Characteristics | Nodal/paranodal antibody | |||
NF155 | Pan-NF | CNTN1 | Caspr1 or CNTN1:Caspr1 complex | |
Clinical features | ||||
Neuropathy | Severe non-length-dependent sensorimotor neuropathy | |||
Age (median; years) | 51.5 | 57.4 | 60 | 57.5 |
Male:female | 1.6:1 | 3.5:1 | 3.3:1 | 2.3:1 |
Initial diagnosis GBS | ||||
Ataxia | ||||
Tremor | ||||
Pain | ||||
Cranial nerve palsies | ||||
Respiratory failure | ||||
Autonomic dysfunction | ||||
Nerve root abnormalities | ‡ | |||
Associated disorders | ||||
Nephrotic syndrome (MGN–CNTN1, FSGS–pan-NF) | § | † | ||
IgG lymphoproliferative disorder | ||||
CCPD | * | |||
Key | ||||
Absent | ||||
+ | <25% | |||
++ | 25%–50% | |||
+++ | 50%–75% | |||
++++ | 75%–100% |
Coloured boxes indicate frequency (as per key) of characteristic from our cohort.
*18 40
†7–10 12
‡17
§1
Caspr1, contactin-associated protein-1; CCPD, combined central and peripheral demyelination; CN, cranial nerve; CNTN1, contactin-1; FSGS, focal segmental glomerulosclerosis; GBS, Guillain-Barré syndrome; IgG, immunoglobulin G; MGN, membranous glomerulonephritis; NF155, neurofascin 155.