Predicting daytime sleepiness and fatigue: a 9-year prospective study in myotonic dystrophy type 1
This study aimed to determine factors that may influence levels of daytime sleepiness and fatigue in a large cohort of DM1 patients followed for 9 years.MethodsThis study included 115 patients with DM1 at baseline (Time 1,T1) and at Time 2 (T2) who were questioned for daytime sleepiness, fatigue, history of depression, psychological distress, pain, hypothyroidism, and sleep habits. Also, their muscular impairment and intellectual quotient were evaluated. Regression models were used to identify correlates of daytime sleepiness and fatigue while controlling for time effect.ResultsBoth daytime sleepiness and fatigue incr...
Source: Journal of Neurology - January 29, 2020 Category: Neurology Source Type: research

Elevated blood glucose is associated with aggravated brain edema in acute stroke
AbstractBackground and purposeClinical outcome after endovascular thrombectomy in patients with acute ischemic stroke still varies significantly. Higher blood glucose levels (BGL) have been associated with worse clinical outcome, but the pathophysiological causes are not yet understood. We hypothesized that higher levels of BGL are associated with more pronounced ischemic brain edema and worse clinical outcome mediated by cerebral collateral circulation.Methods178 acute ischemic stroke patients who underwent mechanical thrombectomy were included. Early ischemic brain edema was determined using quantitative lesion water upt...
Source: Journal of Neurology - January 29, 2020 Category: Neurology Source Type: research

18 F-AV1451 PET imaging and multimodal MRI changes in progressive supranuclear palsy
We examined the relationship between tau pathology (assessed via18F-AV1451 PET) and multimodal MRI imaging using GM volume, cortical thickness (CTh), and diffusion tensor imaging (DTI).MethodsTwenty-three people with clinically probable PSP-Richardson ’s syndrome (age 68.8 ± 5.8 years, 39% female) and 23 controls underwent structural 3 T brain MRI including DTI. Twenty-one patients also had18F-AV1451 PET imaging. Voxelwise volume-based morphometry, surface-based morphometry, and DTI correlations were performed with18F-AV1451 binding in typical PSP regions of interest (putamen, thalamus ...
Source: Journal of Neurology - January 29, 2020 Category: Neurology Source Type: research

Central nervous system disorders after hematopoietic stem cell transplantation: a prospective study of the Infectious Diseases Working Party of EBMT
AbstractWe performed a prospective study to evaluate the types and characteristics of central nervous system (CNS) disorders in patients after hematopoietic stem cell transplantation. The study included 163 episodes of CNS disorders of which 58 (36%) were infections. Proven or probable infections were documented in 34 patients and included fungi (n = 10, 29%), viruses (n = 12, 35%),Toxoplasma spp. (n = 9, 27%) and bacteria (n = 3, 9%). Non-infectious neurological disorders (n = 105, 64%) frequently encompassed metabolic/drug-induced abnormalities (n&thin...
Source: Journal of Neurology - January 29, 2020 Category: Neurology Source Type: research

Non-motor symptoms are relevant and possibly treatable in hereditary spastic paraplegia type 4 (SPG4)
AbstractHereditary spastic paraplegias (HSP) share as cardinal feature progressive spastic gait disorder. SPG4 accounts for about 25% of cases and is caused by mutations in theSPAST gene. Although HSP is an upper motor neuron disease, the relevance of non-motor symptoms is increasingly recognized because of the potential response to treatment. Our study sets out to evaluate non-motor symptoms and their relevance with regard to health-related quality of life. In 118 genetically confirmed SPG4 cases and age- and gender-matched controls, validated questionnaires were used to evaluate fatigue, depression, pain, and restless le...
Source: Journal of Neurology - January 29, 2020 Category: Neurology Source Type: research

Cerebellum and cognition in Friedreich ataxia: a voxel-based morphometry and volumetric MRI study
ConclusionsWith two different image analysis techniques, we confirmed the presence of cerebellar volume loss in FRDA, mainly affecting the posterior lobe. In particular, Lobule IX atrophy correlated with worse visuo-spatial abilities, further expanding our knowledge about the physiopathology of cognitive impairment in FRDA. (Source: Journal of Neurology)
Source: Journal of Neurology - January 29, 2020 Category: Neurology Source Type: research

Concomitant reversible cerebral vasoconstriction syndrome and transient global amnesia
ConclusionsRCVS and TGA can occur simultaneously, which suggests common mechanisms such as aberrant responses to physical or emotional stress and cerebral vasoconstriction. (Source: Journal of Neurology)
Source: Journal of Neurology - January 29, 2020 Category: Neurology Source Type: research

Levels of 7alpha-hydroxycholesterol and/or 7alpha-hydroxy-4-cholest-3-one are the optimal biochemical markers for the evaluation of treatment of cerebrotendinous xanthomatosis
(Source: Journal of Neurology)
Source: Journal of Neurology - January 29, 2020 Category: Neurology Source Type: research

Stefan Borowiecki (1881 –1937)
(Source: Journal of Neurology)
Source: Journal of Neurology - January 29, 2020 Category: Neurology Source Type: research

Menstrual migraine: what it is and does it matter?
AbstractThe diagnostic criteria of menstrual migraine (MM), migraine related to menstruation and pure menstrual migraine, are placed in the appendix of the International Classification of Headache Disorders and are still primarily considered as research criteria that need validation. Although there is a great wealth of knowledge about the neurobiological processes underlying MM and its symptoms, the mechanisms by which an attack starts during the menstrual cycle remain baffling, and the disease is still undertreated. In this narrative review, we aim to summarize recent data on pathophysiology, epidemiology, burden of disea...
Source: Journal of Neurology - January 28, 2020 Category: Neurology Source Type: research

Correction to: Do pain, anxiety and depression influence quality of life for people with amyotrophic lateral sclerosis/motor neuron disease? A national study reconciling previous conflicting literature
The original version of this article unfortunately contained a mistake. Oliver Hanemann name was incorrect in the in the acknowledgements section of this paper. (Source: Journal of Neurology)
Source: Journal of Neurology - January 28, 2020 Category: Neurology Source Type: research

Gastrointestinal dysfunction in Parkinson ’s disease
ConclusionsPD patients report more prevalent and severe GI dysfunction, although our cohort comprised of many later-stage participants. Earlier recognition of GI dysfunction in PD provides the opportunity to direct treatment for chronic pain and constipation, promote physical activity and rationalise PD therapies for optimal patient care. (Source: Journal of Neurology)
Source: Journal of Neurology - January 27, 2020 Category: Neurology Source Type: research

Bernard Sachs (1858 –1944)
(Source: Journal of Neurology)
Source: Journal of Neurology - January 25, 2020 Category: Neurology Source Type: research

Microalbuminuria: a sentinel of neurocognitive impairment in HIV-infected individuals?
ConclusioncART-treated PLHIVs with a history of microalbuminuria subsequently had worse cognitive performances for the information-processing-speed domain, possibly because of CSVD. Our observations should be considered preliminary findings of a temporal link between microalbuminuria, CSVD, and subsequent cognitive impairment. (Source: Journal of Neurology)
Source: Journal of Neurology - January 24, 2020 Category: Neurology Source Type: research

Functional disconnection of the dentate nucleus in essential tremor
In this study, we evaluated DN functional connectivity (FC) changes and their relationship with motor and non-motor symptoms in ET. We studied 25 ET patients and 26 healthy controls. Tremor severity was assessed using the Fahn –Tolosa–Marin tremor rating scale (FTM-TRS) and tremor amplitude and frequency were evaluated using kinematic techniques. Cognitive profile was assessed by montreal cognitive assessment (MoCA) and frontal assessment battery (FAB). All participants underwent a 3 T MRI protocol including resting- state blood oxygenation level dependent and diffusion tensor sequences. We used a seed-bas...
Source: Journal of Neurology - January 23, 2020 Category: Neurology Source Type: research

Precision medicine in Parkinson ’s disease: emerging treatments for genetic Parkinson’s disease
AbstractIn recent years, numerous clinical trials for disease modification in Parkinson ’s disease (PD) have failed, possibly because of a “one-size-fits all” approach. Alternatively, a precision medicine approach, which customises treatments based on patients’ individual genotype, may help reach disease modification. Here, we review clinical trials that target genetic forms of PD, i.e.,GBA-associated andLRRK2-associated PD. In summary, six ongoing studies which explicitely recruitGBA-PD patients, and two studies which recruitLRRK2-PD patients, were identified. Available data on mechanisms of action...
Source: Journal of Neurology - January 23, 2020 Category: Neurology Source Type: research

The outcome of acute functional neurological disorder: a meta-analysis of stroke-mimic presentations
AbstractObjectiveThe prognosis of motor functional neurological disorder (FND) is considered poor; however, all data come from chronic cohorts collected by specialists. The aim of this study was to determine the clinical outcome of acute FND, captured as ‘stroke-mimics’.MethodsA meta-analysis of studies with outcome data on functional stroke mimics. Authors were contacted to provide more information on their FND mimics, including baseline, discharge and follow-up stroke scales. A random effects meta-analysis of proportion of ‘good outcome’ was the primary measure of interest, with additional descrip...
Source: Journal of Neurology - January 22, 2020 Category: Neurology Source Type: research

Efficacy and safety of mechanical thrombectomy for M2 segment of middle cerebral artery: a systematic review and meta-analysis
AbstractBackground and purposeThe efficacy and safety of mechanical thrombectomy (MT) for M2 segment occlusion of middle cerebral artery in patients with acute ischemic stroke (AIS) was investigated.MethodsWe searched PubMed and EMBASE from inception to 16 April 2019 for relevant studies, calculated the pool relative risks (RRs) of 3-month functional independence (modified Rankin scale score 0 –2), death and symptomatic intracerebral hemorrhage (sICH) in MT for M2 segment occlusion in patients with AIS versus those of M1 segment or best medical care.ResultsNine studies enrolling 2152 patients compared MT for patients...
Source: Journal of Neurology - January 22, 2020 Category: Neurology Source Type: research

Effective long-term treatment with incobotulinumtoxin (Xeomin ®) without neutralizing antibody induction: a monocentric, cross-sectional study
ConclusionsThe present study confirms the low antigenicity of incoBoNT/A, which has immediate consequences for patient management, and the use of higher doses and shorter durations of reinjection intervals in botulinum toxin therapy. (Source: Journal of Neurology)
Source: Journal of Neurology - January 20, 2020 Category: Neurology Source Type: research

Possible N -methyl- D -aspartate receptor antibody-mediated encephalitis in the setting of HIV cerebrospinal fluid escape
We report two adults infected with HIV presenting with encephalopathy and seizures. Case 1 had a monophasic encephalopathy with detection of NMDAR antibodies in the context of HIV CSF escape. There was a clinical response to immunotherapy and anti-retroviral therapy adjustment. Case 2 initially presented in non-convulsive status epilepticus associated with HIV CSF escape. He responded to treatment with anti-epileptic drugs and anti-retroviral therapy alteration, but had two further neurological relapses. NMDAR antibodies were detected during the relapses and a clinical response was observed following treatment with immunot...
Source: Journal of Neurology - January 20, 2020 Category: Neurology Source Type: research

Single mean arterial blood pressure drops during stroke thrombectomy under general anaesthesia are associated with poor outcome
We examined the influence of periprocedural blood pressure (BP), especially critical BP drops, on 3-month functional outcome in stroke patients undergoing mechanical thrombectomy (MT) under general anaesthesia (GA).MethodsWe screened all patients with anterior circulation large vessel occlusion receiving MT under GA at our centre from January 2011 to June 2016 and selected those who had continuous invasive periinterventional BP monitoring. Clinical and radiological data were prospectively collected as part of an ongoing cohort study, monitoring data were extracted from electronic anaesthesia records. We used uni- and multi...
Source: Journal of Neurology - January 18, 2020 Category: Neurology Source Type: research

Cognitive and behavioral status in Japanese ALS patients: a multicenter study
ConclusionsConsidering the high prevalence of in particular cognitive impairment, and the diversity of impairments, the cognitive and behavioral aspects of Japanese ALS patients should be given more attention clinically. (Source: Journal of Neurology)
Source: Journal of Neurology - January 18, 2020 Category: Neurology Source Type: research

Severe Lyme neuroborreliosis with bilateral hemorrhagic temporal encephalitis
(Source: Journal of Neurology)
Source: Journal of Neurology - January 18, 2020 Category: Neurology Source Type: research

Complications in cranioplasty after decompressive craniectomy: timing of the intervention
ConclusionCranioplasty has high complication rates, 23% in our cohort. Contrary to recent systematic reviews, early CP was associated with more complications (41%), explained by the higher incidence of pre-CP CSF flow disturbance and acute subdural hematoma as etiology of DC. CP in such patients should therefore be performed with highest caution. (Source: Journal of Neurology)
Source: Journal of Neurology - January 17, 2020 Category: Neurology Source Type: research

White matter and cerebellar involvement in alternating hemiplegia of childhood
AbstractObjectiveTo determine whether brain volumetric and white matter microstructural changes are present and correlate with neurological impairment in subjects with alternating hemiplegia of childhood (AHC).MethodsIn this prospective single-center study, 12 AHC subjects (mean age 22.9  years) and 24 controls were studied with 3DT1-weighted MR imaging and high angular resolution diffusion imaging at 3T. Data obtained with voxel-based morphometry and tract-based spatial statistics were correlated with motor impairment using the International Cooperative Ataxia Rating Scale (ICARS) and Movement and Disability sub-scal...
Source: Journal of Neurology - January 16, 2020 Category: Neurology Source Type: research

Correction to: Differentiating societal costs of disability worsening in multiple sclerosis
The original version of this article unfortunately contained a mistake. The captions of Fig.  2 and Fig. 3 are mismatched. (Source: Journal of Neurology)
Source: Journal of Neurology - January 16, 2020 Category: Neurology Source Type: research

Brain MRI of multiple system atrophy of cerebellar type: a prospective study with implications for diagnosis criteria
AbstractAimThe second consensus statement for the diagnosis of multiple system atrophy type cerebellar (MSA-C) includes pons and middle cerebellar peduncle (MCP) atrophy as MRI features. However, other MRI abnormalities such as MCP hyperintensity, hot cross bun sign (HCB), putaminal hypointensity and hyperintense putaminal rim have been described.ObjectivesTo evaluate, in patients with sporadic late-onset cerebellar ataxia (SLOCA), the discriminative value of several MRI features for the diagnosis of MSA-C, to follow their evolution during the course of MSA-C, and to search for correlations between these MRI features and c...
Source: Journal of Neurology - January 14, 2020 Category: Neurology Source Type: research

A pooled meta-analysis of GPi and STN deep brain stimulation outcomes for cervical dystonia
AbstractObjectiveTo analyze deep brain stimulation (DBS) outcomes in patients with cervical dystonia (CD), the relationships between motor and disability/pain outcomes, and the differences in outcomes between globus pallidus internus (GPi) and subthalamic nucleus (STN) DBS, and to identify potential outcome predictors.MethodsA systematic literature search identified individual patient data of CD patients who underwent DBS and whose outcomes were assessed with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Then, we performed a pooled meta-analysis on this cohort.ResultsA review of 39 papers yielded 208 pat...
Source: Journal of Neurology - January 14, 2020 Category: Neurology Source Type: research

ALS-derived fibroblasts exhibit reduced proliferation rate, cytoplasmic TDP-43 aggregation and a higher susceptibility to DNA damage
ConclusionsDermic-derived ALS fibroblasts recapitulate relevant cellular features of sALS and show a higher susceptibility to DNA damage and defective DNA repair responses. Altogether, these results support that dermic fibroblasts may represent a convenient and accessible ALS cellular model to study pathogenetic mechanisms, particularly those related to DNA damage response, as well as the eventual response to disease-modifying therapies. (Source: Journal of Neurology)
Source: Journal of Neurology - January 14, 2020 Category: Neurology Source Type: research

Questioning the existence of monophasic neuromyelitis optica spectrum disorder by defining a novel long-term relapse-free form from a large Chinese population
AbstractObjectiveTo clarify the existence of monophasic neuromyelitis optica spectrum disorders (NMOSD) and to identify predictive factors of long-term relapse-free form.MethodsWe retrospectively analyzed 289 Chinese patients with NMOSD. Selected subjects were divided into three groups based on the time interval between disease onset and the first relapse, if any. Clinical and imaging data were acquired from each patient ’s medical record and evaluated as predictive factors for NMOSD.ResultsIn total, none of the participating patients exhibited a monophasic form of NMOSD. Rather, 241 patients were selected for relaps...
Source: Journal of Neurology - January 13, 2020 Category: Neurology Source Type: research

Clinical features and prognosis of late-onset neuromyelitis optica spectrum disorders in a Latin American cohort
AbstractBackgroundWe aimed to assess the clinical, paraclinical, imaging and prognostic features of patients with late-onset neuromyelitis optica spectrum disorder (LO-NMOSD; ≥ 50 years at disease onset) LO-NMOSD, compared with early onset-NMOSD (EO-NMOSD, ≤ 49 years at disease onset), in Latin American (LATAM).MethodsWe retrospectively reviewed the medical records of patients with NMOSD, as defined using the 2015 validated diagnostic criteria. We included patients from Argentina, Brazil and Venezuela. They were divided into: LO-NMOSD and EO-NMOSD and comparison among the groups were performed...
Source: Journal of Neurology - January 13, 2020 Category: Neurology Source Type: research

Quantity and quality of gait and turning in people with multiple sclerosis, Parkinson ’s disease and matched controls during daily living
AbstractClinical trials need to specify which specific gait characteristics to monitor as mobility measures for each neurological disorder. As a first step, this study aimed to investigate a set of measures from daily-life monitoring that best discriminate mobility between people with multiple sclerosis (MS) and age-matched healthy control subjects (MS-Ctl) and between people with Parkinson ’s disease (PD) and age-matched healthy control subjects (PD-Ctl). Further, we investigated how these discriminative measures relate to the disease severity of MS or PD. We recruited 13 people with MS, 21 MS-Ctl, 29 people with id...
Source: Journal of Neurology - January 11, 2020 Category: Neurology Source Type: research

Predicting the antiepileptic drug response by brain connectivity in newly diagnosed focal epilepsy
AbstractObjectiveGrowing evidence has suggested that epilepsy is a disease with alterations in brain connectivity. The aim of this study was to investigate whether the changes in brain connectivity can predict the response to an antiepileptic drug (AED) in patients with a newly diagnosed focal epilepsy of unknown etiology.MethodsThis observational study was independently performed at two tertiary hospitals (Group A and B). Thirty-eight patients with newly diagnosed focal epilepsy of unknown etiology were enrolled in Group A and 46 patients in Group B. We divided these patients into two groups according to their seizure con...
Source: Journal of Neurology - January 10, 2020 Category: Neurology Source Type: research

Effectiveness, retention, and safety of modified ketogenic diet in adults with epilepsy at a tertiary-care centre in the UK
This study demonstrates that MKD can be effective in adults, although, even with regular dietetic support, retention rates remain low, and periods of worsening seizure frequency are common. (Source: Journal of Neurology)
Source: Journal of Neurology - January 10, 2020 Category: Neurology Source Type: research

Placebo and nocebo phenomena in anti- CGRP monoclonal antibody trials for migraine prevention: a meta-analysis
AbstractHigh placebo and low nocebo phenomena mirror high positive expectations for a novel treatment, among other reasons. In a meta-analysis aimed to identify placebo and nocebo phenomena in the placebo-controlled randomized trials (RCTs) with the monoclonal antibodies targeting the calcitonin gene-related peptide pathway (anti-CGRP mAbs) all the placebo-treated patients were pooled and compared with the placebo-treated patients in RCTs with topiramate and onabotulinum toxin A (OBTA). In episodic migraine (EM), the proportion of placebo-treated patients who achieved the 50% responder rate (placebo) was 32.7% (95% CI 28.6...
Source: Journal of Neurology - January 9, 2020 Category: Neurology Source Type: research

Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia
AbstractAlien limb refers to movements that seem purposeful but are independent of patients ’ reported intentions. Alien limb often co-occurs with apraxia in the corticobasal syndrome, and anatomical and phenomenological comparisons have led to the suggestion that alien limb and apraxia may be causally related as failures of goal-directed movements. Here, we characterised the nature of a lien limb symptoms in patients with the corticobasal syndrome (n = 30) and their relationship to limb apraxia. Twenty-five patients with progressive supranuclear palsy Richardson syndrome served as a disease control gro...
Source: Journal of Neurology - January 9, 2020 Category: Neurology Source Type: research

Systematic review of the clinical spectrum of CASPR2 antibody syndrome
ConclusionsMost patients have autoimmune or limbic encephalitis and corresponding abnormalities on neuroimaging. Other presentations include peripheral nerve hyperexcitability or Morvan syndromes, cerebellar syndromes, behavioral and cognitive changes, and more rarely movement disorders. The most commonly associated malignancy was thymoma and suggests a role for thymoma screening in CASPR2-related diseases. (Source: Journal of Neurology)
Source: Journal of Neurology - January 7, 2020 Category: Neurology Source Type: research

Restless legs syndrome: causes and consequences
(Source: Journal of Neurology)
Source: Journal of Neurology - January 7, 2020 Category: Neurology Source Type: research

Expert consensus recommendations to improve diagnosis of ATTR amyloidosis with polyneuropathy
AbstractAmyloid transthyretin (ATTR) amyloidosis with polyneuropathy (PN) is a progressive, debilitating, systemic disease wherein transthyretin protein misfolds to form amyloid, which is deposited in the endoneurium. ATTR amyloidosis with PN is the most serious hereditary polyneuropathy of adult onset. It arises from a hereditary mutation in theTTR gene and may involve the heart as well as other organs. It is critical to identify and diagnose the disease earlier because treatments are available to help slow the progression of neuropathy. Early diagnosis is complicated, however, because presentation may vary and family his...
Source: Journal of Neurology - January 6, 2020 Category: Neurology Source Type: research

Dynamics of impulsive –compulsive behaviors in early Parkinson’s disease: a prospective study
ConclusionsPD patients showed increasing frequency of most ICBs during the 5-year follow-up. Specific risk factors were identified for different types of ICBs. (Source: Journal of Neurology)
Source: Journal of Neurology - January 4, 2020 Category: Neurology Source Type: research

Sensory trick phenomenon in cervical dystonia: a functional MRI study
This study suggests a hyper-connectivity of sensorimotor areas during resting state in CD-no-trick subjects. In CD-trick patients, the sensory trick performance was associated with a decreased connectivity of the sensorimotor network. The increased activation of cerebellum in CD-trick patients during the sensory trick imagination suggests a possible role of this area in modulating cortical activity. (Source: Journal of Neurology)
Source: Journal of Neurology - January 2, 2020 Category: Neurology Source Type: research

Brain activity during lower limb movements in Parkinson ’s disease patients with and without freezing of gait
This study revealed the presence of two different patterns of brain activity during a foot movement task in PD-FoG and P D-noFoG patients, suggesting a possible compensatory role of parieto-occipital networks to overcome the fronto-striatal failure in PD-FoG cases. (Source: Journal of Neurology)
Source: Journal of Neurology - January 2, 2020 Category: Neurology Source Type: research

Vertebral artery dissection from neck self-manipulation presenting with acute severe bilateral hearing loss
(Source: Journal of Neurology)
Source: Journal of Neurology - January 1, 2020 Category: Neurology Source Type: research

Correction to: Longitudinal multi-modal muscle-based biomarker assessment in motor neuron disease
The original version of this article unfortunately contained a mistake. The numbers  in the Table 5 appear to have been reproduced wrongly as dates, rather than percentages. (Source: Journal of Neurology)
Source: Journal of Neurology - January 1, 2020 Category: Neurology Source Type: research

Correction to: Risk factors for lymphopenia in patients with relapsing –remitting multiple sclerosis treated with dimethyl fumarate
Unfortunately, the given name and family name of first author was incorrectly tagged in the xml data, therefore it is abbreviated wrongly as “Morales FS” in Pubmed. The correct given name is Fabian and family name is Sierra Morales. Auhtor name should be abbreviated as Sierra Morales F. (Source: Journal of Neurology)
Source: Journal of Neurology - January 1, 2020 Category: Neurology Source Type: research

Cardiovascular autonomic testing in the work-up of cerebellar ataxia: insight from an observational single center study
ConclusionsA complete cardiovascular autonomic battery with head-up tilt can detect early signs of BP dysregulation which may be missed at bed-side tests, thus warranting its application in the first line work-up of cerebellar ataxias. (Source: Journal of Neurology)
Source: Journal of Neurology - December 31, 2019 Category: Neurology Source Type: research

Correction to: Long-term follow-up of a large cohort with focal epilepsy of unknown cause: deciphering their clinical and prognostic characteristics
The original version of this article unfortunately contained a mistake. (Source: Journal of Neurology)
Source: Journal of Neurology - December 26, 2019 Category: Neurology Source Type: research

Disease-specific profiles of apathy in Alzheimer ’s disease and behavioural-variant frontotemporal dementia differ across the disease course
AbstractApathy is one of the most prevalent and disabling non-cognitive symptoms of dementia. This loss of motivation and pervasive decline in goal-directed behaviour represents a core diagnostic feature of behavioural-variant frontotemporal dementia (bvFTD) and is also common in Alzheimer ’s disease (AD). However, despite growing recognition of a multidimensional model, apathy has typically been examined as a unitary symptom. Here, we employed a cross-sectional design to characterise the multidimensional nature of apathy across syndromes and disease course. 92 participants (44 bvFT D, 20 AD, 28 controls) completed t...
Source: Journal of Neurology - December 23, 2019 Category: Neurology Source Type: research

Premature vascular disease in young adult stroke: a pathology-based case series
ConclusionYoung adult stroke patients with recurrent stroke, fatal stroke, or SVD on imaging have advanced atherosclerosis and arteriolosclerosis-related pathological changes in multiple organ systems. Aggressive control of atherosclerosis risk factors is warranted even in young individuals. (Source: Journal of Neurology)
Source: Journal of Neurology - December 18, 2019 Category: Neurology Source Type: research

Inotersen preserves or improves quality of life in hereditary transthyretin amyloidosis
ConclusionPatients with hATTR amyloidosis with polyneuropathy treated with inotersen showed preserved or improved QOL at 66  weeks compared to those who received placebo. (Source: Journal of Neurology)
Source: Journal of Neurology - December 18, 2019 Category: Neurology Source Type: research