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 contribution | Paraneoplastisches neurologisches Anti-Ri-Syndrom mit beidseitiger Parese des VI. Hirnnervs und Kieferdystonie – ein abgegrenztes Syndrom innerhalb des Anti-Ri-Spektrums?: Kasuistik und Literaturübersicht |
|---|---|
| Original language | English |
| Pages (from-to) | 16-21 |
| Number of pages | 6 |
| Journal | Wiener Medizinische Wochenschrift |
| Volume | 174 |
| Issue number | 1-2 |
| Early online date | 03 Mar 2023 |
| DOIs | |
| Publication status | Published - 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