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












  • Comparison of continuous wave Doppler ultrasound of the radial artery and laser Doppler flowmetry of the fingertips with sympathetic stimulation.

    abstract::Laser Doppler is widely used to evaluate sympathetic vasoconstrictor function. Continuous wave (cw)-Doppler of the radial artery may be an alternative but less expensive approach to quantify sympathetically induced resistance changes in the peripheral vascular system. In order to compare the power of both methods, thi...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Eicke BM,Milke K,Schlereth T,Birklein F

    更新日期:2004-08-01 00:00:00

  • Intravenous thrombolysis for ischemic stroke in the golden hour: propensity-matched analysis from the SITS-EAST registry.

    abstract::As there are scarce data regarding the outcomes of acute ischemic stroke (AIS) patients treated with intravenous thrombolysis (IVT) within 60 min from symptom onset ("golden hour"), we sought to compare outcomes between AIS patients treated within [GH(+)] and outside [GH(-)] the "golden hour" by analyzing propensity s...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Tsivgoulis G,Katsanos AH,Kadlecová P,Czlonkowska A,Kobayashi A,Brozman M,Švigelj V,Csiba L,Fekete K,Kõrv J,Demarin V,Vilionskis A,Jatuzis D,Krespi Y,Liantinioti C,Giannopoulos S,Mikulik R

    更新日期:2017-05-01 00:00:00

  • Trajectories of brain remodeling in temporal lobe epilepsy.

    abstract::Temporal lobe epilepsy has been usually associated with progressive brain atrophy due to neuronal cell loss. However, recent animal models demonstrated a dual effect of epileptic seizures with initial enhancement of hippocampal neurogenesis followed by abnormal astrocyte proliferation and neurogenesis depletion in the...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Roggenhofer E,Santarnecchi E,Muller S,Kherif F,Wiest R,Seeck M,Draganski B

    更新日期:2019-12-01 00:00:00

  • Second opinions and tertiary referrals in neurology: a prospective observational study.

    abstract:BACKGROUND AND OBJECTIVE:The number of neurological second opinions (SO) and tertiary referrals (TR) is increasing. The main purpose of this study was to assess whether a day-care admission made a meaningful contribution to standard neurological outpatient care, for a wide range of second opinions and tertiary referral...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Wieske L,Wijers D,Richard E,Vergouwen MD,Stam J

    更新日期:2008-11-01 00:00:00

  • Cost-of-illness in multiple system atrophy and progressive supranuclear palsy.

    abstract::Multiple system atrophy and progressive supranuclear palsy are disabling neurodegenerative disorders, also known as atypical parkinsonian syndromes. Currently, no health economic evaluations of these diseases are available. The objective of this study was to evaluate disease-related costs in German patients with multi...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Winter Y,Stamelou M,Cabanel N,Sixel-Döring F,Eggert K,Höglinger GU,Herting B,Klockgether T,Reichmann H,Oertel WH,Dodel R,Spottke AE

    更新日期:2011-10-01 00:00:00

  • Affinities of human NMDA receptor autoantibodies: implications for disease mechanisms and clinical diagnostics.

    abstract::Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a common autoimmune encephalitis presenting with psychosis, dyskinesias, autonomic dysfunction and seizures. The underlying autoantibodies against the NR1 subunit are directly pathogenic by disrupting synaptic NMDAR currents. However, antibody titers correlate...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Ly LT,Kreye J,Jurek B,Leubner J,Scheibe F,Lemcke J,Wenke NK,Reincke SM,Prüss H

    更新日期:2018-11-01 00:00:00

  • Neuro-ophthalmology update.

    abstract::This review summarizes the most relevant articles from the field of neuro-ophthalmology published in the Journal of Neurology from January 2012 to July 2013. With the advent of video-oculography, several articles describe new applications for eye movement recordings as a diagnostic tool for a wide range of disorders. ...

    journal_title:Journal of neurology

    pub_type: 杂志文章,评审


    authors: Weber KP,Straumann D

    更新日期:2014-07-01 00:00:00

  • The value of leukocyte adhesion molecules in patients after ischemic stroke.

    abstract::Leukocyte recruitment and inflammatory response play an important patho-physiologic role after cerebral ischemia. This study aimed to evaluate whether leukocyte adhesion molecules can predict clinical outcome in patients after ischemic stroke. We prospectively examined serial changes in p-selectin glycoprotein ligand-...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Tsai NW,Chang WN,Shaw CF,Jan CR,Huang CR,Chen SD,Chuang YC,Lee LH,Lu CH

    更新日期:2009-08-01 00:00:00

  • Subthalamic nucleus stimulation in Parkinson's disease: clinical evaluation of 18 patients.

    abstract::The aim of the present study was to assess the efficacy and safety of chronic subthalamic nucleus deep-brain stimulation (STN-DBS) in patients with Parkinson's disease (PD). 18 consecutive severely affected PD patients were included (mean age, SD: 56.9+/-6 years; mean disease duration: 13.5+/-4.4 years). All the patie...

    journal_title:Journal of neurology

    pub_type: 临床试验,杂志文章


    authors: Thobois S,Mertens P,Guenot M,Hermier M,Mollion H,Bouvard M,Chazot G,Broussolle E,Sindou M

    更新日期:2002-05-01 00:00:00

  • Fixation instability and oculomotor abnormalities in Friedreich's ataxia.

    abstract::Eye movements were studied in 13 patients with Friedreich's ataxia and correlated with MRI findings to investigate whether oculomotor abnormalities can be traced to cerebellar disturbances in this disease. One of the most prominent eye signs was fixation instability (square-wave jerks, SWJ.). Besides SWJ the patients ...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Spieker S,Schulz JB,Petersen D,Fetter M,Klockgether T,Dichgans J

    更新日期:1995-08-01 00:00:00

  • Epidemiology of paraneoplastic neurological syndromes: a population-based study.

    abstract:BACKGROUND:The epidemiology of paraneoplastic neurological syndromes (PNS) remains to be defined. We present here the first population-based incidence study and report the clinical spectrum and antibody profile of PNS in a large area in Northeastern Italy. METHODS:We performed a 9-year (2009-2017) population-based epi...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Vogrig A,Gigli GL,Segatti S,Corazza E,Marini A,Bernardini A,Valent F,Fabris M,Curcio F,Brigo F,Iacono D,Passadore P,Rana M,Honnorat J,Valente M

    更新日期:2020-01-01 00:00:00

  • SMN2 copy number predicts acute or chronic spinal muscular atrophy but does not account for intrafamilial variability in siblings.

    abstract::Spinal muscular atrophy (SMA) is an autosomal recessive disorder that affects motor neurons. It is caused by mutations in the survival motor neuron gene 1 (SMN1). The SMN2 gene, which is the highly homologous SMN1 copy that is present in all the patients, is unable to prevent the disease. An SMN2 dosage method was app...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Cuscó I,Barceló MJ,Rojas-García R,Illa I,Gámez J,Cervera C,Pou A,Izquierdo G,Baiget M,Tizzano EF

    更新日期:2006-01-01 00:00:00

  • Rehabilitation for patients with multiple sclerosis?

    abstract::In the epidemiological area of Southern Lower Saxony 92 patients with clinically definite or probable diagnosis of multiple sclerosis (MS) were interviewed and examined. This group contained a remarkably high percentage of benign cases (52%) in comparison with a sample of hospitalized patients. Neurological examinatio...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Poser S,Bauer HJ,Ritter G,Friedrich H,Beland H,Denecke P

    更新日期:1981-01-01 00:00:00

  • An unusual association of dentato-rubral degeneration with spinal ataxia, ophthalmoplegia and multiple cranial nerve palsies.

    abstract::Clinical and neuropathological data of a 50-year-old woman with an unusual multisystem degeneration are presented. Clinically the illness was characterized by progressive ataxia with ophthalmoplegia and multiple cranial nerve palsies. Neuropathological investigation showed a severe and selective degeneration of the de...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Janzer RC,Barontini F

    更新日期:1985-01-01 00:00:00

  • Breathing patterns and HbSaO2 changes during nocturnal sleep in patients with Duchenne muscular dystrophy.

    abstract::A night-time polygraphic sleep recording with continuous HbSaO2 monitoring was performed in 11 chair-bound Duchenne muscular dystrophy patients with severe restrictive lung disease but with blood gas values within normal limits when awake. No abnormalities of sleep pattern were detected. Nocturnal sleep did not have s...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Manni R,Ottolini A,Cerveri I,Bruschi C,Zoia MC,Lanzi G,Tartara A

    更新日期:1989-10-01 00:00:00

  • Primary DQ effect in the association between HLA and neurological syndromes with anti-GAD65 antibodies.

    abstract::The primary cause of neurological syndromes with antibodies against glutamic acid decarboxylase 65 (GAD65-Ab) is unknown, but genetic predisposition may exist as it is suggested by the co-occurrence in patients and their relatives of other organ-specific autoimmune diseases, notably type 1 diabetes mellitus (T1DM), an...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Muñiz-Castrillo S,Ambati A,Dubois V,Vogrig A,Joubert B,Rogemond V,Picard G,Lin L,Fabien N,Mignot E,Honnorat J

    更新日期:2020-07-01 00:00:00

  • Neutrophil-to-lymphocyte ratio: a marker of neuro-inflammation in multiple sclerosis?

    abstract:BACKGROUND:The significance of neutrophil-to-lymphocyte ratio (NLR) has been explored in different diseases. Few studies addressed its role in patients with multiple sclerosis (MS), with promising results regarding its association with disease activity or disability. OBJECTIVES:We aimed at confirming the role of NLR a...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Gelibter S,Pisa M,Croese T,Dalla Costa G,Orrico M,Preziosa P,Sangalli F,Martinelli V,Furlan R,Filippi M

    更新日期:2021-01-03 00:00:00

  • Lumbar spinal stenosis: assessment of cauda equina involvement by electrophysiological recordings.

    abstract:UNLABELLED:The objective of this study was to investigate the relationship between electrophysiological recordings and clinical as well as radiological findings in patients suggestive to suffer from a lumbar spinal stenosis (LSS). We hypothesise that the electrophysiological recordings, especially SSEP, indicate a lumb...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Egli D,Hausmann O,Schmid M,Boos N,Dietz V,Curt A

    更新日期:2007-06-01 00:00:00

  • Glatiramer acetate induces pro-apoptotic mechanisms involving Bcl-2, Bax and Cyt-c in peripheral lymphocytes from multiple sclerosis patients.

    abstract::Apoptotic deletion of autoreactive T-cells is defective in patients with multiple sclerosis (MS). Glatiramer acetate (GA) treatment seems to restore apoptosis of detrimental T-cells. We analyzed the mitochondria membrane pro- (Bax) and anti-apoptotic (Bcl- 2) and cytosolic pro-apoptotic (Cyt-c, APAF-1) proteins expres...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Ruggieri M,Avolio C,Scacco S,Pica C,Lia A,Zimatore GB,Papa S,Livrea P,Trojano M

    更新日期:2006-02-01 00:00:00

  • Histological investigation of spinal cord lesions in the spinal hyperostotic mouse (twy/twy): morphological changes in anterior horn cells and immunoreactivity to neurotropic factors.

    abstract::We examined the morphology of spinal accessory motoneurons and immunoreactivity to neurotrophins, brain-derived neurotropic factor (BDNF) and neurotrophin (NT)-3, as well as the presence of reactive astrocytosis in 70 tiptoe walking Yoshimura (twy) mice that develop calcification at C1-C2 vertebral level compressing t...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Uchida K,Baba H,Maezawa Y,Furukawa S,Furusawa N,Imura S

    更新日期:1998-12-01 00:00:00

  • IVIG therapy in neurological disorders of childhood.

    abstract::There is growing evidence that intravenous immunoglobulins (IVIG) are effective in some neuroimmunological disorders of childhood. This short review summarizes the evidence-based indications and recommendations of IVIG therapy in these disorders. Despite considerable efforts to define the role and mechanisms of IVIG, ...

    journal_title:Journal of neurology

    pub_type: 杂志文章,评审


    authors: Archelos JJ,Fazekas F

    更新日期:2006-09-01 00:00:00

  • Movement-associated cortical potentials in unilateral cerebral lesions.

    abstract::Movement-associated cortical potentials (MP) associated with thumb-opposition or fist-clenching were studied in 20 patients with unilateral cerebral lesions and in 8 healthy subjects. MP was abnormal in 16 patients. The most frequent abnormality was an attenuation of the N component or readiness potential, recorded on...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Shibasaki H

    更新日期:1975-07-02 00:00:00

  • Cluster headache: pathophysiology, diagnosis and treatment.

    abstract::Cluster headache (CH) is characterized by attacks of severe, strictly unilateral pain that is orbital, supraorbital, temporal, or any combination of these, lasts 15-180 min, and occurs from once every other day to eight times a day. The pain is associated with ipsilateral conjunctival injection, lacrimation, nasal con...

    journal_title:Journal of neurology

    pub_type: 杂志文章,评审


    authors: Ljubisavljevic S,Zidverc Trajkovic J

    更新日期:2019-05-01 00:00:00

  • Biomechanical response of the CNS is associated with frailty in NPH-suspected patients.

    abstract::Frailty is known to predict dementia. However, its link with neurodegenerative alterations of the central nervous system (CNS) is not well understood at present. We investigated the association between the biomechanical response of the CNS and frailty in older adults suspected of normal pressure hydrocephalus (NPH) pr...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Vallet A,Del Campo N,Hoogendijk EO,Lokossou A,Balédent O,Czosnyka Z,Balardy L,Payoux P,Swider P,Lorthois S,Schmidt E

    更新日期:2020-05-01 00:00:00

  • The dynamics of multiple sclerosis. The Charcot Lecture.

    abstract::The history of our understanding of the pathogenesis and pathophysiology of multiple sclerosis are reviewed in the context of Charcot's contribution. The implications for treatment of the new knowledge gained from studies during life of pathology and pathogenesis (by MRI) and pathophysiology (by evoked potentials) are...

    journal_title:Journal of neurology

    pub_type: 传,历史文章,杂志文章


    authors: McDonald WI

    更新日期:1993-01-01 00:00:00

  • Progression of parkinsonism in multiple system atrophy.

    abstract:BACKGROUND:Progression of parkinsonian motor impairment is usually rapid and relentless in multiple system atrophy (MSA). However, it may also be subject to considerable variation. Prospective natural history studies using validated rating scales are required to accurately determine the progression of parkinsonism in M...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Seppi K,Yekhlef F,Diem A,Luginger Wolf E,Mueller J,Tison F,Quinn NP,Poewe W,Wenning GK

    更新日期:2005-01-01 00:00:00

  • Cerebral microbleeds in early Alzheimer's disease.

    abstract::We hypothesize that cerebral microbleeds (CMB) in patients with different neuropsychological profiles (amnestic or non-amnestic) and MRI features of vascular damage could provide important information on the underlying pathological process in early Alzheimer's disease. The study was performed at two trial sites. We st...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Poliakova T,Levin O,Arablinskiy A,Vasenina E,Zerr I

    更新日期:2016-10-01 00:00:00

  • Features of intracranial hemorrhage in cerebral venous thrombosis.

    abstract:BACKGROUND:Cerebral venous thrombosis (CVT) is associated with intracranial hemorrhage. AIM:To identify clinical and imaging features of CVT-associated intracranial hemorrhage. We hypothesized that higher clot burden would be associated with a higher risk of intracranial hemorrhage. METHODS:We performed a retrospecti...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Afifi K,Bellanger G,Buyck PJ,Zuurbier SM,Esperon CG,Barboza MA,Costa P,Escudero I,Renard D,Lemmens R,Hinteregger N,Fazekas F,Conde JJ,Giralt-Steinhauer E,Hiltunen S,Arauz A,Pezzini A,Montaner J,Putaala J,Weimar C,

    更新日期:2020-11-01 00:00:00

  • Progress in Huntington's disease: the search for markers of disease onset and progression.

    abstract::Unlike most neurodegenerative disorders, individuals at risk from Huntington's disease can be identified prior to the onset of clinical signs of the disease by virtue of it being an autosomal dominant condition. This provides the hypothetical opportunity to delay disease onset and/or slow down the progression of the d...

    journal_title:Journal of neurology

    pub_type: 杂志文章,评审


    authors: Mason S,Barker RA

    更新日期:2015-08-01 00:00:00

  • Acute convexity subarachnoid hemorrhage (cSAH) in infectious endocarditis (IE): imaging features and follow-up.

    abstract:AIM:To assess: (1) the prevalence of convexity subarachnoid hemorrhage (cSAH) in infective endocarditis (IE); (2) its relationship with IE features; (3) the associated lesions; (4) whether cSAH is a predictor of future hemorrhage; (5) whether cSAH could cause cortical superficial siderosis (cSS). METHODS:We retrospect...

    journal_title:Journal of neurology

    pub_type: 杂志文章


    authors: Boukobza M,Ilic-Habensus E,Duval X,Laissy JP

    更新日期:2020-10-01 00:00:00