Status dystonicus
| Reference | Age (years)/sex | Aetiology | Precipitating factor | Surgery | Delay in improvement | Outcome | F/U | Complications |
|---|---|---|---|---|---|---|---|---|
| Walcott et al123 n=3 | 14/M 9/F 9/F | Kernicterus perinatal/CP Generalised dystonia CP | Spinal surgery Unknown Upper respiratory illness | GPi bilat GPi bilat GPi bilat | Few days Few weeks Few weeks | + + (R side) + | 12 months 3 months 3 months | L lower contact partially working Infection (explantation R) None |
| Kovacs et al124 n=1 | 18/M | Tardive dystonia | No apparent | GPi bilat | Few days | BFM pre-op (mov/dis) 108/28 BFM post-op 2 weeks 42/18 BFM post-op 1 year 3.5/1 | 12 months | None |
| Grandas et al125 n=1 | 19/M | NBIA (PANK2) | No apparent | GPi bilat | Few days | BFM pre-op (mov/dis) 96/29 BFM post-op 9 months 10/4 | 9 months | None |
| Apetauerova et al116 n=2 | 16/M 26/M | CP CP | Surgery/metoclopramide surgery | GPi bilat GPi bilat | Few hours Few days | + (Returned to baseline functional status) + (Returned to baseline functional status) | 30 months 34 months | None None |
| Jech et al126 n=1 | 12/M | Generalised dystonia | No apparent | GPi bilat | Few weeks | BFM pre-op (mov) 41 BFM post-op 2 months 5 BFM post-op15 months 3 | 15 months | None |
| Elkay et al119 n=1 | 19/F | Batten's disease | No apparent | Pallidotomy GPi bilat | Few days | BFM (mov) off stim 100 BFM on stim 62 | 7 months | None |
| Mariotti et al122 n=1 | 15/M | NBIA (PANK2) | Recurrent infection (upper respiratory illness/pneumonia) | GPi bilat* | N/A | + No recurrence of status dystonicus | 12 months | None |
| Teive et al121 n=1 | 57/M | Generalised dystonia | Stress | GPi bilat | N/A | ++ | N/A | Mild L hemiparesis |
| Zorzi et al21 n=3 | 8.2/M 14.2/M 10.6/M | Generalised dystonia Encephalopathy unknown origin Generalised dystonia | Upper respiratory illness No apparent No apparent | GPi bilat GPi bilat GPi bilat | Few months Few days Few days | BFM pre-op (mov/dis) 91/20 BFM post-op at last F/U 83/19 BFM pre-op (mov/dis) 43/12 BFM post-op at last F/U 43/12 BFM pre-op (mov/dis) 79.5/19 BFM post-op at last F/U 63/10 | 15 months 15 months 19 months | Unpredictable switching off None† Unpredictable switching off |
| Angelini et al127 n=1 | 13/M | Unknown TH deficiency? | N/A | GPi bilat | Few days | + | 7 months | None |
*DBS performed some months after the status dystonicus and not in an emergency situation.
†Reimplantation procedure after an infection 2 years ago.
action, action subscore; BFM, Burke–Fahn–Marsden Dystonia Rating Scale; bilat, bilateral; CP, cerebral palsy; DBS, deep brain stimulation; dis, disability subscore; F, female; F/U, follow-up; funct, functional subscore; GPi, internal globus pallidum; L, left; M, male; mov, movement subscore; n, number of individuals in each study; N/A, not available; pre-op, preoperative state; post-op, postoperative state; R, right; stim, stimulation; TH, tyrosine hydroxylase; = no improvement; + = good outcome; ++ = excellent outcome (according to outcome described by the authors).