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