Anti-Ri-associated paraneoplastic cerebellar and brainstem degenerative syndrome

J R Coll Physicians Edinb. 2012;42(3):221-4. doi: 10.4997/JRCPE.2012.307.

Abstract

We present the case of a female patient with a subacute paraneoplastic brainstem neurological syndrome associated with breast cancer and the development of anti-Ri antineuronal antibodies (ANNAs). It is an important syndrome to identify because of the need for urgent investigation and management to reduce progressive and irreversible neurological deterioration and to recognise the associated risks of bulbar and central respiratory failure. Diagnosis can be confounded if the anticipated normality of imaging and cerebrospinal fluid (CSF) studies is not appreciated. Positive antineuronal screening can provide rapid support for a paraneoplastic aetiology. Urgent and extensive investigation to identify the underlying tumour is imperative since neurological outcome is dependent on the rapidity of commencement and efficacy of tumour therapy. We discuss the symptoms, pathophysiology, diagnosis, treatment and prognosis of paraneoplastic neurological syndromes.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antigens, Neoplasm*
  • Autoantibodies*
  • Brain Stem / cytology
  • Brain Stem / immunology
  • Brain Stem / pathology*
  • Breast Neoplasms / complications*
  • Breast Neoplasms / immunology
  • Breast Neoplasms / pathology
  • Cerebellum / cytology
  • Cerebellum / immunology
  • Cerebellum / pathology*
  • Female
  • Humans
  • Nerve Tissue Proteins*
  • Neuro-Oncological Ventral Antigen
  • Neurodegenerative Diseases / etiology*
  • Neurodegenerative Diseases / immunology
  • Neurons / immunology
  • Paraneoplastic Syndromes* / diagnosis
  • Paraneoplastic Syndromes* / etiology
  • Paraneoplastic Syndromes* / immunology
  • Paraneoplastic Syndromes* / pathology
  • RNA-Binding Proteins*

Substances

  • Antigens, Neoplasm
  • Autoantibodies
  • Nerve Tissue Proteins
  • Neuro-Oncological Ventral Antigen
  • RNA-Binding Proteins