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Journal of the Neurological Sciences
JNS Vol 360 | Jan 2016

JNS-cover.jpgJournal of Neurological Sciences

Vol 360 | 15 January 2016 | Pages 1-288

Review Articles

Retinoids and motor neuron disease: Potential role in amyotrophic lateral sclerosis

Javier Riancho, Maria T. Berciano, Maria Ruiz-Soto, Jose Berciano, Gary Landreth, Miguel Lafarga

Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease affecting motor neurons (MN). This fatal disease is characterized by progressive muscular atrophy and unfortunately it does not have an effective treatment. Although a small proportion of ALS cases have a familiar origin, the vast majority of them are thought to have a sporadic origin. Although the pathogenesis of ALS has not been fully elucidated, various disorders in different cellular functions such as gene expression, protein metabolism, axonal transport and glial cell disorders have been linked to MN degeneration.

Emergence of exosomal miRNAs as a diagnostic biomarker for Alzheimer's disease

Vo Van Giau, Seong Soo A. An

Alzheimer's disease (AD) is the most common progressive degenerative disorder, and is characterized by memory loss and cognitive decline. It is a complex disorder with both environmental and genetic components. Current diagnosis of AD is based primarily on the analysis of the patient's cognitive function using imaging techniques and the biochemical analyses of bodily fluids. Efforts have been made to develop not only an effective therapeutic, but also a diagnostic capable of identifying AD before the onset of irreversible neurological damage.

 

Basic Research Papers

Cognitive and psychiatric comorbidities in neuromyelitis optica

Perry Moore, Abigail Methley, Catherine Pollard, Kerry Mutch, Shahd Hamid, Liene Elsone, Anu Jacob

Our primary objective was to examine the neuropsychological and psychopathological profile of patients with neuromyelitis optica (NMO) and compare these to multiple sclerosis (MS) and healthy control (HC) groups. We also examined for relationships between cognitive and psychiatric variables and clinical factors including accumulated neurological disability and disease duration.

Controlled release of osteopontin and interleukin-10 from modified endovascular coil promote cerebral aneurysm healing

Jingyi Chen, Lijun Yang, Yan Chen, Gengshen Zhang, Zheneng Fan

Cerebral aneurysm is a bulging of the artery inside the brain that results from a weakened or thin area of the artery wall. Ruptured cerebral aneurysm could lead to serious brain damage or even death, thus the proper treatment is essential. Compared with the conventional microsurgical clipping approach, the endovascular coiling treatment has many advantages, however, with a major disadvantage of high recurrence rate. One way to lower the recurrence rate, which has been tried since one decade ago, is to modify the coil to be bioactive and releasing biological molecules to stimulate tissue ingrowth and aneurysm healing.

Ultrasound evaluation of internal jugular valve incompetence (IJVI) in Egyptian patients with idiopathic intracranial hypertension (IIH)

Ibtessam M. Fahmy, Noha T. Abokrysha, Sandra M. Ahmed, Haidy M. El-Shebawy

Idiopathic intracranial hypertension (IIH) is a clinical syndrome with no identified causative factor. Internal jugular valve incompetence (IJVI) has been linked to many neurological disorders such as idiopathic intracranial hypertension (IIH), transient global amnesia and cough-induced headache. Intact valves prevent efficiently retrograde flow into the internal jugular vein.

Increased expression of copine VI in patients with refractory epilepsy and a rat model

Binglin Zhu, Jingsi Zha, Yan Long, Xiaotong Hu, Guojun Chen, Xuefeng Wang

Copine VI (CPNE6) is a member of copines family, a calcium-dependent phospholipids-binding protein group found in many diverse eukaryotic organisms. Although earlier studies have shown that CPNE6 is almost exclusively expressed in brain, the exact biological functions remain unclear. The purpose of this study is to explore the relationship between epilepsy and CPNE6 expression. In present study, we investigated the expression pattern and distribution of CPNE6 in patients with refractory epilepsy and in a chronic pilocarpine-induced epileptic rat model by quantitative real-time PCR, Western blot and immunofluorescence.

Intrathecal lipid-specific oligoclonal IgM synthesis associates with retinal axonal loss in multiple sclerosis

José C. Álvarez-Cermeño, Francisco J. Muñoz-Negrete, Lucienne Costa-Frossard, Susana Sainz de la Maza, Luisa M. Villar, Gema Rebolleda

It has been suggested that autoantibodies may induce axonal damage in multiple sclerosis (MS). Optical coherence tomography (OCT) showed that thinning of peripapillary retinal nerve fiber layer (RNFL) and ganglion cell layer/inner plexiform (GCIPL) measurements reflect axonal loss in the disease. We investigated whether the intrathecal synthesis of lipid-specific oligoclonal IgM bands (LS-OCMB) associates with thinning of these structures in MS patients.

Periostin activates pathways involved in epithelial–mesenchymal transition in adamantinomatous craniopharyngioma

Ming Chen, Shi-hao Zheng, Yi Liu, Jin Shi, Song-tao Qi

Periostin (POSTN) is an extracellular matrix protein (ECM) critical for epithelial–mesenchymal transitions (EMT) in several kinds of tumor cells. Previous studies have indicated that EMT exists in craniopharyngioma (CP), and expression of POSTN is a significant factor in the prognosis of CP. However, it has never been explored whether POSTN exists in CP, or how it activates CP's EMT. The expression of POSTN was examined in adamantinomatous craniopharyngioma (ACP) primary cells and tissues by immunohistochemistry, PCR and Western blot, respectively.

Drugs indicated for mitochondrial dysfunction as treatments for acute encephalopathy with onset of febrile convulsive status epileptics

Taku Omata, Katsunori Fujii, Jun-ichi Takanashi, Kei Murayama, Masaki Takayanagi, Kaori Muta, Kazuo Kodama, Yukiko Iida, Yoshimi Watanabe, Naoki Shimojo
  • DOI: http://dx.doi.org/10.1016/j.jns.2015.11.043
  • p57–60
  • Published online: November 24 2015Open Access
  • Abstract
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We studied the efficacy of drugs indicated for mitochondrial dysfunction in the treatment of 21 patients with acute encephalopathy with onset of febrile convulsive status epilepticus at our hospital from January 2006 to December 2014. Among them, 11 patients had been treated with a mitochondrial drug cocktail consisting of vitamin B1, vitamin C, biotin, vitamin E, coenzyme Q10, and l-carnitine (prescription group) and 10 patients were not treated with the cocktail (non-prescription group). We retrospectively reviewed age, trigger, clinical form, treatment start time, and sequelae.   

JC virus seroprevalence and seroconversion in multiple sclerosis cohort: A Middle-Eastern study

Raed Alroughani, Saeed Akhtar, Samar F. Ahmed, Samia J. Khoury, Jasem Y. Al-Hashel, Mohammad Ali. Sahraian, Mohammed Al Jumah, Maya Zeineddine, Sahar Farhat, Hassan Doumiati, Bassem I. Yamout

To estimate JCV seroprevalence and risk of seroconversion against JCV among MS patients in the Middle East.

Cabazitaxel causes a dose-dependent central nervous system toxicity in rats

Ergun Karavelioglu, Yucel Gonul, Hasan Aksit, Mehmet Gazi Boyaci, Mustafa Karademir, Nejdet Simsek, Mustafa Guven, Tugay Atalay, Usame Rakip

Chemotherapeutic agents may lead to serious neurological side effects, which in turn can deteriorate the quality of life and cause dose limiting. Direct toxic effect or metabolic derangement of chemotherapeutic agents may cause these complications. Cabazitaxel is a next generation semi-synthetic taxane derivative, which is effective in both preclinical models of human tumors sensitive or resistant to chemotherapy and in patients with progressive prostate cancer despite docetaxel treatment.

Progression of non-motor symptoms in Parkinson's disease among different age populations: A two-year follow-up study

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

Non-motor symptoms (NMS) are gaining increasing relevance in the management of Parkinson's disease (PD), but little is known about their progression, especially among patients with different ages.

Functional characterization of two novel mutations in TTF-1/NKX2.1 homeodomain in patients with benign hereditary chorea

Claudia Provenzano, Michela Zamboni, Liana Veneziano, Elide Mantuano, Barbara Garavaglia, Giovanna Zorzi, Javier Pagonabarraga, Paola Giunti, Donato Civitareale

The thyroid transcription factor 1 (TTF-1) is encoded, on chromosome 14q13, by the gene termed TITF-1/NKX2.1. Mutations in this gene have been associated with chorea, hypothyroidism, and lung disease, all included in the "brain–thyroid–lung syndrome." We here describe two cases of novel missense mutations [NM_003317.3:c.516G > T and c.623G > C resulting in p.(Gln172His) and p.(Trp208Ser), respectively] in TITF-1/NKX2-1 in non-consanguineous patients. We provide a functional study of the role of the two mutations on the TTF-1 ability to bind DNA and to trans-activate both thyroid and lung specific gene promoters.

Linear lesions may assist early diagnosis of neuromyelitis optica and longitudinally extensive transverse myelitis, two subtypes of NMOSD

Wei Cai, Sha Tan, Lei Zhang, Yilong Shan, Yanqiang Wang, Yinyao Lin, Fangjing Zhou, Bingjun Zhang, Xiaoyu Chen, Li Zhou, Yuge Wang, Xuehong Huang, Xuejiao Men, Haiyan Li, Wei Qiu, Xueqiang Hu, Zhengqi Lu

Purpose: To investigate the relationship between linear lesions (LL) and the development of longitudinally extensive spinal cord lesions (LESCL) in Chinese patients with neuromyelitis optica or longitudinally extensive transverse myelitis. Method: The clinical records of 143 patients with these conditions were reviewed. Forty-one patients with LL were divided into three groups according to the order of appearance of LL and LESCL (simultaneously [n = 10], LL first [n = 26], or LESCL first [n = 5]). The remaining 102 patients without LL were used as a control group.                

Executive dysfunction, obsessive–compulsive symptoms, and attention deficit and hyperactivity disorder in Systemic Lupus Erythematosus: Evidence for basal ganglia dysfunction?

Ricardo Oliveira Horta Maciel, Gilda Aparecida Ferreira, Bárbara Akemy, Francisco Cardoso

Chorea is well described in a group of patients with Systemic Lupus Erythematosus (SLE). There is less information, however, on other movement disorders as well as non-motor neuropsychiatric features such as obsessive–compulsive symptoms (OCS), executive dysfunction and attention deficit and hyperactivity disorder (ADHD) in subjects with SLE.

Effect of Fampridine-PR (prolonged released 4-aminopyridine) on the manual functions of patients with Multiple Sclerosis

Ziv Savin, Izabella Lejbkowicz, Lea Glass-Marmor, Idit Lavi, Sara Rosenblum, Ariel Miller

Persons with MS (PwMS) commonly present ambulatory and manual dysfunctions. While ambulation is recognized as important to PwMS, manual dysfunction is only lately gaining attention. Fampridine-PR was approved for MS ambulatory impairments. Anecdotal evidences indicate possible therapeutic effects on manual function.

Study of the prevalence of familial autoimmune myasthenia gravis in a Spanish cohort

Maria Salvado, Merce Canela, Jose Maria Ponseti, Laura Lorenzo, Cecilia Garcia, Sonia Cazorla, Gisela Gili, Nuria Raguer, Josep Gamez

Myasthenia gravis (MG) is an autoimmune disease caused by a failure of neuromuscular transmission. Familial clustering has been reported despite MG usually manifesting as a sporadic condition presumed not to be inherited. Our study investigated the prevalence of FAMG in a Spanish cohort, characterizing their phenotype, antibody titres and thymus findings.

Diagnostic accuracy of computed tomography perfusion in patients with acute stroke: A meta-analysis

Ye Xin, Fu-Gang Han
  • DOI: http://dx.doi.org/10.1016/j.jns.2015.11.046
  • p125–130
  • Published online: November 25 2015Open Access
  • Abstract
  • Full-Text HTML
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The purpose of this meta-analysis was to evaluate the sensitivity and specificity of computed tomography perfusion (CTP) in diagnosing acute ischemic stroke in patients presenting to the emergency department with stroke-like symptoms.

SiRNA-mediated serotonin transporter knockdown in the dorsal raphe nucleus rescues single prolonged stress-induced hippocampal autophagy in rats

Zhong-Min Wu, Chun-Hua Zheng, Zhen-Hua Zhu, Feng-Tian Wu, Gui-Lian Ni, Yong Liang

The neurobiological mechanisms underlying the development of post-traumatic stress disorder (PTSD) remain elusive. One of the hypotheses is the dysfunction of serotonin (5-HT) neurotransmission, which is critically regulated by serotonin transporter (SERT). Therefore, we hypothesized that attenuation of SERT gene expression in the hippocampus could prevent hippocampal autophagy and the development of PTSD-like behavior. To this end, we infused SLC6A4 siRNAs into the dorsal raphe nucleus (DRN) to knockdown SERT gene expression after a single prolonged stress (SPS) treatment in rats.

Influence of urinary urgency and other urinary disturbances on falls in Parkinson's disease

Ken Sakushima, Shin Yamazaki, Shingo Fukuma, Yasuaki Hayashino, Ichiro Yabe, Shunichi Fukuhara, Hidenao Sasaki

Falling is one of the most common and serious public health problems. It can cause injuries such as sprains and fractures, and hospitalization may be required for serious injuries. Patients with Parkinson's disease have a higher risk of falls, and urinary incontinence is a known risk factor for falls in the elderly. However, whether other urinary disturbances contribute to the risk of falling remains unclear. The purpose of this study was to identify the association between falls and urinary disturbances in Parkinson's disease.

Development of a neurology rotation for internal medicine residents in Haiti

Aaron L. Berkowitz, Louine Martineau, Michelle E. Morse, Kerling Israel

In many low-income countries where there are few or no neurologists, patients with neurologic diseases are cared for by primary care physicians who receive no formal training in neurology. Here, we report our experience creating a neurology rotation for internal medicine residents in rural Haiti through a collaboration between a public academic medical center in Haiti and a visiting neurologist. We describe the structure of the rotation and the factors that led to its development.

Altered white matter metabolism in delayed neurologic sequelae after carbon monoxide poisoning: A proton magnetic resonance spectroscopic study

Hiroshi Kuroda, Kazuo Fujihara, Shunji Mugikura, Shoki Takahashi, Shigeki Kushimoto, Masashi Aoki

Proton magnetic resonance spectroscopy (1H-MRS) was recently used to examine altered metabolism in the white matter (WM) of patients experiencing carbon monoxide (CO) poisoning; however, only a small number of patients with delayed neurologic sequelae (DNS) were analyzed. We aimed to detect altered metabolism in the WM of patients with DNS using 1H-MRS; to explore its clinical relevance in the management of patients experiencing CO poisoning. Patients experiencing acute CO poisoning underwent 1H-MRS and cerebrospinal fluid (CSF) examination within 1 week and at 1 month after acute poisoning.  

Contribution of plexus MRI in the diagnosis of atypical chronic inflammatory demyelinating polyneuropathies

Pierre Lozeron, Marie-Christine Lacour, Christophe Vandendries, Marie Théaudin, Cécile Cauquil, Christian Denier, Catherine Lacroix, David Adams

Nerve enlargement has early been recognized in CIDP and plexus MRI hypertrophy has been reported in typical CIDP cases. Our aim is to determine plexus MRI value in the diagnosis of CIDP with an initial atypical presentation, which, up to now, has not been demonstrated. Retrospective study of 33 consecutive patients suspected of CIDP. Plexus MRI was performed on the most affected territory (brachial or lumbar). Were assessed: plexus trophicity, T2-STIR signal intensity and gadolinium enhancement. Final CIDP diagnosis was made after comprehensive workup.

Meta-analysis of apolipoprotein E levels in the cerebrospinal fluid of patients with Alzheimer's disease

Puneet Talwar, Juhi Sinha, Sandeep Grover, Rachna Agarwal, Suman Kushwaha, M.V. Padma Srivastava, Ritushree Kukreti

The possible association between Apolipoprotein E (ApoE) levels in the cerebrospinal fluid (CSF) and Alzheimer's disease (AD) has been studied extensively. However, previous findings have been inconsistent. We conducted a meta-analysis of observational studies, seeking to provide insights into ApoE's potential as a biomarker for AD. A systematic literature search of PubMed (MEDLINE), EMBASE, and Web of Science was performed to retrieve relevant studies evaluating ApoE levels in CSF from AD subjects and controls. 

 

Clinical Short Communications

Transient MR-angiography changes associated with morphological alterations in epileptic seizure: A short case series

Simone Marziali, Francesco Garaci, Francesca Di Giuliano, Antonio Chiaravalloti, Tommaso Schirinzi, Alessandro Davoli, Alessandro Bozzao, Roberto Floris

An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.

A spring to summer shift of pro-inflammatory cytokine production in multiple sclerosis patients

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

Higher MS relapse frequency is observed during warmer months in different regions, but evidence for an underlying immunological variation is lacking. Therefore, we investigated seasonal variations of cytokine production in relapsing-remitting MS patients. Twenty-one patients and eight controls had blood samples drawn in each season, evaluating for IL-10, IL-6, TNF-α and IFN-γ. The lowest levels of cytokine production were observed in spring samples, with a significant increase from spring to summer for most cytokines, and especially IFN-γ and TNF-α.

Diffuse corpus callosum infarction — Rare vascular entity with differing etiology

Rohan Mahale, Anish Mehta, Kiran Buddaraju, Aju Abraham John, Mahendra Javali, Rangasetty Srinivasa

Infarctions of the corpus callosum are rare vascular events. It is relatively immune to vascular insult because of its rich vascular supply from anterior and posterior circulations of brain.

Polyglucosan bodies in intramuscular nerves: Association with muscle fiber denervation atrophy

Jian-Qiang Lu, Cecile Phan, Douglas Zochodne, Chuanzhu Yan

Polyglucosan bodies (PB) in the intramuscular nerves have been rarely studied, and their presence particularly in subjects without neurologic disorders has been thought to be age-related. We examined, by using light and electron microscopy, 204 consecutive muscle biopsies. PB was found in 5 quadriceps intramuscular nerves (2.45% of all biopsies). All 5 quadriceps containing PB exhibited varying degrees of muscle fiber denervation atrophy with or without fiber type grouping. These quadriceps with PB, compared with the other 119 quadriceps without PB, showed a significantly greater association with muscle fiber denervation atrophy (5/5 versus 55/119; p = 0.02, by two-tailed Fisher's exact test), for which aging is not confounding.

Striatal glucose hypometabolism in preadolescent-onset dentatorubral–pallidoluysian atrophy

Daichi Sone, Noriko Sato, Kota Yokoyama, Kaoru Sumida, Masahiro Kanai, Etsuko Imabayashi, Yuko Saito, Hiroshi Matsuda

Dentatorubral–pallidoluysian atrophy (DRPLA) is hereditary spinocerebellar degeneration presenting various symptoms in association with expansion of the CAG repeat in Atrophin-1 gene. The functional neuroimaging of DRPLA has been poorly investigated. The purpose of this study was to examine 18F-fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) findings of DRPLA. We retrospectively investigated the cases of 14 consecutive genetically confirmed DRPLA patients at our institute. Four juvenile-onset patients underwent 18F-FDG-PET because of intractable seizures.

Novel mutation of SLC20A2 in a Chinese family with primary familial brain calcification

Xiaomin Liu, Gaoting Ma, Zhangning Zhao, Fei Mao, Jiyou Tang, Xiuhua Li, Meijia Zhu

Primary familial brain calcification (PFBC), also called idiopathic basal ganglia calcification (IBGC), or Fahr's disease, is a rare neurological disorder characterized by bilateral calcification in the basal ganglia and other brain regions, which is associated with motor and neuropsychiatric symptoms. SLC20A2 is a major causative gene linked to PFBC [1]. Here we report a Chinese PFBC family with a novel SLC20A2 mutation (Fig. 1a), in which the proband presented with dizziness and muscle cramps in both lower extremities.

Reversible tongue muscle atrophy accelerated by early initiation of immunotherapy in anti-MuSK myasthenia gravis: A case report

Eiji Kitamura, Yoko Takiyama, Masaaki Nakamura, Takahiro Iizuka, Kazutoshi Nishiyama

Progressive bulbar muscle weakness with tongue muscle atrophy (TMA) is caused by a variety of disorders affecting the lower brainstem, lower cranial nerves, neuromuscular junction, and muscles, and is often irreversible. Myasthenia gravis (MG) is the most common antibody-mediated neuromuscular junction disorder. Approximately 85% of patients with generalized MG have antibodies against muscle acetylcholine receptor (AChR-MG) [1] whereas about 30–70% of AChR-Ab-seronegative MG patients have antibodies against muscle-specific tyrosine kinase (MuSK-MG) [2,3] but its positivity varies among ethnic groups or types of assays [4]. 

Reversible encephalopathy with photoparoxysmal response during imipenem/cilastatin treatment

Markus Gschwind, Federico Simonetta, Serge Vulliemoz

A 70-year-old woman, with no history of epilepsy, was hospitalized due to a Macrophage Activation Syndrome (MAS) of unknown origin. She received imipenem/cilastatine (IPM/CS; 500 mg IV every 6 h) for agranulocytosis with sepsis, and presented, at day 6, a confusional state with irregular multifocal myoclonus in face, trunk, both arms and legs.

From conversion disorder (DSM-IV-TR) to functional neurological symptom disorder (DSM-5): When a label changes the perspective for the neurologist, the psychiatrist and the patient

Benedetta Demartini, Armando D'Agostino, Orsola Gambini

The term "conversion disorder", as used in DSM-IV-TR, describes symptoms such as weakness, epileptic-type attacks, abnormal movements or sensory disturbance that are not attributable to a structural damage to the nervous system or to feigning and that are considered to be associated with psychological factors [1]. The traditional label "conversion disorder" refers to a hypothesis based on a psychological aetiology. In fact, historically, psychological and emotional factors, such as trauma, conflict or distress, have been suggested as causal factors of conversion disorders [2].

The differential diagnosis of myotonic syndromes: A case report-guided and neurophysiologic approach

Giovanni Cirillo, Vincenzo Todisco, Gioacchino Tedeschi

Myotonic syndromes are a heterogeneous group of disorders characterized by myotonic symptoms (myotonia, cold-induced worsening, improvement with warming and activity, i.e. "warm-up" effect) and stiffness.

Isolated cerebellar-type hemiataxia in a thalamic infarction

Keiichi Hokkoku, Kiyoshi Matsukura, Junpei Yamamoto, Midori Kuwabara, Takashi Chiba, Yuki Hatanaka, Masahiro Sonoo

Dejerine–Roussy syndrome presenting with hemiataxia due to loss of proprioception is a classic symptom of a thalamic stroke. In contrast, it has been reported that cerebellar ataxia, which was not directly related to the loss of proprioception, might occur due to a thalamic lesion. Recent studies using modern imaging demonstrated that a cerebellar-type hemiataxia may occur in a thalamic infarction, and the ventral lateral (VL) nucleus, the destination of the dentato-rubro-thalamic tract, has been postulated as the responsible lesion [2,7,10].

Pleocytosis in cerebrospinal fluid following multiple lumbar punctures in healthy volunteers

George Atiee, John Sramek, Joseph Palasota, Sarah Andrews, Michael Murphy, Neal R. Cutler

Pleocytosis (white blood cell [WBC] > 5/μL) [1] is commonly reported in studies involving lumbar puncture (LP), but the contributing role of LP in pleocytosis has been obfuscated by the inclusion of patient populations, which may add confounding factors such as disease state or underlying injury. As LP is often performed as a diagnostic tool, many studies have observed pleocytosis following LP in the context of various diseases and injuries to the central nervous system (CNS) [2–6]. Several medical textbooks suggest pleocytosis may be a normal side effect following repeated lumbar puncture, but do not cite controlled studies in healthy subjects to substantiate this statement [7,8].

Multiple system atrophy presenting initially as spastic paraparesis

Yohei Tsuyusaki, Ryuji Sakakibara, Fuyuki Tateno, Yosuke Aiba, Masahiko Kishi, Hiromi Tateno, Tsuyoshi Ogata, Tsutomu Inaoka, Hitoshi Terada, Yasuo Suzuki

Multiple system atrophy (MSA) is a synucleinopathy with glial cytoplasmic inclusions throughout the brain, but it preferentially affects the basal ganglia, cerebellum, and spinal autonomic and Onuf's nuclei [1]. Clinically MSA is characterized by 1) movement disorder (dopa-unresponsive parkinsonism and/or cerebellar ataxia) and 2) autonomic failure (postural hypotension and/or bladder dysfunction) [1]. In addition, MSA shows pyramidal signs as increased deep tendon reflexes and extensor plantar reflexes in 43–61% of the patients [1,2].

 
 
 
 

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