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Abstract Details

Autoimmune-Mediated Cerebellar Ataxia: Observation of Clinical Response to Intravenous Immunoglobulin
Neural Repair/Rehabilitation
P03 - (-)
269
BACKGROUND: Intravenous immunoglobulin (IVIG) has been used in treating many autoimmune-mediated neurological disorders. However, there are no current guidelines for clinical use of IVIG in treating cerebellar ataxia and the reported clinical response is varied.
DESIGN/METHODS: A clinical observation of response to IVIG therapy in an adult patient with progressive cerebellar ataxia assumed to be autoimmune-related due to the presence of anti-thyroid peroxidase antibodies. Combined follow-up neurological examinations and VNG tests were used to monitor the disease and response to treatment.
RESULTS: A 57-year old woman presented with progressive dysartria, down-beating nystagmus, diplopia, hyporeflexia, dysmetria, and gait ataxia. She was diagnosed with cerebellar ataxia and underwent extensive investigations for possible causes. Brain MRI was normal. Imaging studies for underlying malignancy were negative. Laboratory tests for nutritional deficiencies, neurodegenerative diseases, paraneoplastic syndromes, infectious, and hereditary disorders were unremarkable except elevated myelin basic protein in cerebrospinal fluid, elevated serum thyroid stimulating hormone and anti-thyroid peroxidase antibody titer. Autoimmune-mediated cerebellar pathology was suspected. Initial VNG revealed constant down-beating nystagmus and other findings of markedly impaired oculomotor control. Periodic IVIG treatments yielded consistent clinical improvement in symptoms and cerebellar signs, as noted in physical examinations and repeat VNG.
CONCLUSIONS: Autoimmune-mediated cerebellar ataxia may be related to thyroid disease in the presence of autoantibodies. Immune-modulating treatments such as IVIG can be effective. VNG appears to be valuable in assessing clinical response to IVIG therapy in conjunction with routine physical examination.
Authors/Disclosures
Nabeel Herial, MD, MPH, FAAN (Albany Medical Center)
PRESENTER 		Dr. Herial has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Medtronic.
Mehmood Rashid, MD (Neuroscience Center) Dr. Rashid has nothing to disclose.
Hongyan Li, MD, PhD, FAAN (University of Toledo Coll of Med & Life Sci - Dept of Neurology) Dr. Li has nothing to disclose.
Michael S. Okun, MD, FAAN (University of Florida) Dr. Okun has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH. Dr. Okun has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Parkinson's Foundation. Dr. Okun has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for JAMA Neurology. Dr. Okun has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for NEJM Journal Watch. The institution of Dr. Okun has received research support from NIH. The institution of Dr. Okun has received research support from Parkinson's Foundation. The institution of Dr. Okun has received research support from Tourette Association of America. The institution of Dr. Okun has received research support from Michael J Fox. Dr. Okun has received publishing royalties from a publication relating to health care.