A case of clinical and electrophysiologic improvement following a gluten-free diet in a 34-year-old man with a 7-year history of slowly progressive ataxia is reported from the University of Napoli Federico II, Napoli, Italy. Neurologic exam revealed gait ataxia, positive Romberg’s sign, nystagmus, dysarthria, dysmetria, and mild spasticity. Brain MRI showed vermis atrophy, and nerve conduction studies showed a sensory neuropathy. Vitamin E and B12 serum levels were normal. Antigliadin (AGAs) and antiendomysium antibodies (EMAs) were abnormal, and small bowel biopsy revealed total villous atrophy, crypt hyperplasia, and increased lymphocytes in the lamina propria. These signs of subclinical celiac disease had returned to normal when repeated 1 year later, following gluten-free diet. Neurological findings also improved after 2 years of dietary treatment. [1]
COMMENT. Cerebellar ataxia with classic celiac disease does not respond to the gluten-free diet, whereas the patient with subclinical celiac disease shows a remarkable improvement in neurologic signs and resolution of the villous atrophy.
Multiple cerebellar infarctions in a 7-year-old Japanese boy without cerebellar signs is reported from Shiga University, Hikone, Japan [2]. The child presented with uveitis, of undetermined cause.