From: Treatment of motor and behavioural symptoms in three Lesch-Nyhan patients with intrathecal baclofen
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 |