Demographic, immunological, clinical and therapeutic data of anti-neurofascin-155-IgM-positive patients (in the order of antibody titres)
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | |
| Age at onset (years), gender | 37, male | 76, male | 40, male | 42, male | 22, female |
| Duration of disease | 5 months | 6 years | 36 years | 1.5 weeks | 1 year |
| Diagnosis | CIDP | Suspected CIDP (not fulfilling the INCAT/EFNS criteria) | CIDP | GBS | Suspected CIDP (not fulfilling the INCAT/EFNS criteria) |
| ELISA titre | 1:400 | 1:200 | 1:200 | 1:100 | 1:100 |
| Western blot | Positive | Positive | Positive | Positive | Negative |
| Clinical symptoms | Distal >proximal tetraparesis | Distal >proximal tetraparesis muscle atrophy, fasciculations | Distal >proximal tetraparesis, muscle atrophy, fasciculations hypesthesia 20 years after onset | Severe tetraparesis | Distal >proximal tetraparesis |
| Distal hypesthesia, neuropathic pain | Distal hypesthesia, neuropathic pain | Distal hypesthesia 20 years after onset | Hypesthesia, neuropathic pain | Distal hypesthesia, neuropathic pain | |
| Facial and oculomotor palsy disabling sensory ataxia | Sensory ataxia | Autonomic dysfunction, respiratory insufficiency | Urinary incontinence | ||
| Antecedent infections | None | None | None | Respiratory infection 2 weeks prior to onset | Exacerbation during infections |
| Course of disease | Acute onset, relapsing-remitting | Slowly progressive | Slowly progressive | Acute onset, monophasic | Acute onset, relapsing-remitting |
| Tremor | Action and postural tremor | Action tremor | Disabling postural tremor | Rest and postural tremor during recovery phase | None |
| CSF (normal values: protein <50 mg/dL, cells<5/µl) | Protein 834 mg/dL, 7 cells/µl | Protein 103.9 mg/dL, 2 cells/µl | 58 mg/dL, 1 cell/µl | Protein 37 mg/dL, 3 cells/µl | 31 mg/dL, 2 cells/µl |
| Nerve biopsy | Axonal neuropathy, vasculitic component | Discrete axonal neuropathy | Mixed neuropathy, vasculitic infiltrates | Not done | Axonal neuropathy |
| Nodal/paranodal architecture | Normal | Not done | Not done | Normal | Normal |
| IENFD (distal leg) | 4.9/mm (slightly reduced) | Not done | Not done | 5.6/mm (slightly reduced) | 8.9/mm (normal) |
| NCS, EMG | Spontaneous activity | No spontaneous activity | Spontaneous activity | Spontaneous activity | Spontaneous activity |
| ↑↑DML, ↑F wave latency, ↓F wave persistence, ↓CMAP and SNAP, ↓NCV | ↓CMAP and SNAP, normal DML and F waves, normal NCV | ||||
| Treatment response | Poor response to IVIG, good response to plasmapheresis and rituximab | Response to IVIG | Response to IVIG and plasma exchange | Response to IVIG and plasma exchange | No response to steroids, moderate response to IVIG |
CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; CMAP, compound muscle action potential; CSF, cerebrospinal fluid; DML, distal motor latency; EFNS, European Federation of Neurological Societies; GBS, Guillain-Barré syndrome; IENFD, intraepidermal nerve fibre density; INCAT, Inflammatory Neuropathy Cause And Treatment Group; IVIG, intravenous immunoglobulins; NCS, nerve conduction studies; NCV, nerve conduction velocity; PE, plasma exchange; SNAP, sensory nerve action potential.