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April 17, 2018; 90 (16) Video NeuroImages

Video NeuroImages: Head titubation in anti-mGluR1 autoantibody-associated cerebellitis

José Luiz Pedroso, Lívia Almeida Dutra, Alberto J. Espay, Romana Höftberger, O.G.P. Barsottini
First published April 16, 2018, DOI: https://doi.org/10.1212/WNL.0000000000005338
José Luiz Pedroso
From the Neurology and Ataxia Unit, Department of Neurology (J.L.P., L.A.D., O.G.P.B.), Universidade Federal de São Paulo; Faculdade Israelita de Ciências da Saúde Albert Einstein (L.A.D.), São Paulo, Brazil; Gardner Center for Parkinson's Disease and Movement Disorders (A.J.E.), University of Cincinnati Gardner Neuroscience Institute, OH; and Institute of Neurology (R.H.), Medical University of Vienna, Austria.
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Lívia Almeida Dutra
From the Neurology and Ataxia Unit, Department of Neurology (J.L.P., L.A.D., O.G.P.B.), Universidade Federal de São Paulo; Faculdade Israelita de Ciências da Saúde Albert Einstein (L.A.D.), São Paulo, Brazil; Gardner Center for Parkinson's Disease and Movement Disorders (A.J.E.), University of Cincinnati Gardner Neuroscience Institute, OH; and Institute of Neurology (R.H.), Medical University of Vienna, Austria.
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Alberto J. Espay
From the Neurology and Ataxia Unit, Department of Neurology (J.L.P., L.A.D., O.G.P.B.), Universidade Federal de São Paulo; Faculdade Israelita de Ciências da Saúde Albert Einstein (L.A.D.), São Paulo, Brazil; Gardner Center for Parkinson's Disease and Movement Disorders (A.J.E.), University of Cincinnati Gardner Neuroscience Institute, OH; and Institute of Neurology (R.H.), Medical University of Vienna, Austria.
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Romana Höftberger
From the Neurology and Ataxia Unit, Department of Neurology (J.L.P., L.A.D., O.G.P.B.), Universidade Federal de São Paulo; Faculdade Israelita de Ciências da Saúde Albert Einstein (L.A.D.), São Paulo, Brazil; Gardner Center for Parkinson's Disease and Movement Disorders (A.J.E.), University of Cincinnati Gardner Neuroscience Institute, OH; and Institute of Neurology (R.H.), Medical University of Vienna, Austria.
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O.G.P. Barsottini
From the Neurology and Ataxia Unit, Department of Neurology (J.L.P., L.A.D., O.G.P.B.), Universidade Federal de São Paulo; Faculdade Israelita de Ciências da Saúde Albert Einstein (L.A.D.), São Paulo, Brazil; Gardner Center for Parkinson's Disease and Movement Disorders (A.J.E.), University of Cincinnati Gardner Neuroscience Institute, OH; and Institute of Neurology (R.H.), Medical University of Vienna, Austria.
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Citation
Video NeuroImages: Head titubation in anti-mGluR1 autoantibody-associated cerebellitis
José Luiz Pedroso, Lívia Almeida Dutra, Alberto J. Espay, Romana Höftberger, O.G.P. Barsottini
Neurology Apr 2018, 90 (16) 746-747; DOI: 10.1212/WNL.0000000000005338

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A 39-year-old woman presented with apathy, catatonia, and continuous large-amplitude oscillatory head movements (head titubation) evolving over 1 month (video, links.lww.com/WNL/A353). Severe ataxia developed weeks later. Brain MRI, EEG, and serologies were normal. Cell- and tissue-based assays were positive for anti–metabotropic glutamate receptor 1 (mGluR1) antibodies (serum 1:12,800, CSF 1:512; figure). Head titubation, a slow-frequency cerebellar outflow tremor of the head associated with axial hypotonia, may appear in cerebellar abnormalities (Joubert syndrome, Dandy-Walker syndrome) or disorders affecting the anterior lobe of the cerebellum.1 Subacute head titubation should raise suspicion for an mGluR1-associated autoimmune cerebellitis, especially if associated with psychiatric changes.2

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Figure Tissue and cell-based assay for metabotropic glutamate receptor 1 (mGluR1)

Indirect immunohistochemistry on rat brain with patient's CSF shows a neuropil staining pattern in the dentate gyrus and CA3 sector of the hippocampus (A) and molecular layer of the cerebellum (B). Antibodies of the patient are identified on HEK293T cells transfected with mGluR1-EGFP (C; red: CSF of the patient, green: mGluR1-EGFP, blue: Dapi) (A, B: ×40; C: ×400).

Author contributions

J.L. Pedroso: case report project conception, organization, execution; writing of the first draft, review and critique. L.A. Dutra: case report project conception, organization, execution; writing of the first draft, review and critique. A.J. Espay: writing of the first draft, review and critique. R. Hoftberger: case report project execution; writing of the first draft, review and critique. O.G. Barsottini: writing of the first draft, review and critique.

Study funding

No targeted funding reported.

Disclosure

J. Pedroso and L. Dutra report no disclosures relevant to the manuscript. A. Espay has received grant support from the NIH, Great Lakes Neurotechnologies, and the Michael J. Fox Foundation; personal compensation as a consultant/scientific advisory board member for AbbVie, TEVA, Impax, Acadia, Acorda, Cynapsus/Sunovion, Lundbeck, and USWorldMeds; publishing royalties from Lippincott Williams & Wilkins, Cambridge University Press, and Springer; and honoraria from AbbVie, UCB, USWorldMeds, Lundbeck, Acadia, the American Academy of Neurology, and the Movement Disorders Society. R. Hoftberger received grant support from the Jubilaeumsfonds der Oesterreichischen Nationalbank, project 16919. O. Barsottini reports no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

  • © 2018 American Academy of Neurology

References

  1. 1.↵
    1. Zanni G
    . Head titubation: an unrecognized manifestation of Joubert syndrome and other (midline) cerebellar disorders? Dev Med Child Neurol 2014;56:925–926.
    OpenUrl
  2. 2.↵
    1. Lopez-Chiriboga AS,
    2. Komorowski L,
    3. Kümpfel T, et al
    . Metabotropic glutamate receptor type 1 autoimmunity: clinical features and treatment outcomes. Neurology 2016;86:1009–1013.
    OpenUrlAbstract/FREE Full Text

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