JNS-cover.jpgJournal of Neurological Sciences

Vol 361 | 15 February 2016 | Pages 1-276


Editor's update and selected articles from the Journal of the Neurological Sciences

John D. England

The Journal of the Neurological Sciences will soon begin featuring a section devoted to Global Neurology. The Editorial Board and I have seen a significant increase in the number of high quality submissions from around the world, and many of these address important issues in regions beyond the traditional high income countries. In this new section, we seek to profile original research, topical reviews, and commentaries which address important regional and global neurological topics. We extend a special invitation to individuals who are working in or collaborating with neurologists or scientists in lower or middle income countries.


Review Articles

Sphingosine 1-phosphate signaling in astrocytes: Implications for progressive multiple sclerosis

Mauricio F. Farez, Jorge Correale

Multiple sclerosis is an autoimmune disorder characterized by recurrent attacks against the central nervous system. After many years, certain patients enter a progressive disease phase, characterized by steady clinical deterioration. However, in 10–15% of patients, the disease is progressive from the beginning, and thus diagnosed as Primary Progressive Multiple Sclerosis. Unlike relapsing-remitting forms, progressive MS lacks effective therapy. Astrocytes are a major component of glial cells and are now thought to play a role in disease progression.

Ocular motor assessment in concussion: Current status and future directions

Rachel E. Ventura, Laura J. Balcer, Steven L. Galetta, Janet C. Rucker

Mild head injury such as concussions and subconcussive repetitive impact may lead to subtle changes in brain function and it is imperative to find sensitive and reliable tests to detect such changes. Tests involving the visual system, in particular eye movements, can incorporate higher cortical functioning and involve diffuse pathways in the brain, including many areas susceptible to head impact. With concussions, the clinical neuro-ophthalmic exam is important for detecting abnormalities in vergence, saccades, pursuit, and visual fixation.

Short-term suboptimal response criteria for predicting long-term non-response to first-line disease modifying therapies in multiple sclerosis: A systematic review and meta-analysis

Jordi Río, Juan Luís Ruiz-Peña

There is no consensus about short-term suboptimal response to first-line treatments in relapsing-remitting multiple sclerosis.

Epstein–Barr virus and multiple sclerosis. From evidence to therapeutic strategies

Santiago Fernández-Menéndez, Marta Fernández-Morán, Iván Fernández-Vega, Angel Pérez-Álvarez, Javier Villafani-Echazú

Multiple sclerosis is caused by a complex interaction between genetic predisposition and environmental factors. Epstein–Barr virus (EBV) is an environmental risk factor that is strongly related to multiple sclerosis (MS), since EBV seropositivity is linked to a significant risk of developing MS. EBV may be involved in the pathogenesis of the disease and it is possibly a prerequisite for the development of MS. EBV infection persists in B-cells during the lifetime of the host and can modulate their function.

Laryngeal stridor in multiple system atrophy: Clinicopathological features and causal hypotheses

Tetsutaro Ozawa, Kanako Sekiya, Naotaka Aizawa, Kenshi Terajima, Masatoyo Nishizawa

Laryngeal stridor is recognized as a characteristic clinical manifestation in patients with multiple system atrophy (MSA). However, the pathogenic mechanisms underlying this symptom are controversial. Neurogenic atrophy of the posterior cricoarytenoid muscle has been identified in cases of MSA, suggesting that laryngeal abductor weakness contributes to laryngeal stridor. However, dystonia in the laryngeal adductor muscles has also been reported to cause laryngeal stridor. Depletion of serotonergic neurons in the medullary raphe nuclei, which exert tonic drive to activate the posterior cricoarytenoid muscle, has recently been identified in MSA cases.

Alzheimer's disease: An overview of amyloid beta dependent pathogenesis and its therapeutic implications along with in silico approaches emphasizing the role of natural products

Manika Awasthi, Swati Singh, Veda P. Pandey, Upendra N. Dwivedi

Alzheimer's disease (AD) is a progressive and irreversible neurodegenerative disorder characterized by amyloid beta (Aβ) deposition in brain with subsequent formation of neuritic plaques leading to dementia. A number of therapeutic strategies targeted against Aβ depositions have been rigorously explored which provided successful results corresponding to the existing symptomatic treatments. However, at the same time, several failures corresponding to the disease altering therapies and drugs have also been observed due to potential drawbacks in understanding of the pathogenesis of the disease, development of drug candidates and subsequent designing of clinical trials.


Basic Research Papers

Gait variability across the disability spectrum in people with multiple sclerosis

Alon Kalron

An alternative method suggested to assess changes in walking in people with multiple sclerosis (PwMS) is evaluating gait variability. This is a credible option since gait variability reflects to some degree the quality of gait control.

Two young stroke patients associated with regular intravenous immunoglobulin (IVIg) therapy

Yumiko Nakano, Takeshi Hayashi, Kentaro Deguchi, Kota Sato, Nozomi Hishikawa, Toru Yamashita, Yasuyuki Ohta, Yoshiki Takao, Tomohiro Morio, Koji Abe

We recently experienced 2 young adult patients who developed ischemic stroke after regular intravenous immunoglobulin (IVIg) therapy for agammaglobulinemia with diagnosis of common variable immunodeficiency (CVID) in their childhood. Patient 1 was 26-year-old woman, who developed Wallenberg's syndrome 6 days after the last IVIg therapy, but had no further stroke recurrence with cilostazol later. Patient 2 was 37-year-old man, who developed recurrent cerebral infarction in the territory of bilateral lenticulostriate branches like branch atheromatous disease (BAD) several days after the IVIg therapy.

Continuation and adherence rates on initially-prescribed intensive secondary prevention therapy after Rapid Access Stroke Prevention (RASP) service assessment

Stephen J.X. Murphy, Catherine A. Coughlan, Oliver Tobin, Justin Kinsella, Roisin Lonergan, Myles Gutkin, Dominick J.H. McCabe

Consistent adherence to treatment is essential for effective secondary prevention following TIA/ischaemic stroke. Representative data on long-term treatment continuation and adherence rates are limited.

Ingested (oral) anti-IL-12/23 inhibits EAE

Staley A. Brod

Blocking the activity of IL-12/23 can inhibit autoimmune diseases such as psoriasis.

Rhabdomyolysis featuring muscular dystrophies

Rajat Lahoria, Margherita Milone

Rhabdomyolysis is a potentially life threatening condition of various etiology. The association between rhabdomyolysis and muscular dystrophies is under-recognized in clinical practice.

Comparison of motor and non-motor features between essential tremor and tremor dominant Parkinson's disease

Kyum-Yil Kwon, Hye Mi Lee, Seon-Min Lee, Sung Hoon Kang, Seong-Beom Koh

Differential diagnosis of tremor disorders, including essential tremor (ET) and Parkinson's disease-tremor dominant type (PD-TDT), requires further investigation. Therefore, the current study aimed to compare non-motor and tremor features in order to differentiate between ET and PD-TDT.

Prolonged central sensory conduction time in patients with chronic arsenic exposure

Hitoshi Mochizuki, Kazuhiro Yagi, Kazuhito Tsuruta, Akitoshi Taniguchi, Nobuyuki Ishii, Kazutaka Shiomi, Masamitsu Nakazato
  • DOI: http://dx.doi.org/10.1016/j.jns.2015.12.020
  • p39–42
  • Published online: December 11 2015
  • Open Access
  • Abstract
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Many patients from Toroku, Japan, who have chronic arsenic exposure demonstrate whole-body sensory disturbance that is slightly more pronounced in the extremities. Although previous research in this population showed a mild peripheral neuropathy, it is unknown whether these patients have central nervous system impairment. To investigate the lesion sites underlying sensory disturbance related to chronic arsenic poisoning, we analyzed somatosensory evoked potentials (SEP).

Cognitive impairment and neurovascular function in patients with severe steno-occlusive disease of a main cerebral artery

Mami Ishikawa, Haruna Saito, Tomotaka Yamaguro, Masashi Ikoda, Akira Ebihara, Gen Kusaka, Yuichi Tanaka

Patients with severe steno-occlusive disease of a main cerebral artery may demonstrate cognitive impairment without identification of causative lesions on magnetic resonance imaging. We investigated whether cognitive impairment in these patients is associated with regional cerebral blood flow (rCBF), leukoaraiosis, risk factors of atherosclerosis and cerebrovascular reserve (CVR), which shows so-called clinical neurovascular function.

The association of resting state heart rate variability and 24-hour blood pressure variability in spinal cord injury

Julian F. Thayer, John J. Sollers III, Annika Clamor, Julian Koenig, Kristofer J. Hagglund

Patients with high cervical complete spinal cord injuries (tetraplegia) sustain damage to the autonomic neural pathways that influence cardiovascular functioning and produce variability in the heart rate (HR) and blood pressure (BP). In non-injured individuals, an inverse relationship exists between resting autonomic control of the heart (as evidenced by HR variability (HRV)) and BP variability (BPV). This study examined the relationship between HRV, BP and BPV in individuals with tetraplegic (n = 10) and paraplegic (n = 10) spinal cord injuries, and a group of healthy controls (n = 14).                    

Parkinson's Disease—Cognitive Functional Rating Scale across different conditions and degrees of cognitive impairment

Eva Ruzafa-Valiente, Ramón Fernández-Bobadilla, Carmen García-Sánchez, Javier Pagonabarraga, Saül Martínez-Horta, Jaime Kulisevsky

The Parkinson's Disease—Cognitive Functional Rating Scale (PD-CFRS) was designed to avoid motor biases in capturing the functional impact of cognitive impairment in Parkinson's disease (PD). Its performance capturing functional impairment in other conditions leading to cognitive dysfunction is unknown. We compare it with non-specific Instrumental Activities of Daily Living scales.

Intact emotion recognition and experience but dysfunctional emotion regulation in idiopathic Parkinson's disease

Rottraut Ille, Albert Wabnegger, Petra Schwingenschuh, Petra Katschnig-Winter, Mariella Kögl-Wallner, Karoline Wenzel, Anne Schienle
  • DOI: http://dx.doi.org/10.1016/j.jns.2015.12.007
  • p72–78
  • Published online: December 7 2015
  • Open Access
  • Abstract
  • Full-Text HTML
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A specific non-motor impairment in Parkinson's disease (PD) concerns difficulties to accurately identify facial emotions. Findings are numerous but very inconsistent, ranging from general discrimination deficits to problems for specific emotions up to no impairment at all. By contrast, only a few studies exist about emotion experience, altered affective traits and states in PD.

Freezing of gait in early Parkinson's disease: Nigral iron content estimated from magnetic resonance imaging

Marguerite Wieler, Myrlene Gee, Richard Camicioli, W.R. Wayne Martin

Freezing of gait is a major source of disability associated with the progression of Parkinson's disease (PD). Our objective was to determine whether evolving changes in nigral iron content in association with declining motor function in early PD differentiates subjects who develop freezing from those who do not.

Step-rate cut-points for physical activity intensity in patients with multiple sclerosis: The effect of disability status

Stamatis Agiovlasitis, Brian M. Sandroff, Robert W. Motl

Evaluating the relationship between step-rate and rate of oxygen uptake (VO2) may allow for practical physical activity assessment in patients with multiple sclerosis (MS) of differing disability levels.

Heme oxygenase-1 dependant pathway contributes to protection by tetramethylpyrazine against chronic hypoxic injury on medulla oblongata in rats

Yan Ding, Xuefei Hou, Li Chen, Hua Zhou, Yanju Gong, Liqun Dai, Yu Zheng

Tetramethylpyrazine (TMP), one of the active ingredients of the Chinese herb Lingusticum Wallichii (Chuan Xiong) has been proved to protect the medulla oblongata from chronic hypoxia injury. However, the underlying mechanism remains unclear. The purpose of this study was to determine whether the protective effects of TMP are associated with the heme oxygenase-1 (HO-1) dependant pathway in adult rats. The morphological changes of neurons in the hypoglossal nucleus (12N), the nucleus ambiguus (Amb), the nucleus tractus solitarius (NTS), and the pre-Bötzinger complex (pre-BötC) were investigated by Nissl staining; the malondialdehyde (MDA) content and superoxide dismutase (SOD) activity were measured to evaluate the anti-oxidant effect; some apoptosis parameters, Bax mRNA and Bcl-2 mRNA, were tested; and the double immunochemistry staining of active caspase-3/NeuN was performed.          

White blood cell count and clinical outcomes after intracerebral hemorrhage: The INTERACT2 trial

Sungwook Yu, Hisatomi Arima, Emma Heeley, Candice Delcourt, Martin Krause, Bin Peng, Jie Yang, Guojun Wu, Xiaoying Chen, John Chalmers, Craig S. Anderson, on behalf of the INTERACT2 Investigators

Increased inflammatory reaction can aggravate brain injury after acute intracerebral hemorrhage, but the clinical effect of such response is not fully understood. The aim of this study was to determine associations of peripheral white blood cell (WBC) count on clinical outcome among participants of the INTERACT2 study.

Clusterin CSF levels in differential diagnosis of neurodegenerative disorders

Hana Přikrylová Vranová, Eva Hényková, Jan Mareš, Michaela Kaiserová, Kateřina Menšíková, Miroslav Vaštík, Petr Hluštík, Jana Zapletalová, Miroslav Strnad, David Stejskal, Petr Kaňovský

Clusterin, a heterodimeric glycoprotein, is thought to be involved in many cellular functions, including cell–cell interaction, cell survival and apoptosis. In the brain, post-mortem analysis has found increased clusterin associated with the pathology of many other neurodegenerative diseases (ND) such as Parkinson's disease (PD), dementia with Lewy bodies (DLB), Alzheimer's disease (AD) and multiple system atrophy (MSA). In vivo cerebrospinal fluid (CSF) levels of clusterin in ND diseases may reflect differences in the pathology and thus aid in the differential diagnosis.

Sonographic assessment of the optic nerve sheath diameter in the diagnosis of idiopathic intracranial hypertension

Pablo del Saz-Saucedo, Olga Redondo-González, Ángel Mateu-Mateu, Rafael Huertas-Arroyo, Rafael García-Ruiz, Enrique Botia-Paniagua

Objective: Sonographic assessment of the optic nerve sheath diameter (ONSD) is a useful technique in detecting raised intracranial pressure (ICP) in neurocritical care patients. Its utility in idiopathic intracranial hypertension (IIH) is less known. The aim of this study was to evaluate the diagnostic accuracy of ONSD for detecting IIH. Material and methods: Ultrasound measurement of ONSD was performed in 19 patients with IIH and in 11 patients with different neurological diseases without raised ICP that required undergoing a lumbar puncture.

Preoperative motor strength and time to surgery are the most important predictors of improvement in foot drop due to degenerative lumbar disease

Mohamed Macki, Sbaa Syeda, Panagiotis Kerezoudis, Ziya L. Gokaslan, Ali Bydon, Mohamad Bydon

Palsy of dorsiflexion, or foot drop, may be due to degenerative lumbar disease and amenable to posterior spinal decompression. The objective of this study is to measure prognostic factors of and time to foot drop improvement after posterior lumbar decompression.

Cognitive and neuropsychiatric features of orthostatic tremor: A case–control comparison

Julián Benito-León, Elan D. Louis, Verónica Puertas-Martín, Juan Pablo Romero, Michele Matarazzo, José Antonio Molina-Arjona, Cristina Domínguez-González, Álvaro Sánchez-Ferro

Evidence suggests that the cerebellum could play a role in the pathophysiology of orthostatic tremor. The link between orthostatic tremor and the cerebellum is of interest, especially in light of the role the cerebellum plays in cognition, and it raises the possibility that orthostatic tremor patients could have cognitive deficits consistent with cerebellar dysfunction. Our aim was to examine whether orthostatic tremor patients had cognitive deficits and distinct personality profiles when compared with matched controls.

Increased plasma UCH-L1 after aneurysmal subarachnoid hemorrhage is associated with unfavorable neurological outcome

Heikki Kiiski, Jyrki Tenhunen, Marika Ala-Peijari, Heini Huhtala, Mari Hämäläinen, Jaakko Långsjö, Eeva Moilanen, Susanna Narkilahti, Juha Öhman, Jukka Peltola

Aneurysmal subarachnoid hemorrhage (aSAH) is a common cause of long-term disability and death. After primary hemorrhage, secondary brain injury is the main cause of mortality and morbidity. Despite extensive research, reliable prognostic biomarkers are lacking. We measured ubiquitin carboxy-terminal hydrolase L1 (UCH-L1) levels in aSAH patients to evaluate its prognostic potential. This is the first time that plasma UCH-L1 has been studied as a potential prognostic biomarker in patients with aSAH.

Initial research on the relationship between let-7 family members in the serum and massive cerebral infarction

Zhe Gong, Shaoyun Zhao, Jing Zhang, Xiaoge Xu, Wenjuan Guan, Lijun Jing, Peidong Liu, Jingjing Lu, Junfang Teng, Tao Peng, Yanjie Jia

Eighty-eight ischemic stroke patients with massive cerebral infarction (MCI) who met our selection criteria were included in this study. MCI was assessed using the Glasgow Coma Scale (GCS) at hospital admission and at 2 weeks. The sera of all patients and controls were sampled at 48 h after the patients' attacks, and the sera of patients with MCI who had no severe cardiopulmonary complications, including those with hemorrhagic transformation (HT), were sampled again at 2 weeks. The relative expression of let-7 miRNA in the serum was determined by real-time qRT-PCR, and the blood levels of lipids, glucose, high-sensitivity C-reactive protein (hs-CRP), homocysteine and blood pressure were measured at admission.          

Manual therapy as an effective treatment for fibrosis in a rat model of upper extremity overuse injury

Geoffrey M. Bove, Michele Y. Harris, Huaqing Zhao, Mary F. Barbe

Key clinical features of carpal tunnel syndrome and other types of cumulative trauma disorders of the hand and wrist include pain and functional disabilities. Mechanistic details remain under investigation but may involve tissue inflammation and/or fibrosis. We examined the effectiveness of modeled manual therapy (MMT) as a treatment for sensorimotor behavior declines and increased fibrogenic processes occurring in forearm tissues of rats performing a high repetition high force (HRHF) reaching and grasping task for 12 weeks.         

C9ORF72 repeat expansion is not detected in sporadic ataxia patients in mainland China

Miao He, Wei-Qian Yan, Sheng Zeng, Zhen Liu, Yao Zhou, Xian-Feng Zeng, Jun-Sheng Zeng, Hong Jiang, Lu Shen, Bei-Sha Tang, Jun-Ling Wang

Expansion of a GGGGCC hexanucleotide repeat in the gene C9ORF72 is a common pathogenic mutation in families with autosomal dominant frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). In order to understand whether pathogenic GGGGCC expansions of C9ORF72 are associated with spinocerebellar ataxia (SCA) in mainland China, we performed an experiment to determine the prevalence of pathogenic hexanucleotide expansions of C9ORF72 in a large cohort of Chinese Han patients with SCA. 411 sporadic patients with SCA and 314 healthy controls were screened for pathogenic hexanucleotide expansions of C9ORF72 utilizing a repeat primed polymerase chain reaction assay.

Identifying a therapeutic window in acute and subacute inflammatory sensory neuronopathies

Jean-Christophe Antoine, Florence Robert-Varvat, Thierry Maisonobe, Alain Créange, Jérôme Franques, Stéphane Mathis, Emilien Delmont, Thierry Kuntzer, Jean-Pascal Lefaucheur, Jean Pouget, Karine Viala, Claude Desnuelle, Andoni Echaniz-Laguna, Francesco Rotolo, Jean-Philippe Camdessanché, the French Neuromuscular Network (FILNEMUS)

Patients with inflammatory sensory neuronopathy (SNN) may benefit from immunomodulatory or immunosuppressant treatments if administered timely. Knowing the temporal profile of neuronal loss in dorsal root ganglia will help to ascertain whether a final diagnosis may be reached before the occurrence of irreversible neuronal injuries. Thus, we addressed the evolution of neuronal loss in SNN by using sensory nerve action potentials (SNAPs) as a surrogate marker of neuron degeneration.

The association of pathological laughing and crying and cognitive impairment in multiple sclerosis

Joshua Hanna, Anthony Feinstein, Sarah A. Morrow

Pathological laughing and crying (PLC) is common in multiple sclerosis (MS), defined as emotional expression that is exaggerated/incongruent with underlying mood. In other neurological disorders, PLC is associated with cognitive impairment (CI). Few studies have examined this relationship in MS.

Effect of lumbo-peritoneal shunt surgery on neuropsychiatric symptoms in patients with idiopathic normal pressure hydrocephalus

Hideki Kanemoto, Hiroaki Kazui, Yukiko Suzuki, Syunsuke Sato, Haruhiko Kishima, Toshiki Yoshimine, Kenji Yoshiyama

Patients with idiopathic normal pressure hydrocephalus (iNPH) have neuropsychiatric symptoms that could affect patients' quality of life and caregiver burden. In this study, we assessed the effect of lumbo-peritoneal (L-P) shunt surgery on neuropsychiatric symptoms and the association between neuropsychiatric symptoms and caregiver burden after L-P shunt surgery.

BECLA, a new assessment battery for acquired deficits of language: Normative data from Quebec-French healthy younger and older adults

Joël Macoir, Caroline Gauthier, Catherine Jean, Olivier Potvin

Compared to English, for which there exist numerous tests and batteries for the assessment of acquired deficits of language, the tools available to assess French-speaking individuals are much more limited. The Batterie d'Évaluation Cognitive du Langage (BECLA) was purposely developed to fulfill the need for French assessment tools based on theoretical models of cognitive psychology. It comprises 19 tasks, designed to assess each of the components and routes involved in single word processing in order to identify the functional locus/loci of impairment.   

Effects of soybean ingestion on pharmacokinetics of levodopa and motor symptoms of Parkinson's disease — In relation to the effects of Mucuna pruriens

Yasuhiro Nagashima, Tomoyoshi Kondo, Mayumi Sakata, Jinsoo Koh, Hidefumi Ito

Mucuna pruriens is a levodopa-containing legume and its favorable effects on motor complications in Parkinson disease patients have been reported. The aim of this study was to investigate the effects of another legume, soybeans, on the pharmacokinetics and metabolism of levodopa. Seven parkinsonian patients with the wearing-off phenomenon and dyskinesia and five healthy volunteers participated in this study. We conducted a crossover study of the clinical effects on the participants before and after taking either levodopa (100 mg)/carbidopa (10 mg) only (LD/CD) or levodopa/carbidopa with 11 g of ground soybeans (LD/CD/soy).    

Coping with amyotrophic lateral sclerosis; from diagnosis and during disease progression

Birgitta Jakobsson Larsson, Karin Nordin, Ingela Nygren

To evaluate coping strategies among patients with Amyotrophic lateral sclerosis starting with diagnosis and during the disease progression, as well as investigate changes and correlations between coping strategies, emotional well-being and physical function. A total of 36 patients participated in the study. The patients filled out the Hospital Anxiety and Depression Scale and the Motor Neuron Disease Coping Scale. Physical function was measured using the revised ALS functional rating scale. Data were collected regularly from diagnosis and over a two years period.

Association between polymorphism of COMT gene (Val158Met) with Alzheimer's disease: An updated analysis

Weiqian Yan, Cheng Zhao, Lu Sun, Beisha Tang

Previous studies have proposed the association between catechol-o-methyltransferase (COMT) Val158Met polymorphism and the risk of Alzheimer's disease (AD). However, there has been no solid conclusion from those reports because of the limited sample size and/or racial diversity. Here we aimed to give an in-depth and accurate evaluation of this association by performing a comprehensive literature search on websites including PubMed, EMBASE, Alzgene database, Cochrane library and Ovid database. In further meta-analysis we identified 10 case–control studies which contained total 2777 cases and 2829 controls.                  

Medically unexplained visual loss in a specialist clinic: a retrospective case–control comparison

Éanna D. O'Leary, Benjamin McNeillis, Selma Aybek, Paul Riordan-Eva, Anthony S. David

To compare the clinical and demographic characteristics of adult patients with nonorganic or medically unexplained visual loss (MUVL) to those with other common conditions presenting to a neuro-ophthalmology clinic.


Clinical Short Communications

Relative preservation of finger flexion in amyotrophic lateral sclerosis

Ari Shemesh, David Arkadir, Marc Gotkine

A characteristic pattern of intrinsic hand muscle involvement—known as the split hand sign—is typical of ALS; differential involvement of forearm muscles has not been examined systematically. After observing that finger-flexion was often preserved in ALS, despite severe weakness of finger-extension, we assessed the relative involvement of these two muscle groups in a cohort of patients with ALS. We found finger-flexion to be relatively preserved, when compared with finger-extension, in patients with ALS.

Lower urinary tract dysfunction in patients with functional movement disorders

Amit Batla, Isabel Pareés, Mark J. Edwards, Maria Stamelou, Kailash P. Bhatia, Jalesh N. Panicker

Functional movement disorders (FMD) are not associated with the kind of structural or biochemical alterations seen in other movement disorders and therefore would be unlikely to be associated with lower urinary tract (LUT) dysfunction. However, LUT symptoms have been observed in patients with FMD.

Down-regulation of adenosine A1 and A2A receptors in peripheral cells from idiopathic normal-pressure hydrocephalus patients

Martina Casati, Beatrice Arosio, Cristina Gussago, Evelyn Ferri, Lorenzo Magni, Lara Assolari, Valeria Scortichini, Carolina Nani, Paolo Dionigi Rossi, Daniela Mari

Idiopathic normal-pressure hydrocephalus (iNPH) is a neurological disease that usually develops in the elderly. Natural history of iNPH is still unknown. It has been hypothesized that cerebrovascular diseases could have a role in etiology of chronic hydrocephalus and studies show an increased prevalence of cardiovascular diseases in iNPH patients. Moreover, evidences show a possible alteration of immune system in iNPH patients. Adenosine (Ado) is a metabolite produced in response to metabolic stress and injury.                        


Letters to the Editor

Thrombolysis for acute stroke in patient with systemic lupus erythematosus: A case report

Maja Rubinić Majdak, Vladimira Vuletić

Neurological symptoms and signs are rare at onset of systemic lupus erythematosus (SLE). To our knowledge, just one similar case is reported in literature on the use of recombinant tissue plasminogen activator (rt-PA) in patients with SLE who develop an ischemic stroke. We present a case of 40-year-old woman with an acute stroke who received rt-PA. Magnetic resonance imaging (MRI) revealed a large ischemic stroke in right hemisphere and a magnetic resonance angiography (MRA) revealed signs of vasculitis.

Acute posterior multifocal placoid pigment epitheliopathy presenting with multiple brain and spinal cord infarctions

Pedro Castro, Andreia Costa, Pedro Abreu, Susana Penas, Olinda Faria, Elsa Azevedo

Tumefactive demyelinating brain lesions with multiple closed-ring enhancement in the course of neuromyelitis optica

Yuji Tomizawa, Ryota Nakamura, Yasunobu Hoshino, Fuyuko Sasaki, Sho Nakajima, Sumihiro Kawajiri, Kazuyuki Noda, Masashi Takanashi, Naohide Fujita, Kazumasa Yokoyama, Nobutaka Hattori, Toshiyuki Takahashi, Yasuyuki Okuma

A 54-year-old man, previously diagnosed with neuromyelitis optica (NMO), was referred to Juntendo University Shizuoka Hospital due to difficulty in concentrating at work and a gradually worsening left hemiparesis. His first attack occurred at the age of 42 years, at which point a long extensive spinal cord lesion (LESCL) in the cervical cord was observed. The second attack occurred at the age of 45 years, at which point a small cerebellar lesion was observed. He was diagnosed with multiple sclerosis (MS) because the anti-aquaporin-4 (AQP4) antibody test was not available at the time.   

Primary cerebellar progressive multifocal leukoencephalopathy (PML) shows increased accumulation in 123I-IMP SPECT

Shinjiro Fukami, Jiro Akimoto, Megumi Ichikawa, Hiroyuki Jimbo, Yukio Ikeda, Yohhei Ishimura, Kazunori Nanri, Kazuhiro Tsuchiya, Michihiro Kohno

Progressive multifocal leukoencephalopathy (PML) is a rare infectious demyelinating disorder of the central nervous system caused by JC virus [1]. PML tends to occur in cerebral white matter, and can cause akinetic mutism for a few months, indicating a poor prognosis. In recent years, PML associated with human immunodeficiency virus (HIV) infection and molecular target treatment has been increasing. PML rarely occurs in the cerebellum. In this paper, we describe two cases of primary cerebellar PML without acquired immunodeficiency syndrome (AIDS), focusing on radiographic characteristics, specifically N-isopropyl-p-[123I]-iodoamphetamine (123I-IMP) single photon emission computed tomography (SPECT).

Hemiparetic Guillain–Barré syndrome

Jee-Eun Kim, Nobuhiro Yuki

Guillain–Barré syndrome (GBS) is characterized by symmetric motor weakness. We reported an unusual case of GBS who presented with left hemiparesis at the acute phase and right hemifacial weakness at the recovery phase of the hemiparesis.

Dynamic pattern of clinical and MRI findings in a tumefactive demyelinating lesion: A case report

P. Preziosa, V. Martinelli, L. Moiola, M. Radaelli, S. Gerevini, D. Guidetti, P. Immovilli, E. Michieletti, P. Scagnelli, M. Comola, M.A. Rocca, M. Filippi, G. Comi

Tumefactive demyelinating lesions (TDLs) are atypical brain lesions of large size (generally ≥ 2 cm), for which an idiopathic inflammatory origin is suspected [1,2] and which might develop alone or with other inflammatory lesions.

Neuromyelitis optica spectrum initially diagnosed as antiphospholipid antibody myelitis

Hilda Guerra, Sean J. Pittock, Kevin G. Moder, David A. Froehling, Eoin P. Flanagan

Neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) are autoimmune water channelopathies that predominantly target astrocytes (resulting in secondary demyelination) and are associated with the highly specific serum biomarker and pathogenic autoantibody, aquaporin-4-IgG (AQP4-IgG) [1]. Longitudinally extensive transverse myelitis (LETM) with MRI lesions spanning ≥ 3 vertebral segments is a characteristic feature of NMO/NMOSD [1].