Cognitive and metabolic correlates of emotional vulnerability in patients with temporal lobe epilepsy.
J Neurol Neurosurg Psychiatry. 2012 Jan 31;
Authors: Lanteaume L, Guedj E, Bastien-Toniazzo M, Magalahaes A, Mundler O, Bartolomei F
Abstract
BackgroundRecent investigations have suggested that the occurrence of epileptic seizures is not completely random. In particular, various types of psychological changes or life events may act as triggering factors.ObjectiveTo identify a possible link between self-perception of the impact of affective precipitants, cognitive responses modulated by aversive information and brain metabolic modifications in patients with temporal lobe epilepsy (TLE).MethodsThe extent to which seizures were elicited or not by emotional precipitants was estimated using a self-reported scale, allowing distinction of two groups: ‘Emo-TLE’ group (patients reporting to have seizures triggered by emotional events) and ‘Other-TLE’ group, which were compared with healthy individuals (‘control’ group). Attentional biases were investigated using the probe detection paradigm, using negative and neutral stimuli. Interictal brain metabolism was studied using FDG-PET, and comparison was made between controls, Emo-TLE and Other-TLE groups.ResultsPatients with emotional vulnerability (Emo-TLE) disclosed specific attentional biases towards negative stimuli compared with the Other-TLE and control groups. Patients with Emo-TLE also exhibited specific hypometabolism in the anterior temporal lobe, including amygdala and hippocampus. The degree of attentional biases correlated with decreased metabolism in these regions.ConclusionsThis investigation shows that the impact of affective events is the result of self-perception and also that it might be determined by specific cognitive and brain metabolic modifications in TLE.
PMID: 22298841 [PubMed - as supplied by publisher]
PurposeThe study investigates the effects of genetic factors on the pathology of Alzheimer’s disease (AD) and Lewy body (LB) diseases, including Parkinson’s disease and dementia with Lewy bodies.MethodsA multicentre autopsy series (762 brain samples) with AD, LB or vascular pathology was examined. The effects of the tau gene (MAPT) H1 haplotype, the H1 specific SNP rs242557, APOE and the α-synuclein gene (SNCA) 3′UTR SNP rs356165 on the burden of AD and LB pathology were assessed. Neurofibrillary tangles (NFTs) were counted in four brain regions, senile plaques in five and LBs in four. Braak NFT stage, brain weight and presence of vascular pathology were also documented.ResultsMAPT H1 associated with lower counts of NFTs in the middle frontal (p<0.001) and inferior parietal (p=0.005) cortices, and also with lower counts of senile plaques in the motor cortex (p=0.001). Associations of MAPT H1 with increased LB counts in the middle frontal cortex (p=0.011) and inferior parietal cortex (p=0.033) were observed but were not significant after multiple testing adjustment. The APOE ε4 allele was strongly associated with overall Alzheimer type pathology (all p≤0.001). SNCA rs356165 and the MAPT H1 specific SNP rs242557 did not associate with AD or LB pathology.ConclusionThis study shows for the first time that MAPT H1 is associated with reduced Alzheimer type pathology which could have important implications for the understanding of disease mechanisms and their genetic determinants.
PMID: 22291217 [PubMed - as supplied by publisher]
Social Cognition and Emotional Assessment differentiates frontotemporal dementia from depression.
J Neurol Neurosurg Psychiatry. 2012 Jan 29;
Authors: Bertoux M, Delavest M, de Souza LC, Funkiewiez A, Lépine JP, Fossati P, Dubois B, Sarazin M
Abstract
BackgroundBehavioural variant of frontotemporal dementia (bvFTD) is a neurodegenerative disease that is clinically characterised by progressive behavioural changes and social interpersonal dysfunctions. Its diagnosis remains a clinical challenge, and depression is one of the main causes of misdiagnoses due to the prevalence of apathy in bvFTD.ObjectiveTo evaluate the sensitivity and specificity of the Social Cognition and Emotional Assessment (SEA) and the mini-SEA for differentiating bvFTD from major depressive disorder (MDD).MethodsScores for the SEA and mini-SEA for 37 patients with bvFTD (divided into subgroups of 17 with early bvFTD and 20 with moderate bvFTD according to the normal range of the Mattis Dementia Rating Scale), 19 MDD patients and 30 control subjects were compared to define the discrimination power of these tools compared with other standard neuropsychological tests.ResultsSEA and mini-SEA scores were significantly lower for both the early and moderate bvFTD groups compared with control subjects and the MDD group, and very few scores overlapped between patients in the bvFTD subgroups and patients in the MDD and control subgroups. SEA and mini-SEA scores distinguished early bvFTD from MDD with sensitivity and specificity rates above 94%.ConclusionUnlike standard executive neuropsychological tests, SEA and the mini-SEA can differentiate MDD from bvFTD in the early stages of the disease. The mini-SEA is an easy tool that can be utilised in neurological or psychiatric departments.
PMID: 22291219 [PubMed - as supplied by publisher]
Clinical added value of magnetic source imaging in the presurgical evaluation of refractory focal epilepsy.
J Neurol Neurosurg Psychiatry. 2012 Jan 19;
Authors: De Tiège X, Carrette E, Legros B, Vonck K, Op de Beeck M, Bourguignon M, Massager N, David P, Van Roost D, Meurs A, Lapere S, Deblaere K, Goldman S, Boon P, Van Bogaert P
Abstract
ObjectiveThis prospective, bicentre, blinded, intention to treat study assessed the clinical added value of magnetic source imaging (MSI) in the presurgical evaluation of patients with refractory focal epilepsy (RFE).Methods70 consecutive patients with RFE (42 men; mean age 31.5 years, range 3-63) from two Belgian centres were prospectively included. All patients underwent conventional non-invasive presurgical evaluation (CNIPE) and a whole head magnetoencephalography recording (Elekta Neuromag). Equivalent current dipoles corresponding to interictal epileptiform discharges (IED) were fitted in the patients’ spherical head model and coregistered on their MRI to produce MSI results. Results of CNIPE were first discussed blinded to the MSI results in respective multidisciplinary epilepsy surgery meetings to determine the presumed localisation of the epileptogenic zone and to set surgical or additional presurgical plans. MSI results were then discussed multidisciplinarily. MSI influence on the initial management plan was assessed.ResultsBased on CNIPE, 21 patients had presumed extratemporal epilepsy, 38 had presumed temporal epilepsy and 11 had undetermined localisation epilepsy. MSI showed IED in 52 patients (74.5%) and changed the initial management in 15 patients (21%). MSI related changes were significantly more frequent in patients with presumed extratemporal or undetermined localisation epilepsy compared with patients with presumed temporal epilepsy (p≤0.001). These changes had a clear impact on clinical management in 13% of all patients.ConclusionMSI is a clinically relevant, non-invasive neuroimaging technique for the presurgical evaluation of patients with refractory focal epilepsy and, particularly, in patients with presumed extratemporal and undetermined localisation epilepsy.
PMID: 22262910 [PubMed - as supplied by publisher]
The Kurtzke EDSS rank stability increases 4 years after the onset of multiple sclerosis: results from the MSBase Registry.
J Neurol Neurosurg Psychiatry. 2011 Dec 28;
Authors: Hughes S, Spelman T, Trojano M, Lugaresi A, Izquierdo G, Grand’maison F, Duquette P, Girard M, Grammond P, Oreja-Guevara C, Hupperts R, Boz C, Bergamaschi R, Giuliani G, Rio ME, Lechner-Scott J, van Pesch V, Iuliano G, Fiol M, Verheul F, Barnett M, Slee M, Herbert J, Kister I, Vella N, Moore F, Petkovska-Boskova T, Shaygannejad V, Jokubaitis V, McDonnell G, Hawkins S, Kee F, Gray O, Butzkueven H,
Abstract
BackgroundThe Expanded Disability Status Scale (EDSS) is widely used to rate multiple sclerosis (MS) disability, but lack of disease duration information limits utility in assessing severity. EDSS ranking at specific disease durations was used to devise the MS Severity Score, which is gaining popularity for predicting outcomes. As this requires validation in longitudinal cohorts, we aimed to assess the utility of EDSS ranking as a predictor of 5-year outcome in the MSBase Registry.MethodsRank stability of EDSS over time was examined in the MSBase Registry, a large multicentre MS cohort. Scores were ranked for 5-year intervals, and correlation of rank across intervals was assessed using Spearman’s rank correlation. EDSS progression outcomes at 10 years were disaggregated by 5-year EDSS scores.ResultsCorrelation coefficients for EDSS rank over 5-year intervals increased with MS duration: years 1-6=0.55, years 4-9=0.74, years 7-12=0.80 and years 10-15=0.83. EDSS progression risk at 10 years after onset was highly dependent on EDSS at 5 years; one-point progression risk was greater for EDSS score of >2 than ≤2. Two-point progression was uncommon for EDSS score of <2 and more common at EDSS score of 4.ConclusionsEDSS rank stability increases with disease duration, probably due to reduced relapses and less random variation in later disease. After 4 years duration, EDSS rank was highly predictive of EDSS rank 5 years later. Risk of progression by 10 years was highly dependent on EDSS score at 5 years duration. We confirm the utility of EDSS ranking to predict 5-year outcome in individuals 4 years after disease onset.
PMID: 22205675 [PubMed - as supplied by publisher]
Long term follow-up of deep brain stimulation of the caudal zona incerta for essential tremor.
J Neurol Neurosurg Psychiatry. 2011 Dec 28;
Authors: Fytagoridis A, Sandvik U, Aström M, Bergenheim T, Blomstedt P
Abstract
PurposeThe ventral intermediate nucleus of thalamus is the standard target for deep brain stimulation (DBS) in essential tremor (ET). However, favourable data have recently highlighted the caudal zona incerta (cZi) as an alternative target. Reports concerning the long-term results are however lacking, and we have therefore evaluated the long-term effects in our patients with ET and cZi DBS.Methods18 patients were evaluated using the Essential Tremor Rating Scale (ETRS) before and on-/off-stimulation at 1 and 3-5 years after surgery (mean 48.5±10.6 months). Two patients were operated on bilaterally but all electrodes were evaluated separately. The stimulation parameters were recorded and the stimulation strength calculated.ResultsA baseline total ETRS mean score of 46.0 decreased to 21.9 (52.4%) at the final evaluation. On the treated side, tremor of the upper extremity (item 5 or 6) improved from 6.1 to 0.5 (91.8%) and hand function (items 11-14) improved from 9.3 to 2.0 (78.0%). Activities of daily living improved by 65.8%. There was no increase in stimulation strength over time.ConclusioncZi DBS is a safe and effective treatment for the long term suppression of ET.
PMID: 22205676 [PubMed - as supplied by publisher]
Visual hallucinations in the differential diagnosis of parkinsonism.
J Neurol Neurosurg Psychiatry. 2012 Jan 6;
Authors: Bertram K, Williams DR
Abstract
Visual hallucinations (VH) occur commonly in Parkinson’s disease (PD) and dementia with Lewy bodies (DLB) but are reported much less frequently in other neurodegenerative causes of parkinsonism, such as progressive supranuclear palsy, multiple system atrophy and corticobasal degeneration syndrome. This clinical sign may be helpful when considering the differential diagnosis of patients with parkinsonism. The observation that VH may be specific to Lewy body pathology probably reflects a greater vulnerability of the visual systems to PD and DLB neurodegeneration compared with other diseases. Topographic differences in pathology are probably the major factor producing VH in Lewy body diseases, rather than neurophysiological changes that are specific to α-synuclein protein accumulation. VH correlate with pathology in the limbic system and more specifically the amygdale that is frequently affected in PD and DLB but relatively preserved in other forms of parkinsonism often misdiagnosed as PD. In this review, the published frequencies of VH in these different conditions are compared to put into context the notion of VH as a clinical clue to underlying Lewy body pathology.
PMID: 22228724 [PubMed - as supplied by publisher]
Autopsy confirmed multiple system atrophy cases: Mayo experience and role of autonomic function tests.
J Neurol Neurosurg Psychiatry. 2012 Jan 6;
Authors: Iodice V, Lipp A, Ahlskog JE, Sandroni P, Fealey RD, Parisi JE, Matsumoto JY, Benarroch EE, Kimpinski K, Singer W, Gehrking TL, Gehrking JA, Sletten DM, Schmeichel AM, Bower JH, Gilman S, Figueroa J, Low PA
Abstract
BackgroundMultiple system atrophy (MSA) is a sporadic progressive neurodegenerative disorder characterised by autonomic failure, manifested as orthostatic hypotension or urogenital dysfunction, with combinations of parkinsonism that is poorly responsive to levodopa, cerebellar ataxia and corticospinal dysfunction. Published autopsy confirmed cases have provided reasonable neurological characterisation but have lacked adequate autonomic function testing.ObjectivesTo retrospectively evaluate if the autonomic characterisation of MSA is accurate in autopsy confirmed MSA and if consensus criteria are validated by autopsy confirmation.Methods29 autopsy confirmed cases of MSA evaluated at the Mayo Clinic who had undergone formalised autonomic testing, including adrenergic, sudomotor and cardiovagal functions and Thermoregulatory Sweat Test (TST), from which the Composite Autonomic Severity Score (CASS) was derived, were included in the study.ResultsPatient characteristics: 17 men, 12 women; age of onset 57±8.1 years; disease duration to death 6.5±3.3 years; first symptom autonomic in 18, parkinsonism in seven and cerebellar in two. Clinical phenotype at first visit was MSA-P (predominant parkinsonism) in 18, MSA-C (predominant cerebellar involvement) in eight, pure autonomic failure in two and Parkinson’s disease in one. Clinical diagnosis at last visit was MSA for 28 cases. Autonomic failure was severe: CASS was 7.2±2.3 (maximum 10). TST% was 65.6±33.9% and exceeded 30% in 82% of patients. The most common pattern was global anhidrosis. Norepinephrine was normal supine (203.6±112.7) but orthostatic increment of 33.5±23.2% was reduced. Four clinical features (rapid progression, early postural instability, poor levodopa responsiveness and symmetric involvement) were common.ConclusionThe pattern of severe and progressive generalised autonomic failure with severe adrenergic and sudomotor failure combined with the clinical phenotype is highly predictive of MSA.
PMID: 22228725 [PubMed - as supplied by publisher]
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