Mechanisms underlying cerebellar motor deficits due to mGluR1-autoantibodies.

Abstract:

:Patients with Hodgkin's disease can develop paraneoplastic cerebellar ataxia because of the generation of autoantibodies against mGluR1 (mGluR1-Abs). Yet, the pathophysiological mechanisms underlying their motor coordination deficits remain to be elucidated. Here, we show that application of IgG purified from the patients' serum to cerebellar slices of mice acutely reduces the basal activity of Purkinje cells, whereas application to the flocculus of mice in vivo evokes acute disturbances in the performance of their compensatory eye movements. In addition, the mGluR1-Abs block induction of long-term depression in cultured mouse Purkinje cells, whereas the cerebellar motor learning behavior of the patients is affected in that they show impaired adaptation of their saccadic eye movements. Finally, postmortem analysis of the cerebellum of a paraneoplastic cerebellar ataxia patient showed that the number of Purkinje cells was significantly reduced by approximately two thirds compared with three controls. We conclude that autoantibodies against mGluR1 can cause cerebellar motor coordination deficits caused by a combination of rapid effects on both acute and plastic responses of Purkinje cells and chronic degenerative effects.

journal_name

Ann Neurol

journal_title

Annals of neurology

authors

Coesmans M,Smitt PA,Linden DJ,Shigemoto R,Hirano T,Yamakawa Y,van Alphen AM,Luo C,van der Geest JN,Kros JM,Gaillard CA,Frens MA,de Zeeuw CI

doi

10.1002/ana.10451

keywords:

subject

Has Abstract

pub_date

2003-03-01 00:00:00

pages

325-36

issue

3

eissn

0364-5134

issn

1531-8249

journal_volume

53

pub_type

杂志文章
  • Head injury, α-synuclein Rep1, and Parkinson's disease.

    abstract:OBJECTIVE:To test the hypothesis that variability in SNCA Rep1, a polymorphic dinucleotide microsatellite in the promoter region of the gene encoding α-synuclein, modifies the association between head injury and Parkinson's disease (PD) risk. METHODS:Participants in the Farming and Movement Evaluation (FAME) and the S...

    journal_title:Annals of neurology

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1002/ana.22499

    authors: Goldman SM,Kamel F,Ross GW,Jewell SA,Bhudhikanok GS,Umbach D,Marras C,Hauser RA,Jankovic J,Factor SA,Bressman S,Lyons KE,Meng C,Korell M,Roucoux DF,Hoppin JA,Sandler DP,Langston JW,Tanner CM

    更新日期:2012-01-01 00:00:00

  • Corneal confocal microscopy: Neurologic disease biomarker in Friedreich ataxia.

    abstract:OBJECTIVE:Friedreich ataxia (FRDA), an autosomal recessive neurodegenerative disease caused by mutations in the gene encoding for the mitochondrial protein frataxin, is characterized by ataxia and gait instability, immobility, and eventual death. We evaluated corneal confocal microscopy (CCM) quantification of corneal ...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.25355

    authors: Pagovich OE,Vo ML,Zhao ZZ,Petropoulos IN,Yuan M,Lertsuwanroj B,Ciralsky J,Lai E,Kiss S,D'Amico DJ,Mezey JG,Malik RA,Crystal RG

    更新日期:2018-12-01 00:00:00

  • Febrile seizures impair memory and cAMP response-element binding protein activation.

    abstract::The long-term effects of brief but repetitive febrile seizures (FS) on memory have not been as thoroughly investigated as the impact of single and prolonged seizure in the developing brain. Using a heated-air FS paradigm, we subjected male rat pups to one, three, or nine episodes of brief FS on days 10 to 12 postpartu...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.10789

    authors: Chang YC,Huang AM,Kuo YM,Wang ST,Chang YY,Huang CC

    更新日期:2003-12-01 00:00:00

  • Demonstration of acute ischemic lesions in the fetal brain by diffusion magnetic resonance imaging.

    abstract::The possibility of detecting acute hypoxic-ischemic brain lesions by prenatal magnetic resonance imaging or ultrasound is low. We present a case of a fetus with a vein of Galen arteriovenous malformation in whom prenatal diffusion-weighted magnetic resonance imaging at 33 weeks of gestation clearly detected cerebral a...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.10255

    authors: Baldoli C,Righini A,Parazzini C,Scotti G,Triulzi F

    更新日期:2002-08-01 00:00:00

  • Sensory nerve pathology in multifocal motor neuropathy.

    abstract::The nosological status of multifocal motor neuropathy remains controversial. The clinical and electrodiagnostic hallmarks suggest selective motor fiber involvement. In this study, we asked to what extent sensory nerves might be involved pathologically in multifocal motor neuropathy. Examination of sensory nerve biopsy...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410390308

    authors: Corse AM,Chaudhry V,Crawford TO,Cornblath DR,Kuncl RW,Griffin JW

    更新日期:1996-03-01 00:00:00

  • Phase 2 trial of a DNA vaccine encoding myelin basic protein for multiple sclerosis.

    abstract:OBJECTIVE:To evaluate the efficacy and safety of BHT-3009 in relapsing-remitting multiple sclerosis (MS) and to confirm that BHT-3009 causes immune tolerance. METHODS:BHT-3009 is a tolerizing DNA vaccine for MS, encoding full-length human myelin basic protein. Relapsing-remitting MS patients were randomized 1:1:1 into...

    journal_title:Annals of neurology

    pub_type: 杂志文章,随机对照试验

    doi:10.1002/ana.21370

    authors: Garren H,Robinson WH,Krasulová E,Havrdová E,Nadj C,Selmaj K,Losy J,Nadj I,Radue EW,Kidd BA,Gianettoni J,Tersini K,Utz PJ,Valone F,Steinman L,BHT-3009 Study Group.

    更新日期:2008-05-01 00:00:00

  • Diffuse axonal injury in children: clinical correlation with hemorrhagic lesions.

    abstract::An inception cohort of 40 children and adolescents with traumatic brain injury and suspected diffuse axonal injury were studied using a new high-resolution magnetic resonance imaging susceptibility-weighted technique that is very sensitive for hemorrhage. A blinded comparison was performed between the extent of parenc...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.20123

    authors: Tong KA,Ashwal S,Holshouser BA,Nickerson JP,Wall CJ,Shutter LA,Osterdock RJ,Haacke EM,Kido D

    更新日期:2004-07-01 00:00:00

  • Socioeconomic Status Mediates Racial Differences Seen Using the AT(N) Framework.

    abstract:OBJECTIVES:African Americans are at greater risk for developing Alzheimer's disease (AD) dementia than non-Hispanic whites. In addition to biological considerations (eg, genetic influences and comorbid disorders), social and environmental factors may increase the risk of AD dementia. This paper (1) assesses neuroimagin...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.25948

    authors: Meeker KL,Wisch JK,Hudson D,Coble D,Xiong C,Babulal GM,Gordon BA,Schindler SE,Cruchaga C,Flores S,Dincer A,Benzinger TL,Morris JC,Ances BM

    更新日期:2021-02-01 00:00:00

  • Impaired neuronal operation through aberrant intrinsic plasticity in epilepsy.

    abstract:OBJECTIVE:Patients with temporal lobe epilepsy often display cognitive comorbidity with recurrent seizures. However, the cellular mechanisms underlying the impairment of neuronal information processing remain poorly understood in temporal lobe epilepsy. Within the hippocampal formation neuronal networks undergo major r...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.24348

    authors: Artinian J,Peret A,Mircheva Y,Marti G,Crépel V

    更新日期:2015-04-01 00:00:00

  • Phenotypic heterogeneity in families with the myoclonic epilepsy and ragged-red fiber disease point mutation in mitochondrial DNA.

    abstract::Two families with a point mutation in mtDNA associated with myoclonic epilepsy and ragged-red fiber disease showed pronounced clinical heterogeneity. The mothers of the two families had adult-onset myopathy with ragged-red fibers, partial deficiency of cytochrome c oxidase, and sensory neuropathy. Members of the first...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410330613

    authors: Graf WD,Sumi SM,Copass MK,Ojemann LM,Longstreth WT Jr,Shanske S,Lombes A,DiMauro S

    更新日期:1993-06-01 00:00:00

  • Retinal nerve fiber layer axonal loss and visual dysfunction in optic neuritis.

    abstract::Axonal loss is thought to be a likely cause of persistent disability after a multiple sclerosis relapse; therefore, noninvasive in vivo markers specific for axonal loss are needed. We used optic neuritis as a model of multiple sclerosis relapse to quantify axonal loss of the retinal nerve fiber layer (RNFL) and second...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.20575

    authors: Trip SA,Schlottmann PG,Jones SJ,Altmann DR,Garway-Heath DF,Thompson AJ,Plant GT,Miller DH

    更新日期:2005-09-01 00:00:00

  • Anticerebellar antibodies in serum and cerebrospinal fluid of a patient with oat cell carcinoma of the lung and paraneoplastic cerebellar degeneration.

    abstract::A 56-year-old man was seen with subacute cerebellar degeneration and was found to have oat cell carcinoma of the lung. Antibodies to cerebellar Purkinje cells and granule cells were detected in both serum and cerebrospinal fluid (CSF), and intrathecal antibody synthesis was suggested by serum CSF antibody ratios, CSF ...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410190117

    authors: Greenlee JE,Lipton HL

    更新日期:1986-01-01 00:00:00

  • Time modulation of spike-and-wave activity in generalized epilepsy.

    abstract::Nineteen patients aged 4 to 44 years with generalized epilepsy and generalized 3 Hz spike-and-wave activity, were monitored for 24 or 36 hours on one to nine occasions to determine the time distribution of ictal and interictal electroencephalographic discharges and the consistency of the time patterns observed. Two pa...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410080506

    authors: Kellaway P,Frost JD Jr,Crawley JW

    更新日期:1980-11-01 00:00:00

  • Thalamic degeneration in X-chromosome--linked copper malabsorption.

    abstract::Thalamic degeneration was present in 5 autopsied cases of X-chromosome-linked copper malabsorption (X-cLCM), Menkes' kinky hair disease. Among the thalamic nuclei, those in the formatio paraventricularis, intralamellaris, and extralamellaris were spared. The nuclei projecting to the granular cortices had severe neuron...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410050409

    authors: Iwata M,Hirano A,French JH

    更新日期:1979-04-01 00:00:00

  • Point-of-care reversal treatment in phenprocoumon-related intracerebral hemorrhage.

    abstract:OBJECTIVE:Rapid reversal of the anticoagulatory effect of vitamin K antagonists represents the primary emergency treatment for oral anticoagulant-related intracerebral hemorrhage (OAC-ICH). Predicting the amount of prothrombin complex concentrate (PCC) needed to reverse OAC in individual patients is difficult, and repe...

    journal_title:Annals of neurology

    pub_type: 临床试验,杂志文章

    doi:10.1002/ana.21965

    authors: Rizos T,Jenetzky E,Herweh C,Hug A,Hacke W,Steiner T,Veltkamp R

    更新日期:2010-06-01 00:00:00

  • Alternating skew on lateral gaze (bilateral abducting hypertropia).

    abstract::We report thirty-three patients with alternating skew deviation on lateral gaze. The right eye was hypertropic in right gaze, and the left eye was hypertropic in left gaze. Most patients had associated downbeat nystagmus and ataxia and were diagnosed as having lesions of the cerebellar pathways or the cervicomedullary...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410230213

    authors: Moster ML,Schatz NJ,Savino PJ,Benes S,Bosley TM,Sergott RC

    更新日期:1988-02-01 00:00:00

  • Stimulus rate determines regional brain blood flow in striate cortex.

    abstract::Intravenous bolus administration of oxygen 15-labeled water and positron emission tomography were used to measure changes in brain blood flow induced by two modes of photic stimuli over a wide range of repetition rates. These stimuli (patterned-flash and reversing checkerboard) were chosen in order to determine whethe...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410170315

    authors: Fox PT,Raichle ME

    更新日期:1985-03-01 00:00:00

  • Revised estimate of the prevalence of multiple sclerosis in the United States.

    abstract::Using three adjustments, we have revised a 1976 prevalence count for multiple sclerosis in the United States. The adjustments were made to data from a US national survey; they used 1990 population projections from the US Bureau of the Census, and results of investigations conducted in Weld and Larimer Countries, Color...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410310317

    authors: Anderson DW,Ellenberg JH,Leventhal CM,Reingold SC,Rodriguez M,Silberberg DH

    更新日期:1992-03-01 00:00:00

  • Familial episodic ataxia: clinical heterogeneity in four families linked to chromosome 19p.

    abstract::We describe the clinical and oculographic findings in 4 families with episodic ataxia and interictal nystagmus (EA-2) linked to chromosome 19p. Episodes varied from pure ataxia to combinations of symptoms suggesting involvement of the cerebellum, brainstem, and cortex. Some affected individuals exhibited a progressive...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410410105

    authors: Baloh RW,Yue Q,Furman JM,Nelson SF

    更新日期:1997-01-01 00:00:00

  • B-cell Therapy for Multiple Sclerosis: Entering an era.

    abstract::Monoclonal antibodies that target CD20 expressing B cells represent an important new treatment option for patients with multiple sclerosis (MS). B-cell-depleting therapy is highly effective against relapsing forms of the disease and is also the first treatment approach proven to protect against disability worsening in...

    journal_title:Annals of neurology

    pub_type: 杂志文章,评审

    doi:10.1002/ana.25119

    authors: Greenfield AL,Hauser SL

    更新日期:2018-01-01 00:00:00

  • Outcomes among patients discharged from the emergency department with a diagnosis of peripheral vertigo.

    abstract:OBJECTIVE:We aimed to determine the risk of short- and long-term stroke, as well as accidental injury, in patients discharged from an emergency department who were given a diagnosis of a peripheral vestibular disorder. METHODS:In this population-based, retrospective, cohort study, we identified all adult patients who ...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.24521

    authors: Atzema CL,Grewal K,Lu H,Kapral MK,Kulkarni G,Austin PC

    更新日期:2016-01-01 00:00:00

  • Amyloid imaging in mild cognitive impairment subtypes.

    abstract:OBJECTIVE:We utilized the amyloid imaging ligand Pittsburgh Compound B (PiB) to determine the presence of Alzheimer's disease (AD) pathology in different mild cognitive impairment (MCI) subtypes and to relate increased PiB binding to other markers of early AD and longitudinal outcome. METHODS:Twenty-six patients with ...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.21598

    authors: Wolk DA,Price JC,Saxton JA,Snitz BE,James JA,Lopez OL,Aizenstein HJ,Cohen AD,Weissfeld LA,Mathis CA,Klunk WE,De-Kosky ST

    更新日期:2009-05-01 00:00:00

  • Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathy.

    abstract::We analyzed the prion protein gene (PRNP) region in patients with transmissible spongiform encephalopathy associated with the PRNP D178N mutation. The results suggest that the D178N chromosomes had independent origins in each affected pedigree or apparently sporadic case. A de novo spontaneous PRNP mutation was observ...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.10267

    authors: Dagvadorj A,Petersen RB,Lee HS,Cervenakova L,Shatunov A,Budka H,Brown P,Gambetti P,Goldfarb LG

    更新日期:2002-09-01 00:00:00

  • Randomized trials of dichlorphenamide in the periodic paralyses. Working Group on Periodic Paralysis.

    abstract::Although the carbonic anhydrase inhibitors have been used in the treatment of the primary periodic paralyses (PPs), their efficacy has not been demonstrated in double-blind, placebo-controlled trials. Therefore, we tested the efficacy of dichlorphenamide (DCP; Daranide), a potent carbonic anhydrase inhibitor, in the t...

    journal_title:Annals of neurology

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验

    doi:

    authors: Tawil R,McDermott MP,Brown R Jr,Shapiro BC,Ptacek LJ,McManis PG,Dalakas MC,Spector SA,Mendell JR,Hahn AF,Griggs RC

    更新日期:2000-01-01 00:00:00

  • Neuronal migration abnormality in peroxisomal bifunctional enzyme defect.

    abstract::Patterns of brain dysgenesis that resemble those in the Zellweger syndrome were demonstrated in a boy with an isolated defect of the peroxisomal bifunctional enzyme. There was bilateral centrosylvian pachygyria and polymicrogyria, diffuse hemispheric hypomyelination with heterotopic neurons, Purkinje cell heterotopias...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410390218

    authors: Kaufmann WE,Theda C,Naidu S,Watkins PA,Moser AB,Moser HW

    更新日期:1996-02-01 00:00:00

  • Central levodopa metabolism in Parkinson's disease after administration of stable isotope-labeled levodopa.

    abstract::We report the use of a new stable isotope-labeled form of levodopa (LD) to examine in vivo central LD metabolism in Parkinson's disease (PD). Eight patients representing a wide spectrum of disease severity were administered 50 mg of carbidopa orally followed in 1 hour by an intravenous bolus of 150 mg of stable isotop...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410420305

    authors: Durso R,Evans JE,Josephs E,Szabo GK,Evans BA,Handler JS,Jennings D,Browne TR

    更新日期:1997-09-01 00:00:00

  • Excitotoxicity, free radicals, and cell membrane changes.

    abstract::Neuronal injury resulting from glutamate receptor-mediated excitotoxicity has been implicated in a wide spectrum of neurological disease states, including ischemia, central nervous system trauma, and some types of neurodegenerative diseases. Excitotoxicity may interact with other pathophysiological processes to enhanc...

    journal_title:Annals of neurology

    pub_type: 杂志文章,评审

    doi:10.1002/ana.410350707

    authors: Dugan LL,Choi DW

    更新日期:1994-01-01 00:00:00

  • Outcome of neonatal intraventricular hemorrhage with periventricular echodense lesions.

    abstract::The incidence of periventricular-intraventricular hemorrhage (PV-IVH) in a group of 460 preterm infants with birth weight less than 2,250 gm, studied by cranial ultrasonography, was 39%. Sixty-four (36%) of the infants with periventricular-intraventricular hemorrhage had, in addition, periventricular intraparenchymal ...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410150315

    authors: McMenamin JB,Shackelford GD,Volpe JJ

    更新日期:1984-03-01 00:00:00

  • An operational approach to National Institute on Aging-Alzheimer's Association criteria for preclinical Alzheimer disease.

    abstract:OBJECTIVE:A workgroup commissioned by the Alzheimer's Association (AA) and the National Institute on Aging (NIA) recently published research criteria for preclinical Alzheimer disease (AD). We performed a preliminary assessment of these guidelines. METHODS:We employed Pittsburgh compound B positron emission tomography...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.22628

    authors: Jack CR Jr,Knopman DS,Weigand SD,Wiste HJ,Vemuri P,Lowe V,Kantarci K,Gunter JL,Senjem ML,Ivnik RJ,Roberts RO,Rocca WA,Boeve BF,Petersen RC

    更新日期:2012-06-01 00:00:00

  • Absences in juvenile myoclonic epilepsy: a clinical and video-electroencephalographic study.

    abstract::We report a prospective clinical and electroencephalographic study of 19 patients with juvenile myoclonic epilepsy and absence seizures. Absences began 1 to 9 (4.5 +/- 2.5) years before myoclonic jerks and generalized tonic-clonic seizures. Clinical manifestations during the absence ictus showed great variation, rangi...

    journal_title:Annals of neurology

    pub_type: 杂志文章

    doi:10.1002/ana.410250411

    authors: Panayiotopoulos CP,Obeid T,Waheed G

    更新日期:1989-04-01 00:00:00