Intravenous immunoglobulins in paraneoplastic brainstem encephalitis with anti-Ri antibodies

Excerpt

Sirs: We report the case of a 71 year old man who developed over 2 days severe cerebellar ataxia, dysarthria, dysphagia, opsoclonus-myoclonus, nausea and vomiting. Brain MRI was normal. The cerebrospinal fluid (CSF) contained 39 lymphocytes/mm3 with normal protein and glucose levels. An extensive biological investigation remained negative except for the presence of anti-Ri antibodies. These anti-Ri antibodies were demonstrated by a combination of immunofluorescence analysis and Western blot (anti-neuronal antibody Western blot immunoassay, Immco Diagnostics Inc., Buffalo, NY). Serial dilutions revealed a specific antibody titre of 1 : 320. Thoracic CT revealed a suspect nodular mass in the right lung (middle lobe). Bronchoscopy with broncho-alveolar wash was normal. A full-body PET was planned but due to the severity of dysphagia and the fact that patient became bedridden, intravenous immunoglobulins (IVIg, 400 mg/kg/day for 5 days) were administered 4 weeks after symptom onset. This treatment induced a clear-cut clinical improvement after 1 week, allowing removal of the nasogastric tube and a restart of oral feeding. The patient could walk with some aid. Retest for anti-Ri antibodies was negative. The PET scan was in favour of a right lung neoplasm. Unfortunately, the patient developed a pyelonephritis with septicaemia and died 8 weeks after the onset of symptoms. Autopsy confirmed a diagnosis of lung adenocarcinoma. …