A Case Of Paraneoplastic Neuropathy (PN) And Anti-Glycolipid Antibodies (GLA)
Subacute sensory neuronopathy (SSN) is most commonly seen in association with small cell carcinoma of the lung (SCLC), however lower motor neuron syndrome with or without upper motor neuron involvement may occur. Anti-neuronal antibodies, called “anti-Hu”, can be frequently demonstrated. Other antibodies that react with the carbohydrate epitope of ganglioside GM1, associated with primary motor PN, are not frequent. We describe a 73-year-old man with subacute onset of paraesthesiae starting in his finger and toes and spreading proximally. A few months later he developed generalized muscle weakness and was admitted to another hospital. On examination he was unable to write, as well as to stand or walk without support. Tendon reflexes were absent. He had no cranial nerve involvement and had no complain of pain. Pinprick, vibration and four limb positions were impaired distally. CSF study revealed increased protein (90 mg/dl) and lymphocytes (14/mm3). SCLC was identified in paratracheal lymphnodes and treated with chemotherapy (Carboplatinum and Etopoxide) and mediastinum irradiation (36 Gray). No clinical improvement was observed after i.v. immunoglobulins (400 mg/kg/day for 5 days). He was wheelchair-bound and unable to feed himself or to recognize the four limb position when he was admitted in the Neurological Department, University of Verona. Eye movements and winking were markedly reduced. Median (48.6 m/s) and ulnar (46 m/s) nerve study revealed slow conductions. ESP were undetectable. SSEP could not be evoked from the four limbs. Cyclophosphamide (250 mg/day for 5 days), monthly plasma exchange associated with prednisone for six months, and Azatioprine were used. Ambigous low titre of antibodies against neuronal nuclei was identified but anti-Hu antibodies were not detected. The serum antibody titres against glycolipids (GM1, GM2, GM3, GQ1b, GD1a, GD1b, sulfatides) were elevated (>1:720) and had no substantial change at the bimonthly controls in the last 36 months. As negative control case for GLa, we used the Hu-negative serum from a patient who had electrophysiologically confirmed selective sensory involvement associated with SCLC and long survival (29 months). We hypothesized that the neurological course with prominent motor deficit is probably related to serum anti-GL antibody specificities. The presence of ganglioside mimicry in the SCLC (Fuentes et al., Lung Cancer, 1997) appears to be determinant for the induction of anti-GLa. However the anti-neural immune-response may be related to host factors.
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Document Type: Abstract
Affiliations: Department of Neurological and Visual Sciences, Section of Clinical Neurology, University of Verona.
Publication date: March 1, 2001