Clinical symptoms and histopathological findings
| Neurological symptoms | Sural nerve biopsy | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Sensory involvement | ||||||||||||
| Patient Age/sex | Sjögren’s syndrome, positive test | Motor involvement Muscle atrophy/ strength | Distribution | Painful sensation | Nociception | Vibration/ position sense | Sensory ataxia | Romberg’s sign | Autonomic signs | Myelinated fibre density large, 9 small (no/mm2), Small/large ratio | Unmyelinated fibre density (no/mm2) | Follow up (y) |
| +, Positive findings; −, negative findings. Muscle weakness, atrophy, and painful sensation: +3, severe; +2, moderate; +1, mild; –, absent. Distribution of sensory involvement: F, facial; T, trunk; L, limbs. Sensory signs: −3, severe; −2, moderate; −1, mild; –, absent. Nociception was evaluated by pin pricking. Autonomic dysfunction: 1, Adie’s pupils; 2, loss of 123I-MIBG cardiac accumulation; 3, urinary disturbance; 4, hypohidrosis. SS-A, anti-SS-A antibody; SS-B, anti-SS-B antibody; RB, Rose Bengal test for Sjögren’s syndrome. For nerve biopsy findings, large >6.73 um; small <6.73 μm in fibre diameter; As for typical sensory ataxic neuropathy with Sjögren’s syndrome, large myelinated fibre density was 660 (714) (mean (SD)), small myelinated fibre density was 3263 (2390), and small/large fibre ratio was 14.6 (19.0) for 20 cases. ND, not determined. | ||||||||||||
| *,†Sensory ataxia developed in the legs 9 years later, in the right hand 11 years later respectively. | ||||||||||||
| ‡Result of second sural nerve biopsy examined 12 years later. | ||||||||||||
| 1 | SS-A | +1 / +1 | TL | +3 | −3 | −1 /− | – | – | 1, 2, 4 | axonal degeneration | 3463 | 6 |
| 72/F | SS-B | 1159, 921, 0.79 | ||||||||||
| Sialography | ||||||||||||
| 2 | SS-A | −/− | L | +2 | −3 | −1 / −1 | –* | –* | 4 | axonal degeneration | ND | 2 |
| 68/M | Lip biopsy | 922, 448, 0.49 | ||||||||||
| RB | 79, 169, 2.14‡ | |||||||||||
| 3 | Lip biopsy | −/− | TL | +3 | −3 | −/− | – | – | 3 | axonal degeneration | 2585 | 6 |
| 36/F | RB | 1876, 1332, 0.71 | ||||||||||
| 4 | SS-A | +1 /− | L | +3 | −3 | −2 / −2 | –† | – | 1, 2, 4 | axonal degeneration | 8312 | 11 |
| 71/F | Sialography | 1158, 1553, 1.34 | ||||||||||
| RB | ||||||||||||
| 5 | Lip biopsy | −/− | FL | +3 | −3 | −/− | – | – | – | axonal degeneration | 3232 | 11 |
| 48/F | Gum test | 2541, 1528, 0.62 | ||||||||||
| 6 | SS-A | −/− | FL | +3 | −3 | −/− | – | – | 1 | ND | ND | 6 |
| 25/F | RB | |||||||||||
| 7 | Lip biopsy | −/− | FL | +3 | −3 | −/− | – | – | – | ND | ND | 4 |
| 62/F | Schirmer’s test | |||||||||||
| Controls (mean (SD), n=9) | 3068 (294), | 5122 (438), 1.7 (0.2) | 29913 (3457) | |||||||||