Objective: IVIG is a good treatment for autoimmune cerebellar ataxia (high anti-GAD antibody).

Background: IVIG should be considered as a therapy for cerebellar ataxia related to autoimmune process. It has been shown to improve ataxia. Also IVIG therapy is the safest option for treatment of cerebellar ataxia in pregnancy.

Methods: Patient is a 26 yo RH AA pregnant female with h/o vitiligo and pernicious anemia since age 13 who developed difficulty walking, imbalance, fall spells and limb incoordination at the same age. Also she has been wheelchair bound since age 18. She also developed dysarthria, dysphagia and choking spells at age 20. She presented to ataxia clinic while she was in 18 weeks of her pregnancy. Her exam was remarkable for hypermetric saccades, dysarthria, truncal and limb ataxia with ataxic gait. Her MRI brain showed cerebellar degeneration at age 13 and 17. She denied family history of ataxia. She was found to have high anti-GAD per sporadic ataxia workup in ataxia clinic and and she was admitted in the hospital and treated with IVIG for 5 days with partial improvement of her symptoms. However she did not get enough benefit from treatment due to early-onset of cerebellar ataxia and late presentation for therapy. She was also worked up for other causes of autoimmune diseases and nutritional deficiencies which were insignificant.

Results: MRI brain showed cerebellar degeneration in the past. Further brain images were not obtained due to pregnancy. Anti-GAD 65 AB was elevated >30. She was found to have anti-K antibody and kell isoimmunization in pregnancy but the peripheral smear of her blood was insignificant and neuroacanthocytosis (McLeod syndrome which is a X-linked disorder) was ruled out.

Conclusions: Cerebellar ataxia is related to different etiologies but autoimmune cerebellar syndrome due to high titers of anti-GAD antibodies is a known and common cause of cerebellar ataxia. This autoimmune cerebellar syndrome is often associated with other autoimmune disorders including pernicious anemia and vitiligo that this patient has. Treatment is immunosuppression, including IVIG, imuran, PLEX. IVIG therapy is the safest option for treatment in pregnancy. Therapy should be considered early after diagnosis to get enough benefit from treatment. This patient has early-onset of the disease at age 13 but presented at age 26 for further workup so she had partial improvement of her symptoms after therapy.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/cerebellar-ataxia-and-pregnancy/