Table 1.
Demographic features of CIDP patients with and without anti-NF155 antibodies1
| All patients | NF155 antibody-negative CIDP | NF155 antibody-positive CIDP | P-value | |
|---|---|---|---|---|
| Demographics | N = 54 | N = 41 | N = 13 | |
| Sex ratio (male:female) | 38:16 | 30:11 | 8:5 | NS |
| Age at onset (age range) (years)2 | 42.4 ± 18.4 (13–76) | 47.9 ± 17.0 (13–76) | 25.2 ± 10.7 (13–50) | <0.0001 |
| Age at examination (years) | 44.5 ± 19.3 | 50.3 ± 17.6 | 26.2 ± 11.9 | <0.0001 |
| Follow-up period (months) | 70.1 ± 94.2 | 70.5 ± 89.3 | 69.1 ± 112.2 | NS |
| Clinical phenotype | n/N (%) | n/N (%) | n/N (%) | |
| Typical | 37/54 (68.5) | 30/41 (73.2) | 7/13 (53.8) | NS |
| DADS | 8/54 (14.8) | 2/41 (4.9) | 6/13 (46.2) | 0.0014 |
| MADSAM | 4/54 (7.4) | 4/41 (9.8) | 0/13 (0.0) | NS |
| Focal | 2/54 (3.7) | 2/41 (4.9) | 0/13 (0.0) | NS |
| Pure sensory | 2/54 (3.7) | 2/41 (4.9) | 0/13 (0.0) | NS |
| Pure motor | 1/54 (1.9) | 1/41 (2.4) | 0/13 (0.0) | NS |
| Hughes functional scale score | N = 54 | N = 41 | N = 13 | |
| At the peak of illness | 2.31 ± 0.91 | 2.22 ± 0.88 | 2.62 ± 0.96 | NS |
| At the last visit | 1.63 ± 0.90 | 1.54 ± 0.90 | 1.92 ± 0.86 | NS |
| Mode of onset | n/N (%) | n/N (%) | n/N (%) | |
| Acute | 0/54 (0.0) | 0/41 (0.0) | 0/13 (0.0) | NS |
| Subacute | 5/54 (9.3) | 4/41 (9.8) | 1/13 (7.7) | NS |
| Chronic | 49/54 (90.7) | 37/41 (90.2) | 12/13 (92.3) | NS |
| Symptoms and signs | n/N (%) | n/N (%) | n/N (%) | |
| Visual disturbance | 6/54 (11.1) | 3/41 (7.3) | 3/13 (23.1) | NS |
| Facial sensory disturbance | 10/54 (18.5) | 7/41 (17.1) | 3/13 (23.1) | NS |
| Facial palsy | 4/54 (7.4) | 2/41 (4.9) | 2/13 (15.4) | NS |
| Limb weakness | 52/54 (96.3) | 39/41 (95.1) | 13/13 (100) | NS |
| Muscle atrophy (UE) | 22/54 (40.7) | 19/41 (46.3) | 3/13 (23.1) | NS |
| Muscle atrophy (LE) | 24/54 (44.4) | 16/41 (39.0) | 8/13 (61.5) | NS |
| Drop foot | 22/54 (40.7) | 13/41 (31.7) | 9/13 (69.2) | 0.0242 |
| Gait disturbance | 43/54 (79.6) | 30/41 (73.2) | 13/13 (100) | 0.0484 |
| Cerebellar ataxia | 6/54 (11.1) | 4/41 (9.8) | 2/13 (15.4) | NS |
| Tremor | 15/54 (27.8) | 8/41 (19.5) | 7/13 (53.8) | 0.0300 |
| Disturbance of superficial sensation | 40/54 (74.1) | 32/41 (78.0) | 8/13 (61.5) | NS |
| Disturbance of deep sensation | 48/54 (88.9) | 35/41 (85.4) | 13/13 (100) | NS |
| Blood and CSF tests | n/N (%) | n/N (%) | n/N (%) | |
| Monoclonal protein | 3/49 (6.1) | 3/36 (8.3) | 0/13 (0.0) | NS |
| ANA ≥1:160 | 4/54 (7.4) | 2/41 (4.9) | 2/13 (15.4) | NS |
| CSF protein amounts (mg/dL) | 157.1 ± 132.9 | 103.8 ± 75.8 | 317.0 ± 141.1 | <0.0001 |
| CSF cell counts (/μL) | 3.2 ± 5.1 | 2.7 ± 5.5 | 4.9 ± 3.1 | NS |
| CSF albuminocytologic dissociation | 39/52 (75.0) | 32/39 (82.1) | 7/13 (53.8) | 0.0644 |
| Inflammatory demyelination on MRI3 | n/N (%) | n/N (%) | n/N (%) | |
| Brain lesions | 6/40 (15.0) | 3/31 (9.7) | 3/9 (33.3) | NS |
| Spinal cord lesions | 3/31 (9.7) | 3/24 (12.5) | 0/7 (0.0) | NS |
ANA, antinuclear antibodies; CIDP, chronic inflammatory demyelinating polyneuropathy; DADS, distal acquired demyelinating symmetric neuropathy; LE, lower extremities; MADSAM, multifocal acquired demyelinating sensory and motor neuropathy; n, number of positive patients; N, number of patients collated; NF, neurofascin; NS, not significant; UE, upper extremities; CSF, cerebrospinal fluid; CNS, central nervous system.
All continuous values are shown as mean ± SD.
In several CIDP patients with CNS lesions suggestive of demyelination, the ages at onset of peripheral neuropathy were used.
Conventional brain and spinal cord MRI studies were performed as described previously29 using a 1.5- or 3.0-T whole-body clinical imager (Achieva; Philips Healthcare).