Cerebellar ataxia is a disorder of gait disturbances and uncoordination that often follows viral illnesses, especially in children. In adults, autoimmune conditions of cerebellar ataxia have been increasingly found to occur in patients with co-existing conditions of thyroid disease, gluten sensitivity, enteropathy, and diabetes.
Autoantibody Involvement
Cerebellar ataxia causes a progressive gait disturbance and is accompanied by cerebral cortical atrophy and reduced blood flow. In a recent study, Japanese researchers found anti-gliadin autoantibodies, which are seen in gluten sensitivity and anti-SS-A/Ro antibodies, which are seen in Sjogren’s syndrome, in a 51-year old man with symptoms of cerebellar ataxia. Because this suggested an autoimmune cause, the patient’s physicians used intravenous immunoglobulins (IVIG) as the primary treatment. The treatment was successful and the patient was able to walk unassisted. Other studies have confirmed that thyroid antibodies and glutamic acid decarboxylase (GAD) antibodies, which are seen in diabetes, polyglandular syndromes, and Stiff-man syndrome, are also seen in autoimmune cerebellar ataxia.
Autoimmune Associations
In another recent study, researchers diagnosed autoimmune cerebellar ataxia in a 47-year old woman presenting with cerebellar ataxia associated to late-onset type 1 diabetes and polyendocrine auto-immunity. The patient had a past medical history of vitiligo and Graves' disease and presented with late-onset type 1 diabetes. For two years, she had complained of progressive gait instability and oscillopsia, a visual disturbance in which fixed objects in the visual field show movement. Neurological examination revealed visual disturbances, gait ataxia and mild limb ataxia in the left upper arm.
Immunological screening in this patient showed numerous autoantibodies, including GAD-Ab, thyroid peroxidase (TPO)-Ab, thyroglobulin-Ab, 21-hydroxylase (adrenal)-Ab, gastric parietal cell-Ab and GM1 ganglioside IgG-Ab. GAD antibodies were also seen in the cerebrospinal fluid, suggesting an autoimmune ataxia. Treatment with IVIG therapy showed some improvement.
An Organ-Specific Disorder
In an article published in the July 2008 issue of Movement Disorders, researchers in the UK report that sporadic cases of idiopathic cerebral ataxia may actually be organ-specific autoimmune disorders, targeting the cerebral cortex. By comparing immunological profiles in cases of genetic and idiopathic cerebral ataxia, the researchers concluded that the significantly higher prevalence of autoimmune diseases, the presence of the immune system gene HLA DQ2, and anti-cerebellar antibodies in patients with idiopathic sporadic ataxia compared to genetic ataxia supports the notion that autoimmunity may account for some cases of idiopathic sporadic cerebellar ataxia.
Sources:
K Nanri, T Otsuka, A case of anti-gliadin-antibody-positive cerebellar ataxia effectively treated with intravenous immunoglobulin in which voxel-based morphometry and FineSRT were diagnostically useful, Rinsho Shinkeigaku, 2009 Jan;49(1):37-42.
C Bayeurether, S Hieronimus, P Ferrari, Auto-immune cerebellar ataxia with anti-GAD antibodies accompanied by de novo late-onset type 1 diabetes mellitus, Diabetes Metab, 2008 Sep;34(4): 386-8.
M Hadjivassolioui, S Boscolono, Cerebellar ataxia as a possible organ-specific autoimmune disease, Mov Disord. 2008 Jul 30; 23(10): 1370-7.
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