Disease progression but not physical state per se determines mental wellbeing in ALS.

abstract：INTRODUCTION:The spinocerebellar ataxias (SCAs), are rare neurodegenerative disorders caused by distinct genetic mutations. Clinically, the SCAs are characterised by progressive ataxia and a variety of other features, including cognitive dysfunction. The latter is consistent with a growing body of evidence supporting a...

journal_title：Journal of neurology

authors： Garrard P,Martin NH,Giunti P,Cipolotti L

更新日期：2008-03-01 00:00:00

abstract：:Recessive mutations in GJC2, the gene-encoding connexin 47 (Cx47), cause Pelizaeus-Merzbacher-like disease type 1, a severe dysmyelinating disorder. One recessive mutation (p.Ile33Met) has been associated with a much milder phenotype--hereditary spastic paraplegia type 44. Here, we present evidence that a novel Arg98L...

journal_title：Journal of neurology

authors： Abrams CK,Scherer SS,Flores-Obando R,Freidin MM,Wong S,Lamantea E,Farina L,Scaioli V,Pareyson D,Salsano E

更新日期：2014-10-01 00:00:00

abstract：:The objective of the study was to identify differences in treatment strategies for anti-NMDA receptor encephalitis based on specialty of treating physicians, geographic location, and years in practice. We conducted an anonymous worldwide electronic survey through the Practice Current section of Neurology® Clinical Pra...

journal_title：Journal of neurology

authors： Bartolini L,Muscal E

更新日期：2017-04-01 00:00:00

abstract：OBJECTIVES:Recent retrospective studies found sleep disorders, including obstructive sleep apnea and its symptoms to occur more often in patients following aneurysmal subarachnoid hemorrhage, but studies investigating the incidence of subarachnoid hemorrhage in patients with diagnosed obstructive sleep apnea [OSA] comp...

journal_title：Journal of neurology

authors： Zaremba S,Albus L,Schuss P,Vatter H,Klockgether T,Güresir E

更新日期：2019-06-01 00:00:00

abstract：:The cause of idiopathic intracranial hypertension (IIH) remains unknown, and no consensus exists on how patients should be monitored and treated. Acetazolamide is a common treatment but has never been examined in a randomised controlled trial. The objectives of this pilot trial are to prospectively evaluate the use of...

journal_title：Journal of neurology

authors： Ball AK,Howman A,Wheatley K,Burdon MA,Matthews T,Jacks AS,Lawden M,Sivaguru A,Furmston A,Howell S,Sharrack B,Davies MB,Sinclair AJ,Clarke CE

更新日期：2011-05-01 00:00:00

abstract：:Parkinson's disease (PD) is a neurodegenerative disease caused by both genetic and environmental factors. Sirtuins are highly-conserved, NAD-dependent class III deacetylases that regulate a variety of cellular functions. Most of the known sirtuins have been involved in animal models of neurodegenerative disorders, suc...

journal_title：Journal of neurology

authors： Jesús S,Gómez-Garre P,Carrillo F,Cáceres-Redondo MT,Huertas-Fernández I,Bernal-Bernal I,Bonilla-Toribio M,Vargas-González L,Carballo M,Mir P

更新日期：2013-09-01 00:00:00

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

authors： Archelos JJ,Fazekas F

更新日期：2006-09-01 00:00:00

abstract：BACKGROUND:The nonischemic forearm exercise test (NIFET) has been shown to be as effective as the classic ischemic forearm exercise test (IFET) in the diagnosis of patients with McArdle disease. Recently, the lactate increase normalized to the mechanical energy production in NIFET was suggested to have a intermediate s...

journal_title：Journal of neurology

authors： Hanisch F,Eger K,Bork S,Lehnich H,Deschauer M,Zierz S

更新日期：2006-06-01 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：BACKGROUND AND PURPOSE:Parkinson's disease (PD) reduces independence and quality of life through deterioration of upper limb motor function. Transcranial direct current stimulation (tDCS) may offer an alternative, adjunctive therapy for PD. However, the efficacy of tDCS for upper limb motor rehabilitation in PD is unkn...

journal_title：Journal of neurology

authors： Simpson MW,Mak M

更新日期：2020-12-01 00:00:00

abstract：:Decreased tongue strength (TS) might herald bulbar involvement in patients with amyotrophic lateral sclerosis (ALS) well before dysarthria or dysphagia occur, and as such might be prognostic of short survival. The purpose of this study was to investigate the prognostic value of a decreased TS, in addition to other pro...

journal_title：Journal of neurology

authors： Weikamp JG,Schelhaas HJ,Hendriks JC,de Swart BJ,Geurts AC

更新日期：2012-11-01 00:00:00

abstract：OBJECTIVE:We aimed to assess the effectiveness of conservative treatments in children with tension-type headaches (TTH). METHOD:We searched Medline, Embase and the Cochrane Library and performed reference checking. Two independent authors assessed trial quality and extracted data. Analysis was carried out according to...

journal_title：Journal of neurology

authors： Verhagen AP,Damen L,Berger MY,Passchier J,Merlijn V,Koes BW

更新日期：2005-10-01 00:00:00

abstract：:Prognosis of patients with ischemic stroke requiring mechanical ventilation (MV) has been reported to be poor. However, longterm survival and functional outcome have scarcely been studied and nothing is known about the prevalence of cognitive impairment or depression in survivors and their quality of life (QoL). We id...

journal_title：Journal of neurology

authors： Schielke E,Busch MA,Hildenhagen T,Holtkamp M,Küchler I,Harms L,Masuhr F

更新日期：2005-06-01 00:00:00

abstract：BACKGROUND:Despite advances in the field, diagnosis and management of the wide spectrum of neurological events post allogeneic hematopoietic cell transplantation (alloHCT) remain challenging. Therefore, we investigated their incidence, diagnosis, management and long-term prognosis in alloHCT recipients. METHODS:We ret...

journal_title：Journal of neurology

authors： Sakellari I,Gavriilaki E,Papagiannopoulos S,Gavriilaki M,Batsis I,Mallouri D,Vardi A,Constantinou V,Masmanidou M,Yannaki E,Smias C,Geroukis T,Kazis D,Kimiskidis V,Anagnostopoulos A

更新日期：2019-08-01 00:00:00

abstract：:Pontocerebellar hypoplasia type 2 (PCH2) is a very rare autosomal recessive disorder. We report two unrelated female patients born to consanguineous parents presenting with this condition. Patient 1 showed a classical clinical/neuroradiological phenotype of PCH2 with dyskinesia/dystonia. Patient 2 had a neonatal onset...

journal_title：Journal of neurology

authors： Grosso S,Mostadini R,Cioni M,Galluzzi P,Morgese G,Balestri P

更新日期：2002-05-01 00:00:00

abstract：:Ataxia with oculomotor apraxia type 1 (AOA1) is a rare autosomal recessive neurodegenerative disease, recently associated with mutations in the aprataxin gene. Main features are early onset cerebellar ataxia, oculomotor apraxia and peripheral neuropathy. The presence of choreoathetosis or dystonia in some patients sug...

journal_title：Journal of neurology

authors： Salvatore E,Varrone A,Criscuolo C,Mancini P,Sansone V,Strisciuglio C,Cicala D,Scarano V,Salvatore M,Pappatà S,De Michele G,Filla A

更新日期：2008-01-01 00:00:00

abstract：:Acyclovir treatment was used in a randomized, double-blind, placebo-controlled clinical trial with parallel groups to test the hypothesis that herpes virus infections are involved in the pathogenesis of multiple sclerosis (MS). Sixty patients with the relapsing-remitting form of MS were randomized to either oral treat...

journal_title：Journal of neurology

authors： Lycke J,Svennerholm B,Hjelmquist E,Frisén L,Badr G,Andersson M,Vahlne A,Andersen O

更新日期：1996-03-01 00:00:00

abstract：OBJECTIVES:Lumbar spondylosis (LS) is a common spinal degenerative disorder which causes various types of lower urinary tract dysfunction (LUTD). However, it is not certain whether LS may cause urinary retention in elderly women. METHODS:In a period covering the past 3 years, we retrospectively reviewed: a) urodynamic...

journal_title：Journal of neurology

authors： Sakakibara R,Yamamoto T,Uchiyama T,Liu Z,Ito T,Yamazaki M,Awa Y,Yamanishi T,Hattori T

更新日期：2005-08-01 00:00:00

abstract：:Recent studies reported mutations in the gene encoding the proline-rich transmembrane protein 2 (PRRT2) to be causative for paroxysmal kinesigenic dyskinesia (PKD), PKD combined with infantile seizures (ICCA), and benign familial infantile seizures (BFIS). PRRT2 is a presynaptic protein which seems to play an importan...

journal_title：Journal of neurology

authors： Becker F,Schubert J,Striano P,Anttonen AK,Liukkonen E,Gaily E,Gerloff C,Müller S,Heußinger N,Kellinghaus C,Robbiano A,Polvi A,Zittel S,von Oertzen TJ,Rostasy K,Schöls L,Warner T,Münchau A,Lehesjoki AE,Zara F,Lerch

更新日期：2013-05-01 00:00:00

abstract：:An IgM antibody cytotoxic to normal human lymphocytes was found in 46.2% (male: 61.5%; female: 30.8%) of sera from chronic schizophrenics by means of a complement-dependent cytotoxicity test. The relationship between this antibody and the immunological manifestations of schizophrenia is discussed. ...

journal_title：Journal of neurology

authors： Kagami M,Koike T,Maruyama N,Takabayashi K,Tomioka H,Yoshida S,Kon Y

更新日期：1987-06-01 00:00:00

abstract：:Vitamin B(12) (VitB(12), cobalamin) deficiency has been associated with various neuropsychiatric conditions, such as peripheral neuropathy, subacute combined degeneration, affective disorders, and cognitive impairment. Current assays analyze vitamin B(12), of which only a small percentage is metabolically active. Meas...

journal_title：Journal of neurology

authors： Schrempf W,Eulitz M,Neumeister V,Siegert G,Koch R,Reichmann H,Storch A

更新日期：2011-03-01 00:00:00

abstract：:Eighty patients were studied by angiography within 6 h of an ischaemic stroke. Angiography was carried out with digital equipment and was usually limited to the vascular territory responsible for the neurological deficits. In 12 of 19 patients with internal carotid occlusion, the contralateral side was also studied. T...

journal_title：Journal of neurology

authors： Bozzao L,Fantozzi LM,Bastianello S,Bozzao A,Argentino C,Lenzi GL,Fieschi C

更新日期：1989-09-01 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：BACKGROUND AND PURPOSE:Cerebral microbleeds (CMBs) are a possible predictor of symptomatic intracranial hemorrhage (sICH) and poor function outcome (PFO). We aimed to investigate the presence of CMBs on increased incidence of sICH and PFO in acute ischemic stroke patients receiving intravenous thrombolysis (IVT) treatm...

journal_title：Journal of neurology

authors： Yan J,Qiu J,Wu X,Ge Y,Wang J,Wang Y

更新日期：2020-02-01 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：:Even though trigeminovestibular connections are well established in animals, mastication-induced dizziness has been described only as a vascular steal phenomenon in humans. We determined induction or modulation of nystagmus in two index patients with mastication-induced vertigo, 12 normal controls, and 52 additional p...

journal_title：Journal of neurology

authors： Park SH,Kim HJ,Kim JS,Koo JW,Oh SW,Kim DU,Kim JT,Welgampola M,Deriu F

更新日期：2014-03-01 00:00:00

abstract：:In this work, we investigated motor network structure in patients affected by essential tremor (ET) with or without resting tremor, using probabilistic tractography of the cerebello-thalamo-basal ganglia-cortical loop. Twenty-five patients with ET, twenty-two patients with ET associated with resting tremor (rET), and ...

journal_title：Journal of neurology

authors： Caligiuri ME,Arabia G,Barbagallo G,Lupo A,Morelli M,Nisticò R,Novellino F,Quattrone A,Salsone M,Vescio B,Cherubini A,Quattrone A

更新日期：2017-09-01 00:00:00

abstract：:Current findings suggest that multipotent stem cells may be suitable for cell replacement therapies in the treatment of neurodegenerative disorders. Embryonic stem (ES) cells are pluripotent cells isolated from the inner cell mass of the preimplantation blastocyst, which give rise to all cells in the organism. Similar...

journal_title：Journal of neurology

authors： Gerlach M,Braak H,Hartmann A,Jost WH,Odin P,Priller J,Schwarz J

更新日期：2002-10-01 00:00:00

abstract：:Patients in a minimally conscious state (MCS) show restricted signs of awareness but are unable to communicate. We assessed cerebral glucose metabolism in MCS patients and tested the hypothesis that this entity can be subcategorized into MCS- (i.e., patients only showing nonreflex behavior such as visual pursuit, loca...

journal_title：Journal of neurology

authors： Bruno MA,Majerus S,Boly M,Vanhaudenhuyse A,Schnakers C,Gosseries O,Boveroux P,Kirsch M,Demertzi A,Bernard C,Hustinx R,Moonen G,Laureys S

更新日期：2012-06-01 00:00:00

abstract：:We report the familial occurrence and apparent autosomal dominant inheritance of Sneddon's syndrome with variable clinical expression. The proband, a 40-year-old woman, presented with livedo reticularis and progressive neurological deterioration following a stroke. The diagnosis was confirmed by cerebral angiogram and...

journal_title：Journal of neurology

authors： Lossos A,Ben-Hur T,Ben-Nariah Z,Enk C,Gomori M,Soffer D

更新日期：1995-02-01 00:00:00