Table 1.
Demographic and skin biopsy data
| Medians (range) | CMT1A, n = 48 | CIDP, n = 7 | SFN, n = 16 | Controls, n = 45 | Ranksums, CMT1A–control |
|---|---|---|---|---|---|
| Age (years) | 47 (21–63) | 60 (43–76) | 51 (31–63) | 54 (22–64) | P < 0.05 |
| Sex (male:female) | 20:28 | 6:1 | 6:10 | 21:24 | – |
| CMTNSv2 | 18 (3–27) | – | – | – | – |
| IENFD (1/mm) | 3.7 (0.0–17.3) | 6.1 (2.7–7.5) | 5.85 (0.3–15.3) | 5 (0.8–24.9) | P < 0.01 |
| Merkel cell density (1/mm) | 0.50 (0–3.5) | 0.10 (0–6.5) | 1.05 (0–6.7) | 1.10 (0–11.1) | P < 0.05 |
| Fraction of denervated Merkel cells (CMT1A: 39, CIDP: 5, SFN: 12, control: 40) | 0.80 (0–1) | 0.30 (0–1) | 0.30 (0–0.7) | 0.20 (0–1) | P < 0.01 |
| Meissner corpuscle density (1/papilla) | 0.037 (0–0.154) | 0.029 (0–0.153) | 0.042 (0.012–0.137) | 0.053 (0–0.171) | P = 0.06 |
| Density of bundles with (1/mm2) (CMT1A: 46, CIDP: 7, SFN: 15, control: 41) | 4.7 (0–11.7) | 2.6 (0.5–5.3) | 1.9 (0–5.0) | 2.4 (0–12.2) | P < 0.01 |
| Density of bundles without (1/mm2) (CMT1A: 46, CIDP: 7, SFN: 15, control: 41) | 1.35 (0–6.3) | 1.60 (0–6.2) | 1.60 (0–5.1) | 1.30 (0–6.0) | P = 0.7 |
| Node numbers | 164 | 34 | 75 | 189 | – |
| Axonal diameter (µm) | 1.48 (0.56–3.7) | 1.38 (0.74–3.7) | 1.67 (0.56–3.7) | 1.48 (0.74–4.81) | P = 0.08 |
| Node length (µm) | 1.1 (0.37–2.96) | 1.5 (0.56–12.95) | 1.3 (0.56–4.26) | 1.3 (0.56–4.81) | P < 1 × 10−8 |
| Paranode length (µm) | 8.3 (3.1–16.6) | 9.7 (5.7–22.6) | 8.5 (4.8–47.9) | 9.1 (5.4–22.4) | P < 0.001 |
| Asymmetry index (%) | 14 (0–100) | 11.8 (0–44.8) | 11.1 (0–82.8) | 12.35 (0–100) | P = 0.5 |
| Ratio node length/diameter | 0.70 (0.2–2.2) | 0.84 (0.42–11.67) | 0.78 (0.2–3.33) | 0.80 (0.23–6.5) | P < 1 × 10−4 |
| Fraction of long nodes | 9.8% | 38.2% | 37.3% | 25.4% | Binomial test: P < 1 × 10−6 |
| Langerhans cell density (1/mm) (control: 44) | 10.0 (2.5–37.6) | 14.6 (8.3–28.5) | 8.4 (6.0–20.6) | 9.9 (1.3–131.2) | P = 0.4 |
Note: P-values smaller than 0.05 are bold.