Sclérose latérale primitive et adénocarcinome bronchique. Syndrome paranéoplasique ou association fortuite ?

Abstract

Lung cancer is frequently associated with paraneoplastic syndromes, sometimes uncommon, among which motor neurone disease is sometimes described, including primary lateral sclerosis, a disorder characterized by slowly progressive cortico-spinal dysfunction due to the degeneration of the upper motor neurone.We report a case of primary lateral sclerosis developing in a young woman 9 months after the diagnosis of metastatic adenocarcinoma of the lung. Our patient showed a spastic quadriparesis, dysarthria and bulbar symptoms without amyotrophy nor fasciculation. The electromyogram showed isolated upper motor neurone involvement. Cerebral and medullary imaging and CSF analysis showed no abnormality and onconeuronal antibodies were negative. The neurological symptoms and the cancer deteriorated simultaneously and our patient died 6 months after the onset of neurological symptoms.Motor neurone involvement is rare but some case reports describe an association with neoplasia without formal confirmation of a paraneoplastic syndrome. Our case is the first report of primary lateral sclerosis in a young woman diagnosed during the treatment of lung adenocarcinoma.