International Journal of Neuroscience, 122, 545–546, 2012 Copyright © 2012 Informa Healthcare USA, Inc. ISSN: 0020-7454 print / 1543-5245 online DOI: 10.3109/00207454.2012.683220 Gluten Ataxia: An Important Treatable Etiology of Sporadic Ataxia Faten Abou Ghazal, Swaraj Singh, Shadi Yaghi, and Salah G. Keyrouz Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA ABSTRACT Background: Gluten ataxia is a known cause of sporadic ataxia. Case: We report the case of a patient with imbalance and no abdominal symptoms diagnosed with gluten ataxia. Her neurological symptoms responded to gluten-free diet. Conclusion: Gluten ataxia should be considered in all patients with sporadic ataxia with or without abdominal symptoms, as early diagnosis and treatment may result in neurological improvement. KEYWORDS: ataxia, celiac disease, neurologic complications INTRODUCTION Many neurological symptoms can accompany celiac dis- ease (CD). Ataxia is one such manifestation that could occur in the course of CD, with or without associated enteropathy. We report the case of a patient with ataxia related to underlying CD. SUBJECT A 70-year-old woman with Sjogren syndrome presented with a two-year history of imbalance. The patient de- scribed her complaint as unsteadiness, and swaying to one side, mostly to the right. She denied numbness, weakness, and dysautonomic symptoms. Review of sys- tem was positive for fatigue. Physical examination re- vealed gaze evoked nystagmus and marked dysmetria on heel-knee-shin bilaterally and on left fnger-nose-fnger. She had preserved deep tendon refexes all through and no autonomic dysfunction. Labs showed microcytic anemia; normal Vitamin B12, B1, Vitamin E levels; and HIV was negative. Spine MRI was normal. Brain MRI showed atrophy of the cerebellar vermis (see Figure 1). Cerebrospinal fuid (CSF) analysis revealed no pleocy- tosis with normal protein, and glucose. Autoimmune ataxia was suspected after other causes of sporadic ataxia Received 24 January 2012. Address correspondence to Faten Abou Ghazal, M.D., Department of Neurology, University of Arkansas for Medical Sciences, 4301 West Markham, Slot 500, Little Rock, AR 72205, USA. E-mail: fkabughazal@uams.edu (SA) were ruled out based on history, clinical exami- nation, and investigations (see Table 1). Antigliadin an- tibodies (IgG and IgA) and anti Human tissue trans- glutaminase antibody (IgA) were positive, antithyroid peroxidase (anti-TPO) and anti-Human tissue transglu- taminase antibody (IgG), however, were negative. Duo- denal biopsy was then performed and confrmed the di- agnosis of CD (see Figure 1). Patient was started on a gluten-free diet, which resulted in marked improve- ment in her balance. Two months after starting the diet, physical examination improved, revealing minimal gaze- evoked nystagmus, mildly ataxic heel-knee-shin bilater- ally, and on left fnger-nose-fnger. Antigliadin antibody (AGA) IgG normalized 2 months following gluten-free diet. DISCUSSION CD occurs in 1% of the US population, affecting indi- viduals with heightened sensitivity to gluten, a compo- nent of dietary grains. Autoimmune destruction of the intestinal villi is the hallmark of the disease that man- ifests in diarrhea, fatty stools, malabsorption, abdomi- nal pain, and distension [1]. Numerous nervous system conditions, such as ataxia, peripheral neuropathy, de- mentia, and epilepsy, can be associated with CD [2]. Gluten ataxia (GA) describes patients with positive AGA and SA with no other identifable etiology. It is suggested that AGA cross-reacts with epitopes on purkinje fbers of the cerebellum, thereby causing the dysfunction [3]. Ataxia could develop in the absence 545