Relationship between the strain measures of left atrial function and heart failure worsening.

Background: Two-dimensional speckle tracking evaluation (2D-STE) is a useful tool to evaluate the complexity of atrial function by the analysis of the different phases of atrial deformation and by the combination with Doppler measurements of diastolic function.

Aim Of The Study: To evaluate the role of the left atrial (LA) strain parameters to predict worsening chronic heart failure (CHF).

Methods: We enrolled outpatients affected by CHF referred to our heart failure unit.

Chronic obstructive pulmonary disease may complicate Alzheimer's disease: a comorbidity problem.

Background And Aim: Chronic obstructive pulmonary disease (COPD) may be associated with worsening of cognitive performance. We studied patients with Alzheimer's disease (AD) with and without COPD, and we analyzed, in a retrospective way, clinical and neuropsychological variables to verify if COPD plays a pejorative role on cognitive or functional autonomy in patients with dementia.

Methods: We enrolled 23 adult patients (AD-COPD) with probable AD and COPD and 23 with AD only (AD-only); they were matched for sex, age, educational level, and Mini Mental State Examination (MMSE) at the disease onset.

Frontotemporal Dementia Presenting as Gambling Disorder: When a Psychiatric Condition Is the Clue to a Neurodegenerative Disease.

Behavioral variant frontotemporal dementia, the most common form of frontotemporal dementia, is characterized by executive dysfunction and changes in personality and behavior, sometimes with associated psychiatric disorders. We report a man who suddenly developed a gambling disorder when he was 55 years old. A year later he developed personality changes of agitation, euphoria, and disinhibition, along with binge eating and dysthymia.

Direct proof of spontaneous translocation of lipid-covered hydrophobic nanoparticles through a phospholipid bilayer.

Hydrophobic nanoparticles introduced into living systems may lead to increased toxicity, can activate immune cells, or can be used as nanocarriers for drug or gene delivery. It is generally accepted that small hydrophobic nanoparticles are blocked by lipid bilayers and accumulate in the bilayer core, whereas big nanoparticles can only penetrate cells through slow energy-dependent processes, such as endocytosis, lasting minutes. In contrast to expectations, we demonstrate that lipid-covered hydrophobic nanoparticles may translocate through lipid membranes by direct penetration within milliseconds.

A quantitative assessment of chemical perturbations in thermotropic cyanobiphenyls.

Chemical programming of the temperature domains of existence of liquid crystals is greatly desired by both academic workers and industrial partners. This contribution proposes to combine empirical approaches, which rely on systematic chemical substitutions of mesogenic molecules followed by thermal characterizations, with a rational thermodynamic assessment of the effects induced by chemical perturbations. Taking into account the similarities which exist between temperature-dependent cohesive Gibbs free energy densities (CFEDs) and pressure-temperature phase diagrams modeled with the Clapeyron equation, chemical perturbations are considered as pressure increments along phase boundaries, which control the thermotropic liquid crystalline properties.

Chemical Programming of the Domain of Existence of Liquid Crystals.

This work illustrates how enthalpy and entropy changes responsible for successive phase transitions of cyanobiphenyl-based liquid crystals can be combined to give cohesive free energy densities. These new parameters are able to rationalize and quantify the demixing of the melting and clearing processes that occur in thermotropic liquid crystals. Minor structural variations at the molecular level can be understood as pressure increments that alter either the melting or clearing temperatures in a predictable way.

Abnormal postural reflexes in a patient with pontine ischaemia.

The control of body posture is a complex activity that needs a very close relationship between different structures, such as the vestibular system, and the muscle and joint receptors of the neck. Damage of even one of these structures can lead to abnormal postural reflexes. We describe a case of a woman with a left pontine ischaemia who developed a 'dystonic' extensor posture of the left limbs while turned on the right side.

Synthesis, characterization, and tuning of the liquid crystal properties of ionic materials based on the cyclic polyoxothiometalate [{Mo4O4S4(H2O)3(OH)2}2(P8W48O184)](36-).

A series of compounds resulting from the ionic association of a nanoscopic inorganic cluster of formula [K2NaxLiy{Mo4O4S4(OH)2(H2O)3}2(HzP8W48O184)]((34-x-y-z)-), 1, with several organic cations such as dimethyldioctadecylammonium DODA(+), trimethylhexadecylammonium TMAC16(+), alkylmethylimidazoliums mimCn(+) (n = 12-20) and alkyl-dimethylimidazoliums dmimCn(+) (n = 12 and 16) was prepared and characterized in the solid state by FT-IR, EDX, Elemental analysis, TGA and solid state NMR. The solid state NMR experiments performed on (1)H, (13)C and (31)P nuclei evidenced the interactions between the cations and 1 as well as the organization of the alkyl chains of the cations within the solid. Polarized optical microscopy, DSC and SA-XRD experiments implicated mesomorphic phases for DODA(+) and mimCn(+) salts of 1.

Early onset frontotemporal dementia with psychiatric presentation due to the C9ORF72 hexanucleotide repeat expansion: a case report.

Background: Frontotemporal dementia (FTD) may present with psychiatric symptoms, usually together with neurological ones and in cases with a family history of dementia. We describe the case of an FTD behavioural variant with a psychiatric presentation and a normal neurological examination, due to a C9Orf72 gene mutation.

Case Presentation: The patient was a 57 years-old Caucasian woman with a recent onset of bizarre behaviours and mystic delusions.

Tracking "apolar" NMe4+ ions within two polyoxothiomolybdates that have the same pores: smaller clathrate and larger highly porous clusters in action.

Two nanosized polyoxothiometalates were synthesized based on linking oxomolybdate building blocks with {Mo2O2S2}(2+) groups. Remarkably, both compounds are formed selectively primarily upon changing the related concentrations in a logical way; they exhibit common structural features based on the same {Mo9O6S3}-type pores, which result in connections between {Mo6O21} pentagons and {Mo2O2S2}(2+) linkers. Whereas the much larger spherical Mo132-type Keplerate contains twenty pores, the smaller Mo63 -type cluster remarkably contains only two.

Evidence of pre-synaptic dopaminergic deficit in a patient with a novel progranulin mutation presenting with atypical parkinsonism.

Parkinsonism can be the presenting feature of frontotemporal dementia due to Progranulin (GRN) mutations or develop over the course of the disease, mimicking idiopathic Parkinson's disease or atypical parkinsonism. Here we report on a patient carrying a novel GRN mutation who presented with asymmetric parkinsonism and developed cognitive decline and language alterations two years later. Brain MRI showed mild asymmetric fronto-parietal atrophy.

Adult osteomalacia: a treatable cause of "fear of falling" gait.

A 65-year-old man was hospitalized with a gait disorder, obliging him to shuffle laterally(1) (video on the Neurology Web site at www.neurology.org) because of pain and proximal limb weakness.

Enthalpy-entropy compensation combined with cohesive free-energy densities for tuning the melting temperatures of cyanobiphenyl derivatives.

This work illustrates how minor structural perturbations produced by methylation of 4'-(dodecyloxy)-4-cyanobiphenyl leads to enthalpy-entropy compensation for their melting processes, a trend which can be analyzed within the frame of a simple intermolecular cohesive model. The transformation of the melting thermodynamic parameters collected at variable temperatures into cohesive free-energy densities expressed at a common reference temperature results in a novel linear correlation, from which melting temperatures can be simply predicted from molecular volumes.

Supramolecular organization and magnetic properties of mesogen-hybridized mixed-valent manganese single molecule magnets [Mn(III)8Mn(IV)4O12(L(x,y,z-CB))16(H2O)4].

Single molecule magnets (SMM) may be considered for the construction of future integrated nanodevices, provided however that some degree of ordering is imparted to these molecules (surfaces nanostructuration). Combining such nanoobjects with liquid-crystalline orderings to control their assembly and to potentially address them individually therefore appears as one promising strategy. Four mesomorphic, mixed-valent [Mn(III)(8)Mn(IV)(4)O(12)(L(x,y,z-CB))(16)(H(2)O)(4)] SMM, differing in the number of liquid-crystalline promoters, (L(x,y,z-CB)), were synthesized, and their self-organizing and magnetic properties were investigated.

Epilepsy in Mowat-Wilson syndrome: delineation of the electroclinical phenotype.

Mowat-Wilson syndrome (MWS) is a genetic disease caused by heterozygous mutations or deletions of the ZEB2 gene and is characterized by distinctive facial features, epilepsy, moderate to severe intellectual disability, corpus callosum abnormalities and other congenital malformations. Epilepsy is considered a main manifestation of the syndrome, with a prevalence of about 70-75%. In order to delineate the electroclinical phenotype of epilepsy in MWS, we investigated epilepsy onset and evolution, including seizure types, EEG features, and response to anti-epileptic therapies in 22 patients with genetically confirmed MWS.

Magnetism in gold nanoparticles.

Gold nanoparticles currently elicit an intense and very broad research activity because of their peculiar properties. Be it in catalysis, optics, electronics, sensing or theranostics, new applications are found daily for these materials. Approximately a decade ago a report was published with magnetometry data showing that gold nanoparticles, most surprisingly, could also be magnetic, with features that the usual rules of magnetism were unable to explain.

Protonation and complexation properties of polyaromatic terdentate six-membered chelate ligands.

The successive protonation steps occurring in 2,2′;6′,2″-terpyridine (L1) are characterized by a strong affinity for the first entering proton (ΔG(connect)(H,L1) = −17 kJ/mol) followed by allosteric anticooperativity (ΔE(interaction)(H,H,L1) = 6 kJ/mol), a behavior mirrored by 2,6-bis(azaindolyl)pyridine (L2) despite the extension of the chelate ring size from five members (L1) to six members (L2; ΔG(connect)(H,L2) = −28 kJ/mol and ΔE(interaction)(H,H,L2) = 7 kJ/mol). On the contrary, 2,6-bis(8-quinolinyl)pyridine (L3) is less eager for the initial protonation (ΔG(connect)(H,L3) = −10 kJ/mol), but the fixation of a second proton in [H2L3]2+ is driven to completion by positive cooperativity (ΔE(interaction)(H,H,L3) = −5 kJ/mol). Because of its unusual ability to adopt a cis–cis conformation with a large affinity for the entering protons, L2 has been selected for exploring the reactivity of a terdentate fused six-membered chelate with labile metallic cations possessing increasing electrostatic factors along the series Mz+ = Li+ < Mg2+ ≈ Zn2+ < Y3+.

A case of cranial multinevritis: from the onset to the diagnosis of primary neurolymphomatosis.

Neurolymphomatosis (NL) is a rare peripheral or cranial neuropathy caused by non-Hodgkin's lymphoma (NHL). Diagnosis is often delayed and prognosis is poor. The authors described a woman in her 70s with a facial left peripheral palsy, complete right abducent palsy, left hypoacusia and balance deficit.

Control of the transition temperatures of metallomesogens by specific interface design: application to Mn12 single molecule magnets.

Reaction of the Single Molecule Magnet [Mn(12)O(12)(CH(3)CO(2))(16)(H(2)O)(4)] (Mn(12)) with mesogenic dendritic ligands Li (i = 4, 5) quantitatively yields functional clusters [Mn(12)O(12)(Li-H)(16)(H(2)O)(4)] (i = 4, 5) that self-organize into a thermotropic SmA-type liquid crystalline phase. The perturbation of the molecular interface by methylation of the terminal mesogenic cyanobiphenyl groups induces a significant decrease of the clearing temperature without affecting the magnetic properties and the supramolecular organization of the Mn(12)-based clusters.

Silver baits for the "miraculous draught" of amphiphilic lanthanide helicates.

The axial connection of flexible thioalkyls chains of variable length (n=1-12) within the segmental bis-tridentate 2-benzimidazole-8-hydroxyquinoline ligands [L12(Cn) -2 H](2-) provides amphiphilic receptors designed for the synthesis of neutral dinuclear lanthanides helicates. However, the stoichiometric mixing of metals and ligands in basic media only yields intricate mixtures of poorly soluble aggregates. The addition of Ag(I) in solution restores classical helicate architectures for n=3, with the quantitative formation of the discrete D(3) -symmetrical [Ln(2) Ag2(L12(C3) -2 H)(3) ](2+) complexes at millimolar concentration (Ln=La, Eu, Lu).

Dimerization of dendrimeric lanthanide complexes: thermodynamic, thermal, and liquid-crystalline properties.

A series of 10 different mesomorphic semidendrimeric tridentate ligands L5-L14 grafted with terminal cyanobiphenyl groups have been synthesized. Upon reaction with Ln(NO(3))(3) (Ln = trivalent lanthanide), the central 2,6-bis(N-ethylbenzimidazol-2-yl)pyridine unit is meridionally tricoordinated to the metal to give rodlike monomeric [Ln(Lk)(NO(3))(3)] and H-shaped dimeric [Ln(2)(Lk)(2)(NO(3))(6)] complexes. For the small Lu(III) cation, the monomeric complexes are quantitatively formed in a noncoordinating CD(2)Cl(2) solution.

Moyamoya disease and arteriovenous fistula of the epiaortic vessels.

The association of Moyamoya disease (MD) with other cerebrovascular disease has been reported in literature but not the occurrence of MD and arteriovenous fistula of the epiaortic vessels. A 61-year old Italian woman was admitted to our Department because of the sudden onset of Broca's aphasia. At general examination she presented a murmur above the right clavicle.