Carcinoma associated paraneoplastic peripheral neuropathies in patients with and without anti-onconeural antibodies

Abstract

Objective-When to suspect a paraneoplastic disorder is a puzzling problem that has not recently been studied in a large series of patients referred for peripheral neuropathy. Methods-From 422 consecutive patients with peripheral neuropathy, 26 were analysed who concomitantly had carcinoma but no tumorous infiltration, drug toxicity, or cachexia. Their clinical, pathological, and electrophysiological data were analysed according to the presence of anti-onconeural antibodies, the latency between presentation and cancer diagnosis, and the incidence of carcinoma in the corresponding types of neuropathy of the population of 422 patients. Results-Seven patients (group I) had anti-onconeural antibodies (six anti-Hu, one anti-CV2) and 19 did not (groups IIA and B). In group I, subacute sensory neuropathy (SSN) was the most frequent but other neuropathies including demyelinating neuropathies were present. Patients in group II A had a short latency (mean 7.88 months), and a rapidly and usually severe neuropathy which corresponded in 11/14 to an established inflammatory disorder including neuropathy with encephalomyelitis, mononeuritis multiplex, and acute or chronic inflammatory demyelinating polyneuropathy (CIDP). Patients in group IIB had a long latency (mean 8.4 years) and a very chronic disorder corresponding in four of five to an axonal noninflammatory polyneuropathy. In this population, the incidence of carcinoma occurring with a short latency was 47% in sensory neuronopathy, 1.7% in GuillainBarré syndrome, 10% in mononeuritis multiplex and CIDP, and 4.5% in axonal polyneuropathy. Conclusions-Paraneoplasticneuropathies associated with carcinoma are heterogeneous disorders. Neuropathies occurring with a long latency with tumours probably resulted from a coincidental association. Neuropathies which occurred within a few years of the tumour evolved rapidly and corresponded mostly to inflammatory disorders. As dysimmune neuropathies are probably paraneoplastic in a limited number of cases, patients with these disorders should probably not be investigated systematically for carcinoma in the absence of antionconeural antibodies, except when the neuropathy is associated with encephalomyelitis and probably with vasculitis. Questions remain concerning CIDP. (J Neurol Neurosurg Psychiatry 1999;67:7-14) Keywords: paraneoplastic neurological syndromes; peripheral neuropathy; Guillain-Barré syndrome; chronic inflammatory demyelinating neuropathy Depending on diagnostic criteria, up to 50% of patients with carcinoma develop peripheral neuropathy. 1Treatment toxicity, tumorous infiltration, metabolic disturbances, or terminal cachexia account for most cases.2 Paraneoplastic neuropathies are rare and heterogeneous disorders.3 Some of them are part of complex syndromes involving simultaneously the central (CNS) and peripheral nervous systems (PNS), the most frequent of which is subacute sensory neuronopathy/paraneoplastic encephalomyelitis (SSN/PEM). 4 This disorder, when occurring with small cell lung cancer, is almost i...

Weakness and numbness after chemotherapy for metastatic non-seminoma testis: A new neurological complication

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“…In some cases a relationship with a specific antibody can be established, a so-called anti-onconeural antibody [4].…”

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“…Considering that dyskerin and TERT are both constituents of the active human telomerase enzyme complex [4] and that TERT has been indicated as a potential biomarker for colorectal cancer [6], we wished to check whether dyskerin and TERT mRNA levels would vary in parallel in colorectal tumors. According to the multiple role played by dyskerin in ribosome biogenesis, its expression levels were expected to be highly variable in different patients, possibly depending on age, sex and general metabolic conditions.…”

Polyneuropathie im Alter

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