Letter to the editor
Introduction
Patients and methods
Patient | Remote therapeutic history | Previous therapy before ITB placement | ||||
---|---|---|---|---|---|---|
1
| Trihexyphenidyl | 4 mg x3/day | Started in 2006 Discontinued in 2010 | S-adenosyl methionine | 400 mg x4/day | Started in 2000 |
Initial efficacy, progressively lost | ||||||
Discontinued 24/04/2013 | ||||||
2
| Risperidone | 6 mg /day | Started in 1998 Discontinued in 2011 | Levetiracetam | 250 mg x3/day | Started in 2002 |
No efficacy | ||||||
Weaned from 03/01/2014, discontinued 20/02/2014 | ||||||
Clonazepam | 1 mg /day | Started in 2002 | ||||
Partial efficacy on spasticity | ||||||
Weaned from 12/02/2014, discontinued 21/02/2014 | ||||||
Sertraline | 50 mg /day | Started in 2002 | ||||
Scarce efficacy | ||||||
Weaned from 08/02/2014, discontinued 22/02/2014 | ||||||
Zopiclone | 7.5 mg /day | Started in 2002 | ||||
Good efficacy on sleep improvement | ||||||
Weaned from 12/02/2014, discontinued 21/02/2014 | ||||||
3
| Enzyme replacement therapy | Intrathecal infusion of leukocytes (2/month) | Started in 1996 Discontinued in 2010 | Gabapentin | 400 mg x4/day | Started in May, 2013 |
No efficacy | ||||||
Weaned from 23/01/2014, discontinued 15/03/2014 | ||||||
Intrathecal infusion of mesenchymal stem cells | Single administration in 2012 |
Results
Patient | 1 | 2 | 3 | |
---|---|---|---|---|
Before ITB
|
Dystonia:
| 22 | 23 | 38 |
UDRS total
a
| ||||
Aggression
| Yes | No | Yes | |
Self-injury
b
: | ||||
Mouth/lip biting
| 2-3 | 2 | 1-2 | |
Finger biting
| 10 | 20 | 3-4 | |
Punching
| 10 | 0 | 2-3 | |
Restraint
| Permanent whole body restraint | Permanent finger protection | None | |
Sleep
c
: | ||||
Awakenings
| 7-8 | 5-6 | 5-6 | |
Sleep hours
| 2 | 4 | 4 | |
Date of ITB implantation and age (years)
| 29/04/2013 – 19 | 28/02/2014 – 39 | 19/03/2014 – 20 | |
ITB dosage and follow-up duration (months)
| 380 μg /day – 16 | 270 μg /day – 6 | 550 μg /day – 5 | |
After ITB
|
Dystonia:
| 5 | 6.5 | 11 |
UDRS total
a
| ||||
Aggression
| No | No | No | |
Self-injury
b
: | ||||
Mouth/lip biting
| 0-1 | 0 | 0 | |
Finger biting
| 3 | 5 | 0 | |
Punching
| 0 | 0 | 0 | |
Restraint
| Occasional, right arm | None | None | |
Sleep
c
: | ||||
Awakenings
| 1-2 | 1 | 0 | |
Sleep hours
| 5 | 6 | 5 | |
Notes
| Improved verbal communication | - | Persistent moderate nausea, daytime drowsiness |
Patient | Before ITB | After ITB |
---|---|---|
1
| Duration factor: 3 | Duration factor: 0 |
Motor severity factor: eyes and upper face: 1, lower face: 3, jaw and tongue: 2, larynx: 0, neck: 2, shoulder and proximal arm. 2, distal arm and hand (including elbow): 3, pelvis and proximal leg: 3, distal leg and foot (including knee): 2, trunk: 1. | Motor severity factor: eyes and upper face: 2, lower face: 1, jaw and tongue: 1, larynx: 0, neck: 1, shoulder and proximal arm 0, distal arm and hand (including elbow): 0, pelvis and proximal leg: 0, distal leg and foot (including knee): 0, trunk: 0. | |
Total = 22 | Total = 5 | |
2
| Duration factor: 4 | Duration factor: 0.5 |
Motor severity factor: eyes and upper face: 1, lower face: 4, jaw and tongue: 3, larynx: 0, neck: 4, shoulder and proximal arm. 4, distal arm and hand (including elbow): 4, pelvis and proximal leg: 4, distal leg and foot (including knee): 4, trunk: 1. | Motor severity factor: eyes and upper face: 1, lower face: 0, jaw and tongue: 1, larynx: 1, neck: 1, shoulder and proximal arm. 0, distal arm and hand (including elbow): 0, pelvis and proximal leg: 1, distal leg and foot (including knee): 0, trunk: 0. | |
Total = 23 | Total = 6.5 | |
3
| Duration factor: 4 | Duration factor: 1 |
Motor severity factor: eyes and upper face: 1, lower face: 4, jaw and tongue: 4, larynx: 0, neck: 3, shoulder and proximal arm. 3, distal arm and hand (including elbow): 2, pelvis and proximal leg: 3, distal leg and foot (including knee): 4, trunk: 0. | Motor severity factor: eyes and upper face: 1, lower face: 1, jaw and tongue: 2, larynx: 0, neck: 1, shoulder and proximal arm: 1, distal arm and hand (including elbow): 1, pelvis and proximal leg: 2, distal leg and foot (including knee): 1, trunk: 0. | |
Total = 38 | Total = 11 |