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    Antibodies and Neuronal Autoimmune Disorders of The CNS.: Gra1uus F Saiz A Dalmau J

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    Alejandro Guerrero León

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    Antibodies and Neuronal Autoimmune Disorders of The CNS.: Gra1uus F Saiz A Dalmau J

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    Alejandro Guerrero León
    16 views3 pages

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    Abstract GAD

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    Antibodies and Neuronal Autoimmune Disorders of The CNS.: Gra1uus F Saiz A Dalmau J

    Uploaded by

    Alejandro Guerrero León

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    © All Rights Reserved
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    of 3

    J Neurol. 2010 Apr;257(4):509-17. doi: 10.1007/s00415-009-5431-9. Epub 2009 Dec 25.

    Antibodies and neuronal autoimmune disorders of the CNS.


    Gra1uus F, Saiz A, Dalmau J.

    Source
    Servei y111y1u11111111de Neurologia, Hospital Clinic, Institut d' Investigació Biomèdica August Pi
    i Sunyer, Villarroel 170, 08036, Barcelona, Spain. [email protected]
    Abstract11111111
    We review the neuronal antibodies described in CNS disorders in order to e1111226clarify their diagnostic value, emphasize
    potentials pitfalls1uu111uu1uýand limitations in the diagnosis of paraneoplastic neurological syndromes (PNS), and examine
    the current evidence for a possible pathogenic role. We propose to classify the neuronal antibodies associated with
    syndromes resulting from CNS neuronal dysfunction into two groups according to the location of the antigen: inside the
    neuron or in the cell membrane. Group I includes antibodies which target intracellular antigens and probably are not
    pathogenic. They are further subdivided into three groups. Group Ia comprises well-characterized onconeural antibodies (Hu
    (ANNA1), Yo (PCA1), Ri (ANNA2), CV2 (CRMP5), amphiphysin, Ma2) that are useful for the diagnosis of PNS. Group Ib
    antibodies (SOX and ZIC) are cancer-specific but there is no evidence that the immune response is in any way
    pathogenically related to the PNS. Antibodies in group Ic (glutamic acid decarboxylase (GAD), adenylate kinase 5 and
    Homer 3) identify non-PNS: stiff-person syndrome (SPS), cerebellar ataxia, and limbic encephalitis (LE). Group II antibodies
    recognize neuronal surface antigens. Antibodies in group IIa associate with characteristic CNS syndromes but their detection
    does not indicate that the disorder is paraneoplastic. Antibodies to potassium channels, AMPA and GABA(B) receptors are
    associated with LE, NMDA receptor antibodies identify a well-defined encephalitis, and antibodies against glycine receptors
    associate with SPS with encephalitis. A pathogenic role of the antibodies is suggested by the response of symptoms to
    immunotherapy and the correlation between antibody titers and neurological outcome. Lastly, Group IIb includes antibodies
    that are found in patients with paraneoplastic cerebellar ataxia associated with lung cancer (P/Q type calcium channels
    antibodies) or Hodgkin disease (metabotropic glutamate receptor type 1 antibodies).

    Ds

    Cere2eq21sß4ßbellum. 2003;2(1):77-9.

    Cerebel111lar ataxia associated with sáweq acid


    decarboxylase autoantibodies.
    Vianello M, Tavolato B, Armani M, Giometto B.

    Source1wmsmses
    Department of Neurological and Psychiatric Sciences, University of Padova, Italy.
    Abstract
    Recent reports describe the detection of high titres of antibodies to glutamic acid decarboxylase (GAD-Ab) in the
    serum and cerebrospinal fluid (CSF) of patients with cerebellar ataxia. Most of these cases are females with
    Polyglandular Autoimmune Disorder who develop a chronic cereb11111112111112112ellar syndrome. The CSF
    profile is in keeping with an autoimmune disorder and intrathecal GAD-Ab synthesis has been demonstrated. The
    ataxia could reverse after immunomodulatory treatments suggesting a possible pathogenetic role for GAD-Ab.
    PMID:
     
    12882238
     
    [PubMed - indexed for MEDLINE]

    Lancet Neurol. 2011 Aug;10(8):759-72. doi: 10.1016/S1474-4422(11)70096-5.

    Autoantibodies associated with diseases of the CNS: new


    developments and future challenges.
    Vincent A, Bien CG, Irani SR, Waters P.

    Source
    Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, University of Oxford,
    West Wing, John Radcliffe Hospital, Oxford OX3 9DU, UK. [email protected]
    Abstract
    Several CNS disorders associated with specific antibodies to ion channels, receptors, and other synaptic proteins have been
    recognised over the past 10 years, and can be often successfully treated with immunotherapies. Antibodies to components
    of voltage-gated potassium channel complexes (VGKCs), NMDA receptors (NMDARs), AMPA receptors (AMPARs), GABA
    type B receptors (GABA(B)Rs), and glycine receptors (GlyRs) can be identified in patients and are associated with various
    clinical presentations, such as limbic encephalitis and complex and diffuse encephalopathies. These diseases can be
    associated with tumours, but they are more often non-paraneoplastic, and antibody assays can help with diagnosis. The new
    specialty of immunotherapy-responsive CNS disorders is likely to expand further as more antibody targets are discovered.
    Recent findings raise many questions about the classification of these diseases, the relation between antibodies and specific
    clinical phenotypes, the relative pathological roles of serum and intrathecal antibodies, the mechanisms of autoantibody
    generation, and the development of optimum treatment strategies.
    Copyright © 2011 Elsevier Ltd. All rights reserved.
    PMID:
     
    21777830
     
    [PubMed - indexed for MEDLINE]
    J Neurol Neurosurg Psychiatry. 2007 February; 78(2): 187–189.
    doi:  10.1136/jnnp.2006.089268

    Epilepsy and cerebellar ataxia associated with anti‐


    glutamic acid decarboxylase antibodies
    S Vulliemoz, G Vanini, A Truffert, C Chizzolini, and M Seeck

    Los anticuerpos contra la ácido glutámico decarboxilasa se han descrito en pacientes con el
    síndrome de la persona rígida, y otros como ataxia cerebelosa, y epilepsia.

    Este papel reporta un caso con epilepsia crónica focal, nistagmo de la mirada hacia arriba y ataxia
    cerebelosa asociada con una respuesta poliautoinmune que incluía anticuerpos GAD. La marcha y
    el nistagmo mejoraron bastante después de terapia de inmunosupresión con corticoesteroides y
    azatioprina. Después de la introducción de benzodiacepinas las convulsiones refractarias fueron
    completamente controladas. Los anticuerpos antiGAD deben ser buscado de manera activa en la
    epilepsia resistente a fármacos, particularmente si otras anormalidades neurológicas están
    presentes. El tratamiento con inmunosupresores y agentes acidergicos hidroxibutiricos pueden ser
    altamente efectivos.

    Los anticuerpos GAD se encuentran en DM1 así como varias enfermedades neurológicas, como el
    síndrome de la persona rígida (SPS), ataxia cerebelosa con autoinmunidad poliendocrina (CAPA),
    epilepsia y otras condiciones más raras como encefalomielitis progresiva con rigidez. También se
    ha asociado a miastenia gravis, tiroiditis y anemia perniciosa y anticuerpos paraneoplásicos. El
    tratamiento se enfoca en la modificación de la respuesta inmune y potenciar las vías
    gabaérgicas.

    En este paciente se uso metilprednisolona 500 mg IV por 5 días, seguido de prednisona 60 mg


    diario. Al final del tratamiento con metilprednisolona el nistagmo, la hemiataxia y la marcha
    mejoraron notablemente. Se uso levetiracetam y clobazam.

    [Because of the coexistence of a cerebellar syndrome and seizures in a patient with a polyautoimmune
    disorder, including GAD‐Ab, corticosteroid treatment was initiated: 500 mg intravenous
    methylprednisolone was given for 5 days, followed by 60 mg oral prednisone daily, and gabapentin
    was replaced by valproate. At the end of the methylprednisolone treatment, the nystagmus, hemiataxia
    and gait had improved and the leg pain had disappeared. Azathioprine (1.5 mg/kg/day) was
    introduced and tolerated well, allowing slow tapering of prednisolone to 5 mg/day. None the less,
    control of seizures remained unsatisfactory, prompting replacement of valproate with levetiracetam,
    and clobazam was added after 5 months. Eight months later, at the last follow‐up in November 2005,
    the nystagmus had disappeared and the hemiataxia and gait ataxia had improved markedly. The
    patient was able to walk for 1.5 miles without a walking stick and had had no seizures for the past 6 
    months. No diabetes had developed.]

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