| Paraneoplastic jaw dystonia and laryngospasm with antineuronal nuclear autoantibody type 2 (anti-Ri). | |
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MedLine Citation:
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PMID: 20837856 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Opsoclonus-myoclonus syndrome and breast carcinoma were initially described as neurologic and oncologic accompaniments of antineuronal nuclear autoantibody type 2 (ANNA-2, also known as anti-Ri). However, the neurologic spectrum of ANNA-2 autoimmunity is broader, includes a syndrome of jaw dystonia and laryngospasm, and can be accompanied by lung carcinoma. OBJECTIVE: To describe clinically (with a video) ANNA-2-associated jaw dystonia and laryngospasm, its pathologic correlates, and therapeutic outcomes. DESIGN: Retrospective case series with prospective clinical follow-up. SETTING: Mayo Clinic's Neuroimmunology Laboratory, Rochester, Minnesota. PATIENTS: Consecutive patients with ANNA-2 seropositivity identified since January 1, 1990. Main Outcome METHODS: Clinical (in 9 patients) and neuropathologic (in 2 patients) findings were reviewed. RESULTS: Of 48 patients with ANNA-2 seropositivity, 9 (19%) had multifocal neurologic manifestations that included jaw dystonia and laryngospasm. Among 6 patients with jaw dystonia, 5 had severely impaired nutrition, causing profound weight loss. Five patients had documented laryngospasm, which contributed to 1 patient's death. Neuropathologic examination revealed diffuse infiltration by CD8(+) T lymphocytes, with axonal loss and gliosis in brainstem and descending spinal cord tracts. Some patients improved symptomatically after immunosuppressant or cytotoxic therapies; 1 patient improved after treatment with botulinum toxin. One patient who underwent tracheostomy because of recurrent laryngospasm was alive and well longer than 3 years after symptom onset. CONCLUSIONS: Jaw dystonia and laryngospasm are common accompaniments of ANNA-2 autoimmunity and are associated with significant morbidity. We propose that selective damage to antigen-containing inhibitory fibers innervating bulbar motor nuclei by CD8(+) T lymphocytes (histopathologically observed infiltrating brainstem reticular formation) is the proximal cause of this syndrome. Early and aggressive therapy offers the prospect of neurologic improvement or stabilization. |
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Authors:
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Sean J Pittock; Joseph E Parisi; Andrew McKeon; Shanu F Roemer; Claudia F Lucchinetti; K Meng Tan; B Mark Keegan; Samuel F Hunter; Paul R Duncan; Joachim M Baehring; Joseph Y Matsumoto; Vanda A Lennon |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Archives of neurology Volume: 67 ISSN: 1538-3687 ISO Abbreviation: Arch. Neurol. Publication Date: 2010 Sep |
Date Detail:
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Created Date: 2010-09-14 Completed Date: 2010-10-01 Revised Date: 2011-04-26 |
Medline Journal Info:
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Nlm Unique ID: 0372436 Medline TA: Arch Neurol Country: United States |
Other Details:
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Languages: eng Pagination: 1109-15 Citation Subset: AIM; IM |
Affiliation:
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Mayo Medical School College of Medicine, Mayo Clinic, Rochester, MN 55905, USA. pittock.sean@mayo.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Antibodies, Antinuclear / immunology Antibodies, Neoplasm / immunology* Brain / immunology, pathology* Dystonic Disorders / immunology*, pathology, physiopathology Female Follow-Up Studies Humans Jaw / immunology*, pathology, physiopathology Laryngismus / immunology*, pathology, physiopathology Male Middle Aged Paraneoplastic Syndromes / immunology*, pathology, physiopathology Retrospective Studies |
| Chemical | |
Reg. No./Substance:
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0/ANNA-2 antibody, human; 0/Antibodies, Antinuclear; 0/Antibodies, Neoplasm |
| Comments/Corrections | |
Comment In:
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Arch Neurol. 2011 Mar;68(3):399
[PMID:
21403033
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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