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A follow up study of patients with paraneoplastic neurological disease in the United Kingdom

¹ Institute of Neurology and National Hospital for Neurology and Neurosurgery, London
² Department of Molecular Sciences, Institute of Neurology and National Hospital for Neurology and Neurosurgery, London

Correspondence to:
Correspondence to:
Dr J H Rees
Room 724, Institute of Neurology, Queen Square, London, WC1N 3BG; j.rees{at}ion.ucl.ac.uk

Objectives: To examine the range of clinical phenotypes, tumour associations, relevant investigations, response to therapy and outcome in a large series of non-selected patients with paraneoplastic neurological disease (PND) affecting the central nervous system (CNS) in the United Kingdom.

Methods: Data were obtained on patients either through direct referral or through the British Neurological Surveillance Unit (BNSU) from February 2000 to January 2001. Physicians were asked to supply information about age and sex of patients, presenting neurological syndromes, the basis of the diagnosis of PND, any associated malignancy, and treatment. Case notes were reviewed and follow up data obtained where possible one year after notification.

Results: A total of 63 patients (48 females, 15 males) were identified, 48 through the BNSU and 15 through direct referral. Of these 52 were diagnosed as having definite PND, 10 probable PND, and 1 possible PND. The median age of onset of PND was 66 years (range 30–80 years) and only 7 patients (11%) were less than 50 years at presentation. In 53 patients (84%) the PND preceded the diagnosis of cancer. Paraneoplastic sensory neuronopathy, paraneoplastic encephalomyelitis, and paraneoplastic cerebellar degeneration (PCD) were the most common syndromes reported. The benefit of magnetic resonance imaging in the diagnosis of the disease was limited, while fluorodeoxyglucose positron emission tomography was shown to be useful for the detection of an occult malignancy in 10 out of 14 patients. Antineuronal antibodies were positive in 44/57 (77%) of cases. The following tumours were diagnosed: small cell lung cancer (30%), breast cancer (14%), ovarian cancer (8%), non-small cell lung cancer (8%), Hodgkin’s lymphoma (6%), other (16%). With the exception of PCD associated with mesothelioma all other tumours diagnosed in these patients had been previously documented as being associated with PND. Only treatment of the tumour was found to be associated with a stable or improved neurological outcome at last follow up (Fisher‘s exact test = 4.7, p<0.03). Median survival time was 43 months (95% CI 28 to 57) from onset of neurological disease as calculated using the Kaplan–Meier survival analysis.

Conclusions: PND has a striking female preponderance usually affecting patients in their sixth decade and above. The median survival in our study was 43 months. The majority of patients with PND are not known to have cancer at the time of diagnosis. Our study confirms the importance of diagnosing and treating the underlying tumour.

Abbreviations: AUP, adenocarcinoma of unknown primary; BNSU, British Neurological Surveillance Unit; CI, confidence interval; CIDP, demyelinating polyradiculoneuropathy; CNS, central nervous system; FDG-PET, fluorodeoxyglucose positron emission tomography; GBS, Guillain-Barré syndrome; IVIg, Intravenous immunoglobulin; LEMS, Lambert-Eaton myasthenic syndrome; NSCLC, non-small cell lung cancer; PCD, paraneoplastic cerebellar degeneration; PEM, paraneoplastic encephalomyelitis; PSN, paraneoplastic sensory neuronopathy; PND, paraneoplastic neurological diseases; SCLC, small cell lung cancer

Keywords: cerebellar degeneration; encephalomyelitis; follow up study; outcome; paraneoplastic

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