Table 1.

Summary of clinical and serological data in eight patients with CASPR2 and/or VGKC-complex antibodies

Patient	Age, sex	Duration of disease at sampling	Onset, course	MRI	CSF	Extracerebellar involvement	Other autoimmune diseases	VGKC-complex Ab (pM)	CASPR2 Ab score in CBA	Staining on cerebellar slices
07–223	59, F	<3 months	Subacute, progressive	Mild cerebellar atrophy, no temporal lobe changes	Normal, OCB neg	Limbic encephalitis	Thyroid antibodies	757	4	Pos
06–26	50, F	<3 months	Subacute, progressive	Mild atrophy to severe atrophy	Normal, OCB not determined	None	Sarcoidosis (mediastinal adenopathies), ANA	<100	3	Pos
02–762	35, F	<3 months	Acute, full recovery	Normal	NA	NA	NA	<100	1	Neg
A383	54, M	5 years	Fast progressing	NA	NA	Yes	NA	<100	2.5	NA
A425	55, F	14 years	Subacute, progressive	Normal	Normal	None	Thyroid disease	<100	2	Pos
A327	58, F	9 years	Chronic, progressive	Normal	ND	None	No	<100	2	Neg
A386	59, F	8 years	Chronic, stable	ND	ND	None	No	<100	1	Neg
A220	68, F	9 years	Subacute, progressive	Small vessel disease	ND	Mild bradykinesia	No	<100	1	Pos
A201	76, F	NA	Acute/subacute	NA	NA	NA	NA	<100	1	NA

The serum samples were obtained either at presentation or after several years. The majority of patients had subacute onset with slowly progressive disease, except for two, who had acute onset with full recovery. Four additional patients had low positive antibodies against voltage-gated calcium channels (85–122 pM; nv <45 pM), two had VGKC-complex antibodies (186 pM, 168 pM) and one patient had high titres of GAD antibodies (4000 U/ml; nv <1 U/ml). Onconeural antibodies were negative in all, and tumours were not found. Sera 07–223, 06–26, 02–762 were from the Spanish ataxia cohort; all other sera were from the Welsh idiopathic ataxia cohort.

Ab, antibody; ANA, antinuclear antibody; CBA, cell-based assay; NA, not applicable; ND, not determined; Neg, negative; OCB, oligoclonal bands; Pos, positive.