Variants of windmill nystagmus.

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

authors： Mamoli B,Sonneck G,Lechner K

更新日期：1976-01-14 00:00:00

abstract：OBJECTIVE:To report the clinical features and long-term outcome of 22 newly diagnosed paraneoplastic patients with GABAB receptor antibodies (GABABR-Abs). METHODS:Retrospective clinical study of CSF-confirmed cases of GABABR-Abs encephalitis. RESULTS:We identified 22 patients (4 female) with GABABR-Abs, with a median...

journal_title：Journal of neurology

authors： Maureille A,Fenouil T,Joubert B,Picard G,Rogemond V,Pinto AL,Thomas L,Ducray F,Quadrio I,Psimaras D,Berzero G,Antoine JC,Desestret V,Honnorat J

更新日期：2019-01-01 00:00:00

abstract：:Bcl2-associated athanogene 3 (BAG3) gene mutations cause dilated cardiomyopathy and myofibrillar myopathy. Recently, a novel c.625C>T (p.Pro209Ser) mutation in BAG3 was reported to cause axonal Charcot-Marie-Tooth (CMT) disease in three families. Here, we describe two patients with adult-onset and moderate CMT in a Ch...

journal_title：Journal of neurology

authors： Fu J,Ma M,Song J,Pang M,Li G,Zhang J

更新日期：2020-04-01 00:00:00

abstract：OBJECTIVE:Spinal and bulbar muscular atrophy (SBMA) is caused by an abnormal expansion of the CAG repeat in the androgen receptor gene. This study aimed to systematically phenotype a German SBMA cohort (n = 80) based on laboratory markers for neuromuscular, metabolic, and endocrine status, and thus provide a basis for ...

journal_title：Journal of neurology

authors： Rosenbohm A,Hirsch S,Volk AE,Grehl T,Grosskreutz J,Hanisch F,Herrmann A,Kollewe K,Kress W,Meyer T,Petri S,Prudlo J,Wessig C,Müller HP,Dreyhaupt J,Weishaupt J,Kubisch C,Kassubek J,Weydt P,Ludolph AC

更新日期：2018-05-01 00:00:00

abstract：OBJECTIVE:As metabolic and structural changes in frontotemporal-subcortical pathways have been reported in patients with obsessive-compulsive disorders, we investigated the correlation between complex compulsive behaviour (CCB) and the distribution of atrophy in a group of 90 patients with frontotemporal dementia (FTD)...

journal_title：Journal of neurology

authors： Rosso SM,Roks G,Stevens M,de Koning I,Tanghe HLJ,Kamphorst W,Ravid R,Niermeijer MF,van Swieten JC

更新日期：2001-11-01 00:00:00

abstract：:Additional autoimmune diseases in people with multiple sclerosis (MS) and their relatives have been studied many times. Studies have employed different designs, and yielded conflicting results. We performed a systematic review, and calculated overall risk of additional autoimmune diseases in people with MS and their f...

journal_title：Journal of neurology

authors： Dobson R,Giovannoni G

更新日期：2013-05-01 00:00:00

abstract：:Mal de debarquement (MdD) is a subjective perception of self-motion after exposure to passive motion, in most cases sea travel, hence the name. Mal de debarquement occurs quite frequently in otherwise healthy individuals for a short period of time (several hours). However, in some people symptoms remain for a longer p...

journal_title：Journal of neurology

authors： Van Ombergen A,Van Rompaey V,Maes LK,Van de Heyning PH,Wuyts FL

更新日期：2016-05-01 00:00:00

abstract：:Stiff-person syndrome (SPS) is a rare condition of progressive muscular rigidity and spasm, frequently accompanied by other autoimmune conditions, an association which has been further strengthened by the discovery of anti-GAD antibodies and the response of SPS to immunotherapies. Intravenous immunoglobulin (IVIg) is ...

journal_title：Journal of neurology

authors： Gnanapavan S,Vincent A,Giovannoni G

更新日期：2011-10-01 00:00:00

abstract：:Cognition and gait appear to be closely related. The chronological interplay between cognitive decline and gait dysfunction is not fully understood. The aim of the present prospective study is investigating whether the dysfunction of specific gait parameters, during specific task and medication conditions, may predict...

journal_title：Journal of neurology

authors： Amboni M,Iuppariello L,Iavarone A,Fasano A,Palladino R,Rucco R,Picillo M,Lista I,Varriale P,Vitale C,Cesarelli M,Sorrentino G,Barone P

更新日期：2018-10-01 00:00:00

abstract：:Neurological complications following rubella are only rarely encountered. However, in many cases severe neurological impairment may occur, leading to permanent disability. In a recent epidemic of rubella in Israel during the years 1978-1979, 20 patients with severe neurological complications have been seen. We report ...

journal_title：Journal of neurology

authors： Bechar M,Davidovich S,Goldhammer G,Machtey I,Gadoth N

更新日期：1982-01-01 00:00:00

abstract：:To disclose the neuropathological progression course of Machado-Joseph disease (MJD), magnetic resonance imaging (MRI) findings of six genetically confirmed MJD cases (four males and two females, including an autopsied female, all unrelated to one another) were further investigated on neurodegeneration. Brain MRI stud...

journal_title：Journal of neurology

authors： Horimoto Y,Matsumoto M,Akatsu H,Kojima A,Yoshida M,Nokura K,Yuasa H,Katada E,Yamamoto T,Kosaka K,Hashizume Y,Yamamoto H,Mitake S

更新日期：2011-09-01 00:00:00

abstract：:This paper summarizes the methods we devised for the treatment of psychosis, orthostatic hypotension, and mood disorders among the various non-motor complications of Parkinson's disease. Psychosis may not manifest when a patient believes in his/her delusions. If left untreated over a prolonged period, however, the del...

journal_title：Journal of neurology

authors： Fujimoto K

更新日期：2009-08-01 00:00:00

abstract：OBJECTIVE:To assess the changes of muscle-related biomarkers at the early stage of amyotrophic lateral sclerosis, and to confirm these findings in an experimental animal model. METHODS:Thirty-nine subjects with sporadic amyotrophic lateral sclerosis and 20 healthy controls were enrolled and longitudinally evaluated. W...

journal_title：Journal of neurology

authors： Ito D,Hashizume A,Hijikata Y,Yamada S,Iguchi Y,Iida M,Kishimoto Y,Moriyoshi H,Hirakawa A,Katsuno M

更新日期：2019-12-01 00:00:00

abstract：BACKGROUND:Multiple sclerosis (MS) is an autoimmune, demyelinating disease of the central nervous system. The treatment of MS has always been a focus of neurological research. To date, the US Food and Drug Administration has approved 15 medications for modifying the course of multiple sclerosis. In this study, we exami...

journal_title：Journal of neurology

authors： Li H,Hu F,Zhang Y,Li K

更新日期：2020-12-01 00:00:00

abstract：:Predicting unfavorable outcome is of paramount importance in clinical decision making. Accordingly, we designed this multinational study, which provided the largest case series of tuberculous meningitis (TBM). 43 centers from 14 countries (Albania, Croatia, Denmark, Egypt, France, Hungary, Iraq, Italy, Macedonia, Roma...

journal_title：Journal of neurology

authors： Erdem H,Ozturk-Engin D,Tireli H,Kilicoglu G,Defres S,Gulsun S,Sengoz G,Crisan A,Johansen IS,Inan A,Nechifor M,Al-Mahdawi A,Civljak R,Ozguler M,Savic B,Ceran N,Cacopardo B,Inal AS,Namiduru M,Dayan S,Kayabas U,Par

更新日期：2015-01-01 00:00:00

abstract：:A 37-year-old man presented with an acute amnestic syndrome of Korsakoff's type and an upper brain-stem oculomotor syndrome. After a moderate improvement with steroid therapy, he developed progressive behavioural changes due to a frontal lobe syndrome, in addition to motor and visual impairment. Memory performance was...

journal_title：Journal of neurology

authors： Vighetto A,Charles N,Salzmann M,Confavreux C,Aimard G

更新日期：1991-09-01 00:00:00

abstract：:Patients with multiple system atrophy (MSA) often have evidence of compromised gastrointestinal motility. Ghrelin is a gut hormone that influences gastrointestinal motility in humans. The aim of this study was to determine whether ghrelin secretion is affected in MSA patients, and to investigate the relation between g...

journal_title：Journal of neurology

authors： Ozawa T,Tokunaga J,Arakawa M,Ishikawa A,Takeuchi R,Mezaki N,Miura T,Sakai N,Hokari M,Takeshima A,Utsumi K,Kondo T,Yokoseki A,Nishizawa M

更新日期：2013-08-01 00:00:00

abstract：:Despite the fact that almost one-third of patients suffer from visual deficits following brain damage; neuro-visual rehabilitation to compensate for visual field deficits is relatively neglected in the clinical setting. This is in contrast to physio and speech therapies, which are the bread and butter of rehabilitativ...

journal_title：Journal of neurology

authors： Raz N,Levin N

更新日期：2017-06-01 00:00:00

abstract：INTRODUCTION:Mal de Debarquement Syndrome (MdDS) is the rare condition of enduring rocking sensations and subjective unsteadiness following a lengthy exposure to passive motion. The pathogenesis of MdDS is unknown and the available treatment is limited. Here, we developed an experimental model of MdDS that may facilita...

journal_title：Journal of neurology

authors： Schepermann A,Bardins S,Penkava J,Brandt T,Huppert D,Wuehr M

更新日期：2019-09-01 00:00:00

abstract：:The frequency of malignant diseases among 1866 living and 340 deceased multiple sclerosis (MS) patients was investigated in Finland. The study revealed a low prevalence (0.64%) and mortality (0.07%) rate of cancer among MS patients. The difference between MS patients and general population was significant. The highest...

journal_title：Journal of neurology

authors： Palo J,Duchesne J,Wikström J

更新日期：1977-10-07 00:00:00

abstract：:Parkinson's disease (PD) is characterised both clinically and pathologically by features that distinguish it from other parkinsonian disorders including, for instance, multiple system atrophy and progressive supranuclear palsy. The aetiologies of PD includes both genetic and environmental influences. Several single ge...

journal_title：Journal of neurology

authors： Schapira AH

更新日期：2011-05-01 00:00:00

abstract：:The seasonal occurrence of relapses was analysed retrospectively in a group of 53 patients with treated dermatomyositis (DM) or polymyositis (PM). In DM, the incidence of both myositic and cutaneous relapses was highest in summer whereas in the PM group relapses was more evenly distributed throughout the seasons but l...

journal_title：Journal of neurology

authors： Phillips BA,Zilko PJ,Garlepp MJ,Mastaglia FL

更新日期：2002-04-01 00:00:00

abstract：OBJECTIVE:To characterize the relation between brain functional connectivity and disability in patients with multiple sclerosis; to investigate the existence of critical values of both disability and functional connectivity corresponding to exhaustion of functional adaptive mechanisms. METHODS:Hundred-and-nineteen pat...

journal_title：Journal of neurology

authors： Tommasin S,De Giglio L,Ruggieri S,Petsas N,Giannì C,Pozzilli C,Pantano P

更新日期：2018-12-01 00:00:00

abstract：:Cerebral ischemia of 15 min was produced in rats by increasing intracranial pressure above the systolic blood pressure level by infusing artificial cerebrospinal fluid into the cisterna magna. Pulmonary edema was prevented by blocking the vasomotor response with a ganglioplegic agent, and reducing the fluid load by pe...

journal_title：Journal of neurology

authors： Kawakami S,Hossmann KA

更新日期：1977-12-01 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：:Familial amyloid polyneuropathy (FAP) is a progressive systemic autosomal dominant disease caused by pathogenic mutations in the transthyretin (TTR) gene. We studied clinical, electrophysiological, histopathological, and genetic characteristics in 15 (13 late-onset and two early-onset) patients belonging to 14 familie...

journal_title：Journal of neurology

authors： Dohrn MF,Röcken C,De Bleecker JL,Martin JJ,Vorgerd M,Van den Bergh PY,Ferbert A,Hinderhofer K,Schröder JM,Weis J,Schulz JB,Claeys KG

更新日期：2013-12-01 00:00:00

abstract：:Using high-performance liquid chromatography, adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN) were diagnosed by the analysis of fatty acids in sphingomyelin. The analytical method was simple, sensitive and accurate. In ALD and AMN patients, very long chain fatty acids (C26:0, C25:0 and C24:0 on the base of ...

journal_title：Journal of neurology

authors： Kobayashi T,Katayama M,Suzuki S,Tomoda H,Goto I,Kuroiwa Y

更新日期：1983-01-01 00:00:00

abstract：:Glucocorticoids are the standard of care for multiple sclerosis (MS) relapses, but the most desirable route of administration is still matter of debate. The aim of the study was to compare the efficacy and safety of oral versus intravenous steroids for treatment of acute relapses in patients with MS. Randomized or qua...

journal_title：Journal of neurology

authors： Lattanzi S,Cagnetti C,Danni M,Provinciali L,Silvestrini M

更新日期：2017-08-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Mechanical thrombectomy (MT) improves early clinical outcome in patients with acute ischemic stroke but insights on determinants of long-term outcome after MT treatment are scarce. METHODS:Data from stroke patients with anterior circulation large vessel occlusion of a prospective MT registry (01...

journal_title：Journal of neurology

authors： Fuhrer H,Forner L,Pruellage P,Weber S,Beume LA,Schacht H,Egger K,Bardutzky J,Weiller C,Urbach H,Niesen WD,Meckel S

更新日期：2020-04-01 00:00:00

abstract：BACKGROUND:Body weight reduction after disease onset is an independent predictor of survival in amyotrophic lateral sclerosis (ALS), but significance of weight variation after diagnosis remains to be established. OBJECTIVE:To investigate weight variation after diagnosis and its prognostic significance in patients with...

journal_title：Journal of neurology

authors： Shimizu T,Nakayama Y,Matsuda C,Haraguchi M,Bokuda K,Ishikawa-Takata K,Kawata A,Isozaki E

更新日期：2019-06-01 00:00:00