Summary of the clinical features of patients with adult onset predominant axial dystonia
| Patient | Age/sex | Onset age | Onset site | Predominant axial movements | Prior trauma | Drug regimen /response | Follow up (duration; remarks) |
|---|---|---|---|---|---|---|---|
| 1 | 52/M | 49 | Torticollis, back, | Axial flexion spasms | laminectomy | D, AC, NP-nil | > 5 y; suicidal, ECT, dystonia unchanged |
| 2 | 43/M | 39 | L arm, torticollis, | Lordotic and scoliotic | No | TT, NP -good, but SE | > 5 y; unchanged, died -no postmortem |
| 3 | 59/F | 53 | Back and shoulder | Action lateral flexion | Back sprain | Not known | > 3 y; unchanged |
| 4 | 49/M | 44 | Back and neck | Extensor spasms | No | Lith- good, Triple-good | > 4y; remission on treatment |
| 5 | 28/F | 21 | Torticollis, writing craniocervical | Lateral axial and hyperlordotic | RTA, neck injury whiplash | TT-good but SE, AC-good | 3 y; partial response to treatment |
| 6 | 39/F | 18 | Back and neck | Flexion spasms | No | Lith, AC, D- nil | > 5y; suicidal, depression, dystonia unchanged |
| 7 | 58/F | 54 | Torticollis and back | Flexion spasms | No | Triple-good | > 5y; greatly improved on treatment |
| 8 | 63/M | 57 | Cranio- cervical | Axial extension spasms | RTA | D, dopa, TT-nil | 5 year history of dystonia unchanged |
| 9 | 60/M | 50 | Arm, orofacial | Lordotic axial jerks, kyphosis | No | Triple-benefit | > 5 y; suicidal, dystonia unchanged |
| 10 | 72/M | 30 | Orofacial, neck | Flexion stoop | No | AC-SE | > 3 y; unchanged |
| 11 | 61/F | 30 | Orofacial, arm | Flexion jerks | Mandible fracture | AC, NL-nil | > 5y; depressed, recluse, dystonia unchanged |
| 12 | 55/M | 42 | Foot mild | Action flexion spasms | No | BC, AC-nil | > 5 y; dystonia unchanged |
| 13 | 63/F | 38 | Back | Action flexion spasms | No | Dopa-nil, TT, Ac and Dex-good | > 20y; improved on treatment |
| 14 | 51/M | 40 | Back | Flexion extension jerks | Laminectomy | Not known | Not known |
| 15 | 57/M | 56 | Back | Flexion spasms | No | TT, NP, dopa-nil, AC -good | 1 y; remitted on AC |
| 16 | 62/F | 60 | Back, neck | Flexion spasms | No | Triple-good | 1 y; pronounced improvement on treatment |
| 17 | 40/F | 39 | Back | Extensor spasms | No | NP and AC-nil, triple -good | > 5y; remission on triple, partial relapse later |
| 18 | 22/M | 17 | Neck | Extensor spasms | Neck injury | AC, D, dopa -nil, BT-nil | > 5y; unchanged |
Exam=examination; D=diazepam; AC=anticholinergic drugs; NP=neuroleptic drugs; TT=tetrabenzine; lith=lithium; dex=dexamphetamine
Triple=combination of tetrabenazine; artane, and pimozide; BT=botulinum toxin; SE=side effects; RTA=road traffic accident.