JNS-cover.jpgJournal of Neurological Sciences

Vol 350 | No. 1-2 | 15 March 2015 | Pages 1-120


Employment of higher doses of botulinum toxin type A to reduce spasticity after stroke

Andrea Santamato, Maria Francesca Micello, Maurizio Ranieri, Giovanni Valeno, Antonio Albano, Alessio Baricich, Carlo Cisari, Domenico Intiso, Alberto Pilotto, Giancarlo Logroscino, Francesco Panza

Spasticity is a common disabling symptom for several neurological conditions. Botulinum toxin type A injection represents the gold standard treatment for focal spasticity with efficacy, reversibility, and low prevalence of complications. Current guidelines suggest a dose up to 600 units (U) of onabotulinumtoxinA/incobotulinumtoxinA or up to 1500 U of abobotulinumtoxinA to treat post-stroke spasticity to avoid important adverse effects. However, recently, higher doses of botulinum toxin type A were employed, especially in case of upper and lower limb severe spasticity.  

Stroke and sexual dysfunction — A narrative review

Jong-Ho Park, Bruce Ovbiagele, Wuwei Feng

Sexual function is an essential part of quality of life in adults. However, sexual dysfunction (SD) in stroke survivors is a common but under-recognized complication after stroke. It is frequently neglected by patients and clinicians. The etiology of post-stroke SD, which is multifactorial includes anatomical, physical and psychological factors. Complete return of sexual function is an important target for functional recovery after stroke, so clinicians need to be aware of this issue and take a lead role in addressing this challenge in stroke survivors.


Original Articles

A new simple score (ABS) for assessing behavioral and psychological symptoms of dementia

K. Abe, T. Yamashita, N. Hishikawa, Y. Ohta, K. Deguchi, K. Sato, K. Matsuzono, Y. Nakano, Y. Ikeda, Y. Wakutani, Y. Takao

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.01.029
  • p14–17
  • Published online: January 28, 2015
  • Open Access
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In addition to cognitive impairment, behavioral and psychological symptoms of dementia (BPSD) are another important aspect of most dementia patients. This study was designed for a new simple assessment of BPSD. We first employed a clinical survey for the local community with sending an inquiry letter to all members (n = 129) of dementia caregiver society, and then attempted to create a new BPSD score for dementia with 10 BPSD items. This new simple BPSD score was compared to a standard-detailed BPSD score neuropsychiatric inventory (NPI) for a possible correlation (n = 792) and a time to complete (n = 136).                       

Interleukin-10 but not transforming growth factor-β1 gene expression is up-regulated by vitamin D treatment in multiple sclerosis patients

Zeinab Shirvani Farsani, Mehrdad Behmanesh, Mohammad Ali Sahraian

Multiple sclerosis (MS) is an inflammatory and autoimmune disease. Variety of different genetics and environmental factors are involved in MS pathology. The epidemiological studies demonstrated that vitamin D has immune and immunomodulating effects on MS disease. Therefore, this study aims to evaluate the effect of vitamin D treatment on the expression of interleukin-10 (IL-10) and transforming growth factor-β1 (TGF-β1) genes in MS patients. We found that, the expression level of IL-10 gene in treated patients was up-regulated 3.84 times more than before treatment, but the expression level of TGF-β1 was not affected by vitamin D treatment.

Stroke admission and case-fatality in an urban medical unit in sub-Saharan Africa: A fourteen year trend study from 1999 to 2012

Alain Lekoubou, Clovis Nkoke, Anastase Dzudie, Andre Pascal Kengne

Data on recent stroke trends in the context of rapidly deteriorating risk profile of populations within Africa is very limited. We investigated the admission trend for stroke and related outcomes in a major referral hospital in Cameroon.

Association between ventricular volume measures and pulsatile and static intracranial pressure scores in non-communicating hydrocephalus

Terje Sæhle, Per Kristian Eide

In non-communicating hydrocephalus (HC), enlarged cerebral ventricles are often thought to reflect increased intracranial pressure (ICP) or increased pulsatile ICP. The present study was undertaken to explore the association between ventricular volume measures and pulsatile or static ICP scores in patients with non-communicating HC. Since linear measures of ventricular size have the most widespread use, we also examined how linear and volume measures of ventricular size compare.

Expression of suppressor of cytokine signaling 1 (SOCS1) gene dramatically increases in relapsing–remitting multiple sclerosis

Majid Pahlevan Kakhki, Nahid Rakhshi, Masoumeh Heidary, Mehrdad Behmanesh, Abbas Nikravesh

Suppressor of cytokine signaling 1 (SOCS1) is a key regulator of cytokines signaling and plays the most important role in the regulation of the autoimmune responses. The absence of SOCS1 leads to aberrant thymocyte development and systemic inflammation. This study was conducted to evaluate the expression level of SOCS1 mRNA in peripheral blood mononuclear cells (PBMCs) of relapsing–remitting (RR)-multiple sclerosis (MS) patients. In addition, the association of rs243324 SNP with MS and the assessment of this SNP role on the expression level of SOCS1 were aimed to be evaluated.    

Serum NSE level and disability progression in multiple sclerosis

Marcus W. Koch, Suzanne George, Winona Wall, V. Wee Yong, Luanne M. Metz

: Previous studies suggested that serum neuron specific enolase (NSE) may be a biomarker associated with progression in MS.

Clinico-radiological spectrum and outcome in idiopathic hypertrophic pachymeningitis

Gopal Krishna Dash, Bejoy Thomas, Muralidharan Nair, Ashalatha Radhakrishnan

To elucidate the clinico-radiological features, treatment response and outcome of a large cohort of patients (n = 20) with idiopathic hypertrophic pachymeningitis (IHP) and to examine if any of these features could differentiate between IHP and secondary causes of hypertrophic pachymeningitis (SHP).

LRIG3 modulates proliferation, apoptosis and invasion of glioblastoma cells as a potent tumor suppressor

Dongsheng Guo, Hongkuan Yang, Yang Guo, Qungen Xiao, Feng Mao, Yihu Tan, Xueyan Wan, Baofeng Wang, Ting Lei

Leucine-rich repeats and immunoglobulin-like domains (LRIG) 3 gene is mapped to chromosome 12q13.2, a region that is frequently deleted in a subset of glioblastoma multiforme (GBM). It has been reported that perinuclear LRIG3 staining correlated with low WHO grade of glioma and better survival of the patients. However, the relationship between LRIG3 and glioma is not very clear. The purpose of this study is to demonstrate the impacts of LRIG3 on biological characteristics of glioma and its possible mechanisms.

Hypercholesterolemia in elders is associated with slower cognitive decline: A prospective, population-based study (NEDICES)

Julián Benito-León, Saturio Vega-Quiroga, Alberto Villarejo-Galende, Félix Bermejo-Pareja

Studies investigating the association between hypercholesterolemia in the elderly and cognitive decline report discrepant outcomes. We determined in a prospective population-based cohort (NEDICES) in elders whether hypercholesterolemia was associated with slower cognitive decline.

Blood glucose levels during the initial 72 h and 3-month functional outcomes in acute intracerebral hemorrhage: The SAMURAI–ICH study

Masatoshi Koga, Hiroshi Yamagami, Satoshi Okuda, Yasushi Okada, Kazumi Kimura, Yoshiaki Shiokawa, Jyoji Nakagawara, Eisuke Furui, Yasuhiro Hasegawa, Kazuomi Kario, Shoji Arihiro, Shoichiro Sato, Kazunari Homma, Takayuki Matsuki, Naoto Kinoshita, Kazuyuki Nagatsuka, Kazuo Minematsu, Kazunori Toyoda, for the SAMURAI Study Investigators

Prognostic values of blood glucose levels following admission remain unclear. We investigated associations between blood glucose levels during the initial 72 h and outcomes of acute ICH.

Clinical and laboratory characteristics in patients suffering from general paresis in the modern era

Yu-Yan Chen, Ya-Feng Zhang, Xin-Hui Qiu, Qiao Zhang, Fu-Yi Chen, Long Liu, Jin-Yi Fan, Kun Gao, Xiao-Zhen Zhu, Wei-Hong Zheng, Hui-Lin Zhang, Li-Rong Lin, Li-Li Liu, Man-Li Tong, Jian-Jun Niu, Tian-Ci Yang

No gold standard currently exists for the diagnosis of general paresis (GP), thus often resulting in unnecessarily delayed therapeutic decision.

Receptor-associated protein promotes t-PA expression, reduces PAI-1 expression and improves neurorecovery after acute ischemic stroke

Dan-Dong Li, Hong-Gang Pang, Jin-Ning Song, Yong-Lin Zhao, Bin-Fei Zhang, Xu-Dong Ma, Peng Sun

Receptor-associated protein (RAP) is a receptor antagonist that inhibits ligand interactions with the receptors that belong to the low density lipoprotein receptor gene family. The low-density lipoprotein receptor-related protein 1 (LRP1) has a crucial role in regulating tissue plasminogen activator (t-PA) and plasminogen activator inhibitor (PAI-1) expression. Furthermore, the functional balance of these two proteins is directly associated with the initiation and development of cerebral ischemic stroke.


Short Communications

Rare frequency of downbeat positioning nystagmus in spinocerebellar ataxia type 31

Ichiro Yabe, Masaaki Matsushima, Kunihiro Yoshida, Kinya Ishikawa, Shinichi Shirai, Ikuko Takahashi, Hidenao Sasaki

Spinocerebellar ataxia type 31 (SCA31) and spinocerebellar ataxia type 6 (SCA6) are the most frequent types of spinocerebellar degeneration in Japan. Previous reports described that it was difficult to distinguish SCA6 and SCA31 in clinical situations. There is not much difference except that the onset age of SCA31 is slightly higher than that of SCA6. Therefore we surveyed our medical records retrospectively, and then compared clinical symptoms of SCA6 and SCA31. As previously stated, the onset age of SCA31 is higher than that of SCA6.

Parkinsonism, cognitive deficit and behavioural disturbance caused by a novel mutation in the polymerase gamma gene

Manuel Delgado-Alvarado, Patricia de la Riva, Haritz Jiménez-Urbieta, Belén Gago, Alazne Gabilondo, Belén Bornstein, María Cruz Rodríguez-Oroz

Polymerase γ (POLG) is the enzyme responsible for the replication and maintenance of mitochondrial DNA (mtDNA). Mutations in the POLG1 gene can lead to mitochondrial dysfunction, producing a wide range of neurological and non-neurological phenotypes. Neurological manifestations include ataxia, muscular weakness, epilepsy, progressive external ophthalmoplegia (PEO), ptosis, neuropathy, psychiatric disorders and, more rarely, parkinsonism. We present the case of an 80-year old female patient with a history of PEO, ptosis, childish behaviour, obsessive disorder, cognitive decline, and parkinsonism.

Occurrence of multiple Cerebral Cavernous Malformations in a patient with Neurofibromatosis type 1

K. Rerat, F. Parker, G. Nasser, D. Vidaud, F. Riant, E. Tournier-Lasserve, C. Denier

Neurofibromatosis 1 (NF1) belongs to the autosomal dominant neurocutaneous disorders' group, which mainly includes NF1 and NF2, tuberous sclerosis, von Hippel-Lindau disease and Cerebral Cavernous Malformations (CCMs). NF1 has a major impact on the nervous system, eye, skin, bone or cardiovascular system. Cerebrovascular lesions have been reported in NF1 including aneurysm, pseudoaneurysm, arteriovenous malformations, vascular stenosis or occlusion and Moya moya syndrome.


Letters to the Editor

Paroxysmal dysarthria–ataxia syndrome resolving after fingolimod treatment

Silvia Rossi, Valeria Studer, Caterina Motta, Diego Centonze

Paroxysmal dysarthria–ataxia (PDA) is a rare neurological manifestation of brainstem dysfunction, characterized by stereotyped episodes of altered coordination of the speech associated with limb or gait ataxia. The attacks usually last few seconds, and may occur many times during the day. This condition has been anecdotally reported in multiple sclerosis (MS) and in other neurologic diseases [1,4,5]. Carbamazepine has been described to be effective [4].

Diagnosis and treatment of restless legs syndrome in progressive supranuclear palsy

Marcello Moccia, Marina Picillo, Roberto Erro, Roberto Allocca, Paolo Barone, Carmine Vitale

Restless legs syndrome (RLS) has only been recently investigated in a small cohort of progressive supranuclear palsy (PSP) patients and it has been reported to have variable prevalence (among 3.7–58%). However little is known about its management. Here, we report a case of severe RLS occurring during the course of PSP. Diagnostic issues and therapeutic approaches are discussed.

Anti-Ma-associated encephalomyeloradiculopathy in a patient with pleural mesothelioma

Alberto Vogrig, Sergio Ferrari, Michele Tinazzi, Paolo Manganotti, Gaetano Vattemi, Salvatore Monaco

Anti-Ma2-related paraneoplastic syndrome usually presents with limbic/brainstem encephalitis in patients with testicular or lung cancers [1]. Only four cases have been described with peripheral nervous system involvement [2]. We report the unusual association between paraneoplastic encephalomyeloradiculopathy and pleural mesothelioma in a patient with anti-Ma antibodies.

Eye movement changes in mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)

Claudia Vinciguerra, Pamela Federighi, Francesca Rosini, Elena Pretegiani, Elena Cardaioli, Maria T. Dotti, Francesco Sicurelli, Antonio Federico, Alessandra Rufa

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disease due to ECGF1 gene mutations (chromosome 22) causing multiple deletions and depletion of mitochondrial DNA in skeletal muscle. The determination of the activity of the gene-product tymidine phosphorylase (TP) in leukocytes and genetic analysis are diagnostic. External ophthalmoplegia, severe gastrointestinal dysmotility, cachexia, peripheral sensory-motor neuropathy, diffuse myopathy and leukoencephalopathy are typical findings.

Secondary antibody-induced treatment failure under therapy with incobotulinumtoxinA (Xeomin®) in a patient with segmental dystonia pretreated with abobotulinumtoxinA (Dysport®)

Christoph Kamm, Friederike Schümann, Eilhard Mix, Reiner Benecke

IncobotulinumtoxinA (Xeomin®) is a preparation of botulinumtoxinA (BoNT-A) free of complexing proteins [1]. Based on its biological properties and on previous experience in large clinical studies and in clinical practice worldwide since 2005, its immunogenicity is assumed to be lower compared to other commercially available formulations of botulinum toxin. However, recently one single patient with generalized dystonia has been reported [2] who developed secondary treatment failure after five treatment cycles with incobotulinumtoxinA after pretreatment with abobotulinumtoxinA for many years.

Spontaneous dissection of the bilateral internal carotid and vertebral arteries: A rationale for endovascular management

Spontaneous cervical-artery-dissection (sCAD) is a common cause of stroke in young patients. The incidence of concurrent sCAD of more than one neck artery ranges between 10 and 28% [1]. Multi-vessel sCAD is associated with a higher risk of intracranial hemorrhage as well as a higher risk of recurrent stroke or TIA [1]. Postpartum CAD accounts for 6% of spontaneous CAD in women under 50 years of age [2]. Medical management is the primary treatment approach, while the role of endovascular intervention remains unclear.

Delayed imaging abnormalities of neuro-invasive West Nile virus in cancer patients

Jacob J. Mandel, Sudhakar Tummala, Karin Hoang Woodman, Ivo Tremont-Lukats

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.02.014
  • p115–117
  • Published online: February 13, 2015
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West Nile virus (WNV) is a single-stranded ribonucleic acid virus of the genus Flavivirus and the most significant cause of epidemic encephalitis in the western hemisphere [1]. The majority of infections are clinically silent. Seroprevalence following large human epidemics is low and frequently between 2 and 3% per year [2]. Clinical presentation is most commonly a self-limited illness called West Nile fever [3]. Symptoms can include fever, headache, malaise, back pain, myalgias, anorexia and rash persisting for three to six days [3].

Reversal of leukoencephalopathy induced by liposomal amphotericin B in a patient with cryptococcal meningitis

Masayuki Sato, Kimitoshi Hirayanagi, Kouki Makioka, Yoshio Ikeda

  • DOI: http://dx.doi.org/10.1016/j.jns.2015.02.024
  • p118–119
  • Published online: February 17, 2015
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