J Clin Neurol.  2009 Sep;5(3):151-152. 10.3988/jcn.2009.5.3.151.

Anti-Ri-Antibody-Associated Paraneoplastic Syndrome in a Man with Breast Cancer Showing a Reversible Pontine Lesion on MRI

• Kim H
• Lim Y
• Kim KK

• KMID: 2045423
• DOI: http://doi.org/10.3988/jcn.2009.5.3.151

Abstract

BACKGROUND
Paraneoplastic neurological disorders associated with anti-Ri-antibodies, which are typically present with opsoclonus-myoclonus-ataxia. Most cases with anti-Ri-antibodyassociated paraneoplastic syndrome due to breast cancer occur in women - its occurrence in men is extremely rare. CASE REPORT: We present herein the case of a male patient with breast cancer who had atypical anti-Ri-antibody-associated paraneoplastic syndrome presenting as complete horizontal ophthalmoplegia, left trigeminal sensory symptoms, and truncal ataxia. Following the diagnosis of paraneoplastic syndrome, chemotherapy and immunomodulating treatment including intravenous immunoglobulin and oral prednisolone were administered. Although the patient was negative for serum anti-Ri-antibodies 14 weeks later, his symptoms persisted. CONCLUSIONS: To our knowledge, this is the first case report of ophthalmoplegia without opsoclonus-myoclonus in a male anti-Ri-antibody-positive patient with breast cancer.

Keyword

breast cancer; anti-Ri-antibody; paraneoplastic syndrome

MeSH Terms

Ataxia
Breast
Breast Neoplasms
Female
Humans
Immunoglobulins
Male
Multiple Endocrine Neoplasia Type 1
Nervous System Diseases
Ophthalmoplegia
Paraneoplastic Syndromes
Prednisolone
Immunoglobulins
Prednisolone

Figure

• 

  Fig. 1 T2-weighted magnetic resonance images showing bilateral symmetric hyperintense lesions in the pontine tegmentum. A: Parapontine reticular formation (arrow) in the lower pons. B: Medial longitudinal fasciculus (arrow) in the mid pons.

Reference

1. Luque FA, Furneaux HM, Ferziger R, Rosenblum MK, Wray SH, Schold SC Jr, et al. Anti-Ri: an antibody associated with paraneoplastic opsoclonus and breast cancer. Ann Neurol. 1991. 29:241–251.

2. Escudero D, Barnadas A, Codina M, Fueyo J, Graus F. Anti-Ri-associated paraneoplastic neurologic disorder without opsoclonus in a patient with breast cancer. Neurology. 1993. 43:1605–1606.

3. Ohmer R, Golnik KC, Richards AI, Kosmorsky GS. Ophthalmoplegia associated with the anti-Ri antibody. J Neuroophthalmol. 1999. 19:246–248.

4. Voltz R. Paraneoplastic neurological syndromes: an update on diagnosis, pathogenesis, and therapy. Lancet Neurol. 2002. 1:294–305.

5. Wirtz PW, Sillevis Smitt PA, Hoff JI, de Leeuw B, Lammers GJ, van Duinen SG, et al. Anti-Ri antibody positive opsoclonus-myoclonus in a male patient with breast carcinoma. J Neurol. 2002. 249:1710–1712.

6. Pittock SJ, Lucchinetti CF, Lennon VA. Anti-neuronal nuclear autoantibody type 2: paraneoplastic accompaniments. Ann Neurol. 2003. 53:580–587.

7. Kastrup O, Meyring S, Diener HC. Atypical paraneoplastic brainstem encephalitis associated with anti-ri-antibodies due to thymic carcinoma with possible clinical response to immunoglobulins. Eur Neurol. 2001. 45:285–287.

8. Sutton IJ, Barnett MH, Watson JD, Ell JJ, Dalmau J. Paraneoplastic brainstem encephalitis and anti-Ri antibodies. J Neurol. 2002. 249:1597–1598.

9. Ko MW, Dalmau J, Galetta SL. Neuro-ophthalmologic manifestations of paraneoplastic syndromes. J Neuroophthalmol. 2008. 28:58–68.

10. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 9-1988. A 57-year-old woman with worsening opsoclonus. N Engl J Med. 1988. 318:563–570.

11. Giordano SH, Cohen DS, Buzdar AU, Perkins G, Hortobagyi GN. Breast carcinoma in men: a population-based study. Cancer. 2004. 101:51–57.