Successful immunosuppressant therapy of severe progressive cerebellar degeneration and sensory neuropathy: a case report

A 56 year old woman had a 19 month history of a severe subacute progressive cerebellar degeneration, peripheral sensory neuropathy, and urinary incont inence. She was confined to a wheelchair, needed assistance with eating, an d her speech was almost unintelligible. No underlying cancer was found desp ite repeated investigations, and no autoantibodies were demonstrated. She r eceived a 3-month course of intensive immunosuppressant therapy with intrav enous immunoglobulin 400 mg/kg per day for 5 days every month, oral cycloph osphamide 50 mg twice or three times a day to maintain the total lymphocyte count between 500 and 750/mm(3), and prednisone 60 mg per day. She experie nced dramatic subjective and objective improvement. The dysarthria and the upper extremity dysmetria disappeared, and she regained the ability to writ e and cook. The lower extremity ataxia improved and she became able to walk with a cane. Urinary incontinence disappeared. A trial of intensive immuno suppressant treatment is worth considering in a patient with a clinical syn drome resembling paraneoplastic disorders, even if an underlying neoplasm a nd autoantibodies are not demonstrated. (C) 2000 Elsevier Science B.V. All rights reserved.