Chronic axial stiffness and spasm(stiff man syndrome) (n (%)) | Chronic stiffness and spasm of distal limb (stiff limb syndrome) (n (%)) | Progressive encephalomyelitis with rigidity (n (%)) | |
---|---|---|---|
Number | 8 | 13 | 2 |
Diabetes mellitus | 3/8 (37.5) | 0/13 (0) | 0/2 |
Presence of anti-GAD antibodies | 7/8 (88) | 2/13 (15) | 0/1 |
Other positive auto-antibody serology2-150 | 6/7 (86) | 5/13 (39) | 1/2 |
CSF protein >0.6 g/l | 1/8 (12.5) | 4/11 (36) | 1/2 |
CSF cell count >5 cm3 | 1/8 (12.5) | 1/11 (9) | 1/2 |
CSF oligoclonal bands | 4/7 (57) | 2/12 (17) | 1/2 |
MRI brain abnormality | 2/5 (40) | 1/12 (9) | 1/2 |
MRI spine abnormality | 0/5 (0) | 2/12 (18) | 0/2 |
Denervation on EMG | 0/5 (0) | 2/12 (17) | 1/2 |
Abnormal CMCT | 0/6 (0) | 1/8 (13) | ND |
SEP (lower limb) abnormality | 0/4 (0) | 1/8 (13) | ND |
Continuous motor unit activity: | |||
Back and abdomen | 8/8 (100) | 4 (31) | ND |
Proximal or distal limb | 4/8 (50) | 13 (100) | |
Abnormal cutaneomuscular reflexes from the lower limbs | 8/8 (100) | 10/12 (83) | ND |
Abnormal startle response | 4/6 (67) | 7/11 (63) | ND |
Abnormal interference pattern during spasms | 0/6 (0) | 9/12 (75) | ND |
↵2-150 For details of tests for autoantibodies see text.
ND=not done; n=the number of patients affected out of those tested.