CBF changes in drug naive juvenile myoclonic epilepsy patients.

abstract：:Several reports in the literature describe the effects of low-dose (5 mg/kg/day) idebenone in significantly reducing cardiac hypertrophy in patients with Friedreich ataxia. However, the effects of idebenone on neurological function have not been reliably determined in these studies; when neurological parameters were r...

journal_title：Journal of neurology

authors： Schulz JB,Di Prospero NA,Fischbeck K

更新日期：2009-03-01 00:00:00

abstract：:Autonomic nervous system (ANS) involvement is frequently found in Parkinson's disease (PD), but its causal relationship to the disease itself and its medication is unclear. We evaluated the effects of PD medications on cardiovascular ANS functions. Heart rate (HR) responses to normal and deep breathing, the Valsalva m...

journal_title：Journal of neurology

authors： Haapaniemi TH,Kallio MA,Korpelainen JT,Suominen K,Tolonen U,Sotaniemi KA,Myllylä VV

更新日期：2000-11-01 00:00:00

abstract：:Autonomic dysfunction is frequently observed in patients with multiple sclerosis (MS), but clinical studies disagree on the frequency and type of abnormalities in autonomic function tests. Orthostatic dizziness (OD) has been reported in up to 49% of patients, but the pathophysiological mechanisms are poorly understood...

journal_title：Journal of neurology

authors： Flachenecker P,Wolf A,Krauser M,Hartung HP,Reiners K

更新日期：1999-07-01 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：: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：:Adolescent-onset spastic ataxia is a proposed novel phenotype in compound heterozygous carriers of an intronic mutation (c.1909 + 22G > A) in the POLR3A gene. Here, we present ten new cases of POLR3A-related spastic ataxia and discuss the genetic, clinical and imaging findings. Patients belonged to six pedigrees with ...

journal_title：Journal of neurology

authors： Infante J,Serrano-Cárdenas KM,Corral-Juan M,Farré X,Sánchez I,de Lucas EM,García A,Martín-Gurpegui JL,Berciano J,Matilla-Dueñas A

更新日期：2020-02-01 00:00:00

abstract：:Cobrotoxin-binding protein was isolated by affinity chromatography from human thymoma which had been surgically removed from patients with myasthenia gravis. The protein was composed of polypeptides with a molecular mass of 40, 51, 65, and 74 kilodaltons as determined by polyacrylamide gel electrophoresis in the prese...

journal_title：Journal of neurology

authors： Kawanami S,Kamei H,Oita J,Kurokawa M,Uchida Y,Hayashi K

更新日期：1987-05-01 00:00:00

abstract：:Brains from 54 patients with organic dementia were examined systematically. As in previous investigations a predominance of Alzheimer type changes was observed. Seventeen patients showed Lewy bodies in the nucleus basalis, the substantia nigra and the locus coeruleus as well as other lesions of parkinsonian type. In 5...

journal_title：Journal of neurology

authors： Ulrich J,Probst A,Wüest M

更新日期：1986-04-01 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

abstract：BACKGROUND:Late-onset Pompe disease (LOPD) is a rare, metabolic disease primarily affecting the musculoskeletal and respiratory systems. Forced vital capacity (FVC) is commonly used to measure pulmonary function; however, associations between FVC and other LOPD outcomes remain unclear. METHODS:A systematic literature ...

journal_title：Journal of neurology

authors： Berger KI,Kanters S,Jansen JP,Stewart A,Sparks S,Haack KA,Bolzani A,Siliman G,Hamed A

更新日期：2019-09-01 00:00:00

abstract：:Out of 72 patients treated for Borrelia burgdorferi meningopolyradiculoneuritis facial palsies occurred in 22 (12 unilateral, 10 bilateral). Eleven of the 32 pareses were initially complete. By the follow-up examination patients had recovered well with slight sequelae in 22% without cosmetically disfiguring pareses or...

journal_title：Journal of neurology

authors： Angerer M,Pfadenhauer K,Stöhr M

更新日期：1993-05-01 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：:Multiple sclerosis (MS), a chronic inflammatory disorder of the central nervous system (CNS), 2 results in damage to axons and their surrounding myelin sheath. The exact cause of inflammation remains unclear, but an autoimmune response directed against CNS antigens is suspected. MS can affect the brain, optic nerve an...

journal_title：Journal of neurology

authors： Hartung HP,Bar-Or A,Zoukos Y

更新日期：2004-09-01 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：BACKGROUND AND PURPOSE:Dysphagia is estimated to occur in up to 50% of the stroke neurorehabilitation population. Those patients with severe neurogenic oropharyngeal dysphagia (NOD) may receive feeding gastrostomy tubes (FGT) if noninvasive therapies prove ineffective in eliminating aspiration or sustaining adequate nu...

journal_title：Journal of neurology

authors： Ickenstein GW,Stein J,Ambrosi D,Goldstein R,Horn M,Bogdahn U

更新日期：2005-12-01 00:00:00

abstract：BACKGROUND:Trans-active response DNA-binding protein of 43 kDa (TDP-43) can be detected in up to 63% of autopsy-confirmed Lewy body disease (LBD) cases. It is unclear whether TDP-43 is associated with a decreased likelihood of a clinical diagnosis of probable dementia with Lewy bodies (pDLB) during life. METHODS:In an...

journal_title：Journal of neurology

authors： Buciuc M,Whitwell JL,Boeve BF,Ferman TJ,Graff-Radford J,Savica R,Kantarci K,Fields JA,Knopman DS,Petersen RC,Parisi JE,Murray ME,Dickson DW,Josephs KA

更新日期：2020-05-01 00:00:00

abstract：:Nerve conduction studies (NCS) and electromyography (EMG), often shortened to 'EMGs', are a useful adjunct to clinical examination of the peripheral nervous system and striated skeletal muscle. NCS provide an efficient and rapid method of quantifying nerve conduction velocity (CV) and the amplitude of both sensory ner...

journal_title：Journal of neurology

authors： Kane NM,Oware A

更新日期：2012-07-01 00:00:00

abstract：:Four cases of mild botulinus type B intoxication are reported with the clinical neurophysiological studies. Electromyographically tetanic stimulation of the N. ulnaris initially led to tetanic potentiation of the muscle action potential. After an interval without any symptoms tetanic reduction became apparent between ...

journal_title：Journal of neurology

authors： Hagenah R,Müller-Jensen A

更新日期：1978-02-14 00:00:00

abstract：:It is now 10 years since the first report of mutations in the presenilin genes that were deterministic for familial autosomal dominant Alzheimer's disease. The most common of these mutations occurs in the presenilin-1 gene (PSEN1) located on chromosome 14. In the ensuing decade, more than 100 PSEN1 mutations have been...

journal_title：Journal of neurology

authors： Larner AJ,Doran M

更新日期：2006-02-01 00:00:00

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

authors： Mason S,Barker RA

更新日期：2015-08-01 00:00:00

abstract：BACKGROUND:The symptomatic treatment of myotonia and myalgia in patients with dystrophic and non-dystrophic myotonias is often not satisfactory. Some patients anecdotally report symptoms' relief through consumption of cannabis. METHODS:A combination of cannabidiol and tetrahydrocannabinol (CBD/THC) was prescribed as c...

journal_title：Journal of neurology

authors： Montagnese F,Stahl K,Wenninger S,Schoser B

更新日期：2020-02-01 00:00:00

abstract：:Fatigue is an important contributor to quality of life in patients who survive aneurysmal subarachnoid hemorrhage (SAH), but the determinants of this fatigue are unclear. We assessed the occurrence of fatigue 1 year after SAH and its relation to physical or cognitive impairment, passive coping, and emotional problems,...

journal_title：Journal of neurology

authors： Passier PE,Post MW,van Zandvoort MJ,Rinkel GJ,Lindeman E,Visser-Meily JM

更新日期：2011-06-01 00:00:00

abstract：:Augmented spinal nociception during the "off" phase has been observed early in Parkinson's disease further increasing with disease duration. To find out whether increased spinal nociception represents a premotor feature, experimental pain sensitivity was assessed in idiopathic REM-sleep behavior disorder (IRBD) patien...

journal_title：Journal of neurology

authors： Boura E,Stamelou M,Vadasz D,Ries V,Unger MM,Kägi G,Oertel WH,Möller JC,Mylius V

更新日期：2017-03-01 00:00:00

abstract：:Botulinum neurotoxin (BoNT) is an effective treatment for cervical dystonia (CD). Long-term changes of several variables, including the dose of BoNT, in these patients is largely unknown. We reviewed the clinical charts of 275 patients with CD treated with BoNT type A (BoNT-A) for at least 5 years since 1989 at ten te...

journal_title：Journal of neurology

authors： Ruiz PJ,Castrillo JC,Burguera JA,Campos V,Castro A,Cancho E,Chacón J,Vara JH,del Val JL,Garcia EL,Miquel F,Sanz P,Vela L

更新日期：2011-06-01 00:00:00

abstract：:We analysed the CACNA1A gene, located on chromosome 19p13, in three unrelated families and one sporadic case with episodic ataxia type 2 (EA-2). In two of the families and the sporadic patient, novel truncating mutations, which disrupt the reading frame and result in a premature stop of the CACNA1A protein, were ident...

journal_title：Journal of neurology

authors： van den Maagdenberg AM,Kors EE,Brunt ER,van Paesschen W,Pascual J,Ravine D,Keeling S,Vanmolkot KR,Vermeulen FL,Terwindt GM,Haan J,Frants RR,Ferrari MD

更新日期：2002-11-01 00:00:00

abstract：:Exercise therapy (ET) can be beneficial in disabled multiple sclerosis (MS) patients. Intermittent transcranial magnetic theta burst stimulation (iTBS) induces long-term excitability changes of the cerebral cortex and may ameliorate spasticity in MS. We investigated whether the combination of iTBS and a program of ET ...

journal_title：Journal of neurology

authors： Mori F,Ljoka C,Magni E,Codecà C,Kusayanagi H,Monteleone F,Sancesario A,Bernardi G,Koch G,Foti C,Centonze D

更新日期：2011-07-01 00:00:00

abstract：:Natalizumab, an anti-alpha4 integrin antibody, significantly reduces the number of visibly enhancing multiple sclerosis (MS) lesions. In this substudy of a 2-year trial of natalizumab monotherapy versus placebo, contrast-enhanced imaging investigated for subtle blood brain barrier (BBB) leakage in relapsing remitting ...

journal_title：Journal of neurology

authors： Soon D,Altmann DR,Fernando KT,Giovannoni G,Barkhof F,Polman CH,O'Connor P,Gray B,Panzara M,Miller DH

更新日期：2007-03-01 00:00:00

abstract：:Specific mutations in COL6A3 have recently been reported as the cause of isolated recessive dystonia, which is a rare movement disorder. In all patients, at least one mutation was located in Exons 41 and 42. In an attempt to replicate these findings, we assessed by direct sequencing the frequency of rare variants in E...

journal_title：Journal of neurology

authors： Lohmann K,Schlicht F,Svetel M,Hinrichs F,Zittel S,Graf J,Lohnau T,Schmidt A,Mir P,Krause P,Lang AE,Jabusch HC,Wolters A,Kamm C,Zeuner KE,Altenmüller E,Naz S,Chung SJ,Kostic VS,Münchau A,Kühn AA,Brüggemann N,Kl

更新日期：2016-04-01 00:00:00

abstract：: ...

journal_title：Journal of neurology

authors： Jackson JL,Kay C,Scholcoff C,Nickoloff S,Fletcher K

更新日期：2018-12-01 00:00:00

abstract：:Levamisole seems to regulate cell-mediated immunity by restoring T-cell function. Since a deficiency of T lymphocytes has been described by various authors in multiple sclerosis patients. Of the 85 patients involved in the trial, evaluation of functional and neurological scores was possible in 54 (32 with placebo and ...

journal_title：Journal of neurology

authors： Gonsette RE,Demonty L,Delmotte P,Decree J,de Cock W,Verhaeghen H,Symoens J

更新日期：1982-01-01 00:00:00