the cerebellum in reading for children with dyslexia. In addition, a tendency for higher cerebellar activation in dyslexic readers was found in the phonological task. Moreover, the functional connectivity was stronger for dyslexic readers relative to typically developing readers between the lobule VI of the right cerebellum and the left fusiform gyrus during the orthographic task and between the lobule VI of the left cerebellum and the left supramarginal gyrus during the phonological task. This pattern of results suggests that the cerebellum compensates for reading impairment through the connections with specific brain regions responsible for the ongoing reading task. These findings enhance our understanding of the cerebellum's involvement in reading and reading impairment.

  20. Preserved Glucose Metabolism of Deep Cerebellar Nuclei in a Case of Multiple System Atrophy with Predominant Cerebellar Ataxia: F-18 Fluorodeoxyglucose Positron Emission Tomography Study

    Directory of Open Access Journals (Sweden)

    Oh Dae Kwon

    2010-10-01

    Full Text Available The cerebellar glucose metabolism of multiple system atrophy with predominant cerebellar ataxia (MSA-C is known to be decreased but is not defined among areas of cerebellum. We encountered a 54-year-old man who developed dizziness and progressive ataxia followed by urinary incontinence and orthostatic hypotension, all of those symptoms progressed relentlessly and the symptoms responded poorly to levodopa therapy. Visual analysis and statistical parametric mapping analysis of F-18 fluorodeoxyglucose positron emission tomography showed hypometabolism of both cerebellar hemisphere, severe at cortical area, and pons. There was clear sparing of deep cerebellar nuclei. Our report, as we know, shows the first case of preserved glucose metabolism of deep cerebellar nuclei relative to cerebellar cortex in an MSA-C patient.

  1. Cystic neoplasms of the pancreas: A diagnostic challenge

    Institute of Scientific and Technical Information of China (English)

    Grant F Hutchins; Peter V Draganov

    2009-01-01

    Cystic neoplasms of the pancreas are increasingly recognized due to the expanding use and improved sensitivity of cross-sectional abdominal imaging. Major advances in the last decade have led to an improved understanding of the various types of cystic lesions and their biologic behavior. Despite significant improvements in imaging technology and the advent of endoscopic-ultrasound (EUS)-guided fineneedle aspiration, the diagnosis and management of pancreatic cystic lesions remains a significant clinical challenge. The first diagnostic step is to differentiate between pancreatic pseudocyst and cystic neoplasm.If a pseudocyst has been effectively excluded, the cornerstone issue is then to determine the malignant potential of the pancreatic cystic neoplasm. In the majority of cases, the correct diagnosis and successful management is based not on a single test but on incorporating data from various sources including patient history, radiologic studies, endoscopic evaluation, and cyst fluid analysis. This review will focus on describing the various types of cystic neoplasms of the pancreas, their malignant potential, and will provide the clinician with a comprehensive diagnostic approach.

  2. Mucin profile of the pancreatic mucinous cystic neoplasms

    Institute of Scientific and Technical Information of China (English)

    JI Yuan; XU Jian-fang; KUANG Tian-tao; ZHOU Yan-nan; LU Shao-hua; TAN Yun-shan

    2006-01-01

    @@ Mucinous cystic neoplasms (MCNs) of the pancreas are a distinct entity, account for 1% of pancreatic exocrine tumors. MCNs can be classified histologically as adenomas, borderline tumors, or carcinomas. Because several evidences showing that mucinous cystadenomas are poten- tially malignant and may transform into cystadeno- carcinomas, particularly if treated by drainage, these tumors should be identified accurately.1

  3. Role of Calcium in Cerebellar Learning and Function

    NARCIS (Netherlands)

    Z. Gao (Zhenyu)

    2011-01-01

    textabstractThe cerebellum, which means little brain in Latin, occupies most of the posterior cranial fossa and connects with the dorsal brainstem (Kandel et al., 2000). The cerebellar cortex is one of the most foliated brain structures, which accounts for 10% of the total volume and over half of th

  4. Reevaluating the Role of LTD in Cerebellar Motor Learning

    NARCIS (Netherlands)

    M. Schonewille (Martijn); Z. Gao (Zhenyu); H.J. Boele (Henk-Jan); M.F. Vinueza Veloz (Maria); W.E. Amerika; A. Šimek (Antonia); M.T.G. Jeu (Marcel); J. Steinberg (Jordan); K. Takamiya (Kogo); F.E. Hoebeek (Freek); D. Linden (David); R. Huganir (Richard); C.I. de Zeeuw (Chris)

    2011-01-01

    textabstractLong-term depression at parallel fiber-Purkinje cell synapses (PF-PC LTD) has been proposed to be required for cerebellar motor learning. To date, tests of this hypothesis have sought to interfere with receptors (mGluR1) and enzymes (PKC, PKG, or αCamKII) necessary for induction of PF-PC

  5. Cerebellar Control of Locomotion in Health and Disease

    NARCIS (Netherlands)

    M.F. Vinueza Veloz (Maria)

    2015-01-01

    markdownabstract__Abstract__ Modern neuroscience is paving the way for new insight into cerebellar functions including the control of cognitive, autonomic and emotional processes. Yet, how the cerebellum contributes to complex motor behaviors, such as locomotion, is still only partially understood.

  6. Is a Cerebellar Deficit the Underlying Cause of Reading Disabilities?

    Science.gov (United States)

    Irannejad, Shahrzad; Savage, Robert

    2012-01-01

    This study investigated whether children with dyslexia differed in their performance on reading, phonological, rapid naming, motor, and cerebellar-related tasks and automaticity measures compared to reading age (RA)-matched and chronological age (CA)-matched control groups. Participants were 51 children attending mainstream English elementary…

  7. Very Preterm Birth, Cerebellar Development and Neuropsychological Outcome in Adolescence

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2008-06-01

    Full Text Available Cerebellar volumes were measured on structural MRI at adolescence and adulthood in 65 preterm individuals (born before 33 weeks’ gestation, and a term-born comparison group, in a study at King’s College, Great Ormond Street Hospital, and University College, London; and Seoul National University College of Medicine, Korea.

  8. Early Cerebellar Network Shifting in Spinocerebellar Ataxia Type 6.

    Science.gov (United States)

    Falcon, M I; Gomez, C M; Chen, E E; Shereen, A; Solodkin, A

    2016-07-01

    Spinocerebellar ataxia 6 (SCA6), an autosomal dominant degenerative disease, is characterized by diplopia, gait ataxia, and incoordination due to severe progressive degeneration of Purkinje cells in the vestibulo- and spinocerebellum. Ocular motor deficits are common, including difficulty fixating on moving objects, nystagmus and disruption of smooth pursuit movements. In presymptomatic SCA6, there are alterations in saccades and smooth-pursuit movements. We sought to assess functional and structural changes in cerebellar connectivity associated with a visual task, hypothesizing that gradual changes would parallel disease progression. We acquired functional magnetic resonance imaging and diffusion tensor imaging data during a passive smooth-pursuit task in 14 SCA6 patients, representing a range of disease duration and severity, and performed a cross-sectional comparison of cerebellar networks compared with healthy controls. We identified a shift in activation from vermis in presymptomatic individuals to lateral cerebellum in moderate-to-severe cases. Concomitantly, effective connectivity between regions of cerebral cortex and cerebellum was at its highest in moderate cases, and disappeared in severe cases. Finally, we noted structural differences in the cerebral and cerebellar peduncles. These unique results, spanning both functional and structural domains, highlight widespread changes in SCA6 and compensatory mechanisms associated with cerebellar physiology that could be utilized in developing new therapies.

  9. Long-Term Sequelae after Cerebellar Astrocytoma Surgery

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2004-05-01

    Full Text Available The long-term effects on neurologic, neuropsychological, and behavioral functioning in a consecutive series of 23 children treated surgically for cerebellar pilocytic astrocytoma without additional radio- and chemotherapy are determined in a study at Sophia Children’s Hospital, Rotterdam, The Netherlands, and other medical centers.

  10. [Autosomal dominant cerebellar ataxias in the Netherlands: a national inventory

    NARCIS (Netherlands)

    Warrenburg, B.P.C. van de

    2001-01-01

    OBJECTIVE: To provide a comprehensive estimate of the number of Dutch autosomal dominant cerebellar ataxias (ADCA) families and patients and thus estimate the minimal prevalence of ADCA in the Netherlands. Furthermore, to observe the relative frequency of SCA mutations and to study genotype-phenotyp

  11. Mapping the development of cerebellar Purkinje cells in zebrafish.

    Science.gov (United States)

    Hamling, Kyla R; Tobias, Zachary J C; Weissman, Tamily A

    2015-11-01

    The cells that comprise the cerebellum perform a complex integration of neural inputs to influence motor control and coordination. The functioning of this circuit depends upon Purkinje cells and other cerebellar neurons forming in the precise place and time during development. Zebrafish provide a useful platform for modeling disease and studying gene function, thus a quantitative metric of normal zebrafish cerebellar development is key for understanding how gene mutations affect the cerebellum. To begin to quantitatively measure cerebellar development in zebrafish, we have characterized the spatial and temporal patterning of Purkinje cells during the first 2 weeks of development. Differentiated Purkinje cells first emerged by 2.8 days post fertilization and were spatially patterned into separate dorsomedial and ventrolateral clusters that merged at around 4 days. Quantification of the Purkinje cell layer revealed that there was a logarithmic increase in both Purkinje cell number as well as overall volume during the first 2 weeks, while the entire region curved forward in an anterior, then ventral direction. Purkinje cell dendrites were positioned next to parallel fibers as early as 3.3 days, and Purkinje cell diameter decreased significantly from 3.3 to 14 days, possibly due to cytoplasmic reappropriation into maturing dendritic arbors. A nearest neighbor analysis showed that Purkinje cells moved slightly apart from each other from 3 to 14 days, perhaps spreading as the organized monolayer forms. This study establishes a quantitative spatiotemporal map of Purkinje cell development in zebrafish that provides an important metric for studies of cerebellar development and disease.

  12. Cerebellar Damage Produces Selective Deficits in Verbal Working Memory

    Science.gov (United States)

    Ravizza, Susan M.; Mccormick, Cristin A.; Schlerf, John E.; Justus, Timothy; Ivry, Richard B.; Fiez, Julie A.

    2006-01-01

    The cerebellum is often active in imaging studies of verbal working memory, consistent with a putative role in articulatory rehearsal. While patients with cerebellar damage occasionally exhibit a mild impairment on standard neuropsychological tests of working memory, these tests are not diagnostic for exploring these processes in detail. The…

  13. Cerebellar rTMS disrupts predictive language processing

    Science.gov (United States)

    Lesage, Elise; Morgan, Blaire E.; Olson, Andrew C.; Meyer, Antje S.; Miall, R. Chris

    2012-01-01

    Summary The human cerebellum plays an important role in language, amongst other cognitive and motor functions [1], but a unifying theoretical framework about cerebellar language function is lacking. In an established model of motor control, the cerebellum is seen as a predictive machine, making short-term estimations about the outcome of motor commands. This allows for flexible control, on-line correction, and coordination of movements [2]. The homogeneous cytoarchitecture of the cerebellar cortex suggests that similar computations occur throughout the structure, operating on different input signals and with different output targets [3]. Several authors have therefore argued that this ‘motor’ model may extend to cerebellar nonmotor functions [3–5], and that the cerebellum may support prediction in language processing [6]. However, this hypothesis has never been directly tested. Here, we used the ‘Visual World’ paradigm [7], where on-line processing of spoken sentence content can be assessed by recording the latencies of listeners' eye movements towards objects mentioned. Repetitive transcranial magnetic stimulation (rTMS) was used to disrupt function in the right cerebellum, a region implicated in language [8]. After cerebellar rTMS, listeners showed delayed eye fixations to target objects predicted by sentence content, while there was no effect on eye fixations in sentences without predictable content. The prediction deficit was absent in two control groups. Our findings support the hypothesis that computational operations performed by the cerebellum may support prediction during both motor control and language processing. PMID:23017990

  14. Neurophysiological evidence for cerebellar dysfunction in primary focal dystonia.

    NARCIS (Netherlands)

    Teo, J.T.; Warrenburg, B.P.C. van de; Schneider, S.A.; Rothwell, J.C.; Bhatia, K.P.

    2009-01-01

    Recent studies have suggested that there may be functional and structural changes in the cerebellum of patients with adult onset primary focal dystonia. The aim of this study was to establish whether there is any neurophysiological indicator of abnormal cerebellar function, using the classic eyeblin

  15. Cerebro-cerebellar interactions underlying temporal information processing.

    Science.gov (United States)

    Aso, Kenji; Hanakawa, Takashi; Aso, Toshihiko; Fukuyama, Hidenao

    2010-12-01

    The neural basis of temporal information processing remains unclear, but it is proposed that the cerebellum plays an important role through its internal clock or feed-forward computation functions. In this study, fMRI was used to investigate the brain networks engaged in perceptual and motor aspects of subsecond temporal processing without accompanying coprocessing of spatial information. Direct comparison between perceptual and motor aspects of time processing was made with a categorical-design analysis. The right lateral cerebellum (lobule VI) was active during a time discrimination task, whereas the left cerebellar lobule VI was activated during a timed movement generation task. These findings were consistent with the idea that the cerebellum contributed to subsecond time processing in both perceptual and motor aspects. The feed-forward computational theory of the cerebellum predicted increased cerebro-cerebellar interactions during time information processing. In fact, a psychophysiological interaction analysis identified the supplementary motor and dorsal premotor areas, which had a significant functional connectivity with the right cerebellar region during a time discrimination task and with the left lateral cerebellum during a timed movement generation task. The involvement of cerebro-cerebellar interactions may provide supportive evidence that temporal information processing relies on the simulation of timing information through feed-forward computation in the cerebellum.

  16. Anterior and posterior inferior cerebellar artery infarction with sudden deafness and vertigo.

    Science.gov (United States)

    Murakami, Takenobu; Nakayasu, Hiroyuki; Doi, Mitsuru; Fukada, Yasuyo; Hayashi, Miwa; Suzuki, Takeo; Takeuchi, Yuichi; Nakashima, Kenji

    2006-12-01

    We report a patient with anterior and posterior inferior cerebellar artery infarction, which manifested as profound deafness, transient vertigo, and minimal cerebellar signs. We suspect that ischaemia of the left internal auditory artery, which originates from the anterior inferior cerebellar artery, caused the deafness and transient vertigo. A small lesion in the middle cerebellar peduncle in the anterior inferior cerebellar artery territory and no lesion in the dentate nucleus in the posterior inferior cerebellar artery territory are thought to explain the minimal cerebellar signs despite the relatively large size of the infarction. Thus a relatively large infarction of the vertebral-basilar territory can manifest as sudden deafness with vertigo. Neuroimaging, including magnetic resonance imaging, is strongly recommended for patients with sudden deafness and vertigo to exclude infarction of the vertebral-basilar artery territory.

  17. Post-Plasmodium vivax malaria cerebellar ataxia and optic neuritis: A new form of delayed cerebellar ataxia or cerebellar variant of acute disseminated encephalomyelitis?

    Directory of Open Access Journals (Sweden)

    Gaurav M Kasundra

    2015-01-01

    Full Text Available Acute disseminated encephalomyelitis (ADEM is commonly seen after viral and bacterial infections, immunization, and Plasmodium falciparum (PF malaria. Plasmodium vivax (PV rarely causes ADEM. We report a 14-year-old female patient who presented with acute onset bilateral cerebellar ataxia and optic neuritis, 2 weeks after recovery from PV. Magnetic resonance imaging showed bilateral cerebellar hyperintensities suggestive of ADEM. No specific viral etiology was found on cerebrospinal fluid examination. Patient responded well to treatment without any sequelae. Thus, PV too is an important cause of ADEM along with PF. Two of the previously reported cases had co-infection with falciparum malaria. The only other two reported cases, as also this patient, are from Asia. A geographical or racial predisposition needs to be evaluated. Also, a possibility of post-PV delayed cerebellar ataxia, which is classically described post-PF infection, may be considered as it may be clinically, radiologically, and prognostically indistinguishable from a milder presentation of ADEM.

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  14. Diffusion Tensor Imaging of Human Cerebellar Pathways and their Interplay with Cerebral Macrostructure

    Directory of Open Access Journals (Sweden)

    Zafer eKeser

    2015-04-01

    Full Text Available Cerebellar white matter connections to the central nervous system are classified functionally into the spinocerebellar, vestibulocerebellar, and cerebrocerebellar subdivisions. The Spinocerebellar (SC pathways project from spinal cord to cerebellum, whereas the vestibulocerebellar (VC pathways project from vestibular organs of the inner ear. Cerebrocerebellar connections are composed of feed forward and feedback connections between cerebrum and cerebellum including the cortico-ponto-cerebellar (CPC pathways being of cortical origin and the dentate-rubro-thalamo-cortical (DRTC pathway being of cerebellar origin. In this study we systematically quantified the whole cerebellar system connections using diffusion tensor magnetic resonance imaging (DT-MRI. Ten right-handed healthy subjects (7 males and 3 females, age range 20-51 years were studied. DT-MRI data were acquired with a voxel size = 2mm x 2mm x 2 mm at a 3.0 Tesla clinical MRI scanner. The DT-MRI data were prepared and analyzed using anatomically-guided deterministic tractography methods to reconstruct the SC, DRTC, fronto-ponto-cerebellar (FPC, parieto-ponto-cerebellar (PPC, temporo-ponto-cerebellar (TPC and occipito-ponto-cerebellar (OPC. The DTI-attributes or the cerebellar tracts along with their cortical representation (Brodmann areas were presented in standard Montréal Neurological Institute space. All cerebellar tract volumes were quantified and correlated with volumes of cerebral cortical, subcortical gray matter (GM, cerebral white matter (WM and cerebellar GM, and cerebellar WM. On our healthy cohort, the ratio of total cerebellar GM-to-WM was ~ 3.29 ± 0.24, whereas the ratio of cerebral GM-to-WM was approximately 1.10 ± 0.11. The sum of all cerebellar tract volumes is ~ 25.8 ± 7.3 mL, or a percentage of 1.52 ± 0.43 of the total intracranial volume.

  15. Cerebellar motor learning: when is cortical plasticity not enough?

    Directory of Open Access Journals (Sweden)

    John Porrill

    2007-10-01

    Full Text Available Classical Marr-Albus theories of cerebellar learning employ only cortical sites of plasticity. However, tests of these theories using adaptive calibration of the vestibulo-ocular reflex (VOR have indicated plasticity in both cerebellar cortex and the brainstem. To resolve this long-standing conflict, we attempted to identify the computational role of the brainstem site, by using an adaptive filter version of the cerebellar microcircuit to model VOR calibration for changes in the oculomotor plant. With only cortical plasticity, introducing a realistic delay in the retinal-slip error signal of 100 ms prevented learning at frequencies higher than 2.5 Hz, although the VOR itself is accurate up to at least 25 Hz. However, the introduction of an additional brainstem site of plasticity, driven by the correlation between cerebellar and vestibular inputs, overcame the 2.5 Hz limitation and allowed learning of accurate high-frequency gains. This "cortex-first" learning mechanism is consistent with a wide variety of evidence concerning the role of the flocculus in VOR calibration, and complements rather than replaces the previously proposed "brainstem-first" mechanism that operates when ocular tracking mechanisms are effective. These results (i describe a process whereby information originally learnt in one area of the brain (cerebellar cortex can be transferred and expressed in another (brainstem, and (ii indicate for the first time why a brainstem site of plasticity is actually required by Marr-Albus type models when high-frequency gains must be learned in the presence of error delay.

  16. Cerebellar and pontine tegmental hypermetabolism in miller-fisher syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yu Kyrong; Kim, Ji Soo; Lee, Won Woo; Kim, Sang Eun [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of)

    2007-07-01

    Miller Fisher syndrome (MFS) has been considered as a variant of Guillain-Barre syndrome (GBS), a type of acute immune neuropathies involving peripheral nerve system. Unlike GBS, presence of cerebellar type ataxia and supranuclear ophthalmioplesia in MFS suggests additional involvement of the central nervous system. To determine involvement of the central nervous system in MFS, we investigated the cerebral metabolic abnormalities in patients with MFS using FDG PET. Nine patients who were diagnosed as MFS based on acute ophthalmoplegia, ataxia, and areflexia without other identifiable causes participated in this study. In six patients, serum antibodies possibly related with symptom of MFS (anti- GQ1b or anti-GM1) were detected at the time of the study. With the interval of 25 26 days (range: 3-83 days) from the symptom on set, brain FDG PET were underwent in patients and compared with those from healthy controls. In group analysis comparing with healthy controls, FDG PET of patients revealed increased metabolism in the bilateral cerebellar hemispheres and vermis, and the thalamus. In contrast, the occipital cortex showed decreased metabolism. Individual analyses disclosed hypermetabolism in the cerebellar vermis or hemispheres in 5, and in the pontine tegmentum in 2 of the 9 patients. We also found that the cerebellar vermian hypermetabolism was inversely correlated with the interval between from the symptom on set to PET study. Moreover, follow-up PET of a patient demonstrated that cerebellar hypermetabolism decreased markedly with an improvement of the ophthalmoplegia and ataxia. These findings indicate an involvement of the central nervous system in MFS and suggest an antibody-associated acute inflammatory process as a mechanism of this disorder.

  17. GDNF-induced cerebellar toxicity: A brief review.

    Science.gov (United States)

    Luz, Matthias; Mohr, Erich; Fibiger, H Christian

    2016-01-01

    Recombinant-methionyl human glial cell line-derived neurotrophic factor (GDNF) is known for its neurorestorative and neuroprotective effects in rodent and primate models of Parkinson's disease (PD). When administered locally into the putamen of Parkinsonian subjects, early clinical studies showed its potential promise as a disease-modifying agent. However, the development of GDNF for the treatment of PD has been significantly clouded by findings of cerebellar toxicity after continuous intraputamenal high-dose administration in a 6-month treatment/3-month recovery toxicology study in rhesus monkeys. Specifically, multifocal cerebellar Purkinje cell loss affecting 1-21% of the cerebellar cortex was observed in 4 of 15 (26.7%; 95% confidence interval [CI]: 10.5-52.4%) animals treated at the highest dose level tested (3000μg/month). No cerebellar toxicity was observed at lower doses (450 and 900μg/month) in the same study, or at similar or higher doses (up to 10,000μg/month) in subchronic or chronic toxicology studies testing intermittent intracerebroventricular administration. While seemingly associated with the use of GDNF, the pathogenesis of the cerebellar lesions has not been fully understood to date. This review integrates available information to evaluate potential pathogenic mechanisms and provide a consolidated assessment of the findings. While other explanations are considered, the existing evidence is most consistent with the hypothesis that leakage of GDNF into cerebrospinal fluid during chronic infusions into the putamen down-regulates GDNF receptors on Purkinje cells, and that subsequent acute withdrawal of GDNF generates the observed lesions. The implications of these findings for clinical studies with GDNF are discussed.

  18. Long lasting cerebellar alterations after perinatal asphyxia in rats.

    Science.gov (United States)

    Campanille, Verónica; Saraceno, G Ezequiel; Rivière, Stéphanie; Logica, Tamara; Kölliker, Rodolfo; Capani, Francisco; Castilla, Rocío

    2015-07-01

    The developing brain may be particularly vulnerable to injury before, at and after birth. Among possible insults, hypoxia suffered as a consequence of perinatal asphyxia (PA) exhibits the highest incidence levels and the cerebellar circuitry appears to be particularly susceptible, as the cellular makeup and the quantity of inputs change quickly during days and weeks following birth. In this work, we have used a murine model to induce severe global PA in rats at the time of birth. Short-term cerebellar alterations within this PA model have been previously reported but whether such alterations remain in adulthood has not been conclusively determined yet. For this reason, and given the crucial cerebellar role in determining connectivity patterns in the brain, the aim of our work is to unveil long-term cerebellum histomorphology following a PA insult. Morphological and cytological neuronal changes and glial reaction in the cerebellar cortex were analyzed at postnatal 120 (P120) following injury performed at birth. As compared to control, PA animals exhibited: (1) an increase in molecular and granular thickness, both presenting lower cellular density; (2) a disarrayed Purkinje cell layer presenting a higher number of anomalous calbindin-stained cells. (3) focal swelling and marked fragmentation of microtubule-associated protein 2 (MAP-2) in Purkinje cell dendrites and, (4) an increase in glial fibrillary acidic protein (GFAP) expression in Bergmann cells and the granular layer. In conclusion, we demonstrate that PA produces long-term damage in cellular histomorphology in rat cerebellar cortex which could be involved in the pathogenesis of cognitive deficits observed in both animals and humans.

  19. Mucin-hypersecreting bile duct neoplasm characterized by clinicopathological resemblance to intraductal papillary mucinous neoplasm (IPMN of the pancreas

    Directory of Open Access Journals (Sweden)

    Harimoto Norifumi

    2007-08-01

    Full Text Available Abstract Background Although intraductal papillary mucinous neoplasm (IPMN of the pancreas is acceptable as a distinct disease entity, the concept of mucin-secreting biliary tumors has not been fully established. Case presentation We describe herein a case of mucin secreting biliary neoplasm. Imaging revealed a cystic lesion 2 cm in diameter at the left lateral segment of the liver. Duodenal endoscopy revealed mucin secretion through an enlarged papilla of Vater. On the cholangiogram, the cystic lesion communicated with bile duct, and large filling defects caused by mucin were observed in the dilated common bile duct. This lesion was diagnosed as a mucin-secreting bile duct tumor. Left and caudate lobectomy of the liver with extrahepatic bile duct resection and reconstruction was performed according to the possibility of the tumor's malignant behavior. Histological examination of the specimen revealed biliary cystic wall was covered by micropapillary neoplastic epithelium with mucin secretion lacking stromal invasion nor ovarian-like stroma. The patient has remained well with no evidence of recurrence for 38 months since her operation. Conclusion It is only recently that the term "intraductal papillary mucinous neoplasm (IPMN," which is accepted as a distinct disease entity of the pancreas, has begun to be used for mucin-secreting bile duct tumor. This case also seemed to be intraductal papillary neoplasm with prominent cystic dilatation of the bile duct.

  20. Defects in the CAPN1 Gene Result in Alterations in Cerebellar Development and Cerebellar Ataxia in Mice and Humans

    Directory of Open Access Journals (Sweden)

    Yubin Wang

    2016-06-01

    Full Text Available A CAPN1 missense mutation in Parson Russell Terrier dogs is associated with spinocerebellar ataxia. We now report that homozygous or heterozygous CAPN1-null mutations in humans result in cerebellar ataxia and limb spasticity in four independent pedigrees. Calpain-1 knockout (KO mice also exhibit a mild form of ataxia due to abnormal cerebellar development, including enhanced neuronal apoptosis, decreased number of cerebellar granule cells, and altered synaptic transmission. Enhanced apoptosis is due to absence of calpain-1-mediated cleavage of PH domain and leucine-rich repeat protein phosphatase 1 (PHLPP1, which results in inhibition of the Akt pro-survival pathway in developing granule cells. Injection of neonatal mice with the indirect Akt activator, bisperoxovanadium, or crossing calpain-1 KO mice with PHLPP1 KO mice prevented increased postnatal cerebellar granule cell apoptosis and restored granule cell density and motor coordination in adult mice. Thus, mutations in CAPN1 are an additional cause of ataxia in mammals, including humans.

  1. Cerebellar Transcranial Direct Current Stimulation (ctDCS): A Novel Approach to Understanding Cerebellar Function in Health and Disease.

    Science.gov (United States)

    Grimaldi, Giuliana; Argyropoulos, Georgios P; Bastian, Amy; Cortes, Mar; Davis, Nicholas J; Edwards, Dylan J; Ferrucci, Roberta; Fregni, Felipe; Galea, Joseph M; Hamada, Masahi; Manto, Mario; Miall, R Chris; Morales-Quezada, Leon; Pope, Paul A; Priori, Alberto; Rothwell, John; Tomlinson, S Paul; Celnik, Pablo

    2016-02-01

    The cerebellum is critical for both motor and cognitive control. Dysfunction of the cerebellum is a component of multiple neurological disorders. In recent years, interventions have been developed that aim to excite or inhibit the activity and function of the human cerebellum. Transcranial direct current stimulation of the cerebellum (ctDCS) promises to be a powerful tool for the modulation of cerebellar excitability. This technique has gained popularity in recent years as it can be used to investigate human cerebellar function, is easily delivered, is well tolerated, and has not shown serious adverse effects. Importantly, the ability of ctDCS to modify behavior makes it an interesting approach with a potential therapeutic role for neurological patients. Through both electrical and non-electrical effects (vascular, metabolic) ctDCS is thought to modify the activity of the cerebellum and alter the output from cerebellar nuclei. Physiological studies have shown a polarity-specific effect on the modulation of cerebellar-motor cortex connectivity, likely via cerebellar-thalamocortical pathways. Modeling studies that have assessed commonly used electrode montages have shown that the ctDCS-generated electric field reaches the human cerebellum with little diffusion to neighboring structures. The posterior and inferior parts of the cerebellum (i.e., lobules VI-VIII) seem particularly susceptible to modulation by ctDCS. Numerous studies have shown to date that ctDCS can modulate motor learning, and affect cognitive and emotional processes. Importantly, this intervention has a good safety profile; similar to when applied over cerebral areas. Thus, investigations have begun exploring ctDCS as a viable intervention for patients with neurological conditions.

  2. Abnormality in cerebellar blood flow in solo vertigo patients

    Energy Technology Data Exchange (ETDEWEB)

    Nagahori, Takeshi [Shakaihoken Takaoka Hospital, Toyama (Japan); Nishijima, Michiharu; Endo, Shunro; Takaku, Akira

    1997-03-01

    Little is known about the blood flow of the vertebrobasilar system as a cause of vertigo and dizziness. We used Xe-CT to study cerebellar blood flow in 53 patients who ranged in age from 35 to 85 years. The patients were divided into two groups. One of them was the vertigo group that comprised 28 patients with rotatory sensation, and the other, the non-vertigo group of 25 patients with a sensation other than rotation. At the stage of severe symptoms, there was decreased cerebellar blood flow in all patients of both, the vertigo and the non-vertigo groups, and a decrease in the bilateral cerebellar hemisphere was observed in five patients and in a unilateral hemisphere in three patients of the vertigo group. By comparison, in the non-vertigo group, unilateral decrease of cerebellar blood flow was observed in only one patient, and a bilateral decrease in five. At the stage of severe symptoms, the mean regional cerebellar blood flow was 40.5{+-}8.0 ml/100 g/min (n=16 sides) in the vertigo group and 45.3{+-}9.5 ml/100 g/min (n=12 sides) in the non-vertigo group. At the stage of moderate symptoms, blood flow image was normal in four of 14 vertigo patients and in seven of 12 non-vertigo patients. The mean regional blood flow was 47.8{+-}8.6 ml/100 g/min (n=28 sides) in the vertigo group and 47.1{+-}5.1 ml/100 g/min (n=24 sides) in the non-vertigo group. At the asymptomatic stage, a high proportion of normal blood flow images (nine of 16 vertigo patients and 10 of 10 non-vertigo patients) was observed. The mean regional cerebellar blood flow was 51.6{+-}10.7 ml/100 g/min (n=32 sides) in the vertigo group and 52.8{+-}8.5 ml/100 g/min (n=20 sides) in the non-vertigo group. This study demonstrates that a unilateral or bilateral decrease in blood flow of the vertebrobasilar system may cause vertigo and dizziness. It also shows that Xe-CT of the cerebellum may be a valuable examination modality for the diagnosis and treatment of vertigo and dizziness. (author)

  3. Lesions and Neoplasms of the Penis: A Review.

    Science.gov (United States)

    Heller, Debra S

    2016-01-01

    In addition to practitioners who care for male patients, with the increased use of high-resolution anoscopy, practitioners who care for women are seeing more men in their practices as well. Some diseases affecting the penis can impact on their sexual partners. Many of the lesions and neoplasms of the penis occur on the vulva as well. In addition, there are common and rare lesions unique to the penis. A review of the scope of penile lesions and neoplasms that may present in a primary care setting is presented to assist in developing a differential diagnosis if such a patient is encountered, as well as for practitioners who care for their sexual partners. A familiarity will assist with recognition, as well as when consultation is needed.

  4. Primary Intracranial Myoepithelial Neoplasm: A Potential Mimic of Meningioma.

    Science.gov (United States)

    Choy, Bonnie; Pytel, Peter

    2016-05-01

    Myoepithelial neoplasms were originally described in the salivary glands but their spectrum has been expanding with reports in other locations, including soft tissue. Intracranial cases are exceptionally rare outside the sellar region where they are assumed to be arising from Rathke pouch rests. Two cases of pediatric intracranial myoepithelial neoplasm in the interhemispheric fissure and the right cerebral hemisphere are reported here. Imaging studies suggest that the second case was associated with cerebrospinal fluid dissemination. Both cases showed typical variation in morphology and immunophenotype between more epithelioid and more mesenchymal features. The differential diagnosis at this particular anatomic location includes meningioma, which can show some overlap in immunophenotype since both tumors express EMA as well as GLUT1. One case was positive for EWSR1 rearrangement by fluorescence in situ hybridization. One patient is disease free at last follow-up while the other succumbed to the disease within days illustrating the clinical spectrum of these tumors.

  5. Pancreatic endocrine neoplasms: Epidemiology and prognosis of pancreatic endocrine tumors

    OpenAIRE

    2008-01-01

    Pancreatic endocrine neoplasms (PETs) are uncommon tumors with an annual incidence less than 1 per 100,000 persons per year in the general population. PETs that produce hormones resulting in symptoms are designated as functional. The majority of PETs are nonfunctional. Of the functional tumors, insulinomas are the most common, followed by gastrinomas. The clinical course of patients with PETs is variable and depends on the extent of the disease and the treatment rendered. Patients with comple...

  6. Blastic plasmacytoid dendritic cell neoplasm with absolute monocytosis at presentation

    Directory of Open Access Journals (Sweden)

    Jaworski JM

    2015-02-01

    Full Text Available Joseph M Jaworski,1,2 Vanlila K Swami,1 Rebecca C Heintzelman,1 Carrie A Cusack,3 Christina L Chung,3 Jeremy Peck,3 Matthew Fanelli,3 Micheal Styler,4 Sanaa Rizk,4 J Steve Hou1 1Department of Pathology and Laboratory Medicine, Hahnemann University Hospital/Drexel University College of Medicine, Philadelphia, PA, USA; 2Department of Pathology, Mercy Fitzgerald Hospital, Darby, PA, USA; 3Department of Dermatology, Hahnemann University Hospital/Drexel University College of Medicine, Philadelphia, PA, USA; 4Department of Hematology/Oncology, Hahnemann University Hospital/Drexel University College of Medicine, Philadelphia, PA, USA Abstract: Blastic plasmacytoid dendritic cell neoplasm is an uncommon malignancy derived from precursors of plasmacytoid dendritic cells. Nearly all patients present initially with cutaneous manifestations, with many having extracutaneous disease additionally. While response to chemotherapy initially is effective, relapse occurs in most, with a leukemic phase ultimately developing. The prognosis is dismal. While most of the clinical and pathologic features are well described, the association and possible prognostic significance between peripheral blood absolute monocytosis (>1.0 K/µL and blastic plasmacytoid dendritic cell neoplasm have not been reported. We report a case of a 68-year-old man who presented with a rash for 4–5 months. On physical examination, there were multiple, dull-pink, indurated plaques on the trunk and extremities. Complete blood count revealed thrombocytopenia, absolute monocytosis of 1.7 K/µL, and a negative flow cytometry study. Biopsy of an abdominal lesion revealed typical features of blastic plasmacytoid dendritic cell neoplasm. Patients having both hematologic and nonhematologic malignancies have an increased incidence of absolute monocytosis. Recent studies examining Hodgkin and non-Hodgkin lymphoma patients have suggested that this is a negative prognostic factor. The association between

  7. SCHWANNOMA OF TONGUE, A RARE INTRAORAL NEOPLASM: CASE REPORT

    OpenAIRE

    2015-01-01

    Schwannomas are truly encapsulated neoplasms of the human body and are always solitary. Only 1-2% occur intraorally with tongue being the most common site. A 20yr old male presented with a painless, slow growing swelling on the left side of the tongue for the past 1 year. Fine needle aspiration cytology was done and a benign mesenchymal lesion, possibility of Schwannoma was given. Biopsy of the tumour was performed and sent for histopathological examination which confirmed the diagnosis of Sc...

  8. THYROID NEOPLASMS AND PERITUMORAL MORPHOLOGY IN THYROIDECTOMY SPECIMENS

    Directory of Open Access Journals (Sweden)

    Padmavathi

    2015-10-01

    Full Text Available Thyroid neoplasms represent the most common malignancies of the endocrine system. They are known to occur in association with benign lesions of the thyroid, like multinodular goitre and Hashimoto thyroiditis. AIMS AND OBJECTIVES : To study the neoplasms of thyroid and their peritumoral morphology. MATERIALS AND METHODS : All thyroidectomy specimens received in the Department of Pathology, RRMCH over a period of three years from June 2011 to May 2014 were included in the study. Thyro idectomies for non - neoplastic lesions were also extensively sampled and morphologically studied, with focus on peritumoral morphology, in neoplasms. RESULTS : Of the one hundred and fifty four thyroidectomy specimens received over three years, one hundred a nd thirteen (73.4% were non - neoplastic, and forty one were neoplastic (26.6%. Colloid goitre and lymphocytic infiltrate were the most common features in the peritumoral thyroid tissue, followed by multinodular goitre . Hashimoto thyroiditis and Hurthle cell change were noted in 11.5% of cases. Tumors were multicentric in 11.5% of cases. Malignancy was detected in eight of the fifty nine thyroidectomies performed for multinodular goitre . Of the thirty four surgeries for Hashimoto thyroiditis, four were reported as malignant on histopathology. CONCLUSION : All thyroidectomies, including those operated for benign lesions, need to be extensively sampled and morphology studied due to the possibility of occult malignancy. Larger series need to be st udied to find a causal association between the two.

  9. Primary duodenal neoplasms: A retrospective clinico-pathological analysis

    Science.gov (United States)

    Bal, Amanjit; Joshi, Kusum; Vaiphei, Kim; Wig, JD

    2007-01-01

    AIM: To analyze the clinico-pathological spectrum of primary duodenal neoplasms. METHODS: A total of 55 primary duodenal neoplasms reported in the last 10 years after excluding ampullary and periampullary tumors were included in the study. Clinical details were noted and routine hematoxylin and eosin stained paraffin sections were studied for histological subtyping of the tumors. RESULTS: On histopathological examination primary duodenal neoplasms were categorized as: epithelial tumor in 27 cases (49.0%) including 10 cases of adenoma, 15 cases of adenocarcinoma, and 2 cases of Brunner gland adenoma; mesenchymal tumor in 9 cases (16.3%) consisting of 4 cases of gastrointestinal stromal tumor, 4 cases of smooth muscle tumor and I case of neurofibroma; lymphoproliferative tumor in 12 cases (21.8%), and neuroendocrine tumor in 7 cases (12.7%). CONCLUSION: Although non-ampullary/periampullary duodenal adenocarcinomas are rare, they constitute the largest group. Histopathological examination of primary duodenal tumors is important for correct histological subtyping. PMID:17373748

  10. Primary duodenal neoplasms:A retrospective clinico-pathological analysis

    Institute of Scientific and Technical Information of China (English)

    Amanjit Bal; Kusum Joshi; Kim Vaiphei; JD Wig

    2007-01-01

    AIM:To analyze the clinico-pathological spectrum of primary duodenal neoplasms.METHODS:A total of 55 primary duodenal neoplasms reported in the last 10 years after excluding ampullary and periampullary tumors were included in the study.Clinical details were noted and routine hematoxylin and eosin stained paraffin sections were studied for histological subtyping of the tumors.RESULTS:On histopathological examination primary duodenal neoplasms were categorized as:epithelial tumor in 27 cases(49.0%)including 10 cases of adenoma,15 cases of adenocarcinoma,and 2 cases of Brunner gland adenoma;mesenchymal tumor in 9 cases (16.3%)consisting of 4 cases of gastrointestinal stromal tumor,4 cases of smooth muscle tumor and I case of neurofibroma;lymphoproliferative tumor in 12 cases (21.8%),and neuroendocrine tumor in 7 cases(12.7%).CONCLUSION:Although non-ampullary/periampullary duodenal adenocarcinomas are rare,they constitute the largest group.Histopathological examination of primary duodenal tumors is important for correct histological subtyping.

  11. A Study Of Sporadic Adult Onset Degenerative Cerebellar Ataxias

    Directory of Open Access Journals (Sweden)

    Sinha K K

    1999-01-01

    Full Text Available Twenty-four cases of sporadic olivo-ponto-cerebellar atrophy (OPCA of adult onset were studied over a period of two years. Results suggest that this disorder has its usual onset in the 5th and 6th decade of life with a male: female ratio of 2:1. It manifests clinically with gait ataxia in all, dysarthria, other cerebellar signs and autonomic involvement in vast majority. There were features of basal ganglia involvement in some. No known identifiable environmental cause was found and genetically they are quite distinct from the known autosomal dominant spinocerebellar ataxias though sporadic occurrence in recessive inheritance or a de novo mutation could not be ruled out completely, but it is unlikely.

  12. Palatoglossal fusion with cleft palate and hypoplasia of cerebellar vermis

    Directory of Open Access Journals (Sweden)

    Shailesh Solanki

    2016-01-01

    Full Text Available A new-born male presented within 12 h of birth with respiratory distress. On examination and workup, he had palatoglossal fusion, cleft palate and hypoplasia of the cerebellar vermis. A 2.5 Fr endotracheal tube was inserted into the pharynx through nostril as a nasopharyngeal stent, following which his respiratory distress improved. Once child was optimised, then feeding was started by nasogastric tube and feeds were tolerated well. Elective tracheostomy and gastrostomy were done, followed by release of adhesions between the tongue and palate at a later stage. Review of literature suggests that palatoglossal fusion is uncommon and presents as an emergency. Mostly, these oral synechiae are associated with digital and/or cardiac anomaly. Other disorders associated with intra-oral synechiae include congenital alveolar synechiae, van der Woude syndrome, popliteal pterygium syndrome and oromandibular limb hypogenesis syndrome. The authors report a hitherto undescribed association of palatoglossal fusion with cleft palate and hypoplasia of the cerebellar vermis.

  13. Cerebellar deficits and hyperactivity in mice lacking Smad4.

    Science.gov (United States)

    Zhou, Yong-Xing; Zhao, Mingrui; Li, Dan; Shimazu, Kazuhiro; Sakata, Kazuko; Deng, Chu-Xia; Lu, Bai

    2003-10-24

    Smad4 is a central mediator of TGF-beta signals, which are known to play essential roles in many biological processes. Using a Cre-loxP approach to overcome early embryonic lethality, we have studied functions of TGF-beta/Smad4 signals in the central nervous system (CNS). No obvious deficits were detected in mice carrying the targeted disruption of Smad4 in the CNS. The overall morphology of the hippocampus appeared normal. There was no change in the proliferation of neuronal precursor cells, nor in several forms of synaptic plasticity. In contrast, deletion of Smad4 resulted in a marked decrease in the number of cerebellar Purkinje cells and parvalbumin-positive interneurons. Accompanied by the abnormality in the cerebellum, mutant mice also exhibited significantly increased vertical activity. Thus, our study reveals an unexpected role for Smad4 in cerebellar development and in the control of motor function.

  14. Crossed cerebellar diaschisis demonstrated by SPECT in hemiplegic children

    Energy Technology Data Exchange (ETDEWEB)

    Hamano, Shin-ichiro; Nara, Takahiro; Nozaki, Hidetsugu; Fukushima, Kiyomi (Saitama Children' s Medical Center, Iwatsuki (Japan)); Imai, Masayuki; Kumagai, Koumei; Maekawa, Kihei

    1991-01-01

    Crossed cerebellar diaschisis (CCD) in twenty five children with hemiplegia were studied using single photon emission computed tomography (SPECT) with N-isopropyl-p-I-123-iodoamphetamine. Seven of twenty-five patients had cerebral palsy, and the others were impaired by acquired brain injury between ten months and fourteen years of age. CCD was demonstrated in five patients (20%), who were impaired by acquired brain injury after seven years of age. CCD could never be detected in patients with cerebral palsy. Ipsilateral cerebellar diaschisis was also demonstrated in two patients with cerebral palsy and three with early acquired brain injury before three years of age. It is suggested that diaschisis presents itself as a different form in a contralateral and ipsilateral cerebellum before three years of age from a form which presents after seven years of age. (author).

  15. Abnormal ocular motility with brainstem and cerebellar disorders.

    Science.gov (United States)

    Carlow, T J; Bicknell, J M

    1978-01-01

    The disorders of ocular motility seen in association with brainstem or cerebellar disorders may point to rather specific anatomical or pathological correlations. Pontine gaze palsy reflects involvement of the pontine paramedian reticular formation. Internuclear ophthalmoplegia signifies a lesion in the medial longitudinal fasciculus. Skew deviation may result from a lesion anywhere in the posterior fossa. Ocular bobbing typically results from a pontine lesion. The Sylvian aqueduct syndrome is characteristic of involvement in the upper midbrain-pretectal region, usually a pinealoma. Cerebellar lesions may be manifested by gaze paresis, skew deviation, disturbances of saccadic or smooth pursuit movements, ocular myoclonus, or several characteristic forms of nystagmus. Familiarity with these disorders may be of great help to the physician dealing with a patient with a possible posterior fossa lesion.

  16. Correlation between vestibular habituation and postural recovery in cerebellar patients.

    Science.gov (United States)

    Suarez, H; Caffa, C; Macadar, O

    1992-01-01

    Vestibular habituation was studied in normal subjects and in patients with cerebellar disease using a stimulation paradigm proposed in this paper. Six caloric stimuli were repeated daily in the same ear during six days and electronystagmographic responses at the beginning and the end of that period were compared. The normal behaviour was a clear reduction of the response across time. Two groups of cerebellar patients were identified by their ability to recover from positional imbalance after treatment. Compensated patients responded to repeated caloric stimulation in the same way as normal subjects. Conversely, uncompensated patients increased their response after the stimulation paradigm. The role played by the cerebellum in vestibular plasticity is discussed together with the observed correlation between vestibular habituation and the ability for postural recovery to occur.

  17. Cerebellar damage impairs internal predictions for sensory and motor function

    OpenAIRE

    Therrien, Amanda S.; Bastian, Amy J.

    2015-01-01

    The cerebellum is connected to cerebral areas that subserve a range of sensory and motor functions. In this review, we summarize new literature demonstrating deficits in visual perception, proprioception, motor control, and motor learning performance following cerebellar damage. In particular, we highlight novel results that together suggest a general role of the cerebellum in estimating and predicting movement dynamics of the body and environmental stimuli. These findings agree with the hypo...

  18. Hereditary lissencephaly and cerebellar hypoplasia in Churra lambs

    OpenAIRE

    Pérez Pérez, Valentín; Suárez-Vega, Aroa; Fuertes, M.; Benavides, Julio; Delgado, L.; Ferreras, Mª del Carmen; Arranz, Juan José

    2013-01-01

    Abstract Background Lissencephaly is a rare developmental brain disorder in veterinary and human medicine associated with defects in neuronal migration leading to a characteristic marked reduction or absence of the convolutional pattern of the cerebral hemispheres. In many human cases the disease has a genetic basis. In sheep, brain malformations, mainly cerebellar hypoplasia and forms of hydrocephalus, are frequently due to in utero viral infections. Although breed-related malformations of t...

  19. Degenerative cerebellar diseases and differential diagnoses; Degenerative Kleinhirnerkrankungen und Differenzialdiagnosen

    Energy Technology Data Exchange (ETDEWEB)

    Reith, W.; Roumia, S.; Dietrich, P. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany)

    2016-11-15

    Cerebellar syndromes result in distinct clinical symptoms, such as ataxia, dysarthria, dysmetria, intention tremor and eye movement disorders. In addition to the medical history and clinical examination, imaging is particularly important to differentiate other diseases, such as hydrocephalus and multi-infarct dementia from degenerative cerebellar diseases. Degenerative diseases with cerebellar involvement include Parkinson's disease, multiple system atrophy as well as other diseases including spinocerebellar ataxia. In addition to magnetic resonance imaging (MRI), nuclear medicine imaging investigations are also helpful for the differentiation. Axial fluid-attenuated inversion recovery (FLAIR) and T2-weighted sequences can sometimes show a signal increase in the pons as a sign of degeneration of pontine neurons and transverse fibers in the basilar part of the pons. The imaging is particularly necessary to exclude other diseases, such as normal pressure hydrocephalus (NPH), multi-infarct dementia and cerebellar lesions. (orig.) [German] Klinisch imponieren Kleinhirnsyndrome durch Ataxie, Dysarthrie, Dysmetrie, Intentionstremor und Augenbewegungsstoerungen. Neben der Anamnese und klinischen Untersuchung ist die Bildgebung v. a. wichtig um andere Erkrankungen wie Hydrozephalus und Multiinfarktdemenz von degenerativen Kleinhirnerkrankungen zu differenzieren. Zu den degenerativen Erkrankungen mit Kleinhirnbeteiligung gehoeren der Morbus Parkinson, die Multisystematrophie sowie weitere Erkrankungen einschliesslich der spinozerebellaeren Ataxien. Neben der MRT sind auch nuklearmedizinische Untersuchungen zur Differenzierung hilfreich. Axiale Fluid-attenuated-inversion-recovery(FLAIR)- und T2-gewichtete Sequenzen koennen mitunter eine Signalsteigerung im Pons als Ausdruck einer Degeneration der pontinen Neuronen und transversalen Bahnen im Brueckenfuss zeigen. Die Bildgebung ist aber v. a. notwendig, um andere Erkrankungen wie Normaldruckhydrozephalus

  20. Acute cerebellar ataxia: A neurological manifestation in malaria

    Directory of Open Access Journals (Sweden)

    Peddametla Shravan Kumar

    2014-01-01

    Full Text Available Malaria is a vector-borne disease transmitted by the bite of an infected female anopheles mosquito presents with varied clinical manifestations. Neurological manifestations include headaches, confusion, convulsions, hemiplegia, ataxia, cerebral palsy, cortical blindness, and Guillain-Barre syndrome (GBS. We are presenting a case report of acute cerebellar ataxia in a 20-year-old male patient who presented with fever and positive for Plasmodium vivax and Plasmodium falciparum malaria antibodies.

  1. Phenytoin-induced cerebellar atrophy in an epileptic boy

    Directory of Open Access Journals (Sweden)

    Nithin Kumar

    2013-01-01

    Full Text Available Epilepsy is an important health problem due to its high prevalence and potential for causing long-term morbidity. It is commonly treated in children with phenytoin sodium. It has wide pharmacokinetic variability and a narrow therapeutic range that leads to toxicity. Here, we report a case of phenytoin-induced cerebellar atrophy in a 16-year-old epileptic boy who presented to the hospital with a viral infection.

  2. Successful treatment of isolated cerebellar cysticercosis with albendazole

    Institute of Scientific and Technical Information of China (English)

    朱利平; 石尧忠; 潘孝彰; 莫凌; 翁心华

    2003-01-01

    Neurocysticercosis (NCC) is a parasitic disease of the central nervous system (CNS) found world-wide.1 NCC is the most common cause of parasitic infection of CNS in China. Patients with NCC are treated successfully with chemotherapy. However, isolated cystic lesions sited in the cerebellum are usually treated by direct surgical excision as a primary therapeutic modality.2 We present here a case of isolated cerebellar vermis cysticercosis successfully treated with albendazole.

  3. Patterns of regional cerebellar atrophy in genetic frontotemporal dementia

    Directory of Open Access Journals (Sweden)

    Martina Bocchetta

    2016-01-01

    Conclusion: There appears to be a differential pattern of cerebellar atrophy in the major genetic forms of FTD, being relatively spared in GRN, localized to the lobule VIIa-Crus I in the superior-posterior region of the cerebellum in C9orf72, the area connected via the thalamus to the prefrontal cortex and involved in cognitive function, and localized to the vermis in MAPT, the ‘limbic cerebellum’ involved in emotional processing.

  4. Cerebellar Herniation after Lumbar Puncture in Galactosemic Newborn

    Directory of Open Access Journals (Sweden)

    Salih Kalay

    2011-09-01

    Full Text Available Cerebral edema resulting in elevated intracranial pressure is a well-known complication of galactosemia. Lumbar puncture was performed for the diagnosis of clinically suspected bacterial meningitis. Herniation of cerebral tissue through the foramen magnum is not a common problem in neonatal intensive care units because of the open fontanelle in infants. We present the case of a 3-week-old infant with galactosemia who presented with signs of cerebellar herniation after lumbar puncture.

  5. [Aneurysm of the anterior inferior cerebellar artery: case report].

    Science.gov (United States)

    Adorno, Juan Oscar Alarcón; de Andrade, Guilherme Cabral

    2002-12-01

    The intracranial aneurysms of the posterior circulation have been reported between 5 and 10% of all cerebral aneurysms and the aneurysms of the anterior inferior cerebellar artery (AICA) are considered rare, can cause cerebello pontine angle (CPA) syndrome with or without subarachnoid hemorrhage. Since 1948 few cases were described in the literature. We report on a 33 year-old female patient with subarachnoid hemorrhage due to sacular aneurysm of the left AICA. She was submitted to clipage of the aneurysm without complications.

  6. Distal posterior inferior cerebellar artery aneurysm in a child

    Directory of Open Access Journals (Sweden)

    J. Francisco Salomão

    1992-06-01

    Full Text Available The case of a 7-year-old boy presenting with recurrent episodes of subarachnoid hemorrhage due to a distal posterior inferior cerebellar artery aneurysm (PICA, successfully operated, is reported.' The low incidence of intracranial aneurysms in the first decade of life and the rare occurrence of distal PICA aneurysms are unusual features of this case. The theories regarding the origin of intracranial berry aneurysms are discussed.

  7. Patient adaptable cerebellar retractor system: Use in posterior fossa surgery

    Directory of Open Access Journals (Sweden)

    Hamid Borghei-Razavi

    2015-06-01

    Full Text Available A new patient adaptable dual use soft tissue spreader and cerebellar retractor system designed for use during surgery of the posterior fossa is described. We found that this new retractor design allowed for excellent exposure, plus greater freedom and dexterity during the posterior fossa surgery. This novel instrument is an improvement over the existing instrument, because it provided more force/power transmission from pins/connectors to the brain spatula via the shorter flexible arm.

  8. Successfull management of a life threatening cerebellar haemorrhage following spine surgery - a case report -.

    Science.gov (United States)

    Pallud, Johan; Belaïd, Hayat; Aldea, Sorin

    2009-06-01

    Cerebellar haemorrhages are rare life-threatening complications following spine surgery that present challenges for their diagnostic and their therapeutic management. Their patho-physiology remains unclear.We report a case of a life-threatening cerebellar haemorrhage secondary to an occult dural tear following a planned L5-S1 laminectomy. The patient was treated with emergent external ventriculostomy following by a posterior fossa decompressive craniectomy. Cerebellar haemorrhages have to be suspected systematically when unexpected neurological signs occur after spine surgery since their rapid management lead to favourable outcomes. The present imaging findings allow us proposing that cerebellar haemorrhages result primarily from superior cerebellar venous stretching and tearing, and that cerebellar infarction and swelling occur secondarily.

  9. Magnetic resonance imaging in the diagnosis of cerebellar ataxias

    Energy Technology Data Exchange (ETDEWEB)

    Kato, Hiroyuki; Takase, Sadao; Mochizuki, Hiroshi; Kogure, Kyuya; Yamada, Kenji; Hishinuma, Takashi; Matsuzawa, Taiju.

    1987-11-01

    Radiological evaluation in order to quantitatively analyse the size of structures in the posterior fossa using magnetic resonance imaging (MRI) was performed in the patients with spinocerebellar degeneration (SCD). The subjects consisted of 17 patients including 11 OPCA (olivopontocerebellar atrophy)-type and 6 LCCA (late cortical cerebellar atrophy)-type SCD patients, and their disease was in the initial phase. Using a mid-line sagittal view, quantitative measurements of the cerebellum, pons and the medulla oblongata were performed. In the OPCA-type SCD patients, the area, and the longitudinal and anteroposterior diameters of the cerebellar vermis, the area and the anteroposterior diameter of the pons, the height of the fourth ventricle, and the anteroposterior diameter of the medulla oblongata were significantly smaller than those of normal subjects. In the LCCA-type SCD patients, only the area and the anteroposterior diameter of the cerebellar vermis were smaller than those of the normal. As a result, MRI is useful in the diagnosis of SCD, and in the differential diagnosis between the OPCA-type and the LCCA-type SCDs.

  10. Behavior modification after inactivation of cerebellar dentate nuclei.

    Science.gov (United States)

    Peterson, Todd C; Villatoro, Lee; Arneson, Tom; Ahuja, Brittany; Voss, Stephanie; Swain, Rodney A

    2012-08-01

    Effort-based decision making occurs when subjects are given a choice between a reward available at a high response cost and a reward available at a low response cost and is altered in individuals with disorders such as autism or particular patterns of brain injury. The current study explored the relationship between effort-based decision making and reinforcement characteristics in the T maze. This was done using both normal animals and animals with bilateral inactivation of the cerebellar dentate nuclei. Rats chose between alternatives in which one arm contained high-density reinforcement (HR) and the other arm contained low-density reinforcement (LR). During training, the HR arm was obstructed and the point at which the animal no longer worked for reinforcement (breaking point) was determined. The cerebellar dentate nuclei were then transiently inactivated and once again breaking points were assessed. The results indicated that inactivation of the dentate nucleus disrupted effort-based decision making. Additionally, altering both the palatability and the magnitude of the reinforcement were assessed in an attempt to reestablish the original preinactivation breaking point. It was hypothesized that an increase in the strength or magnitude of the reinforcement would promote an increase in the breaking point of the animal even when the cerebellum was inactivated. The results indicated that with both strategies animals effectively reestablished original breaking points. The results of this study will inform the current literature regarding the modification of behavior after brain injury and further the understanding of the behavioral deficits associated with cerebellar dysfunction.

  11. β-Catenin is critical for cerebellar foliation and lamination.

    Directory of Open Access Journals (Sweden)

    Jing Wen

    Full Text Available The cerebellum has a conserved foliation pattern and a well-organized layered structure. The process of foliation and lamination begins around birth. β-catenin is a downstream molecule of Wnt signaling pathway, which plays a critical role in tissue organization. Lack of β-catenin at early embryonic stages leads to either prenatal or neonatal death, therefore it has been difficult to resolve its role in cerebellar foliation and lamination. Here we used GFAP-Cre to ablate β-catenin in neuronal cells of the cerebellum after embryonic day 12.5, and found an unexpected role of β-catenin in determination of the foliation pattern. In the mutant mice, the positions of fissure formation were changed, and the meninges were improperly incorporated into fissures. At later stages, some lobules were formed by Purkinje cells remaining in deep regions of the cerebellum and the laminar structure was dramatically altered. Our results suggest that β-catenin is critical for cerebellar foliation and lamination. We also found a non cell-autonomous role of β-catenin in some developmental properties of major cerebellar cell types during specific stages.

  12. Cerebellar vermis H₂ receptors mediate fear memory consolidation in mice.

    Science.gov (United States)

    Gianlorenço, A C L; Riboldi, A M; Silva-Marques, B; Mattioli, R

    2015-02-01

    Histaminergic fibers are present in the molecular and granular layers of the cerebellum and have a high density in the vermis and flocullus. Evidence supports that the cerebellar histaminergic system is involved in memory consolidation. Our recent study showed that histamine injections facilitate the retention of an inhibitory avoidance task, which was abolished by pretreatment with an H2 receptor antagonist. In the present study, we investigated the effects of intracerebellar post training injections of H1 and H2 receptor antagonists as well as the selective H2 receptor agonist on fear memory consolidation. The cerebellar vermi of male mice were implanted with guide cannulae, and after three days of recovery, the inhibitory avoidance test was performed. Immediately after a training session, animals received a microinjection of the following histaminergic drugs: experiment 1, saline or chlorpheniramine (0.016, 0.052 or 0.16 nmol); experiment 2, saline or ranitidine (0.57, 2.85 or 5.07 nmol); and experiment 3, saline or dimaprit (1, 2 or 4 nmol). Twenty-four hours later, a retention test was performed. The data were analyzed using one-way analysis of variance (ANOVA) and Duncan's tests. Animals microinjected with chlorpheniramine did not show any behavioral effects at the doses that we used. Intra-cerebellar injection of the H2 receptor antagonist ranitidine inhibited, while the selective H2 receptor agonist dimaprit facilitated, memory consolidation, suggesting that H2 receptors mediate memory consolidation in the inhibitory avoidance task in mice.

  13. Sustained Reduction of Cerebellar Activity in Experimental Epilepsy

    Directory of Open Access Journals (Sweden)

    Kim Rijkers

    2015-01-01

    Full Text Available Clinical and experimental evidence suggests a role for the cerebellum in seizure control, while no data are available on cerebellar activity between seizures. We hypothesized that interictal regional activity of the deep cerebellar nuclei is reduced in epilepsy and tested this in an animal model by using ΔFosB and cytochrome oxidase (COX (immunohistochemistry. The expression of these two markers of neuronal activity was analysed in the dentate nucleus (DN, interpositus nucleus (IN, and fastigial nucleus (FN of the cerebellum of fully amygdala kindled rats that were sacrificed 48 hours after their last seizure. The DN and FN of kindled rats exhibited 25 to 29% less ΔFosB immunopositive cells than their respective counterpart in sham controls (P<0.05. COX expression in the DN and FN of kindled animals was reduced by 32 to 33% compared to respective control values (P<0.05. These results indicate that an epileptogenic state is characterized by decreased activity of deep cerebellar nuclei, especially the DN and FN. Possible consequences may include a decreased activation of the thalamus, contributing to further seizure spread. Restoration of FN activity by low frequency electrical stimulation is suggested as a possible treatment option in chronic epilepsy.

  14. Paraneoplastic cerebellar degeneration with anti-Yo antibodies - a review.

    Science.gov (United States)

    Venkatraman, Anand; Opal, Puneet

    2016-08-01

    The ataxic syndrome associated with Anti-Yo antibody, or Purkinje cell cytoplasmic antibody type 1 (PCA1), is the most common variant of paraneoplastic cerebellar degeneration (PCD). The typical presentation involves the subacute development of pancerebellar deficits with a clinical plateau within 6 months. The vast majority of cases have been reported in women with pelvic or breast tumors. Magnetic resonance imaging of the brain is often normal in the early stages, with cerebellar atrophy seen later. The underlying mechanism is believed to be an immunological reaction to cerebellar degeneration-related protein 2 (CDR2), a protein usually found in the cerebellum that is ectopically produced by tumor cells. Although both B- and T-cell abnormalities are seen, there is debate about the relative importance of the autoantibodies and cytotoxic T lymphocytes in the neuronal loss. Cerebrospinal fluid abnormalities, primarily elevated protein, lymphocytic pleocytosis, and oligoclonal bands, are common in the early stages. The low prevalence of this condition has not allowed for large-scale randomized controlled trials. Immunotherapies, such as steroids, intravenous immune globulins, and plasma exchange, have been extensively used in managing this condition, with limited success. Although some reports indicate benefit from antitumor therapies like surgery and chemotherapy, this has not been consistently observed. The prognosis for anti-Yo PCD is almost uniformly poor, with most patients left bedridden. Further studies are required to clarify the pathophysiology and provide evidence-based treatment options.

  15. The Changeable Nervous System: Studies On Neuroplasticity In Cerebellar Cultures

    Science.gov (United States)

    Seil, Fredrick J.

    2014-01-01

    Circuit reorganization after injury was studied in a cerebellar culture model. When cerebellar cultures derived from newborn mice were exposed at explantation to a preparation of cytosine arabinoside that destroyed granule cells and oligodendrocytes and compromised astrocytes, Purkinje cells surviving in greater than usual numbers were unensheathed by astrocytic processes and received twice the control number of inhibitory axosomatic synapses. Purkinje cell axon collaterals sprouted and many of their terminals formed heterotypical synapses with other Purkinje cell dendritic spines. The resulting circuit reorganization preserved inhibition in the cerebellar cortex. Following this reorganization, replacement of the missing granule cells and glia was followed by a restitution of the normal circuitry. Most of these developmental and reconstructive changes were not dependent on neuronal activity, the major exception being inhibitory synaptogenesis. The full complement of inhibitory synapses did not develop in the absence of neuronal activity, which could be mitigated by application of exogenous TrkB receptor ligands. Inhibitory synaptogenesis could also be promoted by activity-induced release of endogenous TrkB receptor ligands or by antibody activation of the TrkB receptor. PMID:24933693

  16. Inpatient Rehabilitation Performance of Patients with Paraneoplastic Cerebellar Degeneration

    Science.gov (United States)

    Fu, Jack B.; Raj, Vishwa S.; Asher, Arash; Lee, Jay; Guo, Ying; Konzen, Benedict S.; Bruera, Eduardo

    2014-01-01

    Objective To evaluate the functional improvement of rehabilitation inpatients with paraneoplastic cerebellar degeneration. Design Retrospective Review Setting Three tertiary referral based hospitals. Interventions Medical records were retrospectively analyzed for demographic, laboratory, medical and functional data. Main Outcome Measure Functional Independence Measure (FIM) Participants Cancer rehabilitation inpatients admitted to three different cancer centers with a diagnosis of paraneoplastic cerebellar degeneration (n=7). Results All 7 patients were white females. Median age was 62. Primary cancers included ovarian carcinoma (2), small cell lung cancer (2), uterine carcinoma (2), and invasive ductal breast carcinoma. Mean admission total FIM score was 61.0 (SD=23.97). Mean discharge total FIM score was 73.6 (SD=29.35). The mean change in total FIM score was 12.6 (p=.0018). The mean length of rehabilitation stay was 17.1 days. The mean total FIM efficiency was 0.73. 5/7 (71%) patients were discharged home. 1/7 (14%) was discharged to a nursing home. 1/7 (14%) transferred to the primary acute care service. Conclusions This is the first study to demonstrate the functional performance of a group of rehabilitation inpatients with paraneoplastic cerebellar degeneration. Despite the poor neurologic prognosis associated with this syndrome, these patients made significant functional improvements on inpatient rehabilitation. When appropriate, inpatient rehabilitation should be considered. Further studies with larger sample sizes are needed. PMID:25051460

  17. Morphology and biomechanical properties of cerebellar arteries in adults

    Directory of Open Access Journals (Sweden)

    Olga A. Fomkina

    2016-06-01

    Full Text Available The goal was to analyze the variability of a number of morphometric and biomechanical parameters of cerebellar arteries in adults aged 20-74 years. Material and Methods ― 179 samples of cerebellar arteries, obtained by autopsy of adults without acute cerebrovascular pathology have been studied; 24 preparations of arterial complexes «arterial circle – cerebral arteries» from scientific collection of Human Anatomy Department of Saratov State Medical University (Saratov, Russia have been also investigated. Research methods were: preparation, microscopy, experiments on uniaxial longitudinal stretching at a tensile testing machine Tira Test 28005 (TIRA GmbH, Germany. We studied outer diameter, angle of divergence, overall strength and maximal relative deformation of superior (SCA, anterior inferior (AICA and posterior inferior cerebellar arteries (PICA. Results and Conclusion ― It was revealed that SCA was characterized by the largest diameter and angle of divergence, the most strength and extensibility. AICA and PICA had no significant differences of the studied parameters. It was noted that AICA originated in the lower third part of basilar artery 1.5 times more likely than in the middle third part of this artery.

  18. Metalinguistic deficits in patients with cerebellar dysfunction: empirical support for the dysmetria of thought theory.

    Science.gov (United States)

    Guell, Xavier; Hoche, Franziska; Schmahmann, Jeremy D

    2015-02-01

    The cerebellar cognitive affective syndrome (CCAS) includes disruption of linguistic processing such as verbal fluency, verbal working memory, grammar, and speech perception. We set out to examine linguistic capabilities in patients with cerebellar lesions to determine which domains are spared and which impaired and to evaluate the underlying cognitive structure of these deficits. Forty-four patients with cerebellar disease were compared to 40 healthy controls on the Oral Sentence Production Test (OSPT) which assesses production of sentences with correct syntactic structure and semantic quality. Twenty-five of these cerebellar patients and 25 controls received the Test of Language Competence-Expanded (TLC-E) that assesses metalinguistic ability. The OSPT failed to reveal differences between patients and controls. In contrast, all cerebellar patients were impaired on each of the four TLC-E subtests. Differences between isolated cerebellar and complex cerebrocerebellar patients were nonsignificant. These results confirm and extend prior observations of the TLC-E in patients with cerebellar lesions and suggest three separate but related language impairments following cerebellar dysfunction: (1) disruption in automatic adjustment of intact grammatical and semantic abilities to a linguistic context in sentence production, (2) disruption in automatic adjustment to a linguistic context in sentence interpretation, and (3) disruption of cognitive processes essential for linguistic skills, such as analysis and sequential logical reasoning. These findings are consistent with the unifying framework of the universal cerebellar transform and the dysmetria of thought theory and provide new insights into the nature of the cognitive impairments in patients with the CCAS.

  19. Medical management of cerebellar abscess: a case report and review of the literature.

    Science.gov (United States)

    Turner, Ryan C; Dodson, Sean C; Rosen, Charles L

    2011-01-01

    A large abscess of the posterior fossa often warrants surgical intervention. We report a case of a 50-year-old male presenting with a cerebellar abscess measuring 2.8 cm x 1.6 cm located in the left cerebellar hemisphere at the level of the middle cerebellar peduncle that was treated conservatively and successfully with antibiotics. Therapeutic management options are discussed in regards to this case specifically as well as a review of the literature. This case illustrates the successful medical management of a cerebellar abscess of otogenic origin in an adult, a unique result in terms of abscess size and age of the patient.

  20. Serum hyaluronic acid in patients with disseminated neoplasm.

    OpenAIRE

    Manley, G.; Warren, C

    1987-01-01

    Hyaluronic acid concentrations were measured by a laser nephelometric assay in serum samples from 50 patients with advanced disseminated neoplasm and 50 healthy controls matched for age and sex. The identity of hyaluronic acid was confirmed by a combination of electrophoretic and enzymatic techniques. The mean serum hyaluronic acid concentration for the control group was 1.09 mg/l, with a range of 0-4 mg/l. The mean concentration for patients with neoplastic disease was 10.38 mg/l, with a ran...

  1. Histologic and Immunohistochemical classification of 41 bovine adrenal gland neoplasms

    DEFF Research Database (Denmark)

    Grossi, Anette Blak; Leifsson, Páll S.; Jensen, Henrik Elvang;

    2013-01-01

    Tumors of the adrenal glands are among the most frequent tumors in cattle; however, few studies have been conducted to describe their characteristics. The aim of this study was to classify 41 bovine adrenal neoplasms from 40 animals based on macroscopic and histologic examination, including....... An immunohistochemistry panel consisting of antibodies against melan A, synaptophysin, and CNPase was considered most useful to classify bovine adrenal tumors. However, the distinction between benign and malignant adrenocortical tumors was based on histologic features as in human medicine....

  2. Cellular schwannoma: a benign neoplasm sometimes overdiagnosed as sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Alberghini, M. [Dept. of Surgical Pathology, Rizzoli Institute, Bologna (Italy); Anatomia Patologica, Istituto Rizzoli, Bologna (Italy); Zanella, L.; Bacchini, P.; Bertoni, F. [Dept. of Surgical Pathology, Rizzoli Institute, Bologna (Italy)

    2001-06-01

    A case of cellular schwannoma originating in the left lumbar paraspinal region is described. The diagnosis was originally made on needle biopsy material. The histological examination is usually not sufficient to correctly diagnose this benign neoplasm. Bone erosion, neurological symptoms, caused by compression of the spinal roots, together with hypercellularity, pleomorphism and an occasional increase in mitotic activity, may lead to an erroneous diagnosis of malignancy. Immunohistochemistry and ultrastructural analysis are helpful in confirming the diagnosis. The recognition of this entity avoids unnecessary overtreatment of these patients. (orig.)

  3. Distinct molecular features of different macroscopic subtypes of colorectal neoplasms.

    Directory of Open Access Journals (Sweden)

    Kenichi Konda

    Full Text Available BACKGROUND: Colorectal adenoma develops into cancer with the accumulation of genetic and epigenetic changes. We studied the underlying molecular and clinicopathological features to better understand the heterogeneity of colorectal neoplasms (CRNs. METHODS: We evaluated both genetic (mutations of KRAS, BRAF, TP53, and PIK3CA, and microsatellite instability [MSI] and epigenetic (methylation status of nine genes or sequences, including the CpG island methylator phenotype [CIMP] markers alterations in 158 CRNs including 56 polypoid neoplasms (PNs, 25 granular type laterally spreading tumors (LST-Gs, 48 non-granular type LSTs (LST-NGs, 19 depressed neoplasms (DNs and 10 small flat-elevated neoplasms (S-FNs on the basis of macroscopic appearance. RESULTS: S-FNs showed few molecular changes except SFRP1 methylation. Significant differences in the frequency of KRAS mutations were observed among subtypes (68% for LST-Gs, 36% for PNs, 16% for DNs and 6% for LST-NGs (P<0.001. By contrast, the frequency of TP53 mutation was higher in DNs than PNs or LST-Gs (32% vs. 5% or 0%, respectively (P<0.007. We also observed significant differences in the frequency of CIMP between LST-Gs and LST-NGs or PNs (32% vs. 6% or 5%, respectively (P<0.005. Moreover, the methylation level of LINE-1 was significantly lower in DNs or LST-Gs than in PNs (58.3% or 60.5% vs. 63.2%, P<0.05. PIK3CA mutations were detected only in LSTs. Finally, multivariate analyses showed that macroscopic morphologies were significantly associated with an increased risk of molecular changes (PN or LST-G for KRAS mutation, odds ratio [OR] 9.11; LST-NG or DN for TP53 mutation, OR 5.30; LST-G for PIK3CA mutation, OR 26.53; LST-G or DN for LINE-1 hypomethylation, OR 3.41. CONCLUSION: We demonstrated that CRNs could be classified into five macroscopic subtypes according to clinicopathological and molecular differences, suggesting that different mechanisms are involved in the pathogenesis of colorectal

  4. [Intraductal papillary mucinous neoplasm of the pancreas, IPMN].

    Science.gov (United States)

    Sirén, Jukka

    2013-01-01

    With the development and increasing use of imaging techniques, intraductal papillary mucinous neoplasm (IPMN) is being detected with increasing frequency. Two forms of the disease are distinguished, the rare main duct form and the common accessory pancreatic duct form. The former often progresses to malignancy, the latter only seldom. The mixed form of IPMN exhibits features of both forms. In main duct IPMN, mucin production obstructs the pancreatic duct causing its dilatation and often symptoms typical of chronic pancreatitis. Main duct IPMN is always an indication for surgery, whereas monitoring is often sufficient for side duct IPMN.

  5. Clinical and genetic analysis of a four-generation family with a distinct autosomal dominant cerebellar ataxia

    NARCIS (Netherlands)

    Schelhaas, H J; Ippel, P F; Hageman, G; Sinke, R J; van der Laan, E N; Beemer, F A

    2001-01-01

    The autosomal dominant cerebellar ataxias (ADCAs) are a heterogeneous group of neurodegenerative disorders characterised by progressive cerebellar dysfunction in combination with a variety of other associative features. Since 1993 ADCAs have been increasingly characterised in terms of their genetic

  6. Current perspectives on pancreatic serous cystic neoplasms: Diagnosis, management and beyond

    Institute of Scientific and Technical Information of China (English)

    Xiao-Peng Zhang; Zhong-Xun Yu; Yu-Pei Zhao; Meng-Hua Dai

    2016-01-01

    Pancreatic cystic neoplasms have been increasingly recognized recently. Comprising about 16% of all resected pancreatic cystic neoplasms, serous cystic neoplasms are uncommon benign lesions that are usually asymptomatic and found incidentally. Despite overall low risk of malignancy, these pancreatic cysts still generate anxiety, leading to intensive medical investigations with considerable financial cost to health care systems. This review discusses the general background of serous cystic neoplasms, including epidemiology and clinical characteristics, and provides an updated overview of diagnostic approaches based on clinical features, relevant imaging studies and new findings that are being discovered pertaining to diagnostic evaluation. We also concisely discuss and propose management strategies for better quality of life.

  7. Tumour type and size are high risk factors for the syndrome of "cerebellar" mutism and subsequent dysarthria

    OpenAIRE

    1999-01-01

    textabstractOBJECTIVE: "Cerebellar mutis" and subsequent dysarthria (MSD) is a documented complication of posterior fossa surgery in children. In this prospective study the following risk factors for MSD were assessed: type, size and site of the tumour; hydrocephalus at presentation and after surgery, cerebellar incision site, postoperative infection, and cerebellar swelling. METHODS: In a consecutive series of 42 children with a cerebellar tumour, speech and neuroradiological studies (CT and...

  8. Properties of bilateral spinocerebellar activation of cerebellar cortical neurons

    Directory of Open Access Journals (Sweden)

    Pontus eGeborek

    2014-10-01

    Full Text Available We aimed to explore the cerebellar cortical inputs from two spinocerebellar pathways, the spinal border cell-component of the ventral spinocerebellar tract (SBC-VSCT and the dorsal spinocerebellar tract (DSCT, respectively, in the sublobule C1 of the cerebellar posterior lobe. The two pathways were activated by electrical stimulation of the contralateral lateral funiculus (coLF and the ipsilateral LF (iLF at lower thoracic levels. Most granule cells in sublobule C1 did not respond at all but part of the granule cell population displayed high-intensity responses to either coLF or iLF stimulation. As a rule, Golgi cells and Purkinje cell simple spikes responded to input from both LFs, although Golgi cells could be more selective. In addition, a small population of granule cells responded to input from both the coLF and the iLF. However, in these cases, similarities in the temporal topography and magnitude of the responses suggested that the same axons were stimulated from the two LFs, i.e. that the axons of individual spinocerebellar neurons could be present in both funiculi. This was also confirmed for a population of spinal neurons located within known locations of SBC-VSCT neurons and dorsal horn DSCT neurons. We conclude that bilateral spinocerebellar responses can occur in cerebellar granule cells, but the VSCT and DSCT systems that provide the input can also be organized bilaterally. The implications for the traditional functional separation of VSCT and DSCT systems and the issue whether granule cells primarily integrate functionally similar information or not are discussed.

  9. Probabilistic identification of cerebellar cortical neurones across species.

    Directory of Open Access Journals (Sweden)

    Gert Van Dijck

    Full Text Available Despite our fine-grain anatomical knowledge of the cerebellar cortex, electrophysiological studies of circuit information processing over the last fifty years have been hampered by the difficulty of reliably assigning signals to identified cell types. We approached this problem by assessing the spontaneous activity signatures of identified cerebellar cortical neurones. A range of statistics describing firing frequency and irregularity were then used, individually and in combination, to build Gaussian Process Classifiers (GPC leading to a probabilistic classification of each neurone type and the computation of equi-probable decision boundaries between cell classes. Firing frequency statistics were useful for separating Purkinje cells from granular layer units, whilst firing irregularity measures proved most useful for distinguishing cells within granular layer cell classes. Considered as single statistics, we achieved classification accuracies of 72.5% and 92.7% for granular layer and molecular layer units respectively. Combining statistics to form twin-variate GPC models substantially improved classification accuracies with the combination of mean spike frequency and log-interval entropy offering classification accuracies of 92.7% and 99.2% for our molecular and granular layer models, respectively. A cross-species comparison was performed, using data drawn from anaesthetised mice and decerebrate cats, where our models offered 80% and 100% classification accuracy. We then used our models to assess non-identified data from awake monkeys and rabbits in order to highlight subsets of neurones with the greatest degree of similarity to identified cell classes. In this way, our GPC-based approach for tentatively identifying neurones from their spontaneous activity signatures, in the absence of an established ground-truth, nonetheless affords the experimenter a statistically robust means of grouping cells with properties matching known cell classes. Our

  10. Aberrant cerebellar connectivity in motor and association networks in schizophrenia

    Directory of Open Access Journals (Sweden)

    Ann K. Shinn

    2015-03-01

    Full Text Available Schizophrenia is a devastating illness characterized by disturbances in multiple domains. The cerebellum is involved in both motor and non-motor functions, and the cognitive dysmetria and dysmetria of thought models propose that abnormalities of the cerebellum may contribute to schizophrenia signs and symptoms. The cerebellum and cerebral cortex are reciprocally connected via a modular, closed-loop network architecture, but few schizophrenia neuroimaging studies have taken into account the topographical and functional heterogeneity of the cerebellum. In this study, using a previously defined 17-network cerebral cortical parcellation system as the basis for our functional connectivity seeds, we systematically investigated connectivity abnormalities within the cerebellum of 44 schizophrenia patients and 28 healthy control participants. We found selective alterations in cerebro-cerebellar functional connectivity. Specifically, schizophrenia patients showed decreased cerebro-cerebellar functional connectivity in higher level association networks (ventral attention, salience, control, and default mode networks relative to healthy control participants. Schizophrenia patients also showed increased cerebro-cerebellar connectivity in somatomotor and default mode networks, with the latter showing no overlap with the regions found to be hypoconnected within the same default mode network. Finally, we found evidence to suggest that somatomotor and default mode networks may be inappropriately linked in schizophrenia. The relationship of these dysconnectivities to schizophrenia symptoms, such as neurological soft signs and altered sense of agency, is discussed. We conclude that the cerebellum ought to be considered for analysis in all future studies of network abnormalities in SZ, and further suggest the cerebellum as a potential target for further elucidation, and possibly treatment, of the underlying mechanisms and network abnormalities producing symptoms of

  11. A distinctive pattern of cortical excitability in patients with the syndrome of dystonia and cerebellar ataxia

    NARCIS (Netherlands)

    Talelli, P.; Hoffland, B.S.; Schneider, S.A.; Edwards, M.; Bhatia, K.P.; Warrenburg, B.P.C. van de; Rothwell, J.C.

    2011-01-01

    OBJECTIVE: The syndrome of dystonia and cerebellar ataxia (DYTCA) is a recently described condition where cervical dystonia and mild cerebellar ataxia are the major clinical features. Here we attempted to explore the pathophysiology of this condition by comparing measurements of cortical excitabilit

  12. Early onset cerebellar ataxia with retained tendon reflexes : foot deformity in a first grade family member

    NARCIS (Netherlands)

    Schelhaas, HJ; Van der Hulst, M; Ippel, E; Prevo, RL; Hageman, G

    1999-01-01

    Early onset cerebellar ataxia with retained tendon reflexes (EOCA) is a clinical syndrome characterised by progressive cerebellar ataxia with an onset before the age of 25 years and a wide spectrum of associated features. It is distinguished from Friedreich's ataxia (FA) mainly by the preservation o

  13. Parvovirus associated cerebellar hypoplasia and hydrocephalus in day-old broiler chickens

    Science.gov (United States)

    Cerebellar hypoplasia and hydrocephalus were detected in day-old broiler chickens. Brains of chickens evaluated at necropsy appeared to be abnormal; some were disfigured and cerebellae appeared to be smaller than normal. Histopathologic examination of brains revealed cerebellar folia that were sho...

  14. Autosomal recessive cerebellar ataxia caused by mutations in the PEX2 gene

    NARCIS (Netherlands)

    C. Sevin; S. Ferdinandusse; H.R. Waterham; R.J. Wanders; P. Aubourg

    2011-01-01

    ABSTRACT: OBJECTIVE: To expand the spectrum of genetic causes of autosomal recessive cerebellar ataxia (ARCA). Case report: Two brothers are described who developed progressive cerebellar ataxia at 3 1/2 and 18 years, respectively. After ruling out known common genetic causes of ARCA, analysis of bl

  15. Socioeconomic status and the cerebellar grey matter volume. Data from a well-characterised population sample.

    Science.gov (United States)

    Cavanagh, Jonathan; Krishnadas, Rajeev; Batty, G David; Burns, Harry; Deans, Kevin A; Ford, Ian; McConnachie, Alex; McGinty, Agnes; McLean, Jennifer S; Millar, Keith; Sattar, Naveed; Shiels, Paul G; Tannahill, Carol; Velupillai, Yoga N; Packard, Chris J; McLean, John

    2013-12-01

    The cerebellum is highly sensitive to adverse environmental factors throughout the life span. Socioeconomic deprivation has been associated with greater inflammatory and cardiometabolic risk, and poor neurocognitive function. Given the increasing awareness of the association between early-life adversities on cerebellar structure, we aimed to explore the relationship between early life (ESES) and current socioeconomic status (CSES) and cerebellar volume. T1-weighted MRI was used to create models of cerebellar grey matter volumes in 42 adult neurologically healthy males selected from the Psychological, Social and Biological Determinants of Ill Health study. The relationship between potential risk factors, including ESES, CSES and cerebellar grey matter volumes were examined using multiple regression techniques. We also examined if greater multisystem physiological risk index-derived from inflammatory and cardiometabolic risk markers-mediated the relationship between socioeconomic status (SES) and cerebellar grey matter volume. Both ESES and CSES explained the greatest variance in cerebellar grey matter volume, with age and alcohol use as a covariate in the model. Low CSES explained additional significant variance to low ESES on grey matter decrease. The multisystem physiological risk index mediated the relationship between both early life and current SES and grey matter volume in cerebellum. In a randomly selected sample of neurologically healthy males, poorer socioeconomic status was associated with a smaller cerebellar volume. Early and current socioeconomic status and the multisystem physiological risk index also apparently influence cerebellar volume. These findings provide data on the relationship between socioeconomic deprivation and a brain region highly sensitive to environmental factors.

  16. Incidence of Dysarthria in Children with Cerebellar Tumors: A Prospective Study

    Science.gov (United States)

    Richter, S.; Schoch, B.; Ozimek, A.; Gorissen, B.; Hein-Kropp, C.; Kaiser, O.; Hovel, M.; Wieland, R.; Gizewski, E.; Timmann, D.

    2005-01-01

    The present study investigated dysarthric symptoms in children with cerebellar tumors. Ten children with cerebellar tumors and 10 orthopedic control children were tested prior and one week after surgery. Clinical dysarthric symptoms were quantified in spontaneous speech. Syllable durations were analyzed in syllable repetition and sentence…

  17. Cerebellar motor learning deficits in medicated and medication-free men with recent-onset schizophrenia

    NARCIS (Netherlands)

    M.P.H. Coesmans (Michiel); C. Röder (Constantin); A.E. Smit (Albertine Eline); S.K.E. Koekkoek (Bas); C.I. de Zeeuw (Chris); M.A. Frens (Maarten); J.N. van der Geest (Jos)

    2014-01-01

    textabstractBackground: The notion that cerebellar deficits may underlie clinical symptoms in people with schizophrenia is tested by evaluating 2 forms of cerebellar learning in patients with recent-onset schizophrenia. A potential medication effect is evaluated by including patients with or without

  18. Cerebellar motor learning deficits in medicated and medication-free men with recent-onset schizophrenia

    NARCIS (Netherlands)

    Coesmans, Michael; Röder, Christian H; Smit, Albertine E; Koekkoek, Sebastiaan K E; De Zeeuw, Chris I; Frens, Maarten A; van der Geest, Josef N

    2014-01-01

    BACKGROUND: The notion that cerebellar deficits may underlie clinical symptoms in people with schizophrenia is tested by evaluating 2 forms of cerebellar learning in patients with recent-onset schizophrenia. A potential medication effect is evaluated by including patients with or without antipsychot

  19. Optogenetics in the cerebellum: Purkinje cell-specific approaches for understanding local cerebellar functions.

    Science.gov (United States)

    Tsubota, Tadashi; Ohashi, Yohei; Tamura, Keita

    2013-10-15

    The cerebellum consists of the cerebellar cortex and the cerebellar nuclei. Although the basic neuronal circuitry of the cerebellar cortex is uniform everywhere, anatomical data demonstrate that the input and output relationships of the cortex are spatially segregated between different cortical areas, which suggests that there are functional distinctions between these different areas. Perturbation of cerebellar cortical functions in a spatially restricted fashion is thus essential for investigating the distinctions among different cortical areas. In the cerebellar cortex, Purkinje cells are the sole output neurons that send information to downstream cerebellar and vestibular nuclei. Therefore, selective manipulation of Purkinje cell activities, without disturbing other neuronal types and passing fibers within the cortex, is a direct approach to spatially restrict the effects of perturbations. Although this type of approach has for many years been technically difficult, recent advances in optogenetics now enable selective activation or inhibition of Purkinje cell activities, with high temporal resolution. Here we discuss the effectiveness of using Purkinje cell-specific optogenetic approaches to elucidate the functions of local cerebellar cortex regions. We also discuss what improvements to current methods are necessary for future investigations of cerebellar functions to provide further advances.

  20. Cerebellar arteries originating from the internal carotid artery: angiographic evaluation and embryologic explanations

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jae Young; Han, Moon Hee; Yu, In Gyu; Chang, Ki Hyun [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of); Kim, Eui Jong [Kyunghee Univ. College of Medicine, Seoul (Korea, Republic of); Kim, Dae Ho [Soonchunhyang Univ. College of Medicine, Asan(Korea, Republic of)

    1997-06-01

    To find and describe the cerebellar arteries arising from the internal carotid artery, explain them embryologically, and evaluate their clinical implication. To determine the point in the internal carotid artery from which the cereballar artery arose anomalously, consecutive angiographic studies performed in the last three years were reviewed. The distribution of such anomalous cerebellar arteries, the point in the internal carotid artery from which the anomalous vessels originated, and associated findings were analyzed. Five anomalous origins of cerebellar arteries arising arising directly from the internal carotid artery were found in five patients. Three anterior inferior cerebellar arteries (AICA) and one common trunk of an AICA and a posterior inferior cerebellar artery (PICA) were found to originate from the internal carotid artery at a point close to the origin of the primitive trigeminal artery. A PICA arose from an artery presenting a course similar to the proatlantal intersegmental artery. Intracranial aneurysms in two patients, Moyamoya disease in one, and facial arteriovenous malformation in one. In our series, AICAs supplied from the arteries considered to be persistent trigeminal artery variants were the most common type. A correlation between type of anomalous cerebellar artery and type of carotid-vertebrobasilar anastomosis may exist. Cerebellar arteries originating anomalously from the internal carotid artery seem to occur as a result of the persistence of carotid-vertebrobasilar anastomoses associated with incomplete fusion of the longitudinal neural arteries. An understanding of these anomalous cerebellar arteries may help prevent accidents during therapeutic embolization and surgical treatment, as well as misinterpretation.

  1. Cerebellar clear cell ependymoma in a 10 year old girl

    Energy Technology Data Exchange (ETDEWEB)

    Thinzar Aye Nyein; Moon, Ah Rim; Hwang, Sun Chul; Hong, Hyun Sook; Lee, A Leum; Chang, Kee Hyun; Kim, Hee Kyung; Chin, Su Sie [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of); Park, Ji Sang [Soonchunhyang University Gumi Hospital, Gumi (Korea, Republic of)

    2016-01-15

    Clear cell ependymoma (CCE) is a histological rare variant (1–5%) of ependymoma, which is distinguished from other histological subtypes by the presence of fusiform cells arrayed radially around small blood vessels. These alleged perivascular pseudorosettes are significant characteristic features of ependymomas. About 95% of infratentorial ependymomas are found in the fourth ventricle and the remainder occurs as cerebellopontine angle lesions. In previous reports, the cerebellum is found to be a rare location for ependymoma. In this study we report one case of CCE originating from the cerebellar hemisphere, showing unusual morphology on 3T MRI.

  2. Infantile intracranial aneurysm of the superior cerebellar artery.

    Science.gov (United States)

    Del Santo, Molly Ann; Cordina, Steve Mario

    2016-02-29

    Intracranial aneurysms in the pediatric population are rare. We report a case of a 3-month-old infant who presented with inconsolable crying, vomiting, and sunset eye sign. CT revealed a subarachnoid hemorrhage, with CT angiogram revealing a superior cerebellar artery aneurysm. An external ventricular drain was placed for acute management of hydrocephalus, with definitive treatment by endovascular technique with a total of six microcoils to embolize the aneurysm. Serial transcranial Dopplers revealed no subsequent vasospasm. Although aneurysms in the pediatric population are rare, once the diagnosis is established, early treatment results in better outcomes.

  3. Endoscopic evacuation of cerebellar hematoma in a term newborn.

    Science.gov (United States)

    Tanriverdi, Sema Rala; Turhan, Tuncer; Uygur, Ozgun; Koroglu, Ozge Altun; Yalaz, Mehmet; Kultursay, Nilgun

    2013-10-01

    Intracerebellar hemorrhage is very rare in term infants and only severe cases with massive intracranial hemorrhage, posthemorrhagic hydrocephalus and clinical deterioration due to increased intracranial pressure require neurosurgical evacuation. In recent adult studies endoscopic hematoma evacuation has been shown as a rapid, effective, and safe technique. A term newborn hospitalized for meconium aspiration syndrome showed hypertonia, jitteriness and abnormal amplitude integrated electroencephalogram findings. He was diagnosed with cerebellar hematoma which caused hydrocephalus by cranial magnetic resonance imaging (MRI). The hematoma was successfully evacuated neuroendoscopically as the first case in literature to our knowledge. Neurologic, a-EEG and MRI findings resolved.

  4. Dysarthria in children with cerebellar or brainstem tumors.

    Science.gov (United States)

    van Mourik, M; Catsman-Berrevoets, C E; Yousef-Bak, E; Paquier, P F; van Dongen, H R

    1998-05-01

    Speech features were perceptually analyzed in two groups of children. The first group (n = 6) had undergone cerebellar tumor resection, and the second group (n = 6) included children with brainstem tumors. Children belonging to the first group became dysarthric after a postoperative mute phase. Slow speech rate was a specific feature, but scanning speech and irregular articulatory breakdown (i.e., prominent characteristics in adult ataxic dysarthria) were not observed. In the second group, hypernasality was a prominent characteristic and resembled flaccid dysarthria in adults. These findings suggest that acquired childhood dysarthria needs a proper classification.

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    Lifescience Database Archive (English)

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    Lifescience Database Archive (English)

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  9. Changes in a cerebellar peduncle lesion in a patient with Dandy-Walker malformation A diffusion tensor imaging study

    Institute of Scientific and Technical Information of China (English)

    Ah Young Lee; Sung Ho Jang; Sang Seok Yeo; Ensil Lee; Yun Woo Cho; Su Min Son

    2013-01-01

    We report a patient with severe ataxia due to Dandy-Walker malformation, who showed functional recovery over 10 months corresponding to a change in a cerebellar peduncle lesion. A 20-month-old female patient who was diagnosed with Dandy-Walker syndrome and six age- and sex-matched healthy control subjects were enrolled. The superior cerebellar peduncle, the middle cerebellar peduncle, and the inferior cerebellar peduncle were evaluated using fractional anisotropy and the apparent diffusion coefficient. The patients' functional ambulation category was 0 at the initial visit, but improved to 2 at the follow-up evaluation, and Berg's balance scale score also improved from 0 to 7. Initial diffusion tensor tractography revealed that the inferior cerebellar peduncle was not detected, that the fractional anisotropy of the superior cerebellar peduncle and middle cerebellar peduncle decreased by two standard deviations below, and that the apparent diffusion coefficient increased by two standard deviations over normal control values. However, on follow-up diffusion tensor tractography, both inferior cerebellar peduncles could be detected, and the fractional anisotropy of superior cerebellar peduncle increased to within two standard deviations of normal controls. The functional improvement in this patient appeared to correspond to changes in these cerebellar peduncles. We believe that evaluating cerebellar peduncles using diffusion tensor imaging is useful in cases when a cerebellar peduncle lesion is suspected.

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    Lifescience Database Archive (English)

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    Lifescience Database Archive (English)

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  13. [Contemporary management of neuroendocrine neoplasms of the female genital organs].

    Science.gov (United States)

    Kuc-Rajca, Małgorzata; Dańska-Bidzińska, Anna

    2011-09-01

    Neuroendocrine neoplasms are a rare and heterogeneous group of diseases that account for only 2% of all gynecologic malignancies. The most common types are ovarian carcinoid tumor and small cell neuroendocrine carcinoma of the cervix. The tumors are staged according to FIGO clinical staging system. The diagnosis is usually made retrospectively after obtaining the results of histopathological evaluation of the primary tumor They rarely cause syndromes related to hormone overexpression. Neuroendocrine neoplasms are characterized by aggressive behaviour Even at an early stage there is high incidence of nodal and distant metastases. Survival is poor regardless of stage at diagnosis. The most important is to diagnose the neuroendocrine tumor accurately and treat it in multimodal, aggressive approach to control the disease better and reduce the incidence of reccurences. Apart from typical therapeutic approach, treatment may encompass isotope therapy using radiolabeled somatostatin analogs. This method should be reserved for patients with expression of somatostatin receptors detected by the somatostatin receptor scyntygraphy. Data concerning the management of neuroendocrin tumors are based mainly on retrospective studies and clinical case series. Lack of randomized trials makes it impossible to select the best treatment option. Better understanding of the biology of neuroendocrine tumors, especially the molecular genetics, will in the future help to determine the optimal treatment strategies for these tumors.

  14. Frequency of heterozygous TET2 deletions in myeloproliferative neoplasms

    Directory of Open Access Journals (Sweden)

    Joseph Tripodi

    2010-09-01

    Full Text Available Joseph Tripodi1, Ronald Hoffman1, Vesna Najfeld2, Rona Weinberg31The Myeloproliferative Disorders Program, Tisch Cancer Institute, Department of Medicine and 2Department of Medicine and Pathology, Mount Sinai School of Medicine, 3The Myeloproliferative Disorders Program, Cellular Therapy Laboratory, The New York Blood Center, New York, NY, USAAbstract: The Philadelphia chromosome (Ph-negative myeloproliferative neoplasms (MPNs, including polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are a group of clonal hematopoietic stem cell disorders with overlapping clinical and cytogenetic features and a variable tendency to evolve into acute leukemia. These diseases not only share overlapping chromosomal abnormalities but also a number of acquired somatic mutations. Recently, mutations in a putative tumor suppressor gene, ten-eleven translocation 2 (TET2 on chromosome 4q24 have been identified in 12% of patients with MPN. Additionally 4q24 chromosomal rearrangements in MPN, including TET2 deletions, have also been observed using conventional cytogenetics. The goal of this study was to investigate the frequency of genomic TET2 rearrangements in MPN using fluorescence in situ hybridization as a more sensitive method for screening and identifying genomic deletions. Among 146 MPN patients, we identified two patients (1.4% who showed a common 4q24 deletion, including TET2. Our observations also indicated that the frequency of TET2 deletion is increased in patients with an abnormal karyotype (5%.Keywords: TET2, myeloproliferative neoplasms, fluorescence in situ hybridization, cytogenetics

  15. Endoscopic approach for a laryngeal neoplasm in a dog

    Directory of Open Access Journals (Sweden)

    Pedro Paulo Maia Teixeira

    2015-01-01

    Full Text Available Laryngeal and tracheal tumors are rare in pets; some piece of information on their disease behavior, therapy and evolution are limited. Neoplasms in this area are a diagnostic challenge. In many cases, they can be biopsied and excised using endoscopic instruments, but there is no report of this in canines. The goal of this study is to report a successful case of a laryngeal neoplasm removal through endoscopy. A head and neck radiogram revealed a mass in the laryngeal lumen protruding into the trachea. The patient then underwent an endoscopy to confirm the radiographic diagnosis and to surgically remove the tumor. The histopathological diagnosis was poorly differentiated carcinoma. The most appropriate treatment for laryngeal tumors is the resection of the submucosa or a partial laryngectomy however, partial and total laryngectomies are associated with many postoperative complications. In contrast, the endoscopic approach allows for highly magnified visualization of the lesion in situ, which facilitates the surgical removal of the mass through videosurgery. With little manipulation of the affected area, the chances of postoperative complications are reduced, leading to a more rapid recovery.

  16. Emerging tumor entities and variants of CNS neoplasms.

    Science.gov (United States)

    Cenacchi, Giovanna; Giangaspero, Felice

    2004-03-01

    Since the appearance in 2000 of the World Health Organization (WHO) classification for central nervous system (CNS) neoplasms, numerous descriptions of new entities or variants have appeared in the literature. In the group of neuronal and mixed glioneuronal neoplasms are lesions with distinctive morphological features that are still not included in a precise classification, including extraventricular neurocytoma, papillary glioneuronal tumor, rosette-forming glioneuronal of the fourth ventricle, glioneuronal with neuropil-like rosette, and DNT-like tumor of the septum pellucidum. The glioneuronal tumor with neuropil-like rosette and oligodendroglioma with neurocytic differentiation represent morphological variants of genetically proven diffuse gliomas. The lipoastrocytoma and the pilomixoid astrocytoma enlarge the group of astrocytic lesions. Rare, low-grade gliomas of the spinal cord with extensive leptomeningeal dissemination associated with unusual neuroimaging are described. The chordoid glioma of the third ventricle and the papillary tumor of the pineal region seem to be correlated by a common histogenesis from the specialized ependyma of the subcommissural organ. An embryonal tumor with neuropil and true rosettes combining features of neuroblastoma and ependymoblastoma is discussed. These new, recently described lesions indicate that the complex morphologic spectrum of CNS tumors is far from being completely delineated.

  17. Babinski's contributions to cerebellar symptomatology: building the basis of the neurological examination

    Directory of Open Access Journals (Sweden)

    Jose Luiz Pedroso

    2013-12-01

    Full Text Available Several assumptions about the function of the cerebellum and semiotic signs have been described over the centuries. Among the long list of famous researchers who have provided a strong contribution and who have left their names on the highway of cerebellar research, Joseph Babinski appears as a prominent name. The description of various forms of cerebellar symptomatology was a major part of Babinski's work, and clinical terms that he introduced, namely hypermetry , diadochokinesia , and asynergy , remain part of contemporary clinical vocabulary. Babinski studied cerebellar signs in many patients and was able to conduct longitudinal studies that permitted him to understand the evolution of cerebellar dysfunction. Babinski contributions to cerebellar symptomatology continue to influence the most modern theories, including functional and neuropathological studies.

  18. Location of lesion determines motor vs. cognitive consequences in patients with cerebellar stroke

    Directory of Open Access Journals (Sweden)

    Catherine J. Stoodley

    2016-01-01

    Full Text Available Cerebellar lesions can cause motor deficits and/or the cerebellar cognitive affective syndrome (CCAS; Schmahmann's syndrome. We used voxel-based lesion-symptom mapping to test the hypothesis that the cerebellar motor syndrome results from anterior lobe damage whereas lesions in the posterolateral cerebellum produce the CCAS. Eighteen patients with isolated cerebellar stroke (13 males, 5 females; 20–66 years old were evaluated using measures of ataxia and neurocognitive ability. Patients showed a wide range of motor and cognitive performance, from normal to severely impaired; individual deficits varied according to lesion location within the cerebellum. Patients with damage to cerebellar lobules III–VI had worse ataxia scores: as predicted, the cerebellar motor syndrome resulted from lesions involving the anterior cerebellum. Poorer performance on fine motor tasks was associated primarily with strokes affecting the anterior lobe extending into lobule VI, with right-handed finger tapping and peg-placement associated with damage to the right cerebellum, and left-handed finger tapping associated with left cerebellar damage. Patients with the CCAS in the absence of cerebellar motor syndrome had damage to posterior lobe regions, with lesions leading to significantly poorer scores on language (e.g. right Crus I and II extending through IX, spatial (bilateral Crus I, Crus II, and right lobule VIII, and executive function measures (lobules VII–VIII. These data reveal clinically significant functional regions underpinning movement and cognition in the cerebellum, with a broad anterior-posterior distinction. Motor and cognitive outcomes following cerebellar damage appear to reflect the disruption of different cerebro-cerebellar motor and cognitive loops.

  19. Cerebellar gray matter and lobular volumes correlate with core autism symptoms

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    Anila M. D'Mello

    2015-01-01

    Full Text Available Neuroanatomical differences in the cerebellum are among the most consistent findings in autism spectrum disorder (ASD, but little is known about the relationship between cerebellar dysfunction and core ASD symptoms. The newly-emerging existence of cerebellar sensorimotor and cognitive subregions provides a new framework for interpreting the functional significance of cerebellar findings in ASD. Here we use two complementary analyses — whole-brain voxel-based morphometry (VBM and the SUIT cerebellar atlas — to investigate cerebellar regional gray matter (GM and volumetric lobular measurements in 35 children with ASD and 35 typically-developing (TD children (mean age 10.4 ± 1.6 years; range 8–13 years. To examine the relationships between cerebellar structure and core ASD symptoms, correlations were calculated between scores on the Autism Diagnostic Observation Schedule (ADOS and Autism Diagnostic Interview (ADI and the VBM and volumetric data. Both VBM and the SUIT analyses revealed reduced GM in ASD children in cerebellar lobule VII (Crus I/II. The degree of regional and lobular gray matter reductions in different cerebellar subregions correlated with the severity of symptoms in social interaction, communication, and repetitive behaviors. Structural differences and behavioral correlations converged on right cerebellar Crus I/II, a region which shows structural and functional connectivity with fronto-parietal and default mode networks. These results emphasize the importance of the location within the cerebellum to the potential functional impact of structural differences in ASD, and suggest that GM differences in cerebellar right Crus I/II are associated with the core ASD profile.

  20. Pathologic evaluation and reporting of intraductal papillary mucinous neoplasms of the pancreas and other tumoral intraepithelial neoplasms of pancreatobiliary tract: Recommendations of verona consensus meeting

    NARCIS (Netherlands)

    V. Adsay (Volkan); M. Mino-Kenudson (Mari); T. Furukawa (Toru); O. Basturk (Olca); G. Zamboni (Giuseppe); G. Marchegiani (Giovanni); C. Bassi (Claudio); R. Salvia (Roberto); G. Malleo (Giuseppe); S. Paiella (Salvatore); C.L. Wolfgang (Christopher L.); H. Matthaei (Hanno); G.J.A. Offerhaus; I.M. Adham; M.J. Bruno (Marco); M.D. Reid (Michelle D.); A. Krasinskas (Alyssa); G. Kloppel (Günter); N. Ohike (Nobuyuki); T. Tajiri (Takuma); K.-T. Jang (Kee-Taek); J.C. Roa (Juan Carlos); P.J. Allen (Peter J.); C. Fernández-Del Castillo (Carlos); J.-Y. Jang (Jin-Young); D.S. Klimstra (David); R.H. Hruban (Ralph)

    2016-01-01

    textabstractBackground: There are no established guidelines for pathologic diagnosis/reporting of intraductal papillary mucinous neoplasms (IPMNs). Design: An international multidisciplinary group, brought together by the Verona Pancreas Group in Italy-2013, was tasked to devise recommendations. Res

  1. Pathologic evaluation and reporting of intraductal papillary mucinous neoplasms of the pancreas and other tumoral intraepithelial neoplasms of pancreatobiliary tract : Recommendations of verona consensus meeting

    NARCIS (Netherlands)

    Adsay, Volkan; Mino-Kenudson, Mari; Furukawa, Toru; Basturk, Olca; Zamboni, Giuseppe; Marchegiani, Giovanni; Bassi, Claudio; Salvia, Roberto; Malleo, Giuseppe; Paiella, Salvatore; Wolfgang, Christopher L.; Matthaei, Hanno; Offerhaus, G. Johan; Adham, Mustapha; Bruno, Marco J.; Reid, Michelle D.; Krasinskas, Alyssa; Klöppel, Günter; Ohike, Nobuyuki; Tajiri, Takuma; Jang, Kee Taek; Roa, Juan Carlos; Allen, Peter; Fernández-Del Castillo, Carlos; Jang, Jin Young; Klimstra, David S.; Hruban, Ralph H.

    2016-01-01

    Background: There are no established guidelines for pathologic diagnosis/reporting of intraductal papillary mucinous neoplasms (IPMNs). Design: An international multidisciplinary group, brought together by the Verona Pancreas Group in Italy-2013, was tasked to devise recommendations. Results: (1) Cr

  2. Spinal level of myelomeningocele lesion as a contributing factor in posterior fossa volume, intracranial cerebellar volume, and cerebellar ectopia.

    LENUS (Irish Health Repository)

    Sweeney, Kieron J

    2013-02-01

    McLone and Knepper\\'s unified theory of Chiari malformation Type II (CM-II) describes how the loss of CSF via the open posterior neuropore fails to create adequate distending pressure for the developing rhomboencephalic vesicle. The authors of the present article describe the relationship between the posterior fossa volume and intracranial cerebellar volume as being related to the distance from the obex of the fourth ventricle to the myelomeningocele lesion using a common mathematical model, the Hagen-Poiseuille law.

  3. Diffusion spectrum imaging shows the structural basis of functional cerebellar circuits in the human cerebellum in vivo.

    Directory of Open Access Journals (Sweden)

    Cristina Granziera

    Full Text Available BACKGROUND: The cerebellum is a complex structure that can be affected by several congenital and acquired diseases leading to alteration of its function and neuronal circuits. Identifying the structural bases of cerebellar neuronal networks in humans in vivo may provide biomarkers for diagnosis and management of cerebellar diseases. OBJECTIVES: To define the anatomy of intrinsic and extrinsic cerebellar circuits using high-angular resolution diffusion spectrum imaging (DSI. METHODS: We acquired high-resolution structural MRI and DSI of the cerebellum in four healthy female subjects at 3T. DSI tractography based on a streamline algorithm was performed to identify the circuits connecting the cerebellar cortex with the deep cerebellar nuclei, selected brainstem nuclei, and the thalamus. RESULTS: Using in-vivo DSI in humans we were able to demonstrate the structure of the following cerebellar neuronal circuits: (1 connections of the inferior olivary nucleus with the cerebellar cortex, and with the deep cerebellar nuclei (2 connections between the cerebellar cortex and the deep cerebellar nuclei, (3 connections of the deep cerebellar nuclei conveyed in the superior (SCP, middle (MCP and inferior (ICP cerebellar peduncles, (4 complex intersections of fibers in the SCP, MCP and ICP, and (5 connections between the deep cerebellar nuclei and the red nucleus and the thalamus. CONCLUSION: For the first time, we show that DSI tractography in humans in vivo is capable of revealing the structural bases of complex cerebellar networks. DSI thus appears to be a promising imaging method for characterizing anatomical disruptions that occur in cerebellar diseases, and for monitoring response to therapeutic interventions.

  4. Progressive cerebellar atrophy: hereditary ataxias and disorders with spinocerebellar degeneration.

    Science.gov (United States)

    Wolf, Nicole I; Koenig, Michel

    2013-01-01

    The hereditary ataxias with onset in childhood are a group of heterogeneous disorders, usually with autosomal recessive inheritance. In many of them, magnetic resonance imaging (MRI) shows cerebellar atrophy. The most prominent exception to this is Friedreich's ataxia, where MRI shows normal cerebellar volume, but sometimes spinal cord atrophy. In several of the hereditary ataxias, the causative gene plays an important role in DNA repair: ataxia telangiectasia and ataxia telangiectasia-like disorder, and ataxia with oculomotor apraxia type I and II. Mitochondrial metabolism is impaired in another group of inherited ataxias including the emergent group of defects in coenzyme Q10 synthesis. Few of these disorders are amenable to effective treatment, the most important of these being vitamin E-responsive ataxia. The autosomal dominant spinocerebellar ataxias are rare in childhood. Some of them, especially SCA7 and SCA2, may begin in childhood or even infancy, family history being positive in these cases. Additional clinical clues such as presence or absence of neuropathy or oculomotor apraxia still help in making a definitive diagnosis albeit there are still many unsolved cases. In pontocerebellar hypoplasia, a neurodegenerative disease with prenatal onset, the genetic basis of the different subtypes has recently been elucidated and involves genes with different functions.

  5. Remodeling of monoplanar Purkinje cell dendrites during cerebellar circuit formation.

    Directory of Open Access Journals (Sweden)

    Megumi Kaneko

    Full Text Available Dendrite arborization patterns are critical determinants of neuronal connectivity and integration. Planar and highly branched dendrites of the cerebellar Purkinje cell receive specific topographical projections from two major afferent pathways; a single climbing fiber axon from the inferior olive that extend along Purkinje dendrites, and parallel fiber axons of granule cells that contact vertically to the plane of dendrites. It has been believed that murine Purkinje cell dendrites extend in a single parasagittal plane in the molecular layer after the cell polarity is determined during the early postnatal development. By three-dimensional confocal analysis of growing Purkinje cells, we observed that mouse Purkinje cells underwent dynamic dendritic remodeling during circuit maturation in the third postnatal week. After dendrites were polarized and flattened in the early second postnatal week, dendritic arbors gradually expanded in multiple sagittal planes in the molecular layer by intensive growth and branching by the third postnatal week. Dendrites then became confined to a single plane in the fourth postnatal week. Multiplanar Purkinje cells in the third week were often associated by ectopic climbing fibers innervating nearby Purkinje cells in distinct sagittal planes. The mature monoplanar arborization was disrupted in mutant mice with abnormal Purkinje cell connectivity and motor discoordination. The dendrite remodeling was also impaired by pharmacological disruption of normal afferent activity during the second or third postnatal week. Our results suggest that the monoplanar arborization of Purkinje cells is coupled with functional development of the cerebellar circuitry.

  6. Congenital disorders of glycosylation with emphasis on cerebellar involvement.

    Science.gov (United States)

    Barone, Rita; Fiumara, Agata; Jaeken, Jaak

    2014-07-01

    Congenital disorders of glycosylation (CDG) are genetic diseases due to defective glycosylation of proteins and lipids. The authors present an update on these disorders affecting the central nervous system with a focus on cerebellar involvement. The rate of identification of novel CDG shows an exponential increase. Some 76 CDG are actually known, not taking into account the defects in glycan-modifying proteins. Neurologic involvement is present in the large majority of CDG. Screening methods are limited to serum transferrin isoelectrofocusing (for N-glycosylation disorders with sialic acid deficiency), and serum apolipoprotein C-III isoelectrofocusing (for core 1 mucin-type O-glycosylation disorders). Whole exome/genome sequencing is increasingly used in the diagnostic workup of patients with CDG-X. Treatment is greatly lagging behind because only one CDG is efficiently treatable (MPI-CDG). Cerebellar involvement is an important feature of PMM2-CDG, the congenital muscular dystrophies due to dystroglycanopathy, and SRD5A3-CDG. It has also been reported in some patients with ALG1-CDG, ALG3-CDG, ALG9-CDG, ALG6-CDG, ALG8-CDG, PIGA-CDG, DPM1-CDG, DPM2-CDG, B4GALT1-CDG, SLC35A2-CDG, COG1-CDG, COG5-CDG, COG7-CDG, and COG8-CDG.

  7. Pilomyxoid astrocytoma of the cerebellar vermis in an elderly patient

    Directory of Open Access Journals (Sweden)

    Branko Skovrlj

    2014-01-01

    Full Text Available Background: Pilomyxoid astrocytoma (PMA has recently been accepted as an aggressive variant of pilocytic astrocytoma with distinct histopathological features. PMAs have been frequently described in the pediatric population with a predilection for the hypothalamic/chiasmatic region. Case Description: A 72-year-old African American male presented with 6 months of memory loss, difficulty expressing himself, and a progressively worsening gait. Magnetic resonance imaging of the brain demonstrated a heterogeneously enhancing cystic mass centered within the cerebellar vermis with mass effect on the fourth ventricle and ventriculomegaly. The patient underwent placement of a ventriculoperitoneal shunt followed by a surgical resection of the lesion, which after immunohistopathologic evaluation, was diagnosed as a World Health Organization grade II PMA. The patient refused further treatment of the lesion and expired 11 months after initial symptom presentation and 4 months after surgery. Conclusion: To our knowledge, this is the first report of PMA of the cerebellar vermis in a previously unreported age group. This case report describes the natural history of this type of tumor in a patient who refused adjuvant therapy following surgical resection.

  8. Mechanisms of ethanol-induced death of cerebellar granule cells.

    Science.gov (United States)

    Luo, Jia

    2012-03-01

    Maternal ethanol exposure during pregnancy may cause fetal alcohol spectrum disorders (FASD). FASD is the leading cause of mental retardation. The most deleterious effect of fetal alcohol exposure is inducing neuroapoptosis in the developing brain. Ethanol-induced loss of neurons in the central nervous system underlies many of the behavioral deficits observed in FASD. The cerebellum is one of the brain areas that are most susceptible to ethanol during development. Ethanol exposure causes a loss of both cerebellar Purkinje cells and granule cells. This review focuses on the toxic effect of ethanol on cerebellar granule cells (CGC) and the underlying mechanisms. Both in vitro and in vivo studies indicate that ethanol induces apoptotic death of CGC. The vulnerability of CGC to ethanol-induced death diminishes over time as neurons mature. Several mechanisms for ethanol-induced apoptosis of CGC have been suggested. These include inhibition of N-methyl-D-aspartate receptors, interference with signaling by neurotrophic factors, induction of oxidative stress, modulation of retinoid acid signaling, disturbance of potassium channel currents, thiamine deficiency, and disruption of translational regulation. Cultures of CGC provide an excellent system to investigate cellular/molecular mechanisms of ethanol-induced neurodegeneration and to evaluate interventional strategies. This review will also discuss the approaches leading to neuroprotection against ethanol-induced neuroapoptosis.

  9. Primary cerebellar extramedullary myeloid cell tumor mimicking oligodendroglioma.

    Science.gov (United States)

    Ho, D M; Wong, T T; Guo, W Y; Chang, K P; Yen, S H

    1997-10-01

    Extramedullary myeloid cell tumors (EMCTs) are tumors consisting of immature cells of the myeloid series that occur outside the bone marrow. Most of them are associated with acute myelogenous leukemia or other myeloproliferative disorders, and a small number occur as primary lesions, i.e., are not associated with hematological disorders. Occurrence inside the cranium is rare, and there has been only one case of primary EMCT involving the cerebellum reported in the literature. The case we report here is a blastic EMCT occurring in the cerebellum of a 3-year-old boy who had no signs of leukemia or any hematological disorder throughout the entire course. The cerebellar tumor was at first misdiagnosed as an "oligodendroglioma" because of the uniformity and "fried egg" artifact of the tumor cells. The tumor disappeared during chemotherapy consisting of 12 treatments. However, it recurred and metastasized to the cerebrospinal fluid (CSF) shortly after the therapy was completed. A diagnosis of EMCT was suspected because of the presence of immature myeloid cells in the CSF, and was confirmed by anti-myeloperoxidase and anti-lysozyme immunoreactivity of the cerebellar tumor. The patient succumbed 1 year and 3 months after the first presentation of the disease.

  10. Coordinated scaling of cortical and cerebellar numbers of neurons

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    Suzana Herculano-Houzel

    2010-03-01

    Full Text Available While larger brains possess concertedly larger cerebral cortices and cerebella, the relative size of the cerebral cortex increases with brain size, but relative cerebellar size does not. In the absence of data on numbers of neurons in these structures, this discrepancy has been used to dispute the hypothesis that the cerebral cortex and cerebellum function and have evolved in concert and to support a trend towards neocorticalization in evolution. However, the rationale for interpreting changes in absolute and relative size of the cerebral cortex and cerebellum relies on the assumption that they reflect absolute and relative numbers of neurons in these structures across all species – an assumption that our recent studies have shown to be flawed. Here I show for the first time that the numbers of neurons in the cerebral cortex and cerebellum are directly correlated across 19 mammalian species of 4 different orders, including humans, and increase concertedly in a similar fashion both within and across the orders Eulipotyphla (Insectivora, Rodentia, Scandentia and Primata, such that on average a ratio of 3.6 neurons in the cerebellum to every neuron in the cerebral cortex is maintained across species. This coordinated scaling of cortical and cerebellar numbers of neurons provides direct evidence in favor of concerted function, scaling and evolution of these brain structures, and suggests that the common notion that equates cognitive advancement with neocortical expansion should be revisited to consider in its stead the coordinated scaling of neocortex and cerebellum as a functional ensemble.

  11. 78 FR 740 - Prospective Grant of Exclusive License: The Development of Gene Expression Signatures of Neoplasm...

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    2013-01-04

    ... Gene Expression Signatures of Neoplasm Responsiveness to mTOR and HDAC Inhibitor Combination Therapy... entitled, ``Gene Expression Signatures of Neoplasm Responsiveness to Therapy'' , and all continuing... the field of use may be limited to: the use of the licensed patent rights limited to an FDA-cleared...

  12. Helicobacter pylori-related chronic gastritis as a risk factor for colonic neoplasms.

    Science.gov (United States)

    Inoue, Izumi; Kato, Jun; Tamai, Hideyuki; Iguchi, Mikitaka; Maekita, Takao; Yoshimura, Noriko; Ichinose, Masao

    2014-02-14

    To summarize the current views and insights on associations between Helicobacter pylori (H. pylori)-related chronic gastritis and colorectal neoplasm, we reviewed recent studies to clarify whether H. pylori infection/H. pylori-related chronic gastritis is associated with an elevated risk of colorectal neoplasm. Recent studies based on large databases with careful control for confounding variables have clearly demonstrated an increased risk of colorectal neoplasm associated with H. pylori infection. The correlation between H. pylori-related chronic atrophic gastritis (CAG) and colorectal neoplasm has only been examined in a limited number of studies. A recent large study using a national histopathological database, and our study based on the stage of H. pylori-related chronic gastritis as determined by serum levels of H. pylori antibody titer and pepsinogen, indicated that H. pylori-related CAG confers an increased risk of colorectal neoplasm, and more extensive atrophic gastritis will probably be associated with even higher risk of neoplasm. In addition, our study suggested that the activity of H. pylori-related chronic gastritis is correlated with colorectal neoplasm risk. H. pylori-related chronic gastritis could be involved in an increased risk of colorectal neoplasm that appears to be enhanced by the progression of gastric atrophy and the presence of active inflammation.

  13. Photodynamic therapy of early stage oral cavity and oropharynx neoplasms: an outcome analysis of 170 patients

    NARCIS (Netherlands)

    B. karakullukcu (Baris); K. Oudenaarde (Kim); M.P. Copper (Marcel); W.M.C. Klop; R. van Veen (Robert); M. Wildeman (Maarten); I. Bing Tan

    2010-01-01

    textabstractThe indications of photodynamic therapy (PDT) of oral cavity and oropharynx neoplasms are not well defined. The main reason is that the success rates are not well established. The current paper analyzes our institutional experience of early stage oral cavity and oropharynx neoplasms (Tis

  14. [Heterogenous abnormality polymorphism of gene PDGFRB in myeloid neoplasms and its clinical characteristics].

    Science.gov (United States)

    Wang, Quan-Shun; Gao, Li; Jing, Yu; Zhu, Hai-Yan; Yang, Hua; Yu, Li

    2012-04-01

    Myeloid neoplasms with eosinophilia and abnormalities of PDGFRB gene are a new kind of myeloid disorders in the revised 2008 WHO classification. Out of detected 2000 cases of myeloid cell abnormalities in our hospital, 12 cases of myeloid neoplasms with eosinophilia and abnormalities of PDGFRB were found. This study was purposed to summarize and analyze the clinical and laboratorial characteristics of the 12 cases with PDGFRB gene abnormalities. The results indicated that among 12 cases of myeloid neoplasms with PDGFRB abnormalities, 5 cases with TEL/PDGFRB fusion gene, 2 cases with HEPI/PDGFRB, 1 case with PDGFRB mutation, 1 case with RABAPTIN-5/PDGFRB, 1 case with GIT2/PDGFRB, 1 case with TP53/PDGFRB, 1 case with WDR43/PDGFRB fusion gene were detected, showing the polymorphism of PDGFRB gene abnormalities. Among this kind of myeloid neoplasm patients, almost all patients manifested monocytosis and eosinophilia in different degree, the thrombocytosis mainly was observed in atypical myeloid neoplasms, acute leukemia, chromic myelo-monocytic leukemia patients. The treatment with imatinib mesylate for this kind of patients was effective in some cases. It is concluded that the myeloid neoplasms with PDGFRB gene abnormalities are a kind of heterogenetic myeloid neoplasms, their gene abnormal types and clinical manifestations show polymorphism too. The monocytosis and eosinophilia appear in this kind myeloid neoplasms which may be treated with tyrosine kinase inhibitors such as imatinib mesylate.

  15. Targeting angiogenesis-dependent calcified neoplasms using combined polymer therapeutics.

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    Ehud Segal

    Full Text Available BACKGROUND: There is an immense clinical need for novel therapeutics for the treatment of angiogenesis-dependent calcified neoplasms such as osteosarcomas and bone metastases. We developed a new therapeutic strategy to target bone metastases and calcified neoplasms using combined polymer-bound angiogenesis inhibitors. Using an advanced "living polymerization" technique, the reversible addition-fragmentation chain transfer (RAFT, we conjugated the aminobisphosphonate alendronate (ALN, and the potent anti-angiogenic agent TNP-470 with N-(2-hydroxypropylmethacrylamide (HPMA copolymer through a Glycine-Glycine-Proline-Norleucine linker, cleaved by cathepsin K, a cysteine protease overexpressed at resorption sites in bone tissues. In this approach, dual targeting is achieved. Passive accumulation is possible due to the increase in molecular weight following polymer conjugation of the drugs, thus extravasating from the tumor leaky vessels and not from normal healthy vessels. Active targeting to the calcified tissues is achieved by ALN's affinity to bone mineral. METHODS AND FINDING: The anti-angiogenic and antitumor potency of HPMA copolymer-ALN-TNP-470 conjugate was evaluated both in vitro and in vivo. We show that free and conjugated ALN-TNP-470 have synergistic anti-angiogenic and antitumor activity by inhibiting proliferation, migration and capillary-like tube formation of endothelial and human osteosarcoma cells in vitro. Evaluation of anti-angiogenic, antitumor activity and body distribution of HPMA copolymer-ALN-TNP-470 conjugate was performed on severe combined immunodeficiency (SCID male mice inoculated with mCherry-labeled MG-63-Ras human osteosarcoma and by modified Miles permeability assay. Our targeted bi-specific conjugate reduced VEGF-induced vascular hyperpermeability by 92% and remarkably inhibited osteosarcoma growth in mice by 96%. CONCLUSIONS: This is the first report to describe a new concept of a narrowly-dispersed combined

  16. Preliminary study on MR colonography with air enema in detection of colorectal neoplasms

    Institute of Scientific and Technical Information of China (English)

    LUO Ming-yue; LIU Li; YAN Fu-hua; SHEN Ji-zhang; YAO Li-qing; ZHOU Kang-rong

    2010-01-01

    Background The few studies on MR colonography with air enema involved feasibility of bowel distention and imaging quality and lacked detection sensitivity of colorectal neoplasms. The purpose of this prospective study was to assess the detection sensitivity of colorectal neoplasms with the three-dimensional Fourier transform fast spoiled gradient-recalled MR colonography with air enema.Methods A total of 30 patients scheduled for optical colonoscopy due to rectal bleeding, positive fecal occult blood test results or altered bowel habits were recruited and successfully underwent entire colorectal examinations with three-dimensional Fourier transform fast spoiled gradient-recalled MR colonography and subsequent optical colonoscopy on the same day. Detection sensitivity of colorectal neoplasms with MR colonography was statistically analyzed on a per-neoplasm size basis by using findings from optical colonoscopy and histopathological examinations as the reference standards.Results Seventy-six neoplasms were detected with optical colonoscopy, consisting of 1 mm-5 mm (n=11), 6 mm-9 mm (n=29) and >10 mm (n=36) in diameter. Detection sensitivities of 1 mm-5 mm, 6 mm-9 mm, ≥10 mm and >6 mm colorectal neoplasms with MR colonography were 9.1%, 75.9%, 100% and 89.2%, respectively; overall detection sensitivity for all sizes colorectal neoplasms was 77.6%.Conclusions Detection sensitivity of three-dimensional Fourier transform fast spoiled gradient-recalled MR colonography with air enema is low for 1 mm-5 mm colorectal neoplasms, but the detection sensitivity is 89.2% for ≥6 mm neoplasms, and all ≥10 mm neoplasms could be detected.

  17. Subsequent neoplasms of the CNS among survivors of childhood cancer: a systematic review.

    Science.gov (United States)

    Bowers, Daniel C; Nathan, Paul C; Constine, Louis; Woodman, Catherine; Bhatia, Smita; Keller, Karen; Bashore, Lisa

    2013-07-01

    Childhood cancer survivors are at risk for development of subsequent neoplasms of the CNS. Better understanding of the rates, risk factors, and outcomes of subsequent neoplasms of the CNS among survivors of childhood cancer could lead to more informed screening guidelines. Two investigators independently did a systematic search of Medline and Embase (from January, 1966, through March, 2012) for studies examining subsequent neoplasms of the CNS among survivors of childhood cancer. Articles were selected to answer three questions: what is the risk of CNS tumours after radiation to the cranium for a paediatric cancer, compared with the risk in the general population; what are the outcomes in children with subsequent neoplasms of the CNS who received CNS-directed radiation for a paediatric cancer; and, are outcomes of subsequent neoplasms different from primary neoplasms of the same histology? Our search identified 72 reports, of which 18 were included in this Review. These studies reported that childhood cancer survivors have an 8·1-52·3-times higher incidence of subsequent CNS neoplasms compared with the general population. Nearly all cancer survivors who developed a CNS neoplasm had been exposed to cranial radiation, and some studies showed a correlation between radiation dose and risk of subsequent CNS tumours. 5-year survival ranged from 0-19·5% for subsequent high-grade gliomas and 57·3-100% for meningiomas, which are similar rates to those observed in patients with primary gliomas or meningiomas. The quality of evidence was limited by variation in study design, heterogeneity of details regarding treatment and outcomes, limited follow-up, and small sample sizes. We conclude that survivors of childhood cancer who received cranial radiation therapy have an increased risk for subsequent CNS neoplasms. The current literature is insufficient to comment about the potential harms and benefits of routine screening for subsequent CNS neoplasms.

  18. Clinical significance of colonoscopy in patients with upper gastrointestinal polyps and neoplasms: a meta-analysis.

    Directory of Open Access Journals (Sweden)

    Zhen-Jie Wu

    Full Text Available BACKGROUND: Some authors have studied the relationship between the presence of polyps, adenomas and cancers of upper gastrointestinal tract (stomach and duodenum and risk of colorectal polyps and neoplasms; however, the results are controversial, which may be due to study sample size, populations, design, clinical features, and so on. No meta-analysis, which can be generalized to a larger population and could provide a quantitative pooled risk estimate of the relationship, of this issue existed so far. METHODS: We performed a meta-analysis to evaluate risk of colorectal polyps or neoplasms in patients with polyps, adenomas or cancers in upper gastrointestinal tract comparing with controls. A search was conducted through PubMed, EMBASE, reference lists of potentially relevant papers, and practice guidelines up to 27 November 2013 without languages restriction. Odd ratios (ORs were pooled using random-effects models. RESULTS: The search yielded 3 prospective and 21 retrospective case-control studies (n = 37152 participants. The principal findings included: (1 OR for colorectal polyps was 1.15 (95% CI, 1.04-1.26 in the gastric polyps group comparing with control groups; (2 Patients with gastric polyps and neoplasms have higher risk (OR, 1.31 [95% CI, 1.06-1.62], and 1.72 [95% CI, 1.42-2.09], respectively of colorectal neoplasms comparing with their controls; and (3 Positive association was found between the presence of colorectal neoplasms and sporadic duodenal neoplasms (OR, 2.59; 95% CI, 1.64-4.11. CONCLUSIONS: Findings from present meta-analysis of 24 case-control studies suggest that the prevalence of colorectal polyps was higher in patients with gastric polyps than in those without gastric polyps, and the risk of colorectal neoplasms increases significantly in patients with gastric polyps, neoplasms, and duodenal neoplasms. Therefore, screening colonoscopy should be considered for patients with upper gastrointestinal polyps and neoplasms.

  19. JAK2 GGCC haplotype in MPL mutated myeloproliferative neoplasms.

    Science.gov (United States)

    Pietra, Daniela; Casetti, Ilaria; Da Vià, Matteo C; Elena, Chiara; Milanesi, Chiara; Rumi, Elisa

    2012-07-01

    JAK2 (V617F) is associated with a genetic predisposition to its acquisition,as it is preferentially found in subjects with a common constitutional JAK2 haplotype known as 46/1 or GGCC. A recent study suggests that a genetic predisposition to acquisition of MPL mutation may exist in sporadic patients, since an association was found with the JAK2 46/1 haplotype. We genotyped 509 patients with myeloproliferative neoplasms (MPN), 7% of which carrying a somatic mutation of MPL Exon 10. We found that the JAK2 GGCC haplotype was closely associated with JAK2 (V617F) (OR 1.84, P < 0.001) but not with MPL mutations (OR 0.98), suggesting a different genetic background for these molecular lesions.

  20. Uterine Rupture Due to Invasive Metastatic Gestational Trophoblastic Neoplasm

    Directory of Open Access Journals (Sweden)

    David I Bruner

    2013-09-01

    Full Text Available While complete molar pregnancies are rare, they are wrought with a host of potential complications to include invasive gestational trophoblastic neoplasia. Persistent gestational trophoblastic disease following molar pregnancy is a potentially fatal complication that must be recognized early and treated aggressively for both immediate and long-term recovery. We present the case of a 21-year-old woman with abdominal pain and presyncope 1 month after a molar pregnancy with a subsequent uterine rupture due to invasive gestational trophoblastic neoplasm. We will discuss the complications of molar pregnancies including the risks and management of invasive, metastatic gestational trophoblastic neoplasia. [West J Emerg Med. 2013;14(5:444–447.

  1. Large mucinous cystic neoplasm of the pancreas associated with pregnancy

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Mucinous cystic neoplasms (MCNs) of the pancreas occur mostly in females and are potentially sex hormone-sensitive. However, a MCN occurring during pregnancy is quite rare. A 30-year-old woman in the tenth week of pregnancy was referred to us because of a rapid increase in lett hypochondrial distending pain. On ultrasound, the patient had a large intra-abdominal cystic lesion. She was thereafter diagnosed with missed abortion and a computed tomography scan showed that the lesion was a cystic tumor 18 cm in diameter originating from the pancreatic tail. The patient subsequently underwent tumor resection with distal pancreatectomy, sparing the spleen. Histopathological analysis of the specimen revealed a pancreatic MCN with moderate dysplasia. Immunohistochemically, the tumor was positive for both estrogen and progesterone receptors. To our knowledge, this is the first reported case of pancreatic MCN with moderate zysplasia in association with pregnancy. Our case strongly indicates that pancreatic MCN is female-hormone dependent.

  2. Dermoscopy of benign and malignant neoplasms in the pediatric population.

    Science.gov (United States)

    Haliasos, Helen C; Zalaudek, Iris; Malvehy, Josep; Lanschuetzer, Christoph; Hinter, Helmut; Hofmann-Wellenhof, Rainer; Braun, Ralph; Marghoob, Ashfaq A

    2010-12-01

    Dermoscopy is a noninvasive technique that enables visualization of subsurface colors and structures within the skin that are imperceptible to the naked eye. The dermatoscope allows the physician to examine both the macroscopic and microscopic primary morphology of skin lesions, identify subtle clinical clues, confirm naked-eye clinical diagnoses, and monitor treatment progress while posing little threat to the young patient. Dermoscopic findings have been formulated into diagnostic criteria that assist experienced clinicians in differentiating benign and malignant neoplasms. In this review, clinical morphology of melanocytic nevi and melanoma in the pediatric population is examined and the relevant dermoscopic findings and histopathologic correlates that aid in the diagnosis and management of these lesions are described.

  3. Myxoma of the temporal bone: A rare neoplasm

    Directory of Open Access Journals (Sweden)

    Kapil Sikka

    2011-01-01

    Full Text Available Myxomas of the temporal bone are rare mesenchymal tumors. We present a case of a 16-year-old girl who presented with complaint of mass lesion protruding from the right ear canal and from behind the ear for last 2 years. Biopsy of the mass was suggestive of benign mesenchymal lesion with prominent myxoid changes. Radiology of the temporal bone showed soft tissue density involving right side external auditory canal, middle ear, mastoid antrum and both lobes of the parotid gland. Tumour excision along with total parotidectomy was performed. Postoperative histopatholgy was consistent with myxoma. The patient is on regular follow-up for last 5months with no recurrence till date. Temporal bone myxomas are rare neoplasms which are surgically treatable but have a strong tendency to recur, necessiating long-term follow-up.

  4. Selective use of radiation therapy for neoplasms of the skin

    Energy Technology Data Exchange (ETDEWEB)

    Parker, R.G.

    1980-07-01

    Radiation therapy is preferable treatment for a minority of basal cell and epidermoid carcinomas of the skin. Proper use exploits the inherent advantage of preservation of function and cosmesis. Therefore, many cancers involving the eyelid, canthus, nose, nasolabial fold, pinna, ear canal, vermilion surface of the lower lip and skin of the chin can be advantageously treated by radiation therapy as compared to surgery, if pretreatment destruction of normal tissue is minimal. Although irradiation is equally effective, surgery is more expeditious for small lesions and cancers at other sites, which can be excised and followed by primary closure, and for large lesions if reconstruction will be required after destruction of the tumor. Radiation therapy can be effective, and usually is preferable treatment, for several other primary neoplasms of skin such as mycosis fungoides and Kaposi's sarcoma.

  5. A VERY RARE CASE OF SKIN ADNEXAL NEOPLASM: HIDRADENOMA

    Directory of Open Access Journals (Sweden)

    Sunil

    2014-11-01

    Full Text Available DEFINITION: Hidradenoma is a form of benign adnexal neoplasm that is a close relative of poroma, but is characterized by cells with ample cytoplasm1-4. Here we present a very rare and interesting case of a 35 year old female patient who presented to surgical opd with the complaints of swelling in front of the middle of the neck since two years. Swelling was not associated with any other complaints like pain, difficulty in swallowing, difficulty in speaking and difficulty in pronunciation of words. With adequate pre-operative preparation and normal routine blood investigations, patient was posted for surgery under monitored anesthesia care {ie.MAC}. A wide local excision was performed and specimen was sent for histopathological examination. HPR revealed an eccrine clear cell hidradenoma of neck. On follow-up for 2 year there has been no recurrence

  6. CT diagnosis of hyperdense intracranial neoplasms. Review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Ishikura, Reiichi; Ando, Kumiko; Tominaga, Satoru; Nakao, Norio [Hyogo College of Medicine, Nishinomiya (Japan); Ikeda, Jouta; Takemura, Yuriko; Morikawa, Tsutomu

    1999-03-01

    In contrast to typical astrocytic tumors that show hypodense areas on computed tomographic images, some intracranial tumors show hyperdense areas on CT images. The major reasons for hyperdensity on CT images are hypercellular lesions, intratumoral calcification, and intratumoral hemorrhage. Malignant lymphomas, germinomas, and medulloblastomas show homogenous hyperdensity on CT images because of their hypercellularity. Tumorous lesions such as subependymal giant cell astrocytomas, oligodendrogliomas, ependymomas, central neurocytomas, craniopharyngiomas, and meningiomas often present with hyperdense calcified lesions on CT images. Intratumoral hemorrhage also causes hyperdensity on CT images, and is often associated with metastatic brain tumors, glioblastomas, pituitary adenomas, and rarely with any of the other intracranial tumors. Although magnetic resonance imaging is now the major diagnostic tool for diseases of the central nervous system, the first imaging studies for patients with neurologic symptoms are still CT scans. Hyperdense areas on CT images are a clue to making an accurate diagnosis of intracranial neoplasms. (author)

  7. Imaging spectrum of primary malignant renal neoplasms in children

    Directory of Open Access Journals (Sweden)

    Rama Anand

    2012-01-01

    Full Text Available Wilms′ tumor (WT is the most common abdominal tumor in children. Many pediatric renal tumors in the past were categorized as WT; however, in recent years, several specific renal tumors have been recognized as distinct pathological entities. The age and clinical presentation of the child and distinctive imaging features may help in reaching a specific diagnosis in most cases. This is important as it has implications on the pre-operative diagnostic work-up and prognosis of the child. However, it is often not possible to differentiate one from the other pediatric renal tumor on the basis of imaging alone, and the final diagnosis is often made at histological examination of the surgical specimen. This article reviews the imaging features of primary malignant renal neoplasms in children along with their clinical presentation and pathological features.

  8. Mouse models of myeloproliferative neoplasms: JAK of all grades

    Directory of Open Access Journals (Sweden)

    Juan Li

    2011-05-01

    Full Text Available In 2005, several groups identified a single gain-of-function point mutation in the JAK2 kinase that was present in the majority of patients with myeloproliferative neoplasms (MPNs. Since this discovery, much effort has been dedicated to understanding the molecular consequences of the JAK2V617F mutation in the haematopoietic system. Three waves of mouse models have been produced recently (bone marrow transplantation, transgenic and targeted knock-in, which have facilitated the understanding of the molecular pathogenesis of JAK2V617F-positive MPNs, providing potential platforms for designing and validating novel therapies in humans. This Commentary briefly summarises the first two types of mouse models and then focuses on the more recently generated knock-in models.

  9. Lack of BRAF mutations in hyalinizing trabecular neoplasm

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    Brose Marcia

    2006-01-01

    Full Text Available Abstract The hyalinizing trabecular neoplasm (HTN of the thyroid is an unusual and controversial lesion. Some consider it a peculiar type of papillary thyroid carcinoma (PTC because of its nuclear features and presence of psammoma bodies. Others consider it an adenoma. Molecular studies have found RET/PTC translocations in some examples, supporting HTN as a PTC; however mutations in BRAF (another marker for PTC have not been found. We report two cases of classic HTN and a case of trabecular PTC and show BRAF mutations in the latter and not in HTN. Trabecular growth pattern is insufficient for a diagnosis of HTN and lesions with such a pattern and nuclear features of PTC are cancers. Morphologically classic HTN are not associated with metastatic potential and should be considered adenomas.

  10. Regional cerebellar volume and cognitive function from adolescence to late middle age.

    Science.gov (United States)

    Bernard, Jessica A; Leopold, Daniel R; Calhoun, Vince D; Mittal, Vijay A

    2015-03-01

    Cerebellar morphology and function have been implicated in a variety of developmental disorders, and in healthy aging. Although recent work has sought to characterize the relationships between volume and age in this structure during adolescence, young, and older adulthood, there have been no investigations of regional cerebellar volume from adolescence through late middle age. Middle age in particular has been largely understudied, and investigating this period of the lifespan may be especially important for our understanding of senescence. Understanding regional patterns of cerebellar volume with respect to age during this portion of the lifespan may provide important insight into healthy aging and cognitive function as well as pathology from adolescence into later life. We investigated regional cerebellar volume using a highly novel lobular segmentation approach in conjunction with a battery of cognitive tasks in a cross-sectional sample of 123 individuals from 12 to 65 years old. Our results indicated that regional cerebellar volumes show different patterns with respect to age. In particular, the more posterior aspect of the neocerebellum follows a quadratic "inverse-U" pattern while the vermis and anterior cerebellum follow logarithmic patterns. In addition, we quantified the relationships between age and a variety of cognitive assessments and found relationships between regional cerebellar volumes and performance. Finally, exploratory analyses of sex differences in the relationships between regional cerebellar volume, age, and cognition were investigated. Taken together, these results provide key insights into the development and aging of the human cerebellum, and its role in cognitive function across the lifespan.

  11. Self-Organization of Polarized Cerebellar Tissue in 3D Culture of Human Pluripotent Stem Cells

    Directory of Open Access Journals (Sweden)

    Keiko Muguruma

    2015-02-01

    Full Text Available During cerebellar development, the main portion of the cerebellar plate neuroepithelium gives birth to Purkinje cells and interneurons, whereas the rhombic lip, the germinal zone at its dorsal edge, generates granule cells and cerebellar nuclei neurons. However, it remains elusive how these components cooperate to form the intricate cerebellar structure. Here, we found that a polarized cerebellar structure self-organizes in 3D human embryonic stem cell (ESC culture. The self-organized neuroepithelium differentiates into electrophysiologically functional Purkinje cells. The addition of fibroblast growth factor 19 (FGF19 promotes spontaneous generation of dorsoventrally polarized neural-tube-like structures at the level of the cerebellum. Furthermore, addition of SDF1 and FGF19 promotes the generation of a continuous cerebellar plate neuroepithelium with rhombic-lip-like structure at one end and a three-layer cytoarchitecture similar to the embryonic cerebellum. Thus, human-ESC-derived cerebellar progenitors exhibit substantial self-organizing potential for generating a polarized structure reminiscent of the early human cerebellum at the first trimester.

  12. Direct and indirect spino-cerebellar pathways: shared ideas but different functions in motor control

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    Juan eJiang

    2015-07-01

    Full Text Available The impressive precision of mammalian limb movements relies on internal feedback pathways that convey information about ongoing motor output to cerebellar circuits. The spino-cerebellar tracts (SCT in the cervical, thoracic and lumbar spinal cord have long been considered canonical neural substrates for the conveyance of internal feedback signals. Here we consider the distinct features of an indirect spino-cerebellar route, via the brainstem lateral reticular nucleus (LRN, and the implications of this pre-cerebellar ‘detour’ for the execution and evolution of limb motor control. Both direct and indirect spino-cerebellar pathways signal spinal interneuronal activity to the cerebellum during movements, but evidence suggests that direct SCT neurons are mainly modulated by rhythmic activity, whereas the LRN also receives information from systems active during postural adjustment, reaching and grasping. Thus, while direct and indirect spino-cerebellar circuits can both be regarded as internal copy pathways, it seems likely that the direct system is principally dedicated to rhythmic motor acts like locomotion, while the indirect system also provides a means of pre-cerebellar integration relevant to the execution and coordination of de

  13. Modality specificity in the cerebro-cerebellar neurocircuitry during working memory.

    Science.gov (United States)

    Ng, H B Tommy; Kao, K-L Cathy; Chan, Y C; Chew, Effie; Chuang, K H; Chen, S H Annabel

    2016-05-15

    Previous studies have suggested cerebro-cerebellar circuitry in working memory. The present fMRI study aims to distinguish differential cerebro-cerebellar activation patterns in verbal and visual working memory, and employs a quantitative analysis to deterimine lateralization of the activation patterns observed. Consistent with Chen and Desmond (2005a,b) predictions, verbal working memory activated a cerebro-cerebellar circuitry that comprised left-lateralized language-related brain regions including the inferior frontal and posterior parietal areas, and subcortically, right-lateralized superior (lobule VI) and inferior cerebellar (lobule VIIIA/VIIB) areas. In contrast, a distributed network of bilateral inferior frontal and inferior temporal areas, and bilateral superior (lobule VI) and inferior (lobule VIIB) cerebellar areas, was recruited during visual working memory. Results of the study verified that a distinct cross cerebro-cerebellar circuitry underlies verbal working memory. However, a neural circuitry involving specialized brain areas in bilateral neocortical and bilateral cerebellar hemispheres subserving visual working memory is observed. Findings are discussed in the light of current models of working memory and data from related neuroimaging studies.

  14. Spectrum of Intestinal Neoplasms: A study of 400 cases

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    Aminder Singh

    2015-02-01

    Full Text Available Objective: The present study is a five-year analysis of all the tumors of small and large intestine received in the Pathology Department of Dayanand Medical College and Hospital, Ludhiana. Methods: All the cases were grossly and microscopically examined and were staged according to Astler Coller Staging and classified and subtyped according to WHO classification. The important differences between the small and large intestine tumors were also analyzed. Results: There were 400 cases out of which 356 were in large intestine while 44 were in the small intestine. There were only 56 benign neoplasms while 344 were malignant tumors. Adenomas were the most common benign tumors while majority of malignant tumors were adenocarcinomas. Lymphomas, mesenchymal tumors, and carcinoid tumors were much more common in a small intestine as compared to large intestine. Majority of adenocarcinomas were located in the large intestine with most of them being moderately differentiated having Astler Coller Stage B II. Mucinous carcinomas had the worst prognosis as compared to adenocarcinomas. Anal canal had mainly squamous cell carcinomas. Conclusions: Tumors of large intestine were much commoner than of small intestine. There was a higher incidence of tumor in males with M:F ratio of 2.2:1. Mean age of presentation of benign tumor was younger, i.e., 32.6 years when compared to 54.5 years for malignant tumors. Tubular adenoma was the most common benign tumor and adenocarcinoma the commonest malignant neoplasm. [J Interdiscipl Histopathol 2015; 3(1.000: 19-23

  15. Hepatic neoplasms associated with contraceptive and anabolic steroids.

    Science.gov (United States)

    Ishak, K G

    1979-01-01

    This paper evaluates the differences between HCA (hepatocellular adenoma) and FNH (focal nodular hyperplasia) and the association of HCA and FNH with OC (oral contraceptives). FNH occurs at least twice as frequent in females as in males. A study conducted by the author revealed that only 20% of patients with FNH had symptoms and signs related to their neoplasms; in the rest, FNH was accidentally discovered during surgery for diseases of the gallbladder or at necropsy. The highly characteristic gross appearance of FNH is discribed in detail. The etiologic relationship between FNH and OC was cited in the light of frequent findings of FNH in infants and children, and of suggestions by other authors that FNH could be a direct result on OC therapy or that contraceptive steroids or conjugated estrogens accelerate the growth of FNH, a very slow growing neoplasm. Simple excision is the treatment of choice for FNH; in some cases, hepatic artery ligation is indicated. In the case of HCA, statistics show that the incidence of HCA has been increasing since 1960. Majority of patients with HCA have normal tests of hepatic function. Radiographic studies and hepatic scans may reveal HCA, but the best diagnostic method so far is angiography. Although gross appearance of HCA is variable, the features are clearly distinguishable from that of FNH. Other topics discussed include the occasional occurence of nodular regenerative hyperplasia in patients on OC or anabolic steroids (AS), and malignant liver tumors in patients using OC or AS. Further research should be done to clarify the etiologic relationship between androgenic-anabolic steroids and hepatocellular tumors and tumorlike lesions.

  16. Percutaneous thermal ablation of renal neoplasms; Perkutane Thermoablation von Nierentumoren

    Energy Technology Data Exchange (ETDEWEB)

    Tacke, J. [Inst. fuer Diagnostische und Interventionelle Radiologie/Neuroradiologie, Klinikum Passau (Germany); Mahnken, A.H.; Guenther, R.W. [Klinik fuer Radiologische Diagnostik, Universitaetsklinikum Aachen (Germany)

    2005-12-15

    Due to modern examination techniques such as multidetector computed tomography and high-field magnetic resonance imaging, the detection rate of renal neoplasms is continually increasing. Even though tumors exceeding 4 cm in diameter rarely metastasize, all renal lesions that are possible neoplasms should be treated. Traditional treatment techniques include radical nephrectomy or nephron-sparing resection, which are increasingly performed laparoscopically. Modern thermal ablation techniques such as hyperthermal techniques like radiofrequency ablation RFA, laser induced thermal ablation LITT, focused ultrasound FUS and microwave therapy MW, as well as hypothermal techniques (cryotherapy) may be a useful treatment option for patients who are unfit for or refuse surgical resection. Cryotherapy is the oldest and best known thermal ablation technique and can be performed laparoscopically or percutaneously. Since subzero temperatures have no antistyptic effect, additional maneuvers must be performed to control bleeding. Percutaneous cryotherapy of renal tumors is a new and interesting method, but experience with it is still limited. Radiofrequency ablation is the most frequently used method. Modern probe design allows volumes between 2 and 5 cm in diameter to be ablated. Due to hyperthermal tract ablation, the procedure is deemed to be safe and has a low complication rate. Although there are no randomized comparative studies to open resection, the preliminary results for renal RFA are promising and show RFA to be superior to other thermal ablation techniques. Clinical success rates are over 90% for both, cryo- and radiofrequency ablation. Whereas laser induced thermal therapy is established in hepatic ablation, experience is minimal with respect to renal application. For lesions of more than 2 cm in diameter, additional cooling catheters are required. MR thermometry offers temperature control during ablation. Microwave ablation is characterized by small ablation volumes

  17. Restoring cognitive functions using non-invasive brain stimulation techniques in patients with cerebellar disorders

    Directory of Open Access Journals (Sweden)

    Paul A Pope

    2014-04-01

    Full Text Available Numerous studies have highlighted the possibility of modulating the excitability of cerebro-cerebellar circuits bi-directionally using transcranial electrical brain stimulation, in a manner akin to that observed using magnetic stimulation protocols. It has been proposed that cerebellar stimulation activates Purkinje cells in the cerebellar cortex, leading to inhibition of the dentate nucleus, which exerts a tonic facilitatory drive onto motor and cognitive regions of cortex through a synaptic relay in the ventral-lateral thalamus. Some cerebellar deficits present with cognitive impairments if damage to non-motor regions of the cerebellum disrupts the coupling with cerebral cortical areas for thinking and reasoning. Indeed, white matter changes in the dentato-rubral tract correlate with cognitive assessments in patients with Friedreich ataxia, suggesting that this pathway is one component of the anatomical substrate supporting a cerebellar contribution to cognition. An understanding of the physiology of the cerebro-cerebellar pathway previously helped us to constrain our interpretation of results from two recent studies in which we showed cognitive enhancements in healthy participants during tests of arithmetic after electrical stimulation of the cerebellum, but only when task demands were high. Others studies have also shown how excitation of the prefrontal cortex can enhance performance in a variety of working memory tasks. Thus, future efforts might be guided towards neuro-enhancement in certain patient populations, using what is commonly termed 'non-invasive brain stimulation' as a cognitive rehabilitation tool to modulate cerebro-cerebellar circuits, or for stimulation over the cerebral cortex to compensate for decreased cerebellar drive to this region. This article will address these possibilities with a review of the relevant literature covering ataxias and cerebellar cognitive affective disorders, which are characterized by thalamo

  18. Qualitative and quantitative aspects of the microanatomy of the African elephant cerebellar cortex.

    Science.gov (United States)

    Maseko, Busisiwe C; Jacobs, Bob; Spocter, Muhammad A; Sherwood, Chet C; Hof, Patrick R; Manger, Paul R

    2013-01-01

    The current study provides a number of novel observations on the organization and structure of the cerebellar cortex of the African elephant by using a combination of basic neuroanatomical and immunohistochemical stains with Golgi and stereologic analysis. While the majority of our observations indicate that the cerebellar cortex of the African elephant is comparable to other mammalian species, several features were unique to the elephant. The three-layered organization of the cerebellar cortex, the neuronal types and some aspects of the expression of calcium-binding proteins were common to a broad range of mammalian species. The Lugaro neurons observed in the elephant were greatly enlarged in comparison to those of other large-brained mammals, suggesting a possible alteration in the processing of neural information in the elephant cerebellar cortex. Analysis of Golgi impregnations indicated that the dendritic complexity of the different interneuron types was higher in elephants than other mammals. Expression of parvalbumin in the parallel fibers and calbindin expressed in the stellate and basket cells also suggested changes in the elephant cerebellar neuronal circuitry. The stereologic analysis confirmed and extended previous observations by demonstrating that neuronal density is low in the elephant cerebellar cortex, providing for a larger volume fraction of the neuropil. With previous results indicating that the elephants have the largest relative cerebellar size amongst mammals, and one of the absolutely largest mammalian cerebella, the current observations suggest that the elephants have a greater volume of a potentially more complexly organized cerebellar cortex compared to other mammals. This quantitatively larger and more complex cerebellar cortex likely represents part of the neural machinery required to control the complex motor patterns involved in movement of the trunk and the production of infrasonic vocalizations.

  19. Restoring cognitive functions using non-invasive brain stimulation techniques in patients with cerebellar disorders.

    Science.gov (United States)

    Pope, Paul A; Miall, R Chris

    2014-01-01

    Numerous studies have highlighted the possibility of modulating the excitability of cerebro-cerebellar circuits bi-directionally using transcranial electrical brain stimulation, in a manner akin to that observed using magnetic stimulation protocols. It has been proposed that cerebellar stimulation activates Purkinje cells in the cerebellar cortex, leading to inhibition of the dentate nucleus, which exerts a tonic facilitatory drive onto motor and cognitive regions of cortex through a synaptic relay in the ventral-lateral thalamus. Some cerebellar deficits present with cognitive impairments if damage to non-motor regions of the cerebellum disrupts the coupling with cerebral cortical areas for thinking and reasoning. Indeed, white matter changes in the dentato-rubral tract correlate with cognitive assessments in patients with Friedreich ataxia, suggesting that this pathway is one component of the anatomical substrate supporting a cerebellar contribution to cognition. An understanding of the physiology of the cerebro-cerebellar pathway previously helped us to constrain our interpretation of results from two recent studies in which we showed cognitive enhancements in healthy participants during tests of arithmetic after electrical stimulation of the cerebellum, but only when task demands were high. Others studies have also shown how excitation of the prefrontal cortex can enhance performance in a variety of working memory tasks. Thus, future efforts might be guided toward neuro-enhancement in certain patient populations, using what is commonly termed "non-invasive brain stimulation" as a cognitive rehabilitation tool to modulate cerebro-cerebellar circuits, or for stimulation over the cerebral cortex to compensate for decreased cerebellar drive to this region. This article will address these possibilities with a review of the relevant literature covering ataxias and cerebellar cognitive affective disorders, which are characterized by thalamo-cortical disturbances.

  20. Tract Profiles of the Cerebellar White Matter Pathways in Children and Adolescents.

    Science.gov (United States)

    Leitner, Yael; Travis, Katherine E; Ben-Shachar, Michal; Yeom, Kristen W; Feldman, Heidi M

    2015-12-01

    Intact development of cerebellar connectivity is essential for healthy neuromotor and neurocognitive development. To date, limited knowledge about the microstructural properties of the cerebellar peduncles, the major white matter tracts of the cerebellum, is available for children and adolescents. Such information would be useful as a comparison for studies of normal development, clinical conditions, or associations of cerebellar structures with cognitive and motor functions. The goal of the present study was to evaluate the variability in diffusion measures of the cerebellar peduncles within individuals and within a normative sample of healthy children. Participants were 19 healthy children and adolescents, aged 9-17 years, mean age 13.0 ± 2.3. We analyzed diffusion magnetic resonance imaging (dMRI) data with deterministic tractography. We generated tract profiles for each of the cerebellar peduncles by extracting four diffusion properties (fractional anisotropy (FA) and mean, radial, and axial diffusivity) at 30 equidistant points along each tract. We were able to identify the middle cerebellar peduncle and the bilateral inferior and superior cerebellar peduncles in all participants. The results showed that within each of the peduncles, the diffusion properties varied along the trajectory of the tracts. However, the tracts showed consistent patterns of variation across individuals; the coefficient of variation for FA across individual profiles was low (≤20%) for each tract. We observed no systematic variation of the diffusion properties with age. These cerebellar tract profiles of the cerebellar peduncles can serve as a reference for future studies of children across the age range and for children and adolescents with clinical conditions that affect the cerebellum.

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  9. Differences in saccade dynamics between spinocerebellar ataxia 2 and late-onset cerebellar ataxias.

    Science.gov (United States)

    Federighi, Pamela; Cevenini, Gabriele; Dotti, Maria T; Rosini, Francesca; Pretegiani, Elena; Federico, Antonio; Rufa, Alessandra

    2011-03-01

    The cerebellum is implicated in maintaining the saccadic subsystem efficient for vision by minimizing movement inaccuracy and by learning from endpoint errors. This ability is often disrupted in degenerative cerebellar diseases, as demonstrated by saccade kinetic abnormalities. The study of saccades in these patients may therefore provide insights into the neural substrate underlying saccadic motor control. We investigated the different extent of saccade dynamic abnormalities in spinocerebellar ataxia type 2 and late-onset cerebellar ataxias, genetically undefined and with prevalent cerebellar atrophy. Reflexive and voluntary saccades of different amplitude (10°-18°) were studied in seven patients with spinocerebellar ataxia 2, eight patients with late-onset cerebellar ataxia and 25 healthy controls. Quantitative analysis of saccade parameters and measures of saccade accuracy were performed. Detailed neurological, neurophysiological and magnetic resonance imaging assessment was obtained for each patient. Genetic and laboratory screening for spinocerebellar ataxias and other forms of late-onset cerebellar ataxias were also performed. A lower peak saccade velocity and longer duration was observed in patients with spinocerebellar ataxia 2 with respect to those with late-onset cerebellar ataxia and controls. Unlike subjects with spinocerebellar ataxia 2, patients with late-onset cerebellar ataxia showed main sequence relationships to similar saccades made by normal subjects. Saccades were significantly more inaccurate, namely hypometric, in late-onset cerebellar ataxia than in spinocerebellar ataxia 2 and inaccuracy increased with saccade amplitude. The percentage of hypometric primary saccades and of larger secondary corrective saccades were consistently higher in late-onset cerebellar ataxia than in spinocerebellar ataxia 2 and controls. No other significant differences were found between groups. Two different mechanisms were adopted to redirect the fovea as fast

  10. Pediatric cerebellar stroke associated with elevated titer of antibodies to β2-glycoprotein.

    Science.gov (United States)

    Spalice, Alberto; Del Balzo, Francesca; Perla, Francesco Massimo; Papetti, Laura; Nicita, Francesco; Ursitti, Fabiana; Properzi, Enrico

    2011-06-01

    Antibodies to 2-glycoprotein I (anti-2GPI) have been associated with recurrent thrombosis and pregnancy morbidity. However, the prevalence of anti-2GPI in children suffering from cerebral and cerebellar infarction is unknown. We report on a 10-month-old boy who had an ischemic cerebellar stroke, secondary to antiphospholipid syndrome with high titers of immunoglobulin G anti-2GPI (first titer: 132U) anticardiolipin antibodies and lupus anticoagulant tests were negative. All other causes of infarction were excluded. To our knowledge, this is the first reported case of childhood cerebellar ischemic stroke with only anti-2GPI but no antibodies detectable in standard antiphospholipid assays.

  11. A Case of Multiple System Atrophy-Cerebellar Type Preceded by Dementia

    Directory of Open Access Journals (Sweden)

    Eun Hye Jang

    2012-10-01

    Full Text Available Multiple system atrophy (MSA is a sporadic, adult-onset disease characterized by progressive degeneration of nervous systems including cerebellar, pyramidal, extrapyramidal, and autonomic system. Although a few recent studies reported that cognitive impairments could occur in patients with MSA, prominent dementia with progressive decline is not a typical clinical manifestation of MSA. In particular, dementia with MSA-cerebellar type is very rare. We have experienced a patient with 2-year history of severe cognitive impairment, who was finally diagnosed as MSA-cerebellar type.

  12. Cerebellar abnormalities typical of methylmercury poisoning in a fledged saltmarsh sparrow, Ammodramus caudacutus.

    Science.gov (United States)

    Scoville, Sheila A; Lane, Oksana P

    2013-05-01

    A fledged, 12-15 day-old saltmarsh sparrow, Ammodramus caudacutus, was collected from an accidental kill on Cinder Island, Long Island, NY, USA. The sparrow was assessed for feather mercury levels and the brain analyzed for cerebellar abnormalities by microscopic examination. In humans, fetal Minamata disease is caused by maternal ingestion of mercury. It is characterized by disrupted and disordered cerebellar neuronal migration in the fetus or infant. Results from this sparrow show cerebellar abnormalities typical of Minamata disease. It is the first known avian or mammalian specimen taken from the wild to show the abnormalities typical of the human fetal syndrome.

  13. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Saatci, I.; Baskan, O.; Haliloglu, M.; Aydingoz, U. [Department of Radiology, Hacettepe University Hospital, Sihhiye 06100, Ankara (Turkey)

    1999-06-01

    Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T 1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T 2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination. (orig.) With 5 figs., 22 refs.

  14. [Cerebellar abscesses secondary to infection of an occipital dermal sinus].

    Science.gov (United States)

    García Galera, A; Martínez León, M I; Pérez da Rosa, S; Ros López, B

    2013-09-01

    A dermal sinus is a congenital defect arising from a closure failure of the neural tube that results in different degrees of communication between the skin and the central nervous system. A dermal sinus can occur anywhere from the root of the nose to the conus medullaris, and the occipital location is the second most common. Dermal sinuses are often found in association with dermoid or epidermoid cysts and less frequently with teratomas. Patients with an occipital dermoid cyst associated with a dermal sinus can develop meningitis and/or abscesses as the first clinical manifestation of the disease due to the dermoid cyst itself becoming abscessed or to the formation of secondary abscesses; few cases of the formation of secondary abscesses have been reported. We present a case of a dermoid cyst associated with an infected dermal sinus and posterior development of cerebellar abscesses and hydrocephalus.

  15. Middle cerebellar peduncles:Magnetic resonance imaging and pathophysiologic correlate

    Institute of Scientific and Technical Information of China (English)

    Humberto Morales; Thomas Tomsick

    2015-01-01

    We describe common and less common diseases that can cause magnetic resonance signal abnormalities of middle cerebellar peduncles(MCP), offering a systematicapproach correlating imaging findings with clinical clues and pathologic mechanisms. Myelin abnormalities, different types of edema or neurodegenerative processes, can cause areas of abnormal T2 signal, variable enhancement, and patterns of diffusivity of MCP. Pathologies such as demyelinating disorders or certain neurodegenerative entities(e.g., multiple system atrophy or fragile X-associated tremor-ataxia syndrome) appear to have predilection for MCP. Careful evaluation of concomitant imaging findings in the brain or brainstem; and focused correlation with key clinical findings such as immunosuppression for progressive multifocal leukoencephalopahty; hypertension, post-transplant status or high dose chemotherapy for posterior reversible encephalopathy; electrolyte disorders for myelinolysis or suspected toxic-drug related encephalopathy; would yield an appropriate and accurate differential diagnosis in the majority of cases.

  16. Consensus paper on post-operative pediatric cerebellar mutism syndrome

    DEFF Research Database (Denmark)

    Gudrunardottir, Thora; Morgan, Angela T; Lux, Andrew L

    2016-01-01

    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. METHODS: Consensus was obtained using the modified nominal group technique, involving four rounds of online Delphi questionnaires interspersed with a structured consensus conference with lectures, group work, and open discussion sessions. RESULTS: A new, proposed definition of "post-operative...

  17. Cerebellar infarct with neurogenic pulmonary edema following viper bite

    Directory of Open Access Journals (Sweden)

    Salil Gupta

    2012-01-01

    Full Text Available Russell′s viper (Daboia russelli bites are well known to cause bleeding complications. However, thrombotic complications are rare. We present the case details of a female who was bitten by a Russell′s viper (Daboia russelli in her village. She then developed features of envenomation in the form of hemorrhagic episodes. She received 27 vials of polyvalent anti-snake venom to which the hemorrhagic complications responded. After about 48 h of the bite she developed features of cerebellar infarct along with pulmonary edema which was in all probability neurogenic in origin. She was managed with mechanical ventilation and extra ventricular drainage with good recovery. We discuss the likely pathogenesis of the infarct and pulmonary edema occurring in a patient with viper bite and other features of envenomation.

  18. Nerve growth factor enhances DNA synthesis in cultured cerebellar neuroblasts.

    Science.gov (United States)

    Confort, C; Charrasse, S; Clos, J

    1991-10-01

    The cerebellar neuroblasts in primary cultures from five-day-old rats bore NGF receptor immunoreactivity, suggesting a potential responsive to NGF. At low plating density, NGF was found to enhance DNA synthesis in these cells in a dose-dependent manner. As these cells synthesize NGF, one possibility to account for the lack of response of neuroblasts plated at high density is that the amount of endogenous trophic agent produced in this culture condition is sufficient to ensure an optimal effect. The results demonstrate that premitotic neuroblasts in the CNS, as well postmitotic neurons, are responsive to NGF. At the early stage of its development, the cerebellum therefore appears to be a very good autocrine model of NGF action.

  19. Incidence of underlying biliary neoplasm in patients after major hepatectomy for preoperative benign hepatolithiasis

    Science.gov (United States)

    Park, Hyeong Min; Cho, Chol Kyoon; Koh, Yang Seok; Kim, Hee Joon; Park, Eun Kyu

    2016-01-01

    Backgrounds/Aims Despite hepatolithiasis being a risk factor for biliary neoplasm including cholangiocarcinoma, the incidence of underlying biliary neoplasm is unknown in patients with preoperative benign hepatolithiasis. The aim of this study was to evaluate the incidence of underlying biliary neoplasm in patients who underwent major hepatectomy for preoperative benign hepatolithiasis. Methods Between March 2005 and December 2015, 73 patients who underwent major hepatectomy for preoperative benign hepatolithiasis were enrolled in this study. The incidence and pathological differentiation of concomitant biliary neoplasm were retrospectively determined by review of medical records. Postoperative complications after major hepatectomy were evaluated. Results Concomitant biliary neoplasm was pathologically confirmed in 20 patients (27.4%). Biliary intraepithelial neoplasia (BIN) was detected in 12 patients (16.4%), and 1 patient (1.4%) had intraductal papillary mucinous neoplasm (IPMN), as the premalignant lesion. Cholangiocarcinoma was pathologically confirmed in 7 patients (9.6%). Preoperative imaging of the 73 patients revealed biliary stricture at the first branch of bile duct in 31 patients (42.5%), and at the second branch of bile duct in 39 patients (53.4%). Postoperative complications developed in 14 patients (19.1%). Almost all patients recovered from complications, including intra-abdominal abscess (9.6%), bile leakage (4.1%), pleural effusion (2.7%), and wound infection (1.4%). Only 1 patient (1.4%) died from aspiration pneumonia. Conclusions The incidence of underlying biliary neoplasm was not negligible in the patients with hepatolithiasis, despite meticulous preoperative evaluations. PMID:28261696

  20. Molecular mechanisms governing competitive synaptic wiring in cerebellar Purkinje cells.

    Science.gov (United States)

    Watanabe, Masahiko

    2008-03-01

    Cerebellar Purkinje cells (PCs) play a principal role in motor coordination and motor learning. To fulfill these functions, PCs receive and integrate two types of excitatory inputs, climbing fiber (CF) and parallel fiber (PF). CFs are projection axons from the inferior olive, and convey error signals to PCs. On the other hand, PFs are T-shaped axons of cerebellar granule cells, and convey sensory and motor information carried through the pontocerebellar and spinocerebellar mossy fiber pathways. The most remarkable feature of PC circuits is the highly territorial innervation by these two excitatory afferents. A single climbing CF powerfully and exclusively innervates proximal PC dendrites, whereas hundreds of thousands of PFs innervate distal PC dendrites. Recent studies using gene-manipulated mice have been elucidating that the PC circuitry is formed and maintained by molecular mechanisms that fuel homosynaptic competition among CFs and heterosynaptic competition between CFs and PFs. GluRdelta2 (a PC-specific glutamate receptor) and precerebellin or Cbln1 (a granule cell-derived secretory protein) cooperatively work for selective strengthening of PF-PC synapses, and prevent excessive distal extension of CFs that eventually causes multiple innervation at distal dendrites. In contrast, P/Q-type Ca2+ channels, which mediate Ca2+ influx upon CF activity, selectively strengthen the innervation by a single main CF, and expel PFs and other CFs from proximal dendrites that it innervates. Therefore, we now understand that owing to these mechanisms, territorial innervation by CFs and PFs is properly structured and mono-innervation by CFs is established. Several key issues for future study are also discussed.

  1. Cerebellar stimulation for cerebral palsy--double blind study.

    Science.gov (United States)

    Davis, R; Schulman, J; Delehanty, A

    1987-01-01

    Twenty spastic cerebral palsy (CP) patients undergoing chronic cerebellar stimulation (CCS) for reduction of spasticity and improvement in function have participated in a double-blind study. Seven US centers involving 9 neurosurgeons (1984-6) have replaced the depleted Neurolith 601 fully implantable pulse generator (Pacesetter Systems Incorp.-Neurodyne Corp., Sylmar, CA) with new units in 19 CP patients, 1 patient entered the study following his initial implant. A magnetically controllable switch was placed in line between the Neurolith stimulator and the cerebellar lead, so allowing switching sequences for the study. Physical therapists, living in the vicinity of the patient's home, carried out two quantitative evaluations: 1. Joint angle motion measurements (passive and active). 2. Motor performance testing was done when possible and included: reaction time, hand dynamonetry, grooved peg board placement, hand/foot tapping, and rotary pursuit testing. Testing was done presurgery, at 2 weeks postimplant, then the switch was activated either "on" or "off" to a schedule, with testing and reswitching at 1, 2 and 4 months, then the switch was left turned "on". Of the 20 patients, 16 finished the tests, 2 patients failed to finish and 2 had switch problems and were deleted from the study. Two of the 16 patients were "off" through the entire testing. Of the 14 that had periods of the stimulator being "on", 10 patients (72%) had quantitative improvements of over 20%, (1 pt: 50+% improvements; 4 pts: 30-50%, 5 pts: 20-30%); while 1 patient (7%) had improvements in the 10-20% level, whereas 3 patients (21%) showed no improvement.

  2. Synchronous Low-grade Appendiceal Mucinous Neoplasm and Primary Peritoneal Low-grade Serous Carcinoma: A First Description of These 2 Neoplasms Presenting Together as Suspected Peritoneal Carcinomatosis.

    Science.gov (United States)

    Sekulic, Miroslav; Pichler Sekulic, Simona; Movahedi-Lankarani, Saeid

    2016-09-28

    Low-grade appendiceal mucinous neoplasm is a neoplasm typically of appendiceal origin, which is characterized by diffuse peritoneal involvement by pools of mucin with mucinous epithelium lacking high-grade cytologic atypia, and clinically presents as suspected peritoneal carcinomatosis. A similar clinical presentation can sometimes be seen with disseminated low-grade serous carcinomas of the peritoneum, fallopian tubes, or ovaries; however, this neoplasm is histologically characterized by tubal-type epithelium and invasive or confluent growth. In this case report, we describe a patient presenting with a clinical examination and radiologic features suggestive of peritoneal carcinomatosis and a prominent pelvic mass; however, after pathologic review, the patient was proven to have peritoneal involvement by both low-grade appendiceal mucinous neoplasm of appendiceal origin and a low-grade peritoneal primary serous carcinoma. In short, we present the first description of low-grade appendiceal mucinous neoplasm and serous carcinoma of the peritoneum presenting synchronously, providing morphologic characterization and immunohistochemical studies supporting the diagnosis, and illustrating a rare instance in which 2 neoplastic processes are underlying clinically suspected peritoneal carcinomatosis.

  3. Retroperitoneal neoplasm with perivascular epithelioid cell differentiation:A case report and review of literature

    Institute of Scientific and Technical Information of China (English)

    Min Zhao; Jin Huang; Jin Wang

    2013-01-01

    The retroperitoneal neoplasm with perivascular epithelioid cel differentiation (PEComa) is an extremely rare path-ological entity. In this article, we reported one case of a 45-year-old woman who was admitted to our hospital (The Second People’s Hospital of Hefei, China) for retroperitoneal neoplasm with perivascular epithelioid cel dif erentiation. The B ultra-sonic examination showed echopoor in the region of cavitas pelvis. The histologic characteristics and immunohistochemical phenotype both revealed the neoplasm with perivascular epithelioid cel differentiation.

  4. Cerebellar Development and Plasticity: Perspectives for Motor Coordination Strategies, for Motor Skills, and for Therapy

    Directory of Open Access Journals (Sweden)

    J. D. Swinny

    2005-01-01

    Full Text Available The role of the mammalian cerebellum ranges from motor coordination, sensory-motor integration, motor learning, and timing to nonmotor functions such as cognition. In terms of motor function, the development of the cerebellum is of particular interest because animal studies show that the development of the cerebellar cortical circuitry closely parallels motor coordination. Ultrastructural analysis of the morphological development of the cerebellar circuitry, coupled with the temporal and spatial identification of the neurochemical substrates expressed during development, will help to elucidate their roles in the establishment of the cerebellar circuitry and hence motor activity. Furthermore, the convenience of a number of naturally occurring mouse mutations has allowed a functional dissection of the various cellular elements that make up the cerebellar circuitry. This understanding will also help in the approach to possible therapies of pathologies arising during development because tile cerebellum is especially prone to such perturbation because of its late development.

  5. Effects of drotaverine hydrochloride on viability of rat cultured cerebellar granulocytes.

    Science.gov (United States)

    Demushkin, V P; Zhavoronkova, E V; Khaspekov, L G

    2012-02-01

    The neurocytotoxic effect of drotaverine hydrochloride was studied in culture of rat cerebellar granulocytes. Incubation of cells with 100 and 250 μM drotaverine reduced neuronal survival to 60 and 4%, respectively.

  6. Uneven distribution of NG2 cells in the rat cerebellar vermis and changes in aging

    Directory of Open Access Journals (Sweden)

    S. Lomoio

    2012-06-01

    Full Text Available We describe by NG2 (neuron-glia chondroitin sulphate proteoglycan 2 immunocytochemistry an uneven distribution of NG2 glial cells in the rat cerebellum, being them more represented in the central lobules of the cerebellar vermis, belonging to the cerebrocerebellum. The cerebellar distribution of NG2 cells changes in aging rats, in which the area where the cells appear to be densely scattered throughout all cerebellar layers involves also more rostral and caudal lobules. In addition, in aging rats, in the most rostral and caudal lobules belonging to the spinocerebellum, punctate reaction product is present at the apical pole of Purkinje cells, i.e. in the area where the majority of synapses between olivary climbing fibers and Purkinje cells occur. Data suggest that the different distribution of NG2 cells is correlated to differences in physiology among cerebellar areas and reflects changes during aging.

  7. Post-traumatic cerebellar infarction due to vertebral artery foramina fracture: case report

    Directory of Open Access Journals (Sweden)

    Moscote-Salazar Luis Rafael

    2016-03-01

    Full Text Available Posttraumatic cerebral infarction is an uncommon cause of morbidity and mortality and many studies have highlighted that trauma needs to considered as causative factor for cerebellar infarction. We present a case of cerebellar infarction in a 35 year old young patient secondary to vertebral fracture involving the vertebral foramen and vertebral artery injury. CT scan cervical spine showed C2-3 fracture on left side with fracture extending into the left vertebral foramen. A CT scan angiogram could not be performed because of poor neurological status. Possibly the infarction was due to left vertebral artery injury. Without surgical intervention prognosis of these patients remain poor. Prognosis of patients with traumatic cerebellar infarction depends on the neurological status of the patient, intrinsic parenchymal damage and more importantly extrinsic compression of the brainstem by the edematous cerebellar hemispheres.

  8. Evolving Models of Pavlovian Conditioning : Cerebellar Cortical Dynamics in Awake Behaving Mice

    NARCIS (Netherlands)

    Ten Brinke, Michiel M; Boele, Henk-Jan; Spanke, Jochen K; Potters, Jan-Willem; Kornysheva, Katja; Wulff, Peer; IJpelaar, Anna C H G; Koekkoek, Sebastiaan K E; De Zeeuw, Chris I

    2015-01-01

    Three decades of electrophysiological research on cerebellar cortical activity underlying Pavlovian conditioning have expanded our understanding of motor learning in the brain. Purkinje cell simple spike suppression is considered to be crucial in the expression of conditional blink responses (CRs).

  9. A case of cerebellar dysarthria as the presenting symptom of HIV infection.

    Science.gov (United States)

    Siddiqi, Zeba; Karoli, Ritu; Fatima, Jalees; Dey, Rahul; Kazmi, Khursheed

    2014-08-01

    A 37 year old man presented with progressive dysarthria for 2 weeks. A week later he developed ataxia and bilateral cerebellar signs including intention tremors, dysmetria and dysdiadokokinesia. During evaluation for aetiology of cerebellar dysarthria, MRI brain revealed asymmetric altered signal intensities in bilateral cerebellar hemispheres and right side of pons suggesting demyelinating lesions. ELISA for Human Immune Deficiency virus-1 was positive. We kept a presumptive diagnosis of Progressive Multifocal Leukoencephalopathy (PML) on the basis of clinico-radiological picture. PML is an under investigated and under diagnosed CNS infection seen in HIV patients with advanced disease. We present an unusual case report where isolated cerebellar involvement occurred as the first AIDS defining event in the absence of appreciable immunodeficiency in a patient with previously undiagnosed HIV infection.

  10. Uneven distribution of NG2 cells in the rat cerebellar vermis and changes in aging

    Science.gov (United States)

    Lomoio, S.; Necchi, D.; Scherini, E.

    2012-01-01

    We describe by NG2 (neuron-glia chondroitin sulphate proteoglycan 2) immunocytochemistry an uneven distribution of NG2 glial cells in the rat cerebellum, being them more represented in the central lobules of the cerebellar vermis, belonging to the cerebrocerebellum. The cerebellar distribution of NG2 cells changes in aging rats, in which the area where the cells appear to be densely scattered throughout all cerebellar layers involves also more rostral and caudal lobules. In addition, in aging rats, in the most rostral and caudal lobules belonging to the spinocerebellum, punctate reaction product is present at the apical pole of Purkinje cells, i.e. in the area where the majority of synapses between olivary climbing fibers and Purkinje cells occur. Data suggest that the different distribution of NG2 cells is correlated to differences in physiology among cerebellar areas and reflects changes during aging. PMID:23027343

  11. A PET study of cerebellar metabolism in normal and abnormal states

    Energy Technology Data Exchange (ETDEWEB)

    Kushner, M.; Alavi, A.; Chawluk, J.; Silver, F.; Dann, R.; Rosen, M.; Reivich, M.

    1985-05-01

    The authors studied cerebellar metabolism under varying conditions of sensory stimulation. Cerebellar glucose consumption was measured by positron emission scanning and 18F-fluorodeoxyglucose in 64 subjects. Cerebellar metabolism relative to the whole brain (CM), and the asymmetry of metabolism between the cerebellar hemispheres (CA) was determined. The lowest CM occurred with maximal sensory deprivation, eyes and ears closed, (CM=96%, n=6). CM increased nonsignificantly with visual stimulation (CM=99%,n=17) and was highest for auditory stimulation (CM=104%,n=10,p<.05). CA was unaffected by sensory input. Under ambient conditions the CM values were 101%, 113% and 135% respectively for young controls (n=9, age=22), old controls (n=8, age=61) and Alzheimer patients (SDAT, n=14, age=69). This difference was significant for SDAT vs young and old controls and was nearly significant for young vs old controls.

  12. Changes in cerebro-cerebellar interaction during response inhibition after performance improvement.

    Science.gov (United States)

    Hirose, Satoshi; Jimura, Koji; Kunimatsu, Akira; Abe, Osamu; Ohtomo, Kuni; Miyashita, Yasushi; Konishi, Seiki

    2014-10-01

    It has been demonstrated that motor learning is supported by the cerebellum and the cerebro-cerebellar interaction. Response inhibition involves motor responses and the higher-order inhibition that controls the motor responses. In this functional MRI study, we measured the cerebro-cerebellar interaction during response inhibition in two separate days of task performance, and detected the changes in the interaction following performance improvement. Behaviorally, performance improved in the second day, compared to the first day. The psycho-physiological interaction (PPI) analysis revealed the interaction decrease from the right inferior frontal cortex (rIFC) to the cerebellum (lobule VII or VI). It was also revealed that the interaction increased from the same cerebellar region to the primary motor area. These results suggest the involvement of the cerebellum in response inhibition, and raise the possibility that the performance improvement was supported by the changes in the cerebro-cerebellar interaction.

  13. Successful Removal of a Metastatic Gastrointestinal Stromal Tumor in the Craniovertebral Junction using an Occipital Artery to Posterior Inferior Cerebellar Artery Bypass

    Directory of Open Access Journals (Sweden)

    Yukitomo Ishi

    2014-05-01

    Full Text Available Gastrointestinal stromal tumors (GISTs are rare mesenchymal neoplasms arising from the gastrointestinal tract. The authors present a case of the successful removal of a metastatic GIST in the craniovertebral junction, using an occipital artery to posterior inferior cerebellar artery (OA-PICA bypass. The patient is a 54-year-old male who underwent his first surgery for a small-bowel tumor at the age of 45 and was diagnosed with GIST. Nine years after his primary diagnosis, the patient suffered from severe neck pain. MRI demonstrated a large demarcated mass adjacent to the right atlas. The right vertebral artery (VA, completely engulfed by the tumor, showed a narrowing and ended in the PICA. Poor collateral blood supply in the right PICA territory was presumed. To prevent ischemic complications, an OA-PICA bypass was performed prior to the tumor resection. After the OA-PICA bypass, the tumor associated with the right VA was successfully removed, and the patient was discharged without any neurological deficits.

  14. Pediatric adrenocortical neoplasms: can imaging reliably discriminate adenomas from carcinomas?

    Energy Technology Data Exchange (ETDEWEB)

    Flynt, Kelsey A.; Dillman, Jonathan R.; Smith, Ethan A.; Strouse, Peter J. [University of Michigan Health System, Section of Pediatric Radiology, C. S. Mott Children' s Hospital, Department of Radiology, Ann Arbor, MI (United States); Davenport, Matthew S.; Caoili, Elaine M. [University of Michigan Health System, Division of Abdominal Imaging, Department of Radiology, Ann Arbor, MI (United States); Else, Tobias [University of Michigan Health System, Division of Metabolism, Endocrinology and Diabetes, Department of Internal Medicine, Ann Arbor, MI (United States)

    2015-08-15

    There is a paucity of literature describing and comparing the imaging features of adrenocortical adenomas and carcinomas in children and adolescents. To document the CT and MRI features of adrenocortical neoplasms in a pediatric population and to determine whether imaging findings (other than metastatic disease) can distinguish adenomas from carcinomas. We searched institutional medical records to identify pediatric patients with adrenocortical neoplasms. Pre-treatment CT and MRI examinations were reviewed by two radiologists in consensus, and pertinent imaging findings were documented. We also recorded relevant histopathological, demographic, clinical follow-up and survival data. We used the Student's t-test and Wilcoxon rank sum test to compare parametric and nonparametric continuous data, and the Fisher exact test to compare proportions. We used receiver operating characteristic (ROC) curve analyses to evaluate the diagnostic performances of tumor diameter and volume for discriminating carcinoma from adenoma. A P-value ≤0.05 was considered statistically significant. Among the adrenocortical lesions, 9 were adenomas, 15 were carcinomas, and 1 was of uncertain malignant potential. There were no differences in mean age, gender or sidedness between adenomas and carcinomas. Carcinomas were significantly larger than adenomas based on mean estimated volume (581 ml, range 16-2,101 vs. 54 ml, range 3-197 ml; P-value = 0.003; ROC area under the curve = 0.92) and mean maximum transverse plane diameter (9.9 cm, range 3.0-14.9 vs. 4.4 cm, range 1.9-8.2 cm; P-value = 0.0001; ROC area under the curve = 0.92). Carcinomas also were more heterogeneous than adenomas on post-contrast imaging (13/14 vs. 2/9; odds ratio [OR] = 45.5; P-value = 0.001). Six of 13 carcinomas and 1 of 8 adenomas contained calcification at CT (OR = 6.0; P-value = 0.17). Seven of 15 children with carcinomas exhibited metastatic disease at diagnosis, and three had inferior vena cava invasion. Median

  15. A case report of patient with cerebellar variant of stiff person syndrome.

    Science.gov (United States)

    Maludzińska, Ewa; Rudzińska, Monika; Stępień, Artur; Szczudlik, Andrzej

    2016-01-01

    Stiff person syndrome (SPS) is a rare autoimmune neurological disorder with antibodies against antigens involved in neurotransmission of gamma-aminobutyric acid (GABA). About 10% of patients with SPS may develop ataxia. This cerebellar variant is a distinct subset of SPS with more severe and complex clinical phenotype. We report the clinical, neuropsychological and neuroradiological findings in a 39-year-old female with cerebellar variant of SPS.

  16. Cerebellar Lesions of Uremic Encephalopathy on MRI in Hemodialyzed Diabetic Patient: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kil, Min Chul; Lee, Seung Young; Cha, Sang Hoon; Cho, Bum Sang; Kang, Min Ho [Dept. of Radiology, Chungbuk National Universty Hospital, Cheongju (Korea, Republic of)

    2012-01-15

    Uremic encephalopathy (UE) is a well-known complication of uremia, but its pathophysiology remains unknown. It is widely reported that in UE, the bilateral basal ganglia (BG) shows hyperintensities on T2/fluid attenuated inversion recovery magnetic resonance imaging (MRI), but cerebellar lesions are extremely rare, with to the best of our knowledge, only one case reported to date. We describe the findings from computed tomography and MRI for typical BG and cerebellar vermis lesions.

  17. Encephalitis due to antibodies to voltage gated potassium channel (VGKC with cerebellar involvement in a teenager

    Directory of Open Access Journals (Sweden)

    Megan M Langille

    2015-01-01

    Full Text Available Encephalitis due to antibodies to voltage gated potassium channel (VGKC typically presents with limbic encephalitis and medial temporal lobe involvement on neuroimaging. We describe a case of 13 year girl female with encephalitis due to antibodies to VGKC with signal changes in the cerebellar dentate nuclei bilaterally and clinical features that suggested predominant cerebellar involvement. These have never been reported previously in the literature. Our case expands the phenotypic spectrum of this rare condition.

  18. Encephalitis due to antibodies to voltage gated potassium channel (VGKC) with cerebellar involvement in a teenager.

    Science.gov (United States)

    Langille, Megan M; Desai, Jay

    2015-01-01

    Encephalitis due to antibodies to voltage gated potassium channel (VGKC) typically presents with limbic encephalitis and medial temporal lobe involvement on neuroimaging. We describe a case of 13 year girl female with encephalitis due to antibodies to VGKC with signal changes in the cerebellar dentate nuclei bilaterally and clinical features that suggested predominant cerebellar involvement. These have never been reported previously in the literature. Our case expands the phenotypic spectrum of this rare condition.

  19. A Turkish newborn infant with cerebellar agenesis/neonatal diabetes mellitus and PTF1A mutation.

    Science.gov (United States)

    Tutak, E; Satar, M; Yapicioğlu, H; Altintaş, A; Narli, N; Hergüner, O; Bayram, Y

    2009-01-01

    Classical neonatal diabetes mellitus is defined as hyperglycemia that occurs within the first month of life in term infants. It can be either permanent or transient. Cerebellar agenesis and permanent neonatal diabetes has been previously reported as a new autosomal recessive disorder. Pancreas Transcription Factor 1 Alpha (PTF1A) mutations have been related with this constellation of abnormalities. Here we report a new case of cerebellar agenesis and neonatal diabetes mellitus whose parents are PTF1A mutation carriers.

  20. Cerebellar involvement that occurred during treatment of Legionella pneumonia: A case report

    Directory of Open Access Journals (Sweden)

    Ozlem Alici

    2013-06-01

    Full Text Available Legionnaires’ disease can appear with different levels of severity. A case of a previously healthy lady with communityacquiredpneumonia who progressed to severe acute respiratory distress syndrome and developed cerebellar dysfunctionis reported. In patients presenting with neurological symptoms after an episode of pneumonia, Legionella infectionshould be considered. J Microbiol Infect Dis 2013; 3(2: 83-85Key words: Legionella, cerebellar dysfunction, dysarthria, ataxia

  1. Post-traumatic cerebellar infarction due to vertebral artery foramina fracture: case report

    OpenAIRE

    Moscote-Salazar Luis Rafael; Rubiano Andres M.; Calderon-Miranda Willem Guillermo; Agrawal Amit

    2016-01-01

    Posttraumatic cerebral infarction is an uncommon cause of morbidity and mortality and many studies have highlighted that trauma needs to considered as causative factor for cerebellar infarction. We present a case of cerebellar infarction in a 35 year old young patient secondary to vertebral fracture involving the vertebral foramen and vertebral artery injury. CT scan cervical spine showed C2-3 fracture on left side with fracture extending into the left vertebral foramen. A CT scan angiogram c...

  2. Cerebellar Development and Plasticity: Perspectives for Motor Coordination Strategies, for Motor Skills, and for Therapy

    OpenAIRE

    Swinny, J. D; van der Want, J.J.L.; Gramsbergen, A.

    2005-01-01

    The role of the mammalian cerebellum ranges from motor coordination, sensory-motor integration, motor learning, and timing to nonmotor functions such as cognition. In terms of motor function, the development of the cerebellum is of particular interest because animal studies show that the development of the cerebellar cortical circuitry closely parallels motor coordination. Ultrastructural analysis of the morphological development of the cerebellar circuitry, coupled with the temporal and spat...

  3. Intra-cerebellar infusion of the protein kinase Mzeta (PKMζ) inhibitor ZIP disrupts eyeblink classical conditioning

    Science.gov (United States)

    Chihabi, Kutibh; Morielli, Anthony D.; Green, John T.

    2016-01-01

    PKM-ζ, a constitutively active N-terminal truncated form of PKC-ζ, has long been implicated in a cellular correlate of learning, long-term potentiation (LTP). Inhibition of PKM-ζ with Zeta-inhibitory peptide (ZIP) has been shown in many brain structures to disrupt maintenance of AMPA receptors, irreversibly disrupting numerous forms of learning and memory that have been maintained for weeks. Delay eyeblink conditioning (EBC) is an established model for the assessment of cerebellar learning; here, we show that PKC-ζ and PKM-ζ are highly expressed in the cerebellar cortex, with highest expression found in Purkinje cell (PC) nuclei. Despite being highly expressed in the cerebellar cortex, no studies have examined how regulation of cerebellar PKM-ζ may affect cerebellar-dependent learning and memory. Given its disruption of learning in other brain structures, we hypothesized that ZIP would also disrupt delay EBC. We have shown that infusion of ZIP into the lobulus simplex of the rat cerebellar cortex can indeed significantly disrupt delay EBC. PMID:26949968

  4. Adaptive control of 2-wheeled balancing robot by cerebellar neuronal network model.

    Science.gov (United States)

    Tanaka, Yoshiyuki; Ohata, Yohei; Kawamoto, Tomohiro; Hirata, Yutaka

    2010-01-01

    A new adaptive motor controller was constructed, and tested on the control of a 2-wheeled balancing robot in simulation and real world. The controller consists of a feedback (PD) controller and a cerebellar neuronal network model. The structure of the cerebellar model was configured based upon known anatomical neuronal connection in the cerebellar cortex. Namely it consists of 120 granular (Gr) cells, 1 Golgi cell, 6 basket/stellate cells, and 1 Purkinje (Pk) cell. Each cell is described by a typical artificial neuron model that outputs a weighted sum of inputs after a sigmoidal nonlinear transformation. The 2 components of the proposed controller work in parallel, in a way that the cerebellar model adaptively modifies the synaptic weights between Gr and Pk as in the real cerebellum to minimize the output of the PD controller. We demonstrate that the proposed controller successfully controls a 2-wheeled balancing robot, and the cerebellar model rapidly takes over the PD controller in simulation. We also show that an abrupt load change on the robot, which the PD controller alone cannot compensate for, can be adaptively compensated by the cerebellar model. We further confirmed that the proposed controller can be applied to the control of the robot in real world.

  5. TERRA INCOGNITA - CEREBELLAR CONTRIBUTIONS TO NEUROPSYCHIATRIC AND COGNITIVE DYSFUNCTION IN BEHAVIOURAL VARIANT FRONTOTEMPORAL DEMENTIA

    Directory of Open Access Journals (Sweden)

    Rachel H Tan

    2015-07-01

    Full Text Available Although converging evidence has positioned the human cerebellum as an important relay for intact cognitive and neuropsychiatric processing, changes in this large structure remain mostly overlooked in behavioural variant frontotemporal dementia (bvFTD, a disease which is characterized by cognitive and neuropsychiatric deficits. The present study assessed whether degeneration in specific cerebellar subregions associate with indices of cognition and neuropsychiatric performance in bvFTD. Our results demonstrate a relationship between cognitive and neuropsychiatric decline across various domains of memory, language, emotion, executive, visuospatial function and motivation and the degree of grey matter degeneration in cerebellar lobules V-VII. Most notably, bilateral cerebellar lobule VII and the posterior vermis emerged as distinct for memory processes, the right cerebellar hemisphere underpinned emotion, and the posterior vermis was highlighted in language dysfunction in bvFTD. Based on cortico-cerebellar connectivity maps, these findings in the cerebellum are consistent with the neural connections with the cortices involved in these domains in patients with bvFTD. Overall, the present study underscores the significance of cortical-cerebellar networks associated with cognition and neuropsychiatric dysfunction in bvFTD.

  6. Integrated plasticity at inhibitory and excitatory synapses in the cerebellar circuit

    Directory of Open Access Journals (Sweden)

    Lisa eMapelli

    2015-05-01

    Full Text Available The way long-term potentiation (LTP and depression (LTD are integrated within the different synapses of brain neuronal circuits is poorly understood. In order to progress beyond the identification of specific molecular mechanisms, a system in which multiple forms of plasticity can be correlated with large-scale neural processing is required. In this paper we take as an example the cerebellar network , in which extensive investigations have revealed LTP and LTD at several excitatory and inhibitory synapses. Cerebellar LTP and LTD occur in all three main cerebellar subcircuits (granular layer, molecular layer, deep cerebellar nuclei and correspondingly regulate the function of their three main neurons: granule cells (GrCs, Purkinje cells (PCs and deep cerebellar nuclear (DCN cells. All these neurons, in addition to be excited, are reached by feed-forward and feed-back inhibitory connections, in which LTP and LTD may either operate synergistically or homeostatically in order to control information flow through the circuit. Although the investigation of individual synaptic plasticities in vitro is essential to prove their existence and mechanisms, it is insufficient to generate a coherent view of their impact on network functioning in vivo. Recent computational models and cell-specific genetic mutations in mice are shedding light on how plasticity at multiple excitatory and inhibitory synapses might regulate neuronal activities in the cerebellar circuit and contribute to learning and memory and behavioral control.

  7. Electron tomographic structure and protein composition of isolated rat cerebellar, hippocampal and cortical postsynaptic densities.

    Science.gov (United States)

    Farley, M M; Swulius, M T; Waxham, M N

    2015-09-24

    Electron tomography and immunogold labeling were used to analyze similarities and differences in the morphology and protein composition of postsynaptic densities (PSDs) isolated from adult rat cerebella, hippocampi, and cortices. There were similarities in physical dimensions and gross morphology between cortical, hippocampal and most cerebellar PSDs, although the morphology among cerebellar PSDs could be categorized into three distinct groups. The majority of cerebellar PSDs were composed of dense regions of protein, similar to cortical and hippocampal PSDs, while others were either composed of granular or lattice-like protein regions. Significant differences were found in protein composition and organization across PSDs from the different brain regions. The signaling protein, βCaMKII, was found to be a major component of each PSD type and was more abundant than αCaMKII in both hippocampal and cerebellar PSDs. The scaffold molecule PSD-95, a major component of cortical PSDs, was found absent in a fraction of cerebellar PSDs and when present was clustered in its distribution. In contrast, immunogold labeling for the proteasome was significantly more abundant in cerebellar and hippocampal PSDs than cortical PSDs. Together, these results indicate that PSDs exhibit remarkable diversity in their composition and morphology, presumably as a reflection of the unique functional demands placed on different synapses.

  8. Integrated plasticity at inhibitory and excitatory synapses in the cerebellar circuit.

    Science.gov (United States)

    Mapelli, Lisa; Pagani, Martina; Garrido, Jesus A; D'Angelo, Egidio

    2015-01-01

    The way long-term potentiation (LTP) and depression (LTD) are integrated within the different synapses of brain neuronal circuits is poorly understood. In order to progress beyond the identification of specific molecular mechanisms, a system in which multiple forms of plasticity can be correlated with large-scale neural processing is required. In this paper we take as an example the cerebellar network, in which extensive investigations have revealed LTP and LTD at several excitatory and inhibitory synapses. Cerebellar LTP and LTD occur in all three main cerebellar subcircuits (granular layer, molecular layer, deep cerebellar nuclei) and correspondingly regulate the function of their three main neurons: granule cells (GrCs), Purkinje cells (PCs) and deep cerebellar nuclear (DCN) cells. All these neurons, in addition to be excited, are reached by feed-forward and feed-back inhibitory connections, in which LTP and LTD may either operate synergistically or homeostatically in order to control information flow through the circuit. Although the investigation of individual synaptic plasticities in vitro is essential to prove their existence and mechanisms, it is insufficient to generate a coherent view of their impact on network functioning in vivo. Recent computational models and cell-specific genetic mutations in mice are shedding light on how plasticity at multiple excitatory and inhibitory synapses might regulate neuronal activities in the cerebellar circuit and contribute to learning and memory and behavioral control.

  9. Second Malignant Neoplasms After Treatment of Childhood Acute Lymphoblastic Leukemia

    DEFF Research Database (Denmark)

    Schmiegelow, K.; Levinsen, Mette Frandsen; Attarbaschi, Andishe

    2013-01-01

    PURPOSE: Second malignant neoplasms (SMNs) after diagnosis of childhood acute lymphoblastic leukemia (ALL) are rare events. PATIENTS AND METHODS: We analyzed data on risk factors and outcomes of 642 children with SMNs occurring after treatment for ALL from 18 collaborative study groups between 1980...... and 2007. RESULTS: Acute myeloid leukemia (AML; n = 186), myelodysplastic syndrome (MDS; n = 69), and nonmeningioma brain tumor (n = 116) were the most common types of SMNs and had the poorest outcome (5-year survival rate, 18.1% ± 2.9%, 31.1% ± 6.2%, and 18.3% ± 3.8%, respectively). Five-year survival...... estimates for AML were 11.2% ± 2.9% for 125 patients diagnosed before 2000 and 34.1% ± 6.3% for 61 patients diagnosed after 2000 (P survival estimates for MDS were 17.1% ± 6.4% (n = 36) and 48.2% ± 10.6% (n = 33; P = .005). Allogeneic stem-cell transplantation failed to improve outcome...

  10. Molecular Pathology of Hepatic Neoplasms: Classification and Clinical Significance

    Directory of Open Access Journals (Sweden)

    Zenta Walther

    2011-01-01

    Full Text Available Recent technological advances have enabled investigators to characterize the molecular genetics and genomics of hepatic neoplasia in remarkable detail. From these studies, an increasing number of molecular markers are being identified that correlate with clinically important tumor phenotypes. This paper discusses current knowledge relevant to the molecular classification of epithelial primary hepatic tumors that arise in adults, including focal nodular hyperplasia (FNH, hepatocellular adenoma (HCA, hepatocellular carcinoma (HCC, cholangiocarcinoma (CC, and combined HCC-CC. Genetic analysis has defined molecular subtypes of HCA that are clinicopathologically distinct and can be distinguished through immunohistochemistry. Gene expression studies have identified molecular signatures of progression from dysplastic nodules (DNs to early HCC in cirrhosis. Analyses of the mutational spectra, chromosomal aberrations and instability, transcriptomics, and microRNA profiles of HCC have revealed the existence of biologically distinct subtypes of this common malignancy, with prognostic implications. Molecular characterization of biliary and hepatic progenitor cell phenotypes in liver cancer has shed new light on the histogenesis of these tumors and has focused attention on novel therapeutic targets. In coming years, the molecular classification of hepatic neoplasms will be increasingly valuable for guiding patient care, as targeted therapies for liver cancer are developed and brought into clinical practice.

  11. Impact of Inflammation on Myeloproliferative Neoplasm Symptom Development.

    Science.gov (United States)

    Geyer, Holly L; Dueck, Amylou C; Scherber, Robyn M; Mesa, Ruben A

    2015-01-01

    Myeloproliferative neoplasms (essential thrombocythemia, ET; polycythemia vera, PV; myelofibrosis, MF) are monoclonal malignancies associated with genomic instability, dysregulated signaling pathways, and subsequent overproduction of inflammatory markers. Acknowledged for their debilitating symptom profiles, recent investigations have aimed to determine the identity of these markers, the upstream sources stimulating their development, their prevalence within the MPN population, and the role they play in symptom development. Creation of dedicated Patient Reported Outcome (PRO) tools, in combination with expanded access to cytokine analysis technology, has resulted in a surge of investigations evaluating the potential associations between symptoms and inflammation. Emerging data demonstrates clear relationships between individual MPN symptoms (fatigue, abdominal complaints, microvascular symptoms, and constitutional symptoms) and cytokines, particularly IL-1, IL-6, IL-8, and TNF-α. Information is also compiling on the role symptoms paradoxically play in the development of cytokines, as in the case of fatigue-driven sedentary lifestyles. In this paper, we explore the symptoms inherent to the MPN disorders and the potential role inflammation plays in their development.

  12. Impact of Inflammation on Myeloproliferative Neoplasm Symptom Development

    Directory of Open Access Journals (Sweden)

    Holly L. Geyer

    2015-01-01

    Full Text Available Myeloproliferative neoplasms (essential thrombocythemia, ET; polycythemia vera, PV; myelofibrosis, MF are monoclonal malignancies associated with genomic instability, dysregulated signaling pathways, and subsequent overproduction of inflammatory markers. Acknowledged for their debilitating symptom profiles, recent investigations have aimed to determine the identity of these markers, the upstream sources stimulating their development, their prevalence within the MPN population, and the role they play in symptom development. Creation of dedicated Patient Reported Outcome (PRO tools, in combination with expanded access to cytokine analysis technology, has resulted in a surge of investigations evaluating the potential associations between symptoms and inflammation. Emerging data demonstrates clear relationships between individual MPN symptoms (fatigue, abdominal complaints, microvascular symptoms, and constitutional symptoms and cytokines, particularly IL-1, IL-6, IL-8, and TNF-α. Information is also compiling on the role symptoms paradoxically play in the development of cytokines, as in the case of fatigue-driven sedentary lifestyles. In this paper, we explore the symptoms inherent to the MPN disorders and the potential role inflammation plays in their development.

  13. SIRT1与肿瘤%SIRT1 and neoplasms

    Institute of Scientific and Technical Information of China (English)

    郭淑芹; 朱春英; 张云良

    2011-01-01

    SIRT1 (Sirtuin type 1 ), a member of histone deacetylase, dependents on nicotinamide adenine dinucleotide ( NAD + ). It involves in the covalent modification of histones, participates in tumor development and progression through transcription, translation and post-translational modification and so on. Therefore, the expression of SIRT1 in tumor cells or abnormal function could be one of the important mechanisms of tumor development, and may become a new potential therapeutic target for neoplasms.%SIRT1 (Sirtuin type 1)是一种依赖于烟酰胺腺嘌呤二核苷酸(NAD+)的组蛋白脱乙酰酶,参与组蛋白的共价修饰,并可通过转录、翻译及翻译后修饰等多种途径参与肿瘤的发生发展过程。肿瘤细胞内SIRT1的表达或功能异常是肿瘤发生发展的重要机制之一,并可能成为治疗肿瘤的潜在靶点。

  14. Defining the Thrombotic Risk in Patients with Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Fabrizio Vianello

    2011-01-01

    Full Text Available Polycythemia vera (PV and essential thrombocythemia (ET are two Philadelphia-negative myeloproliferative neoplasms (MPN associated with an acquired mutation in the JAK2 tyrosine kinase gene. There is a rare incidence of progression to myelofibrosis and myeloid metaplasia in both disorders, which may or may not precede transformation to acute myeloid leukemia, but thrombosis is the main cause of morbidity and mortality. The pathophysiology of thrombosis in patients with MPN is complex. Traditionally, abnormalities of platelet number and function have been claimed as the main players, but increased dynamic interactions between platelets, leukocytes, and the endothelium do probably represent a fundamental interplay in generating a thrombophilic state. In addition, endothelial dysfunction, a well-known risk factor for vascular disease, may play a role in the thrombotic risk of patients with PV and ET. The identification of plasma markers translating the hemostatic imbalance in patients with PV and ET would be extremely helpful in order to define the subgroup of patients with a significant clinical risk of thrombosis.

  15. The spectrum of JAK2-positive myeloproliferative neoplasms.

    Science.gov (United States)

    Kiladjian, Jean-Jacques

    2012-01-01

    The discovery of the JAK2V617F mutation triggered an unexpected flowering of basic and clinical studies in the field of myeloproliferative neoplasms (MPNs), resulting after just a few years in an exceptional amount of new information. One important consequence of those new findings was the modification of the World Health Organization classification and diagnostic algorithms for these diseases, which is still based on the original concept developed by William Dameshek in 1951 and keeps distinct entities under the umbrella of classical Philadelphia-negative MPNs. These MPNs are essential thrombocythemia, polycythemia vera, and primary myelofibrosis. Could a new molecular classification be a better tool to manage MPN patients? Several studies have shown that essential thrombocythemia and primary myelofibrosis can be divided into distinct subtypes based on the presence of the JAK2V617F mutation. Can we now define JAK2-positive diseases to depict a distinct entity from JAK2-negative MPNs? This chapter reviews the significance of JAK2 mutation positivity in the diagnosis, prognosis, and therapy of MPNs.

  16. Pathogenesis of Myeloproliferative Neoplasms: Role and Mechanisms of Chronic Inflammation.

    Science.gov (United States)

    Hermouet, Sylvie; Bigot-Corbel, Edith; Gardie, Betty

    2015-01-01

    Myeloproliferative neoplasms (MPNs) are a heterogeneous group of clonal diseases characterized by the excessive and chronic production of mature cells from one or several of the myeloid lineages. Recent advances in the biology of MPNs have greatly facilitated their molecular diagnosis since most patients present with mutation(s) in the JAK2, MPL, or CALR genes. Yet the roles played by these mutations in the pathogenesis and main complications of the different subtypes of MPNs are not fully elucidated. Importantly, chronic inflammation has long been associated with MPN disease and some of the symptoms and complications can be linked to inflammation. Moreover, the JAK inhibitor clinical trials showed that the reduction of symptoms linked to inflammation was beneficial to patients even in the absence of significant decrease in the JAK2-V617F mutant load. These observations suggested that part of the inflammation observed in patients with JAK2-mutated MPNs may not be the consequence of JAK2 mutation. The aim of this paper is to review the different aspects of inflammation in MPNs, the molecular mechanisms involved, the role of specific genetic defects, and the evidence that increased production of certain cytokines depends or not on MPN-associated mutations, and to discuss possible nongenetic causes of inflammation.

  17. Helicobacter pylori in colorectal neoplasms: is there an aetiological relationship?

    Directory of Open Access Journals (Sweden)

    Tharakan Joseph

    2007-05-01

    Full Text Available Abstract Background This pilot study was carried out to determine whether Helicobacter pylori can be detected in normal colon or in association with colorectal neoplasia. Methods Paraffin processed colonic tissue blocks of normal colonic mucosa (n = 60, and patients diagnosed as adenoma (n = 60, and adenocarcinoma (n = 60 were retrieved from our archive; the adenoma group included tubular (n = 20, tubulovillous (n = 20 and villous adenomas (n = 20. 4 μm sections were stained by immunohistochemical methods using anti-Helicobacter pylori antibodies (polyclonal NCL-HPp and monoclonal NCL-C-jejuni. Results Significant numbers of Helicobacter pylori were identified in tubular adenomas (OR = 11.13; 95%CI = 1.62–76.70, tubulovillous adenomas (OR = 10.45; 95%CI = 1.52–71.52 and adenocarcinomas (OR = 8.13; 95%CI = 1.40–46.99 compared to controls: there was no association in numbers of Helicobacter pylori and villous adenomas (OR = 2.95; 95%CI = 0.29–9.96. Conclusion We conclude that although, in this pilot study, there appears to be an association in the prevalence of Helicobacter pylori with some, but not all, colorectal neoplasms, we can not infer causality from these results. These findings need to be further substantiated with a prospective study and the use of molecular biological techniques to determine a causal association.

  18. Microvessel and mast cell densities in malignant laryngeal neoplasm

    Directory of Open Access Journals (Sweden)

    Balica Nicolae Constantin

    2014-01-01

    Full Text Available Laryngeal neoplasm contributes to 30-40% of carcinomas of the head and neck. Mast cells are normal connective tissue residents, well represented in the respiratory tract. Experimental evidence suggests that the growth of a tumor beyond a certain size requires angiogenesis, which may also permit metastasis. The aim of this study was to evaluate the correlation between mast cell density, microvascular density, histopathological type and histological grade. Our study included 38 laryngeal carcinomas as follows: adenoid cystic carcinoma (2 cases, malignant papilloma (2 cases and squamous cell carcinoma (34 cases. The combined technique of CD 34-alcian blue safranin (ABS was used to identify microvessel and mast cell density, which was quantified by the hot spot method. A significant correlation was found between both mast cell and microvascular density, and G1/G2 histological grade (p=0.002 and p=0.004, respectively. Squamous cell carcinoma was significantly correlated with mast cell density (p=0.003, but not with microvascular density (p=0.454.

  19. Diagnostic and therapeutic endoscopic approaches to intraductal papillary mucinous neoplasm.

    Science.gov (United States)

    Turner, Brian G; Brugge, William R

    2010-10-27

    Pancreatic cystic lesions are increasingly identified on routine imaging. One specific lesion, known as intraductal papillary mucinous neoplasm (IPMN), is a mucinous, pancreatic lesion characterized by papillary cells projecting from the pancreatic ductal epithelium. The finding of mucin extruding from the ampulla is essentially pathognomonic for diagnosing these lesions. IPMNs are of particular interest due to their malignant potential. Lesions range from benign, adenomatous growths to high-grade dysplasia and invasive cancer. These mucinous lesions therefore require immediate attention to determine the probability of malignancy and whether observation or resection is the best management choice. Unresected lesions need long-term surveillance monitoring for malignant transformation. The accurate diagnosis of these lesions is particularly challenging due to the substantial similarities in morphology of pancreatic cystic lesions and limitations in current imaging technologies. Endoscopic evaluation of these lesions provides additional imaging, molecular, and histologic data to aid in the identification of IPMN and to determine treatment course. The aim of this article is to focus on the diagnostic and therapeutic endoscopic approaches to IPMN.

  20. Intraductal Papillary Mucinous Neoplasm of the Pancreas: An Update

    Directory of Open Access Journals (Sweden)

    Shu-Yuan Xiao

    2012-01-01

    Full Text Available Intraductal papillary mucinous neoplasm (IPMN is a cystic tumor of the pancreas. The etiology is unknown, but increasing evidence suggests the involvement of several tumorigenesis pathways, including an association with hereditary syndromes. IPMN occurs more commonly in men, with the mean age at diagnosis between 64 and 67 years old. At the time of diagnosis, it may be benign, with or without dysplasia, or frankly malignant with an invasive carcinoma. Tumors arising from the main pancreatic duct are termed main-duct IPMNs, those involving the branch ducts, branch-duct IPMNs. In general, small branch-duct IPMNs are benign, particularly in asymptomatic patients, and can be safely followed. In contrast, main-duct tumors should be surgically resected and examined carefully for an invasive component. In the absence of invasion, patient’s survival is excellent, from 94 to 100%. For patients with an IPMN-associated invasive carcinoma, the prognosis overall is better than those with a de novo pancreatic ductal adenocarcinoma, with a 5-year survival of 40% to 60% in some series. However, no survival advantage can be demonstrated if the invasive component in an IPMN patient is that of the conventional tubular type (versus mucinous carcinoma. Several histomorphologic variants are recognized, although the clinical significance of this “subtyping” is not well defined.