Association of amyloid angiopathy with microbleeds in logopenic progressive aphasia: an imaging-pathology study.

BACKGROUND AND PURPOSE: Cerebral microbleeds (MB) and superficial siderosis (SS) are frequent neuroimaging findings in patients with logopenic progressive aphasia (LPA), often with frontal lobe predilection. Cerebral amyloid angiopathy (CAA) is hypothesized to be the major pathologic determinant of MB/SS in these patients; however, neuroimaging-pathologic data are limited. METHODS: All patients who had been prospectively recruited by the Neurodegenerative Research Group at the Mayo Clinic (Rochester, MN) between 2010 and 2015 and met the following inclusion criteria were included: (i) received an antemortem LPA diagnosis, (ii) had a gradient-recalled echo T2*-weighted magnetic resonance imaging (MRI) performed, (iii) died and completed a brain autopsy. Demographic, genetic, neuroimaging, and clinical and pathologic characteristics were compared between patients with/without MB/SS. Two-tailed Fisher exact and Wilcoxon rank sum tests were used for comparison of categorical and continuous variables, respectively. RESULTS: Thirteen patients met inclusion criteria, six (46%) had MB/SS on MRI. Moderate/severe CAA was associated with the presence of MB/SS (p = 0.029). As expected, MB/SS most frequently involved the frontal lobes, followed by the parietal lobes. No clear associations were found between regional MB/SS distribution and regional distribution of CAA or hypometabolism on [18 F]-fluorodeoxyglucose-positron emission tomography. There was some evidence for a regional association between MB/SS and uptake on Pittsburgh compound B, although not in all patients. No formal statistical analyses to assess topographic relationships were performed due to the small sample size. CONCLUSIONS: The presence of MB/SS is a strong indicator of underlying moderate/severe CAA in LPA, although the biological mechanisms underlying the topographic distribution of MB/SS remain unclear.

Reproduction of Meloidogyne enterolobii on selected root-knot nematode resistant sweetpotato (Ipomoea batatas) cultivars.

The ability of Meloidogyne enterolobii to reproduce on selected sweetpotato (Ipomoea batatas) cultivars (Beauregard, Covington, Evangeline, Hernandez, and Orleans (LA 05-111)) was evaluated in two greenhouse experiments, each with 10 replicates. All cultivars, except Beauregard (control) and Orleans, were reported previously as moderately resistant or resistant to M. incognita, Fusarium oxysporum f. sp. batatas, and Streptomyces ipomoeae. Plants were inoculated with M. enterolobii (5,000 eggs/plant) and arranged in a completely randomized design in a greenhouse with an average daily temperature of 24.8°C. Galls and egg masses per root system (0-5 scale), eggs per egg mass, eggs per gram of fresh root (gfr), and reproduction factor (RF) were determined. Meloidogyne enterolobii infected and reproduced on all the sweetpotato cultivars. The nematode induced galls on both fibrous and storage roots, regardless of the cultivar, as well as induced necrosis and cracks on storage roots. The lesions and cracks on the storage roots were more visually pronounced on Hernandez than those on other cultivars. Cultivar Orleans sustained less root galling and egg masses than other cultivars (p ≤ 0.01), and both Orleans and Beauregard cultivars had less eggs per gfr and a lower RF than Covington (5,683 eggs/gfr; RF = 16.92), Evangeline (7,161 eggs/gfr; RF = 30.01), and Hernandez (6,979 eggs/gfr; RF = 22.6). The latter two cultivars sustained the largest amount of reproduction of M. enterolobii. The number of eggs per egg mass ranged from 462 to 503 and was similar among all cultivars. In summary, M. enterolobii reproduced well on all sweetpotato cultivars; however, differences were observed among cultivars (p ≤ 0.001). The host status as previously reported for other root-knot nematode species was not a good predictor of host status to M. enterolobii. Some sweetpotato cultivars that were reported as resistant or moderately resistant to M. incognita race 3, such as Evangeline and Hernandez, were among the best hosts to M. enterolobii. Root growth of Evangeline and Orleans, but not of the other cultivars, was negatively correlated with nematode eggs per gfr.The ability of Meloidogyne enterolobii to reproduce on selected sweetpotato (Ipomoea batatas) cultivars (Beauregard, Covington, Evangeline, Hernandez, and Orleans (LA 05-111)) was evaluated in two greenhouse experiments, each with 10 replicates. All cultivars, except Beauregard (control) and Orleans, were reported previously as moderately resistant or resistant to M. incognita, Fusarium oxysporum f. sp. batatas, and Streptomyces ipomoeae. Plants were inoculated with M. enterolobii (5,000 eggs/plant) and arranged in a completely randomized design in a greenhouse with an average daily temperature of 24.8°C. Galls and egg masses per root system (0-5 scale), eggs per egg mass, eggs per gram of fresh root (gfr), and reproduction factor (RF) were determined. Meloidogyne enterolobii infected and reproduced on all the sweetpotato cultivars. The nematode induced galls on both fibrous and storage roots, regardless of the cultivar, as well as induced necrosis and cracks on storage roots. The lesions and cracks on the storage roots were more visually pronounced on Hernandez than those on other cultivars. Cultivar Orleans sustained less root galling and egg masses than other cultivars (p ≤ 0.01), and both Orleans and Beauregard cultivars had less eggs per gfr and a lower RF than Covington (5,683 eggs/gfr; RF = 16.92), Evangeline (7,161 eggs/gfr; RF = 30.01), and Hernandez (6,979 eggs/gfr; RF = 22.6). The latter two cultivars sustained the largest amount of reproduction of M. enterolobii. The number of eggs per egg mass ranged from 462 to 503 and was similar among all cultivars. In summary, M. enterolobii reproduced well on all sweetpotato cultivars; however, differences were observed among cultivars (p ≤ 0.001). The host status as previously reported for other root-knot nematode species was not a good predictor of host status to M. enterolobii. Some sweetpotato cultivars that were reported as resistant or moderately resistant to M. incognita race 3, such as Evangeline and Hernandez, were among the best hosts to M. enterolobii. Root growth of Evangeline and Orleans, but not of the other cultivars, was negatively correlated with nematode eggs per gfr.

Influence of Temperature on Susceptibility of CVS. Tifguard and Georgia-06G Peanut to Meloidogyne arenaria.

Tifguard was released in 2008 as a peanut cultivar with a high level of resistance to Meloidogyne arenaria. Our objective was to determine the role of temperature on infection and development of M. arenaria in Tifguard compared to that in the nematode susceptible cultivar, Georgia-06G. Temperature affected the rate of nematode infection and development in both Tifguard and Georgia-06G (P ≤ 0.05). In Georgia-06G, egg-laying females were observed 25, 20 or 25 days after inoculation at 28°C, 31°C, and 34°C, respectively. There were greater numbers of nematodes entering roots and acceleration of development in response to 31°C compared with that at 28°C. There was, however, a decrease in the number of nematodes entering roots and their development was retarded at 34°C compared with that occurring at 31°C. Although second-stage juveniles penetrated Tifguard roots, they did not develop further at 28°C or 31°C; however, at 34°C both females, males, and a few egg-laying females of M. arenaria were observed. The optimum temperature for nematode infection and development was 31°C in Georgia-06G. In summary, it is unlikely that high soil temperatures would lessen the effectiveness of the nematode resistance gene in Tifguard.

TDP-43 pathology in multiple system atrophy: colocalization of TDP-43 and α-synuclein in glial cytoplasmic inclusions.

AIMS: This study aimed to assess clinicopathologic features of transactive response DNA-binding protein of 43 kDa (TDP-43) pathology and its risk factors in multiple system atrophy (MSA). METHODS: Paraffin-embedded sections of the amygdala and basal forebrain from 186 autopsy-confirmed MSA cases were screened with immunohistochemistry for phospho-TDP-43. In cases having TDP-43 pathology, additional brain regions were assessed. Immunohistochemical and immunofluorescence double-staining and immunogold electron microscopy (IEM) were performed to evaluate colocalization of TDP-43 and α-synuclein. Genetic risk factors for TDP-43 pathology were also analysed. RESULTS: Immunohistochemistry showed various morphologies of TDP-43 pathology in 13 cases (7%), such as subpial astrocytic inclusions, neuronal inclusions, dystrophic neurites, perivascular inclusions and glial cytoplasmic inclusions (GCIs). Multivariable logistic regression models revealed that only advanced age, but not concurrent Alzheimer's disease, argyrophilic grain disease or hippocampal sclerosis, was an independent risk factor for TDP-43 pathology in MSA (OR: 1.11, 95% CI: 1.04-1.19, P = 0.002). TDP-43 pathology was restricted to the amygdala in eight cases and extended to the hippocampus in two cases. The remaining three cases had widespread TDP-43 pathology. Immunohistochemical and immunofluorescence double-staining and IEM revealed colocalization of α-synuclein and TDP-43 in GCIs with granule-coated filaments. Pilot genetic studies failed to show associations between risk variants of TMEM106B or GRN and TDP-43 pathology. CONCLUSIONS: TDP-43 pathology is rare in MSA and occurs mainly in the medial temporal lobe. Advanced age is a risk factor for TDP-43 pathology in MSA. Colocalization of TDP-43 and α-synuclein in GCIs suggests possible direct interaction between the two molecules.

Aberrant accumulation of ErbB4 in progressive supranuclear palsy.

AIMS: The human epidermal growth factor receptor family consists of four members that belong to the ErbB lineage of proteins (ErbB1-4). Neuregulin-1 (NRG1)/ErbB signalling regulates brain development and function. Abnormalities in this signalling have been implicated in the aetiology or development of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease and amyotrophic lateral sclerosis. So, we aimed at investigating whether the expression of NRG1 or ErbB proteins are altered in progressive supranuclear palsy (PSP). METHODS: The brains of 10 PSP and six control patients were investigated by immunohistochemical analysis. RESULTS: Whereas C-terminal ErbB4 immunoreacitivity was partially but distinctly present in the cytoplasm and/or in the nucleus of neurons in control patients, it was rarely observed in the neuronal nuclei in PSP patients. In contrast, neurofibrillary tangles, coiled bodies and threads were robustly immunoreactive for C-terminal ErbB4 in PSP. Double immunofluorescence for C-terminal ErbB4 and phospho-tau revealed co-localization of these proteins within neuronal and glial inclusions. To the contrary, there was no difference in the subcellular localization of NRG1, ErbB1, ErbB2, and N-terminal ErbB4 between control and PSP patients. These proteins were localized in the cytoplasm of neurons. CONCLUSIONS: Our present results suggest that NRG1/ErbB4 signalling could be an important event in the pathogenesis of PSP.

Atypical parkinsonian syndromes: a general neurologist's perspective.

The differential diagnosis of atypical parkinsonian syndromes is challenging. These severe and often rapidly progressive neurodegenerative disorders are clinically heterogeneous and show significant phenotypic overlap. Here, clinical, imaging, neuropathological and genetic features of multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration and frontotemporal lobar degeneration (FTLD) are reviewed. The terms corticobasal degeneration and FTLD refer to pathologically confirmed cases of corticobasal syndrome and frontotemporal dementia (FTD). Frontotemporal lobar degeneration clinically presents as the behavioral variant FTD, semantic variant primary progressive aphasia (PPA), non-fluent agrammatic variant PPA, logopenic variant PPA and FTD associated with motor neuron disease. While progressive supranuclear palsy and corticobasal syndrome have been called Parkinson-plus syndromes in the past, they are now classified as FTD-related disorders, reflecting that they pathologically differ from α-synucleinopathies like multiple system atrophy and Parkinson disease. The contribution of genetic factors to atypical parkinsonian syndromes is increasingly recognized. Genes involved in the etiology of FTLD include MAPT, GRN and C9orf72. Novel neuroimaging techniques, including tau positron emission tomography imaging, are being investigated. Multimodal magnetic resonance imaging approaches and automated magnetic resonance imaging volume segmentation techniques are being evaluated for optimized differential diagnosis. Current treatment options are symptomatic, and disease modifying therapies are under active investigation.

Clinicopathologic heterogeneity in frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) due to microtubule-associated protein tau (MAPT) p.P301L mutation, including a patient with globular glial tauopathy.

AIM: The p.P301L mutation in microtubule-associated protein tau (MAPT) is a common cause of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). We compare clinicopathologic features of five unrelated and three related (brother, sister and cousin) patients with FTDP-17 due to p.P301L mutation. METHODS: Genealogical, clinical, neuropathologic and genetic data were reviewed from eight individuals. RESULTS: The series consisted of five men and three women with an average age of death of 58 years (52-65 years) and average disease duration of 9 years (3-14 years). The first symptoms were those of behavioural variant frontotemporal dementia in seven patients and semantic variant of primary progressive aphasia in one. Three patients were homozygous for the MAPT H1 haplotype; five had H1/H2 genotype. The apolipoprotein E genotype was ϵ3/ϵ3 in seven and ϵ3/ϵ4 in one. The average brain weight was 1015 g (876-1188 g). All had frontotemporal lobar or more diffuse cortical atrophy. Except for one patient, the hippocampus and parahippocampal gyrus had minimal atrophy, whereas there was atrophy of middle and inferior temporal gyri. Dentate fascia neuronal dispersion was identified in three patients, two of whom had epilepsy. In one patient there was extensive white matter tau involvement with Gallyas-positive globular glial inclusions typical of globular glial tauopathy (GGT). CONCLUSIONS: This clinicopathologic study shows inter- and intra-familial clinicopathologic heterogeneity of FTDP-17 due to MAPT p.P301L mutation, including GGT in one patient.

Molecular Characterization of Meloidogyne christiei Golden and Kaplan, 1986 (Nematoda, Meloidogynidae) Topotype Population Infecting Turkey Oak (Quercus laevies) in Florida.

Meloidogyne christiei isolated from turkey oak, Quercus laevies, from the type locality in Florida was characterized using isozyme profiles and ribosomal and mitochondrial gene sequences. The phenotype N1a detected from a single egg-laying female of M. christiei showed one very strong band of malate dehydrogenase (MDH) activity; however, no esterase (EST) activity was identified from macerate of one or even 20 females per well. Phylogenetic relationships within the genus Meloidogyne as inferred from Bayesian analysis of partial 18S ribosomal RNA (rRNA), D2-D3 of 28S rRNA, internal transcribed spacer (ITS) rRNA, and cytochrome oxidase subunit II (COII)-16S rRNA of mitochondrial DNA (mtDNA) gene fragments showed that M. christiei formed a separate lineage within the crown group of Meloidogyne and its relationships with any of three Meloidogyne clades were not resolved.

DNAJC13 p.Asn855Ser mutation screening in Parkinson's disease and pathologically confirmed Lewy body disease patients.

BACKGROUND: Recently, a novel mutation in exon 24 of DNAJC13 gene (p.Asn855Ser, rs387907571) has been reported to cause autosomal dominant Parkinson's disease (PD) in a multi-incident Mennonite family. METHODS: In the present study the mutation containing exon of the DNAJC13 gene has been sequenced in a Caucasian series consisting of 1938 patients with clinical PD and 838 with pathologically diagnosed Lewy body disease (LBD). RESULTS: Our sequence analysis did not identify any coding variants in exon 24 of DNAJC13. Two previously described variants in intron 23 (rs200204728 and rs2369796) were observed. CONCLUSION: Our results indicate that the region surrounding the DNAJC13 p.Asn855Ser substitution is highly conserved and mutations in this exon are not a common cause of PD or LBD among Caucasian populations.

Belonolaimus longicaudatus: An Emerging Pathogen of Peanut in Florida.

Sting nematode (Belonolaimus longicaudatus) is an economically important ectoparasitic nematode that is highly pathogenic on a wide range of agricultural crops in sandy soils of the southeastern United States. Although this species is commonly found in Florida in hardwood forests and as a soilborne pathogen on turfgrasses and numerous agronomic and horticultural crops, it has not been reported infecting peanut. In the summers of 2012 and 2013, sting nematode was found infecting three different peanut cultivars being grown on two separate peanut farms in Levy County, FL. The damage consisted of large irregular patches of stunted, chlorotic plants at both farms. The root systems were severely abbreviated and there were numerous punctate-like isolated lesions observed on pegs and pods of infected plants. Sting nematodes were extracted from soil collected around the roots of diseased peanut over the course of the peanut season at both farm sites. Peanut yield from one of these nematode-infested sites was 64% less than that observed in areas free from sting nematodes. The morphological characters of the nematode populations in these fields were congruous with those of the original and other published descriptions of B. longicaudatus. Moreover, the molecular analyses based on the sequences of D2/D3 expansion fragments of 28S rRNA and internal transcribed spacer (ITS) rRNA genes from the nematodes further collaborates the identification of the sting nematode isolates as B. longicaudatus. The sequences were deposited in GenBank (accession no. KF963097, KF963098 for ITS, and KF96399, KF963100 for D2-D3). The results of the phylogenetic analysis using the sequences of these isolates from peanut compared with those of other isolates from Florida suggests that the sting nematode from both peanut farms are genetically close to B. longicaudatus populations occurring in the state. Peanut plants inoculated with both nematode isolates showed punctate-like isolated lesions on pods and pegs, and an abbreviation of their root systems, whereas those symptoms were not observed on noninoculated peanut plants. To our knowledge, this is the first report of large-scale field damage caused by sting nematode infecting peanut grown under field conditions in Florida.

The Statistical Modeling of Aging and Risk of Transition Project: Data Collection and Harmonization Across 11 Longitudinal Cohort Studies of Aging, Cognition, and Dementia.

Longitudinal cognitive trajectories and other factors associated with mixed neuropathologies (such as Alzheimer's disease with co-occurring cerebrovascular disease) remain incompletely understood, despite being the rule and not the exception in older populations. The Statistical Modeling of Aging and Risk of Transition study (SMART) is a consortium of 11 different high-quality longitudinal studies of aging and cognition (N=11,541 participants) established for the purpose of characterizing risk and protective factors associated with subtypes of age-associated mixed neuropathologies (N=3,001 autopsies). While brain donation was not required for participation in all SMART cohorts, most achieved substantial autopsy rates (i.e., > 50%). Moreover, the studies comprising SMART have large numbers of participants who were followed from intact cognition and transitioned to cognitive impairment and dementia, as well as participants who remained cognitively intact until death. These data provide an exciting opportunity to apply sophisticated statistical methods, like Markov processes, that require large, well-characterized samples. Thus, SMART will serve as an important resource for the field of mixed dementia epidemiology and neuropathology.

Progressive dopaminergic alterations and mitochondrial abnormalities in LRRK2 G2019S knock-in mice.

Mutations in the LRRK2 gene represent the most common genetic cause of late onset Parkinson's disease. The physiological and pathological roles of LRRK2 are yet to be fully determined but evidence points towards LRRK2 mutations causing a gain in kinase function, impacting on neuronal maintenance, vesicular dynamics and neurotransmitter release. To explore the role of physiological levels of mutant LRRK2, we created knock-in (KI) mice harboring the most common LRRK2 mutation G2019S in their own genome. We have performed comprehensive dopaminergic, behavioral and neuropathological analyses in this model up to 24months of age. We find elevated kinase activity in the brain of both heterozygous and homozygous mice. Although normal at 6months, by 12months of age, basal and pharmacologically induced extracellular release of dopamine is impaired in both heterozygous and homozygous mice, corroborating previous findings in transgenic models over-expressing mutant LRRK2. Via in vivo microdialysis measurement of basal and drug-evoked extracellular release of dopamine and its metabolites, our findings indicate that exocytotic release from the vesicular pool is impaired. Furthermore, profound mitochondrial abnormalities are evident in the striatum of older homozygous G2019S KI mice, which are consistent with mitochondrial fission arrest. We anticipate that this G2019S mouse line will be a useful pre-clinical model for further evaluation of early mechanistic events in LRRK2 pathogenesis and for second-hit approaches to model disease progression.

Midbrain atrophy is not a biomarker of progressive supranuclear palsy pathology.

BACKGROUND AND PURPOSE: Midbrain atrophy is a characteristic feature of progressive supranuclear palsy (PSP), although it is unclear whether it is associated with the PSP syndrome (PSPS) or PSP pathology. The aim of the present study was to determine whether midbrain atrophy is a useful biomarker of PSP pathology, or whether it is only associated with typical PSPS. METHODS: All autopsy-confirmed subjects were identified with the PSP clinical phenotype (i.e. PSPS) or PSP pathology and a volumetric MRI. Of 24 subjects with PSP pathology, 11 had a clinical diagnosis of PSPS (PSP-PSPS), and 13 had a non-PSPS clinical diagnosis (PSP-other). Three subjects had PSPS and corticobasal degeneration pathology (CBD-PSPS). Healthy control and disease control groups (i.e. a group without PSPS or PSP pathology) and a group with CBD pathology and corticobasal syndrome (CBD-CBS) were selected. The midbrain area was measured in all subjects. [Correction added on 21 June 2013, after first online publication: the abbreviation of corticobasal degeneration pathology was changed from CBD-PSP to CBD-PSPS.] RESULTS: The midbrain area was reduced in each group with clinical PSPS (with and without PSP pathology). The group with PSP pathology and non-PSPS clinical syndromes did not show reduced midbrain area. Midbrain area was smaller in the subjects with PSPS than in those without PSPS (P < 0.0001), with an area under the receiver operator curve of 0.99 (0.88, 0.99). A midbrain area cut-point of 92 mm(2) provided optimum sensitivity (93%) and specificity (89%) for differentiation. CONCLUSION: Midbrain atrophy is associated with the clinical presentation of PSPS, but not with the pathological diagnosis of PSP in the absence of clinical PSPS. This finding has important implications for the utility of midbrain measurements as diagnostic biomarkers for PSP pathology.

Clinicopathologic correlations in 172 cases of rapid eye movement sleep behavior disorder with or without a coexisting neurologic disorder.

OBJECTIVE: To determine the pathologic substrates in patients with rapid eye movement (REM) sleep behavior disorder (RBD) with or without a coexisting neurologic disorder. METHODS: The clinical and neuropathologic findings were analyzed on all autopsied cases from one of the collaborating sites in North America and Europe, were evaluated from January 1990 to March 2012, and were diagnosed with polysomnogram (PSG)-proven or probable RBD with or without a coexisting neurologic disorder. The clinical and neuropathologic diagnoses were based on published criteria. RESULTS: 172 cases were identified, of whom 143 (83%) were men. The mean±SD age of onset in years for the core features were as follows - RBD, 62±14 (range, 20-93), cognitive impairment (n=147); 69±10 (range, 22-90), parkinsonism (n=151); 68±9 (range, 20-92), and autonomic dysfunction (n=42); 62±12 (range, 23-81). Death age was 75±9 years (range, 24-96). Eighty-two (48%) had RBD confirmed by PSG, 64 (37%) had a classic history of recurrent dream enactment behavior, and 26 (15%) screened positive for RBD by questionnaire. RBD preceded the onset of cognitive impairment, parkinsonism, or autonomic dysfunction in 87 (51%) patients by 10±12 (range, 1-61) years. The primary clinical diagnoses among those with a coexisting neurologic disorder were dementia with Lewy bodies (n=97), Parkinson's disease with or without mild cognitive impairment or dementia (n=32), multiple system atrophy (MSA) (n=19), Alzheimer's disease (AD)(n=9) and other various disorders including secondary narcolepsy (n=2) and neurodegeneration with brain iron accumulation-type 1 (NBAI-1) (n=1). The neuropathologic diagnoses were Lewy body disease (LBD)(n=77, including 1 case with a duplication in the gene encoding α-synuclein), combined LBD and AD (n=59), MSA (n=19), AD (n=6), progressive supranulear palsy (PSP) (n=2), other mixed neurodegenerative pathologies (n=6), NBIA-1/LBD/tauopathy (n=1), and hypothalamic structural lesions (n=2). Among the neurodegenerative disorders associated with RBD (n=170), 160 (94%) were synucleinopathies. The RBD-synucleinopathy association was particularly high when RBD preceded the onset of other neurodegenerative syndrome features. CONCLUSIONS: In this large series of PSG-confirmed and probable RBD cases that underwent autopsy, the strong association of RBD with the synucleinopathies was further substantiated and a wider spectrum of disorders which can underlie RBD now are more apparent.

First Report of Laurel Oak as a Host for the Pecan Root-Knot Nematode, Meloidogyne partityla, in Florida.

Roots of laurel oak (Quercus laurifolia Michx.), member of the family Fagaceae, were found to be heavily galled by the pecan root-knot nematode, Meloidogyne partityla, in two separate home gardens between 2010 and 2012, in Alachua Co., FL. Distinct round galls were observed on secondary and tertiary roots. Internally, root-knot nematode females were clearly visible when the roots were thinly sliced and egg masses were seen protruding from the root surfaces. The nematode species identification was performed using morphology of the male stylet, selected characters of the second-stage juveniles (J2), female perineal patterns, and esterase (EST) and malate dehydrogenase (Mdh) isozyme phenotypes. Morphology of perineal patterns of females, body, stylet, and tail length of the J2 and males all matched those of the original description of M. partityla (2). A swollen deeply grooved rectum was observed in the J2. The male stylet had a blunt tip with a prominent thickening at the junction between the cone and shaft. The stylet knobs of males and females were bipartite, each incised by a deep medium longitudinal groove (2). The isozyme phenotypes (EST = Mp3; Mdh = N1a) were consistent with those previously reported for M. partityla from Florida (1). Mitochondrial DNA (mtDNA) (3) and ribosomal internal transcriber spacer (ITS) DNA (4) of females were amplified to further confirm the nematode species identification. The mtDNA amplification using the C2F3/1108 primer set (3) and the ITS amplification using a recently available M. partityla specific primer set (4) produced fragments of approximately 530 bp and 550 bp, respectively. These were consistent with those already reported for this nematode species. This first report of a plant host for the pecan root-knot nematode outside of the family Juglandaceae indicates that the nematode may have migrated from Quercus species to pecan trees during the period when orchards were being established in Florida. To our knowledge, this is the first report of the pecan root-knot nematode infecting laurel oak. References: (1) J. A. Brito et al. Nematology 10:757, 2008. (2) Kleynhans, K. P. N. Phytophylatica 18:103, 1986. (3) T. O. Powers et al., J. Nematol. 37:226, 2005. (4) R. A. Stamler. M. S. thesis, New Mexico State University, Las Cruces, 2009.

Pathology and temporal onset of visual hallucinations, misperceptions and family misidentification distinguishes dementia with Lewy bodies from Alzheimer's disease.

OBJECTIVE: To determine whether the temporal onset of visual phenomena distinguishes Lewy body disease (LBD) from Alzheimer's disease (AD), and to characterize the extent Lewy bodies and neurofibrillary tangles are associated with these clinical features. METHODS: Consecutive cases of autopsy-confirmed LBD (n = 41), AD (n = 70), and AD with amygdala-predominant Lewy bodies (AD-ALB) (n = 14) with a documented clinical history of dementia were included. We mailed questionnaires to next-of-kin asking about symptoms during life. Lewy pathology and neurofibrillary tangle pathology were assessed. RESULTS: The occurrence of visual hallucinations, misperceptions and family misidentification did not distinguish LBD from AD or AD-ALB, but the onset was earlier in LBD compared to AD and AD-ALB. When visual hallucinations developed within the first 5 years of dementia, the odds were 4-5 times greater for autopsy-confirmed LBD (or intermediate/high likelihood dementia with Lewy bodies) and not AD or AD-ALB. In LBD, limbic but not cortical Lewy body pathology was related to an earlier onset of visual hallucinations, while limbic and cortical Lewy body pathology were associated with visual misperceptions and misidentification. Cortical neurofibrillary tangle burden was associated with an earlier onset of misidentification and misperceptions in LBD and AD, but only with earlier visual hallucinations in AD/AD-ALB. CONCLUSION: When visual hallucinations occur within the first 5 years of the dementia, a diagnosis of LBD was more likely than AD. Visual hallucinations in LBD were associated with limbic Lewy body pathology. Visual misperceptions and misidentification delusions were related to cortical Lewy body and neurofibrillary tangle burden in LBD and AD/AD-ALB.

Sporadic diffuse leucoencephalopathy with axonal spheroids: report of a profuse and rapid cortical-spinal degeneration.

Diffuse leucoencephalopathy with axonal spheroids (DLS) is a rare disease affecting the white matter leading to dementia and progressive motor impairment. The neuropathological hallmark includes axonal swelling and spheroids as well as myelin loss. We report a case of a 46-year-old man with memory deficit and behavioral changes followed by a rapid cognitive decline and pyramidal syndrome. Head magnetic resonance imaging showed cortical atrophy of the brain and symmetric corticospinal tract involvement. He died 4 years after the first symptoms. Autopsy was performed and the brain revealed cortical and corpus callosum atrophy, a grayish granular appearance of the white matter and ventricular enlargement. Myelin stains showed a significant demyelination of the centrum ovale and corticospinal tract. Such degeneration was accompanied by axonal loss, axonal swelling, and numerous spheroids. There was no pigment overload or inflammation. We discuss this new DLS case with bilateral, severe, and rapid cortical-spinal involvement.

Neuropathological analysis of brainstem cholinergic and catecholaminergic nuclei in relation to rapid eye movement (REM) sleep behaviour disorder.

AIMS: Rapid eye movement sleep behaviour disorder (RBD) is characterized by loss of muscle atonia during rapid eye movement sleep and is associated with dream enactment behaviour. RBD is often associated with α-synuclein pathology, and we examined if there is a relationship of RBD with cholinergic neuronal loss in the pedunculopontine/laterodorsal tegmental nucleus (PPN/LDT), compared to catecholaminergic neurones in a neighbouring nucleus, the locus coeruleus (LC). METHODS: This retrospective study utilized human brain banked tissues of 11 Lewy body disease (LBD) cases with RBD, 10 LBD without RBD, 19 Alzheimer's disease (AD) and 10 neurologically normal controls. Tissues were stained with choline acetyl transferase immunohistochemistry to label neurones of PPN/LDT and tyrosine hydroxylase for the LC. The burden of tau and α-synuclein pathology was measured in the same regions with immunohistochemistry. RESULTS: Both the LC and PPN/LDT were vulnerable to α-synuclein pathology in LBD and tau pathology in AD, but significant neuronal loss was only detected in these nuclei in LBD. Greater cholinergic depletion was found in both LBD groups, regardless of RBD status, when compared with normals and AD. There were no differences in either degree of neuronal loss or burden of α-synuclein pathology in LBD with and without RBD. CONCLUSIONS: Whether decreases in brainstem cholinergic neurones in LBD contribute to RBD is uncertain, but our findings indicate these neurones are highly vulnerable to α-synuclein pathology in LBD and tau pathology in AD. The mechanism of selective α-synuclein-mediated neuronal loss in these nuclei remains to be determined.

First Report of Meloidogyne enterolobii Infecting Euphorbia punicea in Florida.

Euphorbia punicea (Euphorbiaceae), commonly known as Jamaican poinsettia, is an evergreen shrub with dark green leaves and flashy red bracts. Bracts are sharply contrasted by rosettes of dark green leaves and can be observed in early summer, spring, fall, and winter. This shrub, native to Jamaica, is suitable in southern climates both in the landscape and as a seasonal patio container plant. Outdoors, the plants can reach as high as 5 meters. In January of 2012, E. punicea plants growing in an ornamental nursery in Dade Co., Florida, were observed with stunted growth and yellowing leaves. Root systems of affected plants were collected and sent to the Florida Department of Agriculture and Consumer Services, Nematology Laboratory, Gainesville, FL. Root systems showing symptoms of root-knot nematode infections were heavily galled and had already started rotting. Galls were observed in the primary, secondary, and tertiary roots. Species identification was initially performed using morphology and morphometrics. The morphology of the perineal patterns and measurements of selected characters of the second-stage juveniles fit those of the original description for M. enterolobii (3). The nematode species identification was confirmed using PCR to amplify mtDNA with the C2F3/1108 primer set (1) and a species-specific SCAR primer set, MK7-F/MK7-R (2). The PCR products were approximately 700 bp for mtDNA and approximately 520 bp for the SCAR, which were identical to those previously reported for M. enterolobii (1, 2). Sanitation practices should be implemented to avoid the spread of this nematode species within and between ornamental nurseries. Planting material should be produced in media free of this pathogen to avoid its introduction into uninfested nurseries and landscape areas. M. enterolobii has a wide host range, including cover and vegetable crops, fruit trees, herbs, and ornamental and weed plants. To our knowledge, this is the first report worldwide of E. punicea as a host of M. enterolobii. References: (1) T. O. Powers et al. J. Nematol. 37:226, 2005. (2) M. S. Tigano et al. Plant Pathol. 59:1054, 2010. (3) B. Yang and J. D. Eisenback. J. Nematol. 15:381, 1983.