Table 2.

Comparison of patients with anti-Caspr1 IgG4 antibodies.

	Emilien Delmont (n = 2)	Andrea Cortese (n = 3)	Kathrin Doppler (n = 2)	Giuseppe Liberatore (n = 1)	Present report (n = 2)
Age at onset (year)	53 and 68	46 (24-57)	30 and 69	57	44 and 59
Male sex	2/2	2/3	1/2	0/1	1/2
Subacute onset	2/2	2/3	1/2	1/1	2/2
Symptoms at onset	NA	NA	Sensory deficits and severe pain, distal numbness	progressive proximal and distal motor involvement	Sensory deficits, severe pain with a cold sensation
Triggering infection/vaccination	0/0	1/3	2/2	NA	1/2
Muscle weakness	2/2	3/3	2/2	1/1	2/2
more severe in distal	0/0	3/3	1/2	NA	2/2
Sensory deficiency	2/2	3/3	2/2	1/1	2/2
Sensory ataxia	1/2	2/3	0/2	1/1	1/2
Tremor	0/2	1/3	0/2	1/1	0/2
Pain	1/2	0/5	2/2	0/1	2/2
Neuropathic pain accompanying cold sense	0/2	0/5	0/2	0/1	2/2
Respiratory failure	2/2	0/5	0/2	0/1	0/2
Cranial nerve involvement	2/2	1/3	0/2	NA	0/2
CSF protein (g/L)	12 and 2.5	4.26 (3.43–5.10)	5.2	NA	3.2 and 3.3
Impairment
ONLS (total)	10 and 5	8 (8-9)	NA	NA	5 and 5
MRC sum score	NA	NA	58/60 *	NA	56 and 56
NCS features
Prolonged DML	NA	3/3	2/2	NA	2/2
Reduced CV	NA	3/3	2/2	NA	2/2
Prolonged F wave	NA	1/3	2/2	NA	2/2
Conduction blocks	NA	1/3	2/2	NA	0/2
Temporal dispersion	NA	1/3	1/2	NA	0/0
Response to IVIG	0/2	2/3	0/1	0/1	0/1
Response to steroids	0/1	2/3	1/1	0/1	2/2
Response to PEX	0/1	0/1	1/2	0/1	0/0
Response to immune suppressors	0/0	1/1	1/1	0/1	0/0

CSF, cerebrospinal fluid; CV, conduction velocity; DML, distal motor latency; IVIg, intravenous immunoglobulins; NCS, nerve conduction study; PEX, plasma exchange. *: The MRC sum score of patient 2 was not found in Doppler’s article.

NA, not applicable.