Table 1

Ataxic patients without definite diagnosis (group A)

Patient	Sex	Age (y)	Age of onset (y)	Symptoms at onset	Pyramidal signs	Signs of peripheral neuropathy	NCS	Brain MRI	Gastrointestinal symptoms	Malabsorption signs	AGA/EMA	Duodenal biopsy
1	M	37	12	Aggressiveness, ataxic gait	No	No	P	A	No	No	N	NP
2	M	22	15	Ataxic gait, spasticity	Yes	Yes	N	A+C+WM	Colic pain, constipation	No	N	NP
3	M	38	27	Ataxic gait	No	No	P	A	No	No	AGA and EMA+	P
4	M	43	25	Weakness, dizziness	No	No	NP	A+B+C	Constipation	No	N	NP
5	F	21	16	Ataxic gait	No	No	N	A	No	No	N	NP
6	M	27	6	Ataxic gait	Yes	No	NP	A+B+C	No	No	N	NP
7	F	58	52	Ataxic gait	No	No	NP	A	No	No	N	NP
8	F	37	22	Writing difficulties	No	No	NP	A	No	No	N	NP
9	M	31	15	Ataxic gait	Yes	No	P	A	No	No	N	NP
10	M	58	46	Ataxic gait	No	No	N	A+B+C	No	No	N	NP
11	F	55	50	Ataxic gait	No	No	P	A+B+C	Constipation	No	N	NP
12	M	23	7	Ataxic gait, dysarthria	No	No	NP	A+WM	Constipation	No	N	NP
13	F	59	57	Ataxic gait, dysarthria	No	No	N	A+B	No	No	N	NP
14	F	26	26	Ataxic gait	No	Yes	P	A	No	No	N	NP
15	M	64	49	Ataxic gait, dizziness	No	No	N	A+C	No	No	N	NP
16	M	55	8	Ataxic gait	No	Yes	NP	A	Colic pain, diarrhoea	No	N	NP
17	M	64	62	Ataxic gait	No	No	N	A+C	No	No	IgG AGA+	P
18	M	33	12	Dysarthria, ataxic gait	Yes	No	N	A+C	No	No	N	NP
19	F	34	27	Ataxic gait	No	No	P	A	No	No	IgG AGA+	P
20	M	48	43	Ataxic gait, dysphonia	Yes	No	N	A+C	No	No	N	NP
21	M	41	25	Dysarthria	No	Yes	P	A	Constipation	No	N	NP
22	M	62	39	Ataxic gait	No	Yes	NP	A+B+C	Constipation	No	N	NP
23	M	53	50	Unsteadiness, ataxic gait	No	Yes	P	A	No	No	N	NP
24	M	66	62	Weakness, tremor	No	Yes	P	A	No	No	N	NP

Pyramidal signs=Babinski’s sign or hyperreflexia plus spasticity; signs of peripheral neuropathy=absence or reduction of distal reflexes associated with reduced vibratory sense; NCS= nerve conduction studies
P=pathological; N=normal; NP=not performed; A=cerebellar atrophy; B=brainstem atrophy; C=cortical cerebral atrophy; WM=white matter abnormalities
AGA=antigliadin antibodies; EMA=antiendomysium antibodies.