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Recent items - Journal of Neurology, Neurosurgery & Psychiatry

Facial bradykinesia
07/05/2013 11:15
The aim of this paper is to summarise the main clinical and pathophysiological features of facial bradykinesia in Parkinson's disease (PD) and in atypical parkinsonism. Clinical observation suggests that reduced spontaneous and emotional facial expr
Prevalence of cognitive impairment in Chinese: Epidemiology of Dementia in Singapore study
07/05/2013 11:15
Objective To study the prevalence of and associated factors for cognitive impairment and dementia in community dwelling Chinese from Singapore. Methods This study includes Chinese subjects from the Epidemiology of Dementia in Singapore (EDIS) study
Association between cerebral microbleeds and cognitive function: a systematic review
07/05/2013 11:15
Background Cerebral microbleeds (MBs), defined as haemorrhagic microvascular lesions or microangiopathy in the brain, have traditionally been considered clinically silent. Recent studies, however, suggest that MBs are associated with a decline in co
Long-term benefits and adverse effects of intermittent versus daily glucocorticoids in boys with Duchenne muscular dystrophy
07/05/2013 11:15
Objective To assess the current use of glucocorticoids (GCs) in Duchenne muscular dystrophy in the UK, and compare the benefits and the adverse events of daily versus intermittent prednisolone regimens. Design A prospective longitudinal observation
Unilateral isolated hypoglossal nerve palsy associated with internal carotid artery dissection
07/05/2013 11:15
A 54-year-old healthy man was admitted to our hospital with acute dysarthria and mild impairment in swallowing and tongue movements, which he had noticed 2 days before. He was not taking any drugs, and his medical history was unremarkable exce
Impact of symptoms in patients with functional neurological symptoms on activities of daily living and health related quality of life
07/05/2013 11:15
When designing a randomised clinical study in patients with functional neurological symptoms (FNS) our problem was what the best primary outcome measure is in this group of patients: activities of daily living (ADL) or quality of life (QoL)? In pati
Does rest tremor exclude the diagnosis of adult-onset primary dystonia?
07/05/2013 11:15
The diagnosis of adult-onset primary dystonia (AOPD) is mainly clinical, but requires the exclusion of any secondary causes. AOPD is characterised by sustained involuntary muscle contraction resulting in one or more body parts moved away from their
Daily or alternative, that is the question: steroid therapy for Duchenne muscular dystrophy patients
07/05/2013 11:15
Daily steroid use brings longer ambulation time but also more adverse effects, except for orthopaedic complications Duchenne muscular dystrophy (DMD) is an X-linked muscular dystrophy known to affect one in 3600 live male births and leads to progres
Cerebral microbleeds and cognitive function
07/05/2013 11:15
The assessment of cerebral microbleeds should be included in the evaluation of patients with vascular cognitive dysfunction Cerebral microbleeds (CMB) are focal haemosiderin deposits that result from minimal blood leakage from damaged small vessels,
Eugen Bleuler and evidence-based psychiatry
07/05/2013 11:15
Not so much a pioneer of EVIDENCE BASE MEDICINE as a visionary for evidence-based psychiatry Stam and Vermeulen1 argue persuasively that Eugen Bleuler (1857–1939) is an unrecognised proponent of evidence-based medicine.2 Bleuler saw the self-a
Eugen Bleuler (1857-1939), an early pioneer of evidence based medicine
07/05/2013 11:15
The famous Swiss psychiatrist, Eugen Bleuler (figure 1), is well known for his seminal work on psychosis, for having coined the term ‘schizophrenia’ and for his disputes about psychoanalysis with Sigmund Freud. Less known is the fact tha
Recurrent transient ischaemic attack and early risk of stroke: data from the PROMAPA study
07/05/2013 11:15
Background Many guidelines recommend urgent intervention for patients with two or more transient ischaemic attacks (TIAs) within 7 days (multiple TIAs) to reduce the early risk of stroke. Objective To determine whether all patients with multip
Variations in acute stroke care and the impact of organised care on survival from a European perspective: the European Registers of Stroke (EROS) investigators
07/05/2013 11:15
Background The need for stroke care is escalating with an ageing population, yet methods to estimate the delivery of effective care across countries are not standardised or robust. Associations between quality and intensity of care and stroke outcom
CT perfusion improves diagnostic accuracy and confidence in acute ischaemic stroke
07/05/2013 11:15
Background and objective CT perfusion (CTP) is rapid and accessible for emergency ischaemic stroke diagnosis. The feasibility of introducing CTP and diagnostic accuracy versus non-contrast CT (NCCT) in a tertiary hospital were assessed. Methods All
Lifetime risks for aneurysmal subarachnoid haemorrhage: multivariable risk stratification
07/05/2013 11:15
Objective The overall incidence of aneurysmal subarachnoid haemorrhage (aSAH) in western populations is around 9 per 100 000 person-years, which confers to a lifetime risk of around half per cent. Risk factors for aSAH are usually expressed as
Enlarged perivascular spaces as a marker of underlying arteriopathy in intracerebral haemorrhage: a multicentre MRI cohort study
07/05/2013 11:15
Background and purpose Small vessel disease (mainly hypertensive arteriopathy and cerebral amyloid angiopathy (CAA)) is an important cause of spontaneous intracerebral haemorrhage (ICH), a devastating and still poorly understood stroke type. Enlarge
Memory outcome after hippocampus sparing resections in the temporal lobe
07/05/2013 11:15
Background and objective Epilepsy surgery within the temporal lobe of the language dominant hemisphere bears the risk of postoperative verbal memory decline. As surgical procedures have become more tailored, the question has arisen, which type of re
The prevalence of neurodevelopmental disorders in children prenatally exposed to antiepileptic drugs
07/05/2013 11:15
The aim of this study was to compare the prevalence of diagnosed neurodevelopmental disorders in children exposed, in utero, to different antiepileptic drug treatments. A prospective cohort of women with epilepsy and a control group of women without
Differentiation between idiopathic and atypical parkinsonian syndromes using three-dimensional magnetic resonance spectroscopic imaging
07/05/2013 11:15
Objectives Degeneration of dopaminergic neurons in the substantia nigra (SN) pars compacta is the primary cause of idiopathic Parkinson's disease (iPD). In early stages of disease in particular, presentation of symptoms is non-specific leading to di
The differential diagnosis of Huntington's disease-like syndromes: 'red flags' for the clinician
07/05/2013 11:15
A growing number of progressive heredodegenerative conditions mimic the presentation of Huntington's disease (HD). Differentiating among these HD-like syndromes is necessary when a patient with a combination of movement disorders, cognitive decline,
The long-term safety and efficacy of bilateral transplantation of human fetal striatal tissue in patients with mild to moderate Huntington's disease
07/05/2013 11:15
Huntington's disease (HD) is a fatal autosomal dominant neurodegenerative disease involving progressive motor, cognitive and behavioural decline, leading to death approximately 20 years after motor onset. The disease is characterised pathologic
The Val158Met COMT polymorphism is a modifier of the age at onset in Parkinson's disease with a sexual dimorphism
07/05/2013 11:15
The catechol-O-methyltranferase (COMT) is one of the main enzymes that metabolise dopamine in the brain. The Val158Met polymorphism in the COMT gene (rs4680) causes a trimodal distribution of high (Val/Val), intermediate (Val/Met) and low (Met/Met)
Autonomic dysfunction in parkinsonian disorders: assessment and pathophysiology
07/05/2013 11:15
Parkinson's disease (PD) is a progressive neurodegenerative disorder characterised by motor dysfunction (parkinsonism) and several non-motor features. Dysautonomia is a significant non-motor feature as well as a neuropsychiatric symptom. Autonomic d
Clinical risk predictors for cerebral hyperperfusion syndrome after carotid endarterectomy
09/04/2013 17:21
Background Cerebral hyperperfusion syndrome (CHS) is an important complication of carotid endarterectomy (CEA), yet prior research has been limited to small cohorts and retrospective analyses, or studies using radiographic rather than clinical defin
Clinical features of CIDP with LM1-associated antibodies
09/04/2013 17:21
Background LM1 is the predominant glycolipid in human peripheral nerve myelin and antibodies to LM1 and LM1-containing ganglioside complexes are detected in some patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The clinical fe
Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome
09/04/2013 17:21
In the 1950s, Bickerstaff and Fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous
Bilateral thoracic long nerve involvement in motor multifocal neuropathy
09/04/2013 17:21
A 20-year-old man presented with a 6-month history of progressive weakness in the right hand. Neurological examination showed a bilateral weakness of hand muscles more pronounced in the right hand, slight scapula winging and no sensory disturbances
Aspirin for acute migraine headaches in adults
09/04/2013 17:21
Clinical bottom line A single oral dose of aspirin 900 mg or 1000 mg is more effective than placebo at resolving migrainous headache pain at 2 h and providing relief that is sustained over 24 h.1 Co-administration of metocloprami
The chameleon syndrome: acute convergence paralysis
09/04/2013 17:21
A 78-year-old female patient without vascular risk factors presented with sudden left eye deviation at awakening. Neither diplopia nor other symptoms were reported. On admission, left eye exotropia at primary gaze position and bilateral internuclea
Olfactory impairment in posterior cortical atrophy
09/04/2013 17:21
Introduction Olfactory dysfunction develops in many neurodegenerative diseases, and is an early feature of the most common neurodegenerative disorder, Alzheimer's disease (AD).1–5 Anatomically, the central olfactory pathways traverse brain reg
High-sensitivity troponin assay improves prediction of cardiovascular risk in patients with cerebral ischaemia
09/04/2013 17:21
Background and purpose Clinical scores are recommended for predicting cardiovascular risk in patients with cerebral ischaemia to inform secondary prevention. Blood biomarkers may improve prediction beyond clinical scores. Methods Within the observa
Volume-dependent effect of perihaematomal oedema on outcome for spontaneous intracerebral haemorrhages
09/04/2013 17:21
Introduction It is still unknown whether subsequent perihaematomal oedema (PHE) formation further increases the odds of an unfavourable outcome. Methods Demographic, clinical, radiographic and outcome data were prospectively collected in a single l
Is language impairment more common than executive dysfunction in amyotrophic lateral sclerosis?
09/04/2013 17:21
Background Systematic explorations of language abilities in patients with amyotrophic lateral sclerosis (ALS) are lacking in the context of wider cognitive change. Methodology Neuropsychological assessment data were obtained from 51 patients with A
The NINDS-Canadian stroke network vascular cognitive impairment neuropsychology protocols in Chinese
09/04/2013 17:21
Background and purpose Vascular cognitive impairment (VCI) affects up to half of stroke survivors and predicts poor outcomes. Valid and reliable assessement for VCI is lacking, especially for the Chinese population. In 2005, the National Institute o
Clinical and cognitive correlates of visual hallucinations in dementia with Lewy bodies
09/04/2013 17:21
Background The presence of recurrent complex visual hallucinations (VHs) is a core feature of dementia with Lewy bodies (DLB). The aim of this study was to investigate which clinical and neuropsychological characteristics are associated with VHs and
Efficacy of mitoxantrone in neuromyelitis optica spectrum: clinical and neuroradiological study
09/04/2013 17:21
Objective To evaluate the efficacy of mitoxantrone (MTX) on clinical and neuroradiological parameters of patients who had a relapse of neuromyelitis optica spectrum (NMOS) within the 12 previous months. Methods MTX (12 mg/m2) combined with met
CSF high-mobility group box 1 is associated with intrathecal inflammation and astrocytic damage in neuromyelitis optica
09/04/2013 17:21
Objective High-mobility group box 1 (HMGB1) acts as a proinflammatory mediator when released by cells. Recent studies implicate extracellular HMGB1 in the pathogenesis of various autoimmune diseases. Our main aim of this study is to determine whethe
Transient global amnesia associated with bilateral restricted diffusion in the lateral hippocampus
09/04/2013 17:21
A 48-year-old man with no significant cardiovascular risk factors developed 3 h of severe anterograde and mild retrograde amnesia, repeatedly asking ‘Is this my coat?’ on emerging shivering from the (cold) English Channel. No other
Impaired social cognition in multiple sclerosis
09/04/2013 17:21
Background Multiple sclerosis (MS) is an inflammatory and neurodegenerative disorder of the CNS that is frequently associated with neuropsychiatric symptoms and decreased quality of life. Social support, which has been found to buffer the psychosoci
Long-term outcome of hemispheric surgery at different ages in 61 epilepsy patients
09/04/2013 17:21
Objective Hemispheric neurosurgery is an established treatment for severe epilepsy caused by extended unilateral brain pathology. However, it is still an unresolved question at which age surgery should best be performed. In light of decreasing plast
Cerebrospinal fluid amyloid-{beta} and phenotypic heterogeneity in de novo Parkinson's disease
09/04/2013 17:21
Background In Parkinson's disease (PD), the motor presentation characterised by postural instability/gait difficulties (PIGD) heralds accelerated motor, functional and cognitive decline, as compared with the more benign tremor-dominant (TD) variant.
The Addenbrooke's Cognitive Examination for the differential diagnosis and longitudinal assessment of patients with parkinsonian disorders
09/04/2013 17:21
Objective Differentiating idiopathic Parkinson's disease from atypical parkinsonian syndromes is challenging, especially in the early stages. We assessed whether the Revised Addenbrooke's Cognitive Examination (ACE-R) could differentiate between par
Memantine for axial signs in Parkinson's disease: a randomised, double-blind, placebo-controlled pilot study
09/04/2013 17:21
Background Given that memantine is thought to decrease N-methyl-D-aspartic-acid-related (NMDA) glutamatergic hyperactivity and improve locomotion in rats, we sought to assess the drug's impact on axial symptoms in advanced Parkinson's disease (PD).
Cortical atrophy in presymptomatic Alzheimer's disease presenilin 1 mutation carriers
09/04/2013 17:21
Background Sporadic late-onset Alzheimer's disease (AD) dementia has been associated with a ‘signature’ of cortical atrophy in paralimbic and heteromodal association regions measured with MRI. Objective To investigate whether a similar
The European iNPH Multicentre Study on the predictive values of resistance to CSF outflow and the CSF Tap Test in patients with idiopathic normal pressure hydrocephalus
09/04/2013 17:21
Objective The objective was to determine the sensitivity, specificity, and positive and negative predictive values of the CSF Tap Test (CSF TT) and resistance to CSF outflow (Rout) for the outcome of shunting in a sample of patients with idiopathic
Action discrimination: impact of apraxia
09/04/2013 17:21
Mariella Pazzaglia from the IRCCS Fondazione Santa Lucia and University of Rome ‘La Sapienza’, in Rome describes a rapidly growing body of clinical research on the complex interplay of both production and comprehension mechanisms in a
An old drug for a common disease
09/04/2013 17:21
Migraine is a common and frequently incapacitating neurological disease highly prevalent in people of working age, especially women. For this reason, a paper1 that summarises and highlights a systematic review that considers Aspirin, an old, inexpe
Executive dysfunction in ALS is not the whole story
09/04/2013 17:21
It is well established that selective cognitive impairments may be present in amyotrophic lateral sclerosis (ALS) patients who do not display a full blown frontotemporal dementia (FTD) syndrome.1 2 Over the last 20 years there has been a surge
Motor deficits associated with changes in {beta}-amyloid in Parkinson's disease
09/04/2013 17:21
β-Amyloid (Aβ) deposition in the brain is one of the main pathological hallmarks of Alzheimer's disease (AD), with dominant gene mutations significantly increasing the amount of this protein in the brain and heralding an early onset of dem
Isolated myositis of the superior oblique muscle
26/03/2013 11:42
A 34-year-old woman presented with a 10 day history of right-sided throbbing headaches and right retro-orbital pain with subacute onset. On the day of admission, she had additionally noticed binocular diplopia when looking upwards. Her past med
Tremor in primary adult-onset dystonia: prevalence and associated clinical features
26/03/2013 11:42
Objective To investigate the frequency and the main clinical features of tremor in primary adult-onset dystonia (PAOD). Methods This cross-sectional study was conducted on 429 patients with PAOD from eight Italian movement disorder centres. Result
Parkinson's disease subtypes: lost in translation?
26/03/2013 11:42
Like many neurodegenerative disorders, Parkinson's disease (PD) is clinically highly heterogeneous. A number of studies have proposed and defined subtypes of PD based on clinical features that tend to cluster together. These subtypes present an oppo
Central nervous system abnormalities in patients with PMP22 gene mutations: a prospective study
26/03/2013 11:42
Background Mutations of the peripheral myelin protein-22 (PMP22) gene are the most common cause of inherited disease of the peripheral nervous system (PNS), with its deletion resulting in hereditary neuropathy with liability to pressure palsies (HNP
Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF72
26/03/2013 11:42
Background A GGGGCC hexanucleotide repeat expansion in C9ORF72 occurs on a chromosome 9p21 locus that is linked with frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) in white populations. The diseases resulting from this expansi