There is increasing evidence that there is an antibody mediated, autoimmune component in hereditary and sporadic cerebellar degeneration. The objective of this study is to show clinical improvement or stabilization in ataxia using treatment with intravenous immunoglobulin (IVIG) treatment. This study is a clinical, randomized, placebo-controlled trial of IVIG in adult patients with cerebellar ataxia. Patients will be selected from the motor control clinic based on a diagnosis of sporadic or hereditary cerebellar degeneration. Study drug (placebo versus active) will be given in an intravenous infusion over a 2-day period while the patient is hospitalized. This will be repeated monthly for 2 months. Efficacy measurement will include clinical ataxia rating scales, motor speed testing and clinical gait evaluation. These will be performed at baseline, during each hospitalization, and 1 month following final IVIG infusion. Main outcome measure will be difference from baseline in score on the NINDS ataxia rating scale 1 month following 2 treatments of 2 mg/kg IVIG (total 4 mg/kg).
INCLUSION CRITERIA: Adults over 18 with hereditary or sporadic cerebellar degeneration. Sporadic cerebellar degeneration may include the cerebellar predominant variant of Multiple System Atrophy (MSA-C). Hereditary ataxia is limited to the SCAs (spinocerebellar ataxias) or those patients with clear autosomal dominant ataxia. Patients must also have evidence for an immune component to their condition such as gluten-sensitivity or antiganglioside antibodies. EXCLUSION CRITERIA: Patients on the gluten-free diet. Those who wish to participate in this trial must be off the diet for a period of 3 months prior to the start of the study. Patients with Friedreich's ataxia. To date, this has not been associated with autoimmune phenomena. We would not expect this population to respond. Patients with other autosomal recessive and mitochondrial forms of ataxia, since autoimmunity has not been studied in this population. Patients with hypercoaguable disorders. This includes conditions like Protein C or S deficiency, underlying malignancy and/or paraproteinemia. Patient with acute renal insufficiency or patients on known nephrotoxic drugs. Patients with selective IgA deficiency Known paraneoplastic cerebellar degeneration. Cerebellar ataxia that is congenital, static and/or symptomatic (due to stroke, tumor, demyelinating or infectious). Women who are pregnant or lactating. Those of child-bearing age will be asked to use effective contraception for the duration of the study. Those patients who do not wish to use a product derived from human serum (for example, Jehovah's Witness).