Table 1

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
Number8132
Sex (M:F)5:35:82:0
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 (%)):
Bulbar0021-152
 Back7 (88)1 (8)
 Limb1 (12)12 (92)
Preceding illness (n (%))05 (39)0
Relapsing remitting course (n (%))1 (12)7 (54)0
Brainstem involvement (n (%))1-150 05 (39)2
Upper limb involvement (n (%))1-150 010 (77)2
Sphincter involvement (n (%))1-150 07 (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 (%))013 (100)2
Number wheelchair bound (n (%))01-151 6 (46)11-153
Number died002
  • 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.