Case Report
A 38-year-old woman was referred for evaluation ofabnormal movements ofarms and legs. It was suddenly installed since one month and rapidly progressive. In her history, she had one fœtal death in utero and had a brother with diabetes mellitus type 1. She was on no medication.She complained from chronic headache but she hadn’t any digestive symptome such as diarrhea or abdominal pain. On examination, she had a chorea on her four members (see video, segment1) without other neurologic syndrome. She had a BMI at19 and no othersystems’ abnormality.
Laboratory evaluations showed none absorption syndrome with mixed deficiency anaemia, hypoproteinemia and hypoalbuminemia without proteinuria, hypocalcaemia,vitamin D deficiency and some episodes of hypoglycaemia.Whereas, she had normal blood level of magnesium, triglyceride, cholesterol and homocysteine and normal creatin kinase and thyroid function. On the other hand, she had hypogammaglobulinemia, small cytolysis and no lymphopenia.
Autoimmune research showed the presence of IgG anti-reticulin antibodyin indirect fluorescent antibody assayon liver on the surch of antinuclear antibody.IgA anti-endomisium antibodywas found on stomach by the same assay. By technique of ELISA, we detected height levels of IgA and IgG of anti- desamidaded peptide of gliadine and IgA of anti-transglutaminase.IgA anti-phospholipidantibody had firstly positive with no significant level but was negative on the control after 3 months.CD was diagnosed and we completed by a small bowel exploration. Endoscopy was normal without villous atrophy but biopsy showedsub-total villous atrophy, intraepithelial lymphocytes (IEL), villous height to crypt depth ratio,which are specific of CD. Abdominals can founds omemesenteric adenopathy without digestive thickening or mass. No vascularitis nor cerebral venousthrombosis was detected by MRI with vascular exploration. Genetic study of Huntington’s disease was negative. No etiology for chorea was diagnosed other than CD spetially Wilson disease and neuroancothocytosis was eliminated by laboratory evaluations. Treatment was started with low doses of neuroleptic and gluten free diet was started one week later. Celiac serology was negative for her three kids and her brother with diabetes mellitus. The patient had difficulties for gluten free diet’s compliance. Evolution was partial regression of chorea and headache. Two months later, she developed extrapyramidal syndrome because of neuroleptic treatment