bluuebunny/crossref_metadata_2025 huggingface dataset:
Monoballism revealing multiple sclerosis. Neuroradiological aspects
Authors: Rosa A. , Canaple S. , Gondry C , Touzet G. , Gérard P.
Published: January 2011

A 38‐year‐old woman suddenly showed signs of monoballism in the right upper limb. Examination of CSF showed 13 monocytes per mm3 and 28.7% of IgG in oligoclonal bands. MRI examination showed several high signal lesions in T2‐weighted images. One of these lesions was located in the left subthalamic region. Visual evoked potentials showed increased latencies for the P100 wave on the left eye. Two years later she experienced vertigo, diplopia, gait unsteadiness and left facial dysesthesia. This clinical syndrome almost completely regressed after 10 days of ACTH therapy (Synacthene). Diagnosis of multiple sclerosis was supported by radiological and neurophysiological investigations.
Paraneoplastic cerebal venous and sinus thrombosis
Authors: Kastrup O. , Koeppen S. , Diener H.‐C
Published: January 2011

A rare case of a cerebral venous and sinus thrombosis with a four year course of recurring focal neurological deficits as a paraneoplastic remote effect of a later diagnosed highly malignant lymphoma is presented. Initially heparin‐therapy and anticoagulation led to good clinical recovery. The subsequent failure of this treatment and a surprisingly good recovery immediately after chemotherapy is discussed.
Correlates of leukoaraiosis and ventricular enlargement on magnetic resonance imaging: a study in normal elderly and cerebrovascular patients
Authors: Padovani A. , Di Piero V. , Bragoni M. , Di Biase C. , Trasimeni G. , lannili M. , Laudani G. , Zanette E. , Gualdi G.F. , Lenzi G.L.
Published: January 2011

Several studies have repeatedly demonstrated that leukoaraiosis as well as ventricular enlargement are common findings in normal elderly and in stroke patients, although there is no general consensus on prevalence rate as well as on their clinical correlations. It is also controversial whether white matter changes and ventricular enlargement are reciprocally related. In this study we investigated the prevalence and extent of white matter hyperintensities and the degree of ventricular enlargement on magnetic resonance imaging in 50 normal elderly individuals (mean age 62.1 ± 7.3 years) and in 50 consecutive chronic ischemic stroke patients (mean age 66.1 ± 7.7 years). All subjects underwent extensive clinical assessment. White matter hyperintensities were graded from 0 to 3 on a semi‐quantitative scale, while bifrontal horn, bicaudate, and third ventricle ratio indices were used as measures of brain atrophy. Hypertension, diabetes, alcohol consumption, cardiac disease, carotid pathology occurred significantly more often in patients than in controls. Prevalence rates of white matter hyperintensities were 30% in controls and 82% in patients. Patients had significantly larger ventricular indices than controls. Significant univariate correlations for the extent of white matter hyperintensities were found with age, sex, hypertension, cardiac disease, carotid pathology, diabetes, history of stroke and ventricular enlargement. Age, sex, cardiac disease, alcohol habit, cerebrovascular disease and extent of white matter hyperintensities correlated with severity of ventricular enlargement. Multivariate regression analysis identified age, hypertension and history of stroke as independent predictors of white matter hyperintensities, while history of stroke, age and alcohol consumption were found as the only independent predictors of ventricular enlargement Separate analysis between periventricular, subcortical or deep white matter hyperintensities and each of the three ventricular indices failed to show a significant association after adjustment for clinical and demographic factors. We suggest that leukoaraiosis and ventricular enlargement are independent pathological processes associated with different risk factors in addition to age and stroke disease.
SEPs N30 amplitude in Parkinson's disease and in pharmacologically induced rigidity: relationship with the clinical status
Authors: Stanzione P. , Traversa R. , Pierantozzi M. , Semprini R. , Loberti M. , Peppe A. , Santilli A. M. , Bernardi G.
Published: January 2011

The frontal N30 wave amplitude of somatosensory evoked potentials (SEPs) has been studied in 41 Parkinson's disease (PD) patients (pts) in a basal condition and compared to that of 30 normal subjects; moreover the N30 amplitude and clinical motor score have been evaluated in a subgroup of 30 PD pts before and during apomorphine infusion and in a second subgroup of 22 PD pts also during levodopa chronic therapy. The data show that N30 amplitude is decreased in PD pts in basal condition and increased following both treatments by a percentage proportional to the clinical improvement Analysis by non parametric correlations showed that the increase is well correlated to the clinical score amelioration induced by apomorphine in the more affected side. The best correlation was to rigidity score amelioration in the group of PD pts in medium stage (Hohen and Yahr stage between 2 and 3), suggesting a relationship between the rigidity and N30 amplitude decrease. Non parkinsonian subjects, treated with low (11 aged normal subjects) and high (eight young psychotic pts) doses of antidopaminergic drugs, were studied. N30 amplitude decreases were only found in the group of eight psychotic pts showing clinical extrapyramidal signs, produced by the high dose of drug administered, but not in the group treated with the lower dose not producing extrapyramidal side effects, although this dose was efficacious on different modalities of evoked potentials. We conclude that N30 amplitude decrease in PD reflects the dopaminergic lack paralleled by clinical symptoms. We propose that N30 amplitude variations by dopaminergic agonists may be useful in the clinical evaluation of dopamine related and non related tone alterations.
A novel mutation of SOD‐1 (Gly 108 Val) in familial amyotrophic lateral sclerosis
Authors: Orrell R. W. , Habgood J.J. , Shepherd D.I. , Donnai D. , de Belleroche J.
Published: January 2011

A novel mutation of the SOD‐1 gene which encodes the enzyme copper‐zinc superoxide dismutase was identified in a family manifesting amyotrophic lateral sclerosis (ALS) in three generations. The mutation is a heterozygote point mutation in exon 4, codon 108 (GGA to GTA), predicting the substitution of valine for glycine. The mutation creates a new restriction site for the endonuclease AccI. The mutation was demonstrated in two affected members of the family, who show features of autosomal dominant inheritance of ALS, but variable age at onset ranging from 48 to 72 years. Over 30 different mutations of SOD‐1 have now been identified in families with ALS. The definition of the different mutations causing human disease may allow further investigation of their pathogenicity in transgenic animal models, and also offers insight into the variable phenotypic disease expression both within and between genotypes.
Cytokines and soluble IL‐4 in patients with acute optic neuritis and multiple sclerosis
Authors: Sellebjerg F. , Bendtzen K. , Christiansen M. , Frederiksen J.
Published: January 2011

We measured the cerebrospinal fluid (CSF) and plasma concentrations of interleukin (IL)‐1α, IL‐1β, IL‐2, IL‐4, IL‐6, IL‐10, tumor necrosis factor (TNF)‐α, TNF‐β, interferon (IFN)‐γ, the IL‐1 receptor antagonist, and soluble IL‐4 receptor (sIL‐4r) by ELISA in 12 patients each with acute, monosymptomatic, idiopathic optic neuritis (ON), ON as part of MS, other attack forms of MS, and in neurological control subjects. CSF concentrations of IL‐1β, IL‐2 and IFN‐γ differed significantly between the different patient groups and were detected most commonly at the highest concentrations in patients with non‐ON attacks of MS. TNF‐β was detected exclusively in CSF from neurological control patients. The patients with non‐ON attacks of MS also had significantly elevated concentrations of sIL‐4r in plasma. Increased CSF concentrations of IL‐1β, IL‐2 and IFN‐γ together with increased plasma concentrations of sIL‐4r support the concept of MS as an autoimmune disease with preferential activation of proinflammatory or T‐helper type 1‐like cells. Patients with idiopathic ON or ON as part of MS may, however, differ immunologically from patients with other attack forms of MS.
Change in the pattern of cervical dystonia might be the cause of benefit loss during botulinum toxin treatment
Authors: Kaňovský P. , Dufek J. , Halačková H. , Rektor I.
Published: January 2011

The muscular patterns of cervical dystonia were identified by polymyographic recordings in 76 patients before botulinum toxin treatment. The leading muscles were considered to be those which started dystonic movement and which showed constant and maximal activity during all dystonic movements. The dystonic muscles were repeatedly treated by local Injections of botulinum toxin. Sixteen patients showed (after repeated injections) loss of the benefit of local applications of botulinum toxin after various periods of time. Repeated polymyographic recordings were performed in these patients during the loss of the benefit of injected botulinum toxin. In four patients repeated polymyographic recordings showed an Identical pattern of cervical dystonia, but the activity of previously injected muscles was apparently decreased. In 12 patients only minimum or no activity was recorded in muscles which had previously been treated with botulinum toxin, but the pattern of cervical dystonia was changed. Different patterns of cervical dystonia with different leading muscles, but with identical directions of head deviation, were observed in six patients. In another six patients, the head deviation direction was to the opposite side and was accompanied by a change of the leading muscle and a change of the muscular pattern of dystonia. These results suggest either that dystonic activity from the cerebral generator changes to new effectors during the peripheral blockade of primary dystonic muscles, or that a change of generators at different levels of the CNS occurs. It may be neccessary to carry out repeated polymyographic recordings throughout the period of loss of benefit of previously successful local botulinum toxin injections.
Streptococcus salivarius meningitis: a case report and literature review
Authors: Berrouschot J. , Sterker M. , Schneider D.
Published: January 2011

Twelve hours after spinal anaesthesia, a 61‐year‐old patient developed meningitis with fever, somnolence, headache and stiffness of the neck. The cerebrospinal fluid was found to contain 5.279/mm3 cells (95 granulocytes), 12.800 mg/l protein and 14.0 mmol/l lactate. Streptococcus salivarius was detected in the culture. The patient was treated with antibiotics (initially cefotaxime, fosfomycin and gentamycin, later piperacillin and sulbactam). Restitutio ad integrum took place after 7 days. Thirteen cases of Streptococcus salivarius meningitis over the past 40 years are described in the literature, nine of which occurred following spinal puncture. Streptococcus salivarius was probably communicated by the medical personnel from the oral cavity when working without masks. The outcome in each case was good. In our opinion suitable masks must be worn during spinal puncture. Although iatrogenic infection is uncommon, it cannot be ruled out.
Metabolic and Degenerative Diseases of the Nervous System
Authors: Jellinger K. A.
Published: January 2011

Book reviewed in this article:

Pathology, Biochemistry and Genetics. J. Cervos‐Navarro and H. Ulrich
Neurologic education for the future: a decade of curricular reform at Harvard Medical School
Authors: Glick T. H. , Armstrong E. G. , Hyman S. E. , Hundert E. M. , Furshpan E. J.
Published: January 2011

The field of neurology is undergoing significant changes to which curricular reform is both responding and contributing. We reflect on a decade of experience at Harvard Medical School with integration of neuroscience, behaviour, pathophysiology and introductory clinical skills. As part of Harvard's “New Pathway” curriculum, this coordinated, pre‐clerkship program embraces a “hybrid” form of problem‐based learning. A variety of methods are employed synergistically to meet the two broad goals of preparing for competency in neurologic clerkships and for career‐long learning in clinically relevant neuroscience. We articulate specific ways of elevating the level of intellectual inquiry, involving multi‐disciplinary faculty more productively, and vertically integrating the learning experience through the years of medical school.
Patients with heart attacks are not valid models for medial temporal lobe amnesia. A neuropsychological and FDG‐PET study with consequences for memory research
Authors: Markowitsch H. J. , Weber‐Luxemburger G. , Ewald K. , Kessler J. , Heiss W.‐D.
Published: January 2011

Equating the condition after cardiac arrest with that of medial temporal damage, and consequently medial temporal lobe amnesia, is questioned on the basis of results from a patient who was studied neuropsychologically as well as with static and dynamic imaging methods (MRI, PET) 6–9 months after a heart attack. The patient manifested severe and persistent anterograde and retrograde amnesia, as well as further cognitive deteriorations. While MRI only indicated non‐specific cortical atrophy, PET revealed a severe bilateral affection of the thalamus and of both medial and lateral temporal cortices as well as occipito‐parietal hypometabolism. The neuropsychological status indicates that patients with a diagnosis of cardiac arrest may suffer very severe and persistent cognitive deficits; the imaging analyses show that cardiac arrests may lead to quite severe and widespread brain damage which, however, may not be visible with current magnetic resonance imaging technology, but which is clearly apparent from positron emission tomography. These data suggest that patients with a condition after a heart attack may not be valid models for pure hippocampal—or even medial temporal lobe—pathology, as they may suffer much more widespread brain damage.
Infectious meningitis with atypical cerebrospinal fluid cells
Authors: García‐Moncó J. C. , Beldarrain M. G. , Zabalza I.
Published: January 2011

Cytologic evaluation of the CSF is often difficult when trying to distinguish between truly neoplastic and reactive cells. Several non‐neoplastic conditions may be associated with atypical cells in the CSF, a fact the clinician has to consider to avoid inadequate aggressive theraphies. We report here three patients with infectious meningitis (due to Herpes zoster virus in two, and neuroborreliosis in one) and cytologically atypical cerebrospinal fluid lymphocytes. Further characterization showed that the pleocyrosis in these patients was of reactive origin. Cytomorphology is frequently insufficient and histochemical, immuncytochemical and cellular genome analysis techniques may help differentiate atypical reactive cells from neoplastic cells.
Neurally mediated syncope: pathophysiology, investigations and treatment
Authors: Jellinger K. A.
Published: January 2011

Book reviewed in this article:

The Bakken Research Centre Series, Vol. 10. Edited by J.‐J. Blanc, D. Benditt and R. Sutton.
Handbook of muscle disease
Authors: Jellinger K. A.
Published: January 2011

Book reviewed in this article:

Neurological Disease and Therapy Series, Vol. 42. Edited by R.J.M. Lane.
Follow‐up of 53 Alzheimer patients with the MODA (Milan Overall Dementia Assessment)
Authors: Capitani E. , Manzoni L , Spinnler H.
Published: January 2011

Fifty‐three patients affected by Alzheimer's disease entered a longitudinal survey aimed at studying which factors influence the rate of progression, assessed by means of the MODA (Milan Overall Dementia Assessment). The second examination was carried out, on average, after 16 months from the first assessment. Only age proved to influence the decline rate, which was faster in elders.
Asymmetric dystonia with frontal white matter lesions in Wilson's disease
Authors: Huang C.‐C. , Chu N.‐S. , Chen R.‐S.
Published: January 2011

Asymmetrical manifestation of dystonia was investigated in 26 patients with Wilson's disease with modified dystonia rating scale and correlated with the asymmetricity of CT and/or MRI findings. Ten patients (eight men and two women) had dystonia. The age of disease onset ranged from 11 to 30 years with a mean of 18.6 ± 5.2 years. The duration of the illness was from 2 to 15 years (mean 7.0; S.D. 3.6). Six patients had subcortical white matter lesions in the frontal and/or parietal lobes, as well as lesions in the basal ganglia. Five patients, who had asymmetrical white matter lesions in the frontal lobes and symmetrical lesions in the basal ganglia and the thalamus, developed more severe contralateral dystonia. The other four patients with symmetric lesions in the basal ganglia and the thalamus had nearly‐symmetrical dystonia. One patient with symmetrical lesions in the frontal lobes and the basal ganglia also had symmetrical manifestation of dystonia. We suggest that the interruption of the loop between the thalamus and premotor cortex by subcortical white matter lesions may enhance contralateral dystonia.
What causes hemiataxia after lacunar ischaemic stroke? A cliniconeurophysiological study
Authors: Luijckx G. J. , Spaans F. , Boiten J. , Lodder J.
Published: January 2011

Both cerebellar dysfunction and proprioceptive deficits have been described as the cause of the ataxia in patients with ataxic hemiparesis. In order to determine the cause of the ataxia, we investigated the sensory modalities and performed median nerve somatosensory evoked potentials (SEPs) in 19 patients with hemiataxia following lacunar ischaemic stroke. All patients had computer tomography (CT) and/or magnetic resonance imaging (MRI) of the brain. SEP results were reviewed for correlation with clinical features and infarct location and size. In all patients, eye closure did not worsen the ataxia. There was no difference between the mean latency of the SEP components from the affected and non‐affected hemisphere of the brain in all patients. Only two of the 19 patients had abnormal SEPs. There was no correlation between SEP findings, clinical features and location or size of the infarcts. The discovery of normal SEPs in most cases and the clinical aspects of the ataxia suggest that hemiataxia following lacunar ischaemic stroke is usually of the cerebellar type and that disturbed proprioception is unlikely as the cause in most patients. Cerebellar‐like ataxia is most likely caused by disruption of the cerebellar pathways at the level of the internal capsule or corona radiata, i.e. either the ascending dentatorubrothalamocortical or the descending corticopontocerebellar pathways.
Micturition disorders in multiple sclerosis patients: neurological, neurourodynamic and magnetic resonance findings
Authors: Patti F. , Ventimiglia B. , Failla G. , Genazzani A. A. , Reggio A.
Published: January 2011

Micturition disorders are frequent in an advanced course of multiple sclerosis. Historical records of multiple sclerosis patients reveal that urinary symptoms occur with high frequence. In the present study we have investigated all patients who were admitted to our Multiple Sclerosis Center in a 1 year period. AH patients underwent a neurological examination corroborated by MRI investigation. Out of 101 patients, 75 who reported urinary symptoms, underwent neurourodynamic investigation. Sixty of them exhibited functional micturition abnormalities, which were strictly correlated to EDSS, pyramidal and cerebellar functional statuses scores. Detrusor hyperreflexia was the commonest abnormality. Neurourodynamic findings were also correlated to the burden of lesion, although MRI seems to fail in localizing specific neural pathways which control micturition phases. Moreover, urodynamic evaluation can select different groups of MS patients with different degrees of neurological impairment.
Progressive upper limb monoparesis: a form of primary lateral sclerosis? Two cases with metabolic brain imaging and transcranial magnetic stimulation
Authors: Cohen L , Brazin B. , Sirigu A. , Meininger V. , Pierrot‐Deseilligny C
Published: January 2011

Two patients presenting an isolated, slowly‐progressive clumsiness affecting one upper limb are described. Clinical evidence, regional cerebral blood flow and transcranial magnetic motor stimulation suggested that this condition resulted from a selective unilateral degeneration of the corticospinal tract. Such progressive hemiparesis may possibly be considered as a rare form of primary lateral sclerosis.
Unusual association of neurofibromatosis Type 1 and idiopathic syringomyelia—bulbia—pontia
Authors: Cakmur R. , Fadiloglu S. , Pirnar T.
Published: January 2011

Since the association of syringomyelia and neurofibromatosis is mostly related to intramedullary tumors, syringomyelia in neurofibromatosis type 1 (NF‐1) is much rarer than in neurofibromatosis type 2 (NF‐2). In this paper, we present a case of NF‐1 associated with an idiopathic syringomyelia extending from conus medullaris to the upper pons and discuss the relationship between neurofibromatosis and syringomyelia.
Vascular Dementia
Authors: Jellinger K. A.
Published: January 2011

Book reviewed in this article:

D. Leys and Ph. Scheltens (Eds). In: Current Issues in Neurodegenerative Diseases
The role of facial nerve conduction studies and electromyography in predicting the outcome of Bell's palsy
Authors: Yamout B. I. , Zaytoun G. , Nuweihed I.
Published: January 2011

Many electrophysiological tests have been used to determine prognosis and extent of recovery in Bell's palsy but the reliability and sensitivity of the different parameters used is still controversial. We performed bilateral percutaneous facial nerve conduction studies, and volitional needle electromyography on 23 patients within 10–14 days post onset of their Bell's palsy. The following parameters were assessed: denervation and recruitment of the frontalis and orbicularis oris muscles, latency of the compound muscle action potential (CMAP), and CMAP amplitude ratio. The patients were re‐examined 6 months later and their recovery graded according to the House‐Brackman classification. The CMAP amplitude ratio and the recruitment scores of the frontalis and orbicularis oris muscles were the only parameters to reliably predict outcome (p = 0.016, 0.007 and 0.036, respectively). All patients with a CMAP amplitude ratio above 10% had a complete recovery. Since Bell's palsy is probably caused by herpes simplex virus, the active disease process is completed within 10–14 days; therefore, facial nerve conduction studies and electromyography at that time are appropriate to predict prognosis.
The cost of severe refractory epilepsy: comparison of patient response to adjunct vigabatrin
Authors: Souêtre E. , Taïeb C. , Lozet H. , Boilait C. , Castelain V. , Bacquet P.
Published: January 2011

Data from 112 patients with severe, refractory, partial epilepsy, who were treated with adjunct vigabatrin, were used to compare the cost‐of‐care for patients who responded to the therapy and those who did not. For patients who responded to vigabatrin, the direct and indirect costs were lower than the costs for patients who did not respond to treatment. The major impact on direct costs was a 69% and 59% decrease in rate of hospitalization and length of stay in hospital, respectively, for the responder group. This decrease in costs outweighed the additional drug costs associated with continued vigabatrin therapy.
Bilateral central retinal artery occlusions in a patient with activated protein C resistance
Authors: Artemis N. , Heliopoulos J. , Makris P. , Piperidou H. , Milonas J.
Published: January 2011

We describe the case of a 42‐year‐old woman who had two episodes of visual loss, first in one and later in the other eye, which caused bilateral reduction of visual acuity to 2/10 with optic atrophy. Detailed clinical and laboratory assessment revealed an activated protein C (APC) resistance and no other obvious cause. APC resistance has not been previously reported as an etiologic factor of central retinal artery occlusion.
Lupus anticoagulant and Sneddon's syndrome
Authors: Lousa M. , Gobernado J. M. , Cervero C. , Perez‐Corral F. , Pardo A.
Published: January 2011

We present the coagulation and serological studies of four patients with Sneddon's syndrome, in which antiphospholipid antibodies (anticardiolipin antibodies and lupus anticoagulant) were determined using cardiolipin and a mixture of phospholipid from rabbit brain as antigen for detection of lupus anticoagulant by ELISA. Our results support a relation between Sneddon's syndrome and lupus anticoagulant (IgG subtype) in all cases. The anticardiolipin antibody test was positive only in two cases (one in low level). All patients could be diagnosed as having primary antiphospholipid antibody syndrome. Antiaggregant treatment was not effective in preventing thrombosis in two cases. Three of four patients received long‐term oral anticoagulation therapy, with no recurrence of thrombosis observed for a period of at least 3 years.
Interhemispheric EEG coherence after corpus callosotomy
Authors: Brázdil M. , Brichta J. , Krajča V. , Kuba R. , Daniel P.
Published: January 2011

Interhemispheric coherence analysis of scalp electroencephalograms (EEGs) in three intractable epileptic patients before and after surgical section of the anterior part of the corpus callosum was performed. Equidistant EEG periods recorded under resting conditions free of interictal epileptic activity and periods with generalized spike‐and‐wave discharges were investigated. Mean coherence values for five frequency EEG bands (delta, theta, alpha, beta 1 and beta 2) before and after corpus callosotomy were compared with each other and statistically evaluated. Anterior callosotomy led, in all cases, to a decrease of interhemispheric EEG coherence in most localizations, with the most profound decreases in the region corresponding to the section. These results support the hypothesis that the corpus callosum plays an important role in resting interhemispheric integration and EEG synchronization, similar to its central role in propagation of specific epileptic activity.
Apolipoprotein E genotype of a Portuguese control population and Alzheimer's disease patients
Authors: Rocha L. , de Mendonça A. , Garcia C. , Lechner M.C.
Published: January 2011

The present work first describes the frequency of APOE alleles and genotypes in Portuguese populations in relation to AD pathology. We genotyped a group of late onset sporadic AD cases, their pairwise controls and a larger group of randomly selected individuals, to assess the distribution pattern of APOE alleles in the Portuguese population. APOE ɛ4 relative frequency may significantly vary among different populations—a fact which should be taken into consideration for the correct evaluation of the cossegregation of this allele with AD pathology. We observed a frequency of 0.087 ± 0.029 of the APOE ɛ4 and 0.043 ± 0.021 of the APOE ɛ2 allele in the Portuguese random population which as expected showed a marked prevalence of the APOE ɛ3 form (0.870 ± 0.035). In the AD patients the APOE ɛ4 allele frequency was significantly higher (0.360 ± 0.081) than in the controls (χ2 = 31.000, p < 0.00001, df = 2). Consistently APOE ɛ3 allele frequency (0.640 ± 0.081) was significantly lower than in the controls while APOE ɛ2 was absent in the studied AD population. Taken together our results demonstrate that the Portuguese population is characterized by a relatively low frequency of the APOE ɛ4 allele, in good agreement with previous observations of a gradient of ɛ4 allele frequency in Europe, decreasing from North to South. Several lines of evidence point at APOEɛe4 allele as a major genetic susceptibility factor in AD. The APOE ɛ4 allele was significantly higher [odds ratio (OR) = 5.93, 95% CI 3.55–9.91] in the Portuguese AD patients than in the random non‐demented population. The genotype analysis of the Portuguese AD patients here described reveals a marked, increased frequency of ɛ4 homo‐ and heterozygous individuals consistent with an APOE ɛ4 zigosity effect as a further genetic trait predisposing to AD development.
Onset‐age dependent MRI based hippocampal volume asymmetries in intractable partial complex temporal lobe epilepsy
Authors: Briellmann R. S. , Ozdoba C , Donati F. , Gutbrod K. , Hess C. W. , Schroth G.
Published: January 2011

The purpose of this study was to determine whether the age at onset of temporal lobe epilepsy influences hippocampal volume. Fifteen right‐handed patients with medically intractable partial complex epilepsy of temporal origin were studied in quantified magnetic resonance imaging (MRI) and neuropsychological tests. In seven patients (mean age: 24 years) epilepsy began before the age of three (? YOUNG), in eight patients (mean age: 29 y) after the age of three (= OLDER). Education, social integration, duration of epilepsy and seizure frequency were equal in YOUNG and OLDER. The mean hippocampal volume, after normalization to total intracranial volume was for the right and for the left side smaller in YOUNG than in OLDER (p ≤ 0.02). The side‐to‐side asymmetry of absolute hippocampal volume was in patients with left‐side focus more pronounced in YOUNG than in OLDER (p < 0.03). The number of patients with right‐side focus was too small for statistical analysis. Results of neuropsychological testing showed no significant differences between YOUNG and OLDER. Onset‐age of epilepsy before three years is associated with a higher risk of bilateral hippocampal hypotrophy and unilateral hippocampal asymmetry. Performance in neuropsychological testing seems not to be significantly influenced by hippocampal volume or age at onset.
Unusual presentation and clinical variability in Belgian pedigrees with progressive external ophthalmoplegia and multiple deletions of mitochondrial DNA
Authors: Van Goethem G. , Martin J.‐J. , Löfgren A. , Dehaene I. , Tack P. , Van Zandycke M. , Ververken D. , Ceuterick C. , Van Broeckhoven C.
Published: January 2011

We studied 14 patients from three unrelated Belgian pedigrees with a familial mitochondrial disorder and multiple deletions of mitochondrial DNA (mtDNA). In one family with an oculopharyngeal presentation there is a clear autosomal dominant inheritance. Progressive external ophthalmoplegia (PEO), “ragged red fibres” (RRF) and multiple deletions of mtDNA are common to all three families. Therefore a diagnosis of autosomal dominant progressive ophthalmoplegia with multiple deletions of mtDNA (adPEO) was made in one family at least. Our data confirm the previous observations that adPEO is a systemic disorder rather than a pure myopathy. In our pedigrees frequently associated features include axonal peripheral neuropathy, dysphagia, psychiatric illness, and sudden death. Mild ataxia, pes cavus and mitral valve prolapse with associated mitral insufficiency also occur. In some cases onset is atypical with neuropathy, adolescent onset myopathy or psychiatric illness. In such cases the common features of PEO and muscle weakness always complete the clinical phenotype later during the course of the disease. Biochemical studies on mitochondrial fractions prepared from one patient's muscle, revealed no abnormalities of respiratory chain enzyme activities.
An acute and long‐term study with a dispersible formulation of levodopa/benserazide (Madopar®) in Parkinson's disease
Authors: Monge A. , Barbato L. , Nordera G. , Stocchi F.
Published: January 2011

The clinical efficacy, tolerability and administration regimens of a dispersible formulation of levodopa/benserazide (DM) were investigated in 30 patients with idiopathic Parkinson's disease, complicated by motor fluctuations. All 30 patients showed delayed‐ “on” phenomenon after administration of the first morning dose of standard levodopa (SM), and 20 showed delayed‐ “on” phenomenon after the first afternoon dose. Patients were receiving standard formulations of levodopa as monotherapy or in combination. A double‐dose study of the dispersible vs the standard formulation was performed in 30 patients, 24 of whom participated in a 36‐month, follow‐up clinical study. In the long‐term study, SM was replaced with DM by substituting the first morning dose or the first morning and first afternoon doses. In the double‐dose study, mean latency to “on” after the first morning dose was significantly shorter with DM than with SM (p < 0.001), whereas the duration of “on” was similar with the two preparations. The post‐prandial delayed‐ “on” in the 14 patients who responded to therapy was significantly shorter for DM than for SM (p < 0.001). In the long‐term study, the mean latency to “on” in all patients was significantly shorter than at baseline (p < 0.001). Time spent in “on” during the active day increased significantly, and remained stable during the 36‐month study. No changes were apparent in the mean dosage of levodopa/day or the number of doses/day, and no acute or long‐term adverse events were reported. In conclusion, these results confirm the long‐term safety of the dispersible formulation, and its improved efficacy compared with standard levodopa formulations, as monotherapy and in association with slow‐release formulations.
Nicardipine improves motor tics
Authors: Jiménez‐Jiménez F. J. , García‐Ruiz P.J. , de Yébenes J. Garciá , Vázquez A. , Urra D. García , Morales B. , de Seijas E. Varela
Published: January 2011

Calcium channel blockers can act on dopaminergic systems, and some reports suggest that they could be useful for the treatment of several movement disorders. In order to assess the efficacy of nicardipine in tics disorders we performed a prospective open non‐controlled study which included 10 previously untreated patients. Our results suggest that nicardipine could be a useful and safe treatment for tics.
The prevalence of migraine and tension headache in Saudi Arabia: a community‐based study
Authors: Rajeh S. Al , Awada A. , Bademosi O. , Ogunniyi A.
Published: January 2011

The prevalence of migraine and tension headache was determined in a 2‐stage, door‐to‐door community survey in Thugbah, Saudi Arabia. Out of 22630 subjects surveyed, 2742 individuals had headaches thus yielding a crude prevalence of 12.1% (95% CI = 11.7–12.5%) and with age‐adjustment, it rose to 15.9% (95% CI = 15.4–16.4%). There was female preponderance overall and the peak frequency was in the 3rd decade. The age‐specific rates rose from 2.4% in the first decade to 37.2% in the 7th decade. The prevalence of tension‐type headache (PR 9.5%; 95% CI = 9.1–9.9%) was higher than migraine (PR = 5.0%; 95% CI = 4.7‐5.3%). The low headache prevalence in this community compared to findings in western countries could be ascribed to the young age of the population and/or could possibly reflect the influence of traditional life styles and cultural factors in the Kingdom.
Amnestic episode caused by thrombosis of internal cerebral veins
Authors: Förderreuther S. , Straube A. , Yousry T. A.
Published: January 2011

We report on a patient who suffered an acute‐onset amnestic syndrome without focal neurological deficits and recovered completely within a fortnight. His symptoms were caused by a bilateral paramedian thalamic venous infarction due to a thrombosis of the internal cerebral veins, which was confirmed by cerebral angiography. Thrombosis of the internal cerebral veins should always be suspected in patients presenting with a longer‐lasting amnestic syndrome without any other neurological symptoms. Furthermore, the prognosis of internal cerebral vein thrombosis is not always poor.
Marked improvement of delayed methotrexate‐induced leukoencephalopathy treated with high‐dose folinic acid
Authors: Hasegawa T. , Abe K. , Sakuma R. , Itoyama Y.
Published: January 2011

We report the case of a patient with delayed methotrexate (MTX)‐induced leukoencephalopathy who showed a marked improvement both in clinical and neuroimaging findings after a high‐dose of the active form of folinic acid (leucovorin) treatment. The patient developed progressive affective impairment accompanied by headache, nausea and vomiting after treatment with MTX during the chemotherapy for acute lymphoblastic leukemia, and diagnosed as delayed type MTX‐induced leukoencephalopathy. After an intravenous injection of high‐dose folinic acid (total 1920 mg), neurological deficits and white matter changes dramatically improved in a few weeks. Although delayed MTX‐induced leukoencephalopathy may cause irreversible brain damage, an early treatment with high dose leucovorin may thus facilitate the marked improvement of clinical findings and white matter abnormalities.
Cheiro‐oral syndrome because of cerebral abscess
Authors: Chang G. Y.
Published: January 2011

Cheiro‐oral syndrome usually results from acute small vessel disease with expected favorable outcome. Simultaneous ipsilateral perioral and hand involvement may be not only due to anatomic proximity but also due to differential sensory threshold. A separate compensatory physiologic mechanism may explain the rarity of slower evolving lesions in producing the cheiro‐oral syndrome. Cerebral abscess, surgically treatable with subacute presentation, may produce an identical clinical syndrome.
A neurologist's view on boxing*
Authors: Tichý J.
Published: January 2011

The contemporary practice of boxing discredits the ethos of modern society. An activity, the goal of which is to render your opponent unconscious, or otherwise inflict damage, should not be a sport. Only activities that lead to maintaining a healthy spirit and body should be considered sports. Boxing, in spite of all suggested arrangements and improved medical control and care, is a risky activity that often results in serious health problems. This has already been pointed out by the Nestor of Czechoslovak neurology, Academician Henner, and other specialists in the field.
A review of the efficacy of the dopamine agonists pergolide and bromocriptine in the treatment of Parkinson's disease
Authors: Nohria V. , Partiot A.
Published: January 2011

This review summarizes those studies, completed over the last 15 years, which compare the clinical benefit of the two most commonly used dopamine agonists, pergolide and bromocriptine. In these studies, both drugs were evaluated as adjunctive therapy to levodopa for the treatment of Parkinson's disease (PD). Ten studies are analyzed and the affect of pergolide and bromocriptine on PD compared. Although variation in study design and disease rating scales prevents the opportunity for a true meta‐analysis, this review analyses the outcome of each individual study to assess the benefit of pergolide over bromocriptine. Pergolide improves patients' activities‐of‐daily‐living and their clinical PD symptoms over bromocriptine. Additionally, a large percentage of patients who do not respond to bromocriptine, or whose PD symptoms worsened, improved on pergolide. Furthermore, patients who are adequately treated with bromocriptine experienced additional improvement with pergolide therapy. In summary, pergolide provided benefits to PD patients over bromocriptine in nine studies and was equivalent in the tenth. The benefits associated with pergolide may be partly due to the action of pergolide on dopamine D1 and D2 receptors (bromocriptine is associated with the D2 agonism). In conclusion, in the majority of completed studies to date, pergolide provided greater improvement to the clinical signs and symptoms of PD than bromocriptine.
Multiple sclerosis and exposure to house pets during childhood and adolescence: a case—control study
Authors: De Keyser J. , Zwanikken C.
Published: January 2011

Multiple sclerosis (MS) might be triggered by an infectious agent early in life. In a case–control study, we compared exposure to household pets for the age periods 0–5, 6–10, 11–15 and 16–20 years in 100 patients with MS and 100 controls. Exposure to household dogs between the ages of 0–10 years was associated with an increased risk of MS [odds ratio 1.9 (95% CI 1.1–3.5)]; the association was strongest between the ages of 0–5 years [odds ratio 2.7 (1.4–5.0)]. Exposure to household dogs between the ages of 10–20 years was not significantly different between cases and controls. Risk of MS was not significantly associated with household exposure to cats, caged small birds or other pets. Our results suggest that exposure to household dogs in the first decade of life—in particular the first 5 years of life—is associated with an increase in the risk of MS.
Thrombotic thrombocytopenic purpura in the brain: possible prognostic impact of von Willebrand factor physiology
Authors: Vieregge P. , Neugebauer C. , Reusche E. , Hallermann W. , Budde U. , Heide W. , Otto V. , Mentzel M. , Hütteroth T.
Published: January 2011

We report three patients with CNS involvement in thrombotic thrombocytopenic purpura (TTP). The first two cases presented with fluctuating levels of consciousness and lateralized neurologic deficits. Repeated brain CT scans were normal in case 1, and showed small parietal lucencies in case 2. Both patients recovered to only minor residues after combined treatment of plasma exchange, corticosteroids, immunoglobulins, vincristine (case 1), and cyclophosphamide (case 2), respectively. Case 3 with multiple infarctions in brain CT died of heart failure. Autopsy showed myocardial infarction and multiple hemorrhages in various organs including cerebral cortex. Excess deposition of von Willebrand factor antigen within hyaline thrombi in small and medium‐sized arteries of the cerebral cortex is shown for the first time immunohistologically.
Isolated dementia in two patients with posterior intraventricular meningiomas
Authors: Isella V. , Appollonio I. , Piolti R. , Gaini S. M. , Frattola L.
Published: January 2011

Although patients with brain lesions of neurosurgical interest may sometimes present with a cognitive profile very similar to a primary dementia, they usually have a history and/or a neurologic examination which alert the clinician. The occurrence of mass lesions with purely isolated cognitive deterioration has been mainly reported for locations at the level of the frontal lobes. A potentially positive prognosis is advocated in these cases, in terms of even complete reversibility of the mental impairment when surgical removal is feasible and successful. We report two rare cases of posterior intraventricular meningiomas presenting with isolated cognitive and behavioural changes consistent with a frontal type dementia; the outcome was favourable in one patient, whereas the second one suffered from massive intracerebral bleeding during the neurosurgical removal and required prolonged hospitalization and subsequent intensive neurorehabilitative efforts. By comparing our cases with previously published decision‐rules regarding neuroimaging in dementia, we suggest that criteria should be enlarged, especially those regarding the age of the subject and the time elapsed from the first signs of the cognitive deterioration.
Acute encephalopathy and idiopathic hypereosinophilic syndrome: clinical and MRI response to early steroid treatment
Authors: Moreau Th. , Derex L. , Confavreux Ch.
Published: January 2011

A 31‐year‐old woman with hypereosinophilic syndrome developed left hemiplegia with confusion over a few hours. Brain magnetic resonance imaging (MRI) showed a diffuse meningeal gadolinium enhancement and periventricular nodular lesions. After 20 months of continuous steroid treatment, the patient is clinically asymptomatic and there were an obvious regression of MRI abnormalities. These findings indicate that an early steroid treatment is able to prevent irreversible damage in hypereosinophilic syndrome.
The Problem of Counterfactuals in Substituted Judgement Decision‐Making
Authors: WRIGLEY ANTHONY
Published: January 2011

abstract

The standard by which we apply decision‐making for those unable to do so for themselves is an important practical ethical issue with substantial implications for the treatment and welfare of such individuals. The approach to proxy or surrogate decision‐making based upon substituted judgement is often seen as the ideal standard to aim for but suffers from a need to provide a clear account of how to determine the validity of the proxy's judgements. Proponents have responded to this demand by providing the truth‐conditions for the substituted judgement in terms of counterfactual reasoning using a possible worlds semantics. In this paper, I show how these underpinnings fail to support the substituted judgement approach as a reasonable standard for decision‐making. Firstly, I show how this counterfactual element has been poorly interpreted. I then explain how various accounts have failed to reflect problems and limitations associated with providing an interpretation of their truth‐conditions using counterfactuals. Finally, I argue that, even when we attend to the initial problems of providing a counterfactual analysis, it still deeply problematic as a means of determining the validity of substituted judgements for two main reasons. Firstly, making determinate judgements as to the truth‐value of these judgements will often not be possible and, secondly, there is a strong requirement when interpreting many counterfactual claims to charitably accede to their being true. I conclude that substituted judgements, as interpreted through counterfactual reasoning and possible worlds semantics, do not therefore provide an adequate standard for surrogate decision‐making.
Preparing for Financial Surprise
Authors: Power Michael
Published: January 2011

This essay shows how Todd LaPorte's work on reliability‐seeking organizations contains insights for how we might understand the financial crisis of 2008–2009. In particular, the financial system was not perceived and described as critical infrastructure, there was a regulatory overemphasis on routine operational practices at the entity level, and there was insufficient countercyclical leadership. These and other of LaPorte's ideas bridge the divide between the two ‘cultures’ of financial and non‐financial risk management.
Organizing for Transient Reliability: The Production of Dynamic Non‐Events
Authors: Weick Karl E.
Published: January 2011

Reliability is portrayed as a situation‐specific localized accomplishment that involves continuous management of significant contradictions that are susceptible to interruption. Managing for higher reliability is precarious because it involves the substitution of a conceptual order for a perceptual order. Discrete concepts simplify and lag behind continuous perceptions, which means that our grasp of events is subject to mis‐specification, misidentification, and misunderstanding. The gap between discrete concepts and continuous perceptions can be reduced if attention is organized so that it is directed at failures, simplifications, operations, resilience, and expertise. These micro‐dynamics are discussed as a partial infrastructure that underlies Todd LaPorte's more macro‐concerns with strategy and structure in the quest for failure‐free interorganizational performance.
Impact of processing steps on the composition of volatile compounds in cheese powders
Authors: VARMING CAMILLA , BECK TOVE KJÆR , PETERSEN MIKAEL AGERLIN , ARDÖ YLVA
Published: January 2011

Changes in the composition of volatile compounds during processing of cheese powders, made from Emmental and Danbo cheese were analysed by dynamic headspace gas chromatography–mass spectrometry. Samples comprised cheese, melted cheese slurry, the slurry heat treated using three different time and temperature combinations, and the spray‐dried cheese powder. About 100 volatile compounds were identified. During melting of the cheese the level of some volatile compounds increased significantly; heat treatment mainly leads to a loss of compounds and spray drying leads to a substantial reduction of volatile compounds, with the exception of a range of aldehydes that increased. The final cheese powders could, however, be distinguished according to cheese type.