JNS-cover.jpgJournal of Neurological Sciences

Vol 364 | 15 May 2016 | Pages 1-202


New clinical insights into combined central and peripheral demyelination (CCPD)

Christoph Kamm

Although encountered rather infrequently in clinical practice, patients with autoimmune disorders affecting both the central and peripheral nervous system, also termed combined central and peripheral demyelination (CCPD), frequently pose a challenge for the clinical neurologist concerning differential diagnosis and especially therapeutic options. As of yet, only few case series and case reports reporting clinical characteristics and therapeutic outcome in these patients (adult and pediatric) have been published [1–4].

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

John D. England

One of the most pressing public health issues for 2016 is the Zika virus epidemic, which the World Health Organization (WHO) has designated as a Public Health Emergency of International Concern (PHEIC). The major reasons for world-wide concern are the apparent increases in the number of babies born with microcephaly and the number of cases of Guillain-Barre syndrome (GBS) in the countries where Zika infection is widespread. A direct causal link between infection with the Zika virus and these neurological conditions is strongly suspected, but has not been proven.

What a downer: The dark side of cannabis

David Z. Rose

It has been known for decades that cigarette smoking is associated with an increased risk of stroke. In 1988, the Framingham Heart Study cohort showed that even after age and hypertension were taken into account, smoking was significantly related to stroke [1]. This risk increased with greater number of cigarettes smoked, and conversely, after two years of cessation, decreased significantly, and by five years reached the level of nonsmokers.


Review Articles

Genetic substrates of psychosis in patients with Parkinson's disease: A critical review

Abhishek Lenka, Shyam Sundar Arumugham, Rita Christopher, Pramod Kumar Pal

Patients with Parkinson's disease (PD) may develop several non-motor symptoms such as psychosis, depression, cognitive impairment, autonomic disturbances and sleep disturbances. Psychosis is one of the common non-motor symptoms, which commonly manifests as visual hallucinations and minor hallucinations such as sense of passage and presence. Though long-term dopaminergic therapy, longer duration of PD and cognitive impairment have been described as risk factors for emergence of psychosis in PD, predicting psychosis in PD remains challenging.

Stroke survivors in low- and middle-income countries: A meta-analysis of prevalence and secular trends

Martinsixtus C. Ezejimofor, Yen-Fu Chen, Ngianga-Bakwin Kandala, Benedeth C. Ezejimofor, Aloysius C. Ezeabasili, Saverio Stranges, Olalekan A. Uthman

To provide an up-to-date estimate on the changing prevalence of stroke survivors, and examines the geographic and socioeconomic variations in low and middle-income countries (LMICs).

Autism spectrum disorders: Integration of the genome, transcriptome and the environment

N. Thushara Vijayakumar, M.V. Judy

Autism spectrum disorders denote a series of lifelong neurodevelopmental conditions characterized by an impaired social communication profile and often repetitive, stereotyped behavior. Recent years have seen the complex genetic architecture of the disease being progressively unraveled with advancements in gene finding technology and next generation sequencing methods. However, a complete elucidation of the molecular mechanisms behind autism is necessary for potential diagnostic and therapeutic applications.


Basic Research Papers

The impact of early morning off in Parkinson's disease on patient quality of life and caregiver burden

Rieko Onozawa, Jun Tsugawa, Yoshio Tsuboi, Jiro Fukae, Takayasu Mishima, Shinsuke Fujioka

Early morning off (EMO) is a symptom experienced by patients with Parkinson's disease (PD) in any stage of the illness; however, few studies have explored its prevalence. Thus, the impact of EMO on patient Quality of Life (QOL) and caregiver burden is unclear.

Analysis of the association of fluid balance and short-term outcome in traumatic brain injury

Zilong Zhao, Dong Wang, Ying Jia, Ye Tian, Yi Wang, Yingsheng Wei, Jianning Zhang, Rongcai Jiang

A balance of fluid intake and output (fluid balance) influences outcomes of critical illness, but the level of such influence remains poorly understood for traumatic brain injury (TBI) and was quantitatively examined in this study.

Risk factors associated with acute/subacute cerebral infarction in HIV-negative patients with cryptococcal meningitis

Yan-Fang Chen, Dan-Ni Wang, Zhi-Ting Chen, Zhen-Hua Zhao, Yu Lin, Hua-Yan Wang, Ning Wang

To explore the risk factors associated with acute/subacute cerebral infarction (ASCI) in HIV-negative patients with cryptococcal meningitis (CM).

Prevalence of neurofascin-155 antibodies in patients with multiple sclerosis

O. Stich, S. Perera, B. Berger, S. Jarius, B. Wildemann, A. Baumgartner, S. Rauer

Antibodies against neurofascin, an axo-glial protein located around the node of Ranvier, have been shown to contribute to axonal pathology both in vitro and in experimental autoimmune encephalomyelitis models. Moreover, small case studies have reported anti-NF antibodies in samples from patients with progressive multiple sclerosis (MS).

Late-onset spastic paraplegia type 10 (SPG10) family presenting with bulbar symptoms and fasciculations mimicking amyotrophic lateral sclerosis

Seiji Kaji, Toshitaka Kawarai, Ryosuke Miyamoto, Hiroyuki Nodera, Lucia Pedace, Antonio Orlacchio, Yuishin Izumi, Ryosuke Takahashi, Ryuji Kaji

Pathogenic mutations in the KIF5A-SPG10 gene, encoding the kinesin HC5A, can be associated with autosomal dominant hereditary spastic paraplegia (ADHSP). It accounts for about 10% of the complicated forms of ADHSP. Peripheral neuropathy, distal upper limb amyotrophy, and cognitive decline are the most common additional clinical features. We examined a 66-year-old Japanese woman manifesting gait disturbance and spastic dysarthria for 6 years with positive family history. She showed evidence of upper and lower motor neuron involvement and fasciculations, thus mimicking amyotrophic lateral sclerosis (ALS).  

Recurrent Guillain–Barré syndrome, Miller Fisher syndrome and Bickerstaff brainstem encephalitis

Junko Ishii, Nobuhiro Yuki, Michi Kawamoto, Hajime Yoshimura, Susumu Kusunoki, Nobuo Kohara

Guillain–Barré syndrome (GBS), Miller Fisher syndrome (MFS), and Bickerstaff brainstem encephalitis (BBE) are usually monophasic, but some patients experience recurrences after long asymptomatic intervals. We aimed to investigate clinical features of recurrent GBS, MFS, and BBE at a single hospital.

The diagnostic challenge of Divry van Bogaert and Sneddon Syndrome: Report of three cases and literature review

Anna Bersano, Michela Morbin, Elisa Ciceri, Gloria Bedini, Peter Berlit, Michele Herold, Stefania Saccucci, Valeria Fugnanesi, Hannes Nordmeyer, Giuseppe Faragò, Mario Savoiardo, Franco Taroni, MariaRita Carriero, Battista Boncoraglio Giorgio, Laura Perucca, Luigi Caputi, Agostino Parati Eugenio, Markus Kraemer

Divry van Bogaert Syndrome (DBS) is a familial juvenile-onset disorder characterized by livedo racemosa, white matter disease, dementia, epilepsy and angiographic finding of "cerebral angiomatosis". A similar syndrome including livedo racemosa and cerebrovascular disease, often associated with anticardiolipin antibodies, has been described as Sneddon Syndrome (SS) highlighting the question whether these two conditions have to be considered different entities or indeed different features of a unique syndrome.

MicroRNA-29a: A potential biomarker in the development of intracranial aneurysm

Wei-Hua Wang, Yun-Hua Wang, Li-Li Zheng, Xiao-Wan Li, Fang Hao, Dong Guo

To identify serum microRNA-29a (miR-29a) level in patients with intracranial aneurysm and its role in the development of intracranial aneurysm (IA).

Diffusion tensor imaging of idiopathic normal-pressure hydrocephalus and the cerebrospinal fluid tap test

Kyunghun Kang, Uicheul Yoon, Woohyuk Choi, Ho-Won Lee

We evaluated relationships between diffusion tensor imaging (DTI) findings and clinical profiles in idiopathic normal-pressure hydrocephalus (INPH) patients, along with differences in DTI parameters between cerebrospinal fluid tap test (CSFTT) responders and non-responders. Fifty-four INPH patients constituted the final group for analysis. Fractional anisotropy (FA), axial diffusivity, radial diffusivity, and mean diffusivity were assessed using atlas-based tract-mapping methods on 20 different fiber tracts.

Evoked potentials are useful for diagnosis of neuromyelitis optica spectrum disorder

Keiko Ohnari, Kazumasa Okada, Toshiyuki Takahashi, Kosuke Mafune, Hiroaki Adachi

Neuromyelitis optica spectrum disorder (NMOSD) has been differentiated from relapsing-remitting multiple sclerosis (RRMS) by clinical, laboratory, and pathological findings, including the presence of the anti-aquaporin 4 antibody. Measurement of evoked potentials (EPs) is often used for the diagnosis of RRMS, although the possibility of applying EPs to the diagnosis of NMOSD has not been investigated in detail. Eighteen patients with NMOSD and 28 patients with RRMS were included in this study. The patients' neurological symptoms and signs were examined and their EPs were recorded.

A 12-month prospective, observational study evaluating the impact of disease-modifying treatment on emotional burden in recently-diagnosed multiple sclerosis patients: The POSIDONIA study

Enrico Montanari, Mariarosa Rottoli, Davide Maimone, Paolo Confalonieri, Katrin Plewnia, Maura Frigo, Ada Francia, Antonello Pala, Nunzia Alessandra Losignore, Paolo Ragonese, Antonella Veneziano, on behalf of the POSIDONIA study group

Depression and anxiety are common among patients with multiple sclerosis (MS) and are frequently present at the time of MS diagnosis.

Know thyself: Exploring interoceptive sensitivity in Parkinson's disease

Lucia Ricciardi, Gina Ferrazzano, Benedetta Demartini, Francesca Morgante, Roberto Erro, Christos Ganos, Kailash P. Bhatia, Alfredo Berardelli, Mark Edwards

Although Parkinson's disease (PD) is defined by its motor symptoms, it is now well recognised that cognitive, affective and emotion domains are also impaired. The pathophysiology of these disabling non-motor symptoms (NMS) remains unclear; recently the involvement of limbic areas, including the insula, in the neurodegenerative process has been suggested to have a key role. These areas, and the insula in particular, are also been suggested as key regions for interoception; interoceptive sensitivity (IS) is a measure of the accuracy of perception of sensations from inside the body related to the function of internal organs.

Genetic background of the hereditary spastic paraplegia phenotypes in Hungary — An analysis of 58 probands

Peter Balicza, Zoltan Grosz, Michael A. Gonzalez, Renata Bencsik, Klara Pentelenyi, Aniko Gal, Edina Varga, Peter Klivenyi, Julia Koller, Stephan Züchner, Judit Maria Molnar

Hereditary spastic paraplegias (HSPs) are a clinically and genetically heterogeneous group of neurodegenerative diseases with progressive lower limb spasticity and weakness. The aim of this study is to determine the frequency of different SPG mutations in Hungarian patients, and to provide further genotype-phenotype correlations for the known HSP causing genes.

Polymorphisms of small ubiquitin-related modifier genes are associated with risk of Alzheimer's disease in Korean: A case-control study

Myung-Jin Mun, Jin-Ho Kim, Ji-Young Choi, Min-Seon Kim, Won-Cheoul Jang, Jung Jae Lee, Young Lee Eun, Shang-June Kwak, Ki Woong Kim, Seok Bum Lee
  • DOI: http://dx.doi.org/10.1016/j.jns.2016.03.023
  • p122–127
  • Published online: March 19 2016
  • Open Access
  • Abstract
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Sumoylation regulates transcription factor transactivation, protein-protein interactions, and appropriate subcellular localization of certain proteins. Previous studies have shown that sumoylation of amyloid precursor protein (APP) is associated with decreased levels of amyloid beta (Aβ) proteins, suggesting that sumoylation may play a role in the pathogenesis of Alzheimer's disease (AD). We investigated the association between polymorphisms of the SUMO genes and the risk of AD. Our study subjects consisted of 144 AD patients and 335 healthy controls without dementia.                  

Depression in Tourette syndrome: A controlled and comparison study

John Carlo P. Piedad, Andrea E. Cavanna

Tourette syndrome (TS) is a neurodevelopmental condition characterised by multiple tics and co-morbid behavioural problems. Previous research found that up to 76% of patients with TS experience affective symptoms, with 13% fulfilling diagnostic criteria for depression.

Comparisons between Garcia, Modo, and Longa rodent stroke scales: Optimizing resource allocation in rat models of focal middle cerebral artery occlusion

Salam P. Bachour, Mario Hevesi, Ornina Bachour, Brian M. Sweis, Javad Mahmoudi, Julia A. Brekke, Afshin A. Divani

The use of rodent stroke models allow for the understanding of stroke pathophysiology. There is currently no gold standard neurological assessment to measure deficits and recovery from stroke in rodent models. Agreement on a universal preclinical stroke scale allows for comparison of the outcomes among conducted studies. The present study aimed to compare three routinely used neurological assessments in rodent studies (i.e., Garcia, Modo, and Longa) to determine which is most effective for accurately and consistently quantifying neurological deficits in the context of focal middle cerebral artery occlusion (MCAo) in rats. 

Subjective sleep problems in Huntington's disease: A pilot investigation of the relationship to brain structure, neurocognitive, and neuropsychiatric function

Chaya Rochel Baker, Juan F. Domínguez D, Julie C. Stout, Sanaz Gabery, Andrew Churchyard, Phyllis Chua, Gary F. Egan, Åsa Petersén, Nellie Georgiou-Karistianis, Govinda R. Poudel

Subjective reports of sleep disturbance are a common feature of Huntington's disease (HD); however, there is limited research investigating the relationship between sleep problems with changes in brain and behaviour. This study aimed to investigate whether subjective reports of sleep problems in HD are associated with brain volume, neurocognitive decline, and neuropsychiatric symptoms. This retrospective pilot study used brain volume, neurocognitive and neuropsychiatric data from premanifest (pre-HD) and symptomatic HD (symp-HD).

Sonographic evaluation of peripheral nerves in subtypes of Guillain-Barré syndrome

Atsuko Mori, Hiroyuki Nodera, Naoko Takamatsu, Keiko Maruyama-Saladini, Yusuke Osaki, Yoshimitsu Shimatani, Ryuji Kaji

Sonography of peripheral nerves can depict alteration of nerve sizes that could reflect inflammation and edema in inflammatory and demyelinating neuropathies. Guillain-Barré syndrome (GBS). Information on sonographic comparison of an axonal subtype (acute motor [and sensory] axonal neuropathy [AMAN and AMSAN]) and a demyelinating subtype (acute inflammatory demyelinating polyneuropathy [AIDP]) has been sparse.

Stent-assisted coiling and balloon-assisted coiling in the management of intracranial aneurysms: A systematic review & meta-analysis

Fei Wang, Xun Chen, Yong Wang, Peng Bai, Huan-zhi Wang, Tao Sun, Hua-lin Yu

Stent-assisted coiling and balloon-assisted coiling are well-established minimally invasive techniques for treatment of intracranial aneurysms. The aim of this study was to use meta-analysis methods to compare clinical outcomes of aneurysms treated with stent-assisted coiling versus balloon-assisted coiling.

Recreational marijuana use and acute ischemic stroke: A population-based analysis of hospitalized patients in the United States

Kavelin Rumalla, Adithi Y. Reddy, Manoj K. Mittal

Recreational marijuana use is considered to have few adverse effects. However, recent evidence has suggested that it precipitates cardiovascular and cerebrovascular events. Here, we investigated the relationship between marijuana use and hospitalization for acute ischemic stroke (AIS) using data from the largest inpatient database in the United States.


Clinical Short Communications

Substantia nigra hyperechogenicity does not correlate with motor features in Parkinson's disease

Joana Jesus-Ribeiro, João Sargento-Freitas, Mário Sousa, Fernando Silva, António Freire, Cristina Januário

The evaluation of hyperechogenicity of the substantia nigra (SN) by transcranial sonography (TCS) is validated for the diagnosis of Parkinson's disease (PD). However, its correlation with the severity of motor involvement is still uncertain.

Auras in patients with temporal lobe epilepsy and mesial temporal sclerosis

Ali A. Asadi-Pooya, Maromi Nei, Ashwini Sharan, Michael R. Sperling

We investigated auras in patients with drug-resistant temporal lobe epilepsy (TLE) and mesial temporal sclerosis (MTS). We also investigated the clinical differences between patients with MTS and abdominal auras and those with MTS and non-mesial temporal auras. All patients with drug-resistant TLE and unilateral MTS who underwent epilepsy surgery at Jefferson Comprehensive Epilepsy Center from 1986 through 2014 were evaluated. Patients with good postoperative seizure outcome were investigated. One hundred forty-nine patients (71 males and 78 females) were studied.  

Blinded search for varicella zoster virus in giant cell arteritis (GCA)-positive and GCA-negative temporal arteries

Don Gilden, Teresa White, Nelly Khmeleva, Bradley J. Katz, Maria A. Nagel

Recent analysis of archived temporal arteries (TAs) acquired from 13 pathology laboratories in the US, Canada, Iceland, France, Germany and Israel from patients with pathologically-verified giant cell arteritis (GCA-positive) and TAs from patients with clinical features and laboratory abnormalities of GCA but whose TAs were pathologically negative (GCA-negative) revealed VZV antigen in most TAs from both groups. Despite formalin-fixation, VZV DNA was also found in many VZV-antigen positive sections that were scraped, subjected to DNA extraction, and examined by PCR with VZV-specific primers.

Long-term assessment of No Evidence of Disease Activity with natalizumab in relapsing multiple sclerosis

Luca Prosperini, Fulvia Fanelli, Carlo Pozzilli

In this study we assessed the proportion of patients with relapsing multiple sclerosis (R-MS) who had No Evidence of Disease Activity (NEDA-3), defined as absence of relapses, absence of confirmed disability worsening, and absence of radiological activity (detected by magnetic resonance imaging of the brain and spinal cord) up to 7 years after starting natalizumab. Out of 152 patients considered, 58 were still on treatment and 94 discontinued treatment after a median time of 3 years. According to an intention-to-treat approach, 52 (34%) patients maintained the NEDA status at the end of follow-up.

Malignant cerebral edema related to Systemic Lupus Erythematosus

Lauren Koffman, Richard Prayson, E.M. Manno

The neurological manifestations of Systemic Lupus Erythematosus (SLE) are varied and incompletely described. A few case series report a benign idiopathic intracranial hypertension (IIH) related to SLE, which is responsive to immunotherapy. There are limited reports of patients with malignant cerebral edema, and diffuse white matter changes in the absence of central nervous system (CNS) vasculitis.

What daily activities increase the risk of falling in Parkinson patients? An analysis of the utility of the ABC-16 scale

Chayanin Foongsathaporn, Pattamon Panyakaew, Onanong Jitkritsadakul, Roongroj Bhidayasiri

Although the strongest predictor of falling in Parkinson's disease is the number of falls in the preceding year, little information is available on what types of daily activities (ADLs) that are associated with a significant fall risk in this population.


Letters to the Editor

Clinical and radiological CLIPPERS features after complete remission of peripheral T-cell lymphoma, not otherwise specified

Ryota Nakamura, Yuji Ueno, Jun Ando, Hironari Matsuda, Azuchi Masuda, Kazuhide Iiduka, Naoki Shingai, Masashi Takanashi, Kazumasa Yokoyama, Norio Komatsu, Nobutaka Hattori

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently reported inflammatory disease of the central nervous system (CNS) [1–2]. Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is an uncommon form of non-Hodgkin lymphoma [3]. We report the case of a patient who developed CLIPPERS within 1 year of remission of PTCL-NOS after chemotherapy.

Neck flexion-induced finger tremor: A novel observation in Hirayama disease

Georgios Koutsis, Georgios Velonakis, Marianthi Breza, Efstratios Karavasilis, Thomas Zambelis, Marios Panas

Hirayama disease, also known as juvenile asymmetric segmental spinal muscular atrophy, is a rare, self-limiting, cervical myelopathy predominantly affecting adolescent males. Its main feature is focal upper limb amyotrophy in the distribution of C7, C8, and T1- innervated muscles. It is usually monomelic, but bilateral affection, occasionally symmetric, has also been described [1–5]. An irregular tremor (minipolymyoclonus), present when outstretching the hands, has been reported in the context of Hirayama disease [1–6].

Gerstmann-Sträussler-Scheinker syndrome in an Argentinean family due to mutationat codon 117 of the Prion Protein Gene (PrPA117V)

Michel Saenz-Farret, Carolina Candelaria Ramirez-Gomez, Natalia Araoz-Olivos, Heidi Carrillo-Canedo, Victoria Aldinio, Veronica Gisela Montilla-Uzcategui, Marcelo Kauffman, Federico Micheli

Prion Diseases or Transmissible Spongiform Encephalopathies (TSEs) constitute rare neurodegenerative diseases, the most common being Creutzfeldt–Jakob disease (CJD). Fifteen percent (15%) of the cases worldwide are considered to be of the familial type and the remainder (85%) present as a sporadic disorder (sCJD) [1]. The familial (or genetic) type includes: genetic CJD (gCJD), fatal familial insomnia (FFI), and Gerstmann–Sträussler–Scheinker syndrome (GSS) [2]. Here we present the clinical findings of an Argentinean family with GSS due to mutation at codon 117 of the prion protein gene (PrPA117V), the first in Argentina and in Latin America.

Life-threatening ADEM in an immunocompetent pregnant woman with concomitant asymptomatic Cytomegalovirus infection

A. Macerollo, L. Dalfino, N. Brienza, N. Campobasso, G. Pertosa, F. Dicuonzo, I.L. Simone, C. Tortorella

The acute disseminated encephalomyelitis (ADEM) is an acute inflammatory demyelinating disease affecting the central nervous system [1]. A monophasic abnormal autoimmune reaction directed against normal brain tissue has been proposed as the primary pathogenesis. Here, we present a life-threatening case of ADEM in a pregnant woman.

Intravenous epoprostenol for treatment-refractory reversible cerebral vasoconstriction syndrome (RCVS)

Anya L. Thydén, Ali A. Muhamad, Anne Jacobsen, Daniel Kondziella

Resolution of periodic alternating nystagmus with amantadine

Seung-Han Lee, Sae-Young Lee, Seong-Min Choi, Byeong.C. Kim, Myeong-Kyu Kim, Ji-Soo Kim

Periodic alternating nystagmus (PAN) refers to a horizontal jerk nystagmus that reverses its direction every 90–120 s with a brief transition period [1]. PAN is explained by an unstable velocity-storage mechanism due to loss of cerebellar inhibition, but with an intact adaptive mechanism that normally acts to null any bias that had induced a sustained vestibular nystagmus [1]. Baclofen, a GABA agonist, potentiates the nodulo-uvular inhibition over the velocity storage system, and has been the drug of choice in PAN [1].

Rhabdomyolysis as a late complication of bariatric surgery

Louise Anne Rigney, Carlos El-Haddad, Cecilia Cappelen-Smith, Roger Pamphlett, Ian Gotis-Graham, Dennis J. Cordato

The number of patients undergoing weight loss surgery in Western countries has dramatically increased during the past 20 years [1].

Immune-mediated spastic paraparesis accompanied with high titres of voltage-gated potassium channel complex antibodies and myokymia/fasciculation

Ban-yu Saitoh, Shintaro Hayashi, Katsuya Ogata, Taira Uehara, Hikaru Doi, Osamu Watanabe, Ryo Yamasaki, Hiroyuki Murai, Jun-ichi Kira

The clinical spectrum of anti-voltage gated potassium channel (VGKC) complex antibody-mediated autoimmunity has been known to be broad and affect both the peripheral and central nervous systems [1]. Among these, myelopathy occurs in 2.8% of cases; however, no previously reported cases had high titres of anti-VGKC complex antibodies [2]. We herein report a case of immunotherapy-responsive spastic paraparesis with myokymia/fasciculation accompanied with extremely high titres of anti-VGKC complex antibodies.

Primary AL amyloidosis presenting as lower motor neuron disease

Roberto Fancellu, Paolo Buzzo, Davide Faga, Mauro Morena, Omar Racchi, Sandro Salvarani, Pierangelo Scotto

Primary systemic AL amyloidosis is a hematological disease characterized by diffuse deposition of amyloid fibrils derived from immunoglobulin light chains, mainly in heart, kidney, liver, gastrointestinal tract, and peripheral nervous system. Axonal sensory and autonomic polyneuropathy is associated with AL amyloidosis in up to 35% of cases [1,2]. Atypical presentations of neuropathy include multiple mononeuropathies, chronic progressive autonomic failure, and chronic inflammatory demyelinating neuropathy [3].

Letter to the Editor regarding the paper by Sun G et al: Elevated serum levels of neutrophil elastase in patients with influenza virus-associated encephalopathy. J Neuro Sci 2015;349:190–195

Nobuyuki Nosaka, Kazuki Hatayama, Yousuke Fujii, Masato Yashiro, Hirokazu Tsukahara, Tsuneo Morishima

We read with great interest the discussion of possible therapeutic potential of anti-elastase agents against influenza virus-associated encephalopathy (IE) in the clinical research paper by Sun et al. [1]. Recently, we had an opportunity to examine the kinetics of serum neutrophil elastase (NE) in the acute clinical course of three patients, aged 12 to 14 months, who were diagnosed with human herpes virus 6 (HHV6)-associated acute encephalopathy with febrile convulsive status epilepticus (AEFCSE), one of whom had moderate neurological sequelae with secondary epilepsy and developmental retardation.      

Acute vestibulocochlear neuritis following postexposure rabies prophylaxis

Hasan Tahsin Gozdas, Abdulkerim Gokoglu

The recent article by Mesquita et al. [1] published in this journal is very interesting. Their case developed acute bilateral vestibulocochlear neuritis following cultured cell vaccine administration for rabies postexposure prophylaxis. However, we have some comments regarding this article.

Acute bilateral vestibulocochlear neuritis following cultured cell vaccine administration for rabies post-exposure prophylaxis

Emersom Cicilini Mesquita, Carolina Rouanet, Gilberto Luciano Lucas, Alberto dos Santos de Lemos, Marco Antonio Lima

Rabies is a zoonotic viral disease transmitted only in mammals, caused by RNA virus of the Family Rhabdoviridae, Genus Lyssavirus. The disease occurs in almost all continents and is responsible for approximately 55,000 to 70,000 deaths/year worldwide [1]. In Brazil, rabies is a public health concern with a total of 574 cases registered from 1990 to 2014 [2].



Corrigendum to "Age-related changes of inactivating BK channels in rat dorsal root ganglion neurons" [J. Neurol. Sci 358 (1–2) (November 15 2015) 138–145]

Weiwei Yu, Xianguang Lin, Shangbang Gao, Chenhong Li

The authors of the above paper have noticed errors in Fig. 2. The corrections and the amended figure are below.