Hallervorden-Spatz disease: clinical and MRI study of 11 cases diagnosed in life.

abstract：:The transcranial activation and the conduction properties of corticospinal and callosal neurons were investigated in 12 early-treated adolescents (aged 17.3, SD 3.5 years; range 14-27 years) with phenylketonuria (PKU) by focal transcranial magnetic stimulation (fTMS) of the motor cortex. The patients had no functional...

journal_title：Journal of neurology

authors： Röricht S,Meyer BU,Irlbacher K,Ludolph AC

更新日期：1999-01-01 00:00:00

abstract：PURPOSE:Anti-myelin oligodendrocyte glycoprotein antibodies (anti-MOG), directed against a component of the myelin sheath, are sometimes detected in the blood or cerebrospinal fluid (CSF) of patients with acute demyelinating conditions. Cortical encephalitic presentations in anti-MOG-antibody-positive patients are reco...

journal_title：Journal of neurology

authors： Doig D,McNamara C,Mewasingh L,Beri S,Jones B,Kachramanoglou C,Jan W

更新日期：2020-10-14 00:00:00

abstract：:Little is known about the relationship between cognitive dysfunctions and the non-motor complex in subjects with newly diagnosed untreated Parkinson's disease (PD). The aim of this study was to explore the association between non-motor symptoms (NMS) and cognitive dysfunctions in an incident cohort of de novo, drug-na...

journal_title：Journal of neurology

authors： Erro R,Santangelo G,Picillo M,Vitale C,Amboni M,Longo K,Costagliola A,Pellecchia MT,Allocca R,De Rosa A,De Michele G,Santoro L,Barone P

更新日期：2012-09-01 00:00:00

abstract：:Posterior leukoencephalopathy typically denotes neurotoxicity from immunosuppressive agents, malignant hypertension or eclampsia. It has not been documented in central nervous system angiitis. We present three cases associated with isolated cerebral angiitis after review of all cases of isolated CNS angiitis from 1998...

journal_title：Journal of neurology

authors： Wijdicks EF,Manno EM,Fulgham JR,Giannini C

更新日期：2003-04-01 00:00:00

abstract：:Freezing of gait (FOG) is a disabling motor symptom experienced by a large proportion of patients with Parkinson's disease (PD). While it is known that FOG contributes to lower health-related quality of life (HRQoL), previous studies have not accounted for other important factors when measuring the specific impact of ...

journal_title：Journal of neurology

authors： Walton CC,Shine JM,Hall JM,O'Callaghan C,Mowszowski L,Gilat M,Szeto JY,Naismith SL,Lewis SJ

更新日期：2015-01-01 00:00:00

abstract：:The electrically elicited blink reflex was investigated in 25 patients with ischaemic lesions of the pons or the medulla oblongata. Only patients with a lesion on MRI appropriate to the clinical syndrome were included. Twenty patients had an infarction of the pons, bilateral in 5. Additional 5 patients had an infarcti...

journal_title：Journal of neurology

authors： Meincke U,Ferbert A

更新日期：1993-11-01 00:00:00

abstract：BACKGROUND:Recent research has convincingly shown that the ability to work mainly depends on the cognitive status in multiple sclerosis (MS). An international committee of experts recommended a brief neuropsychological battery to evaluate cognitive performance in MS. BICAMS comprises three tests, the Symbol Digit Modal...

journal_title：Journal of neurology

authors： Filser M,Schreiber H,Pöttgen J,Ullrich S,Lang M,Penner IK

更新日期：2018-11-01 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：INTRODUCTION:Ataxic polyneuropathy, which occurs in endemic form in an area in southwest Nigeria, is attributed to exposure to cyanide from cassava foods. Exposure to cyanide from cassava is, however, not exclusive to this endemic area. In this study, the occurrence of ataxic polyneuropathy was compared in two communit...

journal_title：Journal of neurology

authors： Oluwole OS,Onabolu AO,Cotgreave IA,Rosling H,Persson A,Link H

更新日期：2002-08-01 00:00:00

abstract：:We have studied the relationship between the apolipoprotein E gene (APOE) and the development of myoclonus, tremors, rigidity and seizures in 168 patients with probable early-onset Alzheimer's disease (AD). There was a statistically significantly lower risk of tremor for carriers of the epsilon 4 allele of APOE. This ...

journal_title：Journal of neurology

authors： Bronzova J,van Duijn CM,Havekes LM,de Knijff P,Van Broeckhoven C,Hofman A

更新日期：1996-06-01 00:00:00

abstract：:Thyronine (T4), triiodothyronine (T3), and reverse-triiodothyronine (rT3) levels were evaluated in cerebrospinal fluid (CSF) and in serum of 12 patients with definite amyotrophic lateral sclerosis (ALS) by specific radioimmunoassays. Circulating microsomal and thyroglobulin antibodies were also evaluated. In all patie...

journal_title：Journal of neurology

authors： Malin JP,Ködding R,Fuhrmann H,von zur Mühlen A

更新日期：1989-01-01 00:00:00

abstract：:Continuous intrathecal administration of baclofen with implanted programmable pump systems is recommended in the treatment of severe spasticity of cerebral origin. Prior to pump implantation, a baclofen bolus test (BBT) is used to assess the effectiveness of intrathecal baclofen using the modified Ashworth Scale (MAS)...

journal_title：Journal of neurology

authors： Pohl M,Rockstroh G,Rückriem S,Mehrholz J,Mrass G,Pause M

更新日期：2002-09-01 00:00:00

abstract：:We report a rare association of spinocerebellar ataxia and motor neuron disease (MND) in a woman with genetically confirmed SCA2 who subsequently developed a rapidly progressive and fatal form of MND. Considering the rarity of these two neurological conditions, it is interesting to note that the concomitant occurrence...

journal_title：Journal of neurology

authors： Nanetti L,Fancellu R,Tomasello C,Gellera C,Pareyson D,Mariotti C

更新日期：2009-11-01 00:00:00

abstract：:Although central facial paresis (CFP) is a major symptom of stroke, there is a lack of studies on the motor and non-motor disabilities in stroke patients. A prospective cohort study was performed at admission for inpatient rehabilitation and discharge of post-stroke phase of 112 patients (44% female, median age: 64 ye...

journal_title：Journal of neurology

authors： Volk GF,Steinerstauch A,Lorenz A,Modersohn L,Mothes O,Denzler J,Klingner CM,Hamzei F,Guntinas-Lichius O

更新日期：2019-01-01 00:00:00

abstract：:We describe three patients in whom an isolated sixth nerve palsy was the only clinical symptom or sign of multiple sclerosis (MS). Data were collected prospectively over 6 years on these three patients, who showed no other signs of brainstem dysfunction or prior symptoms; in addition. Retrospective analysis of all pat...

journal_title：Journal of neurology

authors： Barr D,Kupersmith MJ,Turbin R,Bose S,Roth R

更新日期：2000-09-01 00:00:00

abstract：CONTEXT:Polyneuropathy is a common disease and is more prevalent (at least 3 %) in elderly people. However, routine neurological examination of healthy elderly people may show distal sensory loss and absent tendon reflexes, which can obscure the distinction from polyneuropathy. OBJECTIVE:To investigate the relation be...

journal_title：Journal of neurology

authors： Vrancken AF,Kalmijn S,Brugman F,Rinkel GJ,Notermans NC

更新日期：2006-05-01 00:00:00

abstract：:Eight cases of malignancies with concurrent motor neuron disease (MND), derived from an unselected population representing all cases of MND diagnosed during a 15-year period in two Italian provinces, were studied to verify the existence of paraneoplastic forms of the disease. No statistically significant association b...

journal_title：Journal of neurology

authors： Chiò A,Brignolio F,Meineri P,Rosso MG,Tribolo A,Schiffer D

更新日期：1988-07-01 00:00:00

abstract：OBJECTIVE:To determine the prevalence and characteristics of the cricopharyngeal bar (CPB), defined as marked protrusion with lacking relaxation and stricture of the upper esophageal sphincter on videofluoroscopy, in patients with inclusion body myositis (IBM). METHODS:We conducted a case-control study of comprehensiv...

journal_title：Journal of neurology

authors： Taira K,Yamamoto T,Mori-Yoshimura M,Sajima K,Takizawa H,Shinmi J,Oya Y,Nishino I,Takahashi Y

更新日期：2020-09-26 00:00:00

abstract：:Cerebrospinal fluid (CSF) levels of interleukin (IL)-6, IL-11 and leukaemia inhibitory factor (LIF) were evaluated in 43 patients with Alzheimer's disease (AD) and 24 patients with frontotemporal lobar degeneration (FTLD) as compared with 30 agematched controls (CON), and correlated with clinical and demographic data ...

journal_title：Journal of neurology

authors： Galimberti D,Venturelli E,Fenoglio C,Guidi I,Villa C,Bergamaschini L,Cortini F,Scalabrini D,Baron P,Vergani C,Bresolin N,Scarpini E

更新日期：2008-04-01 00:00:00

abstract：:Headache attributed to ingestion of a cold stimulus (ICHD-3 beta 4.5.1) is also known as ice cream headache (ICH). This cross-sectional epidemiological study included 283 students (10-14-year-olds) attending a grammar school in Germany, their parents (n = 401), and 41 teachers. A self-administered questionnaire was us...

journal_title：Journal of neurology

authors： Zierz AM,Mehl T,Kraya T,Wienke A,Zierz S

更新日期：2016-06-01 00:00:00

abstract：:Biomarkers beyond clinical assessment are needed in patients who suffer from amyotrophic lateral sclerosis (ALS). Here, single-voxel proton magnetic resonance spectroscopy ((1)H MRS) of the gray matter of the motor cortex and the white matter including the pyramidal tracts was used to investigate concentrations of N-a...

journal_title：Journal of neurology

authors： Unrath A,Ludolph AC,Kassubek J

更新日期：2007-08-01 00:00:00

abstract：:To further evaluate (1) transcranial sonography (TCS) for (pre)clinical diagnosis of Parkinson's disease (PD) and (2) to examine asymptomatic carriers of Parkin mutations we investigated substantia nigra (SN) hyperechogenicity in PD patients and unaffected subjects with and without Parkin mutations. The area (aSN) of ...

journal_title：Journal of neurology

authors： Hagenah JM,König IR,Becker B,Hilker R,Kasten M,Hedrich K,Pramstaller PP,Klein C,Seidel G

更新日期：2007-10-01 00:00:00

abstract：:Rapid Eye Movement sleep behavior disorder (RBD) is a parasomnia causing sufferers to physically act out their dreams. These behaviors can disrupt sleep and sometimes lead to injuries in patients and their bed-partners. Clonazepam and melatonin are the first-line pharmacological treatment options for RBD based on dire...

journal_title：Journal of neurology

authors： Gilat M,Marshall NS,Testelmans D,Buyse B,Lewis SJG

更新日期：2021-01-07 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：BACKGROUND:In vascular dementia (VaD), assessment of cerebral blood flow by single photon emission computed tomography (CBF SPECT) has been used to detect a patchy decrease of blood flow or a frontal reduction. In addition to reduced blood flow, the heterogeneous distribution of cerebral blood flow is often observed in...

journal_title：Journal of neurology

authors： Yoshikawa T,Murase K,Oku N,Kitagawa K,Imaizumi M,Takasawa M,Rishu P,Hashikawa K,Nishikawa T,Hori M,Matsumoto M

更新日期：2003-02-01 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：:Recent years have seen substantial news and updates in the genetics and diagnosis of schwannomas, even a new hereditary disease with schwannomas; Schwannomatosis has been defined. These developments have consequently led to better evaluation of the incidence of schwannomas. Although there has also been progress in the...

journal_title：Journal of neurology

authors： Hanemann CO,Evans DG

更新日期：2006-12-01 00:00:00

abstract：:Focal transcranial magnetic stimulation (TMS) of the motor cortex was used to study excitatory and inhibitory stimulation effects in 25 patients with writer's cramp and 25 healthy volunteers. We investigated excitatory and inhibitory corticospinally mediated motor effects in muscles contralateral to the stimulation si...

journal_title：Journal of neurology

authors： Niehaus L,von Alt-Stutterheim K,Röricht S,Meyer BU

更新日期：2001-01-01 00:00:00

abstract：BACKGROUND AND AIMS:Most patients with multiple sclerosis presenting with a relapsing-remitting disease course at diagnosis transition to secondary progressive multiple sclerosis (SPMS) 1-2 decades after onset. SPMS is characterized by predominant neurodegeneration and atrophy. These pathogenic hallmarks result in unsa...

journal_title：Journal of neurology

authors： Ferrazzano G,Crisafulli SG,Baione V,Tartaglia M,Cortese A,Frontoni M,Altieri M,Pauri F,Millefiorini E,Conte A

更新日期：2020-06-05 00:00:00

abstract：:The polymorphism of the HLA class II genes was investigated in 97 patients with multiple sclerosis (MS) in Hokkaido, the northernmost main island of Japan. Of these, 80 patients were classified as having conventional MS and 17 as having opticospinal MS (OS-MS). Our findings confirmed a previous report that the DPB 1*0...

journal_title：Journal of neurology

authors： Fukazawa T,Kikuchi S,Sasaki H,Yabe I,Miyagishi R,Hamada T,Tashiro K

更新日期：2000-03-01 00:00:00