Lipoic (thioctic) acid increases brain energy availability and skeletal muscle performance as shown by in vivo 31P-MRS in a patient with mitochondrial cytopathy.

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：:Mutations in C19orf12 have been recently identified as the molecular genetic cause of a subtype of neurodegeneration with brain iron accumulation (NBIA). Given the mitochondrial localization of the gene product the new NBIA subtype was designated mitochondrial membrane protein-associated neurodegeneration. Frequent fe...

journal_title：Journal of neurology

authors： Deschauer M,Gaul C,Behrmann C,Prokisch H,Zierz S,Haack TB

更新日期：2012-11-01 00:00:00

abstract：OBJECTIVE:Crossed aphasia (CA), usually referred to as an acquired language disturbance, is caused by a lesion in the cerebral hemisphere ipsilateral to the dominant hand, and the exact mechanism is not clear. The development of handedness is influenced by education and training and the impact of habitualization, while...

journal_title：Journal of neurology

authors： Tan X,Guo Y,Dun S,Sun H

更新日期：2018-07-01 00:00:00

abstract：:The various clinical features of multiple system atrophy (MSA) make the diagnosis of the disease difficult, especially in its early stages, when signs of differentiated neuroanatomical system involvement have not yet appeared. Mortality studies may be affected by the variability of the diagnostic criteria and selectio...

journal_title：Journal of neurology

authors： Testa D,Filippini G,Farinotti M,Palazzini E,Caraceni T

更新日期：1996-05-01 00:00:00

abstract：:We report the case of a 17.5-year old girl with generalized myasthenia gravis (MG). When she was 13, she started to complain of episodic diplopia, ptosis and mild fatigability of upper and lower extremity muscles. She was diagnosed with MG 3 years later, after exacerbation of her limb muscle weakness. Acetylcholine re...

journal_title：Journal of neurology

authors： Kostera-Pruszczyk A,Kwiecinski H

更新日期：2009-10-01 00:00:00

abstract：:Laser Doppler is widely used to evaluate sympathetic vasoconstrictor function. Continuous wave (cw)-Doppler of the radial artery may be an alternative but less expensive approach to quantify sympathetically induced resistance changes in the peripheral vascular system. In order to compare the power of both methods, thi...

journal_title：Journal of neurology

authors： Eicke BM,Milke K,Schlereth T,Birklein F

更新日期：2004-08-01 00:00:00

abstract：OBJECTIVE:Childhood arterial ischaemic stroke (AIS) is rare, but causes significant morbidity and mortality. We aimed to investigate incidence, age-dependent clinical presentation, and risk factors and to discuss the medical care situation in Germany. METHODS:This prospective epidemiological study was conducted via ES...

journal_title：Journal of neurology

authors： Gerstl L,Weinberger R,Heinen F,Bonfert MV,Borggraefe I,Schroeder AS,Tacke M,Landgraf MN,Vill K,Kurnik K,Sorg AL,Olivieri M

更新日期：2019-12-01 00:00:00

abstract：:Spastic paraplegia type 7 (SPG7) is an autosomal recessive form of hereditary spastic paraparesis (ARHSP) caused by mutations in paraplegin, a subunit of an ATP-dependent AAA-protease located within the inner mitochondrial membrane. We have identified a novel paraplegin mutation, c.1047insC, in a non-consanguineous No...

journal_title：Journal of neurology

authors： Tzoulis C,Denora PS,Santorelli FM,Bindoff LA

更新日期：2008-08-01 00:00:00

abstract：:Epileptic syndromes are distinctive disorders with specific features, which when taken together, permit a specific diagnosis. There is actually a debate on that medial temporal lobe epilepsy with hippocampal sclerosis is an epileptic syndrome. To address this issue, we searched for discriminative semiological features...

journal_title：Journal of neurology

authors： No YJ,Zavanone C,Bielle F,Nguyen-Michel VH,Samson Y,Adam C,Navarro V,Dupont S

更新日期：2017-05-01 00:00:00

abstract：:Hearing deficits associated with cognitive impairment have attracted much recent interest, motivated by emerging evidence that impaired hearing is a risk factor for cognitive decline. However, dementia and hearing impairment present immense challenges in their own right, and their intersection in the auditory brain re...

journal_title：Journal of neurology

authors： Hardy CJ,Marshall CR,Golden HL,Clark CN,Mummery CJ,Griffiths TD,Bamiou DE,Warren JD

更新日期：2016-11-01 00:00:00

abstract：:Currently, several disease-modifying therapies for the treatment of multiple sclerosis are established and more are likely to be introduced. These treatment options differ with respect to their application profile, mechanism of action, efficacy, safety, and tolerance. Here, we review current concepts of MS therapies, ...

journal_title：Journal of neurology

authors： Buck D,Hemmer B

更新日期：2011-10-01 00:00:00

abstract：:Multifocal motor neuropathy (MMN) can be differentiated from motor neuron disease by electrophysiological evidence of conduction block. To increase the probability of recording conduction block, we studied the whole nerve length including proximal segments in 84 patients with pure motor syndromes, using a special stim...

journal_title：Journal of neurology

authors： Jaspert A,Claus D,Grehl H,Neundörfer B

更新日期：1996-10-01 00:00:00

abstract：OBJECTIVE:To assess the association of Medial Temporal lobe Atrophy (MTA) and White Matter Hyperintensities (WMHs) with gray matter perfusion in Mild Cognitive Impairment (MCI). METHODS:56 MCI patients (age = 69.3 +/- 7.0, 32 females) underwent brain MR scan and (99m)Tc ECD SPECT. We evaluated MTA according to Schelte...

journal_title：Journal of neurology

authors： Caroli A,Testa C,Geroldi C,Nobili F,Guerra UP,Bonetti M,Frisoni GB

更新日期：2007-08-01 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 MS-ID (Multiple Sclerosis Information Dividend) project was initiated by the European Multiple Sclerosis Platform (EMSP) in 2007 in order to identify and address major inequalities of MS treatment and care and thus eliminate disparities across the EU. One major approach to reach these goals in the longer term is t...

journal_title：Journal of neurology

authors： Flachenecker P,Khil L,Bergmann S,Kowalewski M,Pascu I,Pérez-Miralles F,Sastre-Garriga J,Zwingers T

更新日期：2010-10-01 00:00:00

abstract：:Degeneration of central dopaminergic neurons is the most characteristic pathological feature of Parkinson's disease. It remains to be established to what extent these lesions explain the motor, cognitive and affective disorders observed in patients, but there are now some interesting clues to a possible solution. ...

journal_title：Journal of neurology

authors： Dubois B,Pillon B,Agid Y

更新日期：1992-01-01 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：:Disease management is defined as any medical or pharmaceutical intervention designed to improve both outcomes for the patient and overall cost-effectiveness of the health plan. Disease management focuses on the patient throughout the entire course of the disease, involving both health providers and third-party payers....

journal_title：Journal of neurology

authors： Matheron L,Barrau K,Blin O

更新日期：1998-08-01 00:00:00

abstract：:Whereas the effect of interferons (IFNs) on magnetic resonance imaging (MRI) outcome measures in patients with multiple sclerosis (MS) has been convincingly shown, little work has been done to define the between-patient heterogeneity of treatment response. Our aim was to assess the distribution of the effect of IFNbet...

journal_title：Journal of neurology

authors： Sormani MP,Bruzzi P,Beckmann K,Kappos L,Miller DH,Polman C,Pozzilli C,Thompson AJ,Wagner K,Filippi M

更新日期：2005-12-01 00:00:00

abstract：:There have been few reports concerning the characteristics of cerebral infarction associated with migraine (CIAM), and especially about the subsequent fate of these patients. We studied 14 patients (9 female) with CIAM. In all these patients the onset of cerebral infarction was accompanied by a unilateral throbbing he...

journal_title：Journal of neurology

authors： Hoekstra-van Dalen RA,Cillessen JP,Kappelle LJ,van Gijn J

更新日期：1996-07-01 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：:Idiopathic hypereosinophilic syndrome (IHES) is a primary haematological condition characterised by persistent, otherwise unexplained hypereosinophilia sufficient to cause organ damage. Various neurological complications are reported, but very few have mentioned CNS pathology and none has included CNS vasculitis. Our ...

journal_title：Journal of neurology

authors： Rice CM,Kurian KM,Renowden S,Whiteway A,Price C,Scolding NJ

更新日期：2015-05-01 00:00:00

abstract：:Movement-associated cortical potentials (MP) associated with thumb-opposition or fist-clenching were studied in 20 patients with unilateral cerebral lesions and in 8 healthy subjects. MP was abnormal in 16 patients. The most frequent abnormality was an attenuation of the N component or readiness potential, recorded on...

journal_title：Journal of neurology

authors： Shibasaki H

更新日期：1975-07-02 00:00:00

abstract：:Ten female patients suffering from catamenial epilepsy were treated with a synthetic analogue of the gonadotrophin releasing hormone (GnRH) in addition to antiepileptic drugs. Three of the patients became seizure free, in four patients seizure frequency decreased and in one patient seizures were of shorter duration. I...

journal_title：Journal of neurology

authors： Bauer J,Wildt L,Flügel D,Stefan H

更新日期：1992-05-01 00:00:00

abstract：:We performed the first population-based case-control study on clinical risk factors and drug exposure in Guillain-Barré syndrome (GBS). Sixty patients with GBS were collected through an incidence survey performed in the Emilia-Romagna region of northern Italy. GBS patients were compared with 109 hospital controls (HC)...

journal_title：Journal of neurology

authors： D'Alessandro R,Casmiro M,Guarino M

更新日期：1999-11-01 00:00:00

abstract：:Aquaporin-4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorder (NMOSD) is a rare but often severe autoimmune disease with median onset around 40 years of age. We report characteristics of three very-late-onset NMOSD (including complete NMO) patients >75 years of age, in whom this diagnosis initially se...

journal_title：Journal of neurology

authors： Krumbholz M,Hofstadt-van Oy U,Angstwurm K,Kleiter I,Jarius S,Paul F,Aktas O,Buchholz G,Kern P,Straube A,Kümpfel T

更新日期：2015-05-01 00:00:00

abstract：:Postpartum lower limb motor and/or sensory deficit is an uncommon obstetrical complication. We aimed to identify its incidence, etiology, and precipitating factors, as well as the neurological prognosis by retrospectively analyzing the successive neurological evaluations, electrophysiological, and MRI data from all th...

journal_title：Journal of neurology

authors： Richard A,Vellieux G,Abbou S,Benifla JL,Lozeron P,Kubis N

更新日期：2017-03-01 00:00:00

abstract：:The greatest unmet need in multiple sclerosis (MS) are treatments that delay, prevent or reverse progression. One of the most tractable strategies to achieve this is to therapeutically enhance endogenous remyelination; doing so restores nerve conduction and prevents neurodegeneration. The biology of remyelination-cent...

journal_title：Journal of neurology

authors： Cunniffe N,Coles A

更新日期：2021-01-01 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：:Fabry disease (FD) is a rare inherited disorder of the metabolism, associated with renal, cardiac, and cerebrovascular complications. Ischemic and hemorrhagic stroke in FD present with a similar proportion to that observed in the general population, but usually at an early age. Ischemic stroke may result from cardiac ...

journal_title：Journal of neurology

authors： Viana-Baptista M

更新日期：2012-06-01 00:00:00