Anti-Ri paraneoplastic neurological syndrome presenting with bilateral cranial nerve VI palsy and jaw dystonia-a distinctive syndrome within the anti-Ri spectrum? Case report and literature review

Research output: Journal article (peer-reviewed)Case report

1 Citation (Scopus)

Abstract

OBJECTIVE: Paraneoplastic neurological syndromes (PNS) are rare disorders associated with various onconeuronal antibodies. Anti-Ri antibodies (ANNA-2) are typically found in patients with opsoclonus myoclonus syndrome (OMS) and ataxia.

CASE REPORT: We present an anti-Ri antibody-positive 77-year-old woman with subacute progressive bilateral cranial nerve VI palsy, gait disturbance and jaw dystonia. MRI of the brain showed hyperintense signals on T2 bitemporal without contrast enhancement. Cerebrospinal fluid (CSF) examination exhibited mild pleocytosis of 13 cells/µl and positive oligoclonal bands. CSF was overall inconspicuous for a malignant or inflammatory etiology. Immunofluorescence analysis revealed anti-Ri antibodies in both serum and CSF. Subsequent diagnostic work up resulted in a newly diagnosed ductal carcinoma of the right breast. PNS in this case partially responded to the anti-tumor therapy.

CONCLUSION: This case shows similarities with recently published anti-Ri syndromes, which might form a distinct triad within the anti-Ri spectrum.

Translated title of the contributionParaneoplastisches neurologisches Anti-Ri-Syndrom mit beidseitiger Parese des VI. Hirnnervs und Kieferdystonie – ein abgegrenztes Syndrom innerhalb des Anti-Ri-Spektrums?: Kasuistik und Literaturübersicht
Original languageEnglish
Pages (from-to)16-21
Number of pages6
JournalWiener Medizinische Wochenschrift
Volume174
Issue number1-2
Early online date03 Mar 2023
DOIs
Publication statusPublished - Feb 2024

Keywords

  • Antineuronal antibodies
  • Intracellular antigens
  • Oculomotor disorder
  • Onconeuronal antibodies
  • Surface antigens
  • Dystonia/diagnosis
  • Autoantibodies
  • Humans
  • Antibodies, Neoplasm/analysis
  • Paraneoplastic Syndromes/pathology
  • Paraneoplastic Syndromes, Nervous System/diagnosis
  • Female
  • Abducens Nerve Diseases
  • Aged

ASJC Scopus subject areas

  • General Medicine

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