DOCSLIB.ORG

Head Drop and Camptocormia

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Head Drop and Camptocormia EDITORIAL 1 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.73.1.1 on 1 July 2002. Downloaded from Head drop Table 2 Causes of ................................................................................... camptocormia Head drop and camptocormia Disorders Neuromuscular Motor neuron Amyotrophic lateral T Umapathi, V Chaudhry, D Cornblath, D Drachman, sclerosis17 18 J Griffin, R Kuncl Muscle IBM19 Nemaline myopathy20 ................................................................................... Facioscapulohumeral dystrophy The spectrum of bent spine disorders Parkinsonism Idiopathic21 Postencephalitic22 23 Villiusk encephalomyelitis24 ead ptosis (drop) results from Sodium valproate CASE A 25 weakness of the neck extensor, or toxicity An 80 year old man developed head pto- Idiopathic increased tone of the flexor mus- H sis insidiously over a period of few cles. It is characterised by marked anterior curvature or angulation of the weeks. A week before this he had an cervical spine and is associated with upper respiratory tract infection and also various neuromuscular (table 1) and experienced transient sharp pain over occasional nuclear sacs, target and tar- extrapyramidal disorders.12–15 Campto- the left and then the right shoulder. He getoid fibres as well as myopathic fea- cormia or the bent spine syndrome was had no diplopia, dysarthria, dysphagia, tures such as the presence of hyper- first described in hysterical soldiers in limb weakness, or fatiguability. Exam- trophic and split muscle fibres; a few 1915 by the French neurologist ination showed severe neck extensor necrotic, degenerating and regenerating Souques.16 Typically there is marked weakness, Medical Research Council fibres; increased internalised nuclei, and anterior curvature of the thoracolumbar (MRC) grade 2. Muscle strength was mild endomysial fibrosis. No type group- spine. In some patients the spine is normal in all other cranial, proximal, and ing was noted, but there was massive angulated forward, the arms propped distal limb muscles. Serum creatine type I predominance. No specific treat- against the thigh for support. More kinase (CK) was 362 IU/l (normal ment was offered. A few months later, he cases, all among soldiers, were reported 24–195) at presentation. Anti- started to improve. At follow up 2 years during the first and second world wars. acetylcholine receptor antibody was not later, he was able to keep his head up for These patients responded well to psycho- detected. Repetitive nerve stimulation of prolonged periods, especially in the therapy. Recently camptocormia arising right biceps, right nasalis and right sitting position. However, he was more symptomatic while standing. He re- as a result of weakness or abnormality in trapezius was normal. A therapeutic trial ported an overall 60% subjective im- the tone of the paraspinal muscles has of pyridostigmine failed. Cervical spine been described (table 2). In contrast with provement in neck strength and weak- MRI did not show significant compres- other skeletal disorders of the spine such ness had not progressed to other sion of the cord, roots, or any specific as kyphosis, the deformity in head ptosis muscles. changes in the paraspinal muscles. An and camptocormia is not fixed and is corrected by passive extension or lying in EMG disclosed positive sharp waves CASE B restricted to lower cervical paraspinal the supine position. It is not possible to A 74 year old man with diabetes mellitus http://jnnp.bmj.com/ straighten the neck or back voluntarily. muscles. To reduce confounding from and hyperlipidaemia (for which he had The evaluation of these disorders can neuropathic changes secondary to coex- been taking a statin for a few years) indeed be challenging and often no defi- istent age related spondylotic changes, complained of progressive anterior cur- nite diagnosis is made, as illustrated by the thoracic paraspinal muscle was cho- vature of the spine associated with four cases of head ptosis and campto- sen for biopsy. The pathological findings proximal limb weakness for few months. cormia seen by us at the Johns Hopkins were mixed. There were neuropathic On examination, he had moderately Hospital. changes such as angular atrophic fibres; severe anterior thoracolumbar curvature that could be extended passively. Diffuse on October 1, 2021 by guest. Protected copyright. weakness was present; proximal limb strength was MRC grade 4 and distal, Table 1 Neuromuscular causes for head ptosis 4+. Electrodiagnostic evaluation only Site of pathology Disorders showed evidence of an axonal polyneu- ropathy, consistent with diabetes. Serum Motor neuron Amyotrophic lateral sclerosis12 CK was persistently raised (367 IU/l). 3 Postpolio syndrome Spine MRI only showed mild degenera- Peripheral nerve Chronic inflammatory polyneuropathy4 Neuromuscular junction Myasthenia gravis12 tive changes. Anti-acetylcholine receptor Muscle antibody was not detected. Biopsy of the (a) Inflammatory Polymyositis12 rectus femoris showed increased fibre (b) Congenital/hereditary Inclusion body myositis2 size variability, necrosis, mild fibrosis, 56 Nemaline myopathy occasional red rimmed vacuoles, and one Mitochondrial myopathy7 Carnithine deficiency8 fibre which stained positive for ubiqui- Unspecified congenital myopathy9 tin, suggestive of inclusion body myosi- (c) Endocrine/metabolic Facioscapulohumeral dystrophy1 tis. However, inflammation or amyloid Cushing syndrome7 Hypothyroid myopathy10 Myotonic dystrophy with severe hypothyroidism2 ........................................ Severe hypokalaemic myopathy11 Abbreviations: CK, creatine kinase; MSA, multisystem atrophy; ALS, amyotrophic lateral sclerosis www.jnnp.com 2 EDITORIAL J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.73.1.1 on 1 July 2002. Downloaded from deposition were not seen. Despite stop- isolated antecollis causing forced anter- patients but other sensory abnormalities ping statin, his weakness continues to oflexion of the neck occurs in parkinso- are not prominent. The weakness is pro- progress and CK has remained raised nian syndromes. Rivest et al have docu- found in the neck extensors, and involve- (239 IU/L). mented antecollis in four of their ment of contiguous proximal muscles is necropsy proved cases of multisystem at most modest. The CK was normal in CASE C atrophy (MSA).12 13 The abnormality de- all but two patients. Katz at al2 reported A 59 year old woman presented with a 4 velops, sometimes in a subacute manner oedema-like changes and atrophy of the year history of progressive, painless over a period of weeks, in the late or neck extensor muscles on MRI. An EMG anterior curvature of the spine. She had middle stages of the disease. The neck of the paraspinal muscles was remark- a family history of facioscapulohumeral can only be passively and forcibly ex- able for the increased reports of sponta- muscular dystrophy but not of bent tended to its normal position with diffi- neous activity. Fibrillations were present spine. Examination showed campto- culty. None of the patients had convinc- in nine and positive sharp waves in one cormia along with scapular winging and ing dystonic spasms of the anterior neck out of the 15 patients for whom EMG mild facial and proximal weakness. Her muscles, although deeper muscles may data are available. Interestingly, such CK was at the upper limit of normal, 186 be involved. Speech, swallowing, and abnormalities were pronounced in the IU/l. An EMG showed short duration, upgaze deficits are often associated. lower cervical segments; and in one case small amplitude motor units with early Trials of botulinum toxin into both ster- reported by Katz et al,2 extended to the recruitment in the proximal muscles. nocleidomastoid muscles are ineffective midthoracic region. The motor unit Abnormal spontaneous activity was seen and response to levodopa generally action potentials in most cases are in the thoracic paraspinal muscles. No disappointing.12 13 Earlier case reports described as having short duration and decremental response to repetitive nerve have also documented the association small amplitude with early recruitment. stimulation was present. Spine MRI was between MSA and head drop.27–29 However, this has to be interpreted with unremarkable. 4q35 gene deletion, con- Yoshiyama et al14 published a series of caution as paraspinal motor unit action sistent with facioscapulohumeral mus- seven patients with parkinsonism exhib- potentials are generally small and may cular dystrophy, was detected. iting head drop, four of whom carried a have multiple turns even in normal indi- diagnosis of probable MSA. Although at viduals. EMG of the limb muscles is CASE D rest there was no anterior neck spasm, mostly unremarkable. The histology of A 63 year old woman developed slowly attempts to extend the head voluntarily limb muscles is normal or reveals mild progressive, painless thoracolumbar or passively was accompanied by con- non-specific myopathic changes such as stoop of about 30 degrees over 8 to 9 traction of the sternocleidomastoid mus- increased fibre size variability. In two of years. The deformity was not fixed and cles on surface EMG in all of these our cases there is some evidence of den- was worse in the standing position, patients. There was no correlation be- ervation. The paraspinal muscle biopsies better sitting, and minimal while lying tween the severity of the head ptosis and show less subtle but again non-specific down. There was no family history of parkinsonism. Three patients had im- changes
Load more

Recommended publications
  • Diagnostic Clues in Multiple System Atrophy
    DO I:10.4274/Tnd.82905 Case Report / Olgu Sunumu Diagnostic Clues in Multiple System Atrophy: A Case Report and Literature Review Multisistem Atrofi Tanısında İpuçları: Bir Olgu Sunumu ve Literatürün Gözden Geçirilmesi Mehmet Yücel, Oğuzhan Öz, Hakan Akgün, Semai Bek, Tayfun Kaşıkçı, İlter Uysal, Yaşar Kütükçü, Zeki Odabaşı Gülhane Military Medical Academy, Ankara, Turkey Sum mary Multiple system atrophy (MSA) is an adult-onset, sporadic, progressive neurodegenerative disease. Based on the consensus criteria, patients with MSA are clinically classified into cerebellar (MSA-C) and parkinsonian (MSA-P) subtypes. In addition to major diagnostic criteria including poor response to levodopa, and presence of pyramidal or cerebellar signs (ataxia) or autonomic failure, certain clinical features or ‘‘red flags’’ may raise the clinical suspicion for MSA. In our case report we present a 67-year-old female patient admitted to our hospital due to inability to walk, with poor response to levodopa therapy, whose neurological examination revealed severe Parkinsonism, ataxia and who fulfilled all criteria for MSA, as rarely seen in clinical practice.(Turkish Journal of Neurology 2013; 19:28-30) Key Words: Multiple system atrophy, autonomic failure, diagnostic criteria Özet Multisistem atrofi (MSA) erişkin dönemde başlayan, ilerleyici, nedeni bilinmeyen sporadik nörodejeneratif bir hastalıktır. MSA kabul görmüş tanı kriterlerine göre klinik olarak serebellar (MSA-C) ve parkinsoniyen (MSA-P) alt tiplerine ayrılmaktadır. Düşük levadopa yanıtı, piramidal, serebellar bulguların (ataksi) ya da otonomik bozukluk olması gibi majör tanı kriterlerininin yanında “red flags” olarak isimlendirilen belirgin klinik bulgular ya da uyarı işaretlerinin olması MSA tanısı için klinik şüpheyi oluşturmalıdır. Olgu sunumunda 67 yaşında yürüyememe şikayeti ile polikliniğimize müracaat eden ve levadopa tedavisine düşük yanıt gösteren ciddi parkinsonizm bulguları ile ataksi bulunan kadın hasta MSA tanı kriterlerini tam olarak karşıladığı ve klinik pratikte nadir görüldüğü için sunduk.
    [Show full text]
  • Pallidal High-Frequency Deep Brain Stimulation for Camptocormia: an Experience of Three Cases
    Acta Neurochir Suppl (2006) 99: 25–28 # Springer-Verlag 2006 Printed in Austria Pallidal high-frequency deep brain stimulation for camptocormia: an experience of three cases C. Fukaya1;2, T. Otaka1;2, T. Obuchi1;2, T. Kano1;2, T. Nagaoka1;2, K. Kobayashi1;2, H. Oshima1;2, T. Yamamoto1;2, and Y. Katayama1;2 1 Department of Neurological Surgery, Nihon University School of Medicine, Itabashi-ku, Japan 2 Division of Applied System Neuroscience, Nihon University Graduate School of Medical Science, Itabashi-ku, Japan Summary arms. Recently, camptocormia has been reported to oc- Introduction. The term ‘‘camptocormia’’ describes a forward- cur in association with various other neurological con- flexed posture. It is a condition characterized by severe frontal flex- ditions, including primary dystonia. The cause of this ion of the trunk. Recently, camptocormia has been regarded as a pathological condition remains unknown, and appropri- form of abdominal segmental dystonia. Deep brain stimulation ate treatment has not been established. (DBS) is a promising therapeutic approach to various types of move- ment disorders. The authors report the neurological effects of DBS Deep brain stimulation (DBS) has been regarded as to the bilateral globus pallidum (GPi) in three cases of disabling a promising therapeutic approach for various types of camptocormia. movement disorders. The authors report the neurolog- Methods. Of the 36 patients with dystonia, three had symptoms similar to that of camptocormia, and all of these patients underwent ical effects of deep brain stimulation to the bilateral GPi-DBS. The site of DBS electrode placement was verified by mag- globus pallidum (GPi-DBS) in three cases of disabling netic resonance imaging (MRI).
    [Show full text]
  • Hereditary Ataxia Multigene Panel Testing
    Lab Management Guidelines V1.0.2021 Hereditary Ataxia Multigene Panel Testing MOL.TS.310.A v1.0.2021 Introduction Hereditary Ataxia Multigene Panel testing is addressed by this guideline. Procedures addressed The inclusion of any procedure code in this table does not imply that the code is under management or requires prior authorization. Refer to the specific Health Plan's procedure code list for management requirements. Procedures addressed by this Procedure codes guideline Hereditary Ataxia Multigene Panel 81443 (including sequencing of at least 15 genes) Genomic Unity Ataxia Repeat Expansion 0216U and Sequence Analysis Genomic Unity Comprehensive Ataxia 0217U Repeat Expansion and Sequence Analysis Unlisted molecular pathology procedure 81479 What are hereditary ataxias Definition The hereditary ataxias are a group of genetic disorders. They are characterized by slowly progressive uncoordinated, unsteady movement and gait, and often poor coordination of hands, eye movements, and speech. Cerebellar atrophy is also frequently seen.1 Incidence and prevalence Prevalence estimates vary. The prevalence of autosomal dominant ataxias is approximately 1-5:100,000.1 One study in Norway estimated the prevalence of hereditary ataxia at 6.5 per 100,000 people.2 © 2020 eviCore healthcare. All Rights Reserved. 1 of 8 400 Buckwalter Place Boulevard, Bluffton, SC 29910 (800) 918-8924 www.eviCore.com Lab Management Guidelines V1.0.2021 Symptoms Although hereditary ataxias are made up of multiple different conditions, they are characterized by slowly progressive uncoordinated, unsteady movement and gait, and often poor coordination of hands, eye movements, and speech. Cerebellar atrophy is also frequently seen.1 Cause Hereditary ataxias are caused by mutations in one of numerous genes.
    [Show full text]
  • Multiple System Atrophy
    Multiple System Atrophy U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES Public Health Service National Institutes of Health Multiple System Atrophy What is multiple system atrophy? ultiple system atrophy (MSA) is M a progressive neurodegenerative disorder characterized by a combination of symptoms that affect both the autonomic nervous system (the part of the nervous system that controls involuntary action such as blood pressure or digestion) and move- ment. The symptoms reflect the progressive loss of function and death of different types of nerve cells in the brain and spinal cord. Symptoms of autonomic failure that may be seen in MSA include fainting spells and prob- lems with heart rate, erectile dysfunction, and bladder control. Motor impairments (loss of or limited muscle control or movement, or limited mobility) may include tremor, rigidity, and/or loss of muscle coordination as well as difficulties with speech and gait (the way a person walks). Some of these features are similar to those seen in Parkinson’s disease, and early in the disease course it often may be difficult to distinguish these disorders. MSA is a rare disease, affecting potentially 15,000 to 50,000 Americans, including men and women and all racial groups. Symptoms tend to appear in a person’s 50s and advance rapidly over the course of 5 to 10 years, with progressive loss of motor function and 1 eventual confinement to bed. People with MSA often develop pneumonia in the later stages of the disease and may suddenly die from cardiac or respiratory issues. While some of the symptoms of MSA can be treated with medications, currently there are no drugs that are able to slow disease progression and there is no cure.
    [Show full text]
  • Haematological Abnormalities in Mitochondrial Disorders
    Singapore Med J 2015; 56(7): 412-419 Original Article doi: 10.11622/smedj.2015112 Haematological abnormalities in mitochondrial disorders Josef Finsterer1, MD, PhD, Marlies Frank2, MD INTRODUCTION This study aimed to assess the kind of haematological abnormalities that are present in patients with mitochondrial disorders (MIDs) and the frequency of their occurrence. METHODS The blood cell counts of a cohort of patients with syndromic and non-syndromic MIDs were retrospectively reviewed. MIDs were classified as ‘definite’, ‘probable’ or ‘possible’ according to clinical presentation, instrumental findings, immunohistological findings on muscle biopsy, biochemical abnormalities of the respiratory chain and/or the results of genetic studies. Patients who had medical conditions other than MID that account for the haematological abnormalities were excluded. RESULTS A total of 46 patients (‘definite’ = 5; ‘probable’ = 9; ‘possible’ = 32) had haematological abnormalities attributable to MIDs. The most frequent haematological abnormality in patients with MIDs was anaemia. 27 patients had anaemia as their sole haematological problem. Anaemia was associated with thrombopenia (n = 4), thrombocytosis (n = 2), leucopenia (n = 2), and eosinophilia (n = 1). Anaemia was hypochromic and normocytic in 27 patients, hypochromic and microcytic in six patients, hyperchromic and macrocytic in two patients, and normochromic and microcytic in one patient. Among the 46 patients with a mitochondrial haematological abnormality, 78.3% had anaemia, 13.0% had thrombopenia, 8.7% had leucopenia and 8.7% had eosinophilia, alone or in combination with other haematological abnormalities. CONCLUSION MID should be considered if a patient’s abnormal blood cell counts (particularly those associated with anaemia, thrombopenia, leucopenia or eosinophilia) cannot be explained by established causes.
    [Show full text]
  • UC San Francisco Previously Published Works
    UCSF UC San Francisco Previously Published Works Title Axial mitochondrial myopathy in a patient with rapidly progressive adult-onset scoliosis. Permalink https://escholarship.org/uc/item/4k83m27z Journal Acta neuropathologica communications, 2(1) ISSN 2051-5960 Authors Hiniker, Annie Wong, Lee-Jun Berven, Sigurd et al. Publication Date 2014-09-16 DOI 10.1186/s40478-014-0137-3 License https://creativecommons.org/licenses/by/4.0/ 4.0 Peer reviewed eScholarship.org Powered by the California Digital Library University of California Hiniker et al. Acta Neuropathologica Communications 2014, 2:137 http://www.actaneurocomms.org/content/2/1/137 CASE REPORT Open Access Axial mitochondrial myopathy in a patient with rapidly progressive adult-onset scoliosis Annie Hiniker1, Lee-Jun Wong2, Sigurd Berven3, Cavatina K Truong2, Adekunle M Adesina4 and Marta Margeta1* Abstract Axial myopathy can be the underlying cause of rapidly progressive adult-onset scoliosis; however, the pathogenesis of this disorder remains poorly understood. Here we present a case of a 69-year old woman with a family history of scoliosis affecting both her mother and her son, who over 4 years developed rapidly progressive scoliosis. The patient had a history of stable scoliosis since adolescence that worsened significantly at age 65, leading to low back pain and radiculopathy. Paraspinal muscle biopsy showed morphologic evidence of a mitochondrial myopathy. Diagnostic deficiencies of electron transport chain enzymes were not detected using standard bioassays, but mitochondrial immunofluorescence demonstrated many muscle fibers totally or partially deficient for complexes I, III, IV-I, and IV-IV. Massively parallel sequencing of paraspinal muscle mtDNA detected multiple deletions as well as a 40.9% heteroplasmic novel m.12293G > A (MT-TL2) variant, which changes a G:C pairing to an A:C mispairing in the anticodon stem of tRNA LeuCUN.
    [Show full text]
  • Lewy Body Diseases and Multiple System Atrophy As ␣-Synucleinopathies
    Molecular Psychiatry (1998) 3, 462–465 1998 Stockton Press All rights reserved 1359–4184/98 $12.00 GUEST EDITORIAL Lewy body diseases and multiple system atrophy as ␣-synucleinopathies Parkinson’s disease and dementia with Lewy bodies synuclein are the first known genetic causes of Parkin- are among the most common neurodegenerative dis- son’s disease. They are located in the amino-terminal eases. They share the Lewy body and the Lewy neurite half of ␣-synuclein, which consists of seven imperfect as their defining neuropathological characteristics. repeats of 11 amino acids each, with the consensus Originally described in 1912 as a light-microscopic sequence KTKEGV (Figure 1). It has been suggested entity, the Lewy body was shown to be made of abnor- that ␣-synuclein may bind through these repeats to mal filaments in the 1960s. Over the past year, the bio- membranes rich in acidic phospholipids.3 chemical nature of the Lewy body filament has been This work raised the question whether ␣-synuclein revealed. is a component of Lewy bodies and Lewy neurites of A large number of different proteins has been idiopathic Parkinson’s disease and of dementia with reported to be present in Lewy bodies and Lewy neur- Lewy bodies. Using well-characterised anti-␣-synuc- ites by immunohistochemical methods. However, these lein antibodies, Spillantini et al showed strong staining findings do not permit us to distinguish between of both Lewy bodies and Lewy neurites in idiopathic intrinsic Lewy body components and normal cellular Parkinson’s disease and dementia with Lewy bodies.4 constituents that get merely trapped in the filaments They also reported the lack of staining of these patho- that make up the Lewy body.
    [Show full text]
  • A Case Report of Thoracolumbar Paraspinal Myopathy As the Cause of Camptocormia in a Patient with Atypical Parkinsonism
    Kim et al. BMC Neurology (2017) 17:118 DOI 10.1186/s12883-017-0899-x CASE REPORT Open Access A case report of thoracolumbar paraspinal myopathy as the cause of camptocormia in a patient with atypical parkinsonism Yoon Kim, Ahro Kim, Aryun Kim and Beomseok Jeon* Abstract Background: Camptocormia is severe flexion of the thoracolumbar spine, exaggerated during standing and walking but minimized in supine position. Even though camptocormia is a relatively common condition during the course of Parkinson’s disease, there is ongoing controversy concerning its mechanisms. The most widely accepted and yet still disputed one is dystonia. However, based on myopathic changes observed in the paraspinal muscle biopsies of some PD patients with camptocormia, the attempt to attribute camptocormia to myopathy has continued. This case presents evidence for paraspinal myopathy as the cause of camptocormia in a patient with atypical parkinsonism. Case presentation: A patient presented with a relatively acute onset of camptocormia and new-onset back pain. Upon examination, she had asymmetric parkinsonism. Magnetic resonance imaging of the lumbar spine revealed alterations in muscle signal intensity in the right paraspinal muscles at the L1–2 level. In the presence of persistent back pain, repeat imaging done two months later showed diffuse enlargement and patchy enhancement of the paraspinal muscles on T1-weighted imaging from T4 through sacrum bilaterally. About fifteen months after the onset of camptocormia, she underwent ultrasound-guided gun biopsy of the paraspinal muscles for evaluation of focal atrophy of the back muscles on the right. The biopsy revealed unmistakable myopathic changes, marked endomysial and perimysial fibrosis of the muscles, and merely mild infiltration of inflammatory cells but no clues regarding the cause of myopathy.
    [Show full text]
  • What You Need to Know
    Multiple System Atrophy: What You Need to Know Publication Date: April 4, 2019 Authors: Daniel Claassen, MD, Allyson Mayeux, MD, and Annette Hoy, MD 2 Table of Contents Overview……………………………………………………………………………………………… 3 Differential Diagnosis…………………………………………………………………………… 9 Evaluation Methods……………………………………………………………………………… 12 Orthostatic Hypotension………………………………………………………………………. 14 Neurogenic Bladder………………………………………………………………………………. 20 MSA-P & MSA-C ..………………………………………………………………………………… 25 Dystonia………………………………………………………………………………………………… 29 Breathing Disorders………………………………………………………………………………… 34 REM Behavior Disorder…………………………………………………………………………… 39 Depression and Cognitive IMpairMent……………………………………………………. 43 Neuroprotective Diet…………………………………………………………………………….. 45 Constipation……………………………………………………………………………………………. 47 Advanced Planning: Advance Directives, Palliative Care, and Brain Donation…………………………………………………………………………………. 50 © Copyright MSA Coalition 2019. All Rights Reserved. www.multiplesystematrophy.org 3 Multiple System Atrophy Overview Multiple System Atrophy (MSA) is a rare neurodegenerative disorder that can cause different symptoms, such as impairments to balance and difficulty with movement, poor coordination, bladder dysfunction, sleep disturbances, and poor blood pressure control. The disease was first known as Shy-Drager Syndrome. At the moment, it is believed that MSA is "sporadic," meaning that there are no established genetic or environmental factors that cause the disease. A few reports have described families with MSA, but this finding is probably
    [Show full text]
  • Bilateral Deep Brain Stimulation of the Subthalamic Nuclei in Parkinson's
    Scientifi c Journal of Neurology & Neurosurgery Case Report Bilateral Deep Brain Stimulation of the Subthalamic Nuclei in Parkinson’s disease Patients with Camptocormic Posture - Fadi Almahariq1, Marina Raguz1*, Vladimira Vuletic2, Darko Oreskovic1, Ivica Franciskovic1, Petar Marcinkovic1 and Darko Chudy1 1Department of Neurosurgery, University Hospital Dubrava, Zagreb, Croatia 2Department of Neurology, University Hospital Center Rijeka, Rijeka, Croatia *Address for Correspondence: Marina Raguz, Department of Neurosurgery, University Hospital Dubrava, Avenija Gojka Suska 6 Zagreb HR-10000, Croatia, Tel: +385 1 290 2476; Fax: +385 1 290 2934; E-mail: Submitted: 05 August 2017; Approved: 17 August 2017; Published: 19 August 2017 Citation this article: Almahariq F, Raguz M, Vuletic V, Oreskovic D, Franciskovic I, et al. Bilateral Deep Brain Stimulation of the Subthalamic Nuclei in Parkinson’s disease Patients with Camptocormic Posture. Scientifi c Journal of Neurology & Neurosurgery. 2017;3(2): 037-040. Copyright: © 2017 Raguz M, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Page - 037 SRL Neurology & Neurosurgery ABSTRACT Background: Camptocormia is a disabling syndrome characterized by forward fl exion that can be an idiopathic or associated with numerous diseases like movement disorders. Posture improvement could be expected after bilateral deep brain stimulation (DBS) of the Globus Pallidus Internus (GPI) or Subthalamic Nucleus (STN) in Parkinson’s disease (PD) patients with camptocormia. The aim of this study was to determine the effi cacy of bilateral STN DBS in alleviating the degree of camptocormia in PD patients.
    [Show full text]
  • Cognitive and Psychiatric Disturbances in Parkinsonian Syndromes
    Cognitive and Psychiatric Disturbances in Parkinsonian Syndromes Richard M. Zweig, MD*, Elizabeth A. Disbrow, PhD, Vijayakumar Javalkar, PhD KEYWORDS Parkinson Dementia with Lewy Bodies (DLB) Parkinsonian Progressive Supranuclear Palsy (PSP) Multiple System Atrophy (MSA) Corticobasal degeneration (CBD) KEY POINTS Executive dysfunction is often measurable in newly diagnosed Parkinson’s disease. Treatment with dopaminergic medications, particularly dopamine agonists, has been associated with hallucinations and impulse control disorder. While sharing many pathological features with Alzheimer’s disease, distinguishing clinical features of Dementia with Lewy bodies include episodic fluctuations of cognition, early hallucinations, and REM behavior disorder symptoms. Quetiapine appears to be effective for hallucinating patients, without worsening parkin- sonism at lower dosages. Clozapine is also effective. The promising 5-HT2A inverse agonist pimavanserin is awaiting FDA approval. The neuropsychiatric profile of progressive supranuclear palsy may closely resemble that of the frontotemporal lobar degenerations. INTRODUCTION As is the case with all of the neurodegenerative disorders, the subset comprising the parkinsonian syndromes of Parkinson disease, dementia with Lewy bodies, progres- sive supranuclear palsy (PSP), corticobasal degeneration (CBD), and multiple system atrophy (MSA) are considered proteinopathies. In these disorders, disease-associated proteins accumulate in the wrong cellular or extracellular compartments, and are often The authors have nothing to disclose. Department of Neurology, Louisiana State University Health Sciences Center - Shreveport, 1501 King’s Highway, Shreveport, LA 71103, USA * Corresponding author. E-mail address: [email protected] Neurol Clin 34 (2016) 235–246 http://dx.doi.org/10.1016/j.ncl.2015.08.010 neurologic.theclinics.com 0733-8619/16/$ – see front matter Ó 2016 Elsevier Inc.
    [Show full text]
  • Principles-Of-Autonomic-Medicine-V
    Principles of Autonomic Medicine v. 2.1 DISCLAIMERS This work was produced as an Official Duty Activity while the author was an employee of the United States Government. The text and original figures in this book are in the public domain and may be distributed or copied freely. Use of appropriate byline or credit is requested. For reproduction of copyrighted material, permission by the copyright holder is required. The views and opinions expressed here are those of the author and do not necessarily state or reflect those of the United States Government or any of its components. References in this book to specific commercial products, processes, services by trade name, trademark, manufacturer, or otherwise do not necessarily constitute or imply their endorsement, recommendation, or favoring by the United States Government or its employees. The appearance of external hyperlinks is provided with the intent of meeting the mission of the National Institute of Neurological Disorders and Stroke. Such appearance does not constitute an endorsement by the United States Government or any of its employees of the linked web sites or of the information, products or services contained at those sites. Neither the United States Government nor any of its employees, including the author, exercise any editorial control over the information that may be found on these external sites. Permission was obtained from the following for reproduction of pictures in this book. Other reproduced pictures were from -- 1 -- Principles of Autonomic Medicine v. 2.1 Wikipedia Commons or had no copyright. Tootsie Roll Industries, LLC (Tootsie Roll Pop, p. 26) Dr. Paul Greengard (portrait photo, p.
    [Show full text]