JNS-cover.jpgJournal of Neurological Sciences

Vol 348 | No. 1-2 | 15 January 2015 | Pages 1-294


Inherited leukoencephalopathies with clinical onset in middle and old age

Serena Nannucci, Ida Donnini, Leonardo Pantoni

The currently widespread use of neuroimaging has led neurologists to often face the problem of the differential diagnosis of white matter diseases. There are various forms of leukoencephalopathies (vascular, inflammatory and immunomediated, infectious, metabolic, neoplastic) and sometimes white matter lesions are expression of a genetic disease. While many inherited leukoencephalopathies fall in the child neurologist's interest, others may have a delayed or even a typical onset in the middle or old age.

Hereditary motor and sensory neuropathies or Charcot–Marie–Tooth diseases: An update

Meriem Tazir, Tarik Hamadouche, Sonia Nouioua, Stephane Mathis, Jean-Michel Vallat

Hereditary motor and sensory neuropathies (HMSN) or Charcot–Marie–Tooth (CMT) diseases are the most common degenerative disorders of the peripheral nervous system. However, the frequency of the different subtypes varies within distinct populations. Although more than seventy clinical and genetic forms are known to date, more than 80% of CMT patients in Western countries have genetic abnormalities associated with PMP22, MPZ, MFN2 and GJB1. Given the considerable genetic heterogeneity of CMT, we emphasize the interest of both clinical and pathological specific features such that focused genetic testing could be performed.

Vascular comorbidities in the onset and progression of multiple sclerosis

Prudence Tettey, Steve Simpson, Bruce V. Taylor, Ingrid A.F. van der Mei

Vascular comorbidities are common in the general population and are associated with adverse health outcomes. In people with multiple sclerosis (MS), an increasing amount of evidence suggests that vascular comorbidities are also common, but an association with MS risk and disability has not been conclusively established. This review aims to critically examine published data on the relationship between vascular comorbidities (including vascular risk factors) and MS. The evidence suggests an increased risk of MS in people with a high BMI during childhood or adolescence but not adulthood.

A novel treatment target for Parkinson's disease

Chandramohan Wakade, Raymond Chong

We hypothesize that GPR109A message and expression are up-regulated in individuals with Parkinson's disease (PD). GPR109A is a high-affinity niacin receptor. Niacin is a precursor for NAD–NADH which is needed for dopamine production. Thus, niacin supplementation may serve three purposes: reduce inflammation through GPR109A-related mechanisms, increase dopamine synthesis in the striatum through NADPH supply and increase NAD/NADH ratio to boost mitochondrial functions. GPR109A and its agonists are known to exert anti-inflammatory actions in the skin, gut and retina.


Original Articles

Can botulinum toxin type A injection technique influence the clinical outcome of patients with post-stroke upper limb spasticity? A randomized controlled trial comparing manual needle placement and ultrasound-guided injection techniques

Andrea Santamato, Maria Francesca Micello, Francesco Panza, Francesca Fortunato, Alessio Baricich, Carlo Cisari, Alberto Pilotto, Giancarlo Logroscino, Pietro Fiore, Maurizio Ranieri

Botulinum toxin type A is a first-line treatment for post-stroke focal spasticity, and the accuracy in delivering the toxin to the target muscles may influence the treatment outcome. Our aim was to compare the reduction of spasticity and the related finger position at rest improvement in post-stroke patients treated with botulinum toxin type A in upper limb muscles using ultrasound guidance and manual needle placement.

Ultrasound of the peripheral nerves in systemic vasculitic neuropathies

Alexander Grimm, Bernhard F. Décard, Antje Bischof, Hubertus Axer

Ultrasound of the peripheral nerves (PNUS) can be used to visualize nerve pathologies in polyneuropathies (PNP). The aim of this study was to investigate, whether PNUS provides additional information in patients with proven systemic vasculitic neuropathies (VN).

Parahippocampal gray matter alterations in Spinocerebellar Ataxia Type 2 identified by voxel based morphometry

Roberto E. Mercadillo, Víctor Galvez, Rosalinda Díaz, Carlos Roberto Hernández-Castillo, Aurelio Campos-Romo, Marie-Catherine Boll, Erick H. Pasaye, Juan Fernandez-Ruiz

Spinocerebellar Ataxia Type 2 (SCA2) is a genetic disorder causing cerebellar degeneration that result in motor and cognitive alterations. Voxel-based morphometry (VBM) analyses have found neurodegenerative patterns associated to SCA2, but they show some discrepancies. Moreover, behavioral deficits related to non-cerebellar functions are scarcely discussed in those reports. In this work we use behavioral and cognitive tests and VBM to identify and confirm cognitive and gray matter alterations in SCA2 patients compared with control subjects.

A multi-disciplinary model of risk factors for fatal outcome in posterior reversible encephalopathy syndrome

Lea M. Alhilali, Arich R. Reynolds, Saeed Fakhran

To evaluate the relative impact of clinical data, imaging findings, and CSF laboratory values on clinical outcome in patients with posterior reversible encephalopathy syndrome (PRES).

Effects of donepezil, an acetylcholinesterase inhibitor, on neurogenesis in a rat model of vascular dementia

Kyoung Ja Kwon, Min Kyeong Kim, Eun Joo Lee, Jung Nam Kim, Bo-Ryoung Choi, Soo Young Kim, Kyu Suk Cho, Jung-Soo Han, Hahn Young Kim, Chan Young Shin, Seol-Heui Han

Vascular dementia (VaD) is the second most common form of dementia caused by cerebrovascular disease. Several recent reports demonstrated that cholinergic deficits are implicated in the pathogenesis of VaD and that cholinergic therapies have shown improvement of cognitive function in patients with VaD. However, the precise mechanisms by which donepezil achieves its effects on VaD are not fully understood. Donepezil hydrochloride is an acetylcholinesterase inhibitor (AChEI) currently used for the symptomatic treatment of Alzheimer's disease (AD).   

Evaluation of the humoral response against mycobacterial peptides, homologous to MOG35–55, in multiple sclerosis patients

Davide Cossu, Giuseppe Mameli, Speranza Masala, Eleonora Cocco, Jessica Frau, Maria Giovanna Marrosu, Leonardo Antonio Sechi

Bacillus Calmette-Guérin (BCG) and Mycobacterium avium subspecies paratuberculosis (MAP) have been associated with multiple sclerosis (MS). Clinical data indicates that BCG vaccination exerts anti-inflammatory effects in MS; conversely, MAP is thought to be one of the possible infectious factors responsible of MS through a molecular mimicry mechanism. A peptide-based indirect ELISA was used to detect antibodies against the encephalitogenic myelin oligodendrocyte glycoprotein (MOG)35–55 epitope, and two mycobacterial peptides sharing sequence homology with the latter: MAP_2619c352–361/BCG_1224355–364 and BCG_3329c64–74.    

Cannabidiol and endogenous opioid peptide-mediated mechanisms modulate antinociception induced by transcutaneous electrostimulation of the peripheral nervous system

Thais Cristina Teixeira Gonçalves, Anna Karla Londe, Rafael Isaac Pires Albano, Artur Teixeira de Araújo Júnior, Mariana de Aguiar Azeredo, Audrey Francisco Biagioni, Thiago Henrique Ferreira Vasconcellos, Célio Marcos dos Reis Ferreira, Dulcinéa Gonçalves Teixeira, José Alexandre de Souza Crippa, Débora Vieira, Norberto Cysne Coimbra

Transcutaneous electrical nerve stimulation (TENS) is a non-pharmacological therapy for the treatment of pain. The present work investigated the effect of cannabidiol, naloxone and diazepam in combination with 10 Hz and 150 Hz TENS. Male Wistar rats were submitted to the tail-flick test (baseline), and each rodent received an acute administration (intraperitoneal) of naloxone (3.0 mg/kg), diazepam (1.5 mg/kg) or cannabidiol (0.75 mg/kg, 1.5 mg/kg, 3.0 mg/kg, 4.5 mg/kg, 6.0 mg/kg and 12.0 mg/kg); 10 min after the acute administration, 10 Hz or 150 Hz TENS or a sham procedure was performed for 30 min.       

Evidence for peripheral immune activation in amyotrophic lateral sclerosis

Xueping Chen, Weihua Feng, Rui Huang, Xiaoyan Guo, Yongping Chen, Zhenzhen Zheng, Huifang Shang

There is evidence of the activity of immune system in the spinal cords of patients with amyotrophic lateral sclerosis (ALS), however; few studies to date have explored the status of peripheral immune response in ALS patients. Blood samples from 284 ALS patients and 217 aged-match controls were evaluated, and parameters of T cell subset, humoral immunity, and complement system activation were observed. CD4 + T lymphocytes and circulating immune complexes (CICs) were significantly decreased, and component C3 was significantly increased in ALS patients compared with normal controls.

Laboratory diagnosis of Lyme neuroborreliosis is influenced by the test used: Comparison of two ELISAs, immunoblot and CXCL13 testing

Nora Wutte, Juan Archelos, Brian A. Crowe, Werner Zenz, Elisabeth Daghofer, Franz Fazekas, Elisabeth Aberer

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.027
  • p96–103
  • Published online: September 25, 2014
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To compare Borrelia-specific intrathecal antibodies by two different ELISAs, an immunoblot (IB) and CXCL13.

C9orf72 hexanucleotide repeat expansion analysis in Chinese spastic paraplegia patients

Yingying Luo, Bin Jiao, Junling Wang, Juan Du, Xinxiang Yan, Kun Xia, Beisha Tang, Lu Shen

Recently, a hexanucleotide repeat expansion in the C9orf72 gene has been identified to cause frontotemporal dementia, amyotrophic lateral sclerosis families and many other neurodegenerative diseases. Owing to the overlapping phenotypes among HSP, frontotemporal dementia and amyotrophic lateral sclerosis we hypothesized that C9orf72 expansions might be a genetic risk factor or modifier of HSP.

Lateralization of temporal lobe epilepsy by multimodal multinomial hippocampal response-driven models

Mohammad-Reza Nazem-Zadeh, Kost V. Elisevich, Jason M. Schwalb, Hassan Bagher-Ebadian, Fariborz Mahmoudi, Hamid Soltanian-Zadeh

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.029
  • p107–118
  • Published online: September 26, 2014
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Multiple modalities are used in determining laterality in mesial temporal lobe epilepsy (mTLE). It is unclear how much different imaging modalities should be weighted in decision-making. The purpose of this study is to develop response-driven multimodal multinomial models for lateralization of epileptogenicity in mTLE patients based upon imaging features in order to maximize the accuracy of noninvasive studies.

3-week whole body vibration does not improve gait function in mildly affected multiple sclerosis patients—a randomized controlled trial

Thomas Wolfsegger, Hamid Assar, Raffi Topakian

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.030
  • p119–123
  • Published online: September 25, 2014
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To investigate the effects of whole body vibration (WBV) training on gait function in persons with mild multiple sclerosis (MS).

Profile of memory impairment as a prognostic marker in amnestic mild cognitive impairment

Manyong Lee, Tae Sung Lim, Hyun Young Lee, So Young Moon

We aimed to evaluate whether recognition memory can be used to identify patients with amnestic mild cognitive impairment (aMCI) at greater risk for converting to dementia. We recruited 2172 aMCI patients. They were divided into two groups: aMCI with impaired recall but normal recognition (aMCI-IRNR) vs aMCI with impaired recall and impaired recognition (aMCI-IRIR). We compared demographic findings and neuropsychological performance and illustrated the difference in converting to dementia between the two groups.    

Sarcoid neuropathy: Correlation of nerve ultrasound, electrophysiological and clinical findings

A. Kerasnoudis, D. Woitalla, R. Gold, K. Pitarokoili, M.-S. Yoon

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.033
  • p129–136
  • Published online: September 27, 2014
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We present the nerve ultrasound findings in sarcoid neuropathy and examine their correlation with electrophysiology and functional disability.

Lefty inhibits glioma growth by suppressing Nodal-activated Smad and ERK1/2 pathways

Guan Sun, Lei Shi, Min Li, Nan Jiang, Linshan Fu, Jun Guo

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.034
  • p137–142
  • Published online: September 27, 2014
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As a common malignant tumor, glioma has long been a refractory disease in the field of neurosurgery. Exploration of its etiology, pathogenesis, biological characteristics, and new treatment measures is a hot topic as well as a problem most difficult to solve in the neurosurgical division. In recent years, the role of Nodal and its feedback inhibitor Lefty in tumors has drawn more and more attention and may form a new target for cancer therapy. Western blot detection indicated that there was almost no expression of Lefty protein in glioma cells.

Reduction of Intraepidermal Nerve Fiber Density (IENFD) in the skin biopsies of patients with fibromyalgia: A controlled study

Michalis L. Kosmidis, Loukia Koutsogeorgopoulou, Harry Alexopoulos, Ioanna Mamali, Panagiotis G. Vlachoyiannopoulos, Michalis Voulgarelis, Haralampos M. Moutsopoulos, Athanasios G. Tzioufas, Marinos C. Dalakas

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.035
  • p143–147
  • Published online: September 27, 2014
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Fibromyalgia (FM) is one of the most common chronic pain syndromes. Various pathogenetic mechanisms have been implicated but none is proven. Our scope was to determine if Intraepidermal Nerve Fiber Density (IENFD) is reduced in the skin of FM patients, as observed in patients with painful small fiber sensory neuropathy (SFSN).

Effects of tissue plasminogen activator timing on blood–brain barrier permeability and hemorrhagic transformation in rats with transient ischemic stroke

Yanrong Zhang, Yi Wang, Zhiyi Zuo, Zhongxing Wang, Jack Roy, Qinghua Hou, Elizabeth Tong, Angelika Hoffmann, Emily Sperberg, Joerg Bredno, Stuart S. Berr, Mingxing Xie, Kevin Lee, Max Wintermark

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.036
  • p148–154
  • Published online: September 27, 2014
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The goal of our study was to determine if the timing of the tissue plasminogen activator (tPA) administration influenced its effect on blood–brain barrier (BBB) permeability and the subsequent risk of hemorrhagic transformation. Thirty spontaneously hypertensive male rats were subjected to a 90-minute unilateral middle cerebral artery occlusion. Six rats did not receive tPA treatment (vehicle control: Group 0), intravenous tPA was administered immediately after reperfusion (Group 1) or 4 h after reperfusion (Group 2).                               

Prospective induction of peripheral neuropathy by the use of Tartarian Buckwheat

Fei Yang, Sheng-yuan Yu, Yan Wang, Rong-fei Wang, Feng Jing

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.037
  • p155–158
  • Published online: September 29, 2014
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Tartarian Buckwheat is an effective hypoglycemic medicinal herb. Its main active ingredients are flavonoids. We report here 5 cases of new onset polyneuropathy with dyskinesia prospectively induced by tartarian buckwheat products. Clinical and electrophysiological evidence along with laboratory tests were reviewed and analyzed. All patients were male, with an average age of 52.2 ± 10.9 years old (range: 40–66 years) and had a recent history of using tartarian buckwheat for diabetes therapy. The average time of use was 2.5 ± 1.0 months (range: 1.5–4 months).    

Poststroke depression and its multifactorial nature: Results from a prospective longitudinal study

A. De Ryck, E. Fransen, R. Brouns, M. Geurden, D. Peij, P. Mariën, P.P. De Deyn, S. Engelborghs

Poststroke depression (PSD) is commonly observed in stroke patients and has a negative impact on functional outcome and quality of life. Therefore, a prospective, longitudinal epidemiological study was conducted aiming to determine prevalence and risk factors for PSD at 1, 3, 6, 12 and 18 months poststroke.

EMG coherence and spectral analysis in cervical dystonia: Discriminative tools to identify dystonic muscles?

S.W.R. Nijmeijer, E. de Bruijn, P.A. Forbes, D.J. Kamphuis, R. Happee, J.H.T.M. Koelman, M.A.J. Tijssen

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.041
  • p167–173
  • Published online: September 30, 2014
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Botulinum toxin injections in the dystonic muscles are the preferred treatment for cervical dystonia (CD), but proper selection of the dystonic muscles remains a challenge. We investigated the use of EMG coherence and autospectral analysis as discriminative tools to identify dystonic muscles in CD patients.

Semi-quantitative data on ethanol consumption in 354 ET cases and 370 controls

Elan D. Louis, Monika Michalec

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.042
  • p174–178
  • Published online: September 29, 2014
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The notion that there is an association between essential tremor (ET) and higher ethanol consumption has crept into the literature; however, the data are limited and conflicted. A total of 354 ET cases and 370 matched controls were enrolled in a clinical-epidemiological study. Average current daily ethanol consumption was estimated using the Willett Semi-quantitative Food Frequency Questionnaire. The proportion of cases and controls who drank any ethanol was similar: 66.7% vs. 64.1%, p = 0.46, as was the proportion who reported heavy ethanol consumption: 4.0% vs.                

Effects of ischemic preconditioning on VEGF and pFlk-1 immunoreactivities in the gerbil ischemic hippocampus after transient cerebral ischemia

Yoo Seok Park, Jun Hwi Cho, In Hye Kim, Geum-Sil Cho, Jeong-Hwi Cho, Joon Ha Park, Ji Hyeon Ahn, Bai Hui Chen, Bich-Na Shin, Myoung Cheol Shin, Hyun-Jin Tae, Young Shin Cho, Yun Lyul Lee, Young-Myeong Kim, Moo-Ho Won, Jae-Chul Lee

Ischemia preconditioning (IPC) displays an important adaptation of the CNS to sub-lethal ischemia. In the present study, we examined the effect of IPC on immunoreactivities of VEGF-, and phospho-Flk-1 (pFlk-1) following transient cerebral ischemia in gerbils. The animals were randomly assigned to four groups (sham-operated-group, ischemia-operated-group, IPC plus (+) sham-operated-group, and IPC + ischemia-operated-group). IPC was induced by subjecting gerbils to 2 min of ischemia followed by 1 day of recovery.              

Under-reporting of Parkinson's disease on death certificates: A population-based study (NEDICES)

Julián Benito-León, Elan D. Louis, Alberto Villarejo-Galende, Juan Pablo Romero, Félix Bermejo-Pareja

Parkinson's disease is frequently omitted as a cause of death from death certificates. A limitation of previous studies that attempted to assess the validity of death certificates is that population-dwelling cases, with milder, undiagnosed Parkinson's disease were likely excluded. As a result, those studies likely overestimated the validity of death certificates because they did not include these milder cases. We assessed the validity of death certificates in a prospective population-based study (NEDICES), which includes previously undiagnosed Parkinson's disease cases detected during the assessment.

Altered spontaneous activity in the default-mode network and cognitive decline in chronic subcortical stroke

Jingchun Liu, Wen Qin, Hong Wang, Jing Zhang, Rong Xue, Xuejun Zhang, Chunshui Yu

The resting-state functional connectivity (rsFC) has been reported to be impaired in the default-mode network (DMN) in stroke patients. However, it remains unclear whether the regional homogeneity (ReHo) of spontaneous activity and gray matter volume (GMV) are also altered in the DMN in these patients. Here we investigated ReHo, rsFC and GMV changes in the DMN and their functional correlations in stroke patients.

miRNA profiles in cerebrospinal fluid from patients with central hypersomnias

Anja Holm, Claus Heiner Bang-Berthelsen, Stine Knudsen, Signe Modvig, Birgitte Rahbek Kornum, Steen Gammeltoft, Poul J. Jennum

MicroRNAs (miRNAs) are involved in the pathogenesis of many human diseases, including some neurological disorders. Recently, we have reported dysregulated miRNAs in plasma from patients with central hypersomnias including type 1 and type 2 narcolepsy, and idiopathic hypersomnia. This study addressed whether miRNA levels are altered in the cerebrospinal fluid (CSF) of patients with central hypersomnias. We conducted high-throughput analyses of miRNAs in CSF from patients using quantitative real-time polymerase chain reaction panels.

Early infarct growth predicts long-term clinical outcome in ischemic stroke

Seung Min Kim, Sun U. Kwon, Jong S. Kim, Dong-Wha Kang

Ischemic lesions dynamically evolve during the acute phase of stroke. Although the ischemic lesion volume has been considered as a predictor of clinical outcome, it is still controversial whether early changes in ischemic lesion have prognostic information in addition to clinical variables. We hypothesized that early infarct growth on diffusion-weighted imaging (DWI) might be independently associated with long-term outcome in acute ischemic stroke patients.

Analysis among cognitive profiles and gray matter volume in newly diagnosed Parkinson's disease with mild cognitive impairment

Sang Woo Noh, Yong Hee Han, Chi Woong Mun, Eun Joo Chung, Eung Gyu Kim, Ki Hwan Ji, Jung Hwa Seo, Sang Jin Kim

To analyze the characteristics of neuropsychological profiles and gray matter volume in de novo, drug-naïve Parkinson's disease with mild cognitive impairment (PD-MCI).

Iron Metabolism in Neuromyelitis Optica Patients

Lei Zhang, Zhongsi Hong, Xiaoyu Chen, Sha Tan, Yinyao Lin, Yanqiang Wang, Shaoyang Sun, Wei Cai, Zhengqi Lu

The aim of this study was to investigate whether there was any relationship between iron metabolism and clinical features of NMO patients.

Impact of tetrabenazine on gait and functional mobility in individuals with Huntington's disease

Deb A. Kegelmeyer, Anne D. Kloos, Nora E. Fritz, Marianne M. Fiumedora, Susan E. White, Sandra K. Kostyk

Chorea may contribute to balance problems and walking difficulties that lead to higher fall rates in individuals with Huntington's disease (HD). Few studies have examined the effects of tetrabenazine (TBZ), an anti-choreic drug, on function and mobility in HD. The purpose of this study was to compare: 1) gait measures in forward walking, 2) balance and mobility measures, and 3) hand and forearm function measures on and off TBZ. We hypothesized that use of TBZ would improve gait, transfers and hand and forearm function.            

Predictors of development of chronic vestibular insufficiency after vestibular neuritis

Ivan Adamec, Magdalena Krbot Skorić, David Ozretić, Mario Habek

To evaluate the role of clinical parameters, MRI and ocular VEMP (oVEMP) and cervical VEMP (cVEMP) as predictors of development of chronic vestibular insufficiency after vestibular neuritis.

Relationship between gray matter volume and cognitive learning in CIS patients on disease-modifying treatment

Tomas Uher, Ralph H.B. Benedict, Dana Horakova, Niels Bergsland, Jana Blahova Dusankova, Michaela Tyblova, Deepa P. Ramasamy, Zdenek Seidl, Manuela Vaneckova, Jan Krasensky, Eva Havrdova, Robert Zivadinov

Supplemental Materials

Repeated administration of Paced Auditory Serial Addition Test (PASAT) results in a considerable learning effect in short- or long-term follow-up studies. However, the relationship between PASAT learning and changes in magnetic resonance imaging (MRI) parameters is yet to be investigated.

Comparison of hemihypoglossal- and accessory-facial neurorrhaphy for treating facial paralysis in rats

Dezhi Li, Hong Wan, Jie Feng, Shiwei Wang, Diya Su, Shuyu Hao, Michael Schumacher, Song Liu

The aim of this study was to determine the effectiveness of hypoglossal-facial nerve "side"-to-end (HemiHN–FN) and accessory-facial nerve end-to-end (AN–FN) neurorrhaphy using a predegenerated nerve graft (PNG) for reanimating facial paralysis in a rat FN injury model. A total of 25 rats with complete unilateral facial paralysis resulting from section of the right FN were divided into 5 groups (n = 5 each) that were submitted to immediate, delayed (3 months after FN injury) or no (control) FN reconstruction procedures involving HemiHN–FN or AN–FN neurorrhaphy. 

Carotid inflammation on 18F-fluorodeoxyglucose positron emission tomography associates with recurrent ischemic lesions

Hye-Jin Kim, Minyoung Oh, Dae Hyuk Moon, Kyung-Ho Yu, Sun U. Kwon, Jong S. Kim, Dong-Wha Kang

Supplemental Materials

Inflammation plays an important role in the development of ischemic stroke. We hypothesized that inflammation of carotid atherosclerosis, as measured by 18F-fluorodeoxyglucose (FDG) uptake on positron emission tomography (PET), associates with the early recurrent ischemic lesions (ERILs).

Information processing speed impairment and cerebellar dysfunction in relapsing–remitting multiple sclerosis

Aurélie Ruet, Delphine Hamel, Mathilde S.A. Deloire, Julie Charré-Morin, Aurore Saubusse, Bruno Brochet

The aim of this work is to study the relationship between information processing speed (IPS) impairment and motor testing that reflects cerebellar function in persons with multiple sclerosis (PwMS).

Melatonin ameliorates oxidative stress, modulates death receptor pathway proteins, and protects the rat cerebrum against bisphenol-A-induced apoptosis

Mohamed A. El-Missiry, Azza I. Othman, Monera A. Al-Abdan, Aml A. El-Sayed

Epidemiological reports have indicated a correlation between the increasing of bisphenol-A (BPA) levels in the environment and the incidence of neurodegenerative diseases. In the present study, the protective effect of melatonin on oxidative stress and the death receptor apoptotic proteins in the cerebrum of the bisphenol-A-treated rats were examined. Adult male rats were orally administered melatonin (10 mg/kg bw) concurrently with BPA (50 mg/kg bw) 3 days a week for 6 weeks. BPA exposure resulted in significant elevations of oxidative stress, as evidenced by the increased malondialdehyde level and the decreased glutathione level and superoxide dismutase activity in the cerebrum.

Usefulness of sacral nerve modulation in a series of multiple sclerosis patients with bladder dysfunction

Elena Andretta, Claudio Simeone, Edoardo Ostardo, Mauro Pastorello, Cristina Zuliani

Bladder dysfunctions are common in multiple sclerosis (MS) often causing the most distressing symptoms. The aim of this paper was to evaluate the effectiveness of sacral nerve modulation (SNM) in this disease.

Single-subject classification of schizophrenia patients based on a combination of oddball and mismatch evoked potential paradigms

Jorne Laton, Jeroen Van Schependom, Jeroen Gielen, Jeroen Decoster, Tim Moons, Jacques De Keyser, Marc De Hert, Guy Nagels

The diagnostic process for schizophrenia is mainly clinical and has to be performed by an experienced psychiatrist, relying primarily on clinical signs and symptoms. Current neurophysiological measurements can distinguish groups of healthy controls and groups of schizophrenia patients. Individual classification based on neurophysiological measurements mostly shows moderate accuracy. We wanted to examine whether it is possible to distinguish controls and patients individually with a good accuracy.

Investigating the minimal important difference in ambulation in multiple sclerosis: A disconnect between performance-based and patient-reported outcomes?

Carolyn E. Schwartz, Armon Ayandeh, Robert W. Motl

We sought to estimate the MID on two patient-reported outcome (PRO) measures that are frequently used in multiple sclerosis (MS) clinical research: the MS Walking Scale and the MS Impact Scale-29. We anchored the Minimally Important Differences with an objective measure of ambulation, the accelerometer.

Enriched environment induces angiogenesis and improves neural function outcomes in rat stroke model

Kewei Yu, Yi Wu, Qi Zhang, Hongyu Xie, Gang Liu, Zhenzhen Guo, Fang Li, Jie Jia, Shenyi Kuang, Ruiping Hu

Increasing evidence shows that exposure to an enriched environment (EE) after cerebral ischemia/reperfusion injury has neuroprotective benefits in animal models, including enhancing functional recovery after ischemic stroke. However, the mechanism underlying this effect remains unclear. To clarify this critical issue, the current study investigated the effects of EE on the improvement of damaged neural function and the induction of angiogenesis. Adult rats were subjected to ischemia induced by middle cerebral artery occlusion followed by reperfusion.  

Central nervous system involvement of hypereosinophilic syndrome: A report of 10 cases and a literature review

Dokyung Lee, Tae-Beom Ahn

The central nervous system (CNS) may be affected in those cases of peripheral eosinophila without a secondary cause (hypereosinophilic syndrome, HES). The pathomechanism of CNS involvement in HES remained uncertain.

Somatosensory evoked magnetic fields following tongue and hard palate stimulation on the preferred chewing side

Hitoshi Maezawa, Yoshiyuki Hirai, Hideaki Shiraishi, Makoto Funahashi

Although oral sensory feedback is essential for mastication, whether the cortical activity elicited by oral stimulation is associated with the preferred chewing side (PCS) is unclear. Somatosensory evoked fields were measured in 12 healthy volunteers (6 with the right side as the PCS and 6 with the left side as the PCS) following tongue and hard palate stimulation. Three components were identified over the contralateral (P40m, P60m, and P80m) and ipsilateral [P40m(I), P60m(I), and P80m(I)] hemispheres.                       

Apathy in multiple sclerosis: A validation study of the apathy evaluation scale

Simona Raimo, Luigi Trojano, Daniele Spitaleri, Vittorio Petretta, Dario Grossi, Gabriella Santangelo

Apathy is defined as lack of motivation affecting cognitive, emotional, and behavioral domains and is usually assessed by standardized scales, such as the Apathy Evaluation Scale (AES). Recently, apathy has been recognized as a frequent behavioral symptom in multiple sclerosis (MS).

Calf muscle involvement in Becker muscular dystrophy: When size does not matter

Mauro Monforte, Eugenio Mercuri, Francesco Laschena, Enzo Ricci, Giorgio Tasca

Calf hypertrophy is a common feature in Becker muscular dystrophy (BMD), and it is still debated to which extent fatty degeneration or true muscle hypertrophy account for it. We wanted to investigate the relative contribution of these two components using a simple image analysis approach and their possible correlation with disease severity.

Diagnosis of cystathionine beta-synthase deficiency by genetic analysis

Fatemeh Suri, Mehrnaz Narooie-Nejad, Iman Safari, Hamidreza Moazzeni, Mohammad-Reza Rohani, Ali Khajeh, Brandy Klotzle, Jian-Bing Fan, Elahe Elahi

Intellectual disability like other common diseases is often complex because they are genetically heterogeneous, with many different genetic defects giving rise to clinically indistinguishable phenotypes. We present diagnosis of cystathionine beta-synthase (CBS) deficiency in a multiply affected Iranian family with obvious intellectual disability based on whole genome SNP homozygosity mapping. Diagnosis based on clinical presentations had not been made because of unavailability of appropriate medical services.

Influencing effect of non-motor symptom clusters on quality of life in Parkinson's disease

Sung Reul Kim, Hui Young So, Eunju Choi, Jeong Hee Kang, Hye Young Kim, Sun Ju Chung

The heterogeneity of non-motor symptoms (NMSs) in patients with Parkinson's disease (PD) has been well established. We investigated the effects of NMS as a cluster on the quality of life (QoL) of patients with PD. We recruited 180 patients with PD and used a descriptive cross-sectional study design. To determine interrelationships between non-motor symptoms, a principal component analysis with varimax rotation was performed based on the Non-Motor Symptoms Scale (NMSS). Among 180 PD patients, 172 patients (96.6%) had experienced at least 2 domains of concurrent non-motor symptoms.

Cognitive correlates of hallucinations and delusions in Parkinson's disease

Stewart A. Factor, Michael K. Scullin, Ann B. Sollinger, Julia O. Land, Cathy Wood-Siverio, Lavezza Zanders, Alan Freeman, Donald L. Bliwise, William M. McDonald, Felicia C. Goldstein

Hallucinations and delusions that complicate Parkinson's disease (PD) could lead to nursing home placement and are linked to increased mortality. Cognitive impairments are typically associated with the presence of hallucinations but there are no data regarding whether such a relationship exists with delusions.

C9ORF72 repeat expansion is not a significant cause of late onset cerebellar ataxia syndrome

Cheng-Tsung Hsiao, Pei-Chien Tsai, Yi-Chu Liao, Yi-Chung Lee, Bing-Wen Soong

The GGGGCC hexanucleotide expansion in the C9ORF72 gene is the most common cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) in Caucasian populations. The phenotypic spectrum of C9ORF72 hexanucleotide repeat expansion mutation has been reported to include parkinsonian syndrome, Huntington's disease-like syndrome and dementia syndrome. Although few individuals with cerebellar ataxia have also anecdotally been found to harbor the mutation, the relationship between the mutation and cerebellar ataxia awaits further clarification.

The contrasting epigenetic role of RUNX3 when compared with that of MGMT and TIMP3 in glioblastoma multiforme clinical outcomes

Uxia Saraiva-Esperón, Alvaro Ruibal, Michel Herranz

Glioblastoma multiforme (GBM) is the most frequent and malignant astrocytic glioma in the adult, with a survival rate at 5 years less than 5%. In the GBM pathogenesis, the importance of genes methylation involved in cell cycle, tumor suppression, DNA repair and genome integrity, as well as tumor invasion and apoptosis has been described. We analyzed epigenetic regulation involvement of two genes related with apoptosis: TIMP3 and RUNX3 in order to define a clinical profile and compare with the most studied gene in GBM: MGMT.      

Voluntary control of facial musculature in Parkinson's disease

Michelle Marneweck, Geoff Hammond

Aside from being measured in the context of producing facial expressions of emotion, the ability to voluntarily control a range of facial muscles in Parkinson's disease (PD) has not been systematically measured. We used in three enrollment phases an adaptation of the Upper and Lower Face Apraxia test, a measure of the ability to make voluntary movements of the upper and lower face in PD patients and healthy controls. Errors were scored due to (1) pauses prior to movement initiation, (2) loss of individuation, (3) impoverished movement, (4) no movement at all, or (5) content errors (likened to ideational apraxia errors).


Short Communications

Stenogyria — Not only in Chiari II malformation

Monika Bekiesinska-Figatowska, Agnieszka Duczkowska, Hanna Brągoszewska, Marek Duczkowski, Hanna Mierzewska

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.022
  • p337–340
  • Published online: September 25, 2014
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Stenogyria, meaning multiple small compacted gyri separated by shallow sulci, is reported in the literature in association with Chiari II malformation (CM II) which in turn is reported in association with myelomeningocele (MMC). The authors present five cases of stenogyria (and other abnormalities found in CM II, like callosal hypoplasia/dysplasia, agenesis of the anterior commissure, hypoplasia of the falx cerebri) in children without the history of MMC or any other form of open spinal dysraphism.

The Virtual Peg Insertion Test as an assessment of upper limb coordination in ARSACS patients: A pilot study

< id="S0022-510X(14)00630-3-au">Cynthia Gagnon, Caroline Lavoie, Isabelle Lessard, Jean Mathieu, Bernard Brais, Jean-Pierre Bouchard, Marie-Christine Fluet, Roger Gassert, Olivier Lambercy

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.032
  • p341–344
  • Published online: September 27, 2014
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This paper introduces a novel assessment tool to provide clinicians with quantitative and more objective measures of upper limb coordination in patients suffering from Autosomal Recessive Spastic Ataxia of Charlevoix–Saguenay (ARSACS). The Virtual Peg Insertion Test (VPIT) involves manipulating an instrumented handle in order to move nine pegs into nine holes displayed in a virtual environment. The main outcome measures were the number of zero-crossings of the hand acceleration vector, as a measure of movement coordination and the total time required to complete the insertion of the nine pegs, as a measure of overall upper limb performance.

Delayed auditory feedback simulates features of nonfluent primary progressive aphasia

Carolina Maruta, Sonya Makhmood, Laura E. Downey, Hannah L. Golden, Phillip D. Fletcher, Pirada Witoonpanich, Jonathan D. Rohrer, Jason D. Warren

The pathophysiology of nonfluent primary progressive aphasia (nfvPPA) remains poorly understood. Here, we compared quantitatively speech parameters in patients with nfvPPA versus healthy older individuals under altered auditory feedback, which has been shown to modulate normal speech output. Patients (n = 15) and healthy volunteers (n = 17) were recorded while reading aloud under delayed auditory feedback [DAF] with latency 0, 50 or 200 ms and under DAF at 200 ms plus 0.5 octave upward pitch shift. DAF in healthy older individuals was associated with reduced speech rate and emergence of speech sound errors, particularly at latency 200 ms.

The circulating level of leptin and blood pressure in patients with multiple system atrophy

Tetsutaro Ozawa, Jun Tokunaga, Musashi Arakawa, Atsushi Ishikawa, Ryoko Takeuchi, Akio Yokoseki, Hirohito Sone, Masatoyo Nishizawa

Patients with multiple system atrophy (MSA) frequently exhibit orthostatic hypotension (OH). Leptin, an adipose-derived hormone, contributes to the sympathetic control of blood pressure (BP), and loss of leptin may cause OH. We aimed to clarify the relationship between leptin and OH in MSA. Serum leptin levels were measured in 36 patients with MSA, 25 patients with other atypical parkinsonian disorders (APDs), including progressive supranuclear palsy-Richardson syndrome and corticobasal syndrome, and 26 control subjects.                       

A complex form of hereditary spastic paraplegia in three siblings due to somatic mosaicism for a novel SPAST mutation in the mother

Anna Aulitzky, Katrin Friedrich, Dieter Gläser, Regina Gastl, Christian Kubisch, Albert C. Ludolph, Alexander E. Volk

Hereditary spastic paraplegias (HSPs) represent a clinically and genetically heterogeneous group of diseases. Major symptoms comprise progressive bilateral leg stiffness, spasticity at rest and diffuse muscle weakness. Complex forms are characterized by additional symptoms like dementia, cerebellar dysfunction or seizures. Autosomal dominant, autosomal recessive, X-linked recessive and possibly mitochondrial inheritance have been described in familial HSP. The most frequently mutated gene in familial cases of uncomplicated autosomal dominant HSP is SPAST, however de novo mutations in SPAST are rarely found.

Should spinocerebellar ataxias be included in the differential diagnosis for Huntington's diseases-like syndromes?

José Luiz Pedroso, Maria Eliza Thomaz de Freitas, Marcus Vinicius Cristino Albuquerque, Maria Luiza Saraiva-Pereira, Laura Bannach Jardim, Orlando G.P. Barsottini

In this article, we describe three patients with different spinocerebellar ataxia (SCA) subtypes presenting with unusual movement disorders predominantly characterized by choreoathetosis, which, together with their autosomal dominant pattern of inheritance, resembled the Huntington-like syndromes. From a large SCA cohort, we have observed chorea in 1/35 SCA2, 1/112 SCA3/MJD, and 1/30 SCA7 patients. Twenty-eight patients with SCA1, 11 patients with SCA6, and 3 patients with SCA10 were also evaluated, and none of them presented chorea.

MRI negative meningeal myeloma with abducens nerve palsies responding to intrathecal chemotherapy

A. Grisold, C. Weber, J. Hainfellner, H. Gisslinger, G. Kasprian, E. Auff, T. Sycha, W. Grisold

Meningeal involvement of multiple myeloma is rare. A patient with multiple myeloma presented with bilateral abducens nerve palsies. In the MRI neither lytic skull lesions nor meningeal enhancement could be found. The diagnosis was based on CSF studies and cytology. A neurologic remission was achieved with intrathecal chemotherapy.

Can dopamine agonists trigger tactile hallucinations in patients with Parkinson's disease?

Hiroshi Kataoka, Nobuhiro Sawa, Kazuma Sugie, Satoshi Ueno

Various hallucinations are unpleasant for patients with Parkinson's disease (PD). Hallucinations are often related to anti-parkinsonian drugs. Tactile hallucinations rarely occur in patients with PD. In contrast to other types of hallucinations, tactile hallucinations often make physicians wonder if a physical abnormality is the underlying cause. However, the relation of tactile hallucinations to anti-parkinsonian drugs remains uncertain because studies are scant. We describe three patients with PD who had tactile hallucinations that were triggered by dopamine agonists.

Acute hemicerebellitis in a young adult: A case report and literature review

Keisuke Suzuki, Toshiki Nakamura, Ayaka Numao, Hiroaki Fujita, Tomoko Komagamine, Takahide Nagashima, Yohei Asakawa, Yuji Watanabe, Hidehiro Takekawa, Koichi Hirata

Acute hemicerebellitis, marked by headache with or without cerebellar signs, is a rare clinical entity involving a unilateral cerebellar hemisphere. The pathogenesis of acute hemicerebellitis remains unclear, and the disease rarely occurs in adults. Here, we report an 18-year-old woman who presented with a lack of coordination of the right hand and leg lasting longer than one week, following a pulsatile headache. A neurological examination disclosed ocular dysmetria, right-sided limb ataxia and slight truncal ataxia.

Genetic analysis of SPG4 and SPG3A genes in a cohort of Chinese patients with hereditary spastic paraplegia

Xingjiao Lu, Zhidong Cen, Fei Xie, Zhiyuan Ouyang, Baorong Zhang, Guohua Zhao, Wei Luo

Hereditary spastic paraplegia (HSP or SPG) is a group of genetically and clinically heterogeneous neurodegenerative disorders. At least 52 different gene loci have been identified so far, involving autosomal dominant (AD), autosomal recessive (AR), X-linked (XL), and maternal inheritance. Mutations in the SPAST (SPG4) and ATL1 (SPG3A) genes are responsible for about 50% of pure AD-HSP patients. In this study, SPAST and ATL1 mutations were screened in 36 unrelated HSP patients (17 probands with AD family history and 19 sporadic HSP patients) by direct sequencing and multiplex ligation dependent probe amplification (MLPA).

A novel strumpellin mutation and potential pitfalls in the molecular diagnosis of hereditary spastic paraplegia type SPG8

Amir Jahic, Friedmar Kreuz, Pia Zacher, Jana Fiedler, Andrea Bier, Silke Reif, Manuela Rieger, Stefan Krüger, Christian Beetz, Jens Plaschke

Hereditary spastic paraplegia (HSP) is a clinically and genetically heterogeneous, neurodegenerative movement disorder. A total of eight KIAA0196/strumpellin variants have thus far been associated with SPG8, a rare dominant HSP. We present a novel strumpellin alteration in a small family with clinically pure HSP. We corroborated its causality by comparing it to rare benign variants at several levels, and, along this line, also re-considered previous genetic reports on SPG8. These analyses identified significant challenges in the interpretation of strumpellin alterations, and suggested that at least two of the few families claimed to suffer from SPG8 may have been genetically misdiagnosed.

Mutational screening of 320 Brazilian patients with autosomal dominant spinocerebellar ataxia

Vívian Pedigone Cintra, Charles Marques Lourenço, Sandra Elisabete Marques, Luana Michelli de Oliveira, Vitor Tumas, Wilson Marques

Autosomal dominant spinocerebellar ataxias (SCAs) are a clinical and genetically heterogeneous group of debilitating neurodegenerative diseases that are related to at least 36 different genetic loci; they are clinically characterized by progressive cerebellar ataxia and are frequently accompanied by other neurological and non-neurological manifestations. The relative frequency of SCA varies greatly among different regions, presumably because of a founder effect or local ethnicities. Between July 1998 and May 2012, we investigated 320 Brazilian patients with an SCA phenotype who belonged to 150 unrelated families with an autosomal dominant inheritance pattern and 23 sporadic patients from 13 Brazilian states.

Myopathy during treatment with the antianginal drug ranolazine

Charles D. Kassardjian, Xia Tian, Georgirene Vladutiu, Lee-Jun Wong, Margherita Milone

Ranolazine is a medication indicated for treatment of chronic angina and is a partial inhibitor of the fatty acid β-oxidation. We present an adult patient who developed subacute progressive muscle weakness and exercise-induced myalgia, soon after increasing the daily dose of ranolazine, in the setting of therapy with simvastatin. CK persisted normal throughout the duration of the weakness and muscle biopsy showed a lipid storage myopathy for which no underlying genetic defect was identified. Discontinuation of both drugs resulted in clinical improvement.


Letters to the Editor

Acute recanalization and hyperperfusion in MCA territory due to sole t-PA bolus. A case report

Carsten M. Klingner, Stefan Brodoehl, Christian Hohenstein, Otto W. Witte, Albrecht Günther

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.026
  • p383–384
  • Published online: September 24, 2014
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Tissue plasminogen activator (t-PA) is the only approved thrombolytic therapy for the treatment of stroke patients. However, there is discussion whether the current dosing of t-PA (0.9 mg/kg) is the optimal choice for all patients [1]. It is also unknown whether the duration of administration (1 h) is being optimal. Moreover, there is no information available about the effectiveness of a sole t-PA bolus. The following case demonstrates effectiveness and importance of the bolus of t-PA.

A case of Andersen–Tawil syndrome presenting periodic paralysis exacerbated by acetazolamide

Satoshi Nagamine, Shunichi Sakoda, Reiji Koide, Akihiro Kawata, Junhui Yuan, Hiroshi Takashima, Imaharu Nakano

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.040
  • p385–386
  • Published online: September 30, 2014
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Andersen–Tawil syndrome (ATS) is a rare autosomal dominant disorder described first by Andersen et al. [1] and Tawil et al. [2]. It has the clinical triad of periodic paralysis (PP), ventricular arrhythmia and dysmorphic features. Patients with ATS, unlike those with other types of PP, may develop fatal ventricular arrhythmia, although the occurrence of this is rare [3]. Plaster et al. identified a heterozygous mutation of KCNJ2 gene encoding an inward-rectifying potassium channel Kir2.1 in ATS patients [4].

Primary progressive aphasia with occipital impairment

David García-Azorín, Jordi A. Matías-Guiu, María Nieves Cabrera-Martín, Marta Fernández-Matarrubia, Teresa Moreno-Ramos, José Luis Carreras, Jorge Matías-Guiu

  • DOI: http://dx.doi.org/10.1016/j.jns.2014.09.043
  • p387–388
  • Published online: September 29, 2014
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Primary progressive aphasia (PPA) is a clinical entity characterized by a neurodegenerative language disorder. The current classification in three variants of PPA has improved the ability to predict the pathological substrate (1,2). Wicklund et al. have recently described two clinical cases of progressive aphasia with left occipitotemporal hypometabolism, suggesting an overlapping form with posterior cortical atrophy (PCA), a subtype of focal onset of Alzheimer's disease (3). We present two cases of progressive aphasia with left parieto-temporo-occipital hypometabolism in fluorodeoxyglucose positron emission tomography (18F-FDG-PET), and amyloid deposition in PET imaging with 18F-Florbetapir.

Infliximab monotherapy for neuro-Behçet's disease: A case report

Massimiliano Di Filippo, Maria Di Gregorio, Carlotta Nannini, Lorenzo Gaetani, Cristina Gallina, Piero Floridi, Paola Sarchielli, Paolo Calabresi, Fabrizio Cantini

Behçet's disease (BD) is a chronic, relapsing, systemic vasculitis of unknown cause involving veins and arteries of all sizes and characterized by protean clinical manifestations. The disease was first described in 1937 as the triad of recurrent oral aphthous ulcers, genital ulceration and uveitis [1]. Neurological involvement is one of the most serious causes of long-term morbidity and mortality in BD [1]. In particular, parenchymal central nervous system (CNS) involvement usually presents with a brainstem syndrome alone or in combination with cognitive-behavioural symptoms [2].

Vitamin D supplementation in multiple sclerosis: Making a case for clarity

S. Kimball, H.E. Hanwell, J.M. Burton, R.P. Heaney, M.F. Holick, B. Hollis, R. Lewanczuk, N. Makhani, S. Venkateswaran

You recently published a letter by Dr. Fragoso and colleagues [1] that provided a short description of 21 patients with relapsing remitting multiple sclerosis (MS) who were treated with "high dose" vitamin D and experienced subsequent adverse events.

Serial MR spectroscopy in hypertensive encephalopathy

Yuko Matsubara, Yasuhiro Manabe, Mizuho Hattori, Yumiko Nakano, Yoshiaki Takahashi, Hisashi Narai, Kosuke Ota, Koji Abe

A 38-year-old woman was admitted to the hospital because of gait disturbance. She had developed a headache, left hemiparesis, ataxia and incontinence ten days previously. She had a history of hypertension since the age of 15, but did not take depressor medication. Her mother also had hypertension. On arrival, she was 164 cm tall and weighted 48 kg. Her blood pressure was 250/160 mm Hg. She was unconscious with Glasgow Coma Scale (GCS) 15 (E4V5M6). Her cranial nerves were intact. Her right arm and both legs were paretic.                 

Helicobacter cinaedi meningitis: A case report and review of previous cases

Hatsumi Okubo, Masahide Goto, Miori Sato, Teruyuki Sugiyama, Mikihiko Kawano, Tetsuro Matsunaga, Takaaki Akaike

In their recent article, Sugiyama et al. [1] described the first known case of Helicobacter cinaedi (H. cinaedi) meningitis in an adult. Citing previous reports [2,3], they suggested that the patient's pet transmitted the microorganism to the patient. No previous report has mentioned that H. cinaedi was detected in both the patient and his or her pet; therefore, our report is the first to include both human and animal surveys for H. cinaedi.

Hypertrophic spinal pachymeningitis: Idiopathic vs. IgG4-related

Mohamad Ezzeldin, Ahmad Shawagfeh, Vicki Schnadig, Robert G. Smith, Xiang Fang

IgG4-related disease is a newly described pathologic condition that can affect almost every organ system [1]. IgG4-related hypertrophic pachymeningitis (IgG4-RHP) has been recognized as a CNS manifestation of IgG4-related disease [1,2]. Most reported cases of IgG4-RHP have intracranial involvement [2]. Only two cases of spinal IgG4-RHP have been described [3,4]. The spinal form of IgG4-RHP can cause spinal cord compression that is similar to idiopathic spinal hypertrophic pachymeningitis (IHP), a rare chronic progressive inflammatory fibrosis of the dura mater.

Pathological features of intracardiac bacterial vegetation and intracranial arterial thrombus due to infective endocarditis in a stroke patient

Takashi Shimoyama, Naoki Saji, Junichi Uemura, Kensaku Shibazaki, Hirotake Nishimura, Kazumi Kimura

Infective endocarditis (IE) in patients receiving hemodialysis is a common stroke mechanism [1]. Although intracardiac bacterial vegetation as an embolic source plays an important role in IE-related stroke [2], the pathological features of bacterial vegetation and occlusive intra-arterial thrombus are not fully understood. We report herein on autopsy and pathological findings for IE-related stroke in a patient receiving hemodialysis.

Faciobrachial dystonic seizures and antibodies to Lgi1 in a 92-year-old patient: A case report

Pawel Fidzinski, Sven Jarius, Christian Gaebler, Friedrich Boegner, Roland Nohr, Klemens Ruprecht

Faciobrachial dystonic seizures (FBDS) have recently been characterized as a distinctive type of seizures, which frequently precede the onset of limbic encephalitis and are associated with antibodies to the leucine-rich glioma inactivated-1 (Lgi1) protein [1]. We here report on a novel patient with FBDS and antibodies to Lgi1, who had a remarkably old age at first manifestation of the disease, but still showed a complete remission of FBDS following immunotherapy with intravenous immunoglobulins (IVIG).

Sensory ataxia associated with Graves' disease

Kristin Samuelsson, Mats Palmer, Rayomand Press

A 76-year-old female with no family history of neuromuscular disease presented in March 2010 with an eight month history of progressive walking difficulty leading to the need of using a walker as well as of progressive numbness and pain in her feet and hands. She had no history of other diseases, except ongoing treatment for Graves' disease (see below). Neurological examination at time of presentation revealed intact strength, reduced deep tendon reflexes, reduced proprioception and pin-prick sensation in the legs and a complete loss of vibration sensation below the elbows and knees.    

Indirect meta-analytical comparison of azathioprine and of beta interferon effectiveness in all forms of multiple sclerosis pooled together

Andrea Messori, Valeria Fadda, Dario Maratea, Sabrina Trippoli

In pharmacotherapy of multiple sclerosis (MS), the advantages of oral administration are increasingly being recognised [1]. Long time ago, oral azathioprine was tested in a series of small clinical studies, and two meta-analyses were published that pooled the information available at that time [2,3]. Thereafter, azathioprine has not attracted the interest of pharmaceutical industry mainly because of its low cost, and so no further randomised trials (RTs) were conducted.

Palatal myoclonus, eight-and-a-half syndrome, and Holmes tremor in a patient from a single brainstem lesion

Robert D. Bolen, Nikhil Balakrishnan, M.D.

A 53 year old African American male with a past history of hypertension, diabetes, hyperlipidemia, end-stage renal disease, seizures and pontine hemorrhage presented to the hospital with progressive abnormal movements in his right arm. The patient noticed symptoms started several months after his pontine hemorrhage. On exam, the patient had left gaze palsy with very limited adduction of the left in right gaze (one and a half syndrome). A dissociated horizontal pendular nystagmus was evident, greater in the right eye.

A case of shunt responsive tremor due to normal pressure hydrocephalus

Aron T. Hill, Maxwell W. Cowey, David R. Williams

Hydrocephalus refers to an abnormal accumulation of cerebral spinal fluid (CSF) within the brain resulting in enlarged ventricles and subsequent distortion of periventricular structures [1], while normal pressure hydrocephalus (NPH) is a specific form of communicating hydrocephalus where intra-ventricular CSF pressure remains in the normal range. As only a small disparity in pressure between the ventricles and surrounding subarachnoid space are needed to trigger absorption of interstitial fluid, ventricular enlargement still occurs in this condition [2], which is typically accompanied by symptoms of gait disturbance, urinary incontinence and cognitive impairment [3].