Unexpected emergence from the vegetative state: delayed discovery rather than late recovery of consciousness.

abstract：:Acid maltase deficiency is described in non-identical adult twins. The onset of the disease can be traced into late infancy; the clinical picture is one of severe muscular dystrophy; respiratory insuficiency was the cause of death in one case. The autopsy showed the central nervous system, heart and liver to be spared...

journal_title：Journal of neurology

authors： Martin JJ,de Barsy T,den Tandt WR

更新日期：1976-08-06 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：BACKGROUND:Conflicting findings have been reported on the prognostic significance of serum 25-hydroxyvitamin D level in patients with stroke. The objective of this meta-analysis was to evaluate the prognostic utility of serum 25-hydroxyvitamin D in stroke patients. METHODS:PubMed and Embase databases were systematical...

journal_title：Journal of neurology

authors： Liu H,Wang J,Xu Z

更新日期：2020-11-01 00:00:00

abstract：:Discontinuation of natalizumab can lead to severe rebound of disease activity in patients with relapsing-remitting multiple sclerosis (RRMS); nevertheless, the treatment regimen in this clinical situation remains controversial. We report the case of a 25-year-old male patient with RRMS who was clinically stable under ...

journal_title：Journal of neurology

authors： Uphaus T,Oberwittler C,Groppa S,Zipp F,Bittner S

更新日期：2017-12-01 00:00:00

abstract：:The problem of making a decision in case of two clinical hypotheses is treated by the application of the Neyman-Pearson criterion. As an example, a pneumoencephalographic index (frontal horn width divided by septum-caudate distance) of two different diseases, Huntington's chorea and Parkinsonism, is analyzed. As the a...

journal_title：Journal of neurology

authors： Gath I

更新日期：1978-10-25 00:00:00

abstract：:We describe the case of a 40-year-old male patient who had presented clinically as primary lateral sclerosis for the past 10 years and neuropathologically as diffuse Lewy body disease (DLBD). Neuropathology demonstrated DLBD as an almost ubiquitous disorder of the neuronal cytoskeleton. ...

journal_title：Journal of neurology

authors： Hainfellner JA,Pilz P,Lassmann H,Ladurner G,Budka H

更新日期：1995-01-01 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：:Familial paroxysmal exercise-induced dyskinesia (PED) is a rare movement disorder that is mostly caused by mutations in the solute carrier family 2, member 1 (SLC2A1) gene and inherited in an autosomal dominant manner. Clinical, laboratory, and genetic studies were performed in three family members. The proband's symp...

journal_title：Journal of neurology

authors： Tacik P,Loens S,Schrader C,Gayde-Stephan S,Biskup S,Dressler D

更新日期：2014-10-01 00:00:00

abstract：:Treatment of multiple sclerosis (MS) with interferon beta (IFNbeta) can be associated with the development of binding antibodies (BAbs) and neutralizing antibodies (NAbs). NAbs are a subset of BAbs that prevent IFNbeta from effectively binding to or activating its receptor, thereby blocking its biologic effects and in...

journal_title：Journal of neurology

authors： Deisenhammer F,Schellekens H,Bertolotto A

更新日期：2004-06-01 00:00:00

abstract：:Cortical tubers are very common in tuberous sclerosis complex (TSC) and widely vary in size, appearance and location. The relationship between tuber features and clinical phenotype is unclear. The aim of the study is to propose a classification of tuber types along a spectrum of severity, using magnetic resonance imag...

journal_title：Journal of neurology

authors： Gallagher A,Grant EP,Madan N,Jarrett DY,Lyczkowski DA,Thiele EA

更新日期：2010-08-01 00:00:00

abstract：:Most inflammatory neuropathies, both acute and chronic, probably result from an immune attack against antigens of the peripheral nervous system. Specific antibodies in serum that react with the peripheral nervous system have been described in a number of inflammatory neuropathies. We review the pathophysiological sign...

journal_title：Journal of neurology

authors： Steck AJ

更新日期：2000-06-01 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：:Altered cerebral vasoreactivity (CVR) has been implicated in migraine. To test this hypothesis, we studied CVR as measured by transcranial Doppler ultrasound (TCD) in 11 migraineurs and 12 healthy controls of similar age. Mean flow velocities (MFV) in the middle cerebral artery (MCA) were recorded during a cognitive a...

journal_title：Journal of neurology

authors： Thie A,Carvajal-Lizano M,Schlichting U,Spitzer K,Kunze K

更新日期：1992-07-01 00:00:00

abstract：OBJECTIVE:Children with neuromuscular disorders have been assumed to be a particularly vulnerable population since the beginning of COVID-19. Although this is a plausible hypothesis, there is no evidence that complications or mortality rates in neuromuscular patients are higher than in the general population. The aim o...

journal_title：Journal of neurology

authors： Natera-de Benito D,Aguilera-Albesa S,Costa-Comellas L,García-Romero M,Miranda-Herrero MC,Rúbies Olives J,García-Campos Ó,Martínez Del Val E,Martinez Garcia MJ,Medina Martínez I,Cancho-Candela R,Fernandez-Garcia MA,Pascual-Pascual

更新日期：2021-01-02 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：:Although post-stroke emotional incontinence (EI) often occurs after lenticulocapsular strokes, what factors determine the development of EI in these patients has not been identified. I prospectively studied the development of EI in 25 patients (13 men and 12 women, mean age 58.5 years) with single, unilateral, first-e...

journal_title：Journal of neurology

authors： Kim JS

更新日期：2002-07-01 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：:Diminished activity of uroporphyrinogen I-synthetase in the liver and other tissues may be regarded to be the primary genetic deficiency of acute intermittent porphyria (AIP). Increased production and renal excretion of delta-aminolevulinic acid (ALA) und porphobilinogen (PBG) are secondary phenomena. The neuropsychia...

journal_title：Journal of neurology

authors： Flügel KA,Druschky KF

更新日期：1977-03-21 00:00:00

abstract：:A double-blind, placebo-controlled multicentre study was carried out to evaluate the efficacy and tolerability of 100, 200 and 300 mg sumatriptan, a selective 5-hydroxytryptamine (5-HT)1-like receptor agonist, given in an oral dispersible form in the acute treatment of migraine attacks. A total of 1130 patients were r...

journal_title：Journal of neurology

authors： Patten JP

更新日期：1991-01-01 00:00:00

abstract：OBJECTIVE:Determine toxicity and efficacy of autologous hematopoietic stem cell transplantation (HSCT) for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are dependent on intravenous immunoglobulins or plasmapheresis. METHODS:Unselected peripheral blood stem cells were re-infused on...

journal_title：Journal of neurology

authors： Burt RK,Balabanov R,Tavee J,Han X,Sufit R,Ajroud-Driss S,Jovanovic B,Quigley K,Arnautovic I,Helenowski I,Sharrack B

更新日期：2020-11-01 00:00:00

abstract：:The diagnosis and management of lesions of the brachial roots and of the brachial plexus is improved by appropriate investigation, both in acute and chronic disorders. The choice of investigation should be determined by the clinical problem. Since they are relatively non-invasive, electrophysiological investigations a...

journal_title：Journal of neurology

authors： Swash M

更新日期：1986-06-01 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：: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

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：:Whole genome screening is increasingly used to identify genetic risk factors for complex diseases. In this study, a genome wide linkage disequilibrium (LD) screen was performed in a cohort of Parkinson's disease (PD) patients from the UK (n = 195) using pooled DNA to facilitate efficient genotyping of 5546 microsatell...

journal_title：Journal of neurology

authors： Foltynie T,Hicks A,Sawcer S,Jonasdottir A,Setakis E,Maranian M,Yeo T,Lewis S,Brayne C,Stefansson K,Compston A,Gulcher J,Barker RA

更新日期：2005-05-01 00:00:00

abstract：:This article describes the clinical features of anterior semicircular canal benign paroxysmal positional vertigo (AC-BPPV) and a new therapeutic maneuver for its management. Our study was a retrospective review of cases from an ambulatory tertiary referral center. Thirteen patients afflicted with positional paroxysmal...

journal_title：Journal of neurology

authors： Yacovino DA,Hain TC,Gualtieri F

更新日期：2009-11-01 00:00:00

abstract：:Polysomnography (PSG) is considered the gold standard for diagnosis of non-rapid eye movement (NREM) parasomnias, however its diagnostic yield has been rarely reported. We aimed to assess the diagnostic value of polysomnography in different categories of patients with suspected NREM parasomnia and define variables tha...

journal_title：Journal of neurology

authors： Fois C,Wright MA,Sechi G,Walker MC,Eriksson SH

更新日期：2015-02-01 00:00:00

abstract：:In order to learn more on the occurrence of pains and motor deficit in severe diabetic polyneuropathy we reviewed the data of a series of 30 diabetic patients with an uncommonly severe length-dependent diabetic polyneuropathy (LDDP). Extensive sensory loss predominated with pains and temperature sensations and affecte...

journal_title：Journal of neurology

authors： Said G,Baudoin D,Toyooka K

更新日期：2008-11-01 00:00:00

abstract：OBJECTIVES:To estimate the socio-economic impact of multiple sclerosis (MS) in Italy. METHODS:Outpatients with MS were enrolled at 44 centres across Italy. Socio-demographic, clinical and resource utilization data were collected using a validated questionnaire. Each patient completed a weekly diary of expenses due to ...

journal_title：Journal of neurology

authors： Amato MP,Battaglia MA,Caputo D,Fattore G,Gerzeli S,Pitaro M,Reggio A,Trojano M,Mu. S. I. C. Study Group.

更新日期：2002-02-01 00:00:00

abstract：:Multiple system atrophy (MSA) manifests as a combination of dysautonomia and motor symptoms/signs. However, rare cases presenting with autonomic failures in absence of motor symptoms/signs until their deaths have been reported and are referred to as non-motor MSA. To clarify pathological findings underlying non-motor ...

journal_title：Journal of neurology

authors： Riku Y,Watanabe H,Mimuro M,Iwasaki Y,Ito M,Katsuno M,Sobue G,Yoshida M

更新日期：2017-11-01 00:00:00