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

abstract：:Cerebral amyloid angiopathy (CAA) was found in 57% of 123 autopsy brains removed from patients aged 59-101 years. The incidence of CAA increased with age. CAA was seen most frequently in the occipital cortex. Immunohistochemically, amyloid of CAA was positive for amyloid P component and negative for human AA protein a...

journal_title：Journal of neurology

authors： Yamada M,Tsukagoshi H,Otomo E,Hayakawa M

更新日期：1987-08-01 00:00:00

abstract：:Serial magnetic resonance imaging (MRI) detects substantial subclinical disease activity in multiple sclerosis (MS) and is presently included in most treatment trials as an objective outcome measure. Our current knowledge of the role of MRI in MS treatment trials is derived from very limited patient studies, and the a...

journal_title：Journal of neurology

authors： Barkhof F,Filippi M,Miller DH,Tofts P,Kappos L,Thompson AJ

更新日期：1997-02-01 00:00:00

abstract：:Four patients from three unrelated families, with clinical and electrophysiological findings compatible with the diagnosis of hereditary motor and sensory neuropathy, are presented. The molecular analysis showed that the affected individuals were homozygous for the mutation in the X25 gene, characteristic of Friedreic...

journal_title：Journal of neurology

authors： Panas M,Kalfakis N,Karadima G,Davaki P,Vassilopoulos D

更新日期：2002-11-01 00:00:00

abstract：:A case of CT-diagnosed brain-stem haematoma associated with signs of dorsal medullary involvement is reported. The patient recovered completely within 1 month. ...

journal_title：Journal of neurology

authors： Cappa SF,Riva M,Sterzi R,Canepari C,Delzanno GB

更新日期：1985-01-01 00:00:00

abstract：:A rare form of plasma cell dyscrasia characterized by associated polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes has been termed the POEMS syndrome. The pathophysiology is unknown; plasma cell dyscrasia is essential; secondary manifestations are unexplained. We report a 67-year-old man with a ...

journal_title：Journal of neurology

authors： Vidaković A,Simić P,Stojisavljević N,Elezović I,Trikić R,Apostolski S

更新日期：1992-01-01 00:00:00

abstract：:In 40 rabbits cerebral ischaemia was induced by autologous blood clot emboli injected into the middle cerebral artery. Autologous blood clot formed spontaneously (within 2 h) in a catheter fixed in the internal carotid artery. The effects of embolization were investigated on arterial blood pressure, respiration, local...

journal_title：Journal of neurology

authors： Fekete I,Hegedüs K,Molnár L

更新日期：1988-05-01 00:00:00

abstract：:We evaluated 49 paired cerebrospinal fluid (CSF) and serum samples of 35 patients infected with the human immunodeficiency virus type 1 (HIV-1) for laboratory evidence of cytomegalovirus (CMV) infection. The patients were grouped according to clinical criteria as probable CMV encephalitis/polyradiculomyelitis, CMV ret...

journal_title：Journal of neurology

authors： Weber T,Beck R,Stark E,Gerhards J,Korn K,Haas J,Lüer W,Jahn G

更新日期：1994-06-01 00:00:00

abstract：:We evaluated the health economic burden of patients with Gilles de la Tourette's syndrome (GTS) in Germany over a 3-month observation period. Direct and indirect costs were evaluated in 200 outpatients with GTS (mean age 35 +/- 11.5 years) in Germany. Patients were recruited from three outpatient departments that spec...

journal_title：Journal of neurology

authors： Dodel I,Reese JP,Müller N,Münchau A,Balzer-Geldsetzer M,Wasem J,Oertel WH,Dodel R,Müller-Vahl K

更新日期：2010-07-01 00:00:00

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

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

更新日期：2009-09-01 00:00:00

abstract：:Two right-handed patients who exhibited language disability after left frontal infarction are described. The patients spoke fluently and exhibited excellent repetition ability from the onset of infarction without exhibiting any oral apraxia, but had deficits in auditory comprehension, naming, reading and writing. In b...

journal_title：Journal of neurology

authors： Otsuki M,Soma Y,Koyama A,Yoshimura N,Furukawa H,Tsuji S

更新日期：1998-02-01 00:00:00

abstract：:Quality of Life (QoL) is the preferred outcome in non-pharmacological trials, but there is little UK population evidence of QoL in epilepsy. In advance of evaluating an epilepsy self-management course we aimed to describe, among UK participants, what clinical and psycho-social characteristics are associated with QoL. ...

journal_title：Journal of neurology

authors： Ridsdale L,Wojewodka G,Robinson E,Landau S,Noble A,Taylor S,Richardson M,Baker G,Goldstein LH,SMILE Team.

更新日期：2017-06-01 00:00:00

abstract：:Rodent oligodendrocytes have a unique susceptibility among glia to the lytic effects of complement, due in part to a deficiency in CD59 (protectin), a key surface inhibitor of the complement membrane attack complex (MAC). The possibility that shedding of CD59 by human oligodendrocytes contributes to complement-mediate...

journal_title：Journal of neurology

authors： Roddy J,Clark I,Hazleman BL,Compston DA,Scolding NJ

更新日期：1994-08-01 00:00:00

abstract：INTRODUCTION:Whether different mechanisms, particularly ocular pathology, could lead to the emergence of visual hallucinations (VH) (defined as false perceptions with no external stimulus) versus visual illusions (VI) (defined as a misperception of a real stimulus) in Parkinson's disease (PD) remains debated. We assess...

journal_title：Journal of neurology

authors： Marques A,Beze S,Pereira B,Chassain C,Monneyron N,Delaby L,Lambert C,Fontaine M,Derost P,Debilly B,Rieu I,Lewis SJG,Chiambaretta F,Durif F

更新日期：2020-10-01 00:00:00

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

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

abstract：INTRODUCTION:Patients with blepharospasm in addition to involuntary contraction of the orbicularis oculi muscle also have non-motor symptoms (psychiatric, sleep, cognitive, and ocular). In this paper, we investigated the relationship of non-motor with motor symptoms and the total burden of non-motor symptoms in patient...

journal_title：Journal of neurology

authors： Ferrazzano G,Berardelli I,Conte A,Baione V,Concolato C,Belvisi D,Fabbrini G,Defazio G,Berardelli A

更新日期：2019-11-01 00:00:00

abstract：:The symptomatology of 18 patients with the severest clinical course after subarachnoid hemorrhage (SAH) is described and analyzed. Seventeen patients died, five with an acute bulbar syndrome with cardiac arrest, and 12 with irreversible breakdown of brain function. One patient had an apallic syndrome with minimal sign...

journal_title：Journal of neurology

authors： Binder H,Gerstenbrand F,Jellinger K,Krenn J,Watzek C

更新日期：1979-01-01 00:00:00

abstract：:Psychiatric complaints are common in Parkinson's disease (PD), and have a significant influence in disease outcome and quality of life. Little attention has been paid to psychiatric symptoms at early stage disease. We aimed to screen a population of early stage PD patients for psychiatric symptoms and to study the rel...

journal_title：Journal of neurology

authors： Bugalho P,da Silva JA,Cargaleiro I,Serra M,Neto B

更新日期：2012-01-01 00:00:00

abstract：:We should like to emphasize the following points: 1. Apathy is defined here as a quantified and observable behavioral syndrome consisting in a quantitative reduction of voluntary (or goal-directed) behaviors; 2. Therefore, apathy occurs when the systems that generate and control voluntary actions are altered; 3. These...

journal_title：Journal of neurology

authors： Levy R,Czernecki V

更新日期：2006-12-01 00:00:00

abstract：:Vertigo is frequently associated with migraine, and sometimes it is the cardinal symptom. This type of migraine is called "vestibular migraine", "migrainous vertigo", or "migraine-associated vertigo". Earlier findings on effective prophylactic medication for such migraine attacks and their clinical features are few an...

journal_title：Journal of neurology

authors： Baier B,Winkenwerder E,Dieterich M

更新日期：2009-03-01 00:00:00

abstract：:Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder of progressive heterotopic ossification (HO) caused by a recurrent activating mutation of ACVR1/ALK2, a bone morphogenetic protein (BMP) type I receptor. FOP is characterized by progressive HO, which is associated with inflammation in the setting o...

journal_title：Journal of neurology

authors： Kan L,Kitterman JA,Procissi D,Chakkalakal S,Peng CY,McGuire TL,Goldsby RE,Pignolo RJ,Shore EM,Kaplan FS,Kessler JA

更新日期：2012-12-01 00:00:00

abstract：:In idiopathic rapid eye movement sleep behaviour disorder (RBD), an association with visuoperceptive disorders has been described. However, such an association has not been clearly established in RBD secondary to Parkinson's disease (PD). We compared visuoperceptive function in four groups of non-demented patients (pa...

journal_title：Journal of neurology

authors： Marques A,Dujardin K,Boucart M,Pins D,Delliaux M,Defebvre L,Derambure P,Monaca C

更新日期：2010-03-01 00:00:00

abstract：OBJECTIVE:Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, autoimmune-inflammatory disease of the peripheral nervous system. Recently, various immunoglobulin G4 (IgG4) type auto-antibodies have been described in patients with CIDP which can effectively be removed by immunoadsorption (IA). Therefore, ...

journal_title：Journal of neurology

authors： Dorst J,Ludolph AC,Senel M,Tumani H

更新日期：2018-12-01 00:00:00

abstract：:Alexander disease (AD) is a rare genetic disorder of the central nervous system due to a dysfunction of astrocytes. The most common infantile form presents as a progressive leukodystrophy with macrocephalus. Recently, heterozygous de novo mutations in the gene encoding glial fibrillary acidic protein (GFAP) have been ...

journal_title：Journal of neurology

authors： Brockmann K,Dechent P,Meins M,Haupt M,Sperner J,Stephani U,Frahm J,Hanefeld F

更新日期：2003-03-01 00:00:00

abstract：BACKGROUND:Cognitive impairment is a debilitating symptom experienced by ~ 50% of multiple sclerosis patients, with processing speed (PS) and memory most affected. Until now, the field has considered cognition in a binary fashion: patients are designated as impaired or not impaired. This designation is typically arrive...

journal_title：Journal of neurology

authors： Leavitt VM,Tosto G,Riley CS

更新日期：2018-03-01 00:00:00

abstract：:Facioscapulohumeral muscular dystrophy (FSHD) is the third most common muscular dystrophy and usually follows an autosomal dominant trait. Clinically, FSHD affects facial muscles and proximal upper limb and girdle muscles, but may present with variable clinical phenotypes even within the same family. Most genetically ...

journal_title：Journal of neurology

authors： Reilich P,Schramm N,Schoser B,Schneiderat P,Strigl-Pill N,Müller-Höcker J,Kress W,Ferbert A,Rudnik-Schöneborn S,Noth J,Lochmüller H,Weis J,Walter MC

更新日期：2010-07-01 00:00:00

abstract：:Dopamine agonists have been proved safe and effective as initial therapy in early stages of Parkinson's disease. Prospective long-term clinical trials demonstrated that initial symptomatic treatment with a dopamine agonist is associated with a significantly reduced risk for motor complications in comparison with levod...

journal_title：Journal of neurology

authors： Junghanns S,Glöckler T,Reichmann H

更新日期：2004-09-01 00:00:00

abstract：:The aim of this study was to assess the cognitive functions of patients with spinocerebellar ataxia type 6 (SCA6). We examined 13 patients with genetically confirmed SCA6 and 13 healthy control subjects matched for age, years of education, global cognitive status, and intellectual ability. We administered verbal memor...

journal_title：Journal of neurology

authors： Tamura I,Takei A,Hamada S,Nonaka M,Kurosaki Y,Moriwaka F

更新日期：2017-02-01 00:00:00

abstract：:Neuroinflammation (microglial activation) and subclinical nigrostriatal dysfunction have been reported in subjects at risk of Parkinsonism. Eight non-manifesting carriers (NMCs) of LRRK2 G2019S mutation had 11C-PK11195 and 18F-DOPA PET to assess microglial activation and striatal dopamine system integrity, respectivel...

journal_title：Journal of neurology

authors： Gersel Stokholm M,Garrido A,Tolosa E,Serradell M,Iranzo A,Østergaard K,Borghammer P,Møller A,Parbo P,Stær K,Brooks DJ,Martí MJ,Pavese N

更新日期：2020-08-01 00:00:00

abstract：OBJECTIVE:Patient reported outcome measures (PROMs) can provide researchers with a direct view of patients' experiences. They are becoming increasingly important tools for evaluating clinical care and research outcomes. There has been little data on the application of PROMs to amyotrophic lateral sclerosis (ALS) care. ...

journal_title：Journal of neurology

authors： De Marchi F,Berry JD,Chan J,Caldwell S,Ellrodt A,Scalia J,Burke K,Fang T,Clark Sisodia R,Schwamm LH,Moura LMVR,Paganoni S

更新日期：2020-06-01 00:00:00

abstract：:Repetitive transcranial magnetic stimulation (rTMS) and intermittent theta-burst stimulation (iTBS) can be used to manage post-stroke spasticity, but a meta-analysis of the recent randomized-controlled trials (RCTs) is lacking. Our aim is to perform a meta-analysis of the RCTs that investigated the efficacy of rTMS in...

journal_title：Journal of neurology

authors： Xu P,Huang Y,Wang J,An X,Zhang T,Li Y,Zhang J,Wang B

更新日期：2020-07-11 00:00:00