[Show abstract][Hide abstract]ABSTRACT: Introduction: Subacute sensory neuronopathy (SSN) is characterized by degeneration of T cells of dorsal root ganglia. This syndrome is associated with autoimmune diseases (i.e., Sjogren's Syndrome), tumours (i.e., small-cell lung cancer), or idiopathic. Paraneoplastic SSN is frequently associated with the presence in the serum of anti-Hu antibodies (or ANNA 1). Conversely, anti-Ri antibodies (or ANNA 2) have been associated with paraneoplastic neurological syndromes as opsoclonus-myoclonus or cerebellar degeneration. Case Report: A 62-year-old man presented subacute back pain, gait difficulty, four limbs movement uncoordination and paresthesias/dysesthesias. Neurological examination also revealed involuntary pseudoathetoid movements at upper limbs, loss of vibration sense at lower limbs and diffuse absence of deep tendon reflexes. Nerve conduction studies revealed an axonal sensory polyneuropathy. Cerebrospinal fluid proteins were raised, with increased cell count. Chest CT disclosed a lung neoplasm. Serum anti-Ri antibodies were detected using indirect immunofluorescence and confirmed by Western blotting, while anti-Hu were absent. Conclusion: We report a case of paraneoplastic subacute sensory neuronopathy associated with serum anti-Ri antibodies. To our knowledge this is the first description of such an association.
No preview · Article · Apr 2004 · Journal of the Peripheral Nervous System