Myoclonus ataxia and refractory coeliac disease

Table 2 Summary of serological and histopathological characteristics of the 9 patients

Case	Age/sex	Gluten related antibodies baseline				Gluten related antibodies on strict gluten-free diet				Duodenal biopsy baseline	Duodenal biopsy on diet (duration on diet in years)
		EMA	TG2	AGA	TG6	EMA	TG2	AGA	TG6
1	50/M	n/a	n/a	n/a	n/a	-ve	-ve	-ve	-ve	Enteropathy	Enteropathy (10), EAL
2	60/F	+ve	n/a	+ve IgG	pos	-ve	-ve	-ve	-ve	Enteropathy	Enteropathy (5), type 1
3	63/M	+ve	+ve	+ve IgG, IgA	pos	-ve	-ve	-ve	-ve	Enteropathy	Enteropathy (3), type 1
4	53/M	n/a	n/a	n/a	n/a	-ve	-ve	-ve	-ve	Enteropathy	Enteropathy (10), type 2
5	76/M	+ve	+ve	+ve IgA	pos	-ve	-ve	-ve	-ve	Enteropathy	Enteropathy (2), type 1
6	46/F	+ve	+ve	n/a	n/a	+ve	-ve	+ve IgG	-ve	Enteropathy	Enteropathy (2), type 1
7	61/M	+ve	+ve	+ve IgG, IgA	n/a	-ve	+ve	+ve IgG	-ve	Enteropathy	Enteropathy (1), type 1
8	52/F	-ve	+ve	+ve IgG, IgA	pos	-ve	+ve	+ve IgG,IgA	-ve	Enteropathy	Enteropathy (1), type 1
9	74/M	+ve	+ve	n/a	n/a	-ve	-ve	+IgA	-ve	Enteropathy	Enteropathy (1.5), type 1

EMA = endomysium antibodies.TG2 = transglutaminase antibodies type 2. AGA = antigliadin antibodies. TG6 = transglutaminase antibodies type 6. EAL = enteropathy associated lymphoma. Enteropathy = triad of villous atrophy, crypt hyperplasia and increased intraepithelial lymphocytes. Type 1 enteropathy = refractory enteropathy. Type 2 enteropathy = refractory enteropathy with abnormal intraepithelial T cells.

ISSN: 2053-8871