JNS-cover.jpgJournal of Neurological Sciences

Vol 351 | No. 1-2 | 15 April 2015 | Pages 1-216


Elderly and forgetful with transient neurological spells: A story of two amyloids?

Andreas Charidimou

Cerebrovascular amyloid-β deposition within small vessels (i.e. sporadic cerebral amyloid angiopathy—CAA) is perhaps one of the most common neuropathological traits in the aging brain [1,2]. In population-based autopsy studies it is found in 20–40% of non-demented and 50–60% of demented older individuals [3]. CAA also seems to form an integral part in Alzheimer's disease (AD), being present in nearly all brains with AD-type pathology [4] and probably suggesting a close molecular relationship between vascular and parenchymal amyloid-β deposits.



Neurobiological studies on the relationship between toxoplasmosis and neuropsychiatric diseases

Silvia Fabiani, Barbara Pinto, Ugo Bonuccelli, Fabrizio Bruschi

Toxoplasma gondii is a widespread protozoan parasite infecting approximately one third of the world population. After proliferation of tachyzoites during the acute stage, the parasite forms tissue cysts in various anatomical sites including the Central Nervous tissue, and establishes a chronic infection. Clinical spectrum normally ranges from a completely asymptomatic infection to severe multi-organ involvement. Many studies have suggested T. gondii infection as a risk factor for the development of some neuropsychiatric disorders, particularly schizophrenia.

L-DOPA-induced dyskinesia, is striatal dopamine depletion a requisite?

Philippe Huot

l-3,4-Dihydroxyphenylalanine (L-DOPA) is currently the most effective drug available to treat the symptoms of Parkinson's disease (PD). A limitation is that chronic administration of L-DOPA almost invariably, but not universally, leads to the development of abnormal involuntary movements, dyskinesia. Research suggests that striatal dopamine depletion is an important aetiological factor leading to dyskinesia development. However, in studies where L-DOPA was administered to normal animals and human subjects not afflicted by PD, abnormal involuntary movements were sometimes elicited.

Statin induced necrotizing autoimmune myopathy

Suma Babu, Yuebing Li

Statin induced necrotizing autoimmune myopathy (SINAM) is a recently characterized entity belonging to the spectrum of statin myotoxicity. It is a more severe form, and is usually associated with significant proximal muscle weakness, strikingly elevated creatine kinase levels and persistent symptoms despite statin discontinuation. The characteristic pathological finding is a marked muscle fiber necrosis with minimal or no inflammation on muscle biopsy. SINAM is an autoimmune disorder associated with an antibody against 3-hydroxy-3-methyglutaryl-coenzyme A reductase (HMGCR), and the antibody titer is a useful marker for assessing treatment response.


Original Articles

The metabolic syndrome in a memory clinic population: Relation with clinical profile and prognosis

Lieza G. Exalto, Wiesje M. van der Flier, Caroline J.M. van Boheemen, L. Jaap Kappelle, Hugo Vrenken, Charlotte Teunissen, Ted Koene, Phillip Scheltens, Geert Jan Biessels

The metabolic syndrome (MetS) refers to a cluster of cardiovascular risk factors that is associated with an increased risk of cognitive impairment and dementia. It is unclear however, if the presence of the MetS is associated with a particular clinical profile or a different prognosis in patients with cognitive complaints or early dementia.

Simultaneous assessment of cognitive and affective functions in multiple system atrophy and cortical cerebellar atrophy in relation to computerized touch-panel screening tests

Yuko Kawahara, Yoshio Ikeda, Kentaro Deguchi, Tomoko Kurata, Nozomi Hishikawa, Kota Sato, Syoichiro Kono, Taijun Yunoki, Yoshio Omote, Toru Yamashita, Koji Abe

Cognitive impairment and affective dysfunction of multiple system atrophy (MSA) and cortical cerebellar atrophy (CCA) have not been simultaneously examined comparing standard test batteries and a sensitive tool to detect subtle cognitive decline in patients. In the present study, we simultaneously examined cognitive and affective ability in MSA with predominant cerebellar ataxia (MSA-C, n = 25), MSA with predominant parkinsonism (MSA-P, n = 8), and CCA (n = 14) patients using computerized touch panel screening tests.

Analysis of the treatment of neuromyelitis optica

Jose Torres, Amy Pruitt, Laura Balcer, Steven Galetta, Clyde Markowitz, Nabila Dahodwala

Treatment options for neuromyelitis optica (NMO) are currently based on small retrospective case series and open label studies, ranging from 10 to 103 patients.

1,2-Dichloroethane-induced toxic encephalopathy: A case series with morphological investigations

Shiyan Chen, Zhijian Zhang, Hong Lin, Zixuan Chen, Zhiqiang Wang, Wei Wang

1,2-Dichloroethane (DCE) is commonly used as an industrial organic solvent and causes occupational diseases. Toxic encephalopathy is the most common and serious disorder resulting from DCE intoxication. Five patients who worked in shoemaking, pipemaking or a paint factory were identified as suffering from severe encephalopathy due to DCE intoxication. DCE-induced toxic encephalopathy manifests as various neurological deficits, with changes observable by neuroimaging. The main clinical manifestation is headache accompanied by intracranial hypertension.

Heterogeneity among patients with Parkinson's disease: Cluster analysis and genetic association

Ling-Yan Ma, Piu Chan, Zhu-Qin Gu, Fang-Fei Li, Tao Feng

The clinical heterogeneity of Parkinson's disease (PD) reveals the presence of several PD subtypes. The objectives of this study were to identify PD subtypes using cluster analysis (CA) and to determine the association between the subtypes and the polymorphisms in LRRK2 (G2385R and R1628P) and GBA (L444P) genes. A k-means CA of demographics, disease progression, motor and non-motor symptoms was performed from 1,510 Chinese PD patients from the Chinese National Consortium on Neurodegenerative Diseases.          

Central amygdalar nucleus treated with orexin neuropeptides evoke differing feeding and grooming responses in the hamster

Raffaella Alò, Ennio Avolio, Maria Mele, Anna Di Vito, Marcello Canonaco

Interaction of the orexinergic (ORXergic) neuronal system with the excitatory (glutamate, l-Glu) or the inhibitory (GABA) neurosignaling complexes evokes major homeostatic physiological events. In this study, effects of the two ORXergic neuropeptides (ORX-A/B) on their receptor (ORX-2R) expression changes were correlated to feeding and grooming actions of the hibernating hamster (Mesocricetus auratus). Infusion of the central amygdala nucleus (CeA) with ORX-A caused hamsters to consume notable quantities of food, while ORX-B accounted for a moderate increase.    

Fatigue in patients with multiple sclerosis: From movement preparation to motor execution

Margherita Russo, Domenica Crupi, Antonino Naro, Laura Avanzino, Maria Buccafusca, Vincenzo Dattola, Carmen Terranova, Fabrizio Sottile, Vincenzo Rizzo, Maria Felice Ghilardi, Paolo Girlanda, Marco Bove, Angelo Quartarone

The neural mechanisms underlying fatigue in multiple sclerosis (MS) are still poorly understood. Cortico-cortical and cortico-subcortical circuitry abnormalities may play a central role in its pathogenesis. Our previous studies suggest that central fatigue may be related to an impairment of volition drive during movement preparation.

The King–Devick test was useful in management of concussion in amateur rugby union and rugby league in New Zealand

D. King, C. Gissane, P.A. Hume, M. Flaws

To use the King–Devick (K–D) test in senior amateur rugby union and rugby league players over a domestic competition season to see if it could identify witnessed and unwitnessed episodes of concussion that occurred from participation in competition matches over three years.

Diagnostic accuracy of diffusion MRI with quantitative ADC measurements in differentiating glioma recurrence from radiation necrosis

Hui Zhang, Li Ma, Cheng Shu, Yu-bo Wang, Lian-qiang Dong

Differentiating radiation necrosis from glioma recurrence remains a great challenge. Several advanced imaging modalities have been developed to differentiate between these two entities with disparate outcomes. We conducted a meta-analysis to evaluate the diagnostic quality of diffusion MRI in differentiating glioma recurrence from radiation necrosis.

Motor neuron dysfunctions in the frontotemporal lobar degeneration spectrum: A clinical and neurophysiological study

C. Cerami, A. Marcone, C. Crespi, S. Iannaccone, C. Marangoni, A. Dodich, M.C. Giusti, M. Zamboni, V. Golzi, S.F. Cappa

Although only a few frontotemporal lobar degeneration (FTLD) patients develop frank amyotrophic lateral sclerosis (ALS), motor neuron dysfunctions (MNDys) occur in a larger proportion of patients. The aim of this study is to evaluate MNDys and ALS in a sample of consecutively enrolled sporadic FTLD patients.

Anti-apoptotic, anti-oxidant, and anti-inflammatory effects of thalidomide on cerebral ischemia/reperfusion injury in rats

Guadalupe Palencia, Juan Ángel Núñez- Medrano, Alma Ortiz-Plata, Dolores Jiménez Farfán, Julio Sotelo, Aurora Sánchez, Cristina Trejo-Solís

Thalidomide has shown protective effects in different models of ischemia/reperfusion damage. To elucidate the mechanisms of such protection, this study assessed the effects of thalidomide on the oxidative stress and inflammatory response induced by ischemia/reperfusion episodes in rats. Rats underwent middle cerebral artery occlusion (MCAO) for 2 hours. All animals were sacrificed after different reperfusion times. Rats were administered either DMSO or thalidomide (20 mg/kg (i.p.)) at different times before or during reperfusion: 1) 1 h before reperfusion; the infarct area was measured 2 h after reperfusion.           

Activation of the Nrf2 defense pathway contributes to neuroprotective effects of phloretin on oxidative stress injury after cerebral ischemia/reperfusion in rats

Yu Liu, Lei Zhang, Jiangjiu Liang

Oxidative stress is considered a major contributing factor in cerebral ischemia/reperfusion injury. Phloretin, a dihydrochalcone belonging to the flavonoid family, is particularly rich in apples and apple-derived products. A large body of evidence demonstrates that phloretin exhibits anti-oxidant properties, and phloretin has potential implications for treating oxidative stress injuries in cerebral ischemia/reperfusion. Therefore, the neuroprotective and antioxidant effects of phloretin against ischemia/reperfusion injury, as well as related probable mechanisms, were investigated.  

Increased interhemispheric resting-state functional connectivity in paroxysmal kinesigenic dyskinesia: A resting-state fMRI study

Jiechuan Ren, Du Lei, Tianhua Yang, Dongmei An, Fenglai Xiao, Lei Li, Xiaoqi Huang, Qiyong Gong, Dong Zhou

Paroxysmal kinesigenic dyskinesia (PKD) is a rare movement disorder. The underlying neural mechanisms have not been fully understood. This study aimed to examine the alteration of resting-state functional connectivity (RSFC) between interhemispheric homotopic regions in PKD using a technique called "voxel-mirrored homotopic connectivity" (VMHC).

Analysis of opa1 isoforms expression and apoptosis regulation in autosomal dominant optic atrophy (ADOA) patients with mutations in the opa1 gene

Patrizia Formichi, Elena Radi, Eleonora Giorgi, Gian Nicola Gallus, Jlenia Brunetti, Carla Battisti, Alessandra Rufa, Maria Teresa Dotti, Rossella Franceschini, Luisa Bracci, Antonio Federico

Autosomal dominant optic atrophy (ADOA) is a hereditary optic neuropathy characterized by bilateral symmetrical visual loss, decrease in retinal ganglion cells and a loss of myelin within the optic nerve. ADOA is associated to mutations in Optic atrophy 1 gene (OPA1), which encodes a mitochondrial protein involved in cristae remodeling, maintenance of mitochondrial membrane integrity, mitochondrial fusion and apoptosis regulation. We thus evaluated the rate of apoptosis and the expression levels of OPA1 isoforms in ADOA and control cells.

Early fibrinogen degradation coagulopathy: A predictive factor of parenchymal hematomas in cerebral rt-PA thrombolysis

Xuhong Sun, Julien Berthiller, Paul Trouillas, Laurent Derex, Laho Diallo, Michel Hanss

The purpose of this study was to systematically determine the correlations between the post-thrombolytic changes of hemostasis parameters and the occurrence of early intracerebral hemorrhage (ICH).

Trajectories of cognitive function in dementia-free subjects: Radiation Effects Research Foundation Adult Health Study

Michiko Yamada, Reid D. Landes, Yasuyo Mimori, Yoshito Nagano, Hideo Sasaki

To investigate associations between age, sex, education, and birth cohort and global cognitive decline among a population that would most likely not progress to dementia.

Adult onset limb-girdle muscular dystrophy — A recessive titinopathy masquerading as myositis

Ron Dabby, Menachem Sadeh, David Hilton-Jones, Paul Plotz, Peter Hackman, Anna Vihola, Bjarne Udd, Esther Leshinsky-Silver

Rarely, inflammation can be present in genetic myopathies, such as dysferlinopathies, facioscapulohumeral muscular dystrophy and GNE-myopathy (hereditary inclusion body myopathy). This may lead to erroneous initial diagnosis and unnecessary therapy which bear serious side effects. We report on an unusual case of mutations in the TTN gene presenting with inflammatory infiltrates in the muscle biopsy. Only after intensive immune-modulating therapies failed, a genetic myopathy was considered. Exome sequencing and search for mutated muscle protein-encoding genes disclosed compound heterozygous mutations in TTN: K26320T and A6135G.

Friedreich's Ataxia (FRDA) is an extremely rare cause of autosomal recessive ataxia in Chinese Han population

Junsheng Zeng, Junling Wang, Sheng Zeng, Miao He, Xianfeng Zeng, Yao Zhou, Zhen Liu, Hong Jiang, Beisha Tang

Friedreich's Ataxia (FRDA) is a very common cause of hereditary autosomal recessive ataxia among western Europeans. We aim to define the frequency of FRDA in Chinese Han population due to the lack of reports of FRDA in China. The GAA trinucleotide repeats in the FXN gene were analyzed by triplet repeat-primed PCR (TP-PCR) in 122 unrelated hereditary ataxia (HA) and 114 unrelated hereditary spastic paraplegia (HSP) patients. The GAA copy numbers in the FXN gene of all the subjects ranged from 5 to 16.

Dopaminergic denervation is not necessary to induce gait disorders in atypical parkinsonian syndrome

Gilles Allali, Valentina Garibotto, Ismini C. Mainta, Stephane Armand, Richard Camicioli, Osman Ratib, Habib Zaidi, Francois R. Herrmann, Frederic Assal

Gait impairment is common in parkinsonian syndromes but not specific to striatonigral dysfunction. The relationship between the dopaminergic system and gait parameters is poorly understood. This cross-sectional study aimed to determine if gait measures are related to the striatal dopamine transporters distribution using [123I]FP-CIT SPECT in patients with parkinsonian syndromes.

Altered intrinsic brain activity in patients with familial cortical myoclonic tremor and epilepsy: An amplitude of low-frequency fluctuation study

Pu Wang, Cheng Luo, Li Dong, Yi Bin, Shi Ma, Dezhong Yao, Fuqiang Guo, Zhenglin Yang

To investigate localized cerebral function abnormalities in patients with familial cortical myoclonic tremor and epilepsy (FCMTE) using resting-state functional magnetic resonance imaging (fMRI).

Congenital mirror movements: Phenotypes associated with DCC and RAD51 mutations

Elizabeth A. Franz, Rachel Chiaroni-Clarke, Stephanie Woodrow, Kelly A. Glendining, Christine L. Jasoni, Stephen P. Robertson, R.J. McKinlay Gardner, David Markie

Congenital mirror movements (CMM) is a disorder characterized by unintentional mirroring in homologous motor systems of voluntary movements on the opposite side, usually affecting the distal upper extremities. Genetic analyses have revealed involvement of three genes (DCC, RAD51, and DNAL4). We sought to distinguish whether different phenotypes of CMM exist, and if so, whether they might map to different causative genes. We studied 14 individuals across five families with dominantly-inherited CMM.

Ischemic preconditioning inhibits expression of Na+/H+ exchanger 1 (NHE1) in the gerbil hippocampal CA1 region after transient forebrain ischemia

Jae-Chul Lee, Jeong-Hwi Cho, In Hye Kim, Ji Hyeon Ahn, Joon Ha Park, Geum-Sil Cho, Bai Hui Chen, Bich Na Shin, Hyun-Jin Tae, Seung Min Park, Ji Yun Ahn, Dong Won Kim, Jun Hwi Cho, Eun Joo Bae, Jun-Hwan Yong, Young-Myeong Kim, Moo-Ho Won, Yun Lyul Lee

The participation of Na+/H+ exchanger (NHE) in neuronal damage/death in the hippocampal CA1 region (CA1) induced by transient forebrain ischemia has not been well established, although acidosis may be involved in neuronal damage/death. In the present study, we examined the effect of ischemic preconditioning (IPC) on NHE1 immunoreactivity following a 5 min of transient forebrain ischemia in gerbils. The animals used in the study were randomly assigned to four groups (sham-operated-group, ischemia-operated-group, IPC plus (+) sham-operated-group and IPC + ischemia-operated-group).          

Two functional promoter polymorphisms of neuregulin 1 gene are associated with progressive forms of multiple sclerosis

Zahra Bahadori, Mehrdad Behmanesh, Mohammad Ali Sahraian

Multiple sclerosis (MS) is an inflammatory, autoimmune, demyelinating disease of the central nervous system (CNS). Spontaneous remyelination happens in most of MS lesions but it is incomplete and inadequate and eventually fails in majority of lesions of damaged areas. According to the fact that the accuracy of this phenomenon depends on distinct temporal expression profiles of molecules, especially growth factors, we decided to study the association of two functional polymorphisms of NRG1 as a myelin-related growth factor which can promote oligodendrocyte proliferation and differentiation in CNS and PNS.

Analysis of differentially expressed lncRNAs in differentiation of bone marrow stem cells into neural cells

Ai-Min Wu, Wen-Fei Ni, Zhe-Yu Huang, Qing-Long Li, Jian-Bo Wu, Hua-Zi Xu, Li-Hui Yin

Many studies have reported micro RNAs involved in the differentiation of bone marrow mesenchymal stem cells (BMSCs) into neural cells; however, the roles of long non-coding RNAs (lncRNAs) in the differentiation of BMSCs into neural cells remain poorly understood. We used microarray assays to compare the lncRNA and messenger RNA (mRNA) expression profiles in BMSCs and neural-induced BMSCs. We found a total of 24 lncRNAs and 738 mRNAs that were upregulated and 32 lncRNAs and 682 mRNAs that were downregulated in samples induced for 3 h; 27 lncRNAs and 864 mRNAs that were upregulated and 37 lncRNAs and 968 mRNAs that were downregulated in 6 h samples; and 23 lncRNAs and 1159 mRNAs that were upregulated or downregulated in both the 3 h and 6 h samples.


Short Communications

High-resolution MRI vessel wall imaging in varicella zoster virus vasculopathy

Esteban Cheng-Ching, Stephen Jones, Ferdinand K. Hui, Shumei Man, Don Gilden, Adarsh Bhimraj, Ken Uchino

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.02.017
  • p168–173
  • Published online: February 16, 2015
  • Abstract
  • Full-Text HTML
  • PDF

Contrast-enhanced vessel wall imaging high-resolution MRI (HRMR) has revealed vessel wall thickening and enhancement in multiple intracranial vasculopathies, including varicella zoster virus (VZV) vasculopathy. We retrospectively reviewed a database of patients with virologically-verified VZV vasculopathy, who underwent initial and follow-up HRMR between April 2011 and May 2014. Six patients were identified. Baseline demographic and clinical characteristics were collected, including stroke risk factors, history of VZV-related disorders, neurological presentation, course and antiviral treatment.

ATON: Results from a Phase II randomized trial of the B-cell-targeting agent atacicept in patients with optic neuritis

Robert C. Sergott, Jeffrey L. Bennett, Peter Rieckmann, Xavier Montalban, Daniel Mikol, Ulrich Freudensprung, Thomas Plitz, Johan van Beek, for the ATON Trial Group

The 36-week ATON study compared the efficacy and safety of atacicept with matching placebo in 34 patients with unilateral optic neuritis as a clinically isolated syndrome. Atacicept (150 mg) was administered twice weekly for 4 weeks (loading period), then once weekly for 32 weeks. The ATON study was terminated prematurely by the sponsor when an independent Data and Safety Monitoring Board review observed increased multiple sclerosis (MS)-related disease activity in the atacicept arms of the concurrent ATAcicept in MS (ATAMS) study.    

Is pushing the wall, the best known method for scapular winging, really the best? A Comparative analysis of various methods in neuromuscular disorders

Satish V. Khadilkar, Chetan R. Chaudhari, Girish Soni, Ashish Bhutada

'Pushing the wall' has found acceptance in medical teachings. Other methods of scapular winging are less known. Comparative evaluation of the five available methods has not been undertaken. This study focuses on evaluation of the available methods in groups of neuromuscular disorders to select the most sensitive method and to characterize patterns of scapular winging. A survey of methods practiced by clinicians also forms a part of the study.

Prevalence of autoimmune disease in moyamoya disease patients in Western Chinese population

Jian-bin Chen, Yi Liu, Liang-xue Zhou, Hong Sun, Min He, Chao You

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.02.037
  • p184–186
  • Published online: February 26, 2015
  • Abstract
  • Full-Text HTML
  • PDF

The pathogenesis of moyamoya remains to be elucidated and an immunologic basis has been suggested. For gaining further insight into the pathogenesis of moyamoya, we explored the epidemiological characteristics of autoimmune disease in moyamoya disease (MMD) in Western Chinese population. Retrospective clinical characteristic analysis of patients with angiographically confirmed MMD was performed and compared with the general Chinese population. A significantly higher prevalence of autoimmune disease was observed, particularly type 1 diabetes mellitus (P < 0.001, 7.0% vs 1.2%, χ2 test) and Graves disease (P < 0.001, 7.0% vs 0.34%, χ2 test) in the general Chinese population.

Cauda equina conduction time in Guillain-Barré syndrome

Hideyuki Matsumoto, Ritsuko Hanajima, Yasuo Terao, Hideji Hashida, Yoshikazu Ugawa

The proximal segment of peripheral nerves is assumed to be involved in both demyelinating and axonal types of Guillain-Barré syndrome (GBS). However, electrophysiological examinations have not yet clarified if this segment is involved. We measured cauda equina conduction time (CECT) in nine demyelinating GBS and seven axonal GBS patients. Compound muscle action potentials (CMAPs) were recorded from the abductor hallucis muscle. Electrical stimulation was given at the ankle and the knee, and magnetic stimulation was given over the first sacral (S1) and first lumbar (L1) spinous processes using a magnetic augmented translumbosacral stimulation (MATS) coil.

Isolated unilateral oculomotor paresis in pure midbrain stroke

Yu Amano, Yosuke Kudo, Hideyuki Kikyo, Ryoko Imazeki, Masahiro Yamamoto, Kazumitsu Amari, Fumiaki Tanaka, Ken Johkura

Pure midbrain stroke can cause isolated unilateral oculomotor paresis. We attempted to determine whether there is a difference in the oculomotor paresis pattern between pure midbrain infarction and midbrain hemorrhage.


Letters to the Editor

Letter to the Editor on a paper by Hsiao C-T, Tsai P-C, Liao Y-C, Lee Y-C, Soong B-W. C9ORF72 repeat expansion is not a significant cause of late-onset cerebellar ataxia syndrome. J Neurol Sci 2014;347:322–324.

Morgane Plutino, Annabelle Chaussenot, Samira Ait-El-Mkadem, Sylvie Bannwarth, Emmanuelle C. Genin, Cécile Rouzier, Gaëlle Augé, Sabrina Sacconi, Jean Pouget, Véronique Paquis-Flucklinger

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.02.025
  • p196–197
  • Published online: February 20, 2015
  • Abstract
  • Full-Text HTML
  • PDF

A GGGGCC hexanucleotide repeat expansion in the C9ORF72 gene has been initially demonstrated to be a common cause of frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) [1,2]. C9ORF72 repeat expansion has also been associated with parkinsonian syndrome, Huntington's disease-like syndrome and dementia syndrome. Several individuals presenting with cerebellar ataxia have also been reported to carry the C9ORF72 expansion mutation. In order to elucidate the relationship between the C9ORF72 mutation and cerebellar ataxia, Hsiao and colleagues screened a Chinese cohort of 429 patients with idiopathic late-onset cerebellar ataxia [3].

Cerebral amyloid angiopathy-associated microbleed mimicking transient ischemic attack

Apostolos Safouris, Marie-Dominique Gazagnes, Nikos Triantafyllou, Georgios Tsivgoulis

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.02.032
  • p198–199
  • Published online: February 26, 2015
  • Abstract
  • Full-Text HTML
  • PDF

A 72-year-old man diagnosed with probable Alzheimer's disease (AD) presented with acute onset motor aphasia. He has been treated for dyslipidemia with statins and he was on aspirin due to a transient ischemic attack (TIA) that presented with motor aphasia a year ago. At that time the patient had a brain MRI scan that showed no acute ischemic lesions but lobar microbleeds on the left hemisphere; the patient also had an extensive work-up with carotid duplex, transcranial Doppler, transthoracic and transesophageal echocardiography and 24-h Holter ECG that were normal.     

Invasive procedures in Parkinson's disease: Let's be aware we are dealing with prion-like proteins

Maria Graciela Cersosimo

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.02.033
  • p200–201
  • Published online: February 25, 2015
  • Abstract
  • Full-Text HTML
  • PDF

Relevant laboratory observations support the hypothesis that misfolded alpha-synuclein (α-SYN) protein is capable of spread from cell to cell within the central nervous system (CNS). Embryonic tissue grafted into the brain of Parkinson's disease (PD) patients showed the presence of α-SYN pathology more than ten years later in postmortem examination [1,2]. In a recent study, the inoculation of misfolded α-SYN obtained from substantia nigra of post-mortem PD cases into the brain of unaffected animals was followed by the spread of the abnormal protein aggregates to distant interconnected brain regions [3].

Autophagic activation may be involved in the mechanism of hesperidin's therapeutic effects on cognitive impairment

Bin Zhu, Chun Yang, Jing Wu, Fei Hua

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.02.036
  • p202–203
  • Published online: February 26, 2015
  • Abstract
  • Full-Text HTML
  • PDF

Recently, we read with great interest the article titled "Effect of hesperidin on neurobehavioral, neuroinflammation, oxidative stress and lipid alteration in intracerebroventricular streptozotocin induced cognitive impairment in mice" by Hayate et al. [1], who concluded that the mechanisms of hesperidin's therapeutic effects on cognitive impairment are probably associated with the modulation of acetylcholine esterase activity (AChE), thiobarbituric acid reactive substances and the inflammatory response.

Methylphenidate prolongs symptom-free period of experimental prion disease in mice

Israel Nissan, Inbar Gahali-Sass, Alexander Rouvinski, Kirill Makedonski, Albert Taraboulos, Yuval Tal

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.02.040
  • p204–205
  • Published online: February 28, 2015
  • Abstract
  • Full-Text HTML
  • PDF

We wish to report an observation regarding the effects of Ritalin™ (methylphenidate, MP) on the course of prion disease in mice. Prions [1] propagate by refolding host protein PrPC into PrPSc, a process occurring on lipid 'rafts'. We studied MP because it reduces serum cholesterol [2] and elevates serum dehydroepiandrosterone (DHEA) [3], and as we have found that DHEA reduces PrPSc in prion-infected neuro2A cells (in preparation). (See Fig. 1.)

Orienting network dysfunction in progressive multiple sclerosis

Samar S. Ayache, Ulrich Palm, Moussa A. Chalah, Rémy Nguyen, Wassim H. Farhat, Alain Créange, Jean-Pascal Lefaucheur

Among patients with multiple sclerosis (MS), cognitive impairment affects up to 70% of the population [1]. The most frequently impaired domains are memory, processing speed and attention. Three main attentional networks were described [2]: the alerting network responsible for controlling vigilance and performance during tasks, the orienting network in charge of orientation to external stimuli, and the executive control network that deals with solving conflicts, detecting targets, and focal attention.

Hepatitis-E virus associated neuralgic amyotrophy with sustained plexus brachialis swelling visualized by high-resolution ultrasound

Bernhard F. Décard, Alexander Grimm, Michaela Andelova, Anna Deman, Bettina Banderet, Meritxell Garcia, Peter Fuhr

Neuralgic amyotrophy (NA) also known as Parsonage–Turner syndrome is an acute brachial plexus disorder characterized by acute onset of neuropathic pain followed by multifocal paresis in the affected limb. Although the exact pathophysiological mechanisms of NA remain unclear, autoimmunological processes triggered by preceding infections, trauma or operations seem to play an important role especially in genetically susceptible patients [1].

Megalencephalic leukoencephalopathy with subcortical cysts caused by compound heterozygous mutations in MLC1, in patients with and without subcortical cysts in the brain

Teruaki Masuda, Mitsuharu Ueda, Hidetsugu Ueyama, Shino Shimada, Masatoshi Ishizaki, Shigehiro Imamura, Toshiyuki Yamamoto, Yukio Ando

Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare hereditary disorder characterized by infantile-onset macrocephaly and a gradual onset of progressive neurological symptoms including ataxia, spasticity, and mild mental decline [1]. As the name of the disorder suggests, patients with MLC invariably had subcortical cysts in the anterior temporal region of the brain [1].

Mononeuritis multiplex associated with primary livedoid vasculopathy: Neuropathological evidence of ischemic nerve damage

Maria C. Malaguti, Tiziana Cavallaro, Luigi Speziali, Maria G. Zorzi, Sabrina Marangoni, Alberto Morini

Mononeuritis multiplex (MM) is a pattern of peripheral neuropathy characterized by multifocal damage to at least two different nerves. It is associated with several distinct systemic disorders such as diabetes, connective tissue disease, vasculitis, amyloidosis, cryoglobulinemia, sarcoidosis, neoplasm and infections. Nerve biopsy is in most cases useful in demonstrating the aetiology of nerve damage and establishing a causal link with the underlying systemic disorder. Conversely, in some forms of MM the pathophysiology is controversial, especially in those cases associated with uncommon types of organ-specific diseases.