Clinical features in stiff people
| Chronic axial stiffness and spasm (stiff man syndrome) | Chronic stiffness and spasms of distal limb (stiff limb syndrome) | Progressive encephalomyelitis with rigidity | |
|---|---|---|---|
| Number | 8 | 13 | 2 |
| Sex (M:F) | 5:3 | 5:8 | 2:0 |
| Age (y, mean (range)) | 36 (22–47) | 41 (18–70) | 57 |
| Duration (y, mean (range)) | 7.5 (1–14) | 6.2 (1–19) | 0.2 |
| Site of first symptom (n (%)): | |||
| Bulbar | 0 | 0 | 21-152 |
| Back | 7 (88) | 1 (8) | |
| Limb | 1 (12) | 12 (92) | |
| Preceding illness (n (%)) | 0 | 5 (39) | 0 |
| Relapsing remitting course (n (%)) | 1 (12) | 7 (54) | 0 |
| Brainstem involvement (n (%))1-150 | 0 | 5 (39) | 2 |
| Upper limb involvement (n (%))1-150 | 0 | 10 (77) | 2 |
| Sphincter involvement (n (%))1-150 | 0 | 7 (54) | 0/11-153 |
| Lumbar hyperlordosis (n (%)) | 7 (88) | 2 (15) | 0 |
| Truncal rigidity (n (%)) | 8 (100) | 2 (15) | 2 |
| Fixed posture of distal limb (n (%)) | 0 | 13 (100) | 2 |
| Number wheelchair bound (n (%)) | 01-151 | 6 (46) | 11-153 |
| Number died | 0 | 0 | 2 |
↵1-150 Either historically or on examination.
↵1-151 One patient with stiff man syndrome required a wheelchair for outdoor use.
↵1-152 Both these patients had bulbar symptoms initially but they were rapidly followed by limb involvement that dominated the clinical presentation of these patients.
↵1-153 The index case deteriorated so rapidly that no assessment of sphincter function was possible and the need for a wheelchair was not applicable.
Unless otherwise specified, the figures in parentheses refer to the number of patients affected out of the total with each condition.