JNS-cover.jpgJournal of Neurological Sciences

Vol 355 | No. 1-2 | 15 August 2015 | Pages 1-222

Cause, effect and consequence? Painful small fiber sensory polyneuropathy and impaired glycemia

Jeffrey A. Cohen, Shao-Hwa Pius Wei

Type II Diabetes Mellitus is the most common cause of polyneuropathy (PN) in the developed world. Prevalence of PN increases with age and duration of diabetes, with over 50% of diabetics over the age of 60 afflicted with neuropathy [9]. The pathophysiology of nerve damage during the persistent hyperglycemia of type II DM consists of oxidative stressors, free radical production and microvascular changes due to circulatory insufficiency [2,7,8]. Sorbitol and fructose cause damage as hyperglycemia causes an activation of the polyol pathway, and microvasculopathy can cause thickening of blood vessel walls and occlusion.

Ataxia-telangiectasia — A historical review and a proposal for a new designation: ATM syndrome

Hélio A.G. Teive, Adriana Moro, Mariana Moscovich, Walter O. Arruda, Renato P. Munhoz, Salmo Raskin, Tetsuo Ashizawa

The authors review ataxia telangiectasia, emphasizing historical aspects, genetic discoveries, and the clinical presentations of the classical and atypical forms. In fact, ataxia telangiectasia represents a multisystem entity with pleomorphic neurological and systemic manifestations. ATM syndrome is proposed as a more adequate designation for this entity.

Epidemiological, clinical, and immunological characteristics of neuromyelitis optica: A review

Wildéa Lice de Carvalho Jennings Pereira, Edna Maria Vissoci Reiche, Ana Paula Kallaur, Damacio Ramón Kaimen-Maciel

The aim of this study was to review the epidemiological and clinical characteristics of neuromyelitis optica (NMO) and the immunopathological mechanisms involved in the neuronal damage. NMO is an inflammatory demyelinating autoimmune disease of the central nervous system that most commonly affects the optic nerves and spinal cord. NMO is thought to be more prevalent among non-Caucasians and where multiple sclerosis (MS) prevalence is low. NMO follows a relapsing course in more than 80–90% of cases, which is more commonly in women.

Molecular and clinical features of inherited neuropathies due to PMP22 duplication

M.M. Watila, S.A. Balarabe

PMP22 is a transmembrane glycoprotein component of myelin, important for myelin functioning. Mutation of PMP22 gene which encodes for the production of PMP22 glycoprotein is associated with a variety of inherited neuropathies. This literature review sought to review the molecular mechanism and clinical features of inherited neuropathies caused by PMP22 duplication. PMP22 duplication causes CMT1A which accounts for more than half of all CMT cases and about 70% of CMT1 cases. It manifests with muscle weakness, depressed reflexes, impaired distal sensation, hand and foot deformities, slowing of NCV and onion bulbs.

Lymphoma-associated dysimmune polyneuropathies

Joerg-Patrick Stübgen

Lymphoma consists of a variety of malignancies of lymphocyte origin. A spectrum of clinical peripheral neuropathy syndromes with different disease mechanisms occurs in about 5% of lymphoma patients. There exists a complex inter-relationship between lymphoproliferative malignancies and autoimmunity. An imbalance in the regulation of the immune system presumably underlies various immune-mediated neuropathies in patients with lymphoma. This article reviews lymphoma and more-or-less well-defined dysimmune neuropathy subgroups that are caused by humoral and/or cell-mediated immune disease mechanisms directed against known or undetermined peripheral nerve antigens.

Collum-caput (COL-CAP) concept for conceptual anterocollis, anterocaput, and forward sagittal shift

Josef Finsterer, Concha Maeztu, Gonzalo J. Revuelta, Gerhard Reichel, Daniel Truong

Anterocollis as a rare subtype of cervical dystonia is difficult to treat and thus less appreciated than other subtypes of cervical dystonia. This review aimed at summarising and discussing recent advances in the management of anterocollis.

Characterization of retinal architecture in Parkinson's disease

Jessica Chorostecki, Navid Seraji-Bozorgzad, Aashka Shah, Fen Bao, Ginny Bao, Edwin George, Veronica Gorden, Christina Caon, Elliot Frohman, M. Tariq Bhatti, Omar Khan

Parkinson's disease (PD) is a neurodegenerative disorder associated with dopaminergic cell loss and α-synuclein aggregation in Lewy bodies, which has been demonstrated in the retina.

Predictive value of MRI parameters in severity and recovery of first-episode myelitis in aquaporin-4 antibody disease

Andrew Murchison, Joanna Kitley, M. Isabel Leite, Wilhelm Küker, Jacqueline Palace

Neuromyelitis optica spectrum disorder (NMOSD) associated with aquaporin-4 antibodies (AQP4-Ab) typically causes longitudinally-extensive transverse myelitis (LETM). Few data exist about the association of MRI features with LETM attack severity and recovery.

Predictors of clinical failure of decompressive hemicraniectomy for malignant hemispheric infarction

Amedeo Merenda, Jon Perez-Barcena, Guiem Frontera, Ronald J. Benveniste

The aim of this study is to identify pre-operative clinical and/or radiological predictors of clinical failure of decompressive hemicraniectomy (DH) in the setting of malignant hemispheric infarction. These predictors could guide the decision for adjunctive internal brain decompression (e.g. strokectomy) at the time of the initial DH.

Longitudinally extensive optic neuritis as an MRI biomarker distinguishes neuromyelitis optica from multiple sclerosis

Maureen A. Mealy, Anna Whetstone, Gunes Orman, Izlem Izbudak, Peter A. Calabresi, Michael Levy

To differentiate MRI characteristics of optic neuritis associated with neuromyelitis optica (NMO) and relapsing remitting multiple sclerosis (RRMS).

Longitudinal ultra-extensive transverse myelitis as a manifestation of neurosarcoidosis

Lei Wang, Yuebing Li

To analyze the clinical characteristics and outcome of patients with neurosarcoidosis manifesting as longitudinal transverse myelitis spanning 6 or more spinal segments.

Radiographic conjugate horizontal eye deviation in patients with acute cerebellar infarction

Kazutaka Nishimura, Tomoyuki Ohara, Kazuyuki Nagatsuka, Kazuo Minematsu, Kazunori Toyoda

Conjugate eye deviation (CED) has not been fully investigated in patients with acute cerebellar infarction. We investigated the incidence of CED on neurological examination and head imaging with acute cerebellar infarction and associations of CED with the involved vascular territory, lesion site and other clinical factors.

Variation in the miRNA-433 binding site of FGF20 is a risk factor for Parkinson's disease in Iranian population

Leyla Haghnejad, Babak Emamalizadeh, Javad Jamshidi, Alireza Zare Bidoki, Hamid Ghaedi, Ehsan Ahmadi, Shokoufeh Abdollahi, Neda Shahmohammadibeni, Shaghayegh Taghavi, Atena Fazeli, Marzieh Motallebi, Amir Ehtesham Shafiei Zarneh, Saeed Mohammadihosseinabad, Mohammad Reza Abbaszadegan, Shahram Torkamandi, Masoumeh Amini Gavenaroudi, Negar Pedram, Gholam-Ali Shahidi, Abbas Tafakhori, Hossein Darvish, Abolfazl Movafagh

DNA variations in the fibroblast growth factor 20 gene have been reported to be associated with Parkinson's disease (PD). The rs12720208, a functional SNP located in the 3′UTR region of the gene, was reported as a risk factor for PD. A number of studies, which tried to replicate the result in different populations, failed to detect any associations. In this study, we genotyped rs2720208 SNP in 520 PD patients and 520 healthy controls both from Iran. Significant differences were found in allele and genotype frequencies between patients and controls (p < 0.0001 for both).

Overground robot assisted gait trainer for the treatment of drug-resistant freezing of gait in Parkinson disease

Manuela Pilleri, Luca Weis, Letizia Zabeo, Konstantinos Koutsikos, Roberta Biundo, Silvia Facchini, Simonetta Rossi, Stefano Masiero, Angelo Antonini

Freezing of Gait (FOG) is a frequent and disabling feature of Parkinson disease (PD). Gait rehabilitation assisted by electromechanical devices, such as training on treadmill associated with sensory cues or assisted by gait orthosis have been shown to improve FOG. Overground robot assisted gait training (RGT) has been recently tested in patients with PD with improvement of several gait parameters. We here evaluated the effectiveness of RGT on FOG severity and gait abnormalities in PD patients. Eighteen patients with FOG resistant to dopaminergic medications were treated with 15 sessions of RGT and underwent an extensive clinical evaluation before and after treatment.       

Granulocyte colony-stimulating factor attenuates spinal cord injury-induced mechanical allodynia in adult rats

Kei Kato, Masao Koda, Hiroshi Takahashi, Tsuyoshi Sakuma, Taigo Inada, Koshiro Kamiya, Mitsutoshi Ota, Satoshi Maki, Akihiko Okawa, Kazuhisa Takahashi, Masashi Yamazaki, Masaaki Aramomi, Masayuki Hashimoto, Osamu Ikeda, Chikato Mannoji, Takeo Furuya

Spinal cord injury (SCI) can cause neuropathic pain (NeP), often reducing a patient's quality of life. We recently reported that granulocyte colony-stimulating factor (G-CSF) could attenuate NeP in several SCI patients. However, the mechanism of action underlying G-CSF-mediated attenuation of SCI-NeP remains to be elucidated. The purpose of the present study was to elucidate the therapeutic effect and mechanism of action of granulocyte colony-stimulating factor for SCI-induced NeP. T9 level contusive SCI was introduced to adult male Sprague Dawley rats.      

Blood circulating microparticle species in relapsing–remitting and secondary progressive multiple sclerosis. A case–control, cross sectional study with conventional MRI and advanced iron content imaging outcomes

J.S. Alexander, R. Chervenak, B. Weinstock-Guttman, I. Tsunoda, M. Ramanathan, N. Martinez, S. Omura, F. Sato, G.V. Chaitanya, A. Minagar, J. McGee, M.H. Jennings, C. Monceaux, F. Becker, U. Cvek, M. Trutschl, R. Zivadinov

Although multiple sclerosis (MS) is thought to represent an excessive and inappropriate immune response to several central nervous system (CNS) autoantigens, increasing evidence also suggests that MS may also be a neurovascular inflammatory disease, characterized by endothelial activation and shedding of cell membrane microdomains known as 'microparticles' into the circulation.

Optimal combination treatment and vascular outcomes in recent ischemic stroke patients by premorbid risk level

Jong-Ho Park, Bruce Ovbiagele

Optimal combination of secondary stroke prevention treatment including antihypertensives, antithrombotic agents, and lipid modifiers is associated with reduced recurrent vascular risk including stroke. It is unclear whether optimal combination treatment has a differential impact on stroke patients based on level of vascular risk.

Nocebo in Alzheimer's disease; meta-analysis of placebo-controlled clinical trials

Panagiotis Zis, Dimos-Dimitrios Mitsikostas

Nocebo is very prevalent among neurological diseases resulting in low adherence and treatment outcome. We sought to examine the AEs following placebo administration in Randomized Controlled Studies (RCTs) for Alzheimer's Disease (AD).

Callosal damage and cognitive deficits in chronic carbon monoxide intoxication: A diffusion tensor imaging study

Pei-Chin Chen, Meng-Hsiang Chen, Hsiu-Ling Chen, Cheng-Hsien Lu, Kun-Hsien Chou, Re-Wen Wu, Nai-Wen Tsai, Ching-Po Lin, Shau-Hsuan Li, Yi-Wen Chen, Yu-Fan Cheng, Wei-Che Lin

To evaluate the correlation between microstructural damage in the corpus callosum (CC) and the cognitive performance of patients with or without delayed encephalopathy (DE) after carbon monoxide (CO) intoxication in the chronic stage.

Prevalence and determinants of diabetic polyneuropathy in a sub-Saharan African referral hospital

C. Kuate-Tegueu, E. Temfack, S. Ngankou, J. Doumbe, V.P. Djientcheu, A.P. Kengne

Diabetic peripheral neuropathy is the commonest complication of diabetes mellitus, and a major cause of limb amputations. In general however, the magnitude of diabetic neuropathy in sub-Saharan Africans with diabetes has been less reliably quantified. We assessed the prevalence and determinants of diabetic polyneuropathy in hospital settings in Cameroon.

Neuroprotective effect of microRNA-99a against focal cerebral ischemia–reperfusion injury in mice

Zhen Tao, Haiping Zhao, Rongliang Wang, Ping Liu, Feng Yan, Chencheng Zhang, Xunming Ji, Yumin Luo

MicroRNA-99a (miR-99a) has been reported to function as a tumor suppressor through regulating cell cycle and apoptosis. But its clinical significance in ischemic stroke and its function in cerebral ischemia–reperfusion (I/R) injury remained unknown. Herein transient middle cerebral artery occlusion was built on C57BL/6 mice, followed by intracerebroventricular injection of miR-99a agomir or antagomir before reperfusion for 24 h. Our clinical analysis indicates that plasma miR-99a level was significantly decreased in ischemic stroke patients as compared to healthy subjects, and a significant correlation was observed between miR-99a and clinical parameters.

Use of interictal 18F-fluorodeoxyglucose (FDG)-PET and magnetoencephalography (MEG) to localize epileptogenic foci in non-lesional epilepsy in a cohort of 16 patients

Yuchun Wang, Bo Liu, Liqi Fu, Zhiqiang Cui

We assessed the efficacy of interictal 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) and magnetoencephalography (MEG) for localizing the epileptogenic foci in a small cohort of patients with non-lesional epilepsy. Sixteen patients, aged 8–32 years, with non-lesional epilepsy underwent MRI, continuous scalp video-electroencephalography (EEG) monitoring, interictal (FDG)-PET and MEG at our institution. Each patient subsequently underwent intracranial grid placement. The data from the intracranial grids was correlated with the previous studies to determine the efficacy of FDG-PET and MEG in localizing the epileptogenic zone.

Misdirected horizontal saccades in pan-cerebellar atrophy

Aasef G. Shaikh, Fatema F. Ghasia

Saccadic dysmetria is a sensitive marker of cerebellar dysfunction. We discovered misdirected horizontal saccades due to cross-coupled orthogonal (vertical) saccades in siblings with pan-cerebellar atrophy. There was an upward drift in vertical eye position after each cross-coupled downward saccade. Such drifts brought the eyes back to the desired target. Due to strong upward bias, downward compensatory slow movements did not follow cross-coupled upward saccades. There was minimal horizontal cross-coupling associated with vertical saccades.

Increased gene expression of growth associated protein-43 in skin of patients with early-stage peripheral neuropathies

Sarah Scheytt, Nadja Riediger, Silvia Braunsdorf, Claudia Sommer, Nurcan Üçeyler

Growth associated protein-43 (GAP-43) is one of the neural proteins associated with nerve injury that is upregulated after nerve injury. To investigate whether GAP-43 quantification in skin biopsies would differentiate subtypes of peripheral neuropathies, we analyzed GAP-43 expression in skin from the lateral thigh and the distal leg. We prospectively enrolled 130 patients with peripheral neuropathies and compared data with healthy controls. Intraepidermal nerve fiber density (IENFD) was determined using antibodies against protein gene product 9.5 (PGP 9.5); anti-GAP-43 antibodies were applied to visualize regenerating nerve fibers.     

Epidemiology and in-hospital outcome of stroke in South Ethiopia

Birrie Deresse, Debebe Shaweno

Although the burden of stroke in Sub-Saharan Africa, including Ethiopia, is increasing, there are few available data on stroke in Ethiopia.

Spontaneous activity in electromyography may differentiate certain benign lower motor neuron disease forms from amyotrophic lateral sclerosis

Manu E. Jokela, Satu K. Jääskeläinen, Satu Sandell, Johanna Palmio, Sini Penttilä, Annamaija Saukkonen, Raija Soikkeli, Bjarne Udd

There is limited data on electromyography (EMG) findings in other motor neuron disorders than amyotrophic lateral sclerosis (ALS). We assessed whether the distribution of active denervation detected by EMG, i.e. fibrillations and fasciculations, differs between ALS and slowly progressive motor neuron disorders. We compared the initial EMG findings of 43 clinically confirmed, consecutive ALS patients with those of 41 genetically confirmed Late-onset Spinal Motor Neuronopathy and 14 Spinal and Bulbar Muscular Atrophy patients.

Trigeminal root entry zone involvement in neuromyelitis optica and multiple sclerosis

Atsuhiko Sugiyama, Masahiro Mori, Hiroki Masuda, Tomohiko Uchida, Mayumi Muto, Akiyuki Uzawa, Shoichi Ito, Satoshi Kuwabara

Trigeminal root entry zone abnormality on brain magnetic resonance imaging has been frequently reported in multiple sclerosis patients, but it has not been investigated in neuromyelitis optica patients. Brain magnetic resonance imaging of 128 consecutive multiple sclerosis patients and 46 neuromyelitis optica patients was evaluated. Trigeminal root entry zone abnormality was present in 11 (8.6%) of the multiple sclerosis patients and two (4.3%) of the neuromyelitis optica patients. The pontine trigeminal root entry zone may be involved in both multiple sclerosis and neuromyelitis optica.

Clinical, polysomnographic, and CPAP titration features of obstructive sleep apnea: Mixed versus purely obstructive type

Sang-Ahm Lee, Gha-Hyun Lee, Yoo-Sam Chung, Woo Sung Kim

To determine whether obstructive sleep apnea syndrome (OSAS) patients with mixed sleep apnea (MSA) have different clinical, polysomnographic, and continuous positive airway pressure (CPAP) titration findings compared to OSAS patients without MSA.

Vitamin D and cognitive function: The Tromsø Study

Rolf Jorde, Ellisiv Bøgeberg Mathiesen, Sigbjørn Rogne, Tom Wilsgaard, Marie Kjærgaard, Guri Grimnes, Henrik Schirmer

There are indications that vitamin D may be important for more than skeletal health, including cognitive function.

Early neurological worsening in patients with Wilson's disease

Tomasz Litwin, Karolina Dzieżyc, Michał Karliński, Grzegorz Chabik, Wojciech Czepiel, Anna Członkowska

Early neurological worsening during treatment initiation for Wilson's disease (WD) is an unresolved problem. Our aim was to establish the frequency and outcome of early neurological worsening in patients with WD.

Anesthesiologic regimen and intraoperative delirium in deep brain stimulation surgery for Parkinson's disease

M. Lange, N. Zech, M. Seemann, A. Janzen, D. Halbing, F. Zeman, C. Doenitz, E. Rothenfusser, E. Hansen, A. Brawanski, J. Schlaier

In many centers the standard anesthesiological care for deep brain stimulation (DBS) surgery in Parkinson's disease patients is an asleep–awake–asleep procedure. However, sedative drugs and anesthetics can compromise ventilation and hemodynamic stability during the operation and some patients develop a delirious mental state after the initial asleep phase. Further, these drugs interfere with the patient's alertness and cooperativeness, the quality of microelectrode recordings, and the recognition of undesired stimulation effects.

Olfactory function and neuropsychological profile to differentiate dementia with Lewy bodies from Alzheimer's disease in patients with mild cognitive impairment: A 5-year follow-up study

Jung Han Yoon, Min Kim, So Young Moon, Seok Woo Yong, Ji Man Hong

Mild cognitive impairment (MCI) is a well-known precursor of Alzheimer's disease (AD) but often also precedes dementia with Lewy bodies (DLB). The early differentiation of DLB from AD is important to delay disease progression. Olfactory dysfunction is a well-known early sign of both AD and Lewy body disorders, including Parkinson's disease (PD) and DLB. Thus, the aim of the present study was to determine whether olfactory and neuropsychological tests can aid in the differentiation of DLB from AD at the MCI stage.

Toll like receptor-4 gene polymorphisms in patients with solitary cysticercus granuloma

Akhilesh Singh, Ravindra Kumar Garg, Amita Jain, Hardeep Singh Malhotra, Shantanu Prakash, Rajesh Verma, Praveen Kumar Sharma

Solitary cysticercus granuloma (SCG) of the brain is the most common type of neurocysticercosis in India. In this study, we evaluated TLR4 polymorphisms in patients with SCG.

Time-dependent test characteristics of neck stiffness in patients suspected of nontraumatic subarachnoid haemorrhage

Daan Backes, Gabriel J.E. Rinkel, A.J. Martijn Sturkenboom, Mervyn D.I. Vergouwen

Neck stiffness can be the only diagnostic clue for subarachnoid haemorrhage (SAH) at neurological examination in patients with a clinical suspicion of nontraumatic SAH who present with a normal level of consciousness and no focal neurologic deficits. Since the clinical impression is that neck stiffness may take several hours to develop, we determined time-dependent test characteristics of neck stiffness.

Neuromyelitis optica shorter lesion can cause important pyramidal deficits

Denis Bernardi Bichuetti, Marcelo Delboni Lemos, Isac de Castro, Gustavo Balthazar da Silveira Carvalho, Renato Sartori de Carvalho, Nitamar Abdala, Enedina Maria Lobato de Oliveira

Evaluate the correlation between spinal cord lesion length and pyramidal function system score in a cohort of patients with NMO.

Assessment of TREM2 rs75932628 association with amyotrophic lateral sclerosis in a Chinese population

Xueping Chen, Yongping Chen, Qianqian Wei, Xiaoyan Guo, Bei Cao, Ruwei Ou, Bi Zhao, Hui-Fang Shang

Although, rs75932628 in triggering receptor expressed on myeloid cells 2 (TREM2) was shown to increase the risk for Alzheimer's disease, there is no agreement on the association between this variant and the risk for amyotrophic lateral sclerosis (ALS). We conducted a large-sample study to investigate if this variant is associated with ALS in a Chinese population. A total of 868 sporadic ALS (SALS) and 869 healthy controls were included. All cases were genotyped for the single nucleotide polymorphisms (SNP) using Sequenom iPLEX Assay technology.

Does prediabetes cause small fiber sensory polyneuropathy? Does it matter?

C.D. Kassardjian, P.J.B. Dyck, J.L. Davies, Rickey E. Carter, P.J. Dyck

The association between prediabetes and distal polyneuropathy (DPN) remains controversial. Here we test whether the prevalence of small fiber sensory distal polyneuropathy is increased in prediabetes.

A novel ABCD1 mutation detected by next generation sequencing in presumed hereditary spastic paraplegia: A 30-year diagnostic delay caused by misleading biochemical findings

Georgios Koutsis, David S. Lynch, Arianna Tucci, Henry Houlden, Georgia Karadima, Marios Panas

To present a Greek family in which 5 male and 2 female members developed progressive spastic paraplegia. Plasma very long chain fatty acids (VLCFA) were reportedly normal at first testing in an affected male and for over 30 years the presumed diagnosis was hereditary spastic paraplegia (HSP). Targeted next generation sequencing (NGS) was used as a further diagnostic tool.

Decreased metabotropic glutamate receptor type 1 availability in a patient with spinocerebellar ataxia type 6: A 11C-ITMM PET study

Kenji Ishibashi, Yoshiharu Miura, Kinya Ishikawa, Kenji Ishii, Kiichi Ishiwata

Imaging of metabotropic glutamate receptor type 1 (mGluR1), localized exclusively in the cerebellar Purkinje cells and related to cerebellar function, has recently become possible using positron emission tomography (PET). We report the initial mGluR1 imaging in a 74-year-old woman with spinocerebellar ataxia type 6 (SCA6).

When should we test patients with familial ataxias for SCA31? A misdiagnosed condition outside Japan?

José Luiz Pedroso, Agessandro Abrahao, Kinya Ishikawa, Salmo Raskin, Paulo Victor Sgobbi de Souza, Wladimir Bocca Vieira de Rezende Pinto, Pedro Braga-Neto, Marcus Vinicius Cristino de Albuquerque, Hidehiro Mizusawa, Orlando G.P. Barsottini

The autosomal dominant spinocerebellar ataxias (SCAs) are a highly heterogeneous group of genetic diseases characterized by progressive gait ataxia and variable degrees of extracerebellar symptoms and signs. SCAs comprise a large number of unusual genetic disorders, and may be considered a diagnostic challenge. Genetics has a significant role to play in the etiology, and up to now, approximately 40 SCA subtypes have been described, and at least 32 different loci were identified [1]. As the knowledge of clinical and genetic features of SCAs is growing, the "What type of SCA should this patient tested for?" question becomes important before ordering a SCA diagnostic panel.

Rare brainstem oligodendroglioma in an adult patient: Presentation, molecular characteristics and treatment response

Sarah D. Hodges, Patrick Malafronte, Jonathan Gilhooly, William Skinner, Corey Carter, Brett J. Theeler

World Health Organization (WHO) Grades II, III, and IV brainstem gliomas are rare in adults, accounting for < 2% of intracranial gliomas. This is in contradistinction to the pediatric population where brainstem gliomas make up 20% of glial neoplasms. Adult brainstem gliomas appear to have a better prognosis compared to pediatric cases with an average survival of 30–40 months in adults compared to 10–12 months in pediatrics. Oligodendrogliomas represent 1.7% of all brain tumors and anaplastic oligodendrogliomas account for only 0.5% [1]. 

A case of hypertensive brainstem encephalopathy presenting with severe headache and unilateral hearing loss

Jung-Ju Lee, Soohyun Cho, Jong-Moo Park, Kyung-Il Park

Hypertensive encephalopathy usually presents as posterior reversible encephalopathy syndrome (PRES), which is characterized by symmetrical vasogenic edema in the parieto-occipital areas where sympathetic innervation is relatively deficient compared with other areas [1,2]. The underlying pathological mechanism of PRES is the breakdown of cerebral autoregulation and endothelial dysfunction [2]. However, several reports have described a rare (about 4%) variant of PRES localized to the cerebellum and brainstem, rather than the posterior cerebrum [3–5].

Rapidly progressive dementia with false-positive PCR Tropheryma whipplei in CSF. A case of Hashimoto's encephalopathy

R. Robles-Cedeño, J. Gich, D. Genís Batlle, Ll. Ramió-Torrentà

Hashimoto's encephalopathy (HE) is a rare neurological syndrome of unknown aetiology coursing with encephalopathy associated to elevated titters of anti-thyroid autoantibodies [1]. It was first described by Brain et al. in 1966 [2] in reporting a patient affected by an autoimmune thyroid disease who had recurrent stroke-like episodes that appeared independently of the thyroid status.

Aggressive Garcin's syndrome by acquired cystic disease of kidney-related renal cell carcinoma in a long-term hemodialytic patient

Yuko Kawahara, Kentaro Deguchi, Kota Sato, Nozomi Hishikawa, Syoichiro Kono, Yasuyuki Ohta, Toru Yamashita, Eiko Hayashi, Yasuharu Sato, Koji Abe

Garcin's syndrome is characterized by the presence of unilateral palsies of the cranial nerves, but not by sensory or motor long tract disturbances, intracranial hypertension, or abnormal skull base radiographs [1]. Our patient revealed Garcin's syndrome by skull base metastasis of ACDK-related RCC, which is a known unique carcinoma that occurs in patients with long-term HD [2]. We report a pathological case unveiled by autopsy.

Posterior reversible encephalopathy syndrome treated with renin–angiotensin system blockade

Eikan Mishima, Junichiro Hashimoto, Yasutoshi Akiyama, Kazumasa Seiji, Kei Takase, Takaaki Abe, Sadayoshi Ito

Posterior reversible encephalopathy syndrome (PRES) is characterized by reversible cerebral vasogenic edema, occurred predominantly in the parieto-occipital region and less commonly in the brainstem [1]. PRES has been associated with various conditions including hypertension, but its pathogenesis is still unclear. Furthermore, although controlling blood pressure is recommended for the treatment of PRES [1], differences in the treatment efficacy among types of antihypertensive therapies have been poorly investigated.