JNS-cover.jpgJournal of Neurological Sciences

Vol 353 | No. 1-2 | 15 June 2015 | Pages 1-192


Magnetization transfer MRI in dementia disorders, Huntington's disease and parkinsonism

Nicola Tambasco, Pasquale Nigro, Michele Romoli, Simone Simoni, Lucilla Parnetti, Paolo Calabresi

Magnetic resonance imaging is the most used technique of neuroimaging. Using recent advances in magnetic resonance application it is possible to investigate several changes in neurodegenerative disease. Among different techniques, magnetization-transfer imaging (MTI), a magnetic resonance acquisition protocol assessing the magnetization exchange between protons bound to water and those bound to macromolecules, is able to identify microstructural brain tissue changes peculiar of neurodegenerative diseases.

Parkinson's disease and intensive exercise therapy – a systematic review and meta-analysis of randomized controlled trials

Anders Uhrbrand, Egon Stenager, Martin Sloth Pedersen, Ulrik Dalgas

To evaluate and compare the effect of 3 intensive exercise therapy modalities - Resistance Training (RT), Endurance Training (ET) and Other Intensive Training Modalities (OITM) - in Parkinson's Disease (PD). Design A systematic review and meta-analysis of randomized controlled trials.

Hemispheric differences in malignant middle cerebral artery stroke

Vaibhav Rastogi, Damon G. Lamb, John B. Williamson, Thor S. Stead, Rachel Penumudi, Sharathchandra Bidari, Latha Ganti, Kenneth M. Heilman, Vishnumurthy S. Hedna

We recently reported that left versus right hemisphere cerebral infarctions patients more frequently have worse outcomes. However our clinical experience led us to suspect that the incidence of malignant middle cerebral artery infarctions (MMCA) was higher in the right compared to the left hemispheric strokes.


Original Articles

Multitarget intervention of Fasudil in the neuroprotection of dopaminergic neurons in MPTP-mouse model of Parkinson's disease

Yong-fei Zhao, Qiong Zhang, Jian-ying Xi, Yan-hua Li, Cun-gen Ma, Bao-guo Xiao

Recent studies have demonstrated that activation of the Rho-associated kinase (ROCK) pathway participates in the dopaminergic neuron degeneration and possibly in Parkinson's disease (PD). In the current study, we tried to observe the therapeutic potential of ROCK inhibitor Fasudil against dopaminergic neuron injury in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-mouse model of PD, and explore possible molecular mechanisms by enzyme-linked immunosorbent assay (ELISA), western blot and immunofluorescent assays.

Direct observation during surgery shows preservation of cerebral microcirculation in patients with traumatic brain injury

Jon Pérez-Bárcena, Eduardo Romay, Juan Antonio Llompart-Pou, Javier Ibáñez, Marta Brell, Pedro Llinás, Elsa González, Amedeo Merenda, Can Ince, Ross Bullock

To describe the alterations of the cortical microcirculation of the brain (blood flow and vessel density) in TBI patients who and compare them with a control group.

Subcortical whiter matter hyperintensities within the cholinergic pathways of patients with dementia and parkinsonism

Hyung-Eun Park, In-Seok Park, Yoon-Sang Oh, Dong-Won Yang, Kwang-Soo Lee, Hyun-Seok Choi, Kook-Jin Ahn, Joong-Seok Kim

White matter hyperintensities (WMHs) in the cholinergic pathways are associated with cognitive performance in Alzheimer's disease (AD) and Parkinson disease dementia (PDD). This study aimed to evaluate the relationship between loss of white matter cholinergic pathways and cognitive function in patients with AD, diffuse Lewy body disease (DLB), and PDD.

Parkinson's disease: disturbed vestibular function and levodopa

Brian J. Lithgow, Mehrnaz Shoushtarian

Evidence indicates Levodopa effects central postural control. As electrophysiological postural control biomarkers, sensory oto-acoustic features were extracted from Electrovestibulography (EVestG) data to identify 20 healthy age and gender matched individuals as Controls from 20 PD subjects before (PDlowmed) and 18 after (PDmed) morning doses of Levodopa. EVestG data was collected using a single tilt stimulus applied in the pitch plane. The extracted features were based on the measured firing pattern, interval histogram and the shape of the average field potential response.  

Identification of novel variants in LRRK2 gene in patients with Parkinson's disease in Serbian population

Milena Z. Janković, Nikola D. Kresojević, Valerija S. Dobričić, Vladana V. Marković, Igor N. Petrović, Ivana V. Novaković, Vladimir S. Kostić

Mutations in LRRK2 (leucine-rich repeat kinase 2) are the most common cause of autosomal dominant Parkinson's disease (PD). Large international studies have revealed that pathogenic mutations are clustered in several exons coding for functional domains of LRRK2 protein, but the mutation frequency differs among populations. Systematic study of LRRK2 mutation prevalence and phenotype in Serbian population has not been performed.

Increase in oxidative stress biomarkers in dogs with ascending–descending myelomalacia following spinal cord injury

Andrew Marquis, Rebecca A. Packer, Richard B. Borgens, Bradley S. Duerstock

Multiple biochemical and immunohistochemical tests were performed to elucidate the role of oxidative stress during ascending–descending (A–D) myelomalacia by comparing dogs with this progressive terminal condition to dogs with chronic, focal spinal cord injuries (SCIs) and controls without SCI. Dogs with A–D myelomalacia exhibited increased biochemical markers for oxidative stress, including 8-isoprostane F2α and acrolein, as well as decreased endogenous glutathione with greatest changes occurring at the lesion center.

A single preoperative dose of diclofenac reduces the intensity of acute postcraniotomy headache and decreases analgesic requirements over five postoperative days in adults: A single center, randomized, blinded trial

Csilla Molnár, Éva Simon, Ágota Kazup, Judit Gál, Levente Molnár, László Novák, Dániel Bereczki, Daniel I. Sessler, Béla Fülesdi

Postcraniotomy headache causes considerable pain and can be difficult to treat. We therefore tested the hypothesis that a single 100-mg preoperative dose of diclofenac reduces the intensity of postcraniotomy headache, and reduces analgesic requirements.

Diurnal drooling in Chinese patients with Parkinson's disease

Ruwei Ou, Xiaoyan Guo, Qianqian Wei, Bei Cao, Jing Yang, Wei Song, Ke Chen, Bi Zhao, Xueping Chen, Huifang Shang

The aim of this study is to explore the prevalence and clinical correlates of diurnal drooling in Chinese patients with Parkinson's disease (PD).

Clinically isolated syndrome — Rethinking the diagnosis

Jagannadha R. Avasarala

A diagnostic lacuna in clinically isolated syndrome (CIS) is the prognostic uncertainty of approximately 20% of patients who do not meet the radiological criteria based on magnetic resonance imaging (MRI) of the brain. A study by Tintore et al. noted that 23% of patients who had normal MRI findings but had oligoclonal bands (OCBs) in cerebrospinal fluid (CSF) developed clinically definite multiple sclerosis (CDMS), compared with 4% who had normal MRI findings but no OCBs in CSF (Tintore et al. 2008).

Extreme delta brush guides to the diagnosis of anti-NMDAR encephalitis

Jianwen Wang, Kang Wang, Dengchang Wu, Hui Liang, Xuning Zheng, Benyan Luo

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is a treatable but often misdiagnosed autoimmune encephalitis. Diagnosis depends on NMDAR antibody testing, which may not be readily available. Alternatively, the electroencephalogram (EEG) extreme delta brush pattern may provide a valuable immediate indicator for the diagnosis of anti-NMDAR encephalitis. A 32-year-old female (case 1) presented with fever, headache, behavioral changes, confusion, intractable seizures, central hypoventilation, dysautonomia, facial and limb dyskinesias, and comorbid ovarian teratoma.

A case of late-onset riboflavin responsive multiple acyl-CoA dehydrogenase deficiency (MADD) with a novel mutation in ETFDH gene

Zhihong Zhuo, Peina Jin, Fengyan Li, Haiying Li, Xiaoxin Chen, Huaili Wang

We report a novel mutation in the electron transfer flavoprotein dehydrogenase (EFTDH) gene in an adolescent Chinese patient with late-onset riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency (MADD) characterized by muscle weakness as early symptom. At the age of 9 years, the patient experienced progressive muscle weakness. Blood creatine kinase level and aminotransferase were higher than normal. The muscle biopsy revealed lipid storage myopathy. Serum acylcarnitine and urine organic acid analyses were consistent with MADD.

Relationship between the non-motor items of the MDS–UPDRS and Quality of Life in patients with Parkinson's disease

Matej Skorvanek, Jaroslav Rosenberger, Michal Minar, Milan Grofik, Vladimir Han, Johan W. Groothoff, Peter Valkovic, Zuzana Gdovinova, Jitse P. van Dijk

The Movement Disorder Society–Unified Parkinson's Disease Rating Scale (MDS–UPDRS) is a newly developed comprehensive tool to assess Parkinson's disease (PD), which covers a wider range of non-motor PD manifestations than the original UPDRS scale. The aim of this study was to assess the relationship between the MDS–UPDRS and Quality of Life (QoL) and to analyze the relationship between individual MDS–UPDRS non-motor items and QoL. A total of 291 PD patients were examined in a multicenter Slovak study.        

Adult brainstem gliomas: Correlation of clinical and molecular features

Brett J. Theeler, Benjamin Ellezam, Isaac Melguizo-Gavilanes, John F. de Groot, Anita Mahajan, Kenneth D. Aldape, Janet M. Bruner, Vinay K. Puduvalli

Brainstem gliomas are rare in adults and overall have superior survival outcomes compared to pediatric brainstem gliomas.

Hypoglycemia with focal neurological signs as stroke mimic: Clinical and neuroradiological characteristics

Tomohiko Ohshita, Eiji Imamura, Eiichi Nomura, Shinichi Wakabayashi, Hiroshi Kajikawa, Masayasu Matsumoto

Our aim was to investigate the clinical and radiological features of patients with hypoglycemia with focal neurological signs (HFNS). Among 80 consecutive hypoglycemic patients (blood glucose levels less than 50 mg/dL), who had been admitted between October 2008 and May 2012, we selected 11 patients (6 men and 5 women; mean age, 73.2 ± 12 years) with focal neurological signs. The mean initial blood glucose level was 27.9 mg/dL (range, 13–39 mg/dL). The most frequent symptom was unilateral motor weakness (n = 9), which was usually accompanied with mild or moderate alteration of consciousness. 

Chronic manganism: A long-term follow-up study with a new dopamine terminal biomarker of 18F-FP-(+)-DTBZ (18F-AV-133) brain PET scan

Chu-Yun Huang, Chi-Hung Liu, Eusden Tsao, Chia-Ju Hsieh, Yi-Hsin Weng, Ing-Tsung Hsiao, Tzu-Chen Yen, Kun-Ju Lin, Chin-Chang Huang

Recent experimental studies revealed that dopamine neuron dysfunction in chronic manganism may be due to a reduced capacity of dopamine release in the striatum. The findings imposed further difficulty in the differential diagnosis between manganism and IPD. We conducted a long-term clinical follow-up study of 4 manganism patients, applying a new tracer 18F-9-fluoropropyl-(+)-dihydrotetrabenazine (18F-AV-133) with positron emission tomography (PET). Twenty age-matched subjects including 4 manganism patients, 8 idiopathic Parkinson's disease (IPD) patients, and 8 healthy controls were enrolled for comparison.

Incidence and prevalence of pediatric onset multiple sclerosis in Kuwait: 1994–2013

R. Alroughani, S. Akhtar, S.F. Ahmed, R. Behbehani, J. Al-Abkal, J. Al-Hashel

This study aimed to assess the incidence and prevalence of pediatric-onset multiple sclerosis (POMS) along with temporal and gender differentials in these estimates in Kuwait.

Gender and age predict outcomes of cognitive, balance and vision testing in a multidisciplinary concussion center

Peter A. Benedict, Natali V. Baner, G. Kyle Harrold, Nicholas Moehringer, Lisena Hasanaj, Liliana P. Serrano, Mara Sproul, Geraldine Pagnotta, Dennis A. Cardone, Steven R. Flanagan, Janet Rucker, Steven L. Galetta, Laura J. Balcer

This study examined components of the Sports Concussion Assessment Tool, 3rd Edition (SCAT3) and a vision-based test of rapid number naming (King–Devick [K-D]) to evaluate sports and non-sports concussion patients in an outpatient, multidisciplinary concussion center. While the Symptom Evaluation, Standardized Assessment of Concussion (SAC), modified Balance Error Scoring System (BESS), and K-D are used typically for sideline assessment, their use in an outpatient clinical setting following concussion has not been widely investigated.

Serious head trauma preceding essential tremor: A population-based study (NEDICES)

Julián Benito-León, Elan D. Louis, Andrés Labiano-Fontcuberta, Félix Bermejo-Pareja

An association between head trauma and essential tremor (ET) has not been formally assessed. Our purpose was to assess the association between serious head trauma and ET.

Serial measurements of phosphorylated neurofilament-heavy in the serum of subjects with amyotrophic lateral sclerosis

P.A. McCombe, C. Pfluger, P. Singh, C.Y.H. Lim, C. Airey, R.D. Henderson
  • DOI: http://dx.doi.org/10.1016/j.jns.2015.04.032
  • p122–129
  • Published online: April 29, 2015 (Open Access)
  • Abstract
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There is a need for a blood biomarker of disease activity in ALS. This marker needs to measure the loss of motor neurones. Phosphorylated neurofilament heavy chain (pNfH) in the serum is a biomarker of axonal injury. Previous studies have found that levels of pNfH are elevated in ALS. We have performed a serial study of pNfH levels in 98 subjects from our ALS clinic. There was significant elevation of levels of pNfH in subjects with ALS compared to controls, although there was considerable variability.

Gait training in subacute non-ambulatory stroke patients using a full weight-bearing gait-assistance robot: A prospective, randomized, open, blinded-endpoint trial

Mitsuhiro Ochi, Futoshi Wada, Satoru Saeki, Kenji Hachisuka

This study was a prospective, randomized, open, blinded-endpoint trial with the aim of examining whether gait training with a gait-assistance robot (GAR) improves gait disturbances in subacute non-ambulatory hemiplegic stroke patients more than overground conventional gait training. The GAR adopts a robot arm control system with full weight bearing and foot pressure visual biofeedback.

Positive correlation between care given by specialists and registered nurses and improved outcomes for stroke patients

Kyu-Tae Han, Sun Jung Kim, Sung-In Jang, Seung Ju Kim, Seo Yoon Lee, Hyo Jung Lee, Eun-Cheol Park

Cerebrovascular diseases are the second-highest cause of death in South Korea (9.6% of all causes of mortality in 2013). South Korea has a shortage of trained medical personnel compared with other countries and the demands for health care are continuously increasing. Our study sought to determine the relationship between hospital human resources and the outcomes of stroke patients.

Link between cerebral blood flow and autonomic function in survivors of internal carotid artery occlusion

Ya-Ting Chang, Wen-Neng Chang, Nai-Wen Tsai, Chih-Cheng Huang, Hung-Chen Wang, Chia-Te Kung, Yu-Jih Su, Wei-Che Lin, Hsueh-Wen Chang, Ben-Chung Cheng, Chih-Min Su, Yi-Fang Chiang, Cheng-Hsien Lu

Symptomatic internal carotid artery occlusion (ICAO) is an important cause of cerebral ischemia with poor long-term outcome. Reductions in baroreflex function is reported in carotid atherosclerosis and implicated in increased risk of recurrent cardiovascular events. A distributed network of forebrain regions can exert modulatory influences over the cardio-vagal and baroreflex functions. The successful clinical translation of these approaches offers insights into underlying modulatory mechanisms and to possible therapeutic strategy.

Exome analysis identified a novel missense mutation in the CLPP gene in a consanguineous Saudi family expanding the clinical spectrum of Perrault Syndrome type-3

Saleem Ahmed, Musharraf Jelani, Nuha Alrayes, Hussein Sheikh Ali Mohamoud, Mona Mohammad Almramhi, Wasim Anshasi, Naushad Ali Basheer Ahmed, Jun Wang, Jamal Nasir, Jumana Yousuf Al-Aama

Perrault syndrome (PRLTS) is a clinically and genetically heterogeneous disorder. Both male and female patients suffer from sensory neuronal hearing loss in early childhood, and female patients are characterized by premature ovarian failure and infertility after puberty. Clinical diagnosis may not be possible in early life, because key features of PRLTS, for example infertility and premature ovarian failure, do not appear before puberty. Limb spasticity, muscle weakness, and intellectual disability have also been observed in PRLTS patients.


Short Communications

Rituximab treatment did not aggravate ongoing progressive multifocal leukoencephalopathy in a patient with multiple sclerosis

F. Asztely, E. Gilland, M.P. Wattjes, J. Lycke

A multiple sclerosis (MS) patient developed progressive multifocal leukoencephalopathy (PML) after 43 months of natalizumab treatment. New clinical and magnetic resonance imaging (MRI) findings were initially misinterpreted as breakthrough MS disease activity and natalizumab treatment was replaced by rituximab treatment. The patient had a single infusion of rituximab 1000 mg before a definite PML diagnosis was confirmed. Despite undetectable levels of B-cells, JC virus DNA became undetectable in the cerebrospinal fluid by quantitative polymerase chain reaction.

Gaze palsy and exotropia in internuclear ophthalmoplegia

Ken Johkura, Yosuke Kudo, Yu Amano, Hideyuki Kikyo, Ryoko Imazeki, Kazumitsu Amari, Masahiro Yamamoto

Unilateral gaze palsy associated with internuclear ophthalmoplegia (INO), i.e., one-and-a-half syndrome, is well known. Exotropia can also be associated with INO, but it has been reported only rarely. We sought to determine the frequencies and courses of gaze palsy and exotropia in INO.

Mutation genotypes of RNF213 gene from moyamoya patients in Taiwan

Ming-Jen Lee, Ya-Fang Chen, Pi-Chuan Fan, Kuo-Chuan Wang, Kai Wang, Jinyuan Wang, Meng-Fai Kuo

Moyamoya disease (MMD) is a disorder characterized by stenosis of bilateral internal carotid arteries with compensatory angiogenesis of the perforating blood vessels. Familial transmission in MMD is common. Recently, mutations in human RNF213 and ACTA2 genes were identified to be responsible for MMD. The present study was to determine whether Taiwanese MMD patients carried mutations in these two genes. Of the 36 MMD patients, eleven was found to have RNF213 mutations. Direct genetic sequencing identified four different RNF213 mutations in the 11 patients from 8 families: five with a p.R4810K, one with p.A1622V, one with p.V3933M, and the other one with p.R4131C.

Disruption of melatonin circadian rhythm production is related to multiple sclerosis severity: A preliminary study

Alfredo Damasceno, Adriel Santos Moraes, Alessandro Farias, Benito Pereira Damasceno, Leonilda Maria Barbosa dos Santos, Fernando Cendes

Sunlight can influence the immune system independently of vitamin D, such as through melatonin production in the pineal gland. Inflammatory disorders can suppress nocturnal melatonin production, but only a few studies have investigated melatonin status in multiple sclerosis (MS). We aimed to study melatonin production in association with clinical and immunological data in MS patients. Eleven treated relapsing-remitting MS (RRMS) patients and eight controls underwent neurological examination and were assessed for fatigue severity and depressive symptoms.   


Case Report

A unique radiological case of intrathecal methotrexate-induced toxic leukoencephalopathy

Jiwon Yang, Gap Su Kim, Hyeon-Mi Park

Methotrexate (MTX) is a necessary antimetabolite for the treatment of oncological disorders in all age groups. It was first introduced in 1948 for treatment of childhood lymphocytic leukemia [1]. Intrathecal methotrexate is used for prophylaxis and treatment of meningeal leukemia. Neurological adverse effects of methotrexate are not uncommon. Chronic leukoencephalopathy is the most serious neurotoxicity, particularly when it is administered intrathecally in combination with cranial radiation therapy, and is characterized by insidious evolution of dementia, pseudobulbar palsy or ataxia with widespread white matter changes.


Letters to the Editor

Extraction of tumour embolus following perioperative stroke

Stella E. Hughes, Annemarie Hunter, Jamie Campbell, Aidan Brady, Brian Herron, Graham Smyth, Ian Rennie, Stephen J. Hunt

We would like to highlight the case of a 69-year old man who was found to have a rare cause of perioperative stroke. The patient was diagnosed with primary adenocarcinoma of the lung, with no evidence of metastases on staging PET CT. His only past medical history was of hypertension. He underwent resection of the left upper lobe tumour. During the six-hour operation, hypotension or hypoxia was not encountered but supraventricular tachycardia was noted both intra- and post-operatively. In the recovery ward, he was noted to have new left-sided limb weakness.

Post-therapy normalization of brain FDG-PET in Morvan's syndrome

L. Benedetti, D. Franciotta, M. Zoccarato, A. Beronio, M. Godani, E. Schirinzi, G. Siciliano, A. Ciarmiello, M. Del Sette

Morvan's syndrome (MoS) is a very rare disorder that manifests with overlapping symptoms involving the CNS, and peripheral nerve hyperexcitability. Historically, MoS had been associated with antibodies to the complex of voltage gated potassium channels (VGKC) [1], which have been recently classified according to subspecificities, so that contactin-associated protein-2 (CASPR-2), leucine-rich glioma inactivated-1, and contactin-2 result the three target proteins of the autoimmune response in MoS [2,3].

Glycine receptor antibodies and progressive encephalomyelitis with rigidity and myoclonus with predominant motor neuron degeneration — Expanding the clinical spectrum

J. Kenda, V. Švigelj, Z. Rodi, B. Koritnik, F. Graus, M. Kojović

The classical phenotype of glycine receptor (GlyR) antibody-associated progressive encephalomyelitis with rigidity and myoclonus (PERM) consists of axial and limb stiffness with stimulus sensitive myoclonus and brain stem signs [1]. We report a patient with PERM who presented with clinical and electrophysiological features of motor neuron disease (MND) and in whom motor neuron involvement continued to progress even after remission of PERM on immunotherapy.

Lymphoma presenting with choroid plexus infiltration and periventricular dissemination

Motohiro Suzuki, Zen Kobayashi, Takayoshi Ito, Miho Akaza, Hiroyuki Tomimitsu, Shuzo Shintani

Choroid plexus infiltration of systemic lymphoma is rare, and its MRI has not been reported previously. A 71-year-old male presented with a 1-month history of cognitive impairment. His past medical history included B-cell type lymphoma involving the adrenal glands, retroperitoneal space, and bones at the age of 68, which was successfully treated with R-CHOP chemotherapy. Diffusion-weighted brain MRI demonstrated high signal intensity areas in the center of the lateral ventricles and anterior periventricular sites (Fig. 1a–c).

Gastrointestinal symptoms in late-onset Pompe disease: Early response to enzyme replacement therapy

Julio Pardo, Tania García-Sobrino, Ana López-Ferreiro

Pompe disease is an autosomal recessive disorder caused by deficiency of the lysosomal enzyme acid alpha glycosidase (GAA) due to mutations in the GAA gene. The main clinical and prognostic features in late-onset Pompe disease (LOPD) are proximal and axial pareses in the limb girdles and respiratory insufficiency due to glycogen storage in the skeletal muscles [1].

A case of papilloedema associated with Chiari I malformation

Andrea Rigamonti, Giuseppe Lauria, Vittorio Mantero, Francesca Piamarta, Michele Parolin, Andrea Salmaggi

Chiari I malformation (CIM) is a rare congenital malformation of the hindbrain manifesting as herniation of the cerebellar tonsils below the foramen magnum [1]. It usually becomes symptomatic in late childhood or early adulthood. Patients may complain of headache or symptoms related to cranial nerve compression, cerebellar dysfunction, myelopathy and syringomyelia [1].

Selective disappearance of medial back muscles in a case of myotonic dystrophy type 1

Ryuta Morihara, Nozomi Hishikawa, Toru Yamashita, Kentaro Deguchi, Tomoko Kurata, Koji Abe

Here, we report a unique case of late-onset myotonic dystrophy type 1 in a 64-year-old woman, with selective disappearance of the medial lower back muscles. We compared the clinical features of this patient with those of a cohort of 29 patients with myotonic dystrophy type 1 to clarify the correlation between clinical features and lower back muscle atrophy. After classification into three subgroups according to muscle atrophy pattern, medial muscle atrophy was present in 17.2% of the patients. Affected patients were older at onset than non-affected patients, and limb muscle power and respiratory function decreased with atrophy progression.

Lingual dystonia as a new clinical feature in corticobasal syndrome

Evangelia Giza, Zoe Katsarou, Ioannis Dagklis, Sevasti Bostantjopoulou

Corticobasal degeneration (CBD) is a clinicopathological entity with characteristic pathological features, but is clinically heterogeneous, associated with various clinical phenotypes [1]. The classical phenotype originally described for CBD is now referred as corticobasal syndrome (CBS) and is characterized by asymmetric levodopa-resistant parkinsonism, dystonia, and myoclonus accompanied by higher cortical signs (i.e., apraxia, alien limb phenomena, cortical sensory loss and cognitive impairment) [1,2].

Anti-N-methyl-d-aspartate receptor encephalitis: Should psychiatrists shoulder the responsibility for a non-psychiatric disorder?

Huei-Sing Chang, Pan-Ming Chen, Ti Lu, Li-Yu Hu

Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis was formally recognized in 2007 as a specific type of autoimmune encephalitis [1]. Epidemiological studies have increasingly identified this entity as a significant cause of autoimmune encephalitis. This disease is most common in young female patients with ovarian teratomas; however, it has been reported in patients of both sexes ranging in age from less than 1 to over 90 years [2]. This type of encephalitis is notorious for its multi-stage presentation of clinical symptoms, which may delay diagnosis.

Neuromyelitis optica overlapping systemic sclerosis with anti-centromere antibodies

Kota Moriguchi, Kenichi Kaida, Naohiko Togashi, Hiroyuki Onoue, Katsunori Ikewaki

Neuromyelitis optica (NMO) is characterized by positivity for anti-aquaporin 4 (AQP4) antibody and is an idiopathic demyelinating disease associated with various systemic autoimmune diseases such as Sjögren syndrome (SS) and systemic lupus erythematosus [1]. It has been proposed that optic neuritis or longitudinal extensive transverse myelitis associated with systemic autoimmune disease should be categorized as NMO spectrum disorder (NMOSD) [2]. Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by excess collagen deposition in the skin and internal organs, [3] and is occasionally reported in cases of NMO/NMOSD [4–6].