Blood harmane (1-methyl-9H-pyrido[3,4-b]indole) concentration in essential tremor cases in Spain

BackgroundEnvironmental correlates for essential tremor (ET) are largely unexplored. The search for such environmental factors has involved the study of a number of neurotoxins. Harmane (1-methyl-9H-pyrido[3,4-b]indole) is a potent tremor-producing toxin. In two prior case–control studies in New York, we demonstrated that blood harmane concentration was elevated in ET patients vs. controls, and especially in familial ET cases. These findings, however, have been derived from a study of cases ascertained through a single tertiary referral center in New York.ObjectiveOur objective was to determine whether blood harmane concentrations are elevated in familial and sporadic ET cases, ascertained from central Spain, compared to controls without ET.MethodsBlood harmane concentrations were quantified by a well-established high performance liquid chromatography method.ResultsThe median harmane concentrations were: 2.09 g⁻¹⁰/ml (138 controls), 2.41 g⁻¹⁰/ml (68 sporadic ET), and 2.90 g⁻¹⁰/ml (62 familial ET). In an unadjusted logistic regression analysis, log blood harmane concentration was not significantly associated with diagnosis (familial ET vs. control): odds ratio = 1.56, p = 0.26. In a logistic regression analysis that adjusted for evaluation start time, which was an important confounding variable, the odds ratio increased to 2.35, p = 0.049.ConclusionsBlood harmane levels were slightly elevated in a group of familial ET cases compared to a group of controls in Spain. These data seem to further extend our observations from New York to a second cohort of ET cases in Spain. This neurotoxin continues to be a source of interest for future confirmatory research.     

Association of the Val66Met polymorphism of the BDNF gene with primary cranial–cervical dystonia patients from South-west China

BackgroundThe etiology of primary dystonia remains unclear. Recent genetic studies suggest that the Val66Met polymorphism of the BDNF gene is a genetic modifier in cranial–cervical dystonia in Caucasians. However, the finding is not consistent.Patients and MethodsA total of 193 patients with primary cranial–cervical dystonia from the Department of Neurology, West China Hospital of Sichuan University was included. From the same region, 216 healthy individuals were recruited as a control group. The Val66Met SNP was identified by polymerase chain reaction-restriction fragment length polymorphism.ResultsIn the present study, cervical dystonia (59.59%) was the most common type of primary cranial–cervical dystonia. No significant difference was found in the genotype and minor allele frequencies between all patients and controls, between cervical dystonia patients and controls, and between craniocervical dystonia patients and controls. However, significant differences were found in the genotype and minor allele frequencies of Val66Met SNP between blepharospasm (BSP) patients and controls (P = 0.0080 and P = 0.0042, respectively), and between BSP patients and patients with craniocervical derived from BSP (P = 0.0010 and P = 0.0002, respectively).ConclusionMinor allele “A” of BDNF Val66Met SNP may increase the risk for developing BSP and may be a protective factor for preventing BSP progressing to craniocervical dystonia. More association studies involving a larger number of participants are needed to confirm the present findings.     

Gaze patterns during scene processing in typical adults and adults with autism spectrum disorders

BackgroundLittle is known about how adults with autism spectrum disorder (ASD) process dynamic social scenes.MethodWe studied gaze behavior in 16 adults with ASD without intellectual impairment and 16 sex- and age-matched controls during passive scene processing.ResultsAdding more characters to a scene resulted in a drop in time spent looking at faces, and an increase in time spent looking at bodies (static trials) or off-person (dynamic trials) [Scene Type × AOI × Mode: F(2, 60) = 3.54, p = .04, η²_p = .11]. Unlike controls, adults with ASD showed only a small drop in the number of fixations made [Mode × Group: F(1, 30) = 11.30, p = .002, η²_p = .27] and no increase in the duration of face fixations [Mode × AOI × Group: F(2, 60) = 3.50, p = .04, η²_p = .11] when dynamic cues were added. Thus, particularly during dynamic trials, adults with ASD spent less time looking at faces and slightly more time looking off-person than did controls [Mode × AOI × Group: F(2, 60) = 3.10 p = .05, η²_p = .09]. Exhibiting more autistic traits and being less empathic were both associated with spending less time fixating on faces [.34 < |r| < .55, p < .05].ConclusionsThese results suggest that adults with ASD may be less sensitive to, or have more difficulty processing, dynamic cues—particularly those conveyed in faces. The findings demonstrate the importance of using dynamic displays in studies involving this clinical population.     

Can we predict intraoperative blood loss in meningioma patients? Application of dynamic susceptibility contrast-enhanced magnetic resonance imaging

PurposeTo evaluate the potential of quantitative dynamic susceptibility contrast (DSC) perfusion MR imaging parameters as imaging biomarkers for predicting intraoperative blood loss in meningioma.MethodsFifty-one non-embolized meningioma patients who had undergone preoperative DSC perfusion MR imaging were retrospectively included. The corrected relative cerebral blood volume (rCBV) and leakage coefficient (K₂) of the entire enhanced tumor were obtained using leakage correction. Tumor volume, location, grade, and other clinical variables, were also analyzed. To investigate the vascularity and vascular permeability of meningiomas, and their correlation with predicting estimated blood loss (EBL) using preoperative DSC perfusion MR imaging, the authors proposed an index reflecting the inherent tendency of meningiomas to bleed after controlling volume (i.e., EBL/cm³). Simple regression was performed to identify predictors of EBL/cm³; subsequently, the relevant variables included in the stepwise multiple linear regression.ResultsOn univariate analysis, EBL/cm³ was correlated with rCBV (r = 0.677; P < 0.001), K₂ (r = 0.294; P = 0.036), and tumor volume (r = –0.312, P = 0.026). EBL/cm³ was not correlated with age (P = 0.873), sex (P = 0.404), tumor location (P = 0.327), or histological grade (P = 0.230). On multiple linear regression, rCBV (β = 0.663 [0.463–0.864], B = 1.293 [0.903–1.684; P < 0.001) and K₂ (β = 0.260 [0.060–0.460], B = 2.277 [0.523–4.031], P = 0.012), were the only independent predictors of EBL/cm³.ConclusionThe rCBV and K₂ derived from DSC perfusion MR imaging in meningiomas may serve as feasible tools for clinicians to predict intraoperative blood loss and facilitate surgical planning.     

Effects of glutathione S-transferase M1 and T1 deletions on Parkinson's disease risk among a North African population

PurposeIn this study, the effects of glutathione S-transferase polymorphisms Mu1 (GSTM1) and glutathione S-transferase polymorphisms Theta1 (GSTT1) on Parkinson's disease (PD) risk factor were evaluated in a Tunisian population.MethodsThese polymorphisms were analyzed in 229 healthy Tunisian subjects and 64 Tunisian patients with PD, using a polymerase chain reaction (PCR). Statistical analysis was performed using SPSS 18.0. The relative associations between the GST genotypes and PD were assessed by calculating the odds ratios (ORs) and 95% confidence intervals (CIs).ResultsThe study results demonstrated that the individuals with GSTM1 [OR = 3.93, 95% CI: 1.98–7.92, P = 10^?6] and GSTT1 [OR = 5.45, 95% CI: 2.90–10.30, p = 10^?6] were statistically associated with the risk of PD. A significant association was also found between the individuals with both GSTM1/T1 null genotypes and PD risk [OR = 22.10, 95% CI: 6.99–73.75, P = 10^?6].ConclusionThese genotyping findings suggest that the absence of both GSTM1 and GSTT1 activity could be a contributory factor for the development of PD.     

Embarrassment in essential tremor: Prevalence,clinical correlates and therapeutic implications

BackgroundEmbarrassment is a commonly described feature of essential tremor (ET) but has not been the focus of clinical research.ObjectiveTo estimate the prevalence, identify susceptible patient groups, and quantify the therapeutic correlates of reported embarrassment.MethodsA total of 106 ET cases from a population-based sample and 349 ET cases from a clinical sample were asked, “Does your tremor often embarrass you?”ResultsIn the clinical sample, the prevalence of embarrassment was high (58.2%). Even in those ET cases with no head tremor and mild arm tremor, nearly one-half (29/61 [47.5%]) reported embarrassment. While the prevalence of embarrassment was lower in the population-based sample, it was not negligible (18.9%). Embarrassment was associated with younger age of onset (p = 0.003) and women were nearly twice as likely as men to report embarrassment (OR = 1.85, p = 0.01). Independent of tremor severity, embarrassment nearly doubled the odds of using tremor medication (OR = 1.86, p = 0.01).ConclusionsEmbarrassment may be a source of disability in ET. Even among clinic patients with mild tremor, nearly one-half reported embarrassment. We identified a number of patient characteristics linked to embarrassment. Embarrassment alone (i.e., independent of tremor severity) was responsible for a doubling of tremor medication usage. The majority of clinical trials do not assess the therapeutic effects of medication on embarrassment. These trials may benefit from scaled assessments of level of embarrassment.     

Increased left striatal dopamine transmission in unaffected siblings of schizophrenia patients in response to acute metabolic stress

A genetic alteration in sensitivity to stress, mediated by mesolimbic hyperdopaminergia, is thought to play a role in the onset, exacerbation and relapse of schizophrenia. Dopamine sensitivity to stress was tested in individuals at higher than average genetic risk for schizophrenia (siblings of patients). Using a PET paradigm of [¹¹C]raclopride in a bolus plus constant infusion tracer injection, the central DA response to acute metabolic stress (bolus of 2-Deoxy-d-Glucose, 40 mg/kg) in unaffected siblings of patients with schizophrenia (n = 8) and healthy controls (n = 10) was measured by BP_ND of [¹¹C]raclopride before and after the 2DG challenge. After metabolic stress, controls but not siblings displayed a significant decrease in BP_ND of [¹¹C]raclopride in the striatum; no such differences were apparent in the ventral striatum. Siblings but not controls displayed significant asymmetry (L > R) in the stress-induced DA release, especially in ventral striatum, which correlated strongly with psychometric measures of psychosis liability. The results suggest that asymmetry in the mesolimbic DA response to stress is associated with genetic risk for schizophrenia, possibly reflecting the functional consequences of structural disconnectivity underlying psychotic symptoms.     

Cobalamin as a regulator of serum and cerebrospinal fluid levels of normal prions

We have previously demonstrated that the concentration of normal prion proteins (PrP^C) is increased in the serum and cerebrospinal fluid (CSF) of rats deficient in vitamin B₁₂ (cobalamin, Cbl). In this study, we investigated whether similar increases also occur in the serum and CSF of patients deficient in Cbl (Cbl-D), and whether the increase in serum levels can be corrected by Cbl therapy. The study involved two sample populations. The first consisted of 45 patients (13 patients with pernicious anemia [PA], 19 with other forms of anemia, and 13 healthy controls); and the second, 68 patients (five with subacute combined degeneration [SCD], 18 with amyotrophic lateral sclerosis, 22 with multiple sclerosis [MS], and 23 neurological controls). Serum PrP^C levels were measured using an enzyme-linked-immunosorbent-assay before as well as after Cbl therapy. The mean serum PrP^C levels in patients with PA were significantly higher than those of the controls (p = 0.0017) but normalized after Cbl therapy; there was no significant change in the patients with other forms of anemia. Mean CSF PrP^C levels in the patients with SCD were significantly higher than in the neurological controls (p < 0.03). The serum and CSF PrP^C levels of patients with PA and those with SCD were correlated significantly with serum (p = 0.004) and CSF (p = 0.0018) Cbl levels. In patients with MS, CSF PrP^C concentrations were significantly lower than those of the controls regardless of their CSF Cbl levels. We found a correlation between Cbl and PrP^C levels in the serum and CSF of Cbl-D patients, which suggests that Cbl may regulate the PrP^C levels in the serum and CSF in humans.     

Sleep bruxism,snoring, and headaches in adolescents: short-term effects of a mandibular advancement appliance

ObjectivesSleep bruxism (SB) frequently is associated with other sleep disorders and pain concerns. Our study assesses the efficacy of a mandibular advancement appliance (MAA) for SB management in adolescents reporting snoring and headache (HA).MethodsSixteen adolescents (mean age, 14.9 ± 0.5) reporting SB, HA (>1 d/wk), or snoring underwent four ambulatory polysomnographies for baseline (BSL) and while wearing MAA during sleep. MAA was worn in three positions (free splints [FS], neutral position [NP], and advanced to 50% of maximum protrusion [A50]) for 1 week each in random order (FS–NP–A50 or NP–A50–FS; titration order, NP–A50). Reports of HA were assessed with pain questionnaires.ResultsOverall, sleep variables did not differ across the four nights. SB index decreased up to 60% with MAA in A50 (P = .004; analysis of variance). Snoring was measured as the percentage of sleep time spent snoring. The subgroup of snorers (n = 8) showed significant improvement with MAA (−93%; P = .002). Initial HA intensity was reported at 42.7 ± 5/100 mm, showing a decreasing trend with MAA (−21% to −51%; P = .07).ConclusionShort-term use of an MAA appears to reduce SB, snoring, and reports of HA. However, interactions between SB, breathing during sleep, and HA as well as the long-term effectiveness and safety of MAA in adolescents need further investigation.     

Dynamic cerebral autoregulation is compromised in ischaemic stroke of undetermined aetiology only in the non-affected hemisphere

Background and purposeTo assess dynamic cerebral autoregulation (CA) in patients with acute ischaemic stroke of undetermined aetiology, within 72 h of stroke onset.Materials and methodsIn 6 patients with ischaemic stroke of undetermined aetiology (aged 66 ± 9 years, National Institutes of Health Stroke Scale [NIHSS] score on admission: 4.0, range: 4–11), selected based on screening of 118 consecutive ischaemic stroke patients and in 14 volunteers (aged 62 ± 10 years), we continuously monitored RR intervals (RRI), mean arterial pressure (MAP) by means of photoplethysmography, mean cerebral blood flow velocity (CBFV) using transcranial Doppler ultrasonography, end-tidal CO₂ (ETCO₂) and respiration during 2-min deep breathing paced at 6 min⁻¹ (0.1 Hz). To assess CA, we evaluated the impact of breathing-induced MAP oscillations on fluctuations of CBFV in the hemispheres with stroke, the non-involved hemispheres and randomly selected hemispheres of controls by applying cross-spectral analysis and calculating coherence, transfer function gain (CBFV–MAP gain) and phase shift angle between the two oscillating signals.ResultsPhase shift angle between MAP and CBFV oscillations showed values >0 and was significantly reduced in the hemispheres without stroke as compared to controls (0.39 ± 0.95 vs. −1.59 ± 0.33 rad, p = 0.015), whereas in the hemispheres with stroke, phase shift angle did not differ significantly from that observed in the control hemispheres. Clinical status of stroke patients significantly improved at discharge from the hospital (NIHSS: 2.0, range: 1–8, p = 0.028).ConclusionsDuring the first days of ischaemic stroke of undetermined aetiology, dynamic cerebral autoregulation is compromised in the non-affected hemisphere, but not in the hemisphere with ischaemic lesion.     

Prenatal exposure to the organophosphate pesticide chlorpyrifos and childhood tremor

BackgroundThe organophosphate insecticide chlorpyrifos (CPF), widely used for agricultural purposes, has been linked to neurodevelopmental deficits. Possible motor effects at low to moderate levels of exposure have not been evaluated.MethodsPrenatal exposure to CPF was measured in umbilical cord blood in a sample of 263 inner-city minority children, who were followed prospectively. At approximately 11 years of age (mean age 10.9 ± 0.85 years, range = 9.0–13.9), during a neuropsychological assessment, children were asked to draw Archimedes spirals. These were rated by a senior neurologist specializing in movement disorders who was blind to CPF exposure level.ResultsCompared to all other children, those with prenatal CPF exposure in the upper quartile range (n = 43) were more likely to exhibit mild or mild to moderate tremor (≥1) in either arm (p = 0.03), both arms (p = 0.02), the dominant arm (p = 0.01), and the non-dominant arm (p = 0.055). Logistic regression analyses showed significant CPF effects on tremor in both arms, either arm, the dominant arm (p-values <0.05), and the non-dominant arm (p = 0.06), after adjustment for sex, age at testing, ethnicity, and medication.ConclusionPrenatal CPF exposure is associated with tremor in middle childhood, which may be a sign of the insecticide's effects on nervous system function.     

Syndrome de Susac : étude de cinq cas

IntroductionSusac syndrome is a rare microangiopathy, responsible for small cerebral, retinal and cochlear infarcts. The classic clinical triad includes multiple neurologic signs (from headaches to coma), retinal branch occlusions and sensorineural hearing loss.MethodsWe report a series of five patients with Susac syndrome followed in our department from 1997 to 2007.ResultsThere were four women and one man (mean age at onset: 35.2 years). Clinical symptoms at onset were neurological (n = 1), ophthalmological (n = 1), auditory (n = 1) and clinical triad (n = 2). Neurologic symptoms included encephalopathy (n = 2), headache (n = 5), transient ischemic attacks (n = 1). Brain MRI showed T2 lesions in the white and grey matter, corpus callosum and gadolinium-enhanced punctiform lesions. Cerebrospinal fluid contained an elevated protein level in three cases. Immunologic treatments (steroids [n = 4], cylophosphamid [n = 3], intravenous immunoglobulins [n = 5]) associated with aspirin and/or oral anticoagulants, despite early relapses (n = 2), led to dramatic clinical improvement (n = 5).ConclusionDue to its polymorphism the SS is difficult to diagnose when the clinical triad is lacking. In the absence of clinical trial and consensus treatment is empiric and based on supposed pathogenesis.     

Sensibilidad y dolor en distonías focales de la mano

IntroductionA growing body of evidence highlights the importance of understanding both the sensory and the motor pathophysiology of focal dystonia in order to improve its treatment. This study aims to evaluate somatosensory afferences in patients with focal or segmental dystonia affecting the upper limbs, to analyse whether the dominant limb is more frequently affected, to analyse pain tolerance, and to examine the potential association with pain perception in patients with hand dystonia.MethodsWe recruited 24 participants: 12 patients with focal hand dystonia and 12 individuals without dystonia. All participants were evaluated with a digital algometer (Somedic SenseLab AB®, Farsta, Sweden), a Semmes-Weinstein monofilament test, and the visual analogue scale for pain.ResultsAccording to our data, patients showed greater impairment in surface sensitivity than controls, both in the dominant and the non-dominant hands, as well as greater presence of pain (P > .001). Furthermore, the dystonia group showed a negative correlation between perceived pain and pressure pain tolerance threshold (rho = ?0.83; P < .001).ConclusionsPatients with focal hand dystonia presented alterations in sensitivity and more severe perceived pain than individuals without dystonia. Future studies with larger samples should aim to analyse the clinical implications and everyday impact of both objective and subjective pain.     

The effect of cigarette smoking on vitamin D level and depression in male patients with acute ischemic stroke

ObjectiveThe association between low vitamin D levels and depression has been well documented in nonstroke subjects. Accumulating evidence shows that low vitamin D levels may be also associated with depression post stroke. Cigarette smoking was associated with lower vitamin D levels. The purposes of this study were to compare vitamin D levels in smokers to nonsmokers and examine the association between vitamin D levels and depression symptoms in patients with acute ischemic stroke.Materials and methodsSerum levels of 25-hydroxyvitamin D [25(OH)D] were measured in 194 males within 24 h after admission: 116 smokers and 78 nonsmokers. Depression symptoms were assessed with the 17-item Hamilton Depression Scale (HAMD-17). Patients with the HAMD-17 score >7 were identified to have depression symptoms.ResultsThe chi-square test showed that the frequency of depression in the smoker group was 23.3% (27/116), which was significantly higher than that in the nonsmoker group (11.5% = 9/78), with an odds ratios (OR) of 2.33 (95% CI: 1.03–5.27; χ² = 4.25, df = 1, p = 0.039, φ = 0.15). Vitamin D levels were significantly lower in smokers than in nonsmokers (52.4 ± 20.8 vs 61.7 ± 19.2; F = 9.88, p = 0.002), with an effect size of 0.05 (η_p²). Patients with depression symptoms showed lower vitamin D levels than those with no depression symptoms (49.2 ± 19.6 vs 57.7 ± 20.6; F = 5.03, p = 0.03), with an effect size of 0.03 (η_p²).ConclusionHigher rates of depression in smokers with acute ischemic stroke may be associated with lower vitamin D levels induced by smoking.     

Abnormal glucose tolerance,white blood cell count,and telomere length in newly diagnosed,antidepressant-naïve patients with depression

Chronic mood disorders have been associated with a shortened telomere, a marker of increased mortality rate and aging, and impaired cellular immunity. However, treatment may confound these relationships. We examined the relationship of glucose tolerance, white blood cell count and telomere length to depression in newly diagnosed, antidepressant-naïve patients. Subjects with major depression (n = 15), and matched healthy control subjects (n = 70) underwent a two-hour oral glucose tolerance test and evaluation of blood cell count and telomere content. The depression group had significantly higher two-hour glucose concentrations and a lower lymphocyte count than control subjects (respective means [SD] for two-hour glucose were 125.0 mg/dL [67.9] vs 84.6 [25.6] (p < .001); for lymphocyte count 2.1 × 10⁹/L [0.6] vs 2.5 × 10⁹/L [0.7] p = .028). Telomere content was significantly shortened in the depression group (87.9 [7.6]) compared to control subjects (101.0 [14.3]; p < 0.01). Abnormal glucose tolerance, lymphopenia and a shortened telomere are present early in the course of depression independently of the confounding effect of antidepressant treatment, supporting the concept of major depression as an accelerated aging disease.     

Olfactory dysfunction in pure autonomic failure: Implications for the pathogenesis of Lewy body diseases

BackgroundPure autonomic failure (PAF) and Parkinson disease (PD) both are Lewy body diseases, and both entail substantia nigra dopaminergic, locus ceruleus noradrenergic, and cardiac sympathetic denervation. Multiple system atrophy (MSA) is a non-Lewy body disease in which alpha-synuclein accumulates in glial cells, with central catecholamine deficiency but preserved cardiac sympathetic innervation in most patients. PD is associated with more severe and consistent olfactory dysfunction than in MSA; whether PAF entails olfactory dysfunction has been unknown. In this study we assessed olfactory function in PAF in comparison with the two other synucleinopathies and whether olfactory dysfunction correlates with neuroimaging evidence of cardiac noradrenergic or nigrostriatal dopaminergic denervation.MethodThe University of Pennsylvania Smell Identification Test (UPSIT) was administered to 8 patients with PAF, 23 with PD, and 20 with MSA. 6-[¹⁸F]Fluorodopamine positron emission tomographic (PET) scanning was used to indicate cardiac noradrenergic innervation and the putamen:occipital cortex (PUT:OCC) and substantia nigra (SN):OCC ratios of 6-[¹⁸F]fluorodopa-derived radioactivity to indicate nigrostriatal dopaminergic innervation.ResultsThe PAF group had a low mean UPSIT score (22 ± 3), similar to that in PD (20 ± 2) and lower than in MSA (31 ± 2, p = 0.004). Individual UPSIT scores correlated positively with cardiac 6-[¹⁸F]fluorodopamine-derived radioactivity (r = 0.63 in the septum, p < 0.0001; r = 0.64 in the free wall, p < 0.0001) but not with PUT:OCC or SN:OCC ratios of 6-[¹⁸F]fluorodopa-derived radioactivity.DiscussionIn synucleinopathies, olfactory dysfunction is related to Lewy body pathology and cardiac sympathetic denervation, independently of parkinsonism or striatal dopamine deficiency.     

Performance on an Alzheimer-selective odor identification test in patients with Parkinson's disease and its relationship with cerebral dopamine transporter activity

BackgroundPrevious studies have shown selective deficits of odor identification in both Parkinson's disease (PD) and Alzheimer's disease (AD). Brief, selective AD smell screening tests have been developed to identify subjects at risk of AD. The disease specificity of such screening tests has not been formally evaluated.ObjectiveTo evaluate the performance of an Alzheimer-selective odor identification test in patients with PD and its relationship with cerebral dopamine transporter (DAT) activity.MethodsPD patients (n = 44; Hoehn and Yahr stages I–III; 13f/31 m; mean age 59.3 ± 10.1) and 44 controls matched for gender and age completed the University of Pennsylvania Smell Identification Test (UPSIT). All patients had PD duration > 1 year and none had evidence of dementia. Using the UPSIT, we calculated performance on the 10 odors previously reported to be selective for AD risk (UPSIT-AD10). A subset of 29 PD patients also underwent brain DAT [¹¹C]β-CFT (2-β-carbomethoxy-3β-(4-fluorophenyl) tropane) PET imaging. DAT binding was assessed in the hippocampus, amygdala, ventral and dorsal striatum.ResultsUPSIT-AD10 scores were significantly lower in the patient (5.8 ± 2.1) compared to the control group (8.6 ± 2.4) (t = 5.8, P < 0.0001). However, UPSIT-AD10 performance in the PD patients did not correlate with striatal or mesolimbic DAT activity.ConclusionsHyposmia in PD and AD overlap and supposed Alzheimer-selective smell screening tests may not be specific for AD. However, the supposed AD-selective hyposmia scores in PD did not correlate with cerebral DAT binding and may reflect a non-dopaminergic olfactory mechanism.     

CSF neurofilament light chain is elevated in OMS (decreasing with immunotherapy) and other pediatric neuroinflammatory disorders

Using a panel of seven brain cell-specific biomarkers in cerebrospinal fluid (CSF), pediatric opsoclonus–myoclonus syndrome (OMS) (n = 234) was compared to pediatric non-inflammatory neurological controls (n = 84) and other inflammatory neurological disorders (OIND) (n = 44). Only CSF NFL was elevated in untreated OMS versus controls (+ 83%). It was 87% higher in OIND than in OMS. On combination treatment with front-loaded ACTH, IVIg, rituximab, median CSF NFL decreased by 60% to control levels. These biochemical data suggest neuronal/axonal injury in some children with OMS without indicators of astrogliosis, and reduction on sufficient immunotherapy.     

The impact of patient suicide and sudden death on health care professionals

ObjectiveTo compare the professional and personal impact of patient suicide and sudden death on health care professionals (HCPs) and determine factors associated with these impacts.MethodThe sample was derived from a sudden death-controlled psychological autopsy study of suicide. HCPs were identified by deceased's next of kin, by other HCPs, from coroners' files and from medical records. The HCPs were interviewed about their last contact with the deceased and the impact of the death on their lives.ResultsTwo hundred eleven HCPs were interviewed following suicide; 92 after sudden death. Suicide deaths were significantly more likely to impact upon the HCP's professional practice [suicide n= 79 (37.4%); sudden death n= 9 (9.9%); χ²= 22.06, P< .001] and personal life [suicide deaths n= 55 (26.1%); sudden death n= 12 (13.0%); χ²= 5.58, P= .018] than sudden deaths. Using multinomial logistic regression, being female and suicide within a week of the consultation predicted professional and personal impacts; having less than 5 years experience predicted professional impact and receipt of support/counseling predicted personal impact.ConclusionSuicide deaths have a greater impact than sudden deaths upon the life of HCPs. Clinical inexperience influences impacts on professional practice and availability of support impacts on personal life.     

Gustatory and olfactory sensitivity in patients with anorexia and bulimia in the course of treatment

BackgroundThe majority of studies on taste and smell in eating disorders have revealed several alterations of olfactory or gustatory functions. Aim of this prospective study was to employ detailed olfactory and gustatory testing in female subjects of three homogenous groups – anorexia nervosa, bulimia nervosa and healthy controls – and to look at the effects of treatment on these measures.MethodsSixteen hospitalized female patients with anorexia (restricting type, mean age [M] = 24.5 years), 24 female patients with bulimia (purging type, M = 24.3 years) as well as 23 healthy controls (M = 24.5 years) received olfactory (“Sniffin’ Sticks”) and gustatory testing (“Taste Strips”). Group differences in olfactory and gustatory sensitivity, body mass index (BMI), the Beck depression inventory, the eating attitudes test (EAT), and the influence of therapy on gustatory and olfactory function were investigated.Results(1) Group differences were present for odor discrimination and overall olfactory function with anorexic patients having the lowest scores. (2) Regarding taste function, controls scored higher than patients with anorexia. (3) At admission small but significant correlations were found between overall olfactory function and body weight (r₆₃ = 0.35), BMI (r₆₃ = 0.37), and EAT score (r₆₃ = −0.27). Similarly, (4) the taste test score correlated significantly with body weight (r₆₃ = 0.48), and BMI (r₆₃ = 0.45). Finally, (5) at discharge overall olfactory and gustatory function were significantly higher compared to admission in anorexic patients.ConclusionsAs compared to healthy controls and bulimic patients our results show lowered olfactory and gustatory sensitivities in anorexic patients that improved with increasing BMI and decreasing eating pathology in the course of treatment.