Abstract

Introduction. Paraneoplastic encephalomyelitis (PEM) and subacute sensory neuronopathy (SSN) are remote effects of cancer, usually associated with small-cell lung carcinoma and positive anti-Hu antibody. We describe the rare association of bladder transitional cell carcinoma (TCC) with anti-Hu antibody positivity resulting in this paraneoplastic neurological syndrome. Patient. A 76-year-old female presented with bilateral muscle weakness and paraesthesia of the upper and lower limbs in a length-dependent “glove and stocking” distribution. Central nervous system symptoms included cognitive problems, personality change, and truncal ataxia. Case notes and the literature were reviewed. Result. Autoantibody screening was positive for anti-Hu antibody (recently renamed antineuronal nuclear antibody 1, ANNA-1). The diagnosis of PEM and SSN was supported by MRI and lumbar puncture results. A superficial bladder TCC was demonstrated on CT and subsequently confirmed on histology. No other primary neoplasm was found on full-body imaging. The neurological symptoms were considered to be an antibody-mediated paraneoplastic neurological syndrome and improved after resection of the tumour. Discussion. The association of anti-Hu positive paraneoplastic neurological syndrome and TCC has not been described in the literature previously. We emphasize the need for detailed clinical examination and the importance of a multidisciplinary thought process and encourage further awareness of this rare association.

Highlights

  • Paraneoplastic encephalomyelitis (PEM) and subacute sensory neuronopathy (SSN) are remote effects of cancer, usually associated with small-cell lung carcinoma and positive anti-Hu antibody

  • The antineuronal nuclear antibody 1 (ANNA-1) previously called as anti-Hu antibody directed against intracellular antigens is a polyclonal IgG (35–40 kD) type antibody which binds to tumours and neural cells [1]

  • In this paper we present a female patient with anti-Hu antibody who had presented with peripheral sensory neuropathy and cerebellar symptoms as part of paraneoplastic neurological process associated with superficial transitional cell carcinoma (TCC) of the bladder

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Summary

Introduction

The antineuronal nuclear antibody 1 (ANNA-1) previously called as anti-Hu antibody directed against intracellular antigens is a polyclonal IgG (35–40 kD) type antibody which binds to tumours and neural cells [1]. The binding can cause neurological symptoms such as sensory neuropathy, cerebellar ataxia, limbic encephalitis, brainstem encephalitis, intestinal pseudoobstruction, parietal encephalitis, or multifocal involvement as part of a paraneoplastic neurological syndrome [2]. These symptoms usually precede the diagnosis of the primary cancer, which is most of the time small in size and is found in an early, nonmetastatic phase. In this paper we present a female patient with anti-Hu antibody who had presented with peripheral sensory neuropathy and cerebellar symptoms as part of paraneoplastic neurological process associated with superficial transitional cell carcinoma (TCC) of the bladder. We demonstrate the importance of maintaining an open mind to additional common and rare diagnoses and to look for rare associations in patients with paraneoplastic syndrome

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