• paraneoplastic cerebellar degeneration, and alcoholic or nutritional cerebellar degeneration. (wikipedia.org)
  • Approximately 50% of all patients experience dementia as a result of paraneoplastic cerebellar degeneration. (wikipedia.org)
  • Paraneoplastic neurologic syndromes include a variety of neurologic disorders, such as paraneoplastic cerebellar degeneration (PCD), that are caused by an immune-mediated mechanism rather than a metastatic complication or medication effect. (medscape.com)
  • Paraneoplastic cerebellar degeneration is a rare nonmetastatic complication of a carcinoma, typically mediated by antibodies generated against tumor antigens (proteins). (medscape.com)
  • An association between paraneoplastic cerebellar degeneration and occult gynecologic cancers (breast or ovarian) was first identified in 1938, and the syndrome was described fully by Brain in 1951. (medscape.com)
  • [ 2 ] Posner found that patients with paraneoplastic cerebellar degeneration can be classified according to the presence or absence of an antibody that reacted with an antigen present in both the tumors and in cerebellar Purkinje neurons obtained from these patients. (medscape.com)
  • The image below illustrates the workup of paraneoplastic cerebellar degeneration. (medscape.com)
  • Paraneoplastic cerebellar degeneration (PCD) is caused by the secondary effects of cancer and is believed to be immune mediated. (medscape.com)
  • Not all patients presenting with paraneoplastic cerebellar degeneration and its clinical features have recognizable antineuronal antibodies. (medscape.com)
  • Paraneoplastic cerebellar degeneration (PCD) is a rare, severely debilitating disease, often with a rapid onset and progression, which predate the diagnosis of malignancy. (soton.ac.uk)
  • Paraneoplastic Cerebellar Degeneration" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (childrensmercy.org)
  • This graph shows the total number of publications written about "Paraneoplastic Cerebellar Degeneration" by people in this website by year, and whether "Paraneoplastic Cerebellar Degeneration" was a major or minor topic of these publications. (childrensmercy.org)
  • Below are the most recent publications written about "Paraneoplastic Cerebellar Degeneration" by people in Profiles. (childrensmercy.org)
  • Paraneoplastic neurologic syndromes include many neurologic disorders including paraneoplastic cerebellar degeneration (PCD) caused by an immune-mediated mechanism other than a metastatic complication in patients with an underlying malignancy. (medscape.com)
  • Paraneoplastic cerebellar degeneration is a rare neurological manifestation of nonmetastatic malignancy. (e-rvs.org)
  • There have been only a few cases of paraneoplastic cerebellar degeneration with acute presentation. (e-rvs.org)
  • This study describes a patient with paraneoplastic cerebellar degeneration, who presented acute vestibular syndrome and then episodically developed horizontal gaze-evoked nystagmus and gait ataxia. (e-rvs.org)
  • Paraneoplastic cerebellar degeneration refers to a rare neurological manifestation of nonmetastatic malignancy. (e-rvs.org)
  • This study aims to describe a patient with paraneoplastic cerebellar degeneration, who initially presented as acute vertigo mimicking unilateral vestibulopathy. (e-rvs.org)
  • Restricted IgG1 subclass of anti-Yo antibodies in paraneoplastic cerebellar degeneration. (ox.ac.uk)
  • Paraneoplastic cerebellar degeneration (PCD) occurs as a non-metastatic manifestation of cancer in a small proportion of patients with certain breast or gynaecological tumours, and is characterised by widespread Purkinje cell loss. (ox.ac.uk)
  • 1 , 2 Furthermore, better understanding of the immune aetiology for some of the acquired ataxias (eg, paraneoplastic cerebellar degeneration, gluten ataxia, anti-GAD ataxia, primary autoimmune cerebellar ataxia) 3-5 meant that the overall diagnostic yield of investigating sporadic ataxias has improved considerably in recent years. (bmj.com)
  • Subacute onset of ataxia is usually seen in paraneoplastic cerebellar degeneration. (bcm.edu)
  • Herdlevær I, Kråkenes T, Schubert N, Vedeler CA. Localization of CDR2L and CDR2 in paraneoplastic cerebellar degeneration. (uib.no)
  • More specifically, the neurological diseases that can cause cerebellar degeneration include: Spinocerebellar ataxia (SCA), which refers to a group of conditions caused by mutations in the genes of a human, and are characterised by degenerative changes to many parts of the central nervous system, inclusive of the cerebellum, brain stem, and spinal cord. (wikipedia.org)
  • Spongy degeneration with cerebellar ataxia (SDCA) is a neurodegenerative disease caused by two known mutations in the KCNJ10 (SDCA1) and ATP1B2 (SDCA2) gene. (labogen.com)
  • Autoimmunity affects the cerebellum, leading to the manifestations of the cerebellar ataxias (CAs), termed immune-mediated cerebellar ataxia (IMCAs). (springer.com)
  • We describe the causes of ataxia in 1500 patients with cerebellar ataxia. (bmj.com)
  • Ataxia is the term used to describe symptoms and signs resulting from cerebellar dysfunction, manifesting with a combination of gait instability, limb incoordination, slurred speech and nystagmus. (bmj.com)
  • Damage or degeneration in the back part of the brain called the cerebellum results in ataxia. (bcm.edu)
  • condition known as cerebellar ataxia (see below). (medfriendly.com)
  • they are not likely to move in a fast and uncontrolled manner as can happen in cerebellar ataxia. (medfriendly.com)
  • All of the syndromes under the NA umbrella are distinguished from the Bassen-Kornzweig syndrome, an autosomal recessive disorder of childhood in which abetalipoproteinemia and acanthocytosis occur along with steatorrhea, retinitis pigmentosa, and cerebellar ataxia. (medscape.com)
  • Cerebellar degeneration is a condition in which cerebellar cells, otherwise known as neurons, become damaged and progressively weaken in the cerebellum. (wikipedia.org)
  • As the cerebellum contributes to the coordination and regulation of motor activities, as well as controlling equilibrium of the human body, any degeneration to this part of the organ can be life-threatening. (wikipedia.org)
  • Patients with cerebellar degeneration experience a progressive loss of nerve cells (Purkinje cells) throughout the cerebellum. (wikipedia.org)
  • GAD65 is involved in the synthesis, packaging, and release of GABA, whereas the other three play important roles in the induction of long-term depression (LTD). Thus, the auto-antibodies toward these synaptic molecules likely impair fundamental synaptic machineries involved in unique functions of the cerebellum, potentially leading to the development of cerebellar ataxias (CAs). (springer.com)
  • The syndrome is characterized by the degeneration of a part of your brain called the cerebellum . (healthline.com)
  • They result from the degeneration of pathways between the spinal cord and cerebellum. (bcm.edu)
  • The participants with cerebellar degeneration, however, could implement the strategy without interference from the "dumb cerebellum. (berkeley.edu)
  • Using a neuropsychological approach, we report that degeneration of two subcortical structures, the cerebellum and basal ganglia, impairs performance in symbolic arithmetic. (jneurosci.org)
  • Overview of Movement and Cerebellar Disorders Voluntary movement requires complex interaction of the corticospinal (pyramidal) tracts, basal ganglia, and cerebellum (the center for motor coordination) to ensure smooth, purposeful movement. (msdmanuals.com)
  • Cerebellar hypoplasia-tapetoretinal degeneration syndrome is a rare syndrome with a cerebellar malformation as a major feature characterized by cerebellar hypoplasia bilateral retinal pigmentary changes intellectual disability that can range from mild to moderate and pronounced language development delay. (globalgenes.org)
  • Newly diagnosed with Cerebellar hypoplasia-tapetoretinal degeneration syndrome? (globalgenes.org)
  • Acanthocytosis has also been associated with the rare hypobetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP) syndrome, a disease of childhood akin to Hallervorden-Spatz disease and a defect in the gene for pantothenate kinase. (medscape.com)
  • We identify distinct computational impairments in male and female participants with cerebellar degeneration (CD) or Parkinson's disease (PD). (jneurosci.org)
  • Participants with Parkinson's disease showed a greater impairment as the number of operations increased, and participants with cerebellar degeneration showed a greater impairment as the magnitude of the operations increased. (jneurosci.org)
  • The presence of auto-antibodies that target synaptic machinery proteins was documented recently in immune-mediated cerebellar ataxias. (springer.com)
  • Background Cerebellar ataxias are the result of diverse disease processes that can be genetic or acquired. (bmj.com)
  • This immune activation in the central nervous system (CNS) results in cerebellar injury and dysfunction. (medscape.com)
  • Although any paraneoplastic antibody may cause PCD, only anti-Yo, anti-Tr, and antimetabotropic glutamate receptor 1 (mGluR1) have been shown to have specific association with isolated cerebellar dysfunction. (medscape.com)
  • The puppies with cerebellar dysfunction have early onset of clinical signs (5-8 weeks of age). (labogen.com)
  • Thus, these findings point towards an involvement of cerebellar dysfunction in ASD symptoms. (nature.com)
  • Histopathologic findings included marked loss of Purkinje neurons with thinning of the molecular and granular layers and increased cellularity of the cerebellar nuclei. (nih.gov)
  • Using an immunofluorescent technique, we found the patient's serum to have antibodies to cerebellar Purkinje cells. (jamanetwork.com)
  • Cerebellar degeneration can result in disorders in fine movement, posture, and motor learning in humans, due to a disturbance of the vestibular system. (wikipedia.org)
  • The root cause of incurring a cerebellar degenerative condition can be due to a range of different inherited or acquired (non-genetic and non-inherited) conditions, including neurological diseases, paraneoplastic disorders, nutritional deficiency, and chronic heavy alcohol use. (wikipedia.org)
  • Treatment of cerebellar disorders depends on the cause. (medlineplus.gov)
  • Biallelic MED27 variants lead to variable ponto-cerebello-lental degeneration with movement disorders. (medscape.com)
  • Few cerebellar studies have examined changes in synaptic organization and function, although it has been recognized that synaptopathies play a central role in developmental brain disorders, including autism 19 . (nature.com)
  • Distal axonopathy" is a term recently introduced to describe those diseases which are expressed as symmetrical, distal, axonal degeneration occurring concurrently in the peripheral nervous system (PNS) and in selected tracts of the central nervous system (CNS). (cdc.gov)
  • HALLETT, M. Procedural learning in Parkinson's disease and cerebellar degeneration. (bvsalud.org)
  • A 21-year-old woman with subacute cerebellar degeneration was found to have Hodgkin lymphoma. (jamanetwork.com)
  • Adult-onset cerebellar cortical degeneration recently has been reported in American Staffordshire Terriers. (nih.gov)
  • Pathologically the cerebellar cortex and subcortical nuclei demonstrate diffuse degenerative changes. (childrensmercy.org)
  • Lesions of lobule HVI of the cerebellar cortex or of its target in the anterior cerebellar nuclei disrupt the acquisition and retention of conditioned nictitating membrane responses (CRs). (bl.uk)
  • Some have argued that the primary site of learning and memory is in the cerebellar nuclei. (bl.uk)
  • Aspiration lesions of the cerebellar cortex cause retrograde degeneration of precerebellar nuclei. (bl.uk)
  • Since the loss of CRs from lesions of the cerebellar cortex might be due to the loss of collateral inputs to the cerebellar nuclei from the precerebellar nuclei, fibre sparing lesions were made which destroyed only cells in the cerebellar cortex. (bl.uk)
  • CRs were lost, demonstrating that the cerebellar cortex is required for retention of CRs and that the cerebellar nuclei on their own cannot maintain CRs. (bl.uk)
  • Fibre sparing lesions of the anterior cerebellar nuclei prevented the acquisition and abolished retention of CRs. (bl.uk)
  • Notably, we discuss how the counting and chaining operations relate to cerebellar and basal ganglia function in other task domains (e.g., motor processes). (jneurosci.org)
  • Degeneration of the basal ganglia is a consistent feature of this disorder. (medscape.com)
  • More commonly, cerebellar degeneration can also be classified according to conditions that an individual may acquire during their lifetime, including infectious, metabolic, autoimmune, paraneoplastic, nutritional or toxic triggers. (wikipedia.org)
  • Despite their immune diversity, the majority of IMCAs is commonly associated with auto-antibodies against cerebellar autoantigens. (springer.com)
  • Patients with cerebellar degeneration or lesions show mild deficits on a range of neuropsychology tests, especially those associated working memory and verbal fluency. (berkeley.edu)
  • Patients with cerebellar degeneration show poor adaptation," Ivry said. (berkeley.edu)
  • The onset of symptoms of cerebellar degeneration indicates the presence of an occult malignancy. (medscape.com)
  • The clinical pattern of cerebellar involvement, rapidity of onset, pace of progression, additional clinical features in combination with appropriate investigations are essential in reaching a diagnosis. (bmj.com)
  • Here we report alterations in motor behaviour and cerebellar synaptic plasticity in a mouse model (patDp/+) for the human 15q11-13 duplication, one of the most frequently observed genetic aberrations in autism. (nature.com)
  • To examine which cerebellar synaptic alterations might contribute to motor deficits in autism, we studied cerebellar synaptic function and plasticity as well as motor coordination and learning in a mouse model for the human 15q11-13 duplication. (nature.com)
  • The non-CTE cases included Alzheimer's disease (AD), Parkinson-dementia complex of Guam, progressive supranuclear palsy, corticobasal degeneration, primary age-related tauopathy and argyrophilic grain disease. (nih.gov)
  • This condition may not only cause cerebellar damage on a temporary or permanent basis, but can also affect other tissues of the central nervous system, those including the cerebral cortex, spinal cord and the brainstem (made up of the medulla oblongata, midbrain, and pons). (wikipedia.org)
  • This presentation will summarize our clinical and pathological studies of the nervous system degeneration (distal dying-back axonopathy) in man and experimental animals produced by acrylamide monomer and certain hydrocarbon compounds (hexacarbons). (cdc.gov)
  • Brock, S. , Ellison, D. , Frankel, J. , Davis, C. and Illidge, T. (2001) Anti-Yo antibody-positive cerebellar degeneration associated with endometrial carcinoma: case report and review of the literature. (soton.ac.uk)
  • A common feature of autism spectrum disorder (ASD) is the impairment of motor control and learning, occurring in a majority of children with autism, consistent with perturbation in cerebellar function. (nature.com)
  • In agreement with these morphological abnormalities, differences in functional connectivity and activation of cerebellar circuits have been observed using functional magnetic resonance imaging 17 . (nature.com)
  • Despite the abundant evidence for cerebellar involvement in ASD, it remains unclear which changes in cerebellar function have a significant behavioural impact. (nature.com)
  • Muscle biopsy showed myopathic changes with vacuolar degeneration and marked adipose tissue proliferation. (nih.gov)
  • Cerebellar degeneration can be attributed to a plethora of hereditary and non-hereditary conditions. (wikipedia.org)
  • How and where the degeneration began in the brain was never clear. (nih.gov)