Further observation of Japanese Creutzfeldt-Jacob disease with widespread amyloid plaques.


:An autopsy case of Creutzfeld-Jacob disease with widespread amyloid plaques is reported. A 45-year-old Japanese man, whose father had died of a similar disease, had a 5-year illness characterized by progressive cerebellar signs. Mental changes and brain-stem signs developed in the late stage. Myoclonus frequently occurred. Akinetic mutism ensued. The autopsy revealed spongiform encephalopathy with widespread amyloid plaques and extensive degeneration of the white matter. This disease, Western Gerstmann-Sträussler-Scheinker disease and panencephalopathic type of Creutzfeld-Jacob disease are discussed.


J Neurol


Journal of neurology


Yagishita S,Iwabuchi K,Amano N,Yokoi S




Has Abstract


1989-03-01 00:00:00












  • Autonomic dysfunctions in dementia with Lewy bodies.

    abstract::Twenty-nine cases of both clinically and neuropathologically diagnosed dementia with Lewy bodies (DLB) were retrospectively examined for autonomic symptoms. Twenty-eight cases showed some kind of autonomic dysfunction. Urinary incontinence (97 %) and constipation (83 %) were the two most common. Although urinary reten...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Horimoto Y,Matsumoto M,Akatsu H,Ikari H,Kojima K,Yamamoto T,Otsuka Y,Ojika K,Ueda R,Kosaka K

    更新日期:2003-05-01 00:00:00

  • Uric acid levels predict survival in men with amyotrophic lateral sclerosis.

    abstract::Elevated uric acid levels have recently been found to be associated with slower disease progression in Parkinson's disease, Huntington's disease, multiple system atrophy, and mild cognitive impairment. The aim of this study is to determine whether serum uric acid levels predict survival in amyotrophic lateral sclerosi...

    journal_title:Journal of neurology

    pub_type: 杂志文章,多中心研究


    authors: Paganoni S,Zhang M,Quiroz Zárate A,Jaffa M,Yu H,Cudkowicz ME,Wills AM

    更新日期:2012-09-01 00:00:00

  • MS patient management: the Analog Model.

    abstract::The Analog Model was developed for use in patients with multiple sclerosis (MS) at an international roundtable meeting in 2001. Three key disease components are monitored, namely relapses, disability progression and magnetic resonance imaging activity. Its use provides a rapid, straightforward means of assessing the e...

    journal_title:Journal of neurology

    pub_type: 杂志文章,评审


    authors: Sharief MK

    更新日期:2004-09-01 00:00:00

  • The Brief International Cognitive Assessment in Multiple Sclerosis (BICAMS): results from the German validation study.

    abstract:BACKGROUND:Recent research has convincingly shown that the ability to work mainly depends on the cognitive status in multiple sclerosis (MS). An international committee of experts recommended a brief neuropsychological battery to evaluate cognitive performance in MS. BICAMS comprises three tests, the Symbol Digit Modal...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Filser M,Schreiber H,Pöttgen J,Ullrich S,Lang M,Penner IK

    更新日期:2018-11-01 00:00:00

  • Early diagnosis of secondary progressive multiple sclerosis: focus on fluid and neurophysiological biomarkers.

    abstract:BACKGROUND AND AIMS:Most patients with multiple sclerosis presenting with a relapsing-remitting disease course at diagnosis transition to secondary progressive multiple sclerosis (SPMS) 1-2 decades after onset. SPMS is characterized by predominant neurodegeneration and atrophy. These pathogenic hallmarks result in unsa...

    journal_title:Journal of neurology

    pub_type: 杂志文章,评审


    authors: Ferrazzano G,Crisafulli SG,Baione V,Tartaglia M,Cortese A,Frontoni M,Altieri M,Pauri F,Millefiorini E,Conte A

    更新日期:2020-06-05 00:00:00

  • Clinical experience with oral sumatriptan: a placebo-controlled, dose-ranging study. Oral Sumatriptan Dose-defining Study Group.

    abstract::A double-blind, placebo-controlled multicentre study was carried out to evaluate the efficacy and tolerability of 100, 200 and 300 mg sumatriptan, a selective 5-hydroxytryptamine (5-HT)1-like receptor agonist, given in an oral dispersible form in the acute treatment of migraine attacks. A total of 1130 patients were r...

    journal_title:Journal of neurology

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验


    authors: Patten JP

    更新日期:1991-01-01 00:00:00

  • Do the effects measured by intraoperative and postoperative STN macrostimulation in Parkinson's disease match?

    abstract::The aim of our study was to compare the results obtained by intraoperative and postoperative subthalamic nucleus (STN) macrostimulation in Parkinson's disease (PD). One hundred three PD patients implanted with bilateral STN stimulation were included. The thresholds for efficacy and side effects (motor contraction; par...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Xie J,Adamec D,Decullier E,Bin-Dorel S,Mertens P,Polo G,Poisson A,Broussolle E,Thobois S

    更新日期:2010-09-01 00:00:00

  • Ataxia telangiectasia in identical twins: unusual features.

    abstract::The cases of identical twins with ataxia telangiectasia, early intellectual impairment and progressive spasticity are reported. Immunological tests revealed reduced levels of serum and salivary IgA, increased B cells, reduced T cells and raised alpha-fetoprotein. CT scan performed in one of the twins was normal. The p...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Meshram CM,Sawhney IM,Prabhakar S,Chopra JS

    更新日期:1986-10-01 00:00:00

  • Antibodies and neuronal autoimmune disorders of the CNS.

    abstract::We review the neuronal antibodies described in CNS disorders in order to clarify their diagnostic value, emphasize potentials pitfalls and limitations in the diagnosis of paraneoplastic neurological syndromes (PNS), and examine the current evidence for a possible pathogenic role. We propose to classify the neuronal an...

    journal_title:Journal of neurology

    pub_type: 杂志文章,评审


    authors: Graus F,Saiz A,Dalmau J

    更新日期:2010-04-01 00:00:00

  • Cerebral infarcts associated with migraine: clinical features, risk factors and follow-up.

    abstract::There have been few reports concerning the characteristics of cerebral infarction associated with migraine (CIAM), and especially about the subsequent fate of these patients. We studied 14 patients (9 female) with CIAM. In all these patients the onset of cerebral infarction was accompanied by a unilateral throbbing he...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Hoekstra-van Dalen RA,Cillessen JP,Kappelle LJ,van Gijn J

    更新日期:1996-07-01 00:00:00

  • Somatosensory evoked response in controlled A-alpha sensory fiber disease.

    abstract::In contrast to the distal sensory response, the somatosensory evoked response (SSER) is detectable in patients with A-alpha sensory fiber diseases such as Friedreich's ataxia. In four patients with this disease a combined histological and electro-physiological investigation of sensory propagation was performed. The su...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Colon E,Joosten E,de Weerd J

    更新日期:1978-12-22 00:00:00

  • Impact of pre-stroke dependency on outcome after endovascular therapy in acute ischemic stroke.

    abstract:BACKGROUND AND PURPOSE:Current demographic changes indicate that more people will be care-dependent due to increasing life expectancy. Little is known about impact of preexisting dependency on stroke outcome after endovascular treatment (EVT). METHODS:We compared prospectively collected baseline and outcome data of pr...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Oesch L,Arnold M,Bernasconi C,Kaesmacher J,Fischer U,Mosimann PJ,Jung S,Meinel T,Goeldlin M,Heldner M,Volbers B,Gralla J,Sarikaya H

    更新日期:2020-08-31 00:00:00

  • Early atrophy of pallidum and accumbens nucleus in Huntington's disease.

    abstract::In Huntington's disease (HD) atrophy of the caudate nucleus and putamen has been described many years before clinical manifestation. Volume changes of the pallidum, thalamus, brainstem, accumbens nucleus, hippocampus, and amygdala are less well investigated, or reported with contradicting results. The aim of our study...

    journal_title:Journal of neurology

    pub_type: 杂志文章,多中心研究


    authors: van den Bogaard SJ,Dumas EM,Acharya TP,Johnson H,Langbehn DR,Scahill RI,Tabrizi SJ,van Buchem MA,van der Grond J,Roos RA,TRACK-HD Investigator Group.

    更新日期:2011-03-01 00:00:00

  • Compound heterozygous PNPLA6 mutations cause Boucher-Neuhäuser syndrome with late-onset ataxia.

    abstract::PNPLA6 mutations, known to be associated with the development of motor neuron phenotypes, have recently been identified in families with Boucher-Neuhäuser syndrome. Boucher-Neuhäuser is a rare autosomal recessive syndrome characterized by the co-occurrence of cerebellar ataxia, hypogonadotropic hypogonadism, and chori...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Deik A,Johannes B,Rucker JC,Sánchez E,Brodie SE,Deegan E,Landy K,Kajiwara Y,Scelsa S,Saunders-Pullman R,Paisán-Ruiz C

    更新日期:2014-12-01 00:00:00

  • Wilson's disease: evoked potentials and computed tomography.

    abstract::Multi-modality evoked potentials and computed cranial tomography (CT) were performed in ten patients with Wilson's disease to determine if any of these studies would correlate reliably with neurologic status. While all four patients with CT abnormality had neurologic signs, two additional patients with neurologic find...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Roach ES,Ford CS,Spudis EV,Riela AR,McLean WT Jr,Gilliam J,Ball MR

    更新日期:1985-01-01 00:00:00

  • [Intracranial and spinal hemorrhage in haemophilia (author's transl)].

    abstract::Among 234 hemophiliacs, 14 patients (5.9%) with intracranial and 1 patient (0.4%) with intramedullary hemorrhage were observed and examined. The average age at the time of hemorrhage was 17 years in patients with severe hemophilia and 43 years in patients with moderate hemophilia. The patients with mild hemophilia and...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Mamoli B,Sonneck G,Lechner K

    更新日期:1976-01-14 00:00:00

  • Palatal myoclonus occurring during complex partial status epilepticus.

    abstract::Palatal myoclonus is thought to occur after damage to certain brain-stem structures, and with a delay following the causative lesion. A case of palatal myoclonus, probably of epileptic nature, is described. ...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Emre M

    更新日期:1992-04-01 00:00:00

  • Hematopoietic stem cell transplantation for multiple sclerosis. A retrospective multicenter study.

    abstract:RATIONALE:Phase I/II studies of autologous hematopoietic stem cell transplantation (HSCT) for multiple sclerosis ( MS) were initiated, based on results of experimental transplantation in animal models of multiple sclerosis and clinical observations in patients treated concomitantly for malignant disease. PATIENTS:Eigh...

    journal_title:Journal of neurology

    pub_type: 临床试验,杂志文章,多中心研究


    authors: Fassas A,Passweg JR,Anagnostopoulos A,Kazis A,Kozak T,Havrdova E,Carreras E,Graus F,Kashyap A,Openshaw H,Schipperus M,Deconinck E,Mancardi G,Marmont A,Hansz J,Rabusin M,Zuazu Nagore FJ,Besalduch J,Dentamaro T,Fouill

    更新日期:2002-08-01 00:00:00

  • Proton magnetic resonance spectroscopy in frontotemporal dementia.

    abstract::This study of frontotemporal dementia (FTD) was carried out to determine whether MR spectroscopy can provide an in vivo marker for the neuronal loss and gliosis that occur in this condition. We compared spectra in frontal and temporal regions known to be affected early in the course of the disease with spectra in the ...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Coulthard E,Firbank M,English P,Welch J,Birchall D,O'Brien J,Griffiths TD

    更新日期:2006-07-01 00:00:00

  • Relationship between cortex and pulvinar abnormalities on diffusion-weighted imaging in status epilepticus.

    abstract::The aim of this study was to analyze the pattern of magnetic resonance diffusion-weighted imaging (DWI) findings in status epilepticus in terms of clinical characteristics. Participants comprised 106 patients with status epilepticus who were admitted to our hospital and underwent DWI. Forty-five patients (42.5 %) show...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Nakae Y,Kudo Y,Yamamoto R,Dobashi Y,Kawabata Y,Ikeda S,Yokoyama M,Higashiyama Y,Doi H,Johkura K,Tanaka F

    更新日期:2016-01-01 00:00:00

  • Spatial correlation and segregation of multimodal MRI abnormalities in multiple system atrophy.

    abstract:OBJECTIVE:The variability of the severity and regional distribution of pathological process in basal ganglia (BG) and brainstem-cerebellar systems results in clinical heterogeneity and represents the motor subtype of multiple system atrophy (MSA). This study aimed to quantify spatial patterns of multimodal MRI abnormal...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Lee MJ,Kim TH,Mun CW,Shin HK,Son J,Lee JH

    更新日期:2018-07-01 00:00:00

  • Arterial ischemic stroke in infants, children, and adolescents: results of a Germany-wide surveillance study 2015-2017.

    abstract:OBJECTIVE:Childhood arterial ischaemic stroke (AIS) is rare, but causes significant morbidity and mortality. We aimed to investigate incidence, age-dependent clinical presentation, and risk factors and to discuss the medical care situation in Germany. METHODS:This prospective epidemiological study was conducted via ES...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Gerstl L,Weinberger R,Heinen F,Bonfert MV,Borggraefe I,Schroeder AS,Tacke M,Landgraf MN,Vill K,Kurnik K,Sorg AL,Olivieri M

    更新日期:2019-12-01 00:00:00

  • Patterns of cortical activity differ in ALS patients with limb and/or bulbar involvement depending on motor tasks.

    abstract::Functional magnetic resonance imaging (fMRI) of hand movements in amyotrophic lateral sclerosis (ALS) has repeatedly demonstrated increased activation in cortical and subcortical areas, whereas a single study has suggested decreased rather than increased activations for tongue movements in patients with bulbar involve...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Kollewe K,Münte TF,Samii A,Dengler R,Petri S,Mohammadi B

    更新日期:2011-05-01 00:00:00

  • Secondary prevention after ischemic stroke. Evolution over time in practice.

    abstract:BACKGROUND AND OBJECTIVE:An optimal management of vascular risk factors, associated with antithrombotic drugs and carotid surgery when appropriate, reduces the risk of a new vascular event after stroke. Although secondary prevention is not optimal in many patients in practice, the question of whether there is an improv...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Girot M,Mackowiak-Cordoliani MA,Deplanque D,Hénon H,Lucas C,Leys D

    更新日期:2005-01-01 00:00:00

  • Epidemics and outbreaks of peripheral nervous system disorders: II. Toxic and nutritional causes.

    abstract::Peripheral neuropathies have various causes, both infectious and non-infectious. When we think of "epidemics", we often refer to an infectious or even post-infectious origin. Nevertheless, the history of mankind is marked by episodes of epidemics of peripheral neuropathies of non-infectious nature, either of nutrition...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Mathis S,Soulages A,Vallat JM,Le Masson G

    更新日期:2020-09-11 00:00:00

  • Aspirin plus clopidogrel may reduce the risk of early neurologic deterioration in ischemic stroke patients carrying CYP2C19*2 reduced-function alleles.

    abstract:OBJECTIVES:The mechanisms of early neurologic deterioration (END) and prevention strategies for END are not completely understood. The aim of this study was to investigate the association between CYP2C19*2 variants and END, and the effectiveness of antiplatelet therapy for prevention of END according to CYP2C19*2 genot...

    journal_title:Journal of neurology

    pub_type: 杂志文章,多中心研究,随机对照试验


    authors: Yi X,Zhou Q,Wang C,Lin J,Chai Z

    更新日期:2018-10-01 00:00:00

  • Retrospectively acquired cohort study to evaluate the long-term impact of two different treatment strategies on disability outcomes in patients with relapsing multiple sclerosis (RE.LO.DI.MS): data from the Italian MS Register.

    abstract:BACKGROUND:The increase in disease-modifying drugs (DMDs) allows individualization of treatment in relapsing multiple sclerosis (RMS); however, the long-term impact of different treatment sequences is not well established. This is particularly relevant for MS patients who may need to postpone more aggressive DMD strate...

    journal_title:Journal of neurology

    pub_type: 杂志文章,多中心研究


    authors: Paolicelli D,Lucisano G,Manni A,Avolio C,Bonavita S,Brescia Morra V,Capobianco M,Cocco E,Conte A,De Luca G,De Robertis F,Gasperini C,Gatto M,Gazzola P,Lus G,Iaffaldano A,Iaffaldano P,Maimone D,Mallucci G,Maniscalco

    更新日期:2019-12-01 00:00:00

  • Factors associated with employment and expected work retention among persons with multiple sclerosis: findings of a cross-sectional citizen science study.

    abstract:BACKGROUND:Multiple sclerosis (MS) notably affects adults of working age. For persons with MS (PwMS), being employed enhances their quality of life and it may be regarded as an indicator of overall functioning. Thus, ensuring work participation in PwMS is of general public health interest. OBJECTIVE:To examine relevan...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Lehmann AI,Rodgers S,Kamm CP,Mettler M,Steinemann N,Ajdacic-Gross V,Kaufmann M,Kesselring J,Calabrese P,Salmen A,Gobbi C,Zecca C,Bauer GF,von Wyl V

    更新日期:2020-10-01 00:00:00

  • Efficacy of anti-epileptic drugs in patients with gliomas and seizures.

    abstract::Although seizures in brain tumor patients are common, the knowledge on optimal anti-seizure therapy in this patient group is limited. An observational study was carried out using a database of all patients from the neuro-oncology service during the period 2000-2005, with data on seizure characteristics, therapy with A...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: van Breemen MS,Rijsman RM,Taphoorn MJ,Walchenbach R,Zwinkels H,Vecht CJ

    更新日期:2009-09-01 00:00:00

  • Eradication of Helicobacter pylori may be beneficial in the management of Alzheimer's disease.

    abstract::Infectious agents have been proposed as potential causes of Alzheimer's disease (AD). Recently, we documented a high prevalence of Helicobacter pylori (Hp) infection in patients with AD. We aim to access the effect of Hp eradication on the AD cognitive (MMSE: Mini Mental State Examination and CAMCOG: Cambridge Cogniti...

    journal_title:Journal of neurology

    pub_type: 临床试验,杂志文章


    authors: Kountouras J,Boziki M,Gavalas E,Zavos C,Grigoriadis N,Deretzi G,Tzilves D,Katsinelos P,Tsolaki M,Chatzopoulos D,Venizelos I

    更新日期:2009-05-01 00:00:00