49. Effectiveness of subcutaneous immunoglobulins in paraneoplastic CIDP caused by signet ring cell adenocarcinoma

Abstract

We describe a women 54-year-old, heavy smoker (20 cigarettes/day) who was admitted to our hospital after emergency department admission because of an acute flaccid tetra paresis. Lumbar puncture revealed albumino-cytologic dissociation with 4, 3 gr/L proteins and no cells in CSF. Electromyography concluded for a demielinating poliradicoloneuropathy. Intravenous immunoglobulins (ivIg) 2 gr/Kg in 5 days produced discrete improvement of weakness. Cerebral and cervical spine MRI showed only a left neurinoma of left acustic nerve. Chest and abdomen CT scan revealed a non-specific lung nodule. She was transferred in a rehabilitation department with a near-complete resolution of strength deficits. Patient came back after one year with a severe strength worsening and wheel-chair bounded (MRC score: 22/60). She started 2 gr/Kg and then subcutaneous Ig 1 mg/Kg/months(0.2 mg/Kg every 5 days). In 6 months patient showed a progressive improvement up to MRCscore 40/60.TC-PET scan showed multiple areas of hypermetabolism in the back bone and in anterior-superior iliac spine. The latter was biopsied showing signet-ring cells. Clinical course of patient quickly worsened with drug-resistant seizures and obstructive hydrocephalus which required neurosurgery. After few weeks she died at home. Before neoplastic radiological evidence, subcutaneous Ig were effective in treating strength deficits of this paraneoplastic CIDP.